This is an edited post from my other blog, Planet Crazy. Since the focus of this blog is on KTS and all the other fun little medical things that may or may not come along with it, I figured it should have a cosy home here as well. Again...I'm not a doctor because, well, cutting up any type of animal makes me queasy. So please go to your own health care professional regarding questions about your own health.
I'm
hoping to give a basic overview of what Klippel-Trenaunay looks like in
Meat's case and will write more in the future about some of the
problems we've had living with K-T, some ways we've adapted our lives
and supplements he takes. Currently, the biggest problem facing those
with K-T is ignorance. Unless you've heard of Martin vs. The PGA
or read Carla Sosenko's article about it, likely you've never heard of
Klippel-Trenaunay either. Unlike, well, diabetes, hyperlipidemia,
rheumatoid arthritis, even bipolar. Which is fine. Except that
doctors know about as much as the general public. Not a good thing, nor
very helpful when faced with a rare and potentially life-threatening
condition! Only with awareness, will we have a chance to get proper care
for our loved ones.
The Basics
Meat
has Klippel-Trenaunay Syndrome, also known as Osteo-angiohypertrophy.
If you're interested in reading about it from a clinical point of view, here
is as good a start as any. When he was diagnosed a few years ago (most
K-T diagnoses are given at birth or early childhood) Meat's doctor wrote
down the name of the condition and told him to look it up on the
internet - then he would have about the same amount of knowledge
regarding it as the doctor did. Yes, it's that unusual a condition. Most
doctors we have spoken with have never seen it before and have told us
that they have no idea what they are dealing with or how to treat it.
Even doctors who claim to have knowledge about K-T or have had
experience don't know how to treat it. In our case, we've had more
doctors who have totally been out of the ballpark on diagnosing
complaints associated with K-T than we've had doctors who knew what to
do. Meat appeared asymptomatic as a child so his doctors simply
diagnosed him as having a "birthmark". When he went into the military,
many of the hallmarks of Klippel-Trenaunay became evident although
doctors still did not diagnose him. In fact, a VA doctor had written
"Klippel-Trenaunay?" in his records but no VA doctor ever followed up on
it. It was actually my doctor (a DO) that brought up the subject of
Meat's condition during one of my appointments. Without his leads, we
might still not know what we've been dealing with.
Appearances Aren't Everything
I
know I'm biased and I adore my husband so it's only natural for me to
say that he's a unique fella and a one-of-a-kind model. But I'm talking
about his spirit when I say that. Most people only see the outside of
Meat, and many of them have a lot of questions. It's an undeniable fact
that Meat is a mutant. At first glance he may look pretty human, but
there's the large matter of his birthmark...so large that it takes up
(more or less) the entire right side of his body. Those who have no
experience with birthmarks have asked if he was burned, injured, or had a
rash. His doctor refers to it as an "arterio-venous malformation". It
tends to be red or pink and will become more red when he's warm and
purple when he's cold. I really don't like it when friends or
acquaintances ask me about his birthmark instead of asking Meat
outright. I presume they are trying to be delicate but it ends up
feeling like gossip. I think like anything else, people with K-T have
days that they feel more approachable than others. But overall, Meat
doesn't mind it, every question is an opportunity to raise awareness!
The
skin AVM/birthmark requires a little care, he's susceptible to eczema
and cellulitis. He's been prescribed lanolin and steroid creams,
originally his doctor told him to just schmear a bunch of vegetable
shortening on it to keep it supple. We use coconut oil as it seems to
work the best and smells lovely. He has a vaso-constrictor spasm
problem....which is really saying he has a similar condition to
Reynaud's Disease but not quite. During the winter we make sure his hand
and foot are covered well because he loses sensation in his fingers and
toes. The last two fingers don't recover well from cold. Oh, and how
could I almost forget about the Bleeding Issue: where his skin AVM
covers is much more likely to bleed from any small scratch than his
"normal" side. If he nicks his hand doing any everyday job, he bleeds.
Sometimes a lot. My son's dog jumped up for attention and his claw
grazed Meat's side. It bled profusely. Excessive bleeding can be a big
problem for K-T folks, Meat's been told to be vigilant because while not
probable, it is possible to exsanguinate. Say, in his sleep. Not
probable though...
'Those are really groovy shoes...'
After
the birthmark, strangers are likely to comment on Meat's groovy
footwear. I will be the first to cringe at his love for '70s American
music and his past brushes with heavy metal but believe me when I tell
you his Kiss Boots are not an expression of his Inner Ace. When he wears
his boots, he is 183 cm* which is his height as measured by his right
leg. Without his boots, he's 177 cm** Because his care for his growing
right leg was sporadic and mostly ignored during his military service,
he also has scoliosis, kyphosis and arthritis brought about by his body
attempting to compensate for the difference in lengths. His right leg is
still growing, as is his right arm. Both his right arm and leg are also
perceptibly larger in circumference than the left. His trunk is
affected and is larger than the other side. If he were a kid, he might
have surgery or some other corrective measure for it. He's an adult and
his arm and leg are still growing. He keeps getting higher lifts. I
don't know how long this will be practical.
There's
quite a bit of pain associated with this, he has osteo-arthritis in his
hip and knee. His back hurts. He's been diagnosed with osteopenia. Due
to the excessive growing of his right leg, it is brittle, weak and his
ankle twists easily. He wears fitted casts on both legs. Over his
compression stockings to keep the swelling down and protect his numerous
varicose veins on his right foot and leg.
*that's 6' for all you Americans, Liberians and Burmese who may not speak metric
**and that's 5'10"
The Human Time Bomb
To
round out the joy, Meat also has a spinal AVM, it's located between T-4
and T-6. We know very little about it overall, after it was initially
diagnosed, no VA doctor has followed up on it despite our repeated
asking. Each doctor refers us to another doctor before 'anything can be
done'. I kind of think it's like what we deal with when we save up the
money to see outside doctors: they really have no idea what they're even
looking at. Steve Slocum of Spinal AVM Information
compares having an AVM to having a time bomb in your body. It's an
excellent analogy. We're hoping that the placement of his AVM would mean
that it's low enough to not affect his brain if it bleeds. But it could
result in paralysis. There have been some symptoms and lately there
seem to be more of them: headaches, back pain, bathroom issues, pain,
numbness and tingling in an arm, visual disturbances. The most
frustrating thing about this is not having any doctors take us seriously
and not having the money or insurance to go out of state. Aside from
the small fact that many of the treatment options leave AVM sufferers as
bad or worse off than before treatment. But the risks without
treatment...it's a scary place to be.
Every time I get
my hopes up, when we scrape together enough money to see a doctor or
write to an out-of-state doctor or hospital in hopes of guidance, it
results in a dead end. But it's important to not give up, to keep asking
questions and to do what we can do to handle the condition on our own
in the meantime.
Awareness is the Key
To
complicate matters more, every case of Klippel-Trenaunay is unique.
Some K-T folks are impossible to miss while others blend easily into the
crowd. This is why awareness is so important. After all, if few doctors
even have a clue how to manage this condition, how can family, friends
or the public help? For starters, we can talk.
Tell your stories, tell your problems, fears, tell about the mistakes and about what works.
Learn
everything you can - learn how the human body works, read research
papers, connect with other K-T'ers and their families. Be the
pre-eminent lay person in your area on Klippel-Trenaunay. If you have a
child with K-T, don't let fear keep helpful information from him. He
already knows he has something going on with his body, help him to
positively deal with it rather than fear and feel like a freak.
Be an advocate
and if you have a child with K-T, give her the information she needs to
be her own advocate. Educate yourself. Educate your family. No matter
who you are in the K-T picture, having as much information as possible
will allow you to confidently speak up to doctors, ask questions and
seek the best treatment possible. It is infinitely better to be an
advocate than to be a victim. Speaking up can be the difference between
getting treatment and getting blown off. As little progress as we feel
we've made, it has been our perseverance and unwillingness to have
doctors shrug that has gotten us this far. There's a long way to go but
we have so much more now than we did at the initial diagnosis.
Take pride
in who you are. If you have K-T, don't hide, don't be ashamed. You are
magnificent, you are much more than 'just' a K-T'er. Don't let others'
reactions to your K-T stop you from being fully you. You are not a second-class citizen just because you are different!
If you have a child with K-T: Your child is amazing and trying to make
him 'normal' will only lead to frustration for you both. If he looks
unique, feels unique and/or has a unique way of living (medications,
medical treatments, wheelchair/cane) raise him to be proud of who he is
rather than having him compare himself with kids who don't have a
chronic condition. Truly living is so much more different than what we
often think it is. Help him to embrace life and truly live and enjoy it.
It's frightening to receive the diagnosis of
Klippel-Trenaunay. If you allow it, it's the opportunity to open so many
doors and to become a greater person than you ever thought you could
be. You can be more learned, open-minded, thoughtful, empowered. Don't
give up, don't stop seeking and never be ashamed.
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