… to the parents of kids with Port Wine Stains
… to the parents of kids with Klippel-Trenaunay Syndrome
Dear New Parents,
Welcome to my personal journal. I have been diagnosed with Klippel-Trenaunay Syndrome. My journey there has been unusual. You may be feeling a bit apprehensive right now. I’m encouraging you to take a deep breath and to trust that your journey will be easier. Many answers to your questions will come soon, others will likely have to wait until your baby is a little older. The good news is medical science today is so much further down the road than when I was a kid. Even better, social networks will provide you contacts with the world-wide K-T community. We are happy to walk the road with you.
Let’s begin. Was your beautiful child born with muted red and deep purple stains on their skin? If so, are you noticing patches of color that vary in size or maybe even extend from head to toe? When you asked the doctor, did he/she likely described these mosaics as Port Wine Stains, Vascular Birthmarks, Birthmarks or Hemangiomas? Did a doctor give you a preliminary or speculative diagnosis, say Klippel-Trenaunay Syndrome. In any case, I’m sure your level of curiosity is heightened because you don’t recall seeing other babies with similar anomalies. I understand your interest. If you stumble around my journal here, you’ll see that I have a deeply personal interest in helping parents of kids, as well as, kids with Klippel-Trenaunay Syndrome including those with Port Wine Stains, a common manifestation of this congenital aberration.
Born in 1954, I too had a similar mosaic of color. My parents were equally curious. In their day access to quality medical information about babies like us meant they were given wrong information about me. Sure, my parent’s experience could have been attributed to the relatively small number of kids with conditions like mine; and certainly the general practitioners of the day were generally taught little about these unusual conditions. Truth be told, a curious practitioner even in those days could have provided more complete information and could have been more forthcoming. The condition was first described by French physicians Maurice Klippel and Paul Trénaunay in 1900, a full 54 years before I was born.
“It was, what it was” to put a twist on a modern-day expression. As such, I banged through my childhood and early adulthood with little practical knowledge about my condition. I had a “birthmark”, to be exact, nothing more than a superficial stain on my skin. Wow ….. , did my life dramatically change just after my 18th birthday. What had been nothing more than nature’s artwork, turned out to be a stealthy cover for complex systemic aberrations. These hidden conditions had long been degrading multiple systems within my body. What had been explained as “just a birthmark” was anything but. The details of what I call my early-period revelations are being saved for future writings.
The point being made here is that you and your baby will not repeat this legacy as you have access to very informed doctors these days, albeit they’re only a handful, but they’re outstanding. Also, you will soon meet street-level friends in the World Community. It’s a virtual (in every sense of the word) neighborhood of soon-to-be friends. These are KT-kids, as well as their parents, who have lived through the days when little was known and therefore are motivated and happy to share the things they’ve learned along the way. You can find many wonderful resources listed here as various links to support groups and doctors. A resource created by myself with the contributions of many is made available at no charge to the Klippel-Trenaunay community – look for the copyrighted name K-T Connections.
Let me start simple. I will never use the word birthmark, in any form, to describe the painted legs that define me, nor any other person like me. That word more than anything in my life betrayed and misinformed not only this child but medical providers and, as it turned out, government institutions commissioned to care for me and kids like me. As for your baby, know one thing for certain, like every child he/she is unique. This is ever so true of us K-T kids. Some of us will be challenged early, yet others will be challenged more as we reach puberty and then adulthood. All of us will certainly be challenged sometime in our life and usually no later than midlife. Rest assured we’ll let you know when to worry. You’re raising a hero, which will become more and more apparent to you as the days go by. Your child will show you strength of character many people will never understand and evidence of that strength will be your baby passing off the difficult as routine.
As a kid I always marveled at my built-in and very visual thermometers. Muted red means warm enough for shorts. Deep purple means cold enough for long johns. I even enjoyed grossing out my buddies by closing down my varicose veins with one thumb while pushing the blood up my leg with the other. “Poof”, like magic, there they were back to normal gloriously filled with blood. Somewhere in this journal I have pictures of those veins – you’ll get the picture (so to speak) after seeing just how obvious these things are. If only then I had known I was giving my friends valuable biology lessons, I would have charged admission to the show. I respectfully submit that given a chance at a healthy and balanced childhood your baby will see themself as simply another kid in the neighborhood. Yes, of course there are things they will do and things they will adopt to better fit into the picture, but what kid doesn’t do that anyway.
A bit of personal reflection by this old man, a favorite band of mine as a young boy growing up was Deep Purple. I say this choice was seriously subliminal as my funny colored legs didn’t define me. Sure, there was the occasional adult who had to ask, or at least stare for what seemed endless minutes. And, there was the once-in-awhile bully who thought the retort “cherry legs” was the best putdown he could come up with. But, quite frankly, friends and strangers rarely took notice or cared to call me out on my funny looking legs. I kid you not – I was regularly ridiculed about my big ears and big smile. I was all teeth and ears. These more regular distractions left me little time to over worry about having painted legs.
I have attended numerous conferences on Klippel-Trenaunay Syndrome, Vascular Anomalies and Vascular Birthmarks. The one constant I have witnessed and heard is that K-T is a tricky syndrome. The crux of the problem is that it is a congenital syndrome that takes on common traits but with wide variability as to aberration within these traits. It impacts a wide variety of body systems. Doctors speculate these clinical realities are because the “triggers” for this congenital syndrome turn on at different times and for different durations. This behavior leads to the variability we see. Some of us have K-T caused aberrations in the lower extremities, some the upper, some the torso, some right through to the neck. K-T requires patience and thorough examinations. It is not a superficial condition and as such it often involves complications in our internal organs and muscular-skeletal systems. The common thread among us is our vascular abnormalities and a strong propensity to be marked by Port Wine Stains. In its most extreme condition K-T has caused hypertrophy, hemitrophy, and/or girth differentials in a number of my K-T friends.
I’m not a doctor. It bears stating that any counsel given here is based on my personal experiences. In short, my condition informs my counsel. Looking at my legs you see a condition that is bi-lateral from hip-to-toes with significant pelvic involvement. It is multiple-system involved, including peripheral and deep systems. These include venous, lymphatic and capillary malformations along with neurological complications. I have 50% presentation of a Port Wine Stain on both extremities. My deep vein is missing in the right extremity and grossly incompetent in the left extremity. I have severe varicose veins throughout both extremities and feet – these clinically have led to Chronic Venous Insufficiency. Additional complications for me now include chronic and degenerative peripheral neuropathy which shows in a small subset of patients. The sad news for me is that this latter complication was avoidable; it was accelerated by improperly prescribed compression stockings, which I wore from age 19 until 42.
My short-list advise is to stay clear of doctors whose primary experience is exclusively with Hemangiomas and Vascular Birthmarks. The medical expertise to deal with Klippel-Trenaunay Syndrome is most often not resident in these doctors. In no way am I discounting another group of special kids with these medical conditions and who need attention every bit as much as kids like us. Kids with Hemangiomas have very challenging lives as well and are benefiting from wonderful and relatively new technologies. Specialized lasers are resolving these vascular tumors. I’ve visited with several of these kids who have undergone laser treatments. Their before pictures and live results show absolutely miraculous results.
To be clear, the treatment of Klippel-Trenaunay Syndrome is commonly more intrusive than laser treatments. If you haven’t already, please seek out Vascular Surgeons and Intervention Radiologists who advertise Klippel-Trenaunay Syndrome in their Resume’. The K-T Connections directory may help you get started on this endeavor. As a general rule, avoid Dermatologists. I mean these talented folks no disrespect, but their expertise goes basically skin deep (humor intended) and you’ll learn soon enough that Klippel-Trenaunay is a deeply systemic, deeply complex, multi-system disorder.
All this said, an Army OB/GYN Doctor advised my Mom and Dad saying, “He’s a child, raise him like any other child.” And my parents did exactly that. We did not know my “birthmark” was anything special then and in a weird way this naivety provided us with a useful prescription. It allowed me to grow up with a healthy and normal ego. And to be honest, that foundation has been far more beneficial to me than any of the downsides to not knowing exactly what was wrong with me. Don’t get me wrong, it would have been better to know the gory details and still be raised as I was. But given the realities of the day, the healthy ego thing has really paid off.
Congratulations on parenthood. Feel free to ask away. Future articles here will be a best effort at documenting my history with K-T and some hints I’ve picked up along the way.
William Anton Lee
aka: Bill, Will, Willi, or anything else that gets my attention.