Adoption Interview

Adoption Bloggers Interview Project 2012

Sunday, September 19, 2010

Ehler's Danlos Syndrome

If you are a regular reader here you know that Hannah is EXTREMELY flexible, like you can take her thumb and bend it sideways to lay it flush with her wrist kind of flexible. She also has really flat feet and her feet collapse outward significantly. An orthopedist had been watching this since she was about 18 months old using the wait and see approach. Last October when Hannah starting complaining of pain in her hips we were back to the doctor again and she was given AFOs for her shoes and pronounced at the extreme edge of normal as far as joint laxity. The doctor called it hypermobility syndrome. Well it was time for her yearly follow-up last week and I believed that Hannah had outgrown her AFOs because she was complaining that they hurt her feet which she hasn't done since getting used to wearing them last year. Besides checking out the AFOs the doctor completely reevaluated her because of some concerning trends he had noticed in her gait. Because she's heavier now her ligaments are under even greater strain and her feet, ankles, and knees are showing the stress and are starting to show the effects of over compensation. It was hoped that her ligaments would tighten some with age and that the AFOs would be a temporary thing. Unfortunately, things did not improve. In fact, they got worse. After the evaluation the doctor informed me that Hannah had a mild form of a genetic disorder called Ehler's Danlos Syndrome. I don't know much about the disorder yet, but from what I understand it is mainly a defect in the production of collagen. Because the collagen is defective ligaments, blood vessels, skin, and anything else made of collagen are suspect to problems. So far, it appears that Hannah really only has issues with her ligaments and skin, but the doctor wants her to probably go see a cardiologist in about 6 months to get a baseline idea of her heart health in case any issues crop up as she gets older. There is no cure for Ehler's Danlos unfortunately, but the symptoms can be treated individually. So, Hannah had her AFO's adjusted, thank heavens she hadn't outgrown them since they cost $600 a pair and are NOT covered by insurance. She is also going to have to wear something called a Dennis Brown bar at night for 6 moths to a year (then she'll be reevaluated to see if it's helping or if she'll need surgery). I am SO NOT looking forward to this bar. It is basically a pair of shoes attached to a metal bar that holds the legs in a fixed position. They used to be used for infants a lot in past generations to correct bowlegs, in toeing, etc, but now they are basically a thing of the past and I can find absolutely NO information on them being used in a child as old as Hannah. Everything I have read about them show them being used on immobile kids. In fact, the idea of kid Hannah's age using them is so far fetched that when I went to order the special shoes that have to be bolted to the bar, the medical equipment guy had to look through several manufacturer catalogs before he found a place that even made Hannah's size (size 12 in case you were wondering!). Hannah hates wearing shoes and we have a hard enough time keeping her in her shoes during the day so I'm dreading trying to get her to wear what look like very uncomfortable shoes at bedtime. To make matters worse, she's a belly and side sleeper and with this bar she will have to sleep on her back. She also wakes at least once a night to go to the bathroom and I'm not sure how she's going to let us know she's awake if she can't get up out of bed and walk to the door to knock on it like she does now. We are definitely going to have a big learning curve here soon once the shoes come in. Wish us luck.


pixiemama said...

Wow. I bet this all threw you for a big loop. Hope you are feeling OK with the added diagnosis.

Good luck with the night issues. Maybe break out the baby monitor again?

Mama said...

Pixiemama, Yeah it's a lot to digest although I've known in my gut that something wasn't quite right with her joints for a long time even though all the docs were saying she's just a little extra flexible. Unfortunately our baby monitor died years ago, but getting an inexpensive one to try out might be a good solution. She could just quietly say, "I have to go to the bathroom," and we could come get her. Now to just figure out a way to get a kid who doesn't sleep well to begin with to change her sleeping position and a kid with sensory issues who hates shoes an socks to wear them both 24/7! Say a quick prayer for us if you wouldn't mind!

m said...

hello you. it's upsetting to hear about this new direction things have taken, can't imagine how you guys feel. the lack of information about the disorder is especially frustrating, hopefully there are some resources somewhere, i don't know. i just know that hannah, as she goes through all of this, is so lucky have such wonderful, loving parents...that's going to make such a huge difference.

talk to you soon, take care.

Mama said...

M, I've managed to find some more info out there on EDS and I'm feeling much less panicked now :) It's the heart part that scares me, but at the moment she doesn't appear to be having any issues with that. Once we see the cardiologist I think I'll be able to rest easy. Dislocated joints I can deal with. . . . That's not life threatening. It's just the heart and blood vessel abnormalities that completely scare the crap out of me!