Mutant Meat meets Klippel and Trenaunay

I believe I've mentioned that our amazing life keeps us pretty wrapped up in daily activities, many of them having nothing to do with the internet. Living in a very Unenlightened area means that I appreciate my Cyber-Sanity Souls that much more but honestly, I'm extremely thankful that my Real Life is full and satisfying. Well, mostly satisfying. Three members of The Fam deal with congenital health issues that are managed much better alternatively than via the Allopathic Road. While some of the conditions we're dealing with (say...Celiac, for one) are the hot topic in print and on the web, I thought I'd give a little basic information on a lesser-known condition.

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 manic-depression. 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 aseptic 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.

You can help your chronically ill child thrive!

Bu was two when he had his first migraine; Jane has battled auto-immunity issues much of her life. Many parents feel helpless when they find out their children have chronic illnesses but really, there is no need to panic. If a loving parent is willing to adapt and adjust her/his lifestyle a bit, their kids can thrive. If you're already an attachment/peaceful-parenting, weirdo hippie like me, most of what I'm going to say may seem familiar, even if your kids don't have chronic illness.

I'm not going to share tips on how to help your kid deal with being made fun of, how to be Prom Queen or how to be Just Like The Other Kids because that's just not the world our family chooses to live in. I'm going to talk about how to help your kid thrive and become the best person he can be despite being ill. These are things that have worked for our family. Keep in mind that to the average American we're Crazy Hippie Freaks so these tips may not help you in the slightest.

Because it's heinously awkward to constantly write s/he or his/hers, I've tried to use 'him', 'her' and the like alternately. Hopefully it isn't awkward to read!

First Things First

It's not politically correct or often done these days in the medical field but the first thing both you and your child need to accept is that there are limits to what he can do. More often than not, it will harm him to “just push through it”. In my son's case, it could be life-threatening. Living with limits is generally much more preferable than not living at all.

Hopefully you already have an open and good relationship with your kid, it's imperative that you're open to listen to her when she talks about her health. Even better if he's in touch with his symptoms and pains. If she can tell you when the pain is different or he needs to rest. It's that teamwork that will help you and your child thrive. Help him to develop a good “pain vocabularly” or to express exactly what he feels he can do at the time. You can aid her by showing how to break down big tasks into easily manageable ones on days that the pain or fatigue are overwhelming.

As a parent, there often is a terrible sense of guilt and sometimes feels there's more stress than you can handle. Often that's due to the fact that you're trying to do too much. Change your expectations and be willing to alter your life a bit. Remember, your job as a parent is to show your kid the ropes. She will learn by watching you more than she will ever retain all that you say. So show her how life is done. Most kids are bright enough to figure out that too many adults run themselves ragged simply trying to impress someone else. Lighten up and have fun. Tomorrow isn't guaranteed for anyone. Cherish what you have this minute, this day, particularly if you have ill family!


Eat to Live

Regardless of the condition, make sure you're giving her the healthiest food possible. It can be hard to say no to junk food and non-food, but truly, you're not doing her any favours by giving these foods. When your schedule is hectic it may be tempting to get some fast food, don't give in. If your kid is already on any type of medication, her liver and kidneys are already doing plenty trying to process unnatural ingredients. They don't need to have more to do and since most fast food is made up of things our bodies don't recognise as “food”. They simply have a hard time processing it.

Most chronic conditions can be aided by the regular use of organic, REAL foods. Both our kids (17 ½ and 16 by Western age reckoning) are celiac and Bu experiences migraine flares from additional foods. As much as they might like the taste of something, both know that the pay-off isn't worth it. Since I was already a Real Food junkie and had raised the kids the same as much as it was possible on my end, it wasn't that difficult to convince the kids that there are just as many yummy things they could eat that wouldn't harm them. It took a little longer to convince Meat: Giver of Bad Foods, though!

Definitely though, axe the soda pop, candy, flourescent salty snacks and fast food. Try it for a month and see if you and your kid don't feel better. Have fun browsing the local farmer's markets and growing your own fruits and veggies. Try making different ethnic foods since most other cultures have much healthier meals than what's considered “food” in the States.


Thrive on Real Life

If your child is in public/private school, understand that if he says he doesn't want to go or he feels he can't make it, that is a very real feeling for him. It doesn't necessarily mean he's trying to scam. It's often too much to ask an ill child to take on that environment. If it was simply about learning, that might be one thing, but school entails so much more than that. Don't forget, for thousands of years kids did not go to school and somehow they were able to survive and interact in social settings.

Open your mind to homeschooling/unschooling. It's an outright lie that putting any kid, let alone a chronically ill one, in school will help them feel “normal” and “just like other kids”. School was not created, nor is it designed to be a leveling ground for kids. It exists to get children used to the idea of consuming and following the crowd. It doesn't teach them to think, to get along, to be kind or thoughtful. Meat, who spent his childhood on ritalin and singled out as a hyper-active kid, can attest to exactly how little help a chronically ill child is offered in the school system (really, just ask him). Do NOT trust the teacher or the school administration when they tell you that everything is wonderful and the environment is groovy. These people are paid per child enrolled. Just like anyone working on comission, their job is to bring more heads in so they get more money. They don't want you to teach your child yourself, it's counter-productive to their mission.

Homeschooling or unschooling allows kids – who, by the way are already humans, not miniature people or sub-standard humans – to learn at their own pace and ability. It allows for those days when illness would otherwise prohibit education. Because it IS educational to read a book, watch tv or research things on the computer, even though they seem fun. If you have a Learning Mentality, you will learn from any media – if you're a curious person rather than the type who are happy following the crowd. Bu has repeatedly commented that he loves unschooling because every day he's learning something new, even the days when his migraines are killer. Additionally, he's never had the hurt of being left out of school activities due to illness. He creates his own fun and others join in. He and Jane don't live in a world where their worth is determined by their sporting abilities or social status. They've made friends and talked to people as equals, whether the friends are their age, 40 or 60.

Unschooling/homeschooling the chronically ill child helps him/her to learn limits. This is a vital skill for the future as most chronically ill kids will carry over some of that illness to adult life. Bu is very aware of his limitations and is realistic about what types of jobs he could reasonably take on. He knows that it's not a weakness to let others know about his condition and to be honest about what he can and cannot do.

Some parents have expressed concern that their kids wouldn't have the motivation to learn or take the initiative. I really can't speak for any family other than my own but it's never, EVER been an issue for us. Maybe because life has always been an adventure and a learning experience for me. Perhaps that has bubbled over into my kids' lives. Life is about learning and trying and that was their childhood. If you personally take no delight in learning or think that it is only something that can be doled out by Learned Individuals via Educational Texts between certain hours on certain days of the week during certain calendar months, then you may not be cut out for homeschooling. However, if you're willing to take belly dancing because you think it looks like fun (not to impress your husband/friends), you have your TV or radio tuned to a foreign-language channel and jot down words to look up, enjoy making new recipes or exploring the artsy part of town, then your chronically ill child will likely thrive unschooling or homeschooling.


It Works For Us

While we're still dealing with health issues, life is more bearable for the kids. Not because the kids are sitting on their butts eating candy and playing video games all day. Bu and Jane are fun people to be around - their open minds and insatiable interest in any subject allow them to carry their own in conversations on a variety of topics no matter who they are speaking with. They have abundant self-discipline and wake up early because they're looking forward to what each day offers. They are also aware of how to listen to their Body Signals, when to rest, when to eat, when to get a little extra done. They are well-rounded humans who know their limitations. I look at them every day and see that they are truly thriving. They are happy. They are truly living life. They know what is important and what not to waste their energy on. That's more than enough for me.