Facing My Fear of Using a Mobility Aid for Ehlers-Danlos Syndrome

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Imagine having a condition where you slowly lose the ability to do things that you have always been able to do with ease. While losing all of this function, the amount of pain you experience on a day-to-day basis increases to the point it feels you no longer have a tolerable quality of life. Then you realize there is something that can help increase your quality of life a little bit, and it can help make things easier, but then everyone calls you a “faker,” and say you aren’t sick enough to use the aid. You are told that you were walking yesterday, so why do you need it today?

• What is Ehlers-Danlos Syndrome?
• What Are Common Ehlers-Danlos Syndrome Symptoms?

My Story

My name is Lj, and I have a degenerative disease called kyphoscoliosis type Ehlers-Danlos syndrome (kEDS). Ehlers-Danlos syndrome (EDS) is a systemic congenital illness that is degenerative in nature. Every person with EDS is affected differently. Some have a very mild case, while others are significantly impacted leading to severe disability. Over time, pain and symptoms tend to get worse. Each form of EDS is slightly different, but each form also has its own unique characteristics. I have kEDS. I am considered an atypical kEDS case and they believe I could have a new form of EDS that has not yet been named. All we know is that I have the PLOD1 mutation linked to kEDS in addition to other “never seen before” (or “grey area”) mutations at the time when I was tested.

kEDS is one of the rarest forms of EDS and is also considered one of the more debilitating forms in addition to being one of the very few types of EDS that are considered autosomal recessive disorders. With this type of EDS, those who are more severely affected tend to lose their ability to ambulate independently in their 20s or 30s. With all forms of EDS, there is a chance of needing an assistive device like a walker, wheelchair, brace or some other form of assistance to walk due to severe joint instability, autonomic dysfunction, severe pain, severe fatigue and to reduce the frequency and severity of falls.

I turn 30 years old in a couple of weeks, and I consider myself lucky to still move as well as I do, but recently things have really started to change. I have kEDS and spina bifida occulta. At this point in my life, I have had 56 surgeries. Four of those surgeries were on my back, seven were on my left knee, and two on my right knee in addition to over 10 other procedures that I have had on my back. Unfortunately, the last attempt that was made at detethering my spinal cord was a failed back surgery. This means I will continue to lose function of my legs slowly over time and the pain associated with it will continue to worsen. I also have severe osteoarthritis in almost every joint of my body and experience multiple joint dislocations on a daily basis. I am considered a quadriplegic by my physicians, but what most people don’t understand is that does not mean I can’t move. Autonomic dysfunction and autoimmune neuropathy also play a role in me losing my ability to get around as easily.

When is the time right for a new mobility aid?

So, how does one with a disorder like mine know when it’s time to accept a new mobility aid? How does one know when the time is right to start using a wheelchair part-time? Some doctors will make the recommendation when the time is right, and some will wait for the patient to ask. At first, mine would make the recommendation, but then he learned that I am stubborn and will not agree to try a new aid until I am mentally ready. Now, he waits for me to come to him.

In my experience, it takes awareness and realization that everything is no longer OK and that quality of life is starting to be impacted beyond what one feels is bearable (this varies from person to person). It’s time when the pros of using the device and how the device will improve one’s quality of life outweigh the pros of not using the device.

For me, it has been realizing that without the mobility aid, I have no chance of doing what I enjoy. I am so low down and fatigued that I am not able to enjoy life or function well enough to do daily things. I knew it was time to ask for a new mobility aid when I started debating not getting my second degree due to the struggle to navigate my college campus. The amount of energy and time it takes to safely get around campus would leave me way too fatigued to be able to focus in class and succeed in school.

The last thing I have to take into consideration is in what ways the device would change my life. The device would allow me to better manage my pain, reduce the number of dislocations I have on a daily basis, reduce my number of falls, increase my stamina throughout the day, allow me to be able to continue functioning throughout the day after going out, allow me to keep up with my peers/family and allow me to have enough energy to still focus in class and do other things I enjoy.

The fear of asking for a new mobility aid

Asking for help and asking for a new mobility aid is one of the scariest things I have to do. It’s hard to understand why I have so much fear when doing so. However, I have started to gain some insight into this for myself. I have a strong value of independence. Asking for a mobility aid violates this value because I feel like I am giving up some of my independence. I am a very harm-avoidant person. I fear rejection and I fear not making others happy. I don’t fear someone saying “no.” I fear what they will think of me for asking.

When it comes to my illness, I am always afraid people will think I am “faking” things or over-exaggerating them. This is common among those with EDS, since so many of us have had to battle physicians not believing us and saying it’s in our heads when they can’t figure out what’s going on with our bodies. As our condition progresses, people don’t tend to understand why we need something that wasn’t needed before. A lot of EDS patients are ambulatory wheelchair users, which a lot of people don’t understand. They use their wheelchair as needed. It’s not uncommon for people to see us as “imposters” due to their lack of understanding around ambulatory wheelchair users. I fear this because of how long I fought to finally get a diagnosis and how hard I had to fight against people saying it was “all in my head.” I naturally want to avoid anything that will result in others questioning the validity of my illness.

My fears are irrational and yet very real

I know my fears are irrational, and that it shouldn’t matter to me what others think. They are still very real for me and continue to be the biggest barrier to me getting the things that could make living with my disease easier. This is an ongoing battle I will have to face as my disease continues to get worse. I am working hard on overcoming it with the providers I trust most. Focusing on how using assistive devices can help me live my life according to my values is helping me overcome the fears. Over time I know it may get a little easier, or it may not get easier at all, but it’s what I have to do in order to live my life to the fullest.

It is time for me to tackle my fears in order to get the life I deserve!