PWS/KTS - Infant - What to Expect your first visit
My wife and I received some great help and support from the community here 5 months ago when our daughter was born. We’ve been watching the forums, but haven’t contributed much over the past months as we’re really at the beginning of this process. Well, we had our first visit with a specialist, and thought that a rundown of our experience might help others just starting to explore PWS’s and KTS.
Any comments, or corrections to info are appreciated. We were surprised that the specialist was pushing laser treatment out by years (she even mentioned 10 years at one point). We hope that she is accurate.
Backstory: Our daughter was born 5 months ago, perfectly healthy, large port-wine stain covering her outer right leg, bit of back, and some inside leg. Contradictory information from pediatrician so we booked a specialist appointment. Took till now to meet with the specialist.
Disclaimer: This is a second hand account by memory, and not direct quotes. We are by no means speaking for our specialist.
- the normal meet and greet with backstory. Why we booked, what we’re looking for.
- the doctor physically examined our child. This was a careful examination where she was looking both at the breadth and depth(color, signs of abnormalities deeper in the skin) of the stain.
- She then looked for outward signs of abnormalities (KTS). We could see her checking leg length and circumference from the front and back. She didn’t use any measuring instruments. It was just by eye.
- The doctor then briefly reviewed the case. Definitely a PWS, no outward signs of KTS, signs could show up at any time into teens (generally the earlier the more serious) so we should book yearly appts with her (6 months for the first one).
- a nurse came in to take informal pictures with a digicam for future records.
- She then started to talk about options, which prompted a lot of QandA.
Q and A Session
1) Imaging: She didn’t recommend imaging at this point. Why? No outward signs, imaging at this point involves sedation for the child which is more serious. As there isn’t really anything preventative that can be done, imaging can wait until symptoms or our child is older.
2) KTS Prevalence Question: We’ve been told KTS is very rare, but then so too is this kind of PWS. How worried should we be? [there was some hesitation as she thought]
Answer: I see a couple children each month with a PWS like this, but only a handful have developed KTS. [This was probably the most unsatisfying aspect of my visit. I was surprised a hospital couldn’t say we’ve seen x patients with this symptom and y% developed KTS. What does a couple and a handful mean? This could be anywhere from 1% to 20%? With everything else in our thoughts, we didn’t press on]
3) Laser Treatment: At this young age, it would involve general anaesthesia as it is painful. [at any rate, as we found, where we live there’s a 2-year waiting period anyways]. Treatment for leg pws are not always as effective. Of course, we could pursue it, but her opinion is there’s no advantage in outcome vs. waiting until the child can decide for herself. [With a two-year wait period, we went ahead and booked a consult anyways. We hope her assessment is accurate, we could look for a private laser clinic, but it sounds like waiting till our daughter is 2-1/2 won’t affect outcome. Fingers crossed]
Q: I’ve read that the earlier the treatment, the better the results, down to 6 months. Should we push for treatment [I had read a study to this effect]? A: There have been some studies demonstrating that, and some not. She was chatting about this with an expert from NY and the consensus seems to be that there really is no conclusive evidence that early intervention helps (whether 6months or 6 years).
Q: I’ve heard general anaesthesia in childhood can have harmful effects [ I read a study]. A: Yes, there have been studies, but they mostly looked at older drugs. No evidence that newer drugs do [or don’t].
Q: Will laser treatment have any effect on KTS A: None whatsoever. The problems caused by KTS are deeper in the skin.
4) Misc Q: Anything else we can do? A: Not really, just monitor visually as she develops, make yearly appointments, book something sooner if you see a visual indication of KTS.
Q: What changes can we expect to the PWS over time. We’ve heard that if untreated or untreatable the colour and texture of the area can change. A: Yes, it can. It will probably get darker, but beyond that it can follow a different course for different people. Some of the more advanced cases though, you’re looking decades out, not in the next few years.