Since Meat was diagnosed with Klippel-Trenaunay Syndrome as an adult, he and I have done a lot of learning as we go. Since most of the doctors he encounters have no clue what the condition even is, let alone experience in treating it, we've had to research, ask questions and apply what we've learned. Sometimes we have to try different therapies before we find one that works for us. One thing we can say with certainty about our life with Klippel-Trenaunay Syndrome is that it is a condition that requires constant altering, adjusting, re-thinking and learning.
The most obvious 'symptom' of Meat's K-T is the birthmark. It covers his skin, to those who see him the first time, it is his skin. It's what draws the most stares, the most questions. Thankfully despite being the outermost part of him, most of the problems we've had with his skin have been manageable.
How Do You Keep It Safe From Harm?
The biggest challenge we have is keeping his skin safe from harm. The typical wounds we take for granted - paper cuts, scratches, kitchen injuries - all tend to cause Meat's vascular malformation to bleed profusely. This is quite different from the venous ruptures he's had from his varicose veins. It would be easier if we were talking about an isolated area but we're talking about his entire right arm down his chest, half his back around his side...and his buttock down his leg. Which leaves a lot of area to protect. Some memorable bleeding events have involved our son's dog jumping up on Meat, our cat barely scratching him and a scrape on our fence. The bleeding from his affected side is much worse than that of the other side. He has scraped both hands and not bled on his left hand while his right hand injury quickly pooled and dripped.
His compression garments help with some protection, covering his skin at all helps with some protection. This is much easier in the cooler months than during our hot summer. Sometimes his compression garments, cannot be worn since they aggravate his ulcers and dermatitis. So we haven't come up with a foolproof method to protect his skin.
Weather also affects his skin. Heat and cold impact him more than the rest of us, the vascular malformation being particularly touchy when the weather is too warm or too cold.
Dermatitis
Meat's skin itches. A lot. He also suffers from crawling sensations. Some of this is caused by neuropathy, but to him it is something he feels on his skin. Keeping him from aggravating dermatitis and/or causing wounds from a simple itch is a challenge. I've mentioned that we use coconut oil to keep his skin hydrated and that does help. We do have to be careful of what we put on his skin as products with excessive ingredients can irritate it. We've had success using some lotions from the health food store (Aubrey and Desert Essence are two good brands) and pure olive oil soap (which can also be found at health food stores or ethnic grocers for a little less money) helps to keep his skin clean without drying it out.
Ulcers and Aseptic Cellulitis
Ulcers and aseptic cellulitis have caused us the most frustration in dealing with doctors that have little experience treating Klippel-Trenaunay. Since most doctors are used to treating diabetes-related ulcers or septic cellulitis, not only do they not immediately consider it an option, but have often been incredibly degrading to Meat when he brings the subject up. This happens on a regular basis at the Oklahoma City Veterans Administration hospital where he is treated, and the frequent cycling of doctors does not help this.
Meat's ulcers have ranged from tiny to nickel-sized. When he begins to complain of the pain or notices one, we get him to rest with the leg up. The first doctor who diagnosed and treated one (an internist with an interest in vascular conditions) prescribed an Unnas Boot and rest. We've found out since that this is the generally the indicated treatment for these types of ulcers. Since we have moved into the OKC Veterans Hospital system, however, the doctors here are quick to ridicule and belittle him first and apologise later. When he has gone in for ulcers, he's been told there is nothing to worry about and that all he needs is a regular latex bandage. Even his vascular doctors are little help as none of them have experience treating Klippel-Trenaunay or conditions involving vascular malformations. Often we are left to do what we can and so we keep the ulcer bandaged and his limb elevated.
Aseptic cellulitis is an even stranger beast to get treatment for. We have been dealing with this particular problem for a little over two years. His first vascular doctor insisted he had bug bites and prescribed NSAIDS, which are contraindicated for him. After her repeated insistence that the spreading was still bug bites she referred him to dermatology, who also had no firm answers about what it was. Earlier this year he saw a doctor at the VA hospital's Acute Care clinic who laughed at him when he asked if it could be aseptic cellulitis as it has been reported in K-T patients. She told him that in 20 years of practicing medicine she had never seen it so while yes, it certainly hypothetically exists, she personally did not believe in it. It was hard not to laugh since this same doctor had an ash smudge on her forehead and was telling her colleagues that she needed to leave early for an Ash Wednesday service. I guess it's easier to believe in some things you've never seen than others.
With a rare condition, so much learning is really done as we go along. It's challenging to deal with doctors who believe they have all the answers yet have none for you. It can get disheartening. Support is very important, whether it's from family, friends or a support group. If you're a family member of a newly diagnosed K-T patient, I would encourage you to research as much as possible, ask your doctors and most of all, be understanding and kind. What may seem typical or average to you may not be for your loved one. If you have Klippel-Trenaunay, don't be afraid to speak up, don't be afraid of looking weak or not being the same as others. Be honest with how you feel.
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