Saturday, November 8, 2008

Lymphedema- My Story

As many of you know I have lymphedema, Primary Bilateral Lower Limb Lymphedema to be precise. You probably have no idea what it really is. Don't feel bad. I've had a doctor tell me I probably knew as much about it as he did. Not really what you're looking to hear from the person you've been sent to by your primary doctor since she didn't know anything either. I've been told it's just something you're going to have to learn to live with. This was from a surgeon, which from his point of view, was essentially correct. He couldn't repair or cure my lymphedema with surgery so it was not something he could affect therefore it was just something I would have to live with. He gave me a prescription for a lymphatic pump and walked out of the room.

I've had lymphedema since I was about 5. As a child, I had all sorts of tests done, most of which I blissfully do not remember. I was tested for cancers, heart disease and just about anything else they could think of since no one was thinking Lymphedema. I even had exploratory surgery at one point to rule out I'm not sure what. That was in third grade. I spent every day from about 1st grade onward in a compression garment until middle school when I rebelled. I don't really remember the day to day struggle with the garment except that it was hot (I grew up in Houston) and uncomfortable. In fact, it wasn't until shortly after my mother died that in a discussion with my sisters I learned that they had been recruited to help me get the garment on when I was a young child. Let me try to explain why this was so and what it means to me. First off, a compression garment in appearance looks like a pair of tights, except much heavier and made of, at the time, much coarser material. They were kind of like the material of a knee or wrist brace you can buy a Walgreen's expanded into the form of panty hose. They were difficult to put on and as a child I required help to do so.

Here's the neat part: I have absolutely no recall of either of my sisters ever doing this.   None. I don't doubt that they did it. It only makes sense when I look back, knowing how difficult it was for me as an adult to get a garment on. I think this struggle and the resulting discomfort is what created such a high pain threshold for me as a teenager, and my reluctance to tell my mother when I was in pain. Dude, the psychological impact of this is a post unto itself. Suffice it to say that I was conditioned to ignore the pain, was subliminally aware of the resentment my sister's felt with out knowing why and grew up being "different" which as we all know pretty much sucks.

My lymphedema was well controlled throughout elementary and middle school. When I got to middle school and encountered the demon of gym, where we had to "dress out" I soon discovered that I was going to have to explain on a near daily basis what the hell I was wearing. It got old very quickly. So, I moved into a knee high compression garment that I could effectively cover up with socks (ah, the 70s!) and that was the end of that. Except that I gradually took to wearing them less and less and by the time I was in college, was rarely wearing them at all.

Again, my lymphedema gave me little problem. My ankles were almost always swollen but that was really about the extent. But gradually as I grew older, had children and started gaining weight, my lymphedema starting getting worse. I sought treatment once after my eldest was born consisting of a compression garment. More like a Chinese torture device. I don't know if you got from the description above how grossly uncomfortable these damn things were. Actually, it was more than uncomfortable. It hurt. It was most likely not well fitted but I was quickly discouraged enough to refuse to wear it.

As it continued to grow worse, I tried again. This is when my MD sent to the doc who was so smart to tell me I knew as much as him. He gave me diuretics and dismissed my questions about a new treatment I had heard about, Complete Decongestive Therapy. His explanation was that it was not a cure, worked well only as long as you were actively undergoing treatment and was not worth the time. Of course, he was thinking cure. Not management. This is important to understand. Since so little was and is known about Lymphedema, it has taken a very long time to shift the focus from cure and the general lack thereof, to management, which for the time being is where the focus needs to be.

So, the drugs didn't work. More years went by and I learned more information. I discovered a clinic in Austin that performed CDT and started the process with my insurance. They refused to cover it, calling the treatment non-standard and experimental. I would have to prove standard treatment a failure first. At my demand, they sent me to their "expert", the surgeon. The pump he prescribed for me came with no instruction on its use or potential misuse. It made things worse.

A few more years went by. On and off insurance over this time, I was left with very little I could productively do. I then had an episode of cellulitis that put me in the hospital. Low and behold, insurance was now interested in helping. So, once again I sought out the clinic and with some back and forth between we will pay, we won't pay, okay if you make me, I finally got some treatment. It was great. I was fitted for a compression garment and discovered that there had been some serious advances in their manufacture, as in, they were no longer Chinese torture devices.

Now I knew what to do and the only real handicap in keeping up with my treatment lay in my own commitment to the garment, bandages, etc and insurance. Because of the advanced state of my condition, I require a custom-fitted garment. These cost between $200 and $400 a piece and must be replaced at minimum twice a year. Without insurance, that cost was too great some years.

For almost a decade, we were on and off insurance as the job market allowed. In that time, I started having recurring bouts of cellulitis and other complications, that I lovingly referred to as leaking. A small nick on my leg would leak lymphatic fluid and sometimes continue for hours at a time. Fun times.

It was in this decade that we discovered one of our daughters had inherited this condition. At the time I was having children, no one knew enough about Primary Lymphedema to know that it was genetic. So when my daughter started experiencing swelling in one her legs, I was immediately concerned. My worst fears were confirmed and the progression of her condition has continued at a far faster pace than mine at her age.

All this was topped off with my most serious bout of cellulitis. I was in the hospital for three weeks. I became septic, the infection acted in ways it had never before and spread to the other leg. I spent a week in ICU, most of which I do not remember. It almost killed me.

Since then, both my daughter and I have received CDT and are faithfully wearing our compression garments, Nothing like a little near death experience to wake you up.

Next, a more medical discussion on the condition, where research is headed and how important education is for the medical community and patients.

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