Siblings & Classmates

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We have three children: Addy is 7, Clarence is 5, and Eloise is 3.  The younger siblings’ awareness of and reactions to Addy’s port wine stain are amusing: a combination of childhood oblivion and concerned observation.

When Clarence was younger, he’d point to Addy’s baby pictures: “That me!”  “No, sweetie, that’s Adelaide.”  “NO! That ME!”  We’d point to the rather obvious port wine stain plastered on the baby’s face: “Clarence, look at that stain – you don’t have one of those, only Addy does.”  He’d stare at it for a minute, think about it, then firmly shake his head.  “No, that me.”  Okay, dude.

Eloise did the exact same thing when she saw family pictures.  It wasn’t until they were about 3 that either one showed any signs of recognizing the port wine stain’s existence in the picture and connecting it to their sister.

Two or three days after her latest surgery, which left her cheek quite bruised, we were sitting around the dinner table when Elly (age 3) suddenly stopped eating, stared at Addy for a long moment,  pointed to her cheek and asked, “Addy, what that?”  Yes, it took three years of life and three days of bruising for the youngest sibling to notice anything out of the ordinary.

When Clarence was three, he saw Addy’s bruised face after a particularly intense laser treatment and became quite concerned: “Addy! That blood!”  She laughed it off and shrugged, “No, that’s just my port wine stain.”  He stared.  “No, Addy, that blood!”  It took some work to convince him that his sister was fine, and that such bruising had, in fact, happened regularly in his first three years of life.  He’d just never noticed it before.

Clarence recently told me, “I want to have a port wine stain, mom.”  “Really?  Why is that?”  “Because port wine stains are good.”  Awww!  My mommy heart swelled with pride knowing that clearly, I’ve done such a stupendous job parenting tha–   “And because Addy gets toys like her new Olaf toy and her new Olaf blanket when she goes to the hospital, and I want an Olaf blanket.”

Priorities, right?  He just wants the goodies.  I can’t blame him; that new Olaf blanket is pretty sweet, as you can see in the picture.  (By the way, on that thought: A million thanks to the volunteers and donors who keep Children’s Hospital stocked with the awesome toys that comfort & distract kids during medical procedures; it’s stuff like that that keeps our Addy looking forward to her hospital trips!)

As far as classmates go, whenever Addy has a surgery, we prepare her for the fact that, because she’s going to school with a newly dark-purple face, people will probably notice it and ask her about it and that’s totally okay, because it means they’re concerned, and that’s awesome.

But recently, no one has asked.  And I realized something – it’s old news already.  She’s at a small school; all the K-12 class sizes are 10-20 kids each, she knows upperclassmen by name, and they know her.  In other words, pretty much everyone in that building has seen her bruising before.  She walks in with a purple face?  Nothing new.  She gets more double-takes when she leaves Children’s hospital, which makes sense, because those strangers have never seen her before.  (In fact, one little girl in the lobby gasped excitedly, “Mom! Look! That girl has a painted face!” like it was the coolest thing she’d ever seen.  I love kids.)

While familiarity may sometimes breed contempt, it can also breed boredom; in our case, that’s a very good thing.  As long as Addy is small, surrounding her with the same people regularly for whom her suddenly-purple face is ‘nothing new’ minimizes the stares and questions she gets throughout her day.  I will always be happy to expose her to the world, to empower her to answer strangers’ questions with grace, and to build her confidence in facing society with a unique face, but I’m also relieved that in her daily routine, she can relax among a few friends and family who know her face so well that there’s nothing left to ask.

Unless she comes home with a sweet new Olaf blanket and stuffed toy.  Apparently, that’s enough to pique brother’s curiosity all over again.

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Posted on March 5, 2015, in 3. Addy Stories & Experiences and tagged , , . Bookmark the permalink. 8 Comments.

  1. Hi! Thank you very much for writing this blog. My son is 3 months old and was born with very similar markings as you daughter. So happy to read your experiences and I must say your little girl is very charming. I wouldn’t worry about her for a second (easier said than done), she has a lovely personality, is beautiful and seems very secure with herself. You’re a wonderful mother and it shows. We plan to start treatments at a year, hopefully not too many, as it has faded quite a lot since birth. From what I’ve read, that’s not usually what happens with PWS, so I was surprised when I looked back at old pictures and realized the difference. If you’re interested to see, I can send some photos you’re way as you have been so kind to share with us.

    Thanks again!
    Sarah

    • Sarah – Thank you so much for your comments! I love to hear from other mothers. 🙂 I (finally!) have an email address at whathappenedtoherface@gmail.com, so you can send photos my way. (Full disclosure that any photos sent there may be published in later posts, unless you clearly tell me not to in the message.) I’d love to see pictures of your son!

      One thing we’ve noticed in raising our three children is the “oddness” of growth: it seems that when one part of the child is growing, another part of them takes a break. For example, during a ‘growth spurt’ week, Addy would eat three times her normal amount and shoot up an inch, but over the course of the week would seem to be dippy & ditzy, and to have generally regressed about 6 months mentally. (Two steps forward, one step back!) Then, when her body had accomplished the task, her brain would go through a growth spurt, widening her cognitive skills and awareness in leaps and bounds while her size remained unchanged. Even within physical growth, we noticed that kids don’t grow evenly; sometimes they widen first, then they shoot up in height. Or sometimes their legs grow so fast that their gross motor skills can’t keep up, and the kid with great balance finds himself stumbling every time he runs.

      You may be observing something similar with your son – you’re correct that if it’s a Port Wine Stain, it won’t fade on its own, but you may be seeing growth elsewhere first. Don’t be surprised if it suddenly darkens. 🙂 That’s our “growth spurt” indicator with Addy, along with her sudden uptick in ravenous hunger. It may also be a more “shallow” stain. The depth and intensity of a port wine stain depends on when the error occurred in utero; if the error occurred early, then as the child develops & grows, the stain also develops with the face, leading to a stain that is deeper and more extensive. Addy’s stain is deep! Many kids are able to zap off their port wine stains easily, but Addy’s keeps growing back. (It’s also in her mouth, tongue, throat… more on that in a later post.)

      I always advise parents to start zapping as soon as possible; the stain will only grow with time, even if the growth is happening ‘behind the scenes’ and isn’t as visible, making the stain harder to treat (requiring more treatments overall) later. If you can start weed-whacking while the blood vessels themselves are still young, before they get older and tougher and more established, treatment will be easier and you’ll probably need fewer overall. (Addy’s first treatment was at 5 weeks old.) Also, and this is just a mom’s observation here, I think it’s easier to do treatments with a baby than a toddler. They’ll get put under anesthesia, which requires fasting ahead of time, and that’s a lot more complicated when they’re argumentative, tantrumy toddlers. 🙂 But as with anything, follow your gut; you’re his mother, and you know him (and his face) best. If you choose to treat early, I can help you prepare for treatments with baby; if you choose to treat later, I can help you prepare for treatments with toddler. 🙂 Been there, done that, either way!

      I look forward to hearing more about your son; thanks again for stopping by!

      • Thank you so much for your thoughtful reply, I can’t even tell you how helpful your story has been for me. When I realized my son had a birthmark (not a bruise as the nurses told me), I wasn’t that worried. My older son had some developmental delays (language and motor) and I have spent the past 2 years doing everything I could to help him reach his potential. He has quite literally defied the odds and is doing extremely well. He has basically caught up with his peers and is surpassing them in many ways. Parenting him has flipped my world on its head and I have to say I have a very different perspective on life after having him. Enter baby number two with a huge PWS on his face. I thought to myself, “well, I’m sure there are lasers to treat this, and if not, we will prepare him for the world in the best way we know how.” I was really impressed with myself, I loved him so intensely that no birthmark would impede my joy… until, that is, I googled PWS. Seizures, learning disabilities and mental retardation crushed my joy and caused a full on panic attack. I couldn’t believe it, I had never heard of a birthmark causing such health problems. I was devastated. Now after everything we had been through with our first son (and trust me it was a lot) I had to worry about a “stain on the brain.” Luckily the children’s hospital got us an appointment within 2 weeks of birth and told me that he wasn’t high risk for SWS and that lasers can be very effective. I live in Canada and everything is covered by our healthcare system (I never even see a bill), and our children’s hospital has it’s own vascular birthmark clinic. My son is developing beautifully and that helps my anxiety immensely. We will do an MRI when he turns one to rule out anything on his brain, will continue monitoring him for glaucoma and will begin laser treatments at a year, as well. I wish we could start earlier but because of the risk of anesthesia, the doctors will not perform treatments until this age. The hardest part is waiting for the MRI and trying not to let my fears consume me.

        I’m interested to hear about Addy’s oral PWS, my son has a little bit on the white of his eye, but the doctors have said that too is only cosmetic, at this point.

      • Ohmygoodness!!! You have to wait a year for peace of mind?? AGH! I would go crazy. But man, that’s awesome that your hospital has its own vascular birthmark clinic. You’ve been better about googling the problem than I was — I was blissfully naiive. 🙂 I was such an overwhelmed new mom (Addy was my first) that the port wine stain, honestly, wasn’t a high priority for me. I was vaguely aware of it and knew we should probably do something about it… (isn’t postpartum daze great?), but luckily my mom was on the ball. She’s an M.D., and as soon as Addy was born she went into ‘research & diagnosis’ mode, and helped us track down Dr. Zelickson, a dermatologist who specially treats port wine stain kids. He started treating Addy at about 5 weeks old, which we’re thankful for, because Addy’s stain is so deep and stubborn. I saw your handsome son’s pictures! Yes, his stain is definitely lighter than Addy’s was. Even as an infant, her port wine stain was taking on a “3D” quality. I suspect that waiting a few extra months won’t be the end of the world for him. 🙂 Especially if you’re burned out from seeing specialists, with all the activity your first son has required!

        When Addy was born, our nurses told us, “Oh, that’s just a hemangioma, that’ll go away.” It was only the pediatrician doing her rounds who confirmed my mom’s guess of what Addy’s mark was, and confirmed the pressing need to go see a specialist ASAP. Also luckily, they didn’t tell me too many details of Sturge-Weber… I heard the term, “calcifications in the brain”, but had no idea the extent to which a port wine stain could totally mess with a brain. So I was blissfully unaware. Addy got her MRI when she was about 4 months old, at her 4th surgery. Only after she got the all-clear did anyone explain to us exactly what a PWS could do. Sheesh.

        In her first year or two, we did surgeries every month. Then we slowed down to every other month, then every 3 months. When school started and schedules got complicated, we missed a few, but we try to keep to the once-every-three-months schedule. Her stain is so deep and stubborn that we can see it darken as she grows (“growth spurt!”).

        We’re in the U.S., so we have to deal with insurance at every surgery. The treatments are covered and therefore mostly paid by the insurance company, though we have to pay a portion after the fact. Minnesota (where Addy’s Children’s Hospital is located) requires insurance companies to cover PWS treatments, so that simplifies things.

        Addy’s mouth definitely has the port wine stain. When she opens her mouth, you can practically see a line down the middle of the tongue, with dark red on the right and light red on the left. The roof of her mouth is half-and-half also, as is the uvula dangling in the back of the throat. Her teeth came in on the right side first! It was funny, this little teething baby with 3 teeth on the right side (early!), with nothing yet on the left. Amazing what some extra blood flow can do… A few other things we’ve noticed that may or may not be related: as a toddler, she had some difficulty articulating words (we almost got her a speech therapist), though this has since improved; she occasionally gets sensitive to the taste and texture of foods in surprising ways; her teeth are more ‘assertive’ on the right side, so they’re more advanced (the top row is lower, as if fully grown out), and her smile and speech are a bit skewed to the left, as if it’s easier to talk there.

        Addy’s right eye reddens more easily than her left, though she doesn’t have glaucoma. We have to get her checked for glaucoma every year, but so far, she’s in the clear!

      • Yes waiting a year to know if he has lesions on his brain is extremely hard, especially since I suffer from anxiety as it is. It was so bad I considered telling the doctors I was worried my son had a seizure in order to force an MRI. I can’t do that though, it feels like bad karma. However worrying myself sick also takes it’s toll.

        I find PWS extremely interesting, I can’t get enough information on them so thank you for your thoughts on growth/changes and treatments. I find it strange that doctors in the US and Canada have a different timeline regarding laser therapy. I have read some places in Canada won’t touch them until 6 years of age, when research shows the younger the better. Part of me feels like we are missing an important window in waiting until he’s a year (or more), but another part of me is worried about the risk of putting him under at such a young age.

        I personally don’t see his birthmark as a bad thing appearance wise, now that I’m used to it, it’s just him, and he is pretty much the best thing in the entire world. The only comment that has bothered me is from my Mom. My sister said she thought his PWS made him look like a bad ass and my mom could only say, ‘No, it really needs to go.” While I agree, we need to treat it for social and medical reasons, I don’t like that she is so insistent that it should be removed. It’s him and he’s wonderful.

        Can I ask what you meant when you said her PWS had a “3d quality?” Was it raised? Our doctor told us that the darker red the stain, usually the better the results because the laser is attracted to red. I thought that was interesting. I guess some are just more resilient but Addy’s has lightened a lot when compared to her baby photo. Have you used other lasers or just the pulse dye? I heard the gemini laser can treat stains that are stubborn.

      • Hi there! We’ve only used pulse dye lasers, which you’re correct are ‘attracted’ to the red. (For this reason we don’t zap the PWS on her lip – the laser beam can’t distinguish between legitimate lip color and PWS vessels!) When Addy was a baby her PWS was so thick that it almost felt raised (but wasn’t quite yet), and was so dark that it looked like a mask, hence the 3D appearance. And yes, the greatest strides in lightening the stain occurred when it was super-red; now that it’s lighter, the progress after each treatment is still there, but isn’t as dramatic.

        As far as treatment timing: It’s possible that some doctors won’t touch them until they’re older because they don’t use anesthesia at all. I’ve heard of some cases when PWS patients are lasered without any anesthesia — that’s always the story I hear when someone tells me of the treatment they tried once 30 years ago and never tried again. (It’s never a good experience! Apparently it feels like a rubber band snapping against the skin over and over quickly. Many tears!) But some doctors might do it still. Our doctor insists on anesthesia with every child, because he’s firing powerful lasers at squirmy childrens’ faces; definitely a good reason to sedate!

        And yes, research shows that hitting the PWS earlier is better. But some doctors are nervous about the effects of anesthesia on young children; in fact, there’s been research to suggest that children who go under anesthesia multiple times before Age 2 are at higher risk for ADD — however, there’s no accounting for the fact that most children who go under anesthesia multiple times before Age 2 do so because they have significant health issues that are likely to interfere with development; problems like port wine stains are the exception. (Addy doesn’t seem to be affected at all, but I’m her mother, so I might not be an objective judge of her sheer brilliance and awesomeness, though her teachers say she’s doing great.) And, of course, whoever’s paying gets to weigh in on treatment. 😉 The doctors who developed early guidelines on treating port wine stains with lasers, being the first, grabbed an arbitrary, “Well, this might be good if done every three months” as a starting point. Over the years they found that more frequent treatments early were more effective, which is why they offer monthly treatments; however, because the 3 month number what was set & published, our insurance companies can point to that “once every three months” guideline, and occasionally won’t pay if the next treatment comes within 90 days of the last one. You can get pre-approval with a doctor’s note, but we’ve settled into the once-every-three-months routine (school! activities!), so we didn’t bother trying. I know you don’t have to deal with insurance companies, but there might be some medical guidelines based on older research governing treatment options that are helping determine available treatments in some places. But since you have a vascular center right where you are, I’d trust that you’re in good hands!

        Man, it would frustrating to meet with such insistence from grandma! She probably has his best interest at heart, and is afraid of the social stigma he’ll encounter… I’ve noticed that grandparents, having gone through the cycle of raising kids, and having observed the cruelty of older children later in that cycle (in older grades), tend to be loudly insistent that their kids hustle to zap off grandbaby’s stain. They’re afraid that the new parents, naiive of the painful years far ahead in junior high, will be so in love with their baby and the stain that they won’t treat. Complicating that sentiment, I think that most people aren’t naturally able to say, “That’s a lovely stain! Zap it off!” They think that you must either love it & leave it, or hate it & zap it. So when your mom hears any positive remarks about the stain, her knee-jerk reaction is to voice the “zap it off!” choice. When Addy was a baby, even my parents got a look of nervousness on their face whenever I said anything positive about the stain; over time, however, they realized that we did, in fact, intend to zap her and that they didn’t need to convince us over and over again. 🙂 One can see the beauty in the error while still trying to fix the error.

  2. Thank you so much for your comment! (Sorry for the delay in responding here, we were traveling on vacation.) I’m so glad to hear Sylvie’s stain has been responding well to treatment! Addy’s has also been hardest to treat around her midline,right above her lip. (But hey, if she ends up with a half-dark-red lip, then she’s halfway to gorgeously dramatic dark-red lips, right?)

    Ugh, drugs can be such a pain! We liked Versed when it worked for us, until it didn’t; as you’ve probably read here, the memory loss that comes with Versed was rough on our girl. You mention the taste – in fact, Addy complains about the disgusting taste of Versed, and this is our daughter who will eat practically anything cheerfully (including kids’ cold meds), so when she says a medicine is “yucky!” we know it must be gagworthy! That stuff must be truly disgusting. Addy has gagged it down without any flavorings, but the nurse gave her a wet washcloth to wipe down her tongue (since, of course, she can’t eat or drink anything that might chase away the bad taste before going under anesthesia).

    About the Versed (and anything else that seems to be iffy) — This is where your “mom instinct” comes in handy: honestly, nurses will sometimes recommend whatever will make “the procedure” easier, not “your life” easier; in all our medical experience, we’ve encountered nurses who do a quick & dirty job of administering shots to Addy because the whole “shot” experience is shorter, never minding that it takes us two months to unravel her newfound horror of needles. So, keep in mind that the nurses might be recommending Versed for the nurses’ sake, and not for Sylvie’s, or yours.

    Also remember that kids change rapidly; every month brings new brain growth & emotional development, so the kid you’re putting under surgery now is quite different from the kid you put under surgery 3 months ago. If the Versed was convenient in 2014, it might be pointless in 2015, and perhaps not even worth the annoyance. If she hates the taste so much, you can score huge mom points by giving her what she wants – no Versed – and trying a procedure without it to see how she does.

    If Sylvie truly freaks out at being separated from you for a surgery, there are a few things you can do. One is to ask if you can go back there with her; it might not be possible at your hospital, but it’s worth a shot; I’m able to walk back into the O.R. with Addy, so I’m standing by her side when they put the mask on her face and hit her with gases. Then they walk me back to the recovery room. If you can accompany her, she might be calm. (But it will depend on their sterile protocols, etc.) Anyway.

    Another option is to spend the few months before surgery gently preparing her for the procedure, knowing that you won’t want her having the icky Versed, so she’ll be awake & aware when they take her away. She probably knows the routine now, so you can totally work with that, and put a positive spin on it all. Grab an anesthesia mask and play with it at home; take turns putting it on your faces like it’s the coolest thing in the world, and ask her: “Does the doctor put this on you? I wish I could see that! How special!”. Talk about how awesome it is to get to ride on a hospital bed (if that’s how they ‘take her away’ from you for the procedure), and sound bummed when you confess that you don’t get to ride on one, because you’re not a patient. Then have someone special (dad? grandma?) “take Sylvie away for *something special*” like ice cream; pay attention to the language — “take Sylvie away” and “something special” go together. If your daughter is *attached* to you (as my youngest can be), this will be a gentle way to introduce the idea that a trustworthy person can take her away from your arms and have a happy ending. It’s special, it’s exciting, it’s an adventure, it’s short, and she ends up back with you soon.

    Funny enough, the next post I’m prepping is about how to get a kid comfortable around medical stuff; I’ll keep Sylvie in mind as I edit it to see if there’s anything else that pops into my mind to add. 🙂

  3. I love your posts. It is so nice to read about another mommy’s experiences with a daughter with a port-wine stain. Sylvie has another laser treatment in a few weeks. We have been very fortunate because Sylvie’s port-wine stain isn’t extensive (as far as port-wine stains go..) and it has been very responsive to the laser treatments. The area above her lip has been the least reapnsove, but our Dr warned us that the lip area tends to be harder to lighten. We are now doing the treatments every 6 months, rather than every 3. Our biggest obstacle is the versed/midazolam premed that the nurses like is to give Sylvie, so she is more relaxed when they take her away from us. It apparently tastes AWFUL and Sylvie ends up gagging and spitting and hysterical. So, we face the dilemma of making her take this awful medicine so she isn’t as afraid and anxious when they wheel her away from us OR we don’t give it and risk our (quite attached) 3 year old a hysterical mess with multiple nurses holding her down when they take her away. I remember in a previous post, you talked about Addy’s experience with the versed, but it seemed like the problem was more the way she woke up, than actually getting it in her. Do they flavor it for her? Have you experienced any alternative ways to administer it? Any advice is appreciated! I would love to share pics of Sylvie with you, but not sure gow to post those on your blog. I have showed Sylvie Addy’s pictures and she loves seeing another little girl with a port-wine stain 😍
    Felicia

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