Life Expectancy???????

EDS is a genetic condition and with this comes the expectancy of "How long am I going to last with this or when am I going to get my ticket off this planet?" Well it is a question I have never really wanted to know, especially with sayings like...."You have more chance of being killed by a terrorist than getting married after 35". Considering I am 37 and have yet to find my 'Prince Charming' those odds suck. However, when genetics play such a large roll in any ones life it is something to consider. EDS has many types, as I am sure you now know, but the good news for me is..........I have a completely normal life expectancy. YAHOOOO!!!!

In July 2006 I simply popped my neck back into place (as all Classic EDS have loose joints) and as I was rushing to make dinner for guests, I was very vigorous on the popping. I felt a strange 'whoosh' in the base of my head and within the hour my head felt very heavy. A couple of glasses of wine seemed to fix the dull pain and I went to bed. The next day I had a terrible headache and thought that perhaps I had a wee 'wine headache'. That night I went to bed and could not get comfortable. The pain in my head was heavy, and it felt like I was lying on concrete. Monday turned up right on time and I went off to school. I told my babies (my 5 year olds I taught) that Miss Brewer had a terrible headache today and made sure to remind them to talk in there very quiet inside voices. By 10am I started to have the on set of a migraine, I had blurred vision and my head felt like lead. I left my class and went to the sickbay for more panadol. I spoke to the Assistant Principal and she was convinced my neck was out and was going to ring for one of our Chiropractor parents. I said I had to go home and lye down, it was just a migraine and I was going to be fine. That night I was not fine and I called my Mum crying with the pain in my head. "Go to the Hospital now!" were her exact words. So I did. My boyfriend at the time took me. I was so moody and in so much pain in the base of my head.
At the hospital the Desk Nurse asked me out of 10, what was my pain like. I held my head as I was convinced at any moment it was going to fall off and said it was a definite 50 out of 10. I was ushered straight into A & E and the rest was a blur. I was given morphine and the amount of test I had was astronomical. The moment I said I have EDS the blank faces emerged and in my slurred, drugged speech, I told them to 'goggle' it. I had a spinal tap, many bloods taken every couple of hours, M.R.I's, absolutely e.v.e.r.y.t.h.i.n.g!!!! I was a little embarrassed in my drugged state as I thought, "What a lot of fuss over a headache, they better find something".

They certainly did, I had a 'left vertebral artery dissection', I had broken one of the main arteries in my head by putting my neck back into place too roughly. The heavy feeling in my head was blood congealing and it was so much more than just a headache. The scariest thing was, I had to then wait for 6 days in hospital to see if I was going to have a stroke. I was only 35 and I couldn't possibly have a stroke, worst of all the doctor told me if I had not come to the hospital where I was being pumped with blood thinners, I could have died.

The amazing thing is, is I didn't, I didn't have a stroke, I didn't have speech or gross motor skill problems, I was OK and after a few months of warfrain (prescribed blood thinner), 101 blood test (I looked like a heroin junkie with all my track marks), a few more months of Cartia (more blood thinners), I was fine! At my 1 year check with up Dr Singh, he was impressed and so was I. I had suffered no side affects what so ever.

Almost two years on, I don't put my neck back in anymore, and I am still 100% fine. It was a huge fright and I am careful now not to lift heavy things and when I am exercising I don't put any strain on my neck. I was really lucky and I know I was and that has helped me to become even more positive about my life. I don't 'sweat the small stuff', I don't spend quality time with people who bring me down, and I love the people even more that hold me in there hearts. There's nothing like a near death experiences to make you see rainbows where ever you go!

Love Rowena

x x x x

Stitches and Scars!

Stitches are a big deal when you have Classic EDS. Here are my super model legs!!! Nice calves though I always thought. Now the scarring that occurs is called 'widened atrophic scars' or referred to as 'cigarette paper' scarring, as they are terribly thin and without using any imagination they look like crumpled cigarette papers. No hair grows back on the wide scars (which is great as there is less to shave) and they are so sensitive that I can tell someone is going to touch them without even looking. No tears over my scars though, as I wear them like a badge of honour. I have had over 300+ stitches in my life and my left knee has been split open 3 times.

Here are my knees a little closer. The skin is very stretchy and I can pull my knee skin away from the knee cap about 2 inches. It was amazing for 'party tricks' as a child but I don't tend to show me scars off at parties anymore!

Love Rowena

x x x x

Here are the Medical Characteristics of Classic EDS!

The Classic Type:

Major diagnostic criteria
Hyperextensibility of the skin
Widened atrophic scars
Joint hypermobility
Can lead to osteoarthritis in the 3rd or 4th decade
Other features
Poor wound healing
½ of affected individuals are delivered up to 1 month premature due to premature rupture of fetal membranes
Some have cardiac abnormalities
Mitral valve prolapse
Aortic root dilation with occasional rupture
Scoliosis
Pes planus (flatfoot)
Molluscoid pseudotumors (calcified hematomas) may be associated with scars
Inheritance
Autosomal dominant single-gene disorder
Etiology
A major cause is mutations in type V collagen
At least 3 loci are involved
Biochemical Defects
Thickened collagen fibrils in skin as well as "cauliflower" deformities of collagen fibrils
Mutations in COL5A1 and COL5A2 have been seen in some families
No biochemical or molecular based testing methods have been devised to provide reliable results

Sounds very yucky really, however my case is classed as "mild" even though I have many of these characteristics.

Love Rowena

x x x x

In The Beginning!

If my beautiful Mother had one thing to describe finally being pregnant with me, it was this...."I was beside myself". She had waited 7 years and I finally arrived. If only she knew the amount of hospital visits, doctor visits and worry I had in store for her.

You see it all come out in 'the wash' the day my mother took me to her regular plunket visit. The Plunket Nurse had said to my mother "It seems very strange that your child has these bruises". I had finger prints all over my small frame from just my mother picking me up for some love. She went on the tell my mother that she would need to discuss this with someone else. Well that someone else was Social Services and my mother again was 'beside herself', she still refers to it as "The Worst Day of Her Life!" They asked a myriad or questions and all my mother could say was "I waited 7 years to have her". You see I have 2 older sisters and obviously the lack of EDS in them meant they never had bruises, so they left satisfied with my mother crying. Mum then made a speedy doctors appointed.

Good old Dr Greenfield and I would become 'Kindred Spirits', but the day my mother took me to him was the start of many hospital visits. Dr Greenfield contacted Auckland Hospital and Dr Gluckman was to be the man who discovered what I had. He took one look at me and within a moment of pulling at my velveteen skin he told the nurse...."We've got one, don't tell anyone, we've got an Ehlerlos Danlos Syndrome in New Zealand!" I was the first one and I believe I appear in some dusty medical journals somewhere in a Medical Library in Auckland.

Well you can imagine my Mothers reactions, it wasn't of worry at this point, it was of great relief that she was not a 'child beater' and you guessed it, she marched back to the Plunket Rooms in Howick, waving the piece of paper of evidence, 'SHE DID NOT HURT HER BABY'. Then the truth sunk in, no ones life was going to be straight forward anymore.

37 years ago there was hardly any medical knowledge of EDS but this is not the case today. However, saying that still doesn't prepare myself or my gorgeous son the freedom to have an accident, go see a doctor, say my EDS speel and for every single doctor to stare back at me with the "What the hell are you talking about" face. Once you begin to explain it, they will then use the "Yes it looks like I am listening to you...but I'm not".

I really wanted to start this Blog just to say that "I am here"! I am living EDS everyday, I have Classic EDS (Type 2) and it is not all 'doom and gloom' like you see on 'youtube'! It's okay living with EDS, it can be painful, annoying and just a pain, but I don't know anything different, so this is my life and it's a lovely one!

Love Rowena

x x x x