When you read the professional literature on Klippel-Trenaunay Syndrome (K-T), regardless of who publishes it, the content will likely confuse you, scare you and in some cases have you in tears. Stop for a minute here and read my take. It’s an unconventional approach, for no other reason than this syndrome, and many of the people who have suffered through it, deserved better than the prolifically poor information passed off as subject matter expertise. You are likely a new parent trying to understand what your child’s needs and challenges might be and you deserve better. Take note: I’m not saying the professional literature is wrong; that’s above my pay grade, as the saying goes. I am saying that it is way too clinical – way too focused on the worse-case scenarios because that’s what the doctors have seen – way too incomplete.
Muted Reds & Deep Purples, or Not
First, when you look at your child what do you see? Does he/she have odd-looking reddish to purplish stains on their body? Are these stains a single splotch of color? Does it extend from hip to toe on a leg? Does it reside on an arm or neck or back or buttocks? Does the affected area look like it has veins that bubble up like grandma’s? Does the affected area look like it’s bigger or bulkier? Does the affected area look like it might not have developed normally as in maybe a missing toe? Are there no stains, but one of the other things I said is a reasonable description of your son’s/daughter’s condition? Quite bluntly, each and every one of these conditions is a plausible picture of Klippel-Trenaunay Syndrome. It’s a very tricky and complex disorder.
Within this brief article, there is no practical way to show the wide-ranging conditions of K-T using pictures. The variability within the K-T community is huge. On the flip side showing a limited number of pictures might give folks the false sense that this is all there is. Were the public to draw this conclusion, it would be unfair to those K-T kids with conditions that are more remarkable than those presented. All things considered, I decided to place a few pictures here because the abstract view of K-T is far more confusing than the limited view. In time I hope to show a more complete album, although this goal is subject to help from the K-T support groups I participate in. I’m hoping K-T friends will agree to have their story in pictures published alongside mine.
How does this happen? How can K-T look so different, yet be the same syndrome? In short, K-T screws up the construction of our normal plumbing during pregnancy. This feature of the syndrome is the one constant we all share and, interestingly, it is this common thread that is the basis for the wide variety in our topologies, our conditions. Apparently, the unknown mechanism that causes K-T has a nasty habit of turning on at different times and for different durations during pregnancy. The earlier and the longer the K-T mechanism is active the more screwed up our plumbing gets. And the more screwed up or plumbing gets the more screwed up our muscular-skeletal systems and internal organs get.
Klippel-Trenaunay is unpredictable. During gestation a baby develops multiple layers of body systems in concert. A co-dependence exists between our plumbing and the skin cells, nerve cells, muscle cells, and so on. Each of these multi-level systems relies heavily on the availability of healthy and regular capillary, vascular and lymphatic systems. If these fail to deliver timely and balanced levels of nutrients, then co-related body systems tend to follow suit. It’s a delicate balance . With an over-abundance of blood delivered, an over-development of the targeted muscular-skeletal structures and internal organs often occurs. In these latter cases, doctors use the terms hypertrophy or hemitrophy to respectively describe issues of overgrowth in limbs and the torso. Conversely, under-development or no-development occurs when the opposite conditions exist. There are, and will be dramatic cases where limbs and appendages don’t regularly develop.
What’s a Person to Do
To the new parent it has to be difficult to know what to do, or what to think. If the port wine stain is the most visible sign, you imagine your baby having one set of challenges. If your child is born with hypertrophy, you may imagine a whole different set of challenges. These are proper feelings. I wish I had permission to write and publish each of the many K-T stories I have been told over these latter years. The most challenged of us Kt kids leads a life inspired. They go to school, have a circle of friends, go to senior proms, write books, teach school, open businesses, have kids, and in many cases accomplish things kids without K-T never aspire to. Don’t get me wrong sometimes the path-walked is scary, other times it’s easy. What makes the going good are the parents and family that walk the road with us.
The one constant for all of us is the “waiting game.” For most of us imaging technologies are not, as of yet, able to map the aberrant vascular structures until we’re young children. Babies veins are too small for invasive procedures and other less invasive imaging equipment is not discreet enough to show intricate malformations. This means that at times children are growing irregularly after birth and the underlying mechanisms that are feeding or starving this maturation remain unknown. For others, the body remarkably compensates for aberrations and these KTS kids will tell you that they find little distinction between their life and their siblings or neighbors.
Patience and diligence are key!