Ignorance is bliss....or is it?

The last 7 months have been a bit of a fact finding whirlwind..... an emotional roller coaster that has taken me for a ride. First I felt relief and satisfaction that I had figured out the mystery of my many health issues. After a bit of research I felt overwhelmed and sad by the difficult road ahead. As I visit with doctor after doctor to determine baselines and discuss a game plan for my future care I have slowly found myself accepting that which I cannot change with a new found resolve. I am comforted by the fact that my findings will help my younger relatives live more of their life. Through awareness they will be able to avoid lifestyle factors that could jeopardize their health if they find they have indeed inherited EDS.
Like many others I spent Thanksgiving surrounded by relatives.
My recent diagnosis of EDS made it seem that much more important to load up myself and my newly acquired knowledge and travel the four hours to Oklahoma to see my family. Much to my surprise,my health was not a topic of discussion. Ehlers Danlos Syndrome was to be the 500 pound gorilla in the room that everyone ignored. My niece and nephew, both new parents, hadn't heard of the newly discovered genetic defect growing on our family tree. My beautiful and curious 21 year old cousin asked "Is this something I should be concerned about?" Why hadn't this information been shared with them? Why wouldn't you want to spare others pain by empowering them with information?
Why?
I drove home another four hours pondering this question. No good answer came to me.

It is frustrating to reach deep in your heart, past the sadness, to find the silver lining in a less than ideal situation... only to find that nobody wants to see it.

better living through chemicals...as needed

While listing my medications in preparation for a visit to the eye doctor It hit me just how much a genetic defect in your collagen can benefit your local pharmacy. The prescriptions for pain,inflammation,and sleep medications have quickly filled up my bedside table. They stand alongside my daily ADHD pills and many other non prescription remedies for me to pick and choose from...as needed. Excedrin or Maxalt as needed for migraine. Benadryl, Domeboro powder, Caladryl, or a Medrol dose pack as needed for allergic reactions. Albuteral inhaler or Advair as needed for asthma. Bio freeze, mineral ice, Advil, Mobic,and Darvocet as needed for pain. Tylenol pm or sonata for sleep as needed. Vitamin C is taken daily now for the possible chance that it can strengthen the vascular system. My sensitive skin requires special soap, lotion, and shampoo that is free of dyes, fragrance, masking fragrance, lanolin, parabens, and formaldehyde. Vanicream and Free and Clear products can all be ordered at the pharmacy counter. The staff at my local CVS have learned to recognize me after 11 years and 11 surgeries worth of visits. And I am very comfortable calling my pharmacist at the drop of a hat to make sure I don't mix and match what I shouldn't. He is always happy to help me at any time of the night. I just love 24hour pharmacies!

don't protease me

I stumbled upon a bit of information that is definitely worth sharing. It seems that pineapple can be hazardous to those diagnosed with EDS. More specifically the proteolytic enzyme bromelain that is found in the fruit and in higher concentrate in the stem. At first glance you might think that bromelain is the magic cure for many of the problems associated with EDS. Do a quick web search and you will see a laundry list of ailments it can be taken for and more than one advertisement that promotes the pill form of bromelain as a cure for Ehlers Danlos Syndrome. What they fail to mention is that bromelain is a protease enzyme....a protein digesting enzyme. EDS is a defect of the major structural protein in our bodies known as collagen. Duh! Even the pineapple growers of Hawaii list a warning on their web site against eating pineapple if you have EDS or a number of other ailments. Take note that meat tenderizer is made from bromelain too and similar enzymes can be found in papaya, mango,and kiwi fruit.
You can still eat pineapple if you follow one rule. Only eat it if it has been canned. Seems the heat from the canning process kills the enzyme. Good to know I can still have pineapple upside down cake. A small but tasty victory!

splint personality

I received my first seven silver ring splints.They should help support my finger joints and keep the dislocations and pain to a minimum. They are indeed beautiful and feel nice too but a few of them don't exactly fit and the right thumb splint just can't seem to keep my thumb in place. So.... we'll be tweaking them and hopefully my aching joints will get some relief once I am able to wear them daily. I've been thinking about how to describe the pain in my fingers. The best comparison I can come up with is the same tenderness you might have the day after you jam your finger on a basketball. Each joint in every finger becomes sore and swollen in a similar way when I use them. Ice helps. Ice packs, ice in a cocktail,and I'm sure ice cream would help too!

million dollar baby

My new ankle and wrist braces make me feel like jumping in the ring and going a few rounds....running up steps Rocky style....and dancing in place in a torn sweatshirt.
They are for added support while I do crazy joint stressing stunts such as walking and picking up things. The ankle supports are the same kind that place kickers use. Cool huh? They feel fabulous! The wrist support also has a handy dandy thumb support to keep my thumb from hyper extending which will help reduce pain and the groovy corset style lacing on the sides reminds me of a stage costume that Madonna might have worn on her Vogue tour. I just adore accessories! Speaking of which....my silver ring splints are in the works. Ooh La La.

Needy Women

Women have a way of stepping up to the plate with such gentle strength when they are needed. Their nurturing instinct prepared to do service when duty calls. These days the women in my life have rallied around me with their gentle loving spirit and kindness. They seem to know what it is that I need and more importantly what I don't need. I do not need sympathy.I need understanding and respect for what it is I am going through. I do not need doubt and questions.I need friends that care enough to inform themselves about my condition so my illness will not consume our every conversation. I need trust. Although it is not ideal, those closest to me know the relief and sadness that came hand in hand with my diagnosis of EDS and they trust that I have come to this place honestly. It is not my job to convince anyone of my illness. I need someone to listen when I'm frustrated by the absurd and insensitive things people say. I do not need advice. I need a little help around the house. I do not need to give up my life. But most of all...I need to laugh, a cold beer, live music, and a heaping plate of nachos.

Water Bored

Well, I am about to complete my 6 water therapy sessions. My therapist is showing me what I can and cannot do without damaging my joints because even a water class designed for the elderly would be considered too stressful on my failing joints. Then I will be on my own to continue at the community center pool. The exercises (and I use the term loosely) are a bit silly. Especially with my athletic history of track, soccer, tennis, and bodybuilding. I remember working out so hard that I couldn't walk a straight line to my car... Doing 400 sit ups a day....spending a sweaty hour on the treadmill. Now, here I am holding onto the handicap railing in the pool and walking to the end of the pool and back. Then I get to change it up and do it backwards! Before my 20 minutes are up I have performed several more humiliating exercises such as lifting my foot 45 degrees behind me and lifting my straightened arms from down at my sides to the surface of the water. And work it.... and own it.....one and a two. An elderly man is over a few lanes doing laps. Something is wrong with this picture! Believe it or not,those silly little exercises kick my behind! And it is nice to spend twenty minutes of pain free movement. The water is cool and soothing. And in the water, I hardly notice the extra 30 pounds that came quickly after I had to quit exercising. My second dad Ted taught me a quote that I find myself repeating a lot these days- "Things work out best for people who make the best of the way things work out." Thanks for the positive attitude Dad! It's coming in handy.

My people

I have found my people on the facebook Ehlers Danlos group page. Their post
You know you have EDS if...... told me so. For your enjoyment and a little insight into EDS here is a bit of the list.

You know you have EDS if......
The highlight of your day is bedtime.

Even your dish detergent is fragrance free.

Your most prized possessions are your wrist braces.

You can't lose weight because all of your meds say to take with food

You didn't realize that not everyone can put sun tan lotion on their own back.

You leave the caps off of your medications so you won't have to take them off again.

While bending over to tie your shoes you wonder what else you can accomplish while you are down there.

Your favorite game is guess how I got this bruise

You can't remember if the post it note reminding you to take your pills is from today or the one you just wrote for tomorrow.

You head to the bathroom, stub your toe, sit down to check out your injury then start to watch TV and forget to go to the bathroom.

Medical students flock to the examination room to ooh and ahhh at you.

You are on a first name basis with your doctors.

Your calendar has more doctor's appointments than meetings

Every guy you ever dated remembers you soft skin.

You've ever laughed and dislocated something.

Your "to do" list has two things on it and you don't get one done.

brace yourself

One of the issues associated with Hypermobile Ehlers Danlos Syndrome is joint laxity. When I read that my joints would one day start to dislocate spontaneously I was certain this would be a bit down the road. Surprise!....my left middle finger decided
it would be the "ceremonial first" to introduce me to the joys of dislocation. Not to be upstaged, my right knee joined the festivities soon after. My hand therapist began the splinting process with some plastic make shift finger splints that will help her determine the type of splints I need. The plastic splints will be replaced by groovy silver ones that will make me look like a gypsy. The hand splint (which looks like a Jason mask) is to keep my thumb from hyper extending. I also have a lovely new knee brace. I apologize to whoever is behind me the next time I have to walk through the metal detectors in the airport. It may take me awhile to shed all of the metal gadgets adorning my body. Brace yourself....it's going to be a bumpy ride!

Bull in a china closet

All my life I have been considered clumsy, klutzy, accident prone.... a "bull in a china closet" my mom always said. If there was an uneven surface or a pebble in my path I would trip, fall, and sprain my ankle. If I came into contact with anything remotely sharp my skin would tear. "You could cut yourself on a rubber ball" my best friend would tease. And the bruises! I can't remember the last time I didn't have at least a few bruises on my body.On occasion enough that it made my male friends afraid to be seen with me for fear people would deem them an abuser. An active life full of sports, a "can do" attitude, and a labor intensive career path made my many scrapes and injuries seem justified. But now, at 41, I can laugh in the face of such ridicule. I am not an unaware klutz. I was watching where I was going. I will no longer be labeled a bull in a china closet! But come to think of it......right now, I'd love it if I were indeed just clumsy. It's kind of a cute word.

but you don't look sick

I have had a handicap placard for some time now due to my many knee surgeries. But I allowed an incident several years ago to rob me of what I now know would have been
a great benefit to my long term health. Not long after a knee scope a woman followed me into a Target store from my handicap spot and proceeded to verbally assault me. "You don't look handicapped!" she yelled in an attempt to shame and embarrass me. I'm certain what she considered a suitable punishment for my wrong doing and a noble act on her part. Those who know me, know that I am both non confrontational and an extreme introvert. So, needless to say I was both hurt and humiliated. Somehow I mustered up the courage to walk up to her. With tears in my eyes I informed her that I had just had knee surgery and that she shouldn't speak when she has no idea what she is talking about. I knew my words were wasted as she nodded in disbelief and judgement. And she continued to spew ignorance as her husband pulled at her arm and mouthed a silent apology to me. When I told my Doctor of the incident he shared with me a thought that has served me well in many circumstances "How dare she pretend to know your truth."Doesn't that just say it all? How many times do we make judgments about people when we don't know the whole story.....know their truth. That little statement has led me to live my life with a bit more understanding. It has led me to assume in the direction of understanding. And it makes for a pretty good retort "You don't know my truth!" Very dramatic. Now that I will be using my handicap spot more often I will be dusting off that phrase as well as a few others such as..."Ehlers Danlos look it up jerk!" or "You can't see pain!" and just for fun "guess which leg is fake!" In a continued effort to look at the bright side of Ehlers Danlos Syndrome I have to admit that I do love my rock star parking.

Physical Therapy

I met with a very kind Physical therapist yesterday to discuss deep water therapy.
It seems to be the preferred method of cardio for EDS patients. She impressed me immediately with the fact that she had researched EDS and knew that I was going to have to be my own advocate. She was gentle with me...she noticed how I held my pen and could see the discomfort in my hands, she noticed the changes in my skin coloring when I over heated while walking past the hot tub. She noticed tears in my eyes when we discussed keeping me out of a wheelchair for as long as possible and then I noticed her sympathetic tears. She was soft spoken and completely present.Offered me tips on saving my joints and let me know that every little thing I don't do now will keep me out of a wheelchair that much longer. Things like mowing the lawn, pruning trees, fixing things around the house, walking up the stairs,lifting heavy items, washing my car. She also gave me a great piece of advice. "do the things your heart truly wants to
do and forget the rest" The EDS patient can go to a concert or Worlds of fun but the
handicap "hangover" may require two days of bed rest to recover. I appreciated her gentle candor. The last thing I need is someone telling me "everything will be okay"
when, to loosely quote my magic 8 ball -all sources say otherwise.

Chronic Pain

It's hard to explain chronic pain. We've all been sore after a hard work out, sprained an ankle,or stubbed a toe. It hurts a while then gets better and the memory of the pain disappears soon after the bruises fade. What if that sprained ankle didn't stop throbbing? What if your ankles, knees, wrists, shoulders, and elbows all felt as if you had hurt them yesterday? It's hard to imagine and even harder to live with. Unfortunately, chronic pain is a very real part of living with Ehlers Danlos Syndrome.The pain is there when you go to sleep and there when you wake up. Pain can rob you of hours and hours of much needed sleep. Like a slow and steady flashing light
attached to a sawhorse in a construction zone the pain pulses. Wrist...knuckle...elbow...ankle...knee...shoulder...thumb...jaw...over and over in random order my body tells me how it feels. Relief comes in various forms. Pain killers, biofreeze, ice, and a few too many beers all help to give me a little comfort.
A sense of humor helps too. It's hard to cry when you are laughing right? At the end of the day I'm happy to have made it through... ever mindful of those who are feeling worse and grateful for the little things that bring me joy everyday.

Symptoms of Ehlers Danlos

After meeting with the genetic counselors I have a diagnosis of Hypermobility type EDS
with enough Vascular type factors to be considered highly probable Vascular type EDS

My symptoms include
hernia
Fragile and soft skin that tears and bruises easily
Hypermobility of joints/loose unstable ankles
Feet Pronate
tendinitis in wrists, elbows, and shoulders
early onset varicose veins
veins visible through skin
fragile veins (phlebitis)
dilated aorta
fibrous growths on shins
piezongenic papules
family history of brain aneurysm/heart conditions
Asthma
TMJ
Family members with hypermobile joints and mitral valve prolapse
Near sighted
Thin hair
Joint problems-nine knee surgeries for cartilage tears-meniscus transplant in right knee-two shoulder surgeries for bone spurs

My story

After nine knee surgeries, two shoulder surgeries, and one hernia surgery I finally know what is really wrong with me. While watching an episode of Mystery Diagnosis I began to recognize myself in the many symptoms of a rare genetic disorder called Ehlers Danlos Syndrome. Multiple joint surgeries, hernia, family history of brain aneurysm, joint hypermobility (knees bend backward, thumb can bend back and touch arm),fragile skin that tears and bruises easily, veins easily visible through skin...and that was just a few of the many symptoms I had in common with the patient on the show. When I approached one of my doctors with the idea that I might have EDS
he couldn't believe that no one had ever suspected EDS before. With his encouragement I went to see a cardiologist at KU Med. After a thorough exam I was indeed diagnosed with EDS. And so my journey begins.