Living with Ehlers Danlos Syndrome

I AM MOVING

2019-08-26T22:29:00.002-04:00

My blog is moving to www.justsparklejlm.com! New location, new name, new look...more content!

I hope to see you over there...

Gastroparesis Awareness Month: Smash GP

2019-08-20T00:20:00.002-04:00

August is Gastroparesis Awareness Month.

To understand gastroparesis, you need to have a general understanding of how the digestive system works...

The digestive tract is like a plumbing system. Each part of the digestive tract has its own specific function in digestion. When everything works properly, food travels down the esophagus and into the stomach where it is broken down into small food particles suitable for the small intestine. Once in the small intestine, the muscles work to push the food through all 22 feet and into the large intestine (colon). The process continues until the nutrients have been absorbed and waste has been expelled from the body.

Gastroparesis (GP)- delayed stomach emptying
Literally translated, gastroparesis means "stomach paralysis"

Relating this to a plumbing system...if there is a blockage somewhere in the system and waste is not moving out fast enough, there will undeniably be a backup of waste material. Because it has to go somewhere, it generally comes back up the way it came.

The same concept applies to gastroparesis. If the stomach doesn't empty food in a timely manner (delayed stomach emptying), stomach contents will backup in the digestive tract leading to nausea, vomiting, bloating, feeling of fullness after just a few bites, abdominal pain, belching, lack of appetite, weight loss and malnutrition.

I was diagnosed with GP in 2018 after years of struggling to tolerate food. [To read more about my journey to a diagnosis-- click here]. It is not clear what leads to gastroparesis, though a lot of patients with EDS and/or POTS have delayed motility. I tried diet changes, lifestyle modifications, and every available medication with no success.

Ultimately, due to significant weight loss and malnutrition, it was determined that I needed a feeding tube. Though I was not happy with the decision, it was necessary, and ultimately has given me a piece of my life back. It provided steady nutrition, something my body was missing for 2 years, and has actually allowed me to tolerate more food orally.

Black and Gold is a way of life...

[If you want to learn more about feeding tubes, I wrote about them here.]

As part of Gastroparesis Awareness Month, I took part in the Gastroparesis Pie Face Challenge
[Twitter and Instagram: @gppieface]

Help us raise awareness for gastroparesis by taking part in the GP Pie Face Challenge and upload your video to social media (tagging the accounts above and using #SmashGP)

I nominated three people to complete this challenge:
1. Brad Marchand of the Boston Bruins and my favorite hockey player...#TeamBrad
*As some of you may recall, I met Brad in the hospital in December 2018- to read that story -- click here
2. Dale Arnold, Host of Boston Bruins games on NESN - video of challenge
*Dale is also an author- If These Walls Could Talk: Boston Bruins, Stories from the Boston Bruins Ice, Locker Room, and Press Box
3. Todd Angilly, anthem singer for the Boston Bruins - video of challenge
*Best anthem singer in the NHL - learn more about Todd

Together we can Smash GP

But are we really fine?

2019-07-22T23:16:00.000-04:00

"How are you doing/feeling?"

This is a pretty normal opening question to any conversation, right? For the most part, people respond with either "Good," "okay," "fine," "alright."

A pretty typical answer for me would be "I'm fine, you?" I am sure many people with chronic illness have a similar response.

But are we really fine?

The answer is probably not. For me, I rarely have a "good" day, despite how I may look (which is an entirely different topic that I previously addressed here). A "good" day is really just the best of the bad days. I live with constant pain (yes, every second of every day). I understand this can be difficult to comprehend as I don't typically show that I am in pain. I have become very good at hiding my discomfort and adapting to my baseline. I experience numerous joint dislocations, daily headaches, neurological symptoms, and debilitating fatigue. Some days I don't regulate my blood pressure and heart rate correctly and feel as though I am going to faint. I still struggle with nausea, vomiting, abdominal pain, and bloating with food and tube feeds. This is all part of my "normal." Everyday. For people living with chronic illness, perhaps we have convinced ourselves or accepted the fact that our "fine" is very different from a healthy person's definition of the word. But realistically, does all of that seem "fine?"

So why don't I just tell the truth about how I am doing? Sometimes I just don't want to talk about my health. I live with this reality everyday. I don't want/need to spend all of my energy talking about how I am feeling, especially given I have come to learn that most people really don't want to hear the truth. With or without chronic illness, it's used as a conversation starter and the expected response is "good," "fine," or "okay." Who wants to hear that someone is struggling or in pain?

And truthfully, people are so hung up on how a person "looks" that they have already made their own assumptions on how I am doing and usually ignore any attempts at explaining how I really feel. I cannot even begin to explain how many times I hear, "but you look so good," and when I try to explain that I don't feel as good as I look, I am met with either confusion or more comments about how I must be doing better because I look good. Just because someone doesn't look sick or disabled, doesn't mean that they aren't. Don't get me wrong...I want to look good. I just also want people to understand that the way I look doesn't necessarily correlate with how I actually feel. Yet the two seem to go hand in hand, and the concept of invisible illness seems impossible to understand for those who do not live with chronic health problems.

[Again, if you want to read more about invisible illness I wrote about that here].

Sure, there are those people (largely family and close friends) that genuinely want an update on how I am feeling; although, I am not always forthcoming with them either. Even family and friends don't really want to hear that you're not feeling well all the time...for a variety of reasons. For one, it's not a positive conversation and people tend to prefer when the mood is light. That's not to say that sharing my symptoms is being negative. It's not. Saying that I have a headache or pain is a fact. It's part of my reality. It's just not the positive and light discussion that most people are hoping for and often makes people sad/upset. It's also awkward and uncomfortable when someone is expecting you to answer with the normal, mainstream response of "good" and you hit them with your most recent symptoms or overwhelming list of daily struggles.

Because I do not like disappointing people or making people uncomfortable, I often just keep my answers short and sweet or throw in some sarcastic humor.

Well, I'm alive.

Hanging in there.

Same as always.

I'm fine.

...but the reality is very different.

Jillie Beanz Gifts

2019-07-12T11:34:00.002-04:00

Jillie Beanz Gifts is back! I first opened my shop on Etsy in 2016 where I sold embroidered kitchen towels! Following some health challenges, I closed my shop indefinitely; however, I have decided to reopen on a new platform, Instagram (@jilliebeanzgifts). When I first created Jillie Beanz Gifts, I wanted there to be a connection to my dad, who passed away in early 2016. I chose Jillie Bean, as that is what my dad always called me. I will be working to post towel designs in the coming days and weeks. Most of my old designs are already posted. There are some new ones as well that are available or coming soon. I am also working on creating a FB page for those that don’t have Instagram. Feel free to send me a message on IG @jilliebeanzgifts or at jilliebeanzgifts@gmail.com if you have any question or want to place an order. My towels make great housewarming and holiday gifts!

Black and Gold, Always!

2019-06-13T16:57:00.001-04:00

Its hard to describe the emotions of falling just short of the ultimate goal when you put so much of your heart and soul into it. The Bruins are my biggest passion. They are my lifeline. They keep me going in my darkest moments. While the season didn’t end the way we had hoped, it was truly special. I was reminded today of the friendships I have made this year and the experiences I was able to share with my favorite people. I have no doubt we will be back. And I will be waiting, albeit impatiently, for the puck to drop next season. Until then, I will continue to bleed black and gold. I will always love this team and the city of Boston. Thank you Brad, Katrina, and the Bruins organization for the most amazing season. We will bring the cup back to Boston. I know it. I love you all 💛🖤

EDS Awareness Month: Answering Your Questions! PART TWO

2019-05-10T16:40:00.003-04:00

Because connective tissue can be found all throughout the body, there are a lot of secondary or associated conditions that are believed to be connected to EDS--Dysautonomia (POTS, orthostatic hypotension, neurocardiogenic syncope), Chiari Malformation, asthma, heart complications (Mitral Valve Prolapse), Mast Cell Activation Syndrome (MCAS), gastroparesis and other GI motility challenges---and the list continues.

Comorbidities

What other conditions do you have?

In addition to EDS, I have Chiari Malformation, craniocervical instability, neurocardiogenic syncope and POTS, bilateral acetabular hip dysplasia (corrected in a procedure called Periacetabular Osteotomy--PAO), gastroparesis and delayed intestinal motility, Hashimoto's, Graves', autoimmune neutropenia, and complex regional pain syndrome (CRPS).

POTS

What is POTS?

Postural Orthostatic Tachycardia Syndrome (POTS) is a form of dysautonomia (autonomic dysfunction) and a form of orthostatic intolerance. Dysautonomia (autonomic dysfunction) is a term used to describe any disorder/disease/malfunction of the autonomic nervous system. It is associated with Ehlers Danlos Syndrome.

The autonomic nervous system controls functions that we do not consciously think about--blood pressure, heart rate, respiration rate, digestion, etc.

POTS is characterized by an increase in heart rate (tachycardia) of at least 30bpm or more or >120bpm within the first 10 minutes of standing in the absence of orthostatic hypotension.

This is a fantastic video that explains POTS in a simplified way, as disorders of the autonomic nervous system are incredibly complex.

Can you explain what its like to live with POTS? How does it affect you?

POTS is exhausting and unpredictable. Living with POTS is like running a marathon that never ends. My body has to work 3x harder when I am upright as compared to a healthy individual. It leaves my body with very little reserve. It can feel like a never-ending flu. I feel dizzy, lightheaded, weak, shaky, nauseous, and painfully exhausted every single day. I have good days and bad days, but symptoms are always present and can hit at unexpected/unpredictable times.

What is the treatment for POTS? How do you treat your POTS?

POTS can be very difficult to treat/manage. There is no cure, so the goal is to alleviate/manage symptoms. Every patient is different. Treatments for POTS include increasing fluid intake, increasing salt consumption, wearing compression stockings, raising the head of the bed, and graduated exercise. If non-pharmacological methods are not enough, there are a number of different medications that people take to decrease heart rate and/or increase blood pressure.

I went through the standard process of treatment. I started with all of the methods listed above; however, I reached a point where I struggled to get out of bed without fainting and medications were not effective enough. Due to low blood volume, I receive IV fluid 3x/week through a port-a-cath (central line). Though it is not a cure and I still experience symptoms, IV fluid has been the single most effective treatment for me. The use of a central line and IV hydration should be the LAST RESORT as there are risks involved.

Gastroparesis

What is gastroparesis?

Gastroparesis (GP) = delayed stomach emptying. Delayed stomach emptying leads to a backup in the digestive tract and symptoms of nausea, vomiting, bloating, feeling of fullness after just a few bites, abdominal pain, belching, lack of appetite, weight loss and malnutrition.

Literally translated, gastroparesis means "stomach paralysis."

Can you eat?

Yes, I can eat. However, I do not tolerate enough food orally to sustain myself from a nutritional and caloric standpoint. As a result, I have a feeding tube. To learn more about feeding tubes click here.

How is it treated?

Everyone is different. Treatment varies from diet changes to medications to feeding tubes to surgical intervention and gastric pacing.

Chiari and CCI

What is Chiari Malformation?

Chiari Malformation is a neurological disorder where part of the brain (the cerebellar tonsils) herniates or descends out of the skull into the spinal canal. This results in compression of the spinal cord and brain stem. As a result, this compression disrupts the normal flow of cerebrospinal fluid (CSF) leading to disabling neurological symptoms (headaches, occipital neuralgia, numbness and tingling in hands and feet, weakness, nausea/vomiting, dizziness, low blood pressure, and many more).

How were you diagnosed with Chiari?

In 2010, I experienced a CSF leak from an epidural I had placed for hip surgery. This resulted in the most debilitating post-dural puncture headaches. Despite returning to the operating room to have a blood patch to resolve the CSF leak, my headaches never went away. Eventually, a brain MRI confirmed an 8.5mm herniation.

Symptoms of Chiari Malformation are much more than just headaches

Is there a treatment for Chiari Malformation? Is there a cure?

There is no cure. Treatment focuses on alleviating symptoms. This may include medication and/or surgery to relieve the compression on the brainstem and spinal cord. I underwent a brain surgery (posterior fossa decompression) in 2014.

To learn more about my journey with Chiari Malformation you can watch my video:

What is CCI?

CCI stands for craniocervical instability. It is a structural instability of the craniocervical junction, or where the skull and spine meet. This is often seen in conjunction with Chiari Malformation in patients with Ehlers-Danlos Syndrome. Treatment includes the use of a cervical collar, physical therapy (by an EDS knowledgable PT), or a craniocervical fusion.

Concerns about family members

Diet tips for people with POTS?

With POTS it is often common and recommended to increase fluid and salt intake. This helps boost blood volume because where salt goes, water follows. As I am not a physician and cannot offer medical advice, any concerns about dietary changes or the use of multivitamins should be discussed with your doctor.

EDS Awareness Month: Answering Your Questions! PART ONE

2019-05-02T00:08:00.001-04:00

Last month I asked people to send me their questions about EDS and related conditions...you asked some really great questions!

Due to the number of questions and length of some answers, I have separated the questions into 2 posts. Below are the answers to part one!

General EDS Questions

What is EDS?

The Ehlers-Danlos syndromes (EDS) are a group of genetic connective tissue disorders. Connective tissue is found in about 80% of the human body and is made up of proteins that support skin, bones, blood vessels, and other organs. EDS is characterized by joint hypermobility, skin hyperextensibility, and tissue fragility. There are currently 13 subtypes. The specific gene mutations are known for all but one subtype- hypermobile EDS (hEDS). The genetic cause varies depending on the particular subtype, but EDS often involves a defect in the protein collagen. Collagen is the glue that holds the body together.

People with EDS present with joint, skin, and systemic problems. The hypermobile joints are unstable and prone to frequent dislocations and subluxations (partial dislocations). This leads to widespread musculoskeletal pain and early onset of osteoarthritis. People with EDS often have very soft velvety-like skin that is hyperextensible and fragile. The skin may tear or bruise easily, resulting in severe scarring, and slow and poor wound healing.

Depending on the subtype, people with EDS are also more prone to arterial, intestinal, and uterine fragility or rupture, scoliosis, poor muscle tone, and mitral valve prolapse.

EDS is a spectrum disorder. The severity varies from person to person. Some are only mildly affected, while others are completely disabled. No two people with EDS present in exactly the same way, making it incredibly difficult to diagnose and manage. For some, EDS is progressive, with symptoms worsening over time.

What is the treatment for EDS? Is there a treatment?

Treatment plans are individualized and focus on managing symptoms, which are widespread and varied. Treatment for EDS may consist of surgery, physical therapy, and bracing or splinting. Making treatment and management of EDS complicated are the comorbid conditions. I will cover questions related to the associated conditions in part two.

Surgery may involve a particular joint with the goal of stabilizing it as best as possible. This often requires creative thinking from the surgical team and the use of allografts (a tissue graft from a donor of the same species as the recipient but not genetically identical.) Joint fusions may be necessary as a last resort. There are a number of non-orthopedic surgeries that are sometimes required as well due to the comorbid/associated conditions (neurosurgery, cardiovascular surgery, GI surgery, etc.).

PT is incredibly important in EDS, but it is also crucial to find a physical therapist that understands EDS. Often times traditional exercises are not effective or safe. Many with EDS also have comorbid conditions that need to be considered as part of the PT treatment plan as well. The goal of PT is to increase muscle strength to better stabilize joints, address muscular imbalances, and improve proprioception.

Bracing and splinting provides additional support and stability to unstable joints. However, the downside to braces/splints is that it can also lead to muscle atrophy, which leads to more instability.

Is there a cure (or one currently being worked on)?

Unfortunately, to date, there is no cure for any of the 13 subtypes of EDS. One of the reasons I am so passionate about raising awareness and helping people better understand EDS and related conditions is awareness leads to more funding and research. Without research, there will be no cure.

What are the differences between the 13 subtypes?

Each subtype has its own set of clinical criteria; however, there are similarities between the different types. Similarities include joint, skin, and systemic manifestations consistent with connective tissue disorders. To learn more about the differences click here!

Is EDS painful?

Yes, for me it is! I deal with anywhere from 1 to 20+ joint dislocations/subluxations every day. Dislocations and subluxations lead to significant joint pain as a result of micro and macro trauma to the surrounding nerves, soft tissue, and bone structures. I experience pain every minute of every day.

What is the difference between dislocations and subluxations?

A dislocation is a "displacement of a bone from its natural position in the joint." This occurs when the two bones that form a joint fully separate from each other. It requires reduction, or manipulation to relocate the bones back to their proper position.

Anterior shoulder dislocation

A subluxation is a partial dislocation in which the bones begin to separate, though not completely. A subluxation can be just as painful as a dislocation and can even be more difficult to address due to widespread muscular imbalances as the body tries to compensate.

Medial kneecap subluxation

I know someone who is "double jointed." Is that the same thing as EDS?

First, there is no such thing as being "double jointed." The proper term is joint hypermobility (joints that move beyond normal/expected range). Joint hypermobility is a characteristic of EDS; however, just because someone is hypermobile, does not mean that they have EDS. Additional clinical criteria must be met before a diagnosis of EDS can be made. EDS is much more than just hypermobile joints. Many people with EDS live with a number of different chronic illnesses that affect multiple systems (all due to faulty connective tissue).

EDS Research and Awareness

How has the general awareness of EDS changed since you were first diagnosed? Has it gotten better or worse?

Great question! Awareness of EDS has certainly improved since my initial diagnosis in 2010. When I was diagnosed at 19 years old, hardly anyone knew what EDS was (including healthcare providers). I often had to spell it out for my doctors just so that they could "Google it." This made it even more difficult to be accurately diagnosed, much less to find a team that understood how best to manage the many manifestations and widespread systemic problems. While it is still not widely known or understood, it is slightly more recognizable in healthcare; that is my experience at least. However, we are nowhere near where we need to be in regards to awareness. There is not enough research. There is not enough expertise. This needs to change.

In 2017, the clinical criteria were updated and new research/literature was released. This also led to new educational resources for healthcare providers and the general public. My hope is that awareness of EDS continues to improve. Increased awareness leads to more research. Without research, we have no hope for a cure.

Life with EDS

When and how were you diagnosed?

I was diagnosed in 2010 at 19 years old (just before turning 20). I was recovering from major reconstructive hip surgery (as a result of hip dysplasia) and due to immobility from the surgery (being unable to weight bear through my leg), my quad muscles atrophied. This led to spontaneous kneecap dislocations every day. I also struggled with near fainting episodes during that time frame as well. Ultimately, my surgeons referred me to genetics due to the spontaneous kneecap dislocations as well as my past history of gymnastics injuries. I was diagnosed based on clinical examination with hypermobile EDS; however, it is now suspected that I have classical EDS (cEDS).

How do you manage dislocations and EDS?

EDS: First and foremost, physical therapy. I have been in physical therapy for the last 10+ years. The treatment plan and techniques used depend on the particular area we are working on. The goals, as stated above, are to increase strength and stability, improve proprioception, and manage muscular imbalances and dislocations/subluxations. I also utilize braces/splints to provide additional support, and use mobility aids regularly (wheelchair and/or crutches). Though there is no perfect solution and surgery is not a cure, sometimes we reach a point where my quality of life and function with a particular joint is severely impacted and surgery is the best option.

Dislocations: For me, the first step in managing a dislocation is to reduce it (or relocate the joint back to its "normal" position). Sometimes this is easy, and other times it just does not seem to go back in correctly. Depending on the severity of the joint dislocation, I either do nothing (after it is relocated), or I brace/splint/immobilize the joint until the surrounding soft tissue has a chance to settle down. Occasionally I require assistance from my PT to address muscular imbalances that occur as a result of a dislocation/subluxation.

How will EDS affect you in the future?

It is difficult to predict how EDS will affect my future as both EDS and chronic illness are truly unpredictable. I can't predict what my body is going to do 10 minutes from now, so how can I predict my future? With that said, EDS can be progressive. It has been for me. I have watched my health decline over the last 4-5 years and I have not been able to regain any sense of stability. So while I would welcome any improvement, realistically, with a chronic and progressive disorder, it is unlikely that my health improves significantly. This reality is stressful and overwhelming at times because it makes planning my future even more challenging. I want to be able to support myself and be independent. I want to be able to contribute to society in a meaningful way. Unfortunately, with no ability to plan, I do not know what my future will hold.

What is one thing you want someone without EDS to know about EDS or how it impacts your life?

In a perfect world, friends and family (and the general public) would understand everything. But that’s unrealistic. I think the ONE thing I want someone without EDS to know about EDS/how it impacts my life is that it's lifelong. EDS is a genetic condition. I was born with it. It is chronic and progressive. It is not going away. I won’t wake up tomorrow and be better. Surgery isn’t a cure. Physical therapy isn’t a cure. Bracing and splinting is really just a band-aid. There is no medication that will fix this. There is NO cure. The concept of chronic and progressive illnesses seems to baffle people. They really struggle with the idea that there are health conditions that exist without a starting point and an end.

There are so many things I wish people understood. It is not possible to cover them all. It is also not realistic for me to expect people to understand what it is like to live with chronic, unpredictable, progressive illnesses. That is something that you only truly understand when you are living it. I am blessed with family and friends who want to understand and for that, I am forever grateful. Not everyone is as lucky to be surrounded by a wonderful support system.

What is the hardest/worst part about having EDS?

I think the unpredictability of EDS and chronic illness is the hardest part. Every day is different. I have very little control over what my body does on any given day and that makes it very difficult to plan too far in advance. I often feel guilty canceling plans last minute, and sometimes push myself more than I should because of this guilt. For someone who likes to be in control of situations, I have very little control.

What is the biggest lesson you have learned from EDS?

EDS has taught me so much. It has taught me how to adapt and how to live despite my illness and altered reality. It has taught me to persevere and to trust my instincts and fight for what I believe in. Everyday with EDS I learn. I learn more about myself, my abilities, and my strengths.

My life with EDS and chronic illness is not the one I had envisioned for myself. I had envisioned a life in Boston, working as a nurse at my favorite hospital (the hospital that has done so much for me). I envisioned pursuing a Master's in Case Management and/or Genetics to help others like myself navigate a complicated system. I had hoped to make a positive difference in people's lives. That is not my reality though. So the biggest lesson I have learned is how to adapt to life's circumstances to redefine myself, my goals, and my dreams.

How does EDS effect people with respect to their work life? Are people with EDS able to manage to have jobs/careers?

EDS is a spectrum disorder. Some are only mildly affected, while others are completely disabled. Some are able to work full-time with very little difficulty. Others are able to work part-time. Yet, some may not be able to work at all.

I am a Registered Nurse. I worked incredibly hard for my license; however, unfortunately, I am unable to work at this time. I do volunteer as a nurse at a barn that provides programs for special needs children. This allows me to feel purposeful.

My health changes constantly. I live with unrelenting pain and fatigue. In addition to EDS, I also have Chiari Malformation, craniocervical instability, neurocardiogenic syncope and POTS (autonomic dysfunction), gastroparesis and delayed intestinal motility, Hashimoto's, Graves', autoimmune neutropenia, and complex regional pain syndrome (CRPS). With the unpredictability of having so many chronic illnesses, I never know how I am going to feel on a given day or week. It is a frustrating reality, as I want nothing more than to be able to support myself and contribute to society.

What aids do you use regularly? Do you need to use mobility aids and braces consistently?

Due to joint dislocations, joint pain, fatigue, and autonomic dysfunction (POTS) I use a variety of different mobility aids depending on my situation. Sometimes I require a wheelchair for longer outings or circumstances that would require me being on my feet for too long. Due to POTS, I cannot stand for extended periods of time without pre-syncopal/syncopal (fainting) episodes. I am also struggling with right hip dislocations/instability that make weight bearing through my leg very difficult. I am a high fall risk as a result, and a fall leads to other dislocations and injuries.

For shorter outings, I use forearm crutches. The challenge with crutches, however, is that my right shoulder is incredibly unstable and often dislocates when I apply too much weight through my arm. This can also lead to falls, and with joints that don't stay in place, falls are less than ideal.

My wheelchair allows me to do fun things with family and friends!

My Journey with Ehlers-Danlos Syndrome

2019-05-01T00:01:00.000-04:00

*My experiences are my own. Everyone is different. This is a snapshot of my journey. It is not my entire journey.

The Reality of Chronic Illness

2019-04-12T18:01:00.002-04:00

Last night I spent my birthday at the Bruins game with my brother. Anyone that knows me knows that there is quite literally no place I would rather be than at the Garden watching my favorite team play. To be able to do so on my birthday was even more special (even though they didn't play well and lost).

However, the game last night was just another reminder that I pay for almost everything I do. For me that involves increased pain and debilitating fatigue. Sometimes it means a setback in other aspects of my health as well (which may present several days later). It's just the reality of chronic illness.

So why do I still do it? Because it beats the hell out of spending everyday doing nothing (out of fear of a setback or an increase in symptoms) and wasting my life away. Life is happening right now, and there are no guarantees. With a progressive disorder, I can't simply wait for my health to improve before I live my life. In reality, my health likely will never improve all that much. My baseline or "normal" changes regularly but generally trends in the wrong direction.

It can be difficult to find a balance of when to push yourself and when to take a step back and let yourself rest. Sometimes I do have to say "no" or cancel plans last minute. However, if you are passionate about something and realize that there will be consequences later, you find a way to make it work. Sometimes that means utilizing resources that will make it a little bit easier. For me that means using my wheelchair, parking as close as possible (handicapped parking), and asking for help when necessary (which I must admit I am terrible at doing). It's part of accepting life with chronic illness. But at the end of the day, we are only given one life. It's now or never.

Win or Lose...I love my Bruins!

Today I spent the day resting so that I can get back out there on Saturday to cheer on my favorite team. Time to even up the series! Let's go Bruins!

What does Awareness mean to me?

2019-03-31T17:27:00.001-04:00

MAY is EDS Awareness Month...I realize that is a month away.

I am working on some awareness "projects" that I will share on my blog, Twitter, and Instagram. One project is a video of my journey with EDS.

Last year I took part in the EDS Awareness Challenge. I answered questions each day about living with EDS. This year I am opening it up to questions from you.

What do you want to know about EDS and related disorders? The questions can be simple or more complex. They can be related to EDS in general, or my life with EDS and how it impacts me.

You can use the email button to send me an email with questions or awareness suggestions. You can also send me a direct message on Instagram (medicalunicornmurph) and I will address all questions in May.

Invisible Illness

2019-03-22T20:45:00.002-04:00

I had no intention of writing this post, but following a comment made last night I felt the need to make this clear.

JUST BECAUSE SOMEONE DOES NOT LOOK SICK OR DISABLED, DOES NOT MEAN THAT THEY AREN'T....

So what prompted this?

I parked in a handicap spot at the Bruins/Devils game at the Prudential Center in Newark, NJ last night (3/21/19). My handicap placard was visible, and as a result I was instructed to pull into a separate handicap parking section. It was conveniently located on the same level as the bridge that takes you into the Prudential Center from the attached parking garage.

I arrived early, as I always do, so there were only a few other cars in this particular section (no more than 4). As I was sitting in my car, making sure that I had everything I needed for the game -- phone with mobile ticket, wallet, emergency medications, etc. (I will probably write a post later about planning for an event because it's not as easy as just going to the game...it takes preparation when you are disabled), a garage security guard approached my car (the same one that instructed me to pull into the lot in the first place). I opened my door to make sure that everything was okay and to ensure that I was, in fact, in the correct location. I pointed again to my handicap placard and stated that I would be using a wheelchair for game. He looked down at my knee brace (it extends from my thigh down to my ankle so it is hard to miss) and said, "you have a bad leg, okay, you are in the right spot."

He then explained that his manager had told him to make sure that people actually needed handicap parking before allowing them to park there because recently people were utilizing handicap parking but were able to walk.

So, that's not how this works...

First, since when do parking garage attendants get to decide when an individual can utilize their handicap placard? If someone has a handicap placard, they can use it whenever they feel it is necessary. Sometimes that means only on "bad" days, and other times people use them everyday. That is for the individual to decide, but they should not need to justify using one to anyone.

Also, able-bodied individuals do not get to decide who is and isn't disabled just by looking at them. Many disabilities/illnesses are completely invisible; meaning a person may look perfectly healthy, but internally are anything but.

I have more than 8 chronic illnesses. Some of them are visible and some are not. With EDS, for example, I wear braces to stabilize my joints everyday, and I often use a wheelchair or crutches for mobility. Most of the time my braces are visible (though I do conceal them when possible) and, of course, my wheelchair/crutches are as well. However, if you took those mobility devices and braces away, EDS is actually largely invisible. That doesn't make it any less disabling. I still dislocate my joints constantly, live with significant pain, and struggle with countless other symptoms.

I also have a form of Dysautonomia (autonomic dysfunction) called Postural Orthostatic Tachycardia Syndrome (POTS). [If you want to learn more about POTS click here] As a result, my body does not tolerate being upright for very long. Simply standing up is like running a marathon for my body. When I stand up, my heart rate increases rapidly (tachycardia), and I often feel lightheaded, dizzy, and nauseous. This can lead to fainting if I am unable to adjust in time. But if I don't faint...POTS is invisible. It is also just as disabling as my joint dislocations and chronic pain, and incredibly exhausting when your body is constantly trying to stabilize your blood pressure and heart rate.

There is no specific way a disabled person should "look." We come in all shapes and sizes, with visible and invisible illnesses. Also, just because someone CAN walk does not mean that they are not disabled in other ways (ie. autonomic dysfunction/POTS).

Disabled

Still disabled...

I know it can be difficult, but please....don't be so quick to make assumptions or judgments about a person. Just because they look healthy, does not mean that they are. And they do not need to justify it to you...

An Unforgettable Experience

2019-03-09T23:30:00.004-05:00

I’m not even sure where to begin...

Anyone that knows me knows that the Boston Bruins are my #1 passion. They are my biggest distraction from pain and quite literally keep me going. There is hardly a day that goes by that I am not covered head to toe in black and gold, and I would not have it any other way.

It has somehow worked out that every December since 2010 I have had surgery at Boston Children's Hospital, and every year I have missed the Bruins visit by 2-3 days. However, this past December, while inpatient following surgery, I had the pleasure of meeting 4 players (Brad Marchand, Zdeno Chara, David Krejci, and Steven Kampfer). It was such an incredible experience to be able to meet them, and certainly put a smile on my face for the rest of the week while I recovered. We talked about the game the night before against Arizona, and when Chara asked me what I thought I told them I wasn't pleased with the team turnovers, despite the 4-3 win. On the way out, Marchand said: “I’ll think of you yelling at me on TV every time I turn the puck over.”

For Christmas, Shane bought me tickets to the Bruins/Hurricanes on March 5th. As a joke, I sent a tweet to Brad Marchand asking him to limit the turnovers while I’m at the game with a picture from the hospital visit. I didn’t expect him to see my tweet nor did I expect a response. I also didn't think he would remember me. But he did respond and offered me passes to say hi after the game.

The tweet that started it all....

As if that weren't generous enough, he also invited me to the games on Thursday and Saturday as well (I went 3/3). He provided each of us with a signed Winter Classic jersey and took us on a tour of the locker room. He hooked me up with Winter Classic gear and stopped every single Bruins player that walked by so that he could introduce them to us. He even made sure that I got a picture with Patrice Bergeron (and then chirped me for not being able to find words...I got better as the week went on though). His wife Katrina was incredible as well. She even visited us during intermission of Thursday’s game against the Panthers and it has been such a privilege getting to know her during this experience.

I really do not have words to describe the level of appreciation I have for Brad and Katrina. They went above and beyond anything I could have ever imagined. To be able to share this experience with my brother, cousin, and best friend was also special. I only wish that my dad could have been there with me as well. It’s been a rough year, but this was without question the best experience of my life. It was truly unforgettable.

To Brad and Katrina,

From the bottom of my heart, thank you for everything. I will cherish this week for the rest of my life. You are both amazing.

Keep it rolling! Go Bruins!

With the greatest appreciation and gratitude,

Jill

Bruins/Hurricanes with my brother and cousin

Brad Marchand #63

Patrice Bergeron #37

Marchand, Bergeron, Chara...what a crew!

Shane and I with Marchy!

Marchand #63 and McAvoy #73

He's the best!

TUUUUUUKKKK!

Bruins/Panthers with my brother and Steve

Pastrnak, Kampfer (I met him in the hospital), DeBrusk

37 + 63 = 100!

Shawn Thornton

Group shot!

Bruins/Senators with my brother and best friend!

[For those interested, I wrote an article in 2014 about what hockey means to me from a health perspective- Hockey is the Best Medicine].

Eric Russo from the Boston Bruins wrote this article about the experience: Marchand Makes A Dream Come True

---------------------------------------------------------------------------------------------------------------------------
Background:

I was born in Connecticut, though my dad was born in Massachusetts; therefore, you could say that I was born into a Boston sports family. My dad taught me all about the Bruins. Not only did he teach me the game and share with me his favorite Bruins memories dating back to the days of Bobby Orr, but because of the Bruins I shared an incredible bond with my dad. Our collective passion for Boston sports, and specifically the Bruins, is something I will cherish for the rest of my life. I lost my dad to cancer in 2016. Now when I watch the Bruins, I know he is with me, just in a different way. I still feel a connection to my dad when I watch every game.

Hip Instability and Plan

2019-03-08T20:54:00.001-05:00

I have been in Boston this week visiting my specialists at Children's. There was a lot of anticipation leading up to these appointments as I was expecting bad news regarding my right hip.

Background: I was diagnosed with bilateral hip dysplasia in 2009. I underwent periacetabular osteotomies (PAOs) to provide more anterior coverage to the femoral head. This involved breaking my pelvis in 3 places and repositioning it to provide deeper sockets. In theory, this should have provided greater stability and prevented recurrent labral tears....but it did not.

In 2017, after years of hip surgeries to repair the labrum and address impingement, my right hip subluxated (partially dislocated). Later that year I underwent open reconstructive surgery to stabilize my hip with an allograft tendon (a procedure that had been performed successfully 3 times prior at Children's). They also learned during the procedure that my rectus femoris (largest quad muscle) was detached (likely from the subluxation) which explained why my quad didn't function the year leading up to surgery. Unfortunately, only 2 months into recovery, I managed to tear part of the graft. When I was finally able to start weight bearing again (it took 6+ months for my quad to fire) it became blatantly obvious that my hip was still unstable. However, at that time, my left kneecap was causing major problems with lateral and medial dislocations in and out of the brace, and that became the priority for 2018.

Fast forward to 2019...now that we have addressed my left knee, this week we focused on my worsening hip instability and pain. Due to femoral anteversion, my hip sits approxiamtely 5.5 mm out of the socket in neutral. So my hip is always partially dislocated. As a result, in an attempt to provide more stability, I internally rotate my right foot (most of the time I don't realize I am doing this in the moment). I have been falling everyday, which not only leads to more damage to my hip, but my right shoulder dislocates daily as well (I wake up with my shoulder dislocated every morning too). I am starting to lose function in my hand as it is often numb due to the nerves being aggravated. I having been dealing with constant hip pain way above my baseline, and I am not sleeping due to the pain with very few pain control/management options available. My pain is significant, my quality of life and function are declining, and I am struggling to progress with left knee rehab because I am largely stuck in my wheelchair due to my hip. In order to regain quad function I need to be able to weight bear more consistently, but this exacerbates my pain and often leads to falls (I have to protect my other joints as well).

Because of this, my surgeon feels our only option is to proceed with surgical intervention in the next few months. I was concerned coming into this trip that my surgeon was going to have absolutely no idea what to do as we have basically tried everything, and my hip really should not still be this unstable. However, he believes my best chance at stability is a femoral derotational osteotomy and a replacement of the allograft. It will be a long recovery and I will likely be inpatient for 7-10 days based on my history. This will be my 13th hip surgery. Obviously I am not thrilled to need another hip surgery, but given my pain and lack of function, I would rather try something than have no options at all.

The goal of the femoral osteotomy is to position the femoral head deeper into my socket by breaking the femur, rotating it, and plating it in place. This should improve stability. The allograft will act as a capsule because I no longer have one (also providing stability).

Femoral derotational osteotomy

I will update when I have a date for surgery. Until then I will continue to work on regaining strength and range of motion in my left knee.

Left Knee Surgery Update

2019-02-22T23:15:00.001-05:00

This post is long overdue...

Left Kneecap Dislocations
For over a year, my left kneecap dislocated medially (very rare) and laterally everyday. It even took up new residence on the medial aspect of my knee. Bracing and taping did not help, and my kneecap dislocated despite both. I could hardly flex (bend) beyond 20-30 degrees before it jumped out of position. It was incredibly painful.

In 2010, I previously underwent an MPFL reconstruction with a cadaver graft for lateral dislocations. That surgery seemed to work for 7+ years before the dislocations occurred again. This time, when it began dislocating medially as well, my surgeons were a bit more confused as this is really should not be possible. There are very few reported cases of true medial kneecap instability and as a result, the treatment options are even more scarce. But I am blessed with a team that researches and contacts other specialists to determine the best path moving forward.

The kneecap should be positioned in the center of the black line.

Left Knee Surgery
In December, I underwent reconstructive left knee surgery to reposition my kneecap centrally (with a TTT osteotomy), and to provide stabilizing forces to the medial and lateral aspects of my kneecap (MPFL and LPFL*). Due to EDS, cadaver grafts were used. I was inpatient for 6 days. The drain was removed the following day. Because this surgery increases the risk of compartment syndrome, a fasciotomy was performed. This involves cutting the fascia (tissue) to reduce pressure in the tissue (which can result in tissue death). Unfortunately, I still ended up with mild compartment issues and for the first 48 hours I had no feeling in my left foot (which was not expected). The fasciotomy also resulted in a large muscle hernia.

During my hospitalization, I received a visit from my beloved Bruins. Words cannot even begin to describe how excited I was and thankful for their visit. I have had surgery nearly every December for 10 years at Children's and I have missed the Boston Bruins visit by 2-3 days every year until this time.

A bright spot during a painful week in the hospital.

Post-op

Muscle hernia and hematoma

It has been a very slow recovery. I had post-op x-rays at 4 and 8 weeks. My osteotomy site has healed well and I am allowed to fully weight bear through the left knee as long as my brace is locked in extension. I still have no active quad function, though this really is not unusual for me. My quad tends to shut down for an extended amount of time postoperatively. I will need to remain in the post-op knee brace until I have sufficient quad strength, otherwise my leg will give out on me and I will fall on my face (which I do anyway because of my right hip). It has also been a struggle to get my knee to bend; however, we continue to work on this in physical therapy.

Making recovery even more challenging is the fact that my right hip is severely unstable and that has led to quite a few falls. The falls have resulted in more damage to my right shoulder as it dislocates nearly every time.

CRPS Relapse
Leading up to the surgery I was dealing with a significant CRPS flare in my left foot. This was triggered by an avulsion fracture after I rolled my ankle for the millionth time. I was unable to tolerate a sock on my left foot, so went without a left shoe for awhile. It was decided just prior to being put under anesthesia for my knee surgery, that we would try ketamine as part of my anesthesia cocktail. Ketamine has been used for CRPS successfully in studies and I am thrilled that it worked for me as well! I have not had CRPS pain since December.

My foot would swell and turn red daily.

Some days my foot would turn purple.

I will post more about my right hip instability in another post. For now, I will continue to work in physical therapy on regaining strength and flexion in my left knee.

*We don't have an LPFL as part of our anatomy...

Best and Worst Doctor Experiences

2019-01-30T19:30:00.000-05:00

As anyone with chronic illness will tell you....not all doctors are created equal. Some are excellent, others are good, and of course there are a few that are just terrible. People also have mixed views on doctors as we are all looking for something different.

I have had both good and bad experiences with doctors. In fact, I have had more positive experiences than negative and I realize that is not usually the case. This is partly due to the fact that I have had the same team of specialists for years (they are a really good fit for me so there really is no reason to change) and I only add new ones at the suggestion of my PCP (who helps me find the right one when needed).

Because I have had a lot of really good experiences with doctors it is hard to pick just one experience...

The BEST experience, or at least the one that comes to mind first (as it was relatively recent), was with two of my surgeons in Boston. I was scheduled to see both surgeons at different times on the same day. Both have worked together on my case for almost 10 years. This day they came in together and combined two appointments into one (unplanned). This was the first time in years we were able to sit down together to discuss everything as a group (they usually are not in the same place at the same time).

Despite it being a frustrating day with a lot of tears, as we were trying to work through multiple problems (all compounding the situation), it was still a positive experience. They were so patient, so caring, and reminded me that they would not give up and that we would figure it out together. One of them even called my physical therapist and had a 20 minute conversation with him to make sure we all understood the situation and had a plan moving forward. It meant a lot that they took time out of their busy schedule to communicate with other members of my team. After all, that’s what makes this a “team.” They are truly special. I am lucky to have them.

The WORST experience - I am very fortunate that I have not had many bad experiences with doctors. Typically the challenge is they just don’t know what to do with me given the complexity of my health. The worst experience was probably with a general surgeon that I was referred to for a consultation regarding having a port placed for IV fluids. She was unfamiliar with any of my conditions, I waited 2 hours in a room to be seen (she decided to see patients out of order and saw me last) and told me a port was a “stupid” idea. Instead of listening to why we felt this was the best option (my PCP and cardiologist agreed this was the best path moving forward, they are just not capable of placing a port themselves), she tried to convince me that it would be a huge mistake. When I presented literature supporting IV fluids for low blood volume and POTS she changed the subject. She told me to come back in two weeks and she would do research and talk to my cardiologist. Meanwhile she did zero research, never talked to my cardiologist, and wasted my time again. I was discouraged by the appointment but eventually I found the right physicians to help me and everyone on my team agrees that the port was one of the best (and most important) decisions we made.

I am thankful that my positive experiences far outweigh the bad. That is not typically the case for people with chronic illness and I have my team to thank for that.

All About Feeding Tubes

2018-09-20T15:48:00.001-04:00

We have finally reached the point where I need a feeding tube. I have been putting this day off for a very long time (nearly 2 years) despite several GI doctors telling me I would need a feeding tube if I could not manage at least 1700 calories everyday on my own. On a good day I consume about 700 calories and I don't have many good days (and I am still far from my goal intake).

Accepting that I need a feeding tube has been a process. It's a big deal. It's a huge change for me and I wanted to reach a place where I felt at peace with the decision. So I stalled and stalled and kept pretending like I would be able to find ways to consume enough calories to nourish my body. But....it wasn't happening. And I knew that. I am underweight. I have little to no energy. My body has been without steady nutrition for far too long. We have tried everything there is to try. It's time.

I wanted to write a post to explain feeding tubes in more detail and discuss some misconceptions with feeding tubes that exist so as to hopefully help people better understand.

There are different types of feeding tubes...
Types of Feeding Tubes

Nasal Feeding Tubes - NG, NJ, ND - tube enters the nose (N) and travels down to the stomach (G), jejunum (J) or duodenum (D). Feeds can be given through these tubes and they can also be used to decompress the stomach (NG).

Jejunum = small intestine

Abdominal tubes

G-Tube - most common type of feeding tube. They are placed directly through the skin and into the stomach. Those who require tube feeding for more than 3 months are likely to receive a G-tube. A G-tube may consist of a long tube, sometimes called a PEG tube, or a skin-level button.

Mic-Key button

GJ-Tube - very similar to G-tubes, they enter the stomach directly through the skin using the same site or stoma as a G-tube. There are two ports, one into the stomach, and one port with an internal tube that extends into the small intestine. The G port allows for venting to release pressure from the stomach. The longer internal tube allows feeding directly into the small intestine (jejunum- "J") for those who cannot tolerate gastric (stomach) feeds (as is usually the case with gastroparesis)--feeds are given more slowly through the small intestine. This is the type of tube I have!

GJ Mic-Key button

J-Tube - tubes that are directly placed into the small intestine. These are seen in patients with delayed stomach emptying as delivering feeds into the jejunum (small intestine) bypasses the stomach completely. They require surgical placement. Feedings must be given slowly.

There are SO many reasons why people need feeding tubes and I couldn't possibly cover them all.

Reasons for Feeding Tubes
Here are some reasons:

Upper airway disease or obstruction
Gastroparesis and delayed intestinal motility
Cystic fibrosis and other lung diseases
Prematurity
Failure to thrive
Congenital heart defects and other heart conditions
Metabolic disorders
Endocrine disorders
Malformations/diseases of the gastrointestinal tract
Intestinal obstruction
Neurological/neuromuscular impairment
Cancer and chemo
Renal problems
TBI (traumatic brain injuries)
Dysphagia (difficulty swallowing)
Coma patients
Special needs patients
Various autoimmune conditions
Post-op patients
Cleft palate
Severe reflux with aspiration
Genetic conditions
Dysautonomia/autonomic dysfunction
Connective tissue disorders (EDS)
Craniofacial conditions
Nerve damage

As you can see...feeding tubes are used for far more than just gastrointestinal/stomach problems.

Many people have no experience with feeding tubes and there are many misconceptions about them...

Common Myths/Misconceptions about Feeding Tubes
#1 When you have a feeding tube you can't eat
While it is possible that individuals with feeding tubes cannot consume anything by mouth, many people with feeding tubes are encouraged to eat as much by mouth (orally) as possible. So yes, even though I need a feeding tube to ensure that I am receiving adequate nutrition (as I am not able to consume enough on my own), I am allowed to/encouraged to eat food.

A feeding tube takes the pressure away from force feeding myself and dealing with the consequences (vomiting, abdominal pain, bloating, etc.). And yes, I do miss food and I do want to eat food even though I no longer feel hungry and struggle to tolerate it.

#2 Everyone with a nasal feeding tube has an eating disorder
This is simply not true. Nasal feeding tubes are typically used for patients who require a feeding tube for less than 3 months (they can be used longer but the discussion of placing a surgical/abdominal tube then comes into play). They are also used as a trial step to ensure that the patient tolerates feeds.

#3 Feeding tubes are permanent
While it is possible that someone may need a feeding tube permanently, some patients improve enough to where they no longer need the tube. Everyone is different. Their reasons for needing a tube are different and therefore, it's a decision made between the patient and healthcare team if/when the patient is ready to stop tube feeding.

#4 Feeding tubes are only given to people who are underweight
There are so many reasons why people need feeding tubes and not all of them have to do with weight.

#5 Feeding tubes are an easy fix
Feeding tubes are not a simple or easy fix for most people. They are a lot of work. It is not as simple as placing a tube and giving yourself nutrition. Some people do not tolerate formula/feeds well, they can can cause pain, infection, skin breakdown, and a host of other issues. The body doesn’t always like having a foreign object lodged into it. It can be a lifesaving measure, but it is not easy or simple.

#6 Feeding tubes are a cure (all of your symptoms go away)
Feeding tubes are not a cure. They do not magically make all of your symptoms go away. Similar to the answer above, not everyone tolerates formula well and you can still experience nausea, vomiting, pain, bloating, etc.

#7 You don't feel hungry when you have a feeding tube
Some people may not feel hungry due to the nature of their medical condition, while others still do feel hungry. Again, everyone is different. This is not a one-size-fits-all tool.

#8 Feeding tubes restrict what you can do
No, not at all. Kids and adults can lead relatively normal and productive lives with feeding tubes. You can remain active in sports, you can swim, you can work, you can exercise, and travel with a tube! Note: this is in regards to a feeding tube not another medical condition that may prevent any of those activities.

#9 Healthcare providers know everything about feeding tubes
Unfortunately, not all healthcare providers have experience with feeding tubes and some are downright clueless. Parents and patients often know their tube best and figure out the tips and tricks to making life with a feeding tube easier.

#10 Feeding tubes are only used if you're dying or near the end of life
Please refer to the list above about reasons why someone may need a feeding tube. Feeding tubes are tools that help people consume adequate nutrition when they are not able to themselves (for whatever reason). They can be temporary or more long-term but they are not only used for people who are dying or near the end of life.

If you have questions about feeding tubes I encourage you to ask me!

Custom Wheelchair

2018-09-06T23:49:00.003-04:00

Several months ago we decided that it was time to get evaluated for a custom wheelchair. I was recovering from reconstructive hip surgery and was struggling to regain quad function. I had a standard manual wheelchair but was unable to lift it on my own due to my shoulder instability and as such I was becoming more and more frustrated with my lack of independence. My primary care physician wrote a prescription for a custom wheelchair evaluation with an OT/PT and ATP. I have been working with a company called NuMotion throughout the process.

The evaluation took place in the Spring and involved discussing my medical history and specific needs, as well as taking measurements to see what the best size wheelchair would be for me. We talked through all of the options and ultimately all agreed on a TiLite Aero Z wheelchair.

Custom ultralight wheelchairs are built specifically for the user which makes them much more manageable.

I wanted yellow. But they didn't have yellow. So I got the next best color, BLACK. And I added my own touches of yellow!

I am hoping this wheelchair gives me more freedom and allows me to do more with friends and family.

Wheelchair bag

My Journey with Chiari Malformation

2018-09-04T18:11:00.002-04:00

I created this video to document my journey with Chiari Malformation.

Organization: Medical Supplies and Portfolio

2018-08-28T19:51:00.000-04:00

Let's talk about organization. Organization is my thing. Not only am I prepared for nearly every situation but I am ridiculously organized (almost to a fault). I suppose it comes with being a perfectionist. Perhaps its my way of trying to regain some control in my life. Regardless, for me, my life runs more smoothly when everything is in order. In fact, a lot of people have told me they wish I could organize their life.

My closet it color coded, my medical supplies are organized by categories, my medical folder (accordion) is organized by specialty, and I keep all of the most important documents (copies at least) in a portfolio that I always have with me. Even my backpack is organized (see post on What's in my "Purse": Chronic Illness Edition). Basically, everything has a place. I may be the only one who understands my system, but seeing as I am responsible for my own health and treatments, that's okay!

I will break this post down into different sections and explain how I organize each area. Keep in mind that it is not possible to break down every little detail but I will do my best to show you my system. Maybe it will help some people understand how my brain works (because this is how I organize my life...not just medical stuff).

Medical Supplies
I have A LOT of medical supplies. This is largely due to the fact that I have a central line and there are a lot of supplies that are needed to not only care for a port-a-cath but for running infusions through my line. I have a system in place that accounts for daily and weekly needs, as well as a storage system for as needed and extra supplies. This also makes it really easy for me to pack for trips as everything is broken down into categories and I can go through my checklist and grab what I need from my different bins.

Extra Supplies and PRN (as needed) Supplies
My bins are organized by category. I have 3 main bins in my room.
1) Port and infusion supplies


Bin contains: extra access kit, swab caps, alcohol swab sticks and skin prep, chlorhexidine wipes, extra dressings, emesis bags, masks, sterile and non-sterile gloves, Purell, hand wipes, alcohol wipes, thermometer

*Not pictured- bin of extra infusion bags and extra boxes of flushes (saline and heparin)

2) Tubing for infusions
Tubing takes up a lot of room as I have two different types-- dial-a-flow (gravity) tubing and pump tubing. I also change my tubing with each infusion so I go through at least 3 per week.

Dial-a-flow tubing and pump tubing with batteries (for the pump)

3) Allergy and asthma supplies

I have allergic asthma and do daily nebulizer treatments in the Spring and the Fall so I go through this bin quite often. This is also where I store any backup rescue medications (epi-pens, inhalers, nebulizer solutions, benadryl, etc.). If it has to do with breathing or allergies, it goes in this bin!

Bin contains: rescue medications (for nebulizer), tubing and masks (for nebulizer), extra spacers, extra rescue inhalers, extra nasal sprays and other allergy medications and creams, extra epi-pens

How I store my masks (there is another door as well not pictured)

Weekly Supplies
I do weekly infusions (3x/week) and I tend to run my fluids at night while I am in bed. As a result, I have a side table with drawers that has everything I would need to run a bag of fluids. I simply restock these drawers from my extra supplies when I am running low. The drawers typically hold at least 2 weeks worth of infusion supplies.

Tubing, alcohol wipes, flushes (heparin and saline), batteries, tubing caps

Bags of fluid (Lactated Ringer's and Normal Saline)

Daily Supplies
I have various treatments everyday. While I may not be infusing fluids all the time, as long as my port is accessed I have to flush the line daily. I also have daily medications, breathing treatments, and various PRN (as needed) medications that I may take. This is all organized on my bathroom counter.

Daily flushes (saline and heparin), alcohol wipes, swab caps, extra biopatches and injection caps, PRN (as needed) medications, bandaids, Purell, syringes

*On the main floor of our house I have pre-made access kits. I recycle the zip-lock bags my infusion company sends me with monthly deliveries to make these ready-made kits. Because we access my port every Monday (downstairs), it is easier to have them on the same level.

Access bags, extra supplies for the kits

Medical Folder/Accordion
I have accumulated a lot of files over the years. I have visit summaries from appointments, insurance documents (bills, claims, etc.), discharge summaries from hospitalizations, operative reports, physical therapy summaries, and more imaging discs than I can count.

I have organized the accordion by medical specialty and topic. I have a separate binder of all of my x-rays, MRIs, and CT scans. I ALWAYS ask for copies when I have imaging done so that I don't have to track them down later if another specialist requests to see them.

Imaging binder

I also request operative notes/reports at my first post-op visit so that I always have them on hand as well. In the past, I have had issues with some hospitals and getting records when requested. I have found it less stressful to ask for the records during appointments as they can usually print them right then and there. It saves a lot of time spent on the phone trying to figure out why a specialist never received your records.

Two accordion folders: (left) insurance files; (right) specialists and other medical

Inside look

Medical Portfolio
My medical portfolio is a collection of documents I have found important to have with me for new patient appointments with specialists.

What I include:

Complete medical history (combination of everything else on this list)
Diagnoses
Surgeries
Current medications and allergies
Anaphylaxis protocol
Specialist contact information
Port-a-cath placement radiology report and x-ray disc (many facilities have their own policy on using central lines and almost everyone requires proof that the line is in the correct place)- I update this once a year
Neurosurgery operative report
Genetics letter confirming diagnosis of EDS
Current diagnostic criteria for EDS
Information sheets on Dysautonomia, POTS, EDS, and Chiari Malformation
Recent labs
Pad of paper for notes

Information on POTS, Chiari Malformation

EDS diagnostic criteria, information on Dysautonomia

I also have business cards for my specialists so I can give it to other doctors who may want to contact someone else on my health team. I make sure to carry a pen with me as well (both in the binder and in my backpack).

I am constantly updating my portfolio. If I come across something that would be important to have with me, I make copies and add them as necessary.

I am in the process of coming up with a "Treatment Protocol" that my PCP and cardiologist will sign to make it easier for me to seek help in an acute situation. With my complicated history, many facilities simply do not know what to do with me. Having a treatment protocol signed by two members of my healthcare team should help provide some guidance to new providers should I need to seek emergency treatment or care.

When you are complicated, it pays to be organized!

What has EDS taken from me?

2018-08-23T11:27:00.001-04:00

Life with chronic illness can be cruel. In the blink of an eye your life changes. You are forced to reevaluate your life, your goals, your dreams. It takes away all control and predictability. You question why this is happening but ultimately have to accept that life is different than you had envisioned and you have to find a way to adapt to the changes and find a way to move forward and live despite your illness(es).

Here are the Top 3 things EDS and chronic illness have taken from me:

#1 Nursing- The biggest thing EDS has taken from me is the ability to work as a nurse. Due to the unpredictability and unstable nature of my health (at this point), I am unable to work in a clinical capacity as a nurse. Unfortunately, my health declined before I was able to gain experience, which would have opened more doors for me in nursing that may have been more feasible with my health. I hope to one day find a role in nursing as I feel my personal experiences have provided me with a perspective most do not have, but the loss of my dream (in the way in which I had envisioned) is difficult. I continue to keep my license active, my continuing education courses up to date, in the hopes that my health improves to the point where I can find a role in nursing.

#2 Financial Stability- I am 28 years old and I am financially dependent on my mom. While it never escapes me how lucky I am to have a supportive family, I want to be able to support myself and live on my own. That is not my reality, however. It is very difficult to look towards my future without any idea of when or if I will ever be able to support myself.

#3 Ability to Plan- I have said this many times before but chronic illness is unpredictable. Because of this unpredictability, it is very difficult to plan for things too far in advance. For someone who likes to plan (me) and have control, this is frustrating. I don't know how I will feel 5 minutes from now, so it is impossible to know where my health will be in 1 week, 1 month, or 1 year from now. I also feel guilty when I do plan something with friends or family and then I have to cancel or change plans because my health is not stable enough or I am in the middle of a flare. I want to be the type of person that can just get up and go...but that is so far from my reality.

Years ago when I created this blog, my motto became, "you can't always decide what happens to you, but you can decide how you react." This quote still holds true today. It's not perfect. It's not always that easy. But I do my best to remember this.

Discharge Day!

2018-08-22T18:05:00.001-04:00

Surgery to remove my (R) tibia hardware was yesterday. I totally underestimated the pain I would feel as a result of this procedure (thank you Kayla for telling me not to do this at the same time as my left knee surgery in December). I woke up in the PACU (recovery room) with 10/10 pain despite having received IV Tylenol, Dilaudid and Toradol in the OR while still under anesthesia. That meant that I was not due for more medication for almost 4 hours. Unfortunately, even when I was able to take more Tylenol and Toradol, it did absolutely nothing. So I have been in A LOT of pain since waking up from surgery. It is hard to distinguish what is post-op pain and what is CRPS pain but it is likely a combination of both. We are hoping that CRPS will settle down and return to a state of remission soon, but surgery can exacerbate the situation (which we knew going in but because the hardware reaction was the cause of the CRPS we did not really have a choice...it had to come out).

Plate (minus the 7 screws)

The site is covered with a dressing but is very swollen. I have a walking boot (as discussed in my previous post) because I have 7 holes in my bone. These holes will fill in over the next few weeks. I see my PCP in 2 weeks to check the incision. I will get a set of x-rays at 3 weeks post-op to make sure they are filling in. I will send the x-rays to Boston for review. I am allowed to weight bear as tolerated, which is important because my left kneecap is not able to support all of my weight at this point anyway (hence why I will be having another surgery in December to stabilize it).

Dressing and swelling

I did, however, have the BEST room in the hospital with amazing views of the city. It was HUGE. I also kept the hardware as I do with all of my surgeries.

Does not get better than this view

I had my favorite PT this morning to make sure I was safe while crutching. He was surprised to see my name on the list and also to see how much better I was at crutching with this admission vs. hip surgery December 2017 (where I could not advance my leg on my own).

Hopefully CRPS calms down soon, the post-op pain doesn't last too much longer and I can get back to baseline with this leg in the next few weeks.

A HUGE thank you to my uncle for taking me to surgery, waiting around all day and for several hours in the recovery room, and for picking me up today after being discharged. Words are simply not enough.

I return to Boston in mid-October for a post-op and follow-up on my left knee to finalize the surgery plan for December.

Tibia Hardware Removal

2018-08-20T22:55:00.000-04:00

I am back in Boston. I arrived on Sunday. I have surgery tomorrow to remove a plate and screws from my right tibial de-rotational osteotomy (2015). I had hoped to wait until December, when I will be having left knee surgery, but seemingly overnight the hardware has become ridiculously painful, swollen, red and bruised. It also seems to be triggering a relapse of CRPS. So it needs to come out...now!

I did not bump into anything. There was NO injury whatsoever.

Redness and swelling (CRPS)

I had my pre-op today, which involved the usual: vitals, visit with a Nurse Practitioner, anesthesia team, admitting liaison, and an appointment with my surgeon's PA. The surgeon that will be removing the hardware tomorrow was not at the Boston location today, but my other surgeon stopped in to say "Hi" and looked at my hardware site (he is actually the surgeon involved in the original surgery but his schedule was pretty tight so everyone agreed that we would just take the earliest available date given the severity).

The surgeon I spoke to believes I am reacting to the hardware. Somehow, it does not surprise me that my body found a way to be allergic to 3 year old hardware.

There is a chance that surgery could make the current CRPS flare worse. However, we are hopeful that because we are removing the cause, it will only be temporary and will return to a state of "remission" soon.

This procedure is typically done outpatient as a day surgery. However, because of my history, I will be admitted overnight for this procedure. I will be partial weight bearing for 2-4 weeks depending on pain. We will check the bone healing progress with x-rays in 6 weeks. I will wear a walking boot for at least 2 weeks as well.

Hopefully this is just a quick detour!

What's in my "Purse": Chronic Illness Edition

2018-08-15T18:03:00.000-04:00

With chronic illness your health can change minute to minute, hour to hour and day to day. This means your needs change as well. Most people who know me would agree that I am basically prepared for the end of the world. My backpack and car are stocked with everything I could possibly need in case of an emergency, changing health situation, or an apocalypse. Some people make fun of my backpack and how much stuff I carry with me, saying that it is heavier than me. They are not far off. But here's the thing...I have used everything in it and given my health is so unpredictable, I can't justify removing any of the items I carry.

Yes, everything in the list below fits in this backpack

So here is a list of items I carry with me everyday in my "purse" (backpack):

Epi-pens (2)
Anaphylaxis protocol
Emergency medical card (lists emergency contact information, allergies, diagnoses, daily meds, blood type)
Emergency EDS information card (attached to outside of backpack)
Asthma protocol
Inhaler (clipped to outside of backpack so I don't have to panic looking for it at the bottom of my bag when I actually need it)

Spacer for inhaler

Pulse oximeter
Vick's Vapor Rub (I don't know what I would do without Vick's)
Mini clip-on fan and necklace fan (in case I need to cool down but I am using crutches)
Chapstick (I like pumpkin spice)
Feminine products
Pens
Glucometer and blood sugar testing supplies

Sugar/candy/glucose tabs

PRN (as needed) medications

Tylenol
Benadryl (liquid, chewable, tablets)
Prednisone
Phenergan
Granisetron
Excedrin

Purell and hand wipes
Emesis bag
Noise cancelling headphones
Masks
Back-up port and infusion supplies
Wallet (of course)
Planner
(Eye) glasses
Mini bottle of my favorite lotion (either Johnson & Johnson lavender lotion or their baby lotion)
Tissues

Comfy Clothes

2018-08-10T00:36:00.002-04:00

I am a BIG fan of comfortable clothes. Everything I buy is soft and comfortable. I do not try to torture myself with clothing items that look great but don't feel good...so no heels for me (but seriously, I would break my ankle if I tried them anyway). I also don't wear jeans or pants with a bulky waist. I am in enough pain as it is...the last thing I need to do is wear clothing that contributes to my pain. However, I also know that when I like what I am wearing, my mood is better too!

I have compiled a list of clothing items (both style and brand) that I find to be comfortable and affordable, while not looking like I just rolled out of bed (not that there is anything wrong with wearing pajamas outside--I have done that too)!

STYLES

V-Neck Boyfriend Shirts and Oversized Sweaters

$20 from Target (A New Day)

$10-16 at Old Navy

This is a staple in my wardrobe as they are loose, comfortable, and relatively inexpensive. I buy most of mine at Old Navy and Target. With a good sale at Old Navy you can find similar items for $4-6.

Rompers/Jumpsuits

Retails at $60 but sold in Nike Outlets at a lower price

I LOVE rompers and jumpsuits. They are so easy and comfortable. It's an entire outfit with just one item! I have several rompers from Nike and I love them (see picture above). I also have rompers and jumpsuits from Old Navy, Gap and Amazon.com.

Gap "Strappy Jumpsuit" - $40 (I bought it for $20 on sale)

Leggings

No Boundaries Junior Sueded Jersey Leggings $5.88

If I am not wearing a romper or shorts, I am probably wearing leggings. I tend to stick to black leggings from Walmart as they are SUPER soft and comfortable but I also have some fun colored/patterned leggings from a variety of different stores (Target, Nike, Walmart, and Old Navy).

Crew sweatshirts

I love (oversized) crew sweatshirts! I wear them all the time with leggings. My favorite brands are Nike and Target.

Lightweight hoodies

$16.99 at Target

I have a bunch of hoodies from Target that are so soft and comfortable. They are loose and lightweight, which is helpful when I am bloated from gastroparesis. I wear them with leggings.

Zip-up hoodies

Typically starts at $50 but can be found for less at the Nike Outlet!

I LOVE hoodies (both pullover and zip)! Most of my zip-up hoodies are either Nike or from H&M. Zip-ups are really convenient because they are easier to put on and take off, which means I am less likely to dislocate a shoulder in the process.

Slides

$20-30

I do not know what I would do without my slides! I wear them all summer long. I wear Nike slides and I have them in several colors. Of course, I wear them with socks.

Comfortable sneakers

Nike Prestos!

I am not a HUGE sneaker fan but I have 2 pairs of Nike Prestos. The best part about my prestos is that my ankle brace fits inside them and they match my hockey jerseys!

My TOP Brands

Nike

I am a bit of a Nike brat as my dad worked for Nike for 26 years...so I grew up with Nike! But everything is super comfortable and it lasts for a long time!

Target

I am not sure why Target decided to discontinue their Mossimo and Merona brands as they were really comfortable and affordable (I know a lot of people were upset with the change). I like their oversized v-neck sweaters, rompers, and lounge pants (yes, I wear them out)!

Old Navy/Gap

Old Navy has great sales and low prices! I buy the same item in different colors.

Walmart (mainly for leggings)

$5.88 soft leggings for the WIN!

Great for basics and low prices!

Hope this was helpful! This post is not sponsored, nor do I receive any money for promoting these brands! I am simply sharing my favorite go-to items!

Top is from Target (same as the one posted above...but on me!)

Lightweight hoodie from Target (circled)

Heal the World

2018-07-21T13:42:00.003-04:00

A wonderful video with a powerful message!