Wednesday, May 1, 2013

May Is Ehlers-Danlos Syndrome Awareness Month

"When you hear hoof beats behind
you, don't expect to see a zebra"

Over this past year, it has become very clear that I am definitely a zebra. And being a zebra in a land filled with horses is very difficult at times.
"Zebra" is a medical slang term for a surprising diagnosis. Although rare diseases are, in general, surprising when they are encountered, other diseases can be surprising in a particular person and time, and so "zebra" is the broader concept.
Since horses are the most commonly encountered hoofed animal for most people and zebras are comparatively rarely encountered, logically one could confidently guess that the animal making the hoof beats is probably a horse.

I have Ehlers-Danlos Syndrome Classical Type which is a genetic disorder so I’ve had it since birth but it was only diagnosed 2011. May is Ehlers-Danlos Awareness Month and I want to raise awareness as much as I can so by posting this video about Ehlers-Danlos syndrome I am hoping others will understand what EDS is.

Are you or do you know someone who is double-jointed or someone who can put their feet behind their head or do weird things with their body bending it all different ways? If so you’ll want to pay attention to the video that I’m posting, the link to the national Ehlers-Danlos site, and my own story because it may help you or someone you know.

Ehlers-Danlos syndrome is a very horrible thing to have because with it you get many many other things that are wrong with you so many as a matter of fact that people don’t believe you. It’s damaging to your self-esteem, it’s depressing because you always feel like you have the flu and you hurt 24/7, and as kids, you hurt but you don’t really know any different because you have always hurt. As an adult, the pain is so much worse because you haven’t protected your body because you didn’t know this was what you have. If I could’ve known as a child I could’ve learned to pace myself and take care of myself so that I wouldn’t be in as much pain as I am now.

I think back to the time when I had had surgery on both my feet and was in a cast on both legs. I was in 7th grade and the female basketball coach yelled at me and told me she didn’t want me in her class. Of course being overweight already along with being in casts on both legs I was terribly embarrassed as the entire room of classmates laughed and laughed at me.  I have been overweight my entire life, not just a little overweight but a lot overweight and as a result my self-esteem is not so great. Since I have all this weird stuff wrong with me I was almost happy to learn that I have intracranial hydrocephalus, and have since birth, which has caused damage to my pituitary gland which is responsible for helping control weight and tons of other things in the body. I always thought that being overweight was my fault and that I was lazy and didn't care about myself because that is what everyone around me was always telling me. I remember classmates making fun of me because I was slow and couldn’t move as fast as they did. Doctors have treated me like an idiot because of my weight and I have been judged my entire life because of my weight. It is a terrible thing to deal with, not only the extra extra weight, but the judgment that comes with it from others. At least now I know that my being overweight is something that I cannot control on my own.

As an adult, being in so much pain that I can’t function not to mention some of the other things that are wrong with me due to the many debilitating things that EDS offers that keep me from functioning, it is very hard to live my life. There are days when I feel better than others and there are also days when I feel so bad that I cannot even get up to get a drink of water and because I live alone, I’m single and I have no children, and my parents live over an hour away from me and are dealing with their own health issues, I have no other choice but to lie in bed or lie on the couch and not be able to eat or drink for a day or two. Some days just making it to the bathroom is so exhausting it’s overwhelming. I think I have the best parents in the world and I know that if I lived closer to them they would take care of everything that I need.

I don’t say this to make people feel sorry for me, but I want to be open with how it affects my life and how I feel.  It is hard for me to do because to admit that my life is hard to others almost feels as if I’m being defeated and giving into EDS but I am a fighter and I will always fight to not let this disease win. I’m trying to raise awareness of how horrible this disease is and how hard life is for people that have this and how much you have to pay attention to your body so that you don’t hurt yourself. It’s hard to explain but just tying together a plastic bag or putting on a sock can cause a finger to dislocate, or getting a jug of milk out of the refrigerator or reaching in the cabinet for a glass, can cause the wrist or shoulder to dislocate, or talking or yawning can dislocate your jaw. I dislocate something an average of five times a week but thank goodness my body knows how to pop itself back into place and even though I don’t have to seek medical attention each time it leaves your body sore and aching.

This is a rare disease but I believe it’s only because so many go undiagnosed. It is called an invisible disease because the person doesn’t look like there’s anything wrong with them unless they happen to be in a cast, or be wearing a brace or maybe having to use a wheelchair, and when people speak to you, for the most part, your voice sounds fine but all the while you’re very sick. It is common that friends don’t understand what it’s like to have this disease and so some relationships will change because the needier the person with EDS becomes the more their friends back away because it’s so hard to understand what is happening to the EDS person and this is one of the reasons I want to raise awareness. If people, and even the medical community, which usually doesn’t know much about EDS, could understand what Ehlers-Danlos syndrome is and how it affects your body and how it changes your life and how it dictates your future everyone will be much better for it in the end.

This disease will never go away, as of now there’s no cure for it and really no treatment other than managing some of the pain so I’m asking for prayer for wisdom to know what to do, for God to put people in my life that can help me with my basic needs around my apartment, for researchers and doctors to find a cure, and most of all for encouragement for me as I am often very discouraged and very frustrated and very depressed. I’m also asking for prayer for guidance and a fresh vision for my future as I have, like the woman in the video says, lost my identity because everything I thought I was going to do in my future I can no longer do.

Since its Ehlers-Danlos awareness month, I'm going to try to give a little bit of information as often as I can to explain what life is like with Ehlers-Danlos. This picture of me is when I was about 5 years old. My brother and I had been playing in my grandmother's room and my brother pulled on my arm and my shoulder dislocated. I remember that it hurt so badly. We're going to go to Six Flags that day but instead, we went to the emergency room to get my shoulder put back in place. I remember they gave me some really yucky tasting medicine so I got to get a Hershey's bar. I'm laying in the chair because it's the most comfortable position for my arm and you can see my left arm laying over my body. Of course, now I know it wasn't really my brother's fault, he did not hurt me on purpose, and I also remember everybody was disappointed that we didn't get to go to Six Flags. As a five-year-old, I didn't understand anything other than it was my fault that we had to stay home instead of going to Six Flags. No one was telling me that it was my fault and that I had done anything bad, it's just indicative of how the feelings of the EDS patient are invaded by this horrible disorder.

I have dislocations sometimes daily but for sure several times throughout the week.  The most common dislocations for me are shoulders, fingers, wrist, ribs, and my jaw but I can also dislocate just about every other joint. Sometimes I can open my car door and dislocated a finger, sometimes I can yawn and dislocate my jaw or just from talking my jaw will dislocate. It is very common for me to reach for something in a cabinet like a plate or glass or reach down to get something and dislocate a shoulder. For the most part, whenever something dislocates it generally pops back into place but there are times when I'm just not sure if that was a dislocation or break in a bone. I try to only go to the emergency room to have things checked out if the pain continues intensely for more than a week. Before that, I knew that I had EDS I was asked by an ER doctor when I had hurt my foot at what point had I broken my foot. I told him that I had not broken the part of the foot that he was talking about but I had a break in another area of my foot and then he informed me that I had had two broken bones in my foot. I guess that show that I have a pretty high pain tolerance to have walked around on two broken bones and didn't see to know that the pain was worse than my normal pain.  As you'll see below in the information on treatment for EDS there's not much that can be done for the pain that comes from Ehlers-Danlos. Many people with EDS have a high pain tolerance and experience pain that would literally kill other people or at the least cause them to pass out. I do have chronic pain 24 hours a day, I've had in my entire life and it never goes away and it never will go away until a cure is found. The older I get the more I'm learning that pain takes a lot of energy.

Treatment for EDS

Treatment isn’t widely available. For most patients treatment is limited to medications that control the symptoms listed above. For example, a patient that has symptoms of arthritis or joint pain may take anti-inflammatory medications. This is still tricky, however, because many medications can trigger other side effects that an already hard to deal with life can cause to be so much more difficult to work through.

The best type of treatment is monitoring. The patient should be monitored at all times to prevent dangerous aneurysms and cardiac trouble. When a doctor is able to keep close tabs on a patient they are able to research the disease more thoroughly as well as prevent any major problems from occurring.
You keep track of all my sorrows.
You have collected all my tears in your bottle. 
You have recorded each one in your book. 
~Psalm 56:8

Some More Info about EDS.....
It's Ehlers-Danlos syndrome awareness month so here is more information about Ehlers-Danlos syndrome.

Ehlers Danlos Syndrome is a disorder that deals with the connective tissue. It is inherited and cannot be passed on any other way. Ehlers is also known as Cutis Hyperlastica. This disorder essentially causes a severe defect in the production of collagen. Collagen is responsible for providing your muscles and skin with elasticity and firmness. When collagen degenerates or stops being produced properly, the body starts to resemble a limp “sack.” The collagen deficiency can lead to muscle and joint problems, as well as other skin mutations. Ehlers Syndrome is a condition that is either “mild” or life-threatening. Currently, there isn’t a cure, and not enough funding to search for one.

Symptoms of Ehler’s Danlos Syndrome

The symptoms that one experiences with EDS are mostly due to the lack of collagen or issues with collagen production. Therefore, not everyone will experience the same symptoms. Others have worse symptoms, and some individuals are lucky enough to only have mild harmless symptoms.

For patients with EDS, their fingers and toes are likely to be extremely flexible. This isn’t necessarily a bad thing and can actually prove to be beneficial. However, this is often paired with loose joints that are prone to breaking or sprains, making it difficult for the individual to live a normal life. Most children with EDS must be extremely careful and must avoid falling or hurting themselves. This can lead to a very sheltered life. These symptoms are amongst the mildest of symptoms.

Dental crowding is a condition commonly noticed in those with EDS. Children are most likely to experience this problem because their teeth will be very crowded and crooked due to very narrow jaws and palates.

Being easily bruised is often an unfortunate symptom as well, and is caused by narrow blood vessels. Even a small poke can cause a person with EDS to form a tender bruise. Blood vessels are likely to be extremely delicate and require constant monitoring to ensure that the individual doesn’t rupture a vein or develop an aneurysm. Sadly children that have EDS are often not properly diagnosed at a young age. Therefore their parents are typically accused of child abuse, especially if the child has frequent bruises and scarring on the body due to minor injuries.

Patients diagnosed with this disorder also have extremely soft, spongy skin. Their muscles are weak and undefined, and their skin may be easily pulled and “putty” like. Wounds may be difficult to heal, which will result in deep and obvious scarring.
Cardiovascular problems, as well as early onset osteoarthritis, are both common problems associated with this disorder.

Severe Symptoms Associated with EDS

The severe symptoms of EDS are not as commonly noted, and are rarer to the disorder than other symptoms. These symptoms are usually treated with a considerable amount of very specific medication. In some cases, patients may be asked to undergo trial experiments to treat their pain and symptoms.

Brittle bones are a severe problem with EDS, caused by low bone density. These people are likely to suffer broken bones that have a difficult time healing, as well as joint pain. Other bone conditions include spine deformities, such as Kyphosis and Scoliosis. Other spinal mutations are much more severe, but also rare.

Bowel conditions are also problematic for those suffering from EDS. Patients are most commonly afflicted with Irritable Bowel Syndrome as well as Gastritis.

Other symptoms and disorders that arise from having EDS include Carpal tunnel and other nerve problems, skin conditions, bone and blood disorders, as well as difficulty conceiving and giving a live birth.

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