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website of author Paula Offutt

Why Paula Uses a Wheelchair

Good question! And it has a long answer.

Unofficially, I am a genetic mutant with no super powers.

Officially, I have Ehlers-Danlos Syndrome, Hypermobility Type.

(Here comes the “blah blah blah” part)

Ehlers-Danlos Syndrome, often referred to as “EDS” is a collection of heritable connective tissue disorders. Either directly or indirectly, Ehlers-Danlos Syndrome is known or thought to alter the biology of collagen in the body (the most abundant protein), which can lead to multi-systemic symptoms. Each type has certain physical traits and with notable exception to the most common form, the hypermobile type of Ehlers-Danlos Syndrome, most types have a known disease-causing gene.

There are physical characteristics that are common to all types of Ehlers-Danlos Syndrome, including hypermobile joints (joints that move in greater amounts than expected) and skin involvement, such as any of the following: soft, stretchy, saggy, too thin, easy bruising, easy wounding, poor wound healing and/or atrophic scaring.

Each type is a distinct entity and may have very specific and unique features. It is highly improbable to have more than one type of Ehlers-Danlos syndrome, but as they have features and ‘biology’ in common, each type may appear to have variable features of other types.

(source)

Hypermobility (Formerly EDS Type III)

Joint hypermobility is the dominant clinical manifestation. Generalized joint hypermobility that affects large (elbows, knees) and small (fingers, toes) joints is evident in the Hypermobility Type. Recurring joint subluxations and dislocations are common occurrences. Certain joints, such as the shoulder, patella and temporomandibular joint dislocate frequently. The skin involvement (smooth velvety skin with or without hyperextensibility) as well as bruising tendencies in the Hypermobility Type are present but quite variable in severity.

Chronic pain is a well-established and cardinal manifestation of Hypermobility EDS and it is common for pain to be out of proportion to physical and radiological findings. The origin of the pain is not clearly understood, but some of the likely causes include muscle spasm (tender points are sometimes present) and degenerative arthritis; neuropathic pain is also common.

To date, there is no distinctive biochemical collagen finding identified for the majority of Hypermobility cases. The Hypermobility Type of EDS is inherited in an autosomal dominant manner.

(source)

[Hypermobility Type] Affects 1 in 10,000 to 15,000 and is caused by an autosomal dominant mechanism. Mutations in either of two separate genes (which are also involved in Vascular EDS and Tenascin-X deficiency EDS, respectively) may lead to this variant; it is the only type of EDS that cannot be diagnosed through skin / tissue samples but is rather diagnosed through use of clinical observations. Symptoms can include easy bruising, velvety-smooth skin, mildly hyperextensible skin, and loose, unstable joints. Joint dislocations and subluxations are common. Degenerative joint disease can occur; the pain associated with this condition is a serious complication. Some individuals have mitral valve prolapse, which creates an increased risk for infective endocarditis during surgery, particularly dental surgery, as well as possibly progressing to a life-threatening degree of severity of the prognosis of mitral valve prolapse.

(source)

Basically, collagen is in every cell. It makes a cell hard or stiff (such as the cartilage in your ear and nose) or soft (such as skin). It also makes things elastic (such as ligaments and tendons). In people with Ehlers-Danlos Syndrome (EDS), and particularly with people with Hypermobility Type (HEDS), there is too much elasticity. Ligaments and tendons stretch too far and compromise joints. Larger joints, such as shoulders and hips, are frequently subluxed (partial dislocation) or dislocated. Smaller joints such as fingers, bones in the hand and feet, etc, move too much and begin to erode. This all leads to chronic dislocations/subluxations, osteoarthritis, muscle spasms, etc.

Some people with HEDS experience very little pain. My grandfather was a tallish, skinny man who could put his feet behind his head. He had some body pain but not very much. More as he got old, of course. My mother was never physically active and never developed muscle tone needed to hold her together. She dislocates joints easily and has chronic pain because of it. I grew up on a farm and was in decent shape. I was relatively “normal” until I was 15. I had a surgery that caused a limp which caused my body’s balance to get messed up.

There is a theory that the chronic pain associated with HEDS is somehow related to the fascia in what is called “myofascial pain syndrome” but is often confused with fibromyalgia. Fascia is the stuff that covers everything in your body. If everything but that were removed, you’d have a perfect 3D image. They don’t know why the fascia of HEDS patients hurts other than it is made of collagen (and other stuff like proteins and fats).

I have chronic pain. It never ever goes away. Never. Some days are better than others and I can walk to the mail box and back without getting tired or create pain. Other days, I can’t get out of bed. I use a wheelchair because of the damage to my hip sockets, knees, and ankles. I use a power chair because my shoulders can no longer push me without subluxating. I typically only use one forearm crutch because my right hand and shoulder have been damaged. Too many times the shoulder has “popped” and I’ve fallen. The chair prevents falls and eliminates a lot of pain. My current chair has “tilt” which allows me to take the pressure off my hips without having to get out of the chair and go to bed. I have a headrest that allows me to rest my neck. My migraines are caused by me turning my head too many times or bending it too long. I have to be careful when I read a book to ensure I have the book high enough that my head is not bent. I have to sit directly facing the television to make sure my head is not turned at all. My laptop is on a tilted table to raise the screen high enough that my neck isn’t bent. My desktop computer sits on a desk I made to fit me, my chair, and my needs.

Some people, like my mom, are “floppy”. They can bend their bodies in unbelievable positions. Then there are people like me, whose muscles are constantly tensed in an effort to hold everything together. This means that falls, stumbles, etc. result in some muscle damage because they are already tense with very little room for error. My physical therapist and I laugh a lot at our efforts to make my body relax more which seems counter intuitive for someone with my disease. When my muscles spasm, they tend to really mess me up. It is like a wave goes through my body. I wrench my shoulder so my shoulder muscles tense even more. Then my neck and upper back muscles react. Then my lower back and chest muscles. And so on.

So, there you have it. The basics anyway. I don’t mind questions or comments. EDS has made me who I am. I have accepted it (for the most part) and have certainly accepted my chair. Some people see life in a chair as a horrid thing. They would rather die. Not me. Using a chair gave me my life back. The level of pain I am in now is significantly less that what I was in before I started using one. And I cannot imagine the level of pain I’d be in if I didn’t have one now. I do, and can, walk. I use a single forearm crutch (sometimes two) and it takes me a while to get to where I am going. I don’t walk often; more in the summer, less in the winter. Walking comes at a price, though. The pain can get intense fast.

Some day, they’ll figure out which gene is responsible (HEDS is the only type they’ve not figured out yet) which will lead to a cure. Some day.

Linkages:
Ehlers-Danlos National Foundation (EDNF)
Ehlers-Danlos Syndrome (Wikipedia article)
MedlinePlus article
National Organization for Rare Diseases (NORD) – Ehlers-Danlos entry
Fibromyalgia (Wikipedia article)
Myofascial Pain Syndrome (Cleveland Clinic article)

2 Comments

  • On October 15, 2008 at 1:22 am Thyre said

    Saw your entry from a EDS feed, and just got the urge to comment on what you wrote. I have EDS type III, and am considering a wheel chair. I am exactly how you described, not particularly bendy but in chronic pain. I’m only 20 and got my diagnose 4 years ago, stress is what made my condition get much worse. I’m just happy to see someone who is happy about their wheelchair, it makes it less scary to get one.

    Gentle hugs from Norway

  • On May 1, 2017 at 6:51 pm Jackie McBride (AbleTec) said

    Hi, Paula. I’m the WordPress support forum volunteer working w/you regarding your blog site. I also do creative writing, & I also have a disability–I happen to be blind. If you haven’t considered biofeedback/hypnosis for the chronic pain, it might be worth doing. During my career as a physician, I found it helped a lot of my patients.

    Anyway, congrats on your books & best wishes for continued success. & thanks for the accessible captcha, btw.

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