In It For Bennett: Our Journey with Cystic Fibrosis

The Day We Waited 11 Years For...

2021-07-15T21:44:00.010-05:00

This was the moment we've waited his whole life for: On morning of July 1st, Bennett swallowed his first dose of Trikafta, the first medication approved by the FDA for children ages 5-12 that addresses the underlying cause of Bennett's specific type of cystic fibrosis.

Each pill costs about $270 each. Bennett literally swallows nearly $900 a day to keep himself healthy. In total, Trikafta costs about $300,000 a year.

Out of the 8 daily medications Bennett takes, this is the only one that is not prescribed to stop the symptoms of CF. This one is prescribed to actually stop CF itself.

Trikafta is no cure. But we have some real hope that this medicine will significantly slow down the progression of his disease.

Trikafta comes in a pill pack. It's the combination of three meds (think trifecta): ivacaftor, elexacaftor and tezacaftor. Bennett is instructed to take two pills in the morning. He takes an evening pill exactly 12 hours later. To absorb the medicine, he also has to eat something with fat in it. Bennett is expected to take this medication for the rest of his life.

The expectation that Bennett would be getting on Trikafta soon has been a family experience. We've spent lots of time talking about it and looking forward to it.

The night before Bennett's big day, Avonlea (age 7) spent an hour hiding in my bathroom to make Bennett a handmade "Trikafta" sign with which to surprise him the next morning.

We had known this day was coming for months, even years. I thought we were ready until a few nights before the medicine arrived. One night, at dinner during a conversation about our anticipation of Bennett getting on Trikafta, Avonlea reflectively lamented, "I'm going to miss Bennett not doing his Vest anymore."

It was such a sweet comment. She loves her brother dearly. His doing his CF treatments is all she has ever known since she was a baby.

I reassured her his treatments weren't going away, "oh, Bennett will still have to do his Vest. The medicine will help but he won't be able to stop his treatments and taking his medicines for a while."

Bennett abruptly put his fork down and curtly responded, "so this is a downgrade?!"

Part of me wanted to burst into laughter and tears at the same time. It was so funny to me that my Generation Z child was using computer technology language to articulate the impact of Trikafta to his treatment regimen. And part of me was so sad to think that, as exciting Trikafta is to all of us, the reality of the situation for Bennett would be that he would ultimately be adding *more* treatment to his already burdensome treatment regimen.

I reassured him that it wouldn't be like this forever. We don't yet know how Trikafta affects the body long-term. Doctors don't know yet what impact it will be to stop the medications prescribed for addressing the symptoms of CF. When is too early to withdraw meds? What's the impact if we take off this med versus that? Nobody yet knows, not even the researchers.

The night before Bennett was to take his first Trikafta pill, another important conversation came up. As we were saying prayers before bed like we always do, Bennett began to become emotional. He shared about his fears of taking Trikafta. He shared how he likes himself just as he is and how he didn't want to change. He shared how he was afraid of gaining weight, becoming "fat" and even losing his g-tube.

It occurred to me that even though I've wished CF away all his life, CF is all he knows. His body has CF and getting rid of CF is, in some way, risking getting rid of his body as he knows it. I had to really breathe deeply and accept that even in good gifts, there can be losses.

I tried sharing with him why it was ok. I wanted him to be ok. But found the conversation to be futile. He needed time to grief and nothing I said was going to speed that up.

I realized I needed to give him the control of this big change in his life. As much as I had waited for 11 years for him to start this medication, I told myself that we could wait a few days or weeks longer.

I said, "I want you to take Trikafta tomorrow because I think it will make you feel better. But I'm going to let you decide whether or not you want to take it. It's your body. If tomorrow isn't the day, let's wait. You take it when you're ready. It's up to you."

That seemed to help. Bennett seemed to appreciate the space to make the decision that felt right to him.

But, I was pretty quickly reminded that while Bennett is now a pre-teen and has grown up moments, he's still a child who is a sucker for candy. When I asked Bennett if he wanted me to get him a Snickers bar to take with his Trikafta, he quickly agreed tomorrow would be a great day to start.

And that was it! He was all in!

I first learned about the real possibility Bennett getting access to a medicine that could help him back in 2016 (see: http://www.initforbennett.com/2016/03/vlc-2016-state-of-science.html). Here we are, five years later, with that medicine in our hand.

I feel incredibly blessed by the gifts of science and the many blessings in forms of donations to the CF Foundation that came from people who care for Bennett and other people with cystic fibrosis. Thank you to everyone who helped us get to this point. The fight isn't over but we have a pretty powerful tool in the toolbox that we've never had before!

Goodbye Waco, Hello Dallas!

2021-06-29T11:50:00.001-05:00

The worldwide pandemic changed the world. Our world included.

The kids and I faired really well during a year of COVID. No one got sick and everyone was able to make it through a year of homeschooling while I worked from home (thanks to amazing neighborhood friends and a fantastic babysitter). But our time staying safe from COVID gave me an opportunity to recognize some unmet needs in our family during those months of isolation.

After careful consideration, in January, I began the process of moving the children and myself to Dallas, Texas - closer to Bennett's CF care, closer to more educational opportunities and closer to more family support.

I was pregnant with Bennett when we moved to Waco twelve years ago. We had expected the move to be temporary and it became permanent, which I loved. Waco is such a great place to live and raise a family. I am so very grateful for the many years and many friends we made in Waco. We have so many sweet memories.

Our move to Dallas is not too far from Waco, only two hours "up the road". We plan to visit as much as we can. The kids will continue to spend time with their dad there.

This season of change for our family goes beyond just moving. Bennett is currently getting access to Trikafta, the medication that we hope will change his life. And this Fall, Bennett is moving into his last year of middle school while Oliver begins a final year of junior high.

While we are feeling very positive about these changes, we know grief is also a real part of change. Loneliness has a way of settling as the newness of moving in fades away.

What keeps me grounded is knowing that I've felt God's prompting throughout the move. While I was initially hesitant to consider moving away, every curious step I have taken has been met with open doors and a sense of peace.

I've thought several times about Isaiah 43:19 where God says: "Behold, I am doing a new thing..."

I don't totally know what new thing God is doing in this season of our lives. But I do know, whatever God is doing, it is good.

Trikafta Approved!

2021-06-09T23:17:00.000-05:00

Joy! That is what our family feels today!!

This morning, with one eye open and the other still shut, I woke up, rolled over and opened my smartphone. I clicked a notification to find a link sent to me by another mother of a child with cystic fibrosis. My heart lept:

It's approved!!!!!!

A few minutes later, Bennett walked in my room. I was delighted to share with him the good news, "Hey buddy, your medicine has been approved!" He grinned and gave me a high five! It was the news we were waiting for.

Trikafta, the first medication approved by the FDA that benefits Bennett's age group and Bennett's genetic mutation by addressing the underlying cause of his cystic fibrosis, is now available for Bennett to take! We are now within weeks of starting this new potentially life-changing therapy.

If you've prayed for Bennett, walked for Bennett, raised money on behalf of Bennett and/or followed Bennett's blog, THANK YOU!!! It is our hope that you will celebrate this huge gift with us!! You are part of his story! You are part of *this* story!!

We have so much to be grateful for. Bennett is 11 years old and has stayed very healthy so far. His lung function currently sits at 89%. However, he has never had a cough or acquired a lung infection that required IV antibiotics. We hope the Trikafta will increase Bennett's lung function to 100%, which would be considered normal for any child.

Bennett is in the 40th percentile for his height but he struggles most with his weight. Despite a g-tube and daily tube feedings, Bennett's weight is in the 13th percentile (his doctors prefer his weight to be at 50 percentile). So, we hope Trikafta will help Bennett's GI system work more efficiently allowing him to keep the nutrients he takes in. And, if possible, we hope Trikafta might even allow him to get rid of the g-tube (something he's never lived a day in his life without). Additionally, we hope Trikafta will extend Bennett's life expectancy.

Trikafta, which has been approved for people ages 12+ since October 2019, affects every person differently. We don't know if it will work well for Bennett. There have been some side effects that have been difficult enough for some people that they've had to stop taking the medication. But clinical trials seem to indicate that children have tolerated the medicine well. So, we are very hopeful.

I wrote in my blog many years ago about what it feels like when the CF community rejoices at the approval of a drug for those with cystic fibrosis, but a drug in which your child cannot benefit. This was the case in 2012. There was so much excitement and so much hope. But, we couldn't benefit because Bennett didn't have the right gene mutation.

I am very aware that, today, there are around 10% of people with cystic fibrosis and their families for which today is a painful day. They are excited for the CF community. But they know their random genetics mean they must wait longer until they can benefit from a drug that will increase their life expectancy and give them relief from this cruel disease. They are on a clock, a race for a medication like Trikafta and each day that goes by is another day lost. This is also the case for so many CF families around the world who do not yet have this medication available to them in their country. For these families, I just want to say, as exciting as today is, there is a sadness I feel with you, as well. Your time is coming too and I will be anxiously waiting along with you, working to help us find a medication that benefits your child, your family member, you. We are in this together, as the CF Foundation's tagline says, "Until it's done."

Lastly, I want to thank the hard working people at Vertex and the Cystic Fibrosis Foundation who worked so hard to make this dream a reality. Bennett's story began many years before Bennett's born, before we knew we would become a part of the cystic fibrosis community. Thank you to all the scientists, investors and brilliant minds that set forth this venture philanthropy model of discovery to bring this medication forward. Thank you to those clinicians and clinical trial participants who tested this medication so we can feel confident about it's safety. Today's joy isn't only because Bennett has hope like never before. It's because we realize the gift we've been given through the work and sacrifice of so many before us.

We can't wait to share when Bennett receives Trikafta in hand. And we can't wait to see what happens. Trikafta is not a cure. CF is not going away. But it's hope that CF will have just a little less control over Bennett's life, and for that, we are eternally grateful!

Today is a very exciting day!!! We hope you will celebrate with us!! CHEERS!!!!

Emergency Preparedness and Cystic Fibrosis

2021-02-18T00:46:00.010-06:00

Texas is in the middle of a historic winter storm event right now. And I'm in the middle of Texas.

For many many Texans, tonight brings another day and night with no electricity and no heat. On the heels of no heat or electricity, there is an emerging water crisis. While the upcoming weather looks to return to our normal above freezing temperatures in the coming days, we are holding our collective breaths that our frozen pipes won't destroy our homes, businesses and schools. This is day 7 of historic low temperatures. We are weary. Lest we forget we're all in the middle of a COVID pandemic that has forced us to withdraw from our normal life for nearly a year.

The children and I are some of the luckiest ones having not lost heat and electricity during this Winter Storm. I can only assume our power is near a critical city infrastructure and the reason why we have continued to have heat and electricity so far. But I have gone most of the week fully expecting and prepared to lose electricity and a way to warm ourselves.

I wanted to share some of the ways I prepared for this winter storm and some of the ideas I've learned from our CF community about how to cope in an emergency while having cystic fibrosis. While this list won't likely help most of our CF community suffering in Texas right now, I hope it can help my future self and others who randomly find this blog at some point after googling "managing CF during crisis."

Here are ways to prepare for an emergency when considering cystic fibrosis:

* When there is no electricity and you can't do your percussion Vest, use hand Chest Percussion Therapy (CPT).
* Consider getting an battery-powered Afflovest percussion vest. Insurance will usually cover some portion of a new medical device every 5 years. This is a good investment in times like these.
* Keep a list of your medications and access to your medical record/hospital portal on your phone.
* In anticipation of an event, try to fill all prescription medications. Even better, ask your doctor to write a 90 day prescription so you never run out of medicine.
* Store up supplies overtime. While they take up closet space, they can also give you extra time to get supplies if something unexpected happens.
* Boil masks and nebulizers if electricity doesn't allow for baby bottle sterilization
* Remember hand hygiene is so important with CF. Keep baby wipes on hand.
* Consider asking your doctor for a prescription for an albuterol inhaler in case you can't use your nebulizer to do albuterol.
* The eRapid can be a battery powered way to do breathing treatments.
* In an emergency, if you don't have electricity and need to do your treatments from home, try going to your local fire station.
* Remember to reach out to your CF Clinic for help or just to let them know what you're dealing with. A CF social worker's job is to help find community resources to help during a crisis.
* Consider purchasing a generator for backup power at home.
* Use a power inverter to do nebulized medicines in the car.
* If on oxygen, arrange for extra oxygen tanks on hand.
* Create an emergency plan. This link offers some really great suggestions, as well as some good scenarios to think through when you're not in the midst of an emergency.
* Connect to your community. You don't have to fight CF alone.
* Let your utility company know you have a person with a medical condition in your home. Some utility companies will prioritize your home's access to electricity in a crisis, if they know ahead of time.
* Have a 3-day supply consisting of one gallon of water per person per day (or more!). Since electrolyte levels are important for people with CF, consider also having gatorade on hand as well.
* When you're in a difficult situation (emergency or not) and need help related to life with CF, call the CFF's Compass. They are there to help connect you to communicate organizations and resources.
* Read about how the CF Foundation suggests families with CF prepare for emergencies.
* Feeding tube feeds can be hard without electricity. Pumps have batteries but they need to be charged eventually. One electricity-free feeding tube Bennett loves and uses is the bFed bolus system.

Bennett uses the bFed bolus feeding tube system. This feeding bag works through gravity, rather than electricity. It can easily be hands-free with the addition of the "Free Arm." We really love not having to depend on electricity/batteries to do his feeding treatments.

A vaccine is amazing, but that's not the medicine I'm anxiously awaiting

2020-12-16T23:03:00.000-06:00

For nine months, the world has ached and longed for a vaccine for COVID19. It has been thrilling this last week to watch the distribution of a medicine that has the potential to change the world back to the way it's supposed to be. Along with so many others, I look forward to the day when I, too, can have access to this drug.

But, for me, the vaccine, as remarkable as it is, pales in comparison to the drug I'm waiting for.

The drug I'm waiting for is one I've waited for for over a decade. It's a drug that has the potential to change my son's world forever and to extend his life expectancy. The drug I'm waiting for is Trikafta.

Trikfata is the first ever drug to treat the underlying cause of cystic fibrosis to which Bennett will have access. It's not a cure but it's considered "a game changer."

Any day now, Vertex, the pharmaceutical company who makes Trikafta, is expected to submit paperwork to the FDA for approval for it's triple combination modulator therapy. At one point several months ago, Vertex said they expected to do so by end of Quarter 4. I've been on the countdown for months.

As of today, there are only 10 business days left in Quarter 4. (Do you see me pacing the floor?!)

No one outside of Vertex knows when they will submit their application to the FDA. COVID19 and other issues may force Vertex to wait to submit in 2021.

Even after they file with the FDA, it will likely be another few months before the FDA approves the drug giving us access. I have no choice but to be patient.

But knowing access to Trikafta is *so close* but still so far away can feel intolerable for me as a mother of a child with CF. Like a toddler given permission to have candy but forced to wait until it's unwrapped, waiting at this point feels excruciatingly difficult. Every day we wait is one more day Bennett is at risk for further lung damage.

It's bittersweet to listen to the stories of people with CF currently on Trikafta (it's already available for adults with CF with qualifying mutations). Their stories of weight gain, no more coughs, fewer hospitalizations, less treatments, before and after pictures are just amazing. It's super exciting. I want my child to experience it too.

Of course, nothing is for certain. Just like the COVID vaccine isn't 100%, neither are we guaranteed that Trikafta will work for Bennett. We won't know until we give it a good try. But all signs seem to point to this drug greatly benefit Bennett.

Bennett will still have CF and there are a lot of parts to CF that Trikafta won't be able to fix. But it will hopefully stop this progressive disease in its tracks, slowing it's progression and giving Bennett a better quality of life overall.

In March, I watched the world become preoccupied with social distancing, trying to stay well from a respiratory disease. The world began doing many of the things we in the CF community had been doing for years. I felt thankful to no longer feel so alone in it.

Ironically, I feel the same sense of solidarity. Maybe others are looking at the vaccine and I'm looking at Trikafta. But, either way, there's a sense that we're all in this together: each of us is patiently waiting for our own access to a medicine that offers us hope.

Top Takeaways from the 2020 North American Cystic Fibrosis Conference

2020-11-04T14:06:00.003-06:00

For the first time ever, the annual North American Cystic Fibrosis Conference was held virtually (due to the COVID19 pandemic) in October 2020.

Although one of the best parts of NACFC is getting to meet with clinicians, researchers and parents in the hallways between sessions (and sometimes adults with CF who come too), one of the hardest parts of NACFC is not being able to go to all of the great sessions going on simultaneously.

If there was a positive about an all virtual NACFC, it was that, since it was online, I had a chance to watch all of the great sessions I wanted to see. Content was streamlined this year and most sessions were provided OnDemand for several weeks for attendees. I've been told that a good portion of the sessions will be placed on the CFF's Youtube page in the next month for all to see.

While I missed getting to see people in person at NACFC, I loved being able to really soak up the information. I really loved all of the new topics that were discussed this year and those topics that moved forward. I am regularly in awe of the amazing work that happens each year around the world that is presented each October.

Without further adieu, let me present my top takeaways from the 2020 North American CF Conference:

1.) The CF Gut is getting some serious attention in the research and clinical space.

I really loved Plenary 3 this year about all of the new parts of CF Care that haven't yet been fully explored: nutrition, liver, pancreas and intestine.

Considering Bennett's CF has been more GI-related than lung-related, I am particularly thrilled that there is a new focus on understanding how CF works in the digestive system.

There was an excellent video shown during the plenary about the GI system, much like we've had for years about CF and the respiratory system (will post here as soon as it's released).

My favorite take away, however, was the comment that scientists believe dysmotility and dysbiosis in the GI system are the "bronchiectases" of the lung system. Basically, this means that scientists believe that dysmotility (muscles in digestive system don't work properly) and dysbiosis (microbial imbalance) in the CF GI system indicate CF-related damage, just as scientists believe bronchiectases in the lungs indicate CF-related damage. The great thing now is, upon knowing this, researchers will begin to understand these particular markers in the body so they can begin to address CF-related gut problems earlier.

2.) Addressing mental health in people with CF continues to be a priority, with a special awareness of those taking Elex/Tez/Iva.

I really liked hearing more about mental health in the CF community. It's clear that while Trikafta is a blessing to our community, for some people with CF, Trikafta has brought a type of existential crisis of sorts: mental health challenges not previously experienced.

There was a very interesting session regarding mental health for those taking Elex/Tez/Iva (brand name: Trikafta). What I learned is that there are real issues for some people on Trikafta...but so far, it seems those who indicate they are having more mental health problems on Elex/Tez/Iva are those who mental health problems previously existed and were likely exacerbated by the new Trikafta medicine.

This new insight felt reassuring to me as Bennett will hopefully soon have access to Trikafta and I have worried this med might negatively affect his mental health. Reassuringly for pediatric patients, most have not indicated new mental health on this med.

For those who are struggling with mental health on Trikafta, there are many clinical interventions that can be explored to improve mental health, such as changing doses. But this is an area that will continue to be researched, especially with regard to psychotropic drugs.

3.) COVID and Trikafta are a double duo of changes to CF Care in 2020.

The CF Community had two significant events that may have forever affected the trajectory of CF Care: FDA approval of Trikafta for people ages 12+ in 2019 and the COVID19 pandemic in 2020.FDA Approval for Trikafta forever changed CF Care in that it will make it so that most of the CF Community will be significantly more healthy over time and will need less intervention in the future to stay healthy.The COVID19 pandemic initiated widespread implementation of virtual CF visits that will likely continue in some form in the future.

The unfortunate thing about these two confounding events happening at nearly the same time is that, together, they will make data trend origins difficult to identify. However, there is some data between the two events that does show us that Trikafta is having a significant affect on people with CF.

Alex Elbert from the CFF Registry Team explained in the chart above: "Pulmonary exacerbations started a trend lower at the end of 2019, when nobody in the US heard about Sars-Cov-2 virus. The drop is a clear evidence of Trikafta effect on the pulmonary exacerbation rate. The data in 2020 will combine both effects." I thought that was pretty exciting to hear/see!

4.) There is an effort to make sure the diversity of the CF Community is fully appreciated in all areas of CF Care.

There was a great NACFC session called, "Culturally Competent Care" which addressed a variety of issues that clinicians treating CF should and are beginning to think about. For example, how to address unique needs of transgender patients (such as chest binding) and how to be mindful of systemic bias that exists within the healthcare system.

I thought the session was enlightening and really refreshing. One of my favorite quotes was from Susanne Muenzel who said, "Cystic Fibrosis is most prevalent in non-Hispanic White patients. However, as the US population becomes more racially & ethically diverse, minorities represent an increasing portion of patients."

I appreciated hearing the stories of Raeshaun (https://www.instagram.com/spirituallybreathing/) and Terry and Michele White (https://twitter.com/noaacf)

5.) There is movement on the Path to a Cure for whom modulators are not an option.

The first NACFC Plenary addressed the issue of curing CF beyond modulators.

There are three different ways currently being researched to do this: mRNA, DNA and gene editing. mRNA delivery would restore CFTR protein irrespective of mutation. The goal would be restore the CFTR activity to the secretory cells and ionocyte cells. mRNA and DNA therapy would likely be therapy that would need to be repeated (within months).

Gene editing would not likely need to be repeated. Gene editing would be focused in the basal cells and may be performed in either specific or mutation-independent manner.

"One we have developed methodologies that work in the airway, the goal is then to begin to apply them whe ther it's the pancreas, the GI tract or other affected organs." said presenter Brian R. Davis, PhD.

CF is an ideal candidate for genetic based therapies.

"The future is quite bright: what it holds is that the Foundation has set the path for evaluating several therapeutics, in parallel, in an effort to develop, in a very time effective manner, the most efficient and safe molecular therapeutic that can be effectively re-dosed." -presenter, Maria P. Limberis, PhD.

It is expected that a clinical trial for nonsense therapy will begin this year and a first mRNA therapy clinical trial is expected in 2021.

6.) We are learning more about how to better address the needs of people with CF-related Diabetes Care.

As patients with CF grow older, they acquire additional health complications. Diabetes is one of the most common co-morbidity in adult CF patients.

It is thought that modulators may change the course of CF-Related Diabetes disease for CF Patients, especially in those patients who might have early diabetes. However, we will have to wait to see what the data says.

Endocrinologist Marisa Desimone, MD shared in her excellent presentation, "It's important to remember that very few CF patients have a very truly normal glucose metabolism."

Desimone explained studies suggest there is a benefit to screening CF patients for CFRD as early as age 6.

Desimone went on to explain that the primary goals in management of CFRD is to improve weight gain, protein anabolism, pulmonary function and survival. These goals come before the goal of glucose control (which is one way managing CFRD is very different from Type 1 or Type 2 diabetes). She shared that CFRD complications in people with CF are primarily micro-vasular,

I also learned during this session that insulin is the only recommended treatment for CFRD and that some patients may only need treatment during acute illness.

I was really thrilled to learn that, in the same way CF Teams are beginning to recognize the value of having a GI doctor on the multidisciplinary CF team, there is an increased awareness to the value of having an endocrinologist on the CF team.

7.) Recent large-scale study shows CF Carriers may, in fact, affect your health.

In this recent study, researchers have shown to have detected an increase prevalence for 57 out of 59 CF-associated conditions. Just some of the conditions evaluated and found to be related were chronic sinusitis, infertility, bronchiectasis, NTM infections, etc.

CF conditions previously not associated with CF Carriers included constipation, Type 1 or secondary diabetes, short stature, failure to thrive and scoliosis.

The two conditions that CF Carriers did not see odds-ratio increased were: osteoporosis and cirrhosis.

In the picture above, all 59 conditions evaluated are listed. The blue dots indicate conditions that are increased odds-ratio for people with CF. Red dots indicate conditions that are increased odds-ratio for CF Carriers. As demonstrated in the above image, while people with CF had a higher odds-ratio of these conditions, CF Carriers also had an increased odds-ratio.

Morbidity could impact large numbers of people given that there are greater than 10 million CF Carriers in the US alone. The burden of disease related to CF Carrier status may be sufficient enough to consider it it's own well-recognized but less severe disease. "In a way being a CF carrier, at least for the population may raise the possibly of increased risk of CF-related conditions," explained University of Iowa CF Director, Douglas B. Hornick, M.D.

Dr. Hornick says, "Perhaps, it may be time [for genetic counselors] to edit or rethink the recommendation [to CF Carriers] by saying, 'CF may affect your health', rather than does not or categorically does not affect your health."

Lego to Bennett's 11th Birthday!

2020-10-01T17:05:00.005-05:00

Bennett turned 11 on September 30. Since this was Bennett's pandemic birthday (seems like everybody had at least one of those!), we decided to hold a small birthday party outside.

Bennett chose to have a Lego-themed birthday. It's one of his very favorite things to play with.

Party favors included lego-shaped gummies in lego-shaped plastic banks and little bags of lego-shaped hard candy.

Opening presents

Being very gentle when sister presents her present to him.

Make a wish!

If there is a picture to capture Bennett, this just might be it. Bennett loves to make others laugh and never takes himself too seriously. He is pure joy. Happy 11th Bennett!

Did you hear that, Pongo!? Avonlea turned 7!

2020-09-27T15:15:00.009-05:00

These pictures are about two months late but they are too sweet not to share. The baby in our family, Avonlea, turned 7 mid-July. We love birthday party themes so this year's theme was 101 Dalmatians.

Avonlea's birthday couldn't have come at a better time. We were 5 months in to the pandemic and we all needed something to look forward to. I was concerned about keeping our family safe from COVID so we decided to invite our neighbors and have a socially distanced birthday party in our front yard with a pinata. I feel pretty safe from COVID outdoors so it made the party much more enjoyable knowing it was unlikely anyone would get sick.

Avonlea is growing up very quickly. She brings our family such joy. Avonlea is insightful, playful, thoughtful and compassionate. She loves singing and playing with dolls. In order to build up the fun of her upcoming birthday, I suggested we find an dalmatians outfit to go with her birthday theme. I offered to buy her a matching bow just for fun. She requested two bows. She explained she had very clear plans for her hair on the day of her birthday party: she wanted to wear it in two ponytails to look like two dog ears. Her creativity is always delightful.

While we look forward next year when we can invite more friends to celebrate Avonlea's birthday and can have the birthday party inside without the risk of COVID, I was very pleased to be reminded that small celebrations can bring big belly laughs and meaningful moments that will last a lifetime. Happy 7th Birthday, Avonlea!!

Hair in my face, hanging upside down but still holding on

2020-08-10T16:28:00.006-05:00

Up until almost this very moment, I've been single-parenting alone for nearly 6 months without a break. Things changed for the first time this week and it's been a welcomed respite. Parenting 3 children (including one with a chronic health condition) alone 24/7 for months on end in the middle of a world-wide pandemic has taken every bit of my focus. We have had very little face-to-face contact with the outside world and the little contact we have had has been short lived, outdoors in the Texas heat.

I've wanted to blog so many times. I've had so much to say. But sleep, messes and peanut butter and jelly sandwiches have needed my attention more.

Now that I have time to write, I realize there's no organization to my thoughts. I want to pour them out onto the computer screen like pouring a bucket of random Legos onto the floor. Pour them out, assess them, and then try to figure out what to make of them.

From time to time, the blog has become particularly quiet. Sometimes it's been because Bennett's CF has been well-managed and things are peaceful here.

Other times the blog has been quiet because I've been too busy gripping the handle bars as life flung me around a sharp curve or catapulted me into a deep dive before shooting me straight back up again.

In these moments, I just wish I could get off the ride. I'm tired of the "ups and downs" and "around and arounds." I was really hoping I'd be in for a quieter more pleasurable ride, like a ride in a canoe or something. I wanted beautiful views, not whiplash.

But this ride called "Life" doesn't ask you what you want. It just says, "hold on! here we go!"

When I think of how much our life changed with cystic fibrosis, I can look back and say, "wow, that's not the trip I would have planned but I do see such sweet parts of it." In the same way, I am trying these days to pretend like I can "look back" to my life right now as a working single mom separated from friends and family due to COVID19 living in an uncertain world and about to start homeschooling and say, "oh, that's so sweet." But it's hard to have 20/20 vision when you're just trying to keep your eyelids open.

I don't know where this ride is taking me right now. Where will life have me in a couple of years? I'm confident more now than ever before that I have completely no idea.

Exiting the car is not an option. So, I'm making sure the seatbelt is secure. I'm reminding myself outloud that God is in control and that I'm not in a freefall.

Although I can often feel as through I'm suspended in the air - my hair whipping my face, unable to see where I'm going - I know that the truth is, I can lean back into the hard seat, let out a scream and "enjoy" (er, accept) this part of the ride.

Upside down won't last forever.

16 Weeks of Living with CF during COVID19

2020-06-29T16:52:00.002-05:00

As of today, we've been quarantined for almost 4 months. So, I thought I'd share a little bit about how our family has been coping with CF during the COVID19 pandemic and our efforts to "re-open" in the community.

For the majority of our time in quarantine, we've been completely self-quarantined. The CDC has listed cystic fibrosis as one of the diseases that puts a person at high risk for complications of COVID19. It's still too early to know how exactly COVID19 affects people with CF.

At our most strictest quarantine, we never got within 6 feet of others, got our groceries by delivery only and held playdates online. However, after many weeks in self-quarantine, I began to feel more comfortable considering Waco's COVID numbers were consistently much lower than in other parts of the US. (For many weeks, we had no more than 5 new cases a day, pretty impressive for a county of 250,000). As summer approached and I listened to the needs of the children, I began to consider that possibly this moment was the best time (if ever) that we should start thinking about going out and connecting with people again.

The moment I decided to "loosen our belt" really crystalized when Bennett's CF doctor, who was doing a telehealth visit with us at the end of May, suggested that I consider bringing Bennett in to the CF clinic at the end of the summer to do X-rays, PFTs and bloodwork. She said, "you can do it all at once, right before you have to lockdown again during flu season." While I agree, my initial thought was: "Oh, wow. How can we "lock down again" if we've never unlocked ourselves to begin with?"

So, about 4 weeks ago, I started giving serious consideration to restarting hair cuts, dentist appointments and outdoor playdates. Every decision has been agonizing - deciding what is "safe" and "not safe." Safe is very relative. What is worth the risk of my child's health?

I've given up the idea that we can have good mental health and experience completely no COVID risk. At this point, I'm focused more on mitigating the risk and making any risk as low as possible.

Thankfully, deciding to get haircuts for the children was much easier to make when the kid's hairdresser (whom we love) agreed to do the children's hair outside while wearing a mask. In 100 degree heat, that's was a pretty big ask. I'm grateful for a community around us who knows about Bennett's disease and is willing to whatever it takes to keep Bennett safe.

Studies show that ones risk for COVID goes up 19X indoors. So, I've tried to make sure we stay outdoors whenever we can.

Oliver's orthodontist and the kids' upcoming dentist appointments were harder to decide to do. But the flip side of waiting out COVID is possibly having braces on longer (which no one should have to endure) and having tooth decay.

COVID numbers in Texas started to radically rise about a week ago. In one way, it's been scary to feel that COVID is out of control in our state and community. But, in another way, it's meant no difference for us. Behaviorally, we've been acting as though it's been out of control for a while. And we'll continue to, even after the crisis subsides. I'm grateful that a local law requiring masks was recent enacted. It has given me the greatest amount of peace at this time.

While we aren't going out of the house much, there are things we've found to do that are safe. Outside feels freeing. But, in Texas, the heat is so oppressive right now.

We've been thankful for water toys, ice cream and neighbors who have given us access to their pool when they are out of own. Last week, we enjoyed horseback riding together (http://www.brazosbluffsstables.com/guided-trail-ride/). And, while we couldn't travel to Florida this summer as we had planned, we snuck away for a "COVID19-safe vacation" to an empty home in Louisiana for new scenery.

I'm as anxious as the whole world is for this virus to be over. I dream of shopping down aisles in a store again, enjoying friends at dinner in a restaurant and no longer feeling anxiety when I get close to people. There are so many things to miss right now.

I'm afraid our personal quarantine could last at least through next Spring. And that feels very very long. But I'm trying to manage these days by focusing only on the day ahead.

Each day has its hidden gifts, and I'm trying to find and savor them. COVID19, I've found, is full of opportunities to learn resilience, to be reminded that God is in control and to practice gratitude.

Mother's Day

2020-05-13T02:19:00.002-05:00

This Mother's Day blog post was delayed because of...well, caring for my kids. But, even though it's three days later, I've decided to post it anyway.

I had a really lovely quarantined Mother's Day. It was a mix of delight, intention and surprise. It was a completely different one from last year.

One year ago this week, I sat on the floor, alongside Brian, and shared with our children that their father and I had made the decision to end our marriage. It was a complete surprise to the children. Telling them was as painful as I imagined it would be.

This entire last year has been filled with firsts. Alongside each first came unexpected and, at times, paralyzing grief.

While I haven't written publicly about these experiences out of a respect for others' privacy, I have most certainly wanted to. I desire to be authentic in all areas of my life. And, for me, writing is healing.

But I felt it prudent to spend the last twelve months quietly healing with the help of therapy, my faith and the tender care of close friends and family. Our divorce was finalized last month.

What I can share now is that hope is reemerging once again. Grief is less evident than it once was. We are at a new normal and everyone is doing well.

Mother's Day this year was my first as a single mother. And while it was one I had never intended to experience, it was full of blessings.

My sweet boys, Oliver (age 12) and Bennett (age 10), helped me clean the kitchen before we went to bed the night before so that I could wake up to a clean kitchen on Mother's Day morning. Six year old Avonlea, in full delight, gave me a manicure and pedicure. And all day she kept telling me how much she wanted me to have the best day ever.

There were other surprises as well. Neighbors who regularly check on me made sure I felt loved and seen. Friends near and far called or texted to let me know they were thinking of me. I was very overwhelmed by kindness.

The most memorable part of Mother's Day this year, though, was reading four little words that Bennett (who has dyslexia) wrote inside my Mother's Day card:

"I know your trying."

I wanted to simultaneously laugh and cry when I read those words.

I could have interpreted his words as disparaging, a sign of my failure as a mother. But I knew the opposite was true. He meant those words to be affirming.

"I know you're trying."

My children have heard me say the phrase "I'm sorry I'm trying" to them many times: when I've burnt dinner, when I've forgotten to follow through with something I promised I would, when I felt stressed and needed to apologize for my behavior.

I'm a mother. I'm also human.

I do a lot of reminding my children that I'm human. I want to give them permission to be human too.

Our job on earth isn't to be perfect, it is to be our whole ourselves, imperfections and all.

When my children see my brokenness, I want them to see Jesus in me. He is who makes things right.

To hear my child accept my fallibility gives me great joy. It takes away the pressure for me to be supermom or to have everything figured out. It gives me space for me to just be me, just a human who has been given the awesome and sacred opportunity to walk alongside the journey of three little beings as they figure out their way in the world. Sometimes, on this journey, I'm able to protect them from the hurts of the world. Sometimes I'm not. But at all times I am able to trust them to a God who cares for them even more than I do.

As I kept reading my Mother's Day card, I noticed that right below Bennett's words "I know you're trying" were three words that Avonlea added to them: "I love you."

I can't think of a better Mother's Day gift than to be reminded that my kids accept me for who I am and love me anyway,

Happy Mother's Day, fellow Mamas. "I know you're trying" too.

Not just the prison guard...but also, the prisoner

2020-04-02T15:00:00.002-05:00

Everything hit me all at once last Friday. Feelings of depression and fears of panic. "What's the point in getting up today?" I thought as I pulled my covers off and tried to start my day.

I was on week 2 of full isolation, having seen no one and having gone no where. "I can't keep doing this with no end in sight," I said to myself.

My days were bleeding into one another. Every day felt like the one before it. The color was draining from them.

COVID19 had obliterated my calendar, manipulated my routines, and separated me from many of the things that motivate me each morning. Left behind in the rubble was only the promise of a quarantined life inside my home with children at my feet all day and an unknown future in a world that will never be the same.

There are a lot of things I've had to give up since choosing to self-isolate on behalf of Bennett during this pandemic. But, being a big supporter of mental health, one thing I haven't given up is meeting with my therapist.

Thankfully, on the same day I woke up feeling the weight of how my life has forever changed, I climbed in to my SUV in the garage (currently the only place I can cry without my children hearing me) and fired up my laptop in time to see my therapists face through her online video portal.

I cried throughout my session as I explained to my therapist how trapped I felt. Not only was I grieving the stress I had recently undertaken by self-isolating but I was grieving the fact that I had come to accept that our situation won't end for a really long time. However long it takes for the world to feel safe enough to venture back out again, it will take even longer for those of us with family members with health conditions.

My therapist asked how I was caring for myself in the midst of being quarantined. I shared how I hadn't been very focused on myself. Instead, I was focused on taking care of everyone else: making sure we had stored up enough food, protecting the children from accidental interactions with neighbors while outside, making sure the children were not traumatized in this process, making sure I could work, making sure I could do school with them each day, making sure the bills were paid.

I admitted that I had not thought about my own needs during this time. I had not thought about how to fill up my own bucket. I had not thought about relaxing. I had not thought about how to center myself and calm my own heart.

My therapist asked me what I do to relax and I told her how creativity is often how I find myself. "But," I explained, "it's really hard to be creative when you're stressed." My therapist nodded and then wondered aloud: maybe the reason I wasn't giving myself time to do art right now is because it is precisely in those quiet moments that I might start to feel overwhelming feelings. If I can stay busy, I can keep scary feelings at bay.

Then my therapist said, "I think you should remember. You are not just the prison guard - monitoring who and what comes in and who and what goes out, taking care of the prisoners by preparing food and cleaning up. It's important to remember that you too are a prisoner in this."

"What do prisoners do all day to get through their sentence?" she asked rhetorically. "They read. They take classes. They pick up a new hobby."

The image of a lone prisoner inside their jail cell crocheting popped in to my head. It was a terrible and beautiful image. There's tension there: a prisoner doing art.

A prisoner doing art is someone who has accepted their reality and decided to make the most of it.

I felt a sense of peace at the thought of focusing on the beauty and gifts that can be found inside a prison cell, rather than focusing on the prison cell walls themselves that keep me away from beauty and the gifts on the outside. It's a small change of perspective. But it was the very one I needed as I was beginning week 3 of self-isolation, knowing I very well have months more before my family will likely return to life as normal.

In that moment I resolved that I can't just focus on surviving. I have to focus on thriving.

Everything has changed. It's scary and I'm afraid just like everyone else. But there is a lot of opportunity for beauty in this moment in my life. I've been given a gift of time. I've been given a gift of discovering more about myself. I've been given a gift of trusting God. I've been given a gift of getting to know my children on a deeper level. I've been given a gift to connect to friends I don't usually have time to connect to. There are so many tissue flowers I can stick up on my prison cell bars, if I decide to do so.

Instead of feeling trapped, I want to feel secure. Instead of feeling loss, I want to feel gain. The world isn't going on without me. My world is where I am.

Tomorrow starts 4 weeks on self-isolation for my family. I am still having moments of waking up at 4am for no reason and not being able to go to sleep. I'm still having feelings of overwhelm and helplessness. But I now feel more capable of what to do in those moments.

Instead of focusing on what I've lost, I'm focusing on what I have.

How We're Doing CF Care At Home During COVID-19

2020-03-23T21:28:00.004-05:00

Bennett uses a home spirometer.

My family has been self-isolated in our home for nearly two weeks now, an effort to keep Bennett safe from COVID-19. And I am fairly confident it will be many more weeks, possibly months, before our family will be able to safely venture out again.

Besides being homebound and having to go back to homeschooling, one of the most significant changes that COVID-19 has brought to our life is the temporary halt to going to the CF Clinic in person for Bennett's routine care.

Thankfully, we just had a CF appointment a month ago and Bennett is doing well. But, depending on how long COVID-19 goes on, we could find ourselves, like many others, having a CF Clinic appointment from our living room.

Since the COVID-19 virus is overwhelming hospitals and risks the health of people with CF, the CF Foundation is advising people with CF to stay home, even for routine CF care visits. (CF doctors are currently working on providing routine CF care through telemedicine/telehealth.)

I'm not totally sure how telemedicine is going to work (how exactly does a doctor listen to your lungs through a computer, for example?). But I'm looking forward to trying it out. I most definitely want to keep Bennett well and *not* go to the hospital right now.

With that in mind, I've been trying to consider ways to help Bennett stay well at home during COVID-19 and to prepare for telehealth CF Clinic visits. Here's what I'm doing these days:

1.) We aren't slacking on CF care one bit. I am hopeful that Bennett won't get sick with COVID-19. But, just in case he does, I want to make sure his body is in the in optimal condition to fight off infection. Therefore, we are doing our CF treatments religiously and being home is allowing me to increase his g-tube feedings so increase his weight. I also vigilant to make sure Bennett is getting outside to run and play and use his lungs as much as possible.

2.) I'm tracking Bennett's daily symptoms using Folia. Folia, https://www.foliahealth.com/, a free app for caregivers of children with special needs, allows you to track anything you want. I track coughs, nausea, stool consistency, tummy aches, headaches and how adherent he is to his treatment regimen so that I can give a consistent report to my clinic about how he's doing. This is particularly helpful to have should Bennett get sick.

3.) I'm using a scale to capture Bennett's weight over time. I am weighing Bennett once a month just to make sure he's getting good nutrition while quarantined and not losing weight.

4.) I'm using at home spirometer to capture his PFTs. PFTs are typically taken at the CF Clinic every three months when well and more often when sick. Right now, I am using a home spirometer once a week to get some baseline data and to teach Bennett how to get used to a home spirometer which is slightly different than in the clinic. My goal is to have home PFT baseline data to use so I can compare should he get sick. I trust my CF Clinic's PFT machine more, but in absence of clinic PFT data, I appreciate having our home spirometer.

5.) I'm using a thermometer to check for fever as needed. Since we're fully isolated and I am not worried about Bennett getting COVID-19 right now, I am checking for fever only as needed. However, if he was being exposed to anyone coming or leaving the home, I would check his fever daily. We know a very early symptom of COVID-19 is fever.

6.) I will use our home oximeter to capture Bennett's oxygen levels if he gets sick. Low oxygen levels isn't typically a problem for Bennett. But an oximeter can be helpful when breathing issues arise. I will check Bennett's oxygen levels if he was to show signs of being sick.

7.) I have a stethoscope to listen to his lungs...but it's unlikely to ever be much of a benefit to me as one typically needs a trained ear to put it to good use. Nonetheless, I like having it. Maybe it just makes me feel good to know I could listen to his lungs if I wanted to. The reality is that in the worst case scenario, it might allow me to have a doctor friend listen to his lungs if I was really worried about something in the middle of the night. But, in general, I don't think it'll do much for me.

8.) I'm staying on top of medication and formula refills. Since the pandemic can make the most random things scarce quickly (think toilet paper), I try to stay on top of my medication refills for fear one morning I'll wake up and find one of Bennett's medicines is out of stock or on backorder.

9.) I'm keeping Miralax stocked. People with CF are at risk for bowel obstruction. In fact, it's probably the number one thing that could send Bennett to the ER so I always keep Miralax stocked and use it (under doctor's guidance) whenever I think he might be getting backed up.

10.) I change out Bennett's masks and nebulizer cups every three months. I typically change out Bennett's nebulizer cup and masks at each CF Clinic appointment. This time, I've written it on my calendar to change 3 months since our last appointment. And I've written another date 3 months from that to remind myself. I typically keep 2.5 days worth of masks and cup on hand because that's how many can fit in my sterilizer.

11.) I change out Bennett's g-tube button every six months. It's easy to forget to do but I try to remember to change Bennett's g-tube button two times a year so that it doesn't become an emergent situation.

12.) I'm stay connected to the CF community online. Staying connected to the CF community makes me feel much less alone and allows me to get quick answers to things without needing to bother my CF Clinic. If I need something that an expert, such as a doctor, needs to advise me on, typically the CF community will encourage me to ask my doctor. But often I just need a quick answer from someone who has been there before and the CF community is there 24/7.

13.) I'm staying connected to my CF Clinic. I know my CF Clinic is busy right now. I also know they will likely just get busier in the coming weeks. But that won't change my communication with them. I will continue to reach out to them via email or MyChart as I need and will continue to let them know of any changes I make at home. CF Care is a partnership and I want to make sure I'm being a full partner with my team in Bennett's CF Care, even if my team can't get back to me as quickly as they typically have in the past.

14.) I'm always looking for at home protocols for CF Care from other CF families. Sometimes other CF Clinics put out printed at-home protocols. I really appreciate being able to learn from how other CF Centers coach and support their patients. I try to share what my clinic does with other families. I'm always looking for good at-home resources.

Although these are things I'm doing, there are some things I can't do and things I don't know how the clinic with will do them outside of a hospital/clinic either:

I don't know how CF Clinic throat swabs will work with regard to CF telemedicine. But I'm hoping to eventually be able to throat swabs at home, especially since we're 2 hours away from our CF hospital. I suppose if we can do drive-through COVID-19 tests, drive through throat swabs could one-day be an option? I'll wait to hear from my clinic on that.

I also don't know how bloodwork will be done at home. Some people have talked about having home nurses do the bloodwork. But with COVID-19, I don't want to even risk that. So, we'll see. Typically Bennett doesn't need bloodwork but once a year so hopefully we'll be back in the clinic for that. The same goes for an Oral Glucose test (OGTT).

And, how about X-rays? X-rays will always require a clinic visit. Thankfully, we grabbed ours last month at clinic so we have a recent one completed. But, I'd probably opt for a small clinic or urgent care near home at this point if we had to get an x-ray...anything besides going to the hospital. In absence of sickness, x-rays are typically done annually for CF patients.

I'm curious if there are other things I've missed about doing CF care at home and CF Clinic via telehealth during COVID-19. If you know of anything I should be thinking of, please let me know! I love crowd-sourcing and learning from others!

Dear Teachers: Thoughts from an COVID-19 At-Risk Family Just Trying to Survive

2020-03-15T15:26:00.000-05:00

I received the first email from one of my children's teachers today regarding school work that will soon be sent home. The email was a "heads up, it's coming next week" email from Oliver's teacher, which I appreciated.

I replied to the email with thoughts from a mother dealing with a child with special needs during this national emergency.

I wanted to share it on my blog because I hope other teachers will read it. The point of the email: Educators, be compassionate with your expectations, our children and families are just trying to survive right now.

****

Dear Teacher,
One thing I wanted to share with you, and all educators, who are currently making lessons plans is right now is: please be mindful that although children are at home, many are under duress in the current situation. Adults' schedules have changed. Adults' financial situations may have changed. This naturally causes stress among the adults in the home and therefore is stressful to the children.

In our particular case, since Oliver's little brother's underlying respiratory condition cystic fibrosis means COVID-19 could be fatal to him, our family is now fully quarantined (and plan to be for at least 6 weeks). This means that we are having to ration food and supplies to make sure we can make it as long as possible without having to risk exposing Bennett to COVID-19. My children spent much of this morning just grieving - grieving over their Spring Break trip which was cancelled, grieving the loss of their normal schedules, grieving the loss of being able to see their friends and teachers at school and grieving loss of not being able go about life as normal.

Oliver will have no problem with any of the assignments you give him. I know he will welcome them from his favorite subject! But I would very much appreciate if you would share this sentiment with other teachers. Children in distress will struggle to learn and retain new information. We know this is brain science. So many of America's children are in distress right now.

It's also worth noting that, although children may not currently be in school, they are most certainly learning. These days they are getting a front row seat to learning "supply and demand," learning how viruses work in the body, learning about countries around the world and learning how pandemics affect economies, etc. There is so much learning happening, even in the absence of worksheets.

If teachers would be compassionate towards families in crisis and children in crisis when they send work home, it would be so greatly appreciated.

That said, we look forward to hearing from you and to the work you will send.

Thank you,

Breck Gamel

Figuring Out How to Keep Bennett Safe Through the Coronavirus

2020-03-11T09:37:00.001-05:00

It's been a hard to know when and how...but self-quarantining seems nearly impossible to escape at this point. Due to cystic fibrosis, Bennett is part of the at-risk population extremely vulnerable to the coronavirus. If Bennett were to get COVID-19, it would likely, at best, compromise his long term lung health and, at worst, risk his life. (Side note: I realize that most children are being mostly spared from the symptoms of this illness, but doctors are unclear whether or not children with CF will be affected; we can only assume they will be as they are highly vulnerable to respiratory issues)

Although Waco has yet to see any positive coronavirus tests yet, we know it's just a matter of time. Spring Break is this week. Baylor students and many in our city who have been traveling will return this weekend.

For this reason, we have made the very difficult decision to self-quarantine for a time. The plan is to mainly stay at home with a few outings here and there but to eventually stop going out completely, as appropriate.

Questions I've had to recently ask myself are: what does it mean to self-quarantine? Do we just not go out? Do we stop going to school? Do we stop having people over? How far is too far and how far is just right? There are no good answers. It's such a personal decision. Even CF doctors around the nation aren't consistent with their recommendations. Nobody really knows what it is best.

The decision to self-quarantine was a slow progression. It came last week with cancelling of a large conference that I was set to attend in 3 weeks. Then, I decided to cancel our Spring Break trip to the Alamo. On Monday, I decided to cancel our two nights in a hotel in San Antonio. On Tuesday, I decided to cancel all of our plans next week. I don't know what we'll do after that.

Although it's been difficult to know where to draw the line, each day seems to reassure me the line I'm drawing is the right one. It's getting worse out there as the virus spreads.

The goal is to keep Bennett well. Is it even possible to do that? I don't know. Is my goal of staying home until it passes too lofty? Possibly. Is it worth all the trouble to try? Yes, I think so, to the extent that I reasonably can.

One of the things that reassures me that self-quarantining could be valuable for Bennett (outside of the fact, I'm not really sure I have much choice, considering the alternative) is: we've done it before during flu season and it worked.

Here's the thing about the flu, though. We get our flu shot every year. We have tamiflu should we get it. We have neither of these options with the coronavirus. So, if self-quarantining helped with the flu, it's really our only shot with the coronavirus.

I don't really know what our self-quarantining will look like for us over time. But here are the things I've done to try to keep our family safe (on behalf of Bennett):

* Stock up on items to the extent it's reasonable (peanut butter, noodles, canned food, etc.).
* Filled all of Bennett's prescribed medicines for the month
* Make sure we have a working thermometer and cold/flu relief medicines
* Make sure Bennett has all of his durable medical equipment at home
* Stay home as much as possible; stop going out unless necessary
* No big groups, such as church, birthday parties or ballgames; eventually we'll limit even friends
* When able, have needed items delivered

Preparing for a self-quarantine is one thing. Experiencing it is another. It's very easy for everyone to get cabin fever and even to feel depressed. So here are some of the things I learned during our first quarantining experience that helped us cope being at home (and a few other tips I plan to put in to place this time around):

* Get emotional buy-in from everyone, kids included; explain the situation and make sure everyone is on board.
* Set a daily routine/rhythm so that everyone knows what to expect day
* Get up on time, get dressed and make your bed every morning, even if you don't go anywhere (it will make you feel good)
* Set a bedtime for everyone and stick to it; good sleep hygiene is really important to prevent depression
* Develop excitement in the family by finding things to look forward to each day (this is especially important with young children who want to know, "what are we going to do tomorrow?!"); try to make each day slightly different
* Give each family member as much space as possible during the day (it's easy for everyone to become irritable without appropriate space)
* Set a certain period of the day for quiet alone time (necessary for introverts)
* Set a certain period of the day for connection time (necessary for extroverts); ideas include: read a family book together as a family, put together a puzzle as a family or play a board game each night; make a fort in the living room
* To address kid boredom, pull out old toys or suggest playing with old toys in a new way
* Organize.a closet or drawer, kids love playing with new stuff or using old stuff for crafts; besides, at least when all of this is over you'll have a clean house!
* Encourage everyone to keep learning (play educational games online; learn how to play an instrument, how to cook, how to paint, etc. Youtube has instructional videos for all kinds of pasttimes; learning will keep everyone stimulated)
* Use videoconferencing to connect with people, especially the kids' friends
* Bury yourself in a book
* Enjoy the outdoors (there's no coronavirus in the woods)
* Get out safely; you don't even have to go anywhere! Get in the car, roll down the windows and just drive; the change of scenery can be very helpful
* Exercise daily; exercise is important for everyone so make it fun and regular
* At the beginning of the quarantine, create a list of everything you want to do. Come back to it once the newness wears off.

We don't know when this will end. I'm not even quite confidence it's fully gotten started. But I do know that, at at the end of the day, every person and every family should do whatever they feel most comfortable with doing for the people they love.

I know I don't really have much control in keeping Bennett well from a highly contagious respiratory virus that is rapidly affecting the world. Bennett may get the coronavirus despite my best attempt. But, it won't be for lack of trying. And that is what I need to know to bring me peace.

Infection control with COVID-19: What cystic fibrosis has taught me

2020-03-04T19:53:00.001-06:00

Social media and the news circuit is filled with almost hourly updates of the spread of COVID-19 in the US. It can be very scary for anyone, but especially for those of us who have loved ones in the most vulnerable populations.

COVID-19 scares me. But, let's be honest, so does everything when you have CF. The flu. Colds. Coughs. Sick people. Hospitals. Large groups of people. Flying. Pets. Dirt. Hot tubs. I've gotten very used to being scared of things I can't see: bacteria, viruses, illnesses. Those are kryptonite to those with cystic fibrosis.

When you live with CF, the common rhinovirus can take away your lung function and put you in the hospital, so you learn to live life with an elevated level of fear. You also learn very quickly ways to keep yourself safe.

I thought today how strange it feels that the rest of the world is now talking about "infection control," something so commonly talked about in the CF Community. Since people with CF can make other people with CF sick, "infection control" is super important. At the hospital, people with CF are restricted to stay in their rooms without special permission because of "infection control." People with CF can't even be in the same classroom at school because of "infection control."

As annoying as this is, when you have CF, I have found myself quite thankful for it today as I hear about COVID-19. While these things are not fail-safe, they are second nature for our family. For that reason, I thought I should share some of the things we do nearly daily to keep Bennett safe from respiratory viruses, in hopes others might feel comforted there are things they can do to keep themselves safe too.

Here are just some of the things we do in our family to keep Bennett safe from dangerous respiratory viruses:

1.) Wear a mask in the hospital (masks actually don't keep you safe but it does signal to others to keep their distance)
2.) Stay 6 feet apart from others who might get you sick
3.) Sanitize surfaces touched by a potentially sick person (Lysol wipes are our friend!)
4.) Stay out of hospitals/clinics/pharmacies at all costs
5.) Consider withdrawing from school when sickness is circling
6.) Drive, rather than fly
7.) Stay away from crowds
8.) While in the hospital, insist doctors and nurses wear gloves and a plastic gown when they care for you (we ask for them to wear masks during flu season)
9.) Don't be afraid to ask clinicians sanitize stethoscope or any other medical device before it's used on you
10.) Sanitize nebulizer cups and plastic breathing masks before treatments
11.) Increase breathing treatments and chest therapy when sick
12.) Prioritize sleep when susceptible to being sick
13.) Stay hydrated when susceptible to being sick
14.) Pay attention to good nutrition when susceptible to being sick
15.) Make sure meds are not missed
16.) Use good hand hygiene (I keep hand sanitizer on me all the time)
17.) Don't share toothbrushes, eating utensils or drinking cups; wash clothes, towels and sheets more frequently
18.) Don't shake hands, hug or kiss when sick
19.) Don't share common objects with those who are sick or might be sick (such as pens, door handles, computers)
20.) Refrain from being together in enclosed poorly ventilated places such as a car.
21.) Stay away from anyone coughing or sniffling and ask others to identify you as soon as they start feeling symptoms so you can stay away
22.) Quarantine sick family members to one room or part of the house

Here's what the CF Foundation recommends for infection control for people with CF. It is not written with COVID-19 in mind. https://www.cff.org/Care/Clinical-Care-Guidelines/Infection-Prevention-and-Control-Clinical-Care-Guidelines/Infection-Prevention-and-Control-Clinical-Care-Guidelines/

Also, here are some tips for those trying to stay well from any respiratory virus: https://www.cff.org/Life-With-CF/Daily-Life/Germs-and-Staying-Healthy/How-Can-You-Avoid-Germs/

The CF Foundation just put out an announcement about COVID-19 today. See here.

It is my desire for the healthcare and research community to learn more about infection control. While we know some about how viruses spread, there is a lot we don't know. I am very hopeful that this new spotlight on how respiratory viruses spread and how to keep yourself safe will bring insights and understandings that will benefit CF community. While I am confident COVID-19 will eventually be a thing of the past, the CF community will have to continue to think of infection control until there is a cure for CF.

Trikafta is what we're afta!

2019-10-22T08:49:00.001-05:00

Excitement in the CF Community is through the roof! Yesterday, the FDA approved Trikafta, a triple combination medication that is believed to eventually benefit 90% of people with cystic fibrosis! This is EXCITING NEWS!!!! This is no cure. But CF clinicians and patients both say this is a GAME CHANGER!!!

Adults in our CF community who have been in clinical trials with this drug have been talking for months about the significant changes they've seen personally. This drug, for many people, has meant fewer hospitalizations, increased lung function and healthier feelings overall. It's almost hard to wrap my brain around!

In order to benefit from Trikafta (pronounced "Try-Kaft-Uh"), the FDA says one must have two specific CF gene mutations: F508del and a nonsense mutation. Bennett has both.

The FDA also says one must be 12 years old.

Bennett just turned 10 last month.

This means that he will now need to wait two more years to benefit, unless the FDA approves the drug for his age group before then.

To give perspective on what that might look like - there are currently 3 other drugs already on the market for people with cystic fibrosis (none of which benefit Bennett), the latest medicine, Symdecko, was approved for adults ages 12+ in February 2018. It took more than a year, in June 2019 before it was approved in children ages 6-11. So, our wait could be a while.

So, we're in that gray space. The space of almost but not quite. GET EXCITED!!!!! But wait...

It means fewer hospitalizations, increased lung function, healthier feelings overall are to come for Bennett...but not yet.

As a mama, these dual feelings are like what one might feel like, if having waited in line for hours to get on a much anticipated roller coaster ride, the ride stopped working, just as it's finally time to get on it. It's elation met with disappointment. It's a muffled joy. It's hope deferred.

Sure, comfort comes in the form of reassuring thoughts: "they'll get the ride up and going. It will be your turn really soon." But the wait feels particularly difficult to endure now. The build-up so intense. Pinned up hope aches for a place to go. It's so close now. But, knowing it's not our turn just yet gives way to fears: could this thing I've waited for so long actually be illusive?

I have thought many times today and yesterday about a blog post I wrote several years ago when the first CF drug, Kalydeco, came out on the market. I felt a similar feeling then too. But, as I reminded myself then and do again today - rescue helicopters are going to come back for my loved one too. I just have to hold on and wait a bit longer.

To be honest, waiting can be a gift - because it gives me great empathy for the 10% of people with CF with two nonsense mutations who have NO drug available right now from which they benefit. They don't get the privilege of waiting for their son to turn 12 so they can have a medicine too.

I think of my friend and CF adult, Emily, who is fighting tooth and nail to move science forward faster, in time that she herself would benefit. Her work and the work of her nonprofit Emily's Entourage has given us all hope that one day, sooner than later, 100% of people with CF will have something that will slow down this awful disease. But until then, she painfully waits.

The CF Foundation continues to work on this as well, regularly reminding us: we won't stop until it's done. And the job isn't done until ALL people with CF have a drug that makes a meaningful difference on their health...and even still, until there is a cure.

So, while our family waits a little longer, we will know this is the same ache many still feel in the CF community. And while we wait, we will experience the anticipation of getting access to a bottle of hope called Trikafta.

Bennett moves his cart of medicines and machines to his room for bedtime treatments.

I tried to explain to Bennett last night news about this drug. I tried to manage his expectations, just as I try to manage my own. I explained that if this drug works, like people are saying it does, it might mean he wouldn't have to go to the hospital as often. It might mean he could stop some of his medicines at some point. He might even be able to stop doing his twice-daily Vest.

Doctors don't yet know what this drug will really be able to do for a person. Drugs affect people in different ways. And different clinicians are more or less comfortable testing a drug's potential. It will take a series of trials and errors to find out just how this drug will help Bennett, once he gets there. And researchers will begin studying the drug on a larger scale once more people have access.

Bennett asked if he would still have to do his pump. I told him I didn't know. But I hoped this new medicine might allow him to stop having to get so many tube feeds. He is very excited about the idea that anything, even remotely like a cure, might be within reach.

For now, we head in to flu season, praying for God's continued protection. And we will just keep doing his CF meds as normal.

We can't stop now. For Bennett, hope is on its way. Trikafta, we can't wait to get our hands on you!!!!!

**Note: To all those in the CF Community who have come before us, thank you. Thank you for your diligence in fundraising...thank you for putting a laser focus on drug development...thank you for continuing to persevere until a cure is found. We are a part of a larger story that has been happening long before we were here and it feels amazing to be able to experience this particular chapter of the story. Also, to our extended community who loves and supports Bennett, I hope you can take joy in this celebration as well! You have contributed financially and physically to see a cure for Bennett. It's happening, guys!!! So, thank you!

Double Digits!?!

2019-10-01T00:10:00.000-05:00

Today, Bennett turned double digits! Bennett is 10 years old! It's hard to wrap my brain around. He's halfway grown.

When I met him for the first time in the NICU, his body swollen and near death, I didn't have any idea the amazing adventure I would end up going on with him as his mother. Over hills and through dark caves, splashing across creeks and sliding down rocks, I would experience the pains and joys of 10 years of caring for Bennett with cystic fibrosis. Each adventure would reveal a jewel, a tiny gift from the the experience, a sliver revelation of who Bennett is inside. I recognize this adventure is not over. But, ten years in to it, I am incredibly grateful for the trip so far. How lucky I am to be his mother.

So, who is Bennett, at age 10? Bennett is playful, outgoing and loves to be with people. He very tender-hearted and sensitive to other's feelings. He lives in the moment and is always up for something exciting. Bennett is brave, so very brave. He is resilient and strong. Bennett is compliant and easy to please. He is wise. Bennett rarely complains. He is great friend to others and incredibly lovable. Bennett is passionate about superheros, has a weak spot for gummy bears, and loves surprises. In his free time, he enjoys playing with Lego men, singing to The Greatest Showman soundtrack with his sister and dressing up, especially if it makes you laugh.

Today, on Bennett's birthday, I decided to take the children to get flu shots. I hated that it fell on Bennett's birthday but I felt it was important enough for us to get the when we could. Bennett asked to be the first one to get his flu shot, as an example to his sister and to get it over with. He was a rockstar.

Ultimately, the whole crew got their flu shot...despite little sister's brief protest.

Avonlea felt a little better about the process of getting a flu shot today when she learned she could give her doll her own flu shot too. (I guess she reasoned:"if you can't beat 'em, join 'em.") She started by cleaning the area.

We're thankful for Hillcrest Pediatrics for being so kind to let us have a syringe so baby doll could get her shot....

...and a band aid that matched her mama's.

Tears all gone - just smiles and the confidence that we're doing everything we can to keep Bennett healthy!

After flu shots and errands, we celebrated Bennett with a Pizookie from BJ's. Here's to double digits, Bennett! May we find a cure for cystic fibrosis and, maybe one day, even celebrate you in the triple digits! Happy Birthday!!

Bluebonnet Pictures 2019

2019-04-13T11:19:00.000-05:00

One of the best parts of living in Texas comes every Spring when the bluebonnets and other Texas wildflowers pop up alongside the highway and in random fields. The beauty lasts for only a few weeks but when you see them, you know Spring has sprung!

It's a societal norm in Waco to drop off your children in a random field, place them among the flowers and to tell them to say "cheese". So, we did it this year just like we have almost every year since we've been in Waco. Here are this year's pictures and, if you keep scrolling, you'll see how much these nuggets have grown.

Oliver, age 11

Bennett, age 9

Avonlea, age 5

April 2019 (Bennett, 9; Avonlea, 5; Oliver, 11)

April 2015 (Oliver, 7; Avonlea, 21 months; Bennett, 5)

April 2014 (Oliver, 6; Avonlea, 9 months; Bennett, 4)

April 2013 (Bennett, 3; Oliver, 5)

April 2012 (Bennett, 2; Oliver, 4)

April 2010 (Oliver, 2; Bennett, 7 months)

An update on Bennett - March 2019

2019-03-01T17:53:00.000-06:00

Bennett had his quarterly CF appointment last week so I thought I'd share how he's doing health-wise. I also thought I'd share how he's doing all around:

First of all, Bennett has blueish green hair these days. It's starting to fade but after asking consistently for at least a year to dye his hair, I finally relented, shortly after family photos were taken. Santa brought Bennett semi-permanent hair dye. The directions said it would wash out in anywhere between 5-25 washes. What the directions didn't say is how often a 9 year old typically washes their hair when they don't want the dye to come out. Ha!

On one hand, I'm so glad I finally gave in to his very simple request. A friend of mine made the point that allowing him to change his hair color was a great way to give him control over his body, which I hadn't thought about but I agree.

On the other hand, I didn't realize that, in order to put the blueish green hair dye on blonde-ish brown hair, you have to bleach it first. Bleach: what a terrible thing for CF lungs to inhale! Nonetheless, I was already committed at this point when I realized it, so I grinned and beared my fears and went forth. I told him, though, I didn't imagine a time when we'd be doing this again. ;)

Last week, Bennett went to see his CF Team at Dallas Children's Hospital for his quarterly CF appointment. Bennett gained two pounds in 3 months so his GI doc was very happy. Gaining weight is very difficult for people with CF.

We are currently giving Bennett 3 tube feeds daily, that's over 1500 calories and he's eating on top of that, which is wonderful. His ability to gain weight is a good sign he is staying healthy. He's still low in BMI so he needs more calories and more weight gain. But, for now, he's at least on track with his growth.

Bennett's lung health is also looking good. His PFTs were 92%, down from 95%. But, considering that he hasn't gotten sick this winter so far, his Pulmonologist gave a good report and said his lungs sound good. Bennett's throat culture results shows he continues to be free of psuedomonas and MRSA, for now. We're starting back up nasal rinses and Flonase due to headaches, which may indicate sinus build up. But we are hoping we can hedge that off early.

Bennett is doing well in school. This picture shows him sitting next to the board he made to demonstrate his work in robotics. You can see he's wearing his feeding bag. Bennett is very open about his CF at school. He sorta has to be open, considering he requires a tube-feeding each day at lunch. We are grateful that the school is more than willing to help us daily keep him on track with his weight gain!

Oliver and Bennett (and most recently, Avonlea) started a new school this year and it's going super well. They are growing in incredible ways. Acton Academy is a bit of a blend of homeschooling and a gifted/talented program. Their school is very hands-on and self-directed, which requires a lot of motivation to learn on their part. Thankfully, since the school has designed the learning process to be so much fun, my kids have very little problem with motivation. In fact, they beg me to drop them off at school at the first possible moment and to pick them up at the very last moment of the day.

Their school year is split in to 7 different sessions. In addition to the core subjects of Math, Reading and Writing, each session at school has a project-based focus. During the first session, they focused on entrepreneurship. Their second session was focused on learning about Ancient Greek culture. Their third session was all about Robotics. Their current session is about Plants and Gardening (they were making plant cell models out of Play-doh earlier this week).

As naturally awesome as this session is, gardening at school has presented challenges for Bennett due to CF. Many of the bacteria, yeast, molds and allergies that Bennett's CF lungs are susceptible to getting live in soil and decaying things.

These bacteria, yeast, molds and allergies can easily get kicked up into the air when dirt is moved around. And Bennett can easily breathe in these tiny particles into his lungs, which can cause havoc in his body. If and when Bennett gets a bacteria in his lungs, it will likely require weeks on IV antibiotics and/or inhaled meds and may include weeks in the hospital. So, we are very hesitant to risk this happening.

I hated having to explain to the teachers (who are actually called Guides at Acton) that Brian and I believe there's no amount of playing in the dirt or near the dirt that is good for Bennett. Out of abundance of caution, we also don't let Bennett play outside when the lawn is being mowed, either.

Not all families with CF believe it's necessary to withdraw children's playtime in the dirt - "children with CF shouldn't live in a bubble" is often an argument - but, based on what we know about the risks, Brian and I feel more comfortable letting him learn a bit more hands-off during this session.

It's disappointing that Bennett can't play in the dirt. But, we reason that we work too hard to keep him well, to do something that we know has a risk to undermine all of the work we've done so far. I'm thankful that the kid's school partners with us and is willing to do whatever it takes to keep Bennett's lung health optimal for as long as possible.

The last thing I thought I'd share is that we just started Bennett in twice-weekly sports training class for 7-11 year olds to see if he can improve some of the issues that showed up on Bennett's most recent physical therapy assessment.

Although this new sports training class is an added expense and means "one more thing" to Bennett's week and our family's schedule, we are trying to focus on Bennett's long-term health goals and believe that doing so will, not only build his core muscles and improve his lung health, but also teach him that being physically active is caring for himself.

Thankfully, Bennett really enjoys working out and has made new friends. A new friend said to his mother other night about Bennett; "he's a lot of fun."

I'm so glad to have received a good report on Bennett's health and to breath a sigh of relief for another 3 months. Brian and I are incredibly proud of Bennett's resilience and his willingness to try new things!

Transition

2018-11-25T23:56:00.000-06:00

Things have been very peaceful with regard to Bennett's CF as of late. (Peaceful, as in 2 Vest treatments, 3 inhaled medications, 3 tube-feedings and 15+ pills on a daily basis...but you get the point. ;) )

Bennett's GI issues seem to be in check. And, despite my recent fears about potential lung issues, Bennett blew a FEV1 of 95 (out of 100), which seems to indicate he's doing well. Bennett is still in the 3rd percentile for BMI but gained .5 lb and .5 inches in 3 months. We are giving him 3 tube feeds a day so we hope we can increase his weight and height even more. I'm trying to figure out if we can increase it to 4 tube feeds a day without inconveniencing either one of us.

This morning, I sat down with Bennett to go through a worksheet with him. I had received a "adolescent transition worksheet" from a friend whose child is seen at Cincinnati Children's Hospital in Ohio. I learned about this worksheet while in the midst of a conversation with a friend about "transition" in CF Care.

"Transition" is the word that describes moving from pediatric CF care to adult CF care. Until even as recently a decade ago, most CF patients were being cared for, even as adults, at pediatric clinics/hospitals. This is because CF has historically been a childhood disease - people with CF didn't usually live in to adulthood. Thankfully, things have changed. Projected life expectancy for CF is now 41 and these days, most people with CF are expected to eventually move from pediatric CF care given pediatric hospitals to adult CF care provided at adult hospitals. The process of moving from pediatric to adult CF care is often referred to as "Transition."

Bennett is still 10 years away from "transitioning" to an adult CF clinic. But, I tend to see transition in CF as being less about learning how to move from one hospital to another but instead learning how to manage CF as a child to managing CF as an adult. And that transition happens from birth.

In the same way all children are given "teenage years" to practice being an adult. I believe, there should be a similar transition phase for kids with CF to practice being an adult with CF. This is why I was so interested in filling this worksheet out with Bennett this morning.

Transition is still a relatively new concept in CF care. So, there aren't a lot of resources for transition of CF care available yet. That's why I was grateful to get my hands on this resource to try out with Bennett, even if it was a bit premature for his needs. My goal in completing this form together was just to create a path in my own mind, for what transition might look like.

Right now, as his mom, I carry most of his disease (along with his dad). I know most of the information. I make most of the decisions and connections. I address most of the problems. I'm in charge. But, one day, Bennett will be the one who primarily carries his disease, who makes the decisions and the connections, who will be addressing the problems and being in charge. I don't see that happening late in high school or when he goes to college. I see that beginning to happen now, ever so slowly, so that the both of us can get used to our new roles - me in the background, he in the foreground.

This transition worksheet, which Bennett and I did after breakfast this morning, allowed Bennett and me to have a really great conversation about his CF care and his ability to advocate for himself. I saw him completely light up when he shared with me the things he is proud that he could do. Bennett was very honest about the things he can't yet do. And, he asked me to start teaching him about the things he doesn't know much about or how to do.

So, at nine years old, what exactly can Bennett do and what does he understand about his care? What does he still need help with and how can we, as his family, support him to gaining greater independence with CF?

To my delight, Bennett indicated he can describe CF other others. He can speak up for himself and tell others what he needs. He can take part in healthcare discussion about himself (I owe a lot of credit to his CF docs who do an excellent job of talking to him directly when we're in the room). And Bennett indicated he understands the importance of regular exercise and the importance of sleep and rest. This was all really great for both of us to hear! There is a lot that Bennett is already doing well in his transition to owning his own CF care.

There are also areas that Bennett acknowledged he needs help with. Bennett explained that while he can take his own medications, he still needs help to prepare his meds. He also needs help properly cleaning his breathing/respiratory equipment.

Bennett said he mostly understands why he takes most of his medicines but acknowledged he didn't totally understand why he had to take certain meds or why they were important to take. In addition, Bennett asked to learn more about how, if he wants to know more about CF, he can get that information. He suggested: "maybe I could learn how to call the doctors??" I so love this! Truly, this is transition conversation has gone well when my nine year old wants to know how he can call his CF clinic to ask questions.

Things I didn't really know about Bennett, but that I learned from this exercise, is that Bennett doesn't totally understand infection control (the reason why people with CF can't be together and why CF clinicians have to gown up and glove at clinic). In fact, when I tried to explain it to him, his eyes welled up with tears. It was hearing that his "lungs are sick" that made him start to cry. I had to quickly caveat it with, "well, they are sick but they aren't. I mean, you aren't actually sick right now but your lungs are susceptible to others germs so they are kinda sick." He asked me, sort of offhandedly, for the third time recently, "am I going to die?"

I need to answer this question for him but I wasn't ready to at this moment. That's a transition conversation I'm not quite emotionally ready to make. I also skipped the question about whether or not the person with CF understands his/her issues with infertility.

I really appreciated this little checklist for the opportunity it gave Bennett and me to sit down and talk about aspects of his disease that he and I often take for granted that he may already know. Bennett really loves it when I give him my full undivided attention. So, we both really enjoyed the intimacy of the moment of sitting down and talking through how well he takes care of himself and identifying ways he wants to do that even more.

I'm incredibly proud of Bennett and the person he's becoming to be. So few children have to carry such a difficult burden on their shoulders. And he does it with such strength and grace.

A well-known psychologist once said: "the goal of raising children is getting them to adulthood as fully intact as possible." Maybe, I'd add, the goal of raising children with cystic fibrosis is getting them to adulthood as fully independent as possible.

I'm grateful Bennett is making it very easy for me to begin that process now.

NACFC 2018 - My Reflection on the excitement of CF Modulators

2018-11-02T12:36:00.003-05:00

NACFC didn't feel quite so overwhelming to me this year since this was my third year to attend. I was more familiar with the format, the expectations, the pace of sessions.

But, there was a moment that took my breath away when I least expected it to.

It was during Plenary 2. I was sitting in the middle of literally the hugest auditorium I've ever been in. I was listening to the newest information about CF research. A picture of a little baby came up on the screen (see what I'm talking about here at the 53:35 mark). NACFC Speaker Dr. Felix Ratjen had spent the last hour explaining all the success we've seen with the newest CFTR modulator medications and wondered outloud, "how about if we start [these meds] early on in babies, in patients, before the lungs are damaged?""

What if we give newborn babies, still yet free of CF damage, the newest CF drugs available?

It was a hopeful statement.

Suddenly, I had this flash of memory to the previous plenary, Plenary 1, held the night before, when speaker Dr. Lisa Saiman shared other new clinical information in CF by juxtaposing where we were in CF research the decade before.

"Ten years ago..." Dr. Saiman would end up saying over and over again as she explained how much CF meds have changed in a decade. As Dr. Saiman flashed back to the decade prior, I couldn't help but do so in my own life.

It was almost 10 years ago that Bennett was born (he just turned 9). At the time, Brian and I, new CF parents, eagerly listened to and read the newest CF research with expectant hope. At the time, we felt very encouraged by the newest research and genuinely hoped that the newest meds available 10 years ago would prevent CF from damaging his body. And yet, back in the present, it's hard to believe that research is 10 years ago old and no longer relevant.

Pulmozyme, a medicine that we use routinely nightly in our house (and have for years), had only barely been out of trials and become FDA approved when Bennett was born. It felt "new" and "exciting" and now, it's commonplace and part of the standard CF protocol.

I looked up again at that picture of the cute newborn baby up on the screen and re-heard the words "how about if we start [these meds] early on in babies, in patients, before the lungs are damaged?"

All of the sudden, that idea no longer gave me hope. It brought me grief.

"We're losing years!" I felt some deep place in my heart call out. "We've already lost a decade!"

No longer is my family in that group of CF families who are"newly diagnosed." Our family isn't even in that group of families with "young children with CF" anymore.

We're in a new place, a wonderfully joyful place for most parents: a place with a tween!

But, when it comes to CF, time often passes faster than research can answer questions and faster than medicines can be approved. So, while birthdays in CF are a really big deal because they are a huge gift that we celebrate, they also mark time that has passed without a cure.

Bennett is in a really good place, considering. He has had very good lung health compared to many children with CF. But we know silent lung damage is happening and we know that, statistically, he's losing 2% of his lung capacity per year.

More drugs are coming. It's a very exciting time in cystic fibrosis. But it's a hard pill to swallow that we've also lost nearly 10 years of potential benefit for those drugs.

Sitting in the auditorium, surrounded by a sea of professionals quietly listening to the newest information on CF, I felt time compress.

New meds are coming.

But we won't be the lucky ones with newborns.

We won't be the lucky ones with young children.

Fingers crossed, in another few years, we'll be the lucky ones with an older child.

But I want those early years back. I want to be the one with the newborn who benefits. And I want to stop the clock until we have more tools in our arsenal.

Might this be what it feels like for those who have gone before me - parents of adults with cystic fibrosis - whose time compression is marked by many more FDA approvals of CF medicines...or, worse, are marked by the time when there were no new CF medicines at all? How difficult it must be for them to hear of the excitement in the CF community and yet to know their child, whom they've had to say goodbye, will not benefit?

My heart breaks for them. And it breaks for us, all of those with CF still suffering from this awful life-threatening disease.

Can I be sad for time lost, even as I have hope for the future? As a mother, I can hardly help but wanting Bennett to have never suffered.

This is a very very good time for the CF community.

But, as good as it is, this NACFC just reminded me:

The clock is still ticking...

NACFC 2018 - 16 Things I Learned at NACF 2018

2018-10-26T13:12:00.000-05:00

I just returned from being in Denver, Colorado for the 2018 North American Cystic Fibrosis Conference, an annual conference with more than 5,000 scientists, researchers, clinicians, parents and people with CF in attendance this year.

I wanted to do a "brain dump" blogpost so I won't forget all that I learned during my few days there (this post). But I also wanted to reflect on some inner thoughts that emerged while I was there (next post).

For the first time ever, Brian came with me this year (we tried to steal away some time for a very rare weekend getaway for ourselves). Brian was able to attend a few of the microbiology sessions and report back what he learned.

The most important point I should share (something have to remind myself each year) is that, in many ways, CF research is moving fast and, in other ways, CF research is slow. Most of what we learn at NACFC each year, for the most part, isn't necessarily "groundbreaking" or rather, it doesn't often translate in to an immediate behavior change in CF Care. But, taken in totality, it is helpful to learn to better understand the research trends and to get an idea of what is coming down the pipeline.

Here are the 16 things I learned at NACFC this year:

1.) Next year is gonna be a big year for cystic fibrosis!

Probably the biggest news was announced during the first Plenary. We are likely less than one year away from the announcement of the "triple combo" - a Vertex drug (a combination of three drugs) that could benefit up to 90% of the CF Community (those who have 1 copy of the F508del).

We know that the "Triple Combo" drug is highly effective in people with a single copy of F508del. Phase II data results came out in February 2019. Both VX-659 + Tezacaftor/Ivacaftor and VX-445 + Tezacaftor/Ivacaftor achieved similar results.

Data on the VX-659 trial should be available in late 2018. Data on the VX-445 trial should be in the first quarter of 2019. Vertex plans to submit approval no later than mid-2019.

2.) Vertex isn't the only company advancing a triple combo drug.

Proteostasis Therapeutics has multiple trials going on right now. Galapogos does as well. (Learn more about what is happening in this space by watching the NACFC 2018 Plenary 1 here: https://www.youtube.com/watch?v=LlZ5kvJ9AWg - starts about the 25 minute mark)

3.) CFTR Modulators are good. But they aren't gonna make lung decline go away entirely.

We know that lung decline is still an issue for those who are currently benefitting from CFTR modulators. So, our community, even with modulators, needs to keep focused on keeping CF lungs healthy.

This slide, which shows evidence of Ivacaftor treatment and stopping Ivacaftor treatment, reminded me both how much I'm grateful for the CFTR modulators and how they are still not a cure.

This slide is a reminder that, CFTR modulators on the market or not, we still have a lot of reasons why we need to better understand respiratory infections and inflammation.

4.) The CFF is putting $100 million dollars towards an infection research initiative because we have so much more to learn about infection diagnosis, treatment and outcomes.

The CF Foundation recognizes that while CFTR modulators are coming down the pipeline, those for whom cannot benefit from this new drug combination have many years left to wait for their drug to come...and even then, there is some portion of the CF community who won't be able to benefit from these drugs just due to medication interference or some other reason. Many people with CF are being plagued by lung infections and fungal infections. That's why the CF Foundation has decided to invest $100 million to study these life-threatening infections affecting people with CF. (Watch more about this initiative and the research that was presented during this Plenary here: https://www.youtube.com/watch?v=K-u4-QhoYsE - it starts about the 9 minute mark)

5.) Inflammation remains a major cause for lung damage. There are new anti-inflammatory drugs in the pipeline.

There are new mucociliary clearance drugs also in the pipeline.

6.) The CF Foundation is putting resources towards using "the oldest new idea to improve CF Care": CoProduction (true partnership between the person with CF/families and the clinician/clinical team when making decisions in care)

So many people enjoyed the last plenary of NACFC 2018 which was led by Maren Batalden. Watch it here: https://www.youtube.com/watch?v=bC-Lq2LWQcM

Maren talked about the general healthcare ecosystem, calling this slide a "map" of sorts. Notice patients, professionals and the healthcare system overlap but all work towards health outcomes.

This was one of my favorite slides. It demonstrates the relationship between the patient and the clinician. Many times I've heard clinicians explain their interactions with patients as the first image ("communications"). Even at NACFC there was conversation about "educating" the patient and how the patient needed to be further "educated." But, the place where coproduction actually exists is within the last image of "partnering." It is here that the clinician is no longer in the center of or more important to the relationship. In partnering/coproduction, the patient and the clinician are equal. Here they give and take equally. This is the place where decision-making works best, as both parties respect each other's expertise.

This slide was really great. It was the results from the CFF Mind of the Community Survey given last year. It shows that while patients do tend to primarily trust their doctors with regard to CF research/drugs (70%), clinical care (50%) and medical stages of CF (34%), they actually trusted their peers primarily when it came to sticking to a treatment plan (37%), day-to-day life (67%) and CF and relationships (56%). It's clear that in many important areas people/families with CF trust people/families with CF more than their often-central care team. For this reason, we must treat CF differently than we have in the past. We can no longer have a one-way communication relationship, it must be partnership - it must be coproduction.

Alongside Kathy Sabadosa (a CF Mom) and Melanie Abdelnour (a person with CF), Cindy George (CFF's Senior Director of Patient Engagement shared about the partnership experience from the CFF's point of view. Cindy mentioned that the CFF is putting resources toward improving coproduction, most specifically within the CFF's mental health initiative, with the addition of CF resources and guidelines, by way of the Success with Therapies Research Consortium and through the Quality Improvement Learning Networks such as the CF Learning Network.

I can personally attest that the CFF is putting more resources towards building better partnership with patients and their clinicians in order to improve CF care. This new focus from the CF Foundation has supported the work I've been a part of with the CF Learning Network (CFLN) for three years now. In particular, over the last year, I have been working within the CFLN to improve CF Center-level coproduction.

If coproduction is patients and clinicians working together as equals, then "center-level coproduction" is when a group of patients and a group of clinicians work together as a team to improve CF care at the CF Center level (such as improving the wait time in the clinic, beefing up infection control, improving the clinic's communication system, etc). "Individual-level coproduction" is what we refer to as partnership between a individual clinician and a individual patient at point of care/within the clinic room, which is also very important. But I have been working more directly in center-level coproduction.

This year, I took the research I have had the privilege to gather through the CFLN and submitted it as a poster which was accepted at NACFC 2018. It was then accepted as a presentation at the "Quality Improvement Thematic Poster Session." It was really rewarding to be able to teach others about the good work happening within the CF Learning Network through our participating CF quality improvement teams at 27 CF Programs across the US.

I am really hopeful that next year I can work with more of our patient and family members within the CF Learning Network to submit their own posters. We need more people with CF and their family members presenting research at NACFC. I fully believe that, as patients - members of the clinical team, we know a lot about CF and we should be demonstrating that knowledge alongside researchers at NACFC.

7.) There's a company working towards PERT-free food.

What if you could offer your kid a snack without the need for enzymes? That's what the gang at GlycosBio are working on. It's a very interesting concept.

8.) Bowel, bladder and sexual dysfunction is not uncommon in CF. Better posture and strengthening core and pelvic muscles may help.

Physical therapists are being used more and more CF care. There are common issues prevalent in CF that might benefit from work with a physical therapist. For example, bowel and sexual dysfunction is common in CF.

This is what it looks like for your diaphragm and pelvic floor when you cough. This is why for many there is loss of bladder and bowel control, the muscles may be trying to do their job but the pressure from coughing can be too much.

Physical therapist Karen Von Berg explained that building good posture and core muscles may help with the stress the muscles often find themselves under due to CF's effect on the body.

Of course, Karen Von Berg were also reminded us that we should all be sitting at a 35 degree angle when we poop, CF or not. (Credit: Karen Von Berg, D.P.T. The Johns Hopkins Hospital, "Collaborative Approach to GI Management: A Team Perspective")

9.) One way to address DIOS in CF may be massage of the abdomen.

I don't have a ton of information about this session but I thought it was good. Karen Von Berg also explained that massage can help DIOS issues. It's unclear why it works - if it's because massage on the abdomen gets the blood flowing on that area which improves motility or if it's because massaging itself can massage the poop to the right place. While Karen didn't have a video on how to give massage but she suggested massaging along the bowel: up the ascending bowel over to and down the descending bowel. (Credit: Karen Von Berg, D.P.T. The Johns Hopkins Hospital, "Collaborative Approach to GI Management: A Team Perspective")

10.) There is still a lot we don't yet know about pulmonary exacerbations.

How do we define a "pulmonary exacerbation?" Doctors don't agree. What do we treat it with? What do we use to treat? Where should we treat it? Who should monitor treatment? When is treatment stopped and when is the pulmonary exacerbation resolved? How do we classify it as mild, moderate or severe? How does the patient/family weight in? These are just some of the questions doctors and researchers are trying to answer.

11.) There is debate within the clinician community on whether IV antibiotics should be given at home vs. in the hospital.

Before I share more, I must outline what the debate between two really great CF doctors was about. They debated whether research points us to believe IV antibiotics (once started in the hospital and proven to be effective for an individual patient) should be continued at home vs. in the hospital.

The debate was convincing on both sides. It seems that the issue is very patient dependent and results, positive or negative, can due to a complexity of things. Data may tend towards telling us that FEV1 results seem to improve better if you finish a course of IV antibiotics in the hospital. But we know that FEV1 is just one measure of someone's overall health in CF. Data also tends towards telling us that quality of life may improve when a course of IV antibiotics are given at home.

What I came away with for my own family was that IV Antibiotics can be given at home but need to include, at minimum, 1-2 weekly labs, weekly clinic visits, PICC line care and good communication with the home care team.

The conclusion of the debate brought about a few important points both doctors agree on. Both doctors agree that the CF community needs to be more aggressive with treatment of exacerbations. And, if IV antibiotics are to be given at home, there should be shared decision making (partnering between the clinicial team and the patient) and good processes for administering and monitoring.

This debate will likely continue as more research is being done on it. STOP 3 is a study that might provide some insights as well as ongoing quality improvement projects from clinics. (Credit: Elliot Dasenbrook, M.D. M.H.S. Cleveland Clinic, "Exacerbation Treatment at Home is as Effective as Exacerbation Treatment in the Hospital. Pro")

While I was swayed slightly by the data to stay in the hospital for IV antibiotics as long as possible, I find myself more than frustrated that the the hospital is hugely disruptive to sleep and family life. I did gain a better appreciation that IV antibiotics given at home may mean people who get IVs at home tend to push themselves too fast to get back in the saddle (they go back to work, school, family life, etc too fast) and may not fully recognize their own need to rest stress-free. (Credit: Don Sanders, M.D. M.S. Indiana University, "Exacerbation Treatment at Home is as Effective as Exacerbation Treatment in the Hospital. Con")

12.) Respiratory virus infections, including RSV and Influenza A, may promote P. aeruginosa biofilm growth which transitions to chronic infection.

This slide shows the the presence of RSV (which is one of the cold viruses) has a correlation with more PA (bacteria in green). But, scientists have been trying to figure out why.

Apparently, when a virus goes in to the epithelial cells (the outside cell of the lung), the epithelial cells create a substance called interferon. Interferon signals to other cells that an infection is near and helps other cells resist the virus. But some scientists now hypothesize that interferon stimulates PA to develop a biofilm. This biofilm is a protective "bunker" that protects the PA from the body's defenses. Possibly this biofilm establishes chronic infection. (Credit: Brian Kocak, University of Pittsburg, "Interferon Signaling Induces Metabolic Shifts In The CF Airway Epithelium To Promote P. Aeruginosa Biofilm Growth")

13.) Microbial ecology (how bacteria, viruses, etc. interact with each other) is important in CF and shouldn't be ignored.

In the session called, "Mucin as a Nutrient Source for Staphylococcus Aureus Invitro & In CF Associated Chronic Rhinosinusitis," it was presented that, in general, pathology (or CF disease) is determined as much by the conditions of the environment inside the lungs as by the presence of a pathogen (bad bacteria/virus, etc). In other words how sick with CF you are is probably less about whether you have a bad bacteria in your lungs or sinuses but it's more about the interaction of that bacteria with other bacteria. (As Brian explained it to me: it's not whether Johnny is a bad kid or a good kid, it's whether Johnny is a bad kid when he gets around the bad kids at school).

As demonstrated on this slide, the healthy lung environment (left side) has plenty of oxygen (O2) and normal food (GLc/glucose). But when there's a viral infection (such as an infection in the sinuses, also called "rhinosinusitis"), oxygen levels (O2) fall and nitric oxide levels (NO) rise and lactate replaces glucose (GLc). It is here, in this new environment that bacteria that was harmless suddenly becomes disease-causing.

This session also discussed how chronic rhinosinusitis (an infection in the sinuses) can help promote Staph A. infections (see above). For this reason and the reason written above, it's becoming more clear that it's not enough to avoid active Staph A. contamination. We should also be avoiding any respiratory infection at all, even seemingly harmless ones, since chronic diseases are the result of complex microbial interactions. As Brian explained to me: it takes a village to get a chronic disease, so it's best to avoid the entire village if one can. (Credit: Sarah Lucas, PHD Candidate, University of Minnesota, "Mucin as a Nutrient Source for Staphylococcus Aureus Invitro & In CF Associated Chronic Rhinosinusitis")

14.) Nutrition options in the CF world continues to expand.

A few years ago, I learned about Kate Farms at NACFC. We love Kate Farms feeding tube formula at my house and use it nearly every day. Now, there is another company on the market called "Functional Formularies" which offers organic, whole-food feeding tube formulas and whole meal replacements.

15.) Physical therapists are being added to many CF teams and they are doing great things with PT.

Bennett has started benefiting from physical therapy. So, I was very interested to attend this session on physical therapy to learn more about the benefits of physical therapy. What was funny was when I arrived for the session, I learned it was Bennett's CF physical therapist giving the presentation. I guess that's when you know you're at the right place when you want to better understand your clinician's advice and find out that it's your clinician who is teaching others - ha! (Credit: Renalli Arcinas, D.P.T., Children's Medical Center Dallas"Putting the Pieces Together: Comprehensive Application of PT Interventions in CF".)
16. There's a new a college scholarship opportunity for siblings of people with CF through Vertex.

This may not be new news but this was the first time for me to hear that Vertex has a $5,000 scholarship program which includes siblings of people with cystic fibrosis as well as caregivers (yay for Vertex for recognizing CF affects the entire family!). To apply, click here: https://www.vrtx.com/giving-back/cystic-fibrosis-programs

Speaking of Vertex, Brian and had an opportunity to meet Paul Negulescu from Vertex at NACFC. I asked Paul if he would take a picture with Brian and me as we wanted to show the picture to Bennett when we returned home. I always tell Bennett when I go to NACFC that "I am going to meet with the doctors and scientists trying to find a cure for CF." Now that a med that may actually benefit Bennett is likely to be out in the next year or so, I decided to show Bennett the picture of the person who helped create it. We don't know what the future will hold for Bennett but it feels really great to know there are a lot of people like Paul out there trying to find a cure for cystic fibrosis.

In conclusion, I wanted to provide free online resources for anyone who is interested in reading more about the research provided at the 2018 NACFC. Click here to learn more:
Plenary 1: https://www.youtube.com/watch?v=LlZ5kvJ9AWg
Plenary 2: https://www.youtube.com/watch?v=K-u4-QhoYsE
Plenary 3: https://www.youtube.com/watch?v=bC-Lq2LWQcM
The list of NACFC 2018 sessions: https://onlinelibrary.wiley.com/doi/10.1002/ppul.23294
The list of NACFC 2018 posters: https://onlinelibrary.wiley.com/doi/full/10.1002/ppul.23295 (see what research is happening in the CF community)
Poster Session Abstracts: https://onlinelibrary.wiley.com/doi/10.1002/ppul.23297 (read these! They are interesting!)
Symposium Summaries: https://onlinelibrary.wiley.com/toc/10990496/50/S41
Author Index: https://onlinelibrary.wiley.com/doi/10.1002/ppul.24153 (find out what research your clinician might be involved in these days)
Facebook NACFC QA Sessions: https://www.facebook.com/pg/cysticfibrosisfoundation/videos/?ref=page_internal

System Failure

2018-09-12T10:19:00.000-05:00

In the world of cystic fibrosis, the older you get, the more meds they add to your list of things to do. The body gets sicker, the list of medicines and treatments grows.

Until recently, I really struggled with getting in all the things I have to do to keep Bennett healthy on a daily basis. For example, Bennett takes 8 medicines three different ways, all by 8am. It didn't help that that my husband's job demanded that he be gone alot, so it was just me a lot of the time.

I felt so frustrated at myself. It's easy, as a CF mom, to get signals that somehow, by failing at the incredibly burdensome treatment regimen, I was failing, not just my son but as a mom. If mom guilt is bad, I'd argue CF mom guilt is even worse.

In general, I work really hard to give myself grace and recognize I'm only doing what I can. But who wants to hit the bar lower than ideal when your kid's life depends on it?

Several months ago, feeling the pressure that I wasn't doing everything Bennett needed, I said to one of Bennett's CF docs, "Tube feeds ARE a priority. I just have 40 other things that are a priority before that."

I remember thinking right after I said that: Do I *really* have 40 priorities more important that feeding Bennett?!

Then, all of the sudden, a small but very honest voice inside me responded to myself's question with a huge litany of fears and concerns - some I didn't even know existed.

I tried to write them all down. Here's what I came up with as "top priorities that come before feeding my son his g-tube feeds":
* making sure Bennett doesn't vomit
* making sure Bennett doesn't get C.Diff again
* making sure Bennett gets exercise so his lungs don't get sick
* making sure Bennett doesn't stop eating by mouth
* making sure Bennett doesn't get acid reflux

* making sure Bennett's emotional needs are being met
* making sure my other children's emotional and physical needs are met
* making sure my own emotional and physical needs
* making sure we have insurance coverage for the formula
* making sure we have feeding bags
* making sure we have feeding bag extensions
* making sure the feeding pump battery is charged
* making sure I know where the feeding pump backpack is
* making sure we have enzymes or Reliazorb at home for his tube feeds
* making sure I don't give a tube feed before a Vest treatment
* making sure I put the Liquidgen in with the tube feeds
* making sure I don't freeze the Liquidgen on the top shelf of the refrigerator
* making sure I don't leave the Liquidgen out on the counter to spoil
* making sure I throw the Liquidgen away after it expires in 14 days
* making sure we can slow our family schedule down so we can give tube feeds

That's only 20 "priorities" but, I guarantee if I sit here long enough, I could list 20 more...

My point is that failing to be proficient with the CF treatment regimen is never as simple as "I don't want to care for my son" or "I don't know the importance caring for my son." The challenge of doing what the doctors want me to do comes down to organization, time and addressing a list of bigger priorities that is a mile long (some, I don't always even consciously know).

Getting all of the things done that people with CF have to do isn't as easy as just checking them off a list.

It's not about willpower. It's about barriers.

Emotional barriers, physical barriers, insurance barriers, etc.

And something I've recently come to more fully appreciate, it's about system barriers.

Success at home with the CF treatment regimen requires success with aligning the systems we live in.

This is why I have gained much greater success with the treatment regimen lately (I'm now up to being able to give Bennett 3 tube feeds consistently daily). But it's taken a tremendous amount of oversight, self-reflection and effort. It's also taken a good deal of money and required I change our physical surroundings.

I think we're set up from here to move forward more easily. But, I can't help but think about so many of our CF family friends who don't have the time, money or ability to master their entire environment to improve how they take care of CF at home.

For a very long time, it may looked like, on paper, that I hadn't been working diligently to give Bennett tube feeds daily. Until recently, I was able to get in less than 10% of Bennett's tube feedings (1-2 feeds per week).

Every time I would come to clinic, I would cringe. At times, I felt scolded and shamed. It wasn't that my CF team wanted me to feel that way. The reality is they feel the same pressure as I do - to not hit the bar lower than ideal when Bennett's life depends on it.

But, having said that, there have also been times when I didn't feel my efforts were as fully appreciated - how diligently I was working towards improving that 10%, even as that number wasn't changing.

It's because I was working incredibly hard to change, not the number/behavior, but the system. For me, what's the point in getting in 100% of tube feeds consistently for 2 months and then burning out completely? I want 100% for the long term.

CF is a time-bomb. Time is of the essence. But CF is a also marathon, not a sprint. I wanted to find a way to take care of Bennett at a pace that works for him, for me and for our family - and in a way that we can continue to support over time.

As humans, we live in a variety of systems. It's easy to tell myself that I have full control over my life. But the reality is I live and work in a series of systems that create pressures on me to behave in certain ways.

I have come to realize that recognizing that these systems exist helps me to appreciate why I can totally believe in the value of something and fully want to do it but still struggle to get it done. It also helps me understand how I can better align what I want with what can do.

For simplicity sake, I love thinking about the 4 primary systems we live in: the micro-system, the meso-system, the exo-system and the macro level system.

The micro-system (individual) is the system I have the most control over - where I place my hair brush, which color I prefer, my choice of hobbies, etc. This is the place were I made the decision I *want* to give Bennett tube feeds.

The meso-system (social environment) is the system in which my family and friends exist. This is where, for example, the pressures of my children's needs and my husband's desires exists. This is the system that I was struggling with when it came to Bennett's tube feeds.

The exo-system (physical environment) is the name that describes the system where pressures are placed on me by my husband's job, my children's school, my co-workers, our church, etc. This is also where I struggled for a long time to align Bennett's tube feeds.

The macro-level system (community environment) describes the limitations and opportunities that come from my location of where I live and my larger community.

When I want to change something about Bennett's care, I have to evaluate what is and isn't working in a system. And I have to prioritize the particular system's need for balance.

When I couldn't figure out Bennett's tube feeds, I recognized that it wasn't that I could make a change to my micro-system (I *wanted* to give Bennett tube feeds). The change that had to happen was within our meso-system (the system of our family) and our exo-system (our home).

It took Brian and me more than a year to make these changes, changes propelled by more than just the need to give Bennett his tube feeds, but most definitely by our need to take the best care of Bennett's health.

We realigned our meso-system. We withdrew the boys from school and began homeschooling last year to slow down our family life and give us more control over our schedule. I spent time working with each of the children to be more independent in their self-care and in Bennett's care.

We realigned our exo-system. We purchased a new home, specifically with Bennett's CF care in mind. (I cannot even express what I huge difference this has made!) Now I can keep more supplies on hand, have them readily available and identify when I need to restock. A new home layout also allows Bennett to have more space to do his treatments where it feels functional and less in our face or as a mess on the counter.

Even though my micro-system was fully on board with giving Bennett tube feeds, I made changes there too. I started using FolioHealth, a new pilot tracking platform which helps me accurately track how successful I am being with the treatment regimen and identify patterns that prevent me from getting it all done. (Another game changer!!!)

And I designated someone in my life who checks in with me regularly about Bennett's care to help me recognize when Bennett's CF regimen is too much for me to handle emotionally and encourages me to find a way to get a break.

Change cannot be made in a vacuum. We live in the midst of complex systems. When CF families like mine are asked to do "one more thing", it must mean that everything else must move to make room for that one thing. Considering we are asked to do "one more thing" several times at every quarterly appointment, that means we are trying to make room for up to (or even more than) a dozen new things per year.

I am tempted to feel like a failure when I can't do that. But I have to remember that it's not me that's a failure, it's the failure of a system. I just have to change my system somehow.

It might not have looked on paper for a long time that I cared about improving Bennett's nutritional health but the very opposite is true. Now that we've got our working systems in place, hopefully the paper will accurately reflect all our hard work!

First Day of School 2018

2018-09-04T13:54:00.005-05:00

Today was a momentous day in the Gamel household, the start of our newest adventure as a family. It was the first day back to school - at a brand new school - for Oliver and Bennett. (Avonlea had her first day back to school at her school a few weeks ago.)

We are all so grateful for such a wonderful year homeschooling last year. A year of homeschooling was like water to all of our souls. We were able to rest. We were able to gain back family time. And the boys were able to gain back self-confidence after having had challenges the year before with both cystic fibrosis and dyslexia.

A year and a half ago, I struggled to figure out what the best school option for the boys would be moving forward after a difficult year. Should I homeschool? Might another school in our area be able to better meet the boy's needs and the unique needs of our family? I contemplated opening my own school after discovering a fantastic school model in Austin called "Acton Academy" that I would have loved to replicate in Waco. I was even awarded a grant last fall and attended a 3-day think tank in New Orleans with 4.0 Schools for people around the US who want to start their own schools. But, alas, I struggled to feel settled that I could run a school, take care of 3 young kiddos and deal with Bennett's ever-unpredictable cystic fibrosis.

Ultimately, our family chose to homeschool last year and we were very happy with that decision. The only downside was that my boys, having been used to attending school daily, missed learning within a community of their peers. Homeschooling was so good in so many ways. But, since we didn't join a co-op our first homeschooling year, we found it could also be lonely.

Having experienced the beauty and flexibility of homeschool but also the community and connection of traditional school, Brian and I wished there was another option - something in between homeschool and traditional school. This is where that little school in Austin called "Acton Academy" looked so interesting to me.

Well, low and behold, this summer, I heard from a friend that there was someone in Waco who was creating a school called "Acton Academy." I couldn't believe it. I called up that someone, who now is a friend of mine, and shared our story. It was then that I knew, our boys would be 2 of the 22 founding students of the school now officially called "Acton Academy Waco."

Oliver, First Day of 5th Grade

Oliver, 10 years old

Bennett, First Day of 3rd Grade

Bennett, 8 years old

The boys were very excited to go to school this morning. This new school seems like a great match for what they need. It's a school that is focused on helping each child discover their abilities and talents, in addition to mastering their academics. CF brings Bennett a lot of challenges but also brings him a lot of gifts. Dyslexia does this for both boys, as well. I look forward to seeing how Acton Academy will help the boys discover, in a self-directed way, how to use their strengths as leverage over their weaknesses.

Some things I love about Acton are that it offers a mixed-age classroom, gives no homework (yes! let them play!), focuses on building character, uses socratic discussions, uniquely integrates technology, offers alot of freedom to the students and is overall very intentional.

I don't believe there is one school or one school model for all children. Just as children are different, so are their needs for different school models and communities. Brian and I consider it a great privilege to have a choice in the way we educate our children. We realize so many families never get that opportunity. Brian and I are evaluating each child and each school year as we go. I'll homeschool in a heartbeat again in the future if any of my children need me to. But, I'm also just as happy to kiss them goodbye and wish them a really great day at school.

Of course, I can't fail to mention that there's another little Gamel experiencing a new adventure this year. Avonlea started Kindergarten three weeks ago. It's hard to believe our sweet baby is a kindergartner. But it makes it easier when we see how much she loves it!