All the Marletts, Our Life as a Family

Back in again…

2012-02-25T22:56:00.001-05:00

Cultures are cooking, labs were drawn, urine collected, and vitals taken. After a long day spent in the ED it was decided that they wanted to admit Lucy for observation…ugh. She continues to run a fever, but she is not acting “sick”. The attending that is on this weekend is concerned about Lucy, I can appreciate that, but I’m not quite sure what keeping us here over a weekend is going to accomplish.

I am going to take advantage of the wonderful nurses here at the hospital and catch up on some much needed sleep. How’s that for looking on the bright side. I’m so grateful for iPads, Netflix, and Dora the Explorer, we watched all 24 episodes from season one today, some more than once. Drew and the girls came over and had pizza with us for dinner, Jack was at a party for 4th and 5th graders only so he missed out on all of our fun. Lucy has the nurses on the floor hopping getting all of her meds and supplies, and a few extra supplies for baby doll too.

Not sure what tomorrow will bring, but on Monday our main doc will be back.

On the fence…

2012-02-25T09:56:00.001-05:00

That is Lucy’s current stance, all week her body temperature has been on the rise. It is hovering at or above our cut off for what we consider a fever. Last night it went higher than it has all week which prompted a call to the on call doctor. Our options at 11pm were few, she needed to have repeat labs and blood cultures done to rule out infection. Sensing my frustration, he discussed with me my options for bringing her in, if her temps came down then we could wait until the morning, but if they continued to rise she needed to come in, and so began the back and forth discussions in my head of what to do. Lucy has been running a low grade temperature for weeks, she has had blood cultures done weekly for the past three weeks with no growth...yet, and neurologically she has a lot of issues going on which we believe is playing a role in her temperature instability. In addition to the fevers, she is acting manic again, not sleeping. I would describe her behavior as volatile, one moment she is fine the next she is screaming. This is so difficult.

Every fifteen minutes or so we kept rechecking her temperature, she was trending down which we took as a sign to bring her in in the morning. After struggling to find sleep she finally did at around 12:45 pm.

Our plan is to bring her to the ER this morning for labs and cultures. If her temperature is on the rise or lab work is indicating infection then we will stay and start treating her as if she has an infection until things prove otherwise.

Quick update…

2012-02-17T21:32:00.001-05:00

We arrived home tonight at around 6:30pm, exhausted, emotionally drained, nervous, but relieved to be home. We are working on the dosing for Lucy’s anti-seizure meds. She has not had any more seizures since starting her on the Keppra and pheonobarb regimen, for that we are very grateful. She is, however, one miserable baby. Keppra has a nasty side effect of causing increased irritability, this is just what Lucy doesn’t need, more irritability. I have heard this side effect referred to as the Keppra attitude. She was one miserable baby before we started her on these meds. I’m not sure yet if the Keppra is adding to her irritability, or if it is something else, or if her behaviors are heightened because she is exhausted and in need of getting back into her routine. Because of all of these things, we were given the option of coming home tonight and work at sorting out these issues on an out patient basis. Although she is clearly not better, it appears that we have stopped her seizures. We figured if she is going to be miserable and screaming at least we can be miserable in the comforts of our own home.

Thank you for all of your thoughts, prayers, love, and support. I am staring at a large pile of laundry and an even larger pile of medical supplies, meds, and TPN bags that need to be put away. I think I’ll put the med supplies away tonight and save the laundry for tomorrow.

Our day…

2012-02-16T22:52:00.001-05:00

Our day started off ok. Lucy woke up at around 8 am, played with some toys, chatted to people she felt like chatting with, ignored those she felt like ignoring, watched Tinkerbelle for the billionth time, and then started seizing again. The team was rounding when she started seizing so they were able to witness first hand the type and intensity of Lucy’s seizures. In a way I was relieved that they saw them, I no longer have to describe to them what they are like. She seized for almost five minutes when they ordered a loading dose, the maximum amount, of phenobarbital for Lucy’s weight to stop the seizures. Since then she has been sleeping, pretty soundly.

Why is this happening, it’s anyone’s guess. In theory Lucy did not have enough methadone for it to really have this much of an effect on her, but we all know how much Lucy likes to follow theory. It’s possible that this has something to do with starting her on methadone, but not very probable. None the less we have discontinued methadone for the time being. Could it be a paradoxical reaction to the phenobarbital? It’s very unlikely, phenobarbital is a tried and tested medication, this is one of the reasons we agreed to trialing it with Lucy. The words disease progression were uttered too many times today for me to count. I don’t doubt that that could be a likely contributor, but there are also a lot of other things going on that we need to investigate.

Neurology was consulted today and discussed with me the need to put Lucy on another anti-seizure medication. Phenobarbital is a sedative. Lucy is experiencing seizures despite being on phenobarbital. Increasing her phenobarb dosing to control her seizures long term is not the best plan for seizure control, that is if we ever want to see Lucy awake. We are going to continue to use phenobarbital at night, but add in keppra for more seizure control. Keppra is another tried and tested anti-seizure medication that is not as sedating, is used to treat varying types of seizures, and it comes in IV form. For these reasons we are going to be adding this med to Lucy’s already long list of medications tonight.

I was and still am hesitant to start her on anything new tonight as she has so much going on. Dr. C who is covering for our beloved Dr. R came in this evening to discuss with me our options, she really helped me to make this decision for our girl. She believes that the combination of the two anti-seizure medications are what Lucy needs. We are going to be starting Lucy on a loading dose of keppra tonight, and throughout the weekend we are going to be monitoring her to ensure that the combination of these two drugs is working. Yes, you read correctly, we are here for the weekend.

Drew and the kids came over for dinner tonight, unfortunately Lucy was not awake. This made the kids sad, and Lucy even sadder when she finally did wake up at 9:3o pm tonight asking, “when the kids coming?”. Lucy and I called the kids and they said good night and I love you over the phone, be still my mama heart. We are looking forward to seeing everyone tomorrow night.

Sleeping over…

2012-02-16T00:22:00.001-05:00

We are in a room on our regular floor admitted for observation of seizures. Since giving Lucy her phenobarbital dose for the night we have not seen any more seizure episodes. Prior to that we had several episodes that were observed by the admitting team. We are very concerned that she is having a paradoxical reaction to the methadone…ugh. She is extremely agitated and irritable one moment, and then distant and nonresponsive the next. We were very hopeful that methadone would be a great med for Lucy since it is effective at controlling neuropathic pain. I am looking forward to meeting directly with our palliative care doctor tomorrow and discussing things. Not sure if I mentioned this, but our main doc is out of the country(he promises this is the last time for a long time) until February 25th which adds so much more stress to our already stressful life. The doctor that is covering Lucy for him knows our girl well, but apparently didn’t know she was starting methadone yesterday. I wonder how that detail was forgotten? I am not sure I am looking forward to seeing her as much in the morning, but we do need to talk. Hopefully we all come up with some sort of plan to get us to the 25th.

Thanks for all of your thoughts and prayers, love and support! Drew went home to get somewhat of a decent nights sleep and also so that he can bring me a change of clothes and some things to shower with in the morning. The kids are all snuggled in bed at our friends the Puff’s house, and Lucy and I are being well taken care of here at the hotel DuPont.

Too many things…

2012-02-15T18:21:00.001-05:00

Times have been tough for our little one, I believe that is the easiest way to sum things up. This afternoon Lucy was taken to the hospital via ambulance for cluster seizures lasting longer than ten minutes. These continued for the duration of our ride over here. For those of you who have ever had a child or seen a child seize, a second feels like an hour, a minute a year, and well ten minutes an eternity. Like I mentioned in pervious posts, seizures are not something new for Lucy, but the frequency and duration of these seizures are new for our girl. She has been even more irritable and agitated the past few weeks but especially the past few days. We started Lucy on phenobarbital on Saturday and methadone yesterday. Is it med related, surly we are suspicious, we don’t really know yet. There are too many things happening at once, as usual, to say what it is that is causing this. For now she is sleeping, seizing like she did is extremely fatiguing, and when she is awake she is irritable. I will update when we know more.

Today…

2012-02-10T18:38:00.001-05:00

As I sit here, blood is infusing ever so slowly into Lucy so as not to fluid overload her. She is finally sleeping, something that has been scarce for her this week. We are praying that this is the boost her body so desperately needs, but I have been told that even though this will help this is not going to “fix” her. I know this in my mind but in my heart is another matter. Her baseline is changing, I am hesitant to say what her norm is, what was normal last week is different this week.

Cardiology finally chimed in on all areas of Miss Lucy’s life today which officially got the ball rolling on many things. We are going to be starting Lucy on some pretty heavy duty meds for pain control, autonomic control, and seizure control. Lucy has learned to live with a level of pain that is beyond our comprehension. Acute pain is one thing, chronic pain is another. As her parents we too have learned to live with a level of Lucy’s pain. Our hospice team and doctors very delicately pointed out to us that Lucy deserves to be comfortable, and that as difficult as it is going to be to find the right balance of meds, she needs them. Our biggest fear is perhaps loosing the part of Lucy that makes her Lucy. The very real reality is that we need to do something for her and we don’t know what that something is going to do to her until we actually do it.

Our appointment with neurology was pretty straight forward. Lucy’s previous eegs have demonstrated that she has seizures so there really is no need to repeat an eeg, we just need to decided whether or not we are going to start seizure meds, and might I just add that our options for meds are very few. Drew and I left our appointment with neurology and parted ways, he went off to work and Lucy and I headed to day med. We kissed good-bye and said that we will discuss “stuff” later. As Lucy and I were settling into day med we were greeted by our palliative team and Dr. R, they were all coming to discuss “stuff”, so much for doing it later. Decisions were made that took some convincing on my behalf , but are clearly in Lucy’s best interest. We are going to be starting Lucy on phenobarbital tonight because it is an effect seizure med, pain med, and helps with autonomic instability, all areas that she is in need of help with. In a few days we are going to be adding another med for pain control that will hopefully be a great med for for neuropathic pain relief.

I want to thank all of you for thoughts and prayers, love and support. I also want to say a special thanks to all of you who donate blood, without you our little one and so many others would not be alive. Our prayers are for little to no med reactions, negative cultures, and that Lucy is comfortable. If you can also find it in your prayers to pray for our three other loves in our life that they too have peace and comfort with all that is going on in our lives.

Worried…

2012-02-09T23:48:00.001-05:00

You’ve heard the saying “worry does not empty tomorrow of all its troubles, but empties today of all it’s strength”. I’m not really sure who is famous for stating this, but I wonder if this person had a child with a chronic progressive degenerative disease that has them wondering at all hours of the day and night what on earth is going on with their child. I had strength beyond my comprehension today, and yet I am so worried about our girl. Something is wrong, different, changing.

We had a long appointment with our main doc today, which in the grand scheme of things did not amount to any big changes yet, just a lot of what if’s. We are scheduled to see a new neurologist tomorrow one who specializes in seizure disorders. When Lucy was a teeny tiny baby this neurologist was one of the first doctors who clued us in that there was something going on with our little one, but steered us toward a neurologist who specialized in neuromuscular diseases. Ironically our neuromuscular neurologist is steering us back to him. Seizures are not something new in Lucy’s life, but appear to be making a come back.

We are scheduled for a blood transfusion tomorrow, labs and cultures will also be drawn as Lucy is teetering on the fence of a fever. I am worried that she is brewing something, add to that worry that our main doc is going to be away for the next two weeks, and I believe that my worry makes me a stronger advocate for our daughter.

Where I Last Left Off…

2012-02-08T20:48:00.001-05:00

I apologize for my lack of updates, time is just not on my side these days. We appreciate so much the love and support that we receive from all of you who know and love us either from the words here on this blog or in real life!

When I last wrote I shared with you that there is a fungus among us. After several conversations with our main doc it was decided that we needed to investigate other options for treating Lucy’s yeast issues. Systemic/IV antifungals are tough on the body, most of them come with a list of side effects that are concerning for anyone, but even more so for Lucy. Her sensitivities to medications are limiting our choices, as well as her failing GI system and liver disease. Lucy is in intestinal failure, she has little to no function of her GI system and as a result she is unable to take in and absorb meds through her GI tract. Yet it is her GI system that is overgrowing with bacteria and yeast. We need to continue to try and treat the yeast and bacteria in an effort to keep it from translocating from her intestines into her blood stream putting her at risk for life threating infections. It is believed that Lucy’s poor immune function is playing a significant role in her reoccurring yeast infections, these types of reoccurring fungal infections are most commonly found in severely immune compromised individuals such as bone marrow transplant patients, or HIV infected patients, and Lucy. After consulting with some of the other docs in the diagnostic referral service, Dr. R talked with me about putting Lucy on amphotericin B orally. Amphotericin B is typically an IV antifungal that is know to have serious side effects when administered IV, it’s nicknamed ampho-terrible. Interestingly enough when given orally, or in our case via her g and j tubes and swabbing her mouth with it, it should have little to no side effects because it does not get absorbed systemically. Though not common, we agreed to treating her this way because, frankly, all of our other options come with a long list of side effects that will exasperate many of her existing symptoms and create bigger problems . Treating Lucy with an “oral” medication means we are taking a gamble that her failing intestines will be able to take in the very small volumes(.5mls) of ampho and move it through her GI system thus coming in contact with the yeast and eradicating it. In theory this should work; however, any long time reader of this blog knows that Lucy does not follow theory. Most if not all of the meds we are pushing down her tubes are pouring back into her drainage bags no matter how long I clamp her for. We are continuing to research options for systemic antifungals.

As for the yeast in her mouth that does appear to be improving ever so slightly. I am going to venture a guess that oral ampho is perhaps one of the worst tasting medications. Although Lucy hates the taste of the med, she is surprising us with her cooperation in allowing us to swab her mouth with it every eight hours. When we first started swabbing her mouth I said half kidding that there are not enough smelly stickers in the tri-state area to bribe Lucy with for the next two weeks, but she is realizing that it is helping to get rid of the very thick and pasty yeast that has plagued her mouth for months. She never ceases to amaze us with her ability to tolerate a new therapy, intervention, or medication. Once she realizes how much better she feels she is willing to allow us to do it, although not without tears and some back arching, this is both comforting and heart breaking.

The last few weeks have been riddled with worry, if it’s not one things it’s ten more things. Last week her urine had a strange odor to it which prompted me to ask our doc to have it cultured. On the same day, one of the lumens on her central line clotted off for the second time in two weeks. We did a dye study on her line but did not find anything obvious that could be causing the line to occlude. I was not really surprised since seconds before injecting the dye into her line for the study I flushed a med through it first and lo and behold it started flowing like a river. One sure way to fix a line…go through the hassle of scheduling a dye study. We did cultures of her line as well as because we are always suspicious when we have line troubles. Her line cultures have no growth as of today, which is a good thing, but her urine cultures grew positive for an unusual bacteria that is not a typical UTI bacteria found in the bladder but rather in the bowel. Lucy did not present with a high fever which is odd since she has a pretty significant infection, this has us all a little concerned. She was one miserable girly this weekend, which makes sense now that we know about the UTI. We started her on IV bacterium to treat the infection, which is now only going to make the yeast issue a bigger issue…ugh, around and around we go!

Lucy is in need of blood, her counts have dropped pretty significantly in the last few weeks. We are scheduled to have this done on Friday. She is also in the midst of another autonomic storm which has affected her sleeping and mine too, motility or lack there of, body temperatures, and heart rates. She has us all on a roller coaster wondering what on earth we are going to do for her. Talks between Dr. R and our cardiologist are being had, one of our frustrations is whether or not we can really do anything for Lucy cardiac wise. We are looking for some guidance as to how high is too high for her heart rates and how long is too long to let them be high. Pain continues to be a growing issue, Drew and I witness on a daily basis just how strong our little one is when it comes to pain. On Monday night we had her laying between us in bed, she was twitching and shaking she was in so much pain, but was not crying she just wanted us to lay with her.

It has been a tough few weeks around here, but we will get through them. We are scheduled to be at DuPont the next two days for what is going to amount to be loooooong days. We are in the midst of making some changes to her current meds, looking for more effective pain management.

My hope is to update more often about the everyday stuff that happens in our life, it’s just that the everyday stuff happens to be everything I have just written and then some. The then some part will have to wait for another day. Thanks as always for your thoughts and prayers, love and support, we could not do this alone!

What’s been going on…

2012-01-23T08:41:00.001-05:00

This post is for all you who have been wondering what on earth has been going on with All the Marletts…thanks for caring.

If our main doc got paid for each and every e-mail he receives and sends about Lucy he just might have enough to retire, but then we would be without him, and that would be no good! Last night I sent an e-mail to our good doc who just so happens to be in South America helping another family in need, yeah he is amazing like that! He e-mailed me back saying that he is “concerned”, and then discussed a plan that he was going to relay back to the other doctors in the group. Concern, concerning, concerned, those three tenses just about sum up Lucy’s past, present, and future health!

I have mentioned on more than a few occasions how Lucy has been battling reoccurring yeast, she is coated with it from head to toe! In particular she has been suffering with severe oral thrush that we just can’t get rid of! After several e-mails, discussions, appointments, and cultures, we learned that Lucy is growing a very rare form of candida yeast that is resistant to the antifungal that we have been using to treat it. Infectious disease was consulted, and it was decided that we need switch Lucy’s antifungal. This type of med change typically requires a stay in the hospital; however, Dr. R was able to get permission from infectious disease to allow us to have her first infusion given in day med. This meant no overnight hospital admission, but if an admission was the only way she was going to be able to get the med, we were willing to do it. That’s how badly she needed to get started on this med! Poor girly, the corners of her lips and and mouth were covered in blisters.

Since starting Lucy on the new anti-fungal on Monday, she has developed quite a few concerning symptoms: diarrhea, fever, tachycardia, erratic heart rates, hypotension, and vasodilation. Why am I surprised(insert sarcasm, fatigue, and worry)? Antifungals are difficult meds to be on, this particular med that Lucy is on came with an eight page packet of side effects. What’s confusing is that our girl already experiences a lot to the side effects of the medication. After talking with the on call doc this weekend, we have decided to do an on-off-on trial with Lucy to see if indeed her symptoms are related to the new med, if her symptoms improve while off of the med then we are going to have to decide whether to continue or change her to a different anti-fungal. Of course our main doc is still out of the country which makes these decisions all the more challenging.

In other issues, because you know there’s always more, we recently had an appointment with pulmonology. Lucy is not new to pulmonology, early on her life we had a need to definitively rule out cystic fibrosis as a possible diagnosis. However we have been referred to a new pulmonologist, one who specializes in neuromuscular diseases. In the past few months we have struggled with seeing a decline in Lucy’s overall muscle strength and vascular health. She is a shallow breather, she has been most of her life and her body has compensated for this up until now. Her need for supplemental oxygen is not going away post transfusion, she visually looks blue at times, and her profusion in her hands and feet are little to none . The thought is that everything that we are seeing in Lucy is as a result of disease progression, and that Lucy is in need of more support respiratory wise. A sleep study was ordered to get a better picture of what is happening to her when she is sleeping, discussions about which meds we can use to help open up and clear out her lungs were had, and orders for chest PT, a.k.a vest therapy, were given. We don’t disagree with any of these recommendations, we feel that they will help to keep Lucy at her best. What is difficult is knowing that we are having discussions about disease progression and not being able to do anything to prevent it! We can treat the symptoms but not the disease.

Happy New Year!

2012-01-15T08:49:00.001-05:00

On your mark, get set, go go go…that is exactly how our year started, not unlike last year, or the year before that…

Our life is busy, of course it is we have four kids! Our days are full of the typical everyday stuff and the the not so typical everyday stuff. Last year at this time I was begging and pleading God for more time. I specifically prayed not just for time, but for more memory making opportunities, and for time to process and reflect. We have a peace about our life this year that we did not have at this time last year. Prayers big and small get answered everyday, and we are blessed again to be beginning another year as a family of six!

We begin every new year off with the celebration of Jack’s birthday, he graced us with his presence eleven years ago on January 4th. They(whoever “they” are) weren’t kidding when they said they grow up fast! Why is it that I feel older on Jack’s birthday? Jack is growing up to become one fine young man, he makes his mama and his daddy so proud.

So many have asked us about our holidays, thanks for asking. We celebrated a wonderful Christmas at home, the six of us and my parents! The week leading up to Christmas was stressful, of course, but not just because I had a million things to accomplish for the holiday, but because Miss Lucy had to go and make things a little exciting, you know with the whole serotonin syndrome business. If I recall the week leading up to Christmas we could be seen at DuPont almost everyday for one reason or another. Lucy got a new GJ-tube(tube #24, but who’s counting) for Christmas as she was in need of one that would drain! Jack happened to get a virus for Christmas this year that landed him in bed for the better part of Christmas and a few days following, not the best present but he has fully recovered back to the Jack we know and love, so all’s well that ends well.

As Drew and I surveyed the presents under our Christmas tree and the stockings all hung by our chimney with care, I was in awe of all that we had accomplished. How and when we had time to communicate with the North Pole I just don’t remember, but I impressed myself this year! All of our kids were happy, which is the best gift any parent can get for Christmas.

Merry Christmas to all…

2011-12-25T23:50:00.001-05:00

and to all a good night!

A quick update…

2011-12-23T08:46:00.002-05:00

So far cultures are still negative, we would be very surprised if that changes. Lucy’s urine culture did come back with bacteria that she has grown before. We believe that her bladder is most likely colonized with the bacteria, we are not really surprised since she has a neurogenic bladder and is cathed several times a day, everyday. We believe that Lucy experienced a very rare side effect, serotonin syndrome, from the medication that she started on Friday. We started Lucy on Lexapro, it is an SSRI-selective serotonin reuptake inhibitor. These types of meds are most commonly used to treat depression, this is not why we were using this med for Lucy. SSRI’s are also used as an effective pain management medicine for individuals with neuropathic pain, the type of pain that we believe Lucy experiences on a daily basis. In theory this would have been a good alternative to narcotic pain relief for Lucy…if it had worked. Serotonin syndrome can be fatal if it is not detected early, the treatment is discontinuation of the medicine and supportive medicine for the resulting symptoms. Lucy only had one dose of the medicine, thank God, we don’t even want to think about what could have happened if she had any more than that. Lucy’s autonomic system is storming due to her adverse reaction to the med; blood pressures, heart rates, body temperatures, are all over the place. Her neurological system took the brunt of the impact, affecting her balance, speech, movements, and causing seizures. As the medication metabolizes through her system we are seeing a decrease in her symptoms.

When discussing Lucy’s symptoms with our main doc he said that he was surprised, but then again not, it’s Lucy! I know I have mentioned this before, you know the fine print that reads less than 1% of people will experience this, that, or the other when taking this medication, that’s Lucy! Our palliative and hospice care team have officially been initiated into Lucy’s medical world.

No news…

2011-12-18T23:35:00.001-05:00

I have not heard from the hospital or our main doc, which means that cultures have not grow anything as of yet, that’s good. The cardiologist on call last night confirmed, based on the EKG they did in the ED ,that Lucy is experiencing sinus tachycardia. We’re not at all surprised with this finding, but what we are wondering is what is causing it. The fear is that it could become more serious, for now we are monitoring her heart rates very closely, and will be in contact with our main doc and cardiologist tomorrow…I see another holter monitor in Lucy’s near future.

We started Lucy on a new med Friday night which we hoped would help her neuropathic pain. Although very rare(for the general population that is, but we are talking about Lucy here so rare is really not that rare), there is a possibility that Lucy’s symptoms are as a result of an adverse reaction to the new medication. Needless to say, we discontinued the med and will be in touch with our palliative and hospice care team to discuss other options for our girly. Lucy’s urine came back with an impressive number of white blood cells in it, indicating infection but we won’t really know until we get the culture back. Perhaps our biggest fear is that the yeast that has overtaken Lucy’s GI tract is circulating in her blood system. Yeast and central lines are not good, not good at all. Yeast is difficult to grow on a culture, it likes to hide and then surprise you with one really sick kiddo. We are praying that this is not it!

Something is up, what it is we just don’t know yet…this is the hardest part of all of this, the not knowing! Once you know what it is, then you can do something!

Twas a week before Christmas…

2011-12-17T19:01:00.001-05:00

And all through our house sounds of Lucy’s heart rate monitor are alarming us all! We are currently sitting in the ED getting labs and cultures drawn. Lucy’s heart rates are high >170bpm, she is sitting right at the cutoff for what we consider a fever, and she looks like a little tomato. On the plus side, she is not really acting “sick”…yet. We spoke with our main doc, who just happens to be on this weekend, and he agreed to have us come in for labs, cultures, and a EKG and then we can go home and wait for him to call. We both agree that this is out of Lucy’s norm, and with Christmas just a week away we are trying to be proactive.

Update:

Six hours later we are still here, in the ED. We are waiting on lab results. Cultures are cooking and Dr. R has convinced the ED docs to let us go, just as soon as we get a few more lab results back. Ugh…time definitely does not fly when you are in the hospital! We are looking forward to picking up our other three kiddos and going to bed. Please pray for negative cultures, and that things calm down with Lucy’s body.

Update:

We arrived home at around 12:30 am. We are going to be keeping a close eye on our girly. Here’s hoping for a good nights sleep, lord knows we could use one!

The Readers Digest version…

2011-12-06T23:30:00.001-05:00

Sad but true, I don’t have enough time during the day to sit down and write a blog post from start to finish. I very often begin posts only to find that days later they remain incomplete. By the time I sit down to finish writing, I am often too tired to complete a thought much less write one down. My lack of blogging is certainly not because of a lack of things to blog about…quite the contrary. Things have been busy in our house…I am hard pressed to think of a time when they have not been. In an effort to catch up, I am going to attempt to write the Readers Digest version of medical events.

Lucy an I are spending our afternoon at the hospital in day-med getting a blood transfusion, which is why I have time to write. Nothing slows me down more than being on hospital time watching blood drip ever so slowly into our little one’s body. Luc is in need of a boost that blood gives her, we are looking forward to rosier cheeks, lower heart rates, and hopefully a decreased need for oxygen. Hematology-wise Lucy has developed some new issues, aside from her reoccurring need for blood, it appears that she has developed a clotting disorder that has us all wondering why. A while back Lucy occluded all four of her IV lines while they were running, to quote our infusion nurse “That’s impressive, but then again it’s Lucy!”. For the last month we have been running tests on Lucy’s blood to help us understand the cause of her clotting issues. Dr. R is consulting with hematology in regards to all of this. On the plus side, we got approval to use TPA ,the medication that is used to break up blood clots, at home which means that when Lucy’s line clots off we no longer have to sit in the emergency room or day med for hours on end waiting for blood to dissolve.

Lucy is battling yeast ~~again~~ still, she is covered in yeast from head to, umm... bottom. For the last month we added oral antifungals to Lucy’s g and j tubes as well as swabbing her mouth with the med in hopes of eradicating the yeast from her entire GI system. We’ve had Lucy on a daily prophylactic IV antifungal since July in hopes of keeping the yeast from entering her bloodstream thus preventing it from getting to her CVL, but it does not work in her stomach and intestines as it does not go through her GI system. Infectious disease has been consulted on this issue because in theory her IV antifungal should be preventing the thrush in Lucy’s mouth. It is thought that Lucy’s poor immune system is playing a role in this issue. We had recent discussions with Dr.R about adding IVIG therapy to Lucy’s list of therapies. In theory this could help to boost Lucy’s immune system, but as we have learned Lucy’s body has other theories.

We have not had any sleepovers in the hospital as of late and for that we are very grateful! That is not to say that you haven't seen us around the hospital, we are here at least once or twice a week sometimes more. In an effort to keep us from sleeping over we continue to increase Lucy’s level of care at home, trying to be proactive. When Lucy had her most recent central line(CVL) placed in October, our surgeon cautioned us that access is an issue for our girl, she is not easy to get a line into, as a result we need to do everything we can to preserve the integrity of this line. In an effort to keep the infections at bay, we began daily ethanol lock therapy on Lucy’s CVL. This has added another step to our daily med schedule that makes things even more complex, our home nursing agency has expressed to us that they are in uncharted waters when it comes to staffing nursing at our house. Lucy’s level of care is A LOT, but because we have all of the help that we do have we are able to continue to live our life at home!

When you have a child like Lucy you are always waiting for the other shoe to drop, so to speak…we know she is going to have another line infection, get sick with another virus, and end up in the hospital again sooner or later. Drew and I have had some very serious discussions about life with Lucy’s team, we all hope to give Lucy “the best longest life”. Mitochondrial disease is a very difficult disease to try and predict, nobody knows what the prognosis is for our girl. Dr. R uses phrases like “she is not heading in the right direction”, or “she is my sickest patient at the moment”, or “Lucy is the one I am most worried about at the moment,”, but he will also be the one to tell you that you never know what can happen. He is a glass half full kind of guy, we often refer to him as our eternal optimist, but even so he continues to tell us that he is worried about our girl. She is very unpredictable, yeah she likes to mess with your mind and heart like that, after all she is a girl. Knowing what we know about Lucy’s health, hoping for the best, and with the support and encouragement of Lucy’s team Drew and I agreed to put Lucy on pediatric hospice. I will write more about this in a later post, but know that this does not mean that we are giving up on treatments, or that we feel that Lucy’s time here on earth is coming to an end. It is our hope that by adding hospice to our team we will be able to provide Lucy, our entire family for that matter, with supportive care in our home environment. I would be lying if I said that this was an easy decision, nothing about Lucy’s life has been easy, but we have some peace and comfort in knowing that we have the resources available to us that hospice can provide.

Thanks as always for your thoughts and prayers, love and support, we could never do this alone.

Baby has a brand new set of wheels…

2011-12-06T08:39:00.001-05:00

Lucy’s new wheelchair is here! She has outgrown her old chair both in size and medical need. Her new chair is bigger, heavier, more comfortable for her (not quite as comfortable for me to push), can support the weight of all of her medical equipment, has an IV pole, is pink, and has her name embroidered in pink letters on her seat to match her chair. This is just a little something special that our wheelchair clinic likes to do for their kiddos The first thing Lucy said when she saw her new wheelchair, “It my name!”, referring to the embroidery on the seat. She just about feel out of her chair the first time she sat in it because she was so excited to show anyone and everyone her name.

Her new chair has both tilt and recline, which means nothing to those of you who have never ordered a wheelchair before, but to those who have this is the reason that Lucy is more comfortable in her new chair, and also why her chair is so heavy(over 100 lbs.). These two features combined mean that Lucy can be positioned in such a way that we are able to reduce the pressure on her belly, thus allowing her to be more comfortable.

Baby now needs a brand new ride for her new set of wheels. We are nearing the end of the process of purchasing a wheelchair-accessible-van. If you thought buying a car was a process, try buying a car with a wheelchair ramp! Having a ramp van is going to make our life a whole lot easier when it comes to transporting our crew, especially Lucy! We had some specific needs for our new wheelchair-accessible-van, mainly adding a fourth seat to the backseat as opposed to just having the third row back seats. Because of this our options for what type of van and where we could purchase our wheelchair-accessible-van was limited and expensive, the ramp conversion costs almost as much as the van!

Our family has been blessed by the kindness, caring, love, support, and generosity of others in so many ways we feel humbled and honored! There are several fundraisers occurring to “Help Us Lift Lucy into Her Van!”. On Friday night The Hockessin Music School, a school that our family has been involved with for the last 10 years, hosted a family music night in honor of our family in hopes of raising some money for the cost of our van. We never expected the outpouring of support that we received, it’s simply… amazing! Additionally, Musikgarten, the music curriculum that our children have learned from, just released a new lullaby CD. Jill, our friend and children’s music teacher, is selling the new CD for $12.00 as an additional fundraiser. She and her husband are donating $5 for every CD sold, her sister who also teaches music classes offered to donate an additional $3 for every CD sold, and two very loving and supportive music school families each agreed to donating another $1! This now means that for every CD sold $10 is going towards the cost of our wheelchair-accessible-van! I was told that at last count 90 CD’s have been sold! If you are interested in purchasing a CD please leave me a comment in the comments section and we will make this happen.

If you live locally, there is another fundraiser occurring this Thursday, December 8th at Wackadoodles Toy Shop. Betsy, the owner of Wackadoodles, heard of our story and very graciously offered to host a fundraiser for our family. I cannot tell you how amazing it is that our family, our life, our littlest one’s heroic struggle with mitochondrial disease has touched the lives of so many. It is every parents hope that their child make a difference in this world, we could never have imagined that ours would be doing this at such young age! We feel so blessed to have the love and support of so many, to be living among those who care so much, to have a community that wants to reach out to those in need…we could never do this alone! Thank you!

Here are the details for the upcoming Wackadoodle’s fundraiser, feel free to spread the word!

Help us lift Lucy into her Van!

Lucy Marlett is a beautiful three year old girl with Mitochondrial disease. Lucy recently moved to a wheel chair that does not fold and weighs over 100 pounds, which makes a wheelchair-accessible-van a necessity!

Here is where you can help. Wackadoodles Toy Shop has graciously offered to donate a percentage of their total sales for one day to the Marlett family to help with the cost of their new wheelchair-accessible-van.

When: You are invited to attend a fun day of shopping at Wackadoodles Toy Shop on Thursday, December 8, 2011.

Where: Wackadoodle’s Toy Shop 61 Jenner’s Village Center West Grove, PA 19390 (Next to Giant) Phone: 610-869-2404

The Details: Wackadoodles Toy Shop will give you 10% off of your total purchase and donate 10% of your total sale to the Marlett family, or you can opt to have Wackadoodles donate the entire 20% or your total sale to the Marlett family. All you need to do is present this coupon to show your support.

This coupon is good for 10% off of your total purchase at Wackadoodles Toy Shop on Thursday, December 8, 2011. Wackadoodles will donate an additional 10% of your total sale to the Marlett family or you can opt to have Wacakadoodles donate the entire 20% of your total sale to the Marlett family.

Total Sale*__________________________________________

*Offer not valid with any other coupons, promotions, or sale items. This offer is valid for in-stock items only. Total sale does not include tax.

Happy Thanksgiving…

2011-11-25T06:04:00.001-05:00

We have so much to be thankful for…

From our house to yours, Happy Thanksgiving!

Matters of the heart…

2011-11-20T23:39:00.001-05:00

A few weeks ago we had an appointment with a cardiologist who specializes in pediatric cardiac arrhythmias, also know as a cardiac electrophysiologist for those of you medical junkies out there. Dr. R likes to ~~harass~~ quiz me on my medical knowledge anytime he can, I have to remind him that I specialize in Lucy, not medicine.

For over a year we have observed changes in Lucy’s cardiac health. She was diagnosed with tachycardia, accelerated heart rhythm, last year around this time in addition to postural orthostatic tachycardia syndrome (POTS), in which Lucy’s blood pressures and heart rates would change over 20% with changes in position. We originally thought, more like hoped, that Lucy’s tachycardia was related to her chronic anemia. If we corrected her anemia then we hoped we would see an improvement in her heart rhythms. Sadly, we did not see much of an improvement in Lucy’s heart rates post transfusion. After wearing a 24 hour Holter monitor, Lucy was diagnosed with having sinus tachycardia, tachycardia resulting from the sinoatrial node due to autonomic dysfunction. Little did we know how chronic Lucy’s anemia was, her bone marrow is affected by her mitochondrial disease and as such does not have the ability to make an adequate amount of blood for Lucy’s body. Couple that with weekly lab draws and daily procedures in which blood is taken from her body, and she loses more than she can make up for on her own. Lucy becomes symptomatic of anemia(accelerated heart rates, neurological changes, and oxygen dependent just to name a few) anytime her hemoglobin falls below 10g/dL, her need for blood transfusions is now about every four to six weeks, sooner with illness.

Changes in Lucy’s blood pressures, dramatic edema, and heart rhythms, had us all scratching our heads trying to figure out what could be going on with our girl. Lucy was diagnosed with hypertension during her acute illness in April/May which has proven to be persistent. Clonidine is working to keep her pressures low. We had nephrology do a full work up on Lucy’s kidneys to rule out kidney disease as a cause for Lucy’s hypertension. Although Lucy’s kidneys are affected from her mitochondrial disease, they are not the cause for her hypertension. In July, Drew and I noticed prolonged periods in which Lucy’s heart rates were even more elevated than her normal(140’s/150’s). These prolonged periods appeared to be causing her some distress as she seemed agitated and was unable to sleep well during these times. After discussing these symptoms with Dr. R and Lucy’s cardiologist, we decided to do another Holter monitor test. The results from this test showed changes from her pervious Holter monitor done in December, it showed that Lucy had multiple episodes of premature ventricular contractions (PVC’s), in which her heart would skip multiple beats in a row. We look for trends in Lucy’s labs or tests that indicate change, since this was a dramatic change from December we decided to monitor Lucy again in eight weeks to compare the data. The results from this Holter monitor showed what we feared, Lucy has ventricular tachycardia, a type of tachycardia that can be fatal. It was at this time that our cardiologist referred us to the Dr. T, who specializes in these types of arrhythmias.

Before our appointment with Dr. T, it was decided that Lucy would need to wear a cardiac event monitor which recorded her heart rates over a thirty day period of time…oh yeah, we took that monitor with us to the beach. As if Lucy does not have enough stuff coming off of her torso, we had to find room to attach three cardiac leads and pager like device which recorded every beat of her heart for THIRTY days. At the end of each day, or whenever the monitor alarmed we had to call into the monitoring station to transmit the data.

On the day of our appointment with Dr, T, he ordered a repeat EKG and echocardiogram of Lucy’s heart to compare it with her previous tests. Our reason for meeting with Dr. T was to discuss with him what, if any, medical interventions could be done to lessen or improve Lucy’s cardiac issues. It is always difficult meeting new doctors at this stage of the game, granted if you are having to see a sub-specialist cardiologist you have to have some pretty significant medical issues occurring. Dr. T is not new to complex medical train wrecks like our daughter, but he is also not an expert in mitochondrial disease. During our appointment Dr. T discussed with us the reasons for Lucy’s tachycardia and other cardiac issues. First off, he confirmed her diagnosis of dysautonomia, adding to it that it is severe! Your autonomic nervous system is responsible for controlling things such as heart rate, body temperature, blood pressure, and digestion all areas that Lucy’s body clearly struggles with. Lucy’s dysautonomia is as a result of her primary mitochondrial myopathy. Secondly, Dr. T also discovered that Lucy’s heart is significantly undersized for her body, but is not atrophying! He told us that this is not an uncommon finding in kids with severe dysautonomia. Lucy’s heart has to pump faster to circulate blood throughout her body, one reason for the tachycardia. Finally, small heart size is not the reason for Lucy’s arrhythmias, these are caused by failures of Lucy’s autonomic nervous system to signal her brain to tell her heart to beat regularly. Lucy’s heart muscle is “normal”, albeit undersized, for lack of a better description. She does not have a cardiomyopathy(muscle disease) which is what most mitochondrial patients with cardiac disease have, instead Lucy’s cardiac issues are as a result of a cardiac neuropathy (nerve disease).

What if anything can be done about all of this? Dr. T told us that there is an experimental surgery which to his knowledge has never been preformed on a child as young as Lucy. This is where Drew and I stopped him, we are not looking for Lucy to become the first of anything in cardiac surgery, our goal for Lucy is to give her the best quality of life that she can have in her time here on this earth. Dr. T was relieved to hear this, he agreed that any experimental surgery would be just that, an experiment. The first creed in medicine is to do no harm, he believes that if we attempt to correct Lucy’s cardiac issues either by surgery or with medications that we could be doing more harm than good. All of the medications used to correct the cardiac issues that Lucy suffers with have serious side effects, and will interfere with many of Lucy's existing metabolic and cardiac issues. We must continue to monitor Lucy’s cardiac issues closely with regular Holter monitors, and bi-annual cardiac event monitoring. Due to the progressive and degenerative nature of mitochondrial disease, it is assumed that Lucy’s cardiac issues are not going to get better.

Drew and I were not expecting miracles from this appointment, and we very much liked Dr. T. We along with Dr. R were “hoping” that something could be done to improve Lucy’s cardiac symptoms with little or no side effects. When discussing this appointment with Dr. R, he commented that it was “disappointing but not surprising”. The decisions for Lucy are increasing in difficulty. As a mama, it is heart breaking to know that this disease is overtaking our little one’s body, one organ system after another.

Letters to Santa…

2011-11-20T15:02:00.001-05:00

Dear Santa,

For Christmas, we would love to be able to give a fraction of what we have been given…

Here’s a wonderful way of showing your support for an organization that is near and dear to our hearts! Thanks to Macy’s for supporting the Make-A-Wish foundation!

What comes around, goes around…

2011-11-06T01:04:00.000-04:00

I am referring to germs, they found us and apparently are tough enough to stand up to the copious amounts of Lysol used in our house. Jack has been sick for the better part of a week, he has been struck with what we thought was strep throat and some sort of upper respiratory virus (para virus) that is lingering and according to our pediatrician has the reputation of causing secondary pneumonia. The poor kid has not been feeling well at all, but I sent him back to school on Wednesday after being on antibiotics for 36 hours, and no fever for 24 hours. He looked awful and had no appetite, but we worry about him getting too far behind. I know, I am a contender for “worst mother of the year” for sure. The kid is smart, really smart, but slow, really slow. Yesterday, he came home from school and it was just the two of us sitting at the kitchen table sharing a bowl of soup. He was so tired he could not hold his head up. He shared with me that he did not feel well, but he liked that it was just the two us at the table. We talked about some very intense stuff, he cried, I cried, and told him that I was happy that he talked with me. I am not sure if it was the fever or the emotions brewing inside, but he needed me and I was so glad that I could be there for him. His fever is better today, but his symptoms are not. I will bring him back to our pediatrician on Monday morning if he is not any better.

Miss Sophie woke us in the middle of the night on Tuesday stating, “I don’t feel good…”, which is code for I am going to throw-up all over you in less than ten seconds. Thankfully she was on Drew’s side of the bed because it is closer to our bathroom. I yelled at him to get her to the bathroom a.s.a.p, since he was in a deep slumber. Just as he got her there, he yelled back to me “good call babe!”. Poor Soph, she practically turned inside out that night. Drew had to leave for work at 4 am for a very early morning meeting which meant that neither he nor I got any sleep. I washed five loads of laundry between the hours of 2 am and 7 am all the while attending to Sophie, doing Lucy’s 5:30 med schedule, and washing and cleaning. Sophie’s GI bug lasted only 24 hours, but it had me doing a subterranean cleaning for 48 hours. She was back to her happy perky self by Thursday morning.

Germs are the enemy in our life! Illnesses surround us this time of year, and we have to try our best to keep them away as any illness can be detrimental to Lucy’s life! Lucy’s immune issues are severe not only because she has mito, but because of the fact that she has secondary hemophagocytic lymphohistiocytosis (HLH), or macrophage activation syndrome (MAS). What happened to her in April as a result of coming in contact with the flu B virus can and most likely will reoccur when her body comes in contact with another virus. It is a difficult balance of living life and living in a bubble. We isolate our kids when they are sick as a way of protecting Lucy, but as you can imagine it is difficult. Lucy wears a mask whenever she is out in public, it always amazes me that she never complains about it, it is her norm. I have tried wearing a mask for an hour or longer, it’s uncomfortable not to mention hot. Our kids are programed to wash their hands the minute they walk in the door, eat, or want to play with their sister. We use copious amounts of soap, hand sanitizer, and Lysol products. We are aware of the literature out there that states anti bacterial products are to blame for the resistance to antibiotics, I don’t disagree that there is probably some truth to those statements. However, in our situation it is our best defense at keeping Lucy “healthy”.

So far Megan has been spared of any illness. She and Sophie share a bedroom, and on Tuesday night Megan woke up and moved herself to our bed saying that she did not want to breath in whatever Sophie was throwing up. Lucy too has not shown us any signs that she has what Jack or Sophie has, but she is “off”. We were at DuPont on Friday for an appointment with the cardiologist who specializes in arrhythmias(I’ll update in another post about this appointment), while we were there Dr. R saw our girl and ordered labs and blood and urine cultures, he agreed she does not look good and is concerned.

We are praying for good health all the way around, that Jack starts feeling better, and that whatever is causing Lucy’s symptoms disappears without a trace.

Thanks as always for your thoughts and prayers.

Trick or Treat…

2011-10-31T23:44:00.001-04:00

A snow storm, an autonomic storm, a pumpkin shortage, a migraine headache, fever, vomiting, and strep throat tried to trick us into not celebrating Halloween his year, but we managed to find the treat in celebrating. No, Lucy is not the sick one (yet), it’s Jack.

Happy Halloween!

An “Off” Day

2011-10-29T22:00:00.001-04:00

We are bound and determined to celebrate Halloween-at- home this year, and we were going to kick off our home Halloween weekend with a hay ride out to the pumpkin patch, but… it’s snowing, it feels more like we should be getting our Christmas tree. I stepped foot into a Target for the first time in months on Thursday looking for wings for a monarch butterfly costume that Sophie very firmly stated she wanted to be for Halloween. Although yesterday she very firmly stated that she wanted to be a “white kitty cat, the very furry kind”, seriously. Guess where I found the costumes… right next to the Christmas decorations, what!?! I already feel behind the eight ball so to speak all of the time, seeing Christmas decorations out so early just doesn’t seem right, anyone else want to rally around this with me? I have been feeling more than ever that I just want life to s-l-o-w down!

I woke up last Sunday morning fully prepared to go to church when I leaned over to Drew and said lets take the kids to Sesame Place instead today. It was a warm and beautiful fall day, perfect “Lucy weather”, he smiled and in an instant plans changed and a day full of memories were made. I am so grateful that we seized the day knowing what I know now.

Lucy is “off”, something is not right with our girl. She looks, for lack of a better word, “sick”. She is sleepier, grumpier, sneezier…her symptoms are similar to those of the seven dwarfs. Her coloring is, well, whiter than the snow covering our front yard. Her body temps and heart rates have all taken a dive down. Clearly her autonomic nervous system is not happy. We are praying that this is just a bad mito day and nothing worse, but my mama radar is saying something is up.

Hopefully the snow melts in time for trick or treating on Monday, if not our kids are all going trick or treating as ski bums.

Beach Memories…

2011-10-25T22:01:00.001-04:00

Lots of memories were made during our beach vacation… it’s hard to believe that it was just a little over a week ago that we were basking in the sun, with the wind at our backs, and sand in between our toes. We thought long and hard about how a vacation like this would work for our family. As you can imagine bringing four children anywhere is challenging, but having one very “special” child, one who can not get wet because she has four IV lines hanging off of her body at any given time, a urinary catheter taped to her leg for most of her day, two drainage bags attached to her g and j tubes, for the next month a cardiac event monitor strapped to her chest, temperature regulation issues, and at times has sensory issues beyond comprehension….well, you can only imagine the challenges that we faced. Lucy’s first time at the actual beach lasted, at best, ten seconds. She was drawn to it however, I think watching “her kids” run in and out of the waves and play in the sand with such enthusiasm intrigued her, despite her nervous system signaling her brain that it was too much. By the end of the week we were ALL able to enjoy the beach for forty-five minutes as a family of six! We rented a house right on the beach so that our three water babies could enjoy the sun, sand, and surf whenever they desired and for as long as they desired, all the while keeping Lucy on her strict schedule. While Lucy napped, the mama read two novels and buried her feet in the sand, the daddy built sand castles and hunted for sea shells, and the kids were thrilled with just being at the beach!

When we left, more than a few tears were shed, and conversations about doing this again next year were had…all signs of a great vacation!

The view from here…

2011-10-14T09:27:00.001-04:00

Enjoy!