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The Best of Times, and the Worst of Times…

(That’s Addy trying to play it cool while telling me what she’s looking forward to at this surgery.  Before I pulled out my phone, she had been talking a million miles an hour, almost bouncing off the bed with enthusiasm.)

Addy had another laser surgery in January. She had looked forward to it for MONTHS. We try to do them every three months now that she’s older, but occasionally we have to push it back an extra month for scheduling conflicts, like this round. So with 4 months between surgeries here, she had an extra month to get excited about going into Children’s hospital.

“Mom! When do we go to the hospital?”
“Another 6 weeks, sweetie.”
“But I thought we were going sooooooner!”

“Mom! We go see Doctor Zelickson THIS MONTH!”

“Only 3 weeks until we go to Children’s!”

“I get to go see him in 2 weeks!”

“MOM! Only 1 week left!”

“GUESS WHAT!! Only 3 days until I see Doctor Zelickson!”

We came this close to making a paper chain like we do for countdowns to vacations.

This kid loves going to Children’s hospital for her laser surgeries. Her love of all things medical is the result of both some intentional parenting during her early procedures and the awesome folks at Children’s. (I’ll post more details about the parenting side later, in another post.)

Addy underwent her first laser surgery at 5 weeks old, and she’s had over 30 so far. She knows the faces of Doctor Zelickson, the nurses, the anesthesiologists, and the Child Life Specialists, and they all know her. She knows the routine by heart and looks forward to it each time.

Here’s the routine: We get to the hospital early in the morning and check in, where she chats up the receptionist. We wait in the waiting room for a few minutes until they call her name, then we follow them back to the pre-op ward. We stop in the hallway to check her weight and get her some hospital jammies. We settle into the pre-op room, she changes clothes and gets comfy on the hospital bed, and then the nurse starts the Q&A session (“Any loose teeth? Recent colds?”) while checking Addy’s blood pressure, etc. As an hour or so passes, we see a parade of people: the pre-op nurse, the pre-op nursing assistant, the Child Life Specialist (Geri, of whom you’ll see more later here), the nurse anesthetist, the anesthesiologist, and, of course, Dr. Zelickson. Everyone verifies what we’re doing (“laser surgery”) and where (“to the right side of the face”). Somewhere in that hour, either the nurse or Geri hooks Addy up with toys, even remembering from visit to visit which toys are Addy’s favorite (princesses, of course). Then, when it’s time for the procedure, they wheel her back to the Operating Room, and I give her a kiss while they drug her and then leave once she’s asleep so they can do the zapping. They wheel her back to the room within 20 minutes. (It’s a fast procedure.)

I should pause here to explain that in the past, we’ve had some trouble coming out of anesthesia. Addy was 3 or 4 when she experienced ‘emergent delerium’, which means that her body woke up before her brain. Anesthesia is steadily breathed out of the system, and if the body wakes up before it’s all out, the brain is still fogged, drugged, and weird. With Addy, this manifested first as weepiness, then quickly turned to an angry, inconsolable tantrum that lasted an hour. It was like being on the set of “The Exorcist.” It. Was. Miserable.

After that, I’ve always asked the anesthesia team to please keep her asleep as long as possible, to let her sleep off the drugs. At September’s surgery, they gave her Versed, which is a hilariously loopy drug that helped knock her out until her brain was de-fogged. Worked like a dream, and I got some very cute videos of a trippy Addy.

This time, we did the same thing. The nurse gave her Versed at 7:25a.m., and because Versed wipes out memory, he reminded me: “Her memory stopped at seven-fifteen.” Ok, cool, whatever.

Wrong.

When Versed wiped Addy’s memory, she lost the memory of seeing Dr. Zelickson, of being wheeled down to the O.R., and really, of most of the routine she had so intently looked forward to.

When she came out of anesthesia, she was confused and weepy and demanded that we do it all again! She cried because she didn’t believe us when we told her that we’d already done everything, and it was over now. “No, we haven’t! I haven’t seen Doctor Zelickson! You haven’t wheeled me down the hall yet! You need to wheel me down the hall!”

This is where Geri (the Child Life Specialist) comes in. As soon as the staff saw that Addy was distressed, Geri stepped up. Planted next to Addy’s bed, she calmly and clearly spoke to the confused little girl, firmly reiterating that yes, we’ve already done all of that, and then she reminded Addy of the yucky-tasting drug she took earlier, explained to her that it wipes out memory, asked Mommy to pull out the phone with which we’d taken a few pictures ahead of time (to show Addy evidence of the routine that did in fact take place), and explained to Addy in clear step-by-step terms that her big-girl brain doesn’t like being confused about sequence and routine, and that it’s okay to be a little stressed about it. She didn’t mince words, she held her ground when Addy insisted that we do it all again, she was kind, she was patient, she was empathetic, she was firm, she was clear, she was basically everything you’d want someone to be when they’re giving your kid a pep talk like this.

She helped catch Dr. Zelickson between procedures, so he could come back into Addy’s room and we could check that off Addy’s mental checklist (again). She even arranged for the two of us to wheel Addy’s bed down to an induction room (which looked like an O.R. but wasn’t in use) to fill in some of the routine ‘gaps’ in Addy’s brain. Check.

Can you see why I love going to a Children’s Hospital?  These people know their clientele.

When we were all done and Addy was ready to go, I took her down to the hospital cafeteria for a special breakfast, just the two of us. (A rare treat when you’re the eldest of 3 kids.) Her brain was de-fogged and she enjoyed herself.

All in all, I felt awful about wiping Addy’s memory of the experience that she had so wonderfully anticipated. She’s already counting down to the next one, and we’ve all agreed (me, Keith, Children’s staff, and especially Addy) that we’ll skip the Versed next time. Apparently, the risk of emergent delirium peaks around ages 3-4, so it’s less of a risk now anyway.

Each year of treating Addy’s port wine stain brings something different; we’ll see what Age 7 has in store for us.  (So far, it appears we’ll be dealing with a strange tear-drainage problem around the eye, but more on that later.)  Whatever it brings, I know that Addy’s in good hands at Children’s, and that we’ll figure it out together.

 

Having breakfast after Addy's latest laser treatment.

Having breakfast after Addy’s latest laser treatment.

Talking to Addy (part 2)

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In response to a dear reader’s inquiry, my last post discussed how we prepared Addy for the concept of having a huge dark-pink mark on her face; in this one, I tell how we actually talked about the port wine stain itself in more concrete terms.

As you’ve probably noticed in parenting, kids are oblivious to some things for an amazingly long time. For your child with a port wine stain, as long as she ignores it (or is oblivious to it), you can ignore it, too. You don’t want to force the topic and make it ‘An Issue’ before she’s ready. As I mentioned in my last post, only worry about laying the groundwork for her to believe that a distinguishing mark is a *good* thing.

But that oblivion doesn’t last forever, and at some point she notices it. She also becomes aware that all those comments from other kids and adults do, in fact, refer to her. She realizes that there’s something on her face, something others don’t have. This stage I found very, very tricky; you want to beat the world to the punch, you want to be the first voice she hears regarding her stain, her first impression and the last word. But you don’t want to make it ‘An Issue.’

So what do you say? Facts. This is the stage to be factual, neutral, and yet still calmly positive. Let her absorb the facts: That her face is pink (or purple). That it is a port wine stain. That she has a port wine stain. That Mommy does not have a port wine stain. Simple facts can be profound to a toddlers and preschoolers as they order their universe, so offer factual comments with a positive, contented attitude. The universe is in order; all is good.

If you catch her staring in the mirror at her port wine stain, offer a simple comment: “That’s your port wine stain.” Say it with quiet gladness, like you’re a garden-tour guide answering a question about local flora on a summer day: “That’s a purple lilac bush.” Pleasant, and neutral. Just a nice fact.

I found it helpful to focus on the words themselves: make this a language lesson. Toddlers are constantly learning how to speak and annunciate, and they’re thrilled with their accomplishment. “Can you say Port Wine Stain? Good! That’s right, po-oh-rr-t, make sure you say the ‘t’ there…” This accomplishes 4 things: 1) she knows it’s not a taboo topic, 2) you open the door to any questions that might be simmering in the back of her little mind that she keeps forgetting to ask you, 3) you’re empowering her to answer strangers’ questions for herself by speaking it clearly, and 4) there’s no pressure to Have A Talk about it. You’re just working on speech, that’s all. Happily.

After a surgery, she might stare longer at her reflection, observing the new shade of purple on her cheek. Sometimes just a solidly honest “You are so beautiful” is all she needs to hear; it’s enough to tell her that all is good with the world. “Yep, whew, I’m still beautiful, good, moving on.” Sometimes she’ll need a little more from you, as Addy did when she stared bug-eyed at the mirror at some especially intense post-surgery bruising: the reaction I used is the quietly-rolling “Oooooo!” that we adults use when we see an intriguingly lovely exotic fish in an aquarium: calm, admiring, hushed, and affirming yet neutral – “Ooooo, look at that amazing shade of blue.” “Ooooo, look at that, Dr. Zelickson really did a wonderful job with the laser surgery today.”

Then immediately start another language lesson: “Can you say, ‘laser surgery with doctor Zelickson’? That’s right – lay-zerr…” and so on, until she accomplishes the sentence. Then she’ll proudly show her accomplishment off by announcing “It my lay-zer zur-dur-ee wit doctuh Zeckickdon” to anyone who’ll listen. And when you see your daughter proudly (proudly!) telling others that her face is purple from a port wine stain treatment… it feels pretty awesome.

One last piece of advice I got from my mom: don’t call it a beauty mark. I called it that a few times when Addy was a toddler, in an attempt to soften the topic and make her feel good about it. As my mom pointed out, if I were to tell Addy that it was a beauty mark, I’d be setting her up for disappointment at the first reality check when a blunt playground kid impulsively responds: “No, it’s not.” Don’t attach beauty to its title. Call it what it is – a port wine stain. You can separately help her understand that her port wine stain is beautiful (as in my last post), but don’t tie its presence or absence to beauty. Just be factual and honest, with a pleasant demeanor every time. She’ll learn to accept the stain matter-of-factly, she’ll understand that it’s not a bad thing, and she’ll be equipped to face the world herself, big words and all.

Talking to Addy (part 1)

FacePainting

Back after a long hiatus!

In my last post (long ago), I responded to a dear reader’s question regarding what we tell others about the port wine stain. In this one, I’m belatedly following up to respond to the same reader’s question regarding what we tell Addy:

“…Any advice on how you talked to Adelaide about her PWS when she was Sylvie’s age [23 months]? Sylvie doesn’t notice it normally, but after this last treatment, she did touch her face when she looked in the mirror, so she notices that it looks “different.””

Ah, childhood oblivion; it’s a lovely thing.

As I was writing a response to this question, I found my answer to be getting ridiculously long, so I’m splitting it into two blog posts. Number 1 here is how we prepared Addy for the concept of having a huge dark-pink mark on her face; Number 2 will be how we actually talked about the port wine stain in more concrete terms when she was little. So if I seem vague here, or like I’m coming at the question from a 30,000-foot view, don’t worry – I’ll get more specific in my next post.

First, let’s cover the advice I won’t give you. When it comes to facing a blunt world with a unique face, other parents may empower their darling with the same indignance that soaks our culture. When Junior stares at the mirror confused, the parent crashes in with, “You’re PERFECT, don’t ever let ANYONE tell you otherwise.” Before Junior even sees a stranger doing a second take, Indignant Parent chimes in with, “ReMEMber, some people are just plain ignorant. Keep moving.”

I’m not that kind of parent. Indignation is great for making a sassy kid, but I don’t think it empowers them to be comfortable in their own skin. I’m a realist, and the reality is that you don’t need a sassy attitude to have positive encounters with other humans in the world; in fact, if anything, it hinders that goal.

My big-picture advice? Give your daughter a head start on feeling comfortable with her different, stained face. Start early, while she’s still mostly oblivious, and be subtle. We didn’t directly talk about the Port Wine Stain with Addy until she was in preschool, so in her early years I basically trained her to believe that having a different, marked, pink face is a good thing. Then I hoped that, when it finally dawned on her that *she* had a different, marked, pink face, it would be a happy and comfortable realization. So far, it’s worked.

To do this, send lots of little messages gradually, consistently, and frequently. You want to subtly convince her that port wine stains are awesome.

To begin with, I applied blush during my morning makeup routine when Addy was present. A lot of blush. Often. And I made sure to admire myself (think “exaggerated Hollywood starlet” kind of self-admiration) in the mirror. “Ooh, how lovely!” “Do you think that’s pink enough?” “I really want my cheeks to be nice and dark.” “Hm, I should make them pinker.” “Well, a classy lady needs nice pink cheeks!” And then I called in reinforcements: my mother, my mother-in-law, and the daycare lady each admired their pink-blushed cheeks in the mirror when Addy happened to be with them, applying rouge liberally and happily. “You can never have too much pink!”

(I know what some of you may be thinking – “What kind of message is she sending her daughter by relying on something as superficial and false as blush for beauty? True beauty should come from within!” That’s fine and dandy, but in our case, Mother Nature and Cultural Norms conspired together and slapped Addy with a big birthmark in the very color that women around the world aspire to have on their cheeks, so I’m pushing Addy to the front of the pack on this one. We all want pink cheeks? SHE WINS. And I’m not taking that away from her.)

So play up the pink-cheek thing; she might not realize yet that she herself has a super-pink cheek, but for now it’s adequate that she absorb the knowledge that it’s a very, very good thing to have.

Another thing we did was embrace face-painting at every single festival we attended. This one was harder for me at first; I don’t like anything touching my own face, and on top of that I’m a little nervous to have her port wine stain touched or pressured. But once I saw how happy she was to have her (other) cheek painted with elaborate girly unicorns and hearts and stars, I knew we could use this to our advantage.

So we made face-painting into this huge deal, this happy thing that happens at summer festivals. She looks forward to it throughout the year, and when those festivals come, we celebrate with painted faces. Because painted faces are awesome. Because it’s a desirable thing, a worthy thing, a beautiful thing to have SOMETHING on your FACE. And pick your words so that, again, she absorbs the knowledge that having a mark on her cheek can be a good thing: you don’t just say, “I’ll get my face painted, too!” -– you say, “I want something on my cheek, too!”

We also started using more wardrobe statement pieces for ourselves. I realized that my little toddler was watching everything I did down to dressing for the day. And the sweet little copycat would later go into my closet and stand in front of my mirror and mimic my actions, clomping around in my heels. So I decided to talk out loud while choosing an outfit: “Hm, I like this shirt… and I’ll wear these pants because they look nice…Very classy… But, hmm, I think I need to have something noticeable, something bright that will get people’s attention -– here, I’ll wear this!” And I’d grab one of my hundred brightly-colored accessory scarves and tie it around my neck, or my biggest, shiniest, cheapest earrings and secure them saying, “There, this will make people say ‘WOW!’”

I’m a conservative dresser; no one ever said “Wow!” But day after day I told the mirror (and my copycat) that I wanted something to catch peoples’ attention and set me apart, even if just for a moment. Some days it was a bright scarf, other days those big cheap earrings, and sometimes a lovely hat, because no one really wears hats and I said it would make me ‘stand out.’ (Likewise, my husband occasionally started wearing a nice hat, too, telling Addy that he wanted something to distinguish himself from other men that day.)

Her port wine stain never came up while we were dressing in front of that mirror. But now, when her port wine stain comes up in conversation and we mention that it’s ‘a little different’ from the other kids, her face lights up like she’s just won a beauty pageant. And when she was getting ready to go to preschool with a bruised face a couple years ago, she expressed sympathy that the other kids didn’t “get to have purple” on their face like she did.

Embrace distinguishing characteristics; if you wear glasses, then you can tell the mirror (and your copycat) proudly that “not everyone gets to wear glasses,” as if you’re lucky for standing out from the crowd. I showed her my little cross tattoo to prove that I had wanted something unique so badly that I actually employed needles to get it (and I hate needles). I bought face paints and let the kids have a blast painting their cheeks (and noses and foreheads and ears) crazy colors, because face color is fabulous and beautiful and fun and happy. When Addy whispers loudly that she sees a stranger with something different (“Mom LOOK! He’s missing a leg!”) I whisper back like we just saw a movie star (“WHAT?! No WAY!”).

With effort, luck, and time, you can help her know that distinguishing marks are awesome, and that pink cheeks are awesome, and that purple is even more awesome. With that knowledge in place, talking about the port wine stain itself will be easier. Instead of being defensive toward the world, she’ll be comfortable in it with her own unique skin, because she’ll know that unique is fabulous.

FacePaintingComplete

Talking to Others

A dear reader (whose daughter has a similar stain) asked me the following question:

“…I do struggle with how to respond when people ask questions about her face, especially after a treatment. I want to educate them, but at the same time, I don’t want to be judged. I don’t think the average person understands how extensive these port wine stains become with age and without any treatment. Do you have any advice on how you respond to these types of questions?”

Why, certainly. 🙂

First, don’t worry; there will always be people who disapprove of your decisions. They can’t do anything about it, so let them stew.

Second: my rule of thumb is to always (always!) strive to make the other person feel comfortable. Not only is it kind, but in our cynical culture it’s also unexpected, and therefore disarming to any potential jerks. (In other words, if they’re expecting me to be defensive and I’m not, they soften up immediately. Works like magic in most of life, actually.)

So, kindness is key. But how do we make others comfortable when we’re toting a small child who looks like she’s been in a barfight? And in only a quick minute or two of passing conversation?

I’ve found myself using the following lines the most – they’re simple, they sum up the problem quickly, use imagery that people understand, and are casual & humorous enough to put people at ease. Sprinkle them into the conversations as you wish:

• “Oh, she’s fine, she just had another laser treatment for her port wine stain.”
• “It’s a proliferation of blood vessels – basically, they never got the signal to stop growing in utero, so they just keep growing, and growing, and growing.”
• “The laser zaps them – they heat up, explode and die. So then we can zap the next layer – there are a TON of them.”
• “It’s like weed-whacking – those vessels are constantly growing, we’re just beating them back. The sooner, the better.”
• “Yep, as she grows, the blood vessels keep growing with her. The whole thing will get thicker and darker and even nodular over time. It’s crazy!”
• “It’s not a big deal, we just have to keep weed-whacking for a while, that’s all.”
• “We’re going in for another zapping next week.”
• “I think it’s pretty much the same thing they do for varicose veins.”

The ‘weed-whacking’ analogy clicks with people – they suddenly ‘get it’ that this is a long-term process against constant growth, and it makes them smile. (Who hasn’t battled weeds in their yard?)

‘Zapping’ also sounds casual and surprisingly noninvasive, and makes people smile. (What kid hasn’t shuffled their stockingfeet on carpet and zapped a door handle?).

Pointing out the relation to cosmetic surgery seems to make people more comfortable that this is a simple, noninvasive procedure.

When you speak with easy confidence and a smile, rolling your eyes at how these blood vessels just keep growing (and growing, and growing), waving your hand when you tell them “Oh, she’s fine,” and shrugging when you tell them she’s going in for another zapping soon, they’ll usually relax. You’re cool with it, they can be cool with it, too.

When you’re with your close friends and confidants, you can relay your anxieties, fears, and worries – after all, this is your daughter and there’s a lot to worry about. But as long as you’re in casual conversation, just make people comfortable, and you’ll find that most respond with kindness.

Variations: Not all port wine stains are the same…

Okay, so now that we know that a port wine stain is, for lack of simpler words, a ‘proliferation’ of blood vessels along a nerve, I can tell you that recent research (which you’ll stumble across if you Google ‘port wine stain’) has determined that it is not hereditary.

The problem is simply a mutation that happens somewhere along the way as the fetus is developing. If it happens early (and therefore multiplies itself as the fetus grows), then you end up with a bigger, deeper, port wine stain. If it happens later in development, then you see a smaller, lighter port wine stain.

We’ve met people with varied port wine stains, from a little light-colored “Whatdidya spill there?” spot on a cheek, to Addy’s “Woah! What is On That Baby’s Face?” mask, to a half-bodied “Is that a purple tattoo?” job.

Again (and this is really important), these are not hemangiomas, they are not strawberries, and they will not go away on their own. But they are often all lumped together, since they are all ‘vascular malformations.’

Oddly enough (for a problem that’s not genetic), my third child Eloise was born with another “vascular malformation” – in this case, a hemangioma on her tushy. How do we know it’s a hemangioma, and not a port wine stain? Because that bright-pink little splotch is raised & bumpy; a port wine stain is dark and flat (to begin with). So, we can be reasonably assured that this pink little splotch, unlike a port wine stain, will eventually fade away as she grows.

In any case, I don’t care how long it takes that hemangioma to fade. It’s on your butt, sweetheart; I’m not paying to get that sucker to get lasered off.

Treatment

I’m just one parent here, so all I can tell you about is my own experience: we took Baby Addy to a dermatologist who specializes in treating kids with port wine stains (Dr. Zelickson, you’re awesome) with pulse-dye laser treatment.

Basically, he hits Addy’s port wine stain with a yellow laser; because of the color spectrum, the red blood vessels absorb the yellow light, heat up, explode and die. When they die, they leave purple bruising behind, which clears up within a week. Overall effect: fewer blood vessels. And then we hit them again. And again.

In layman’s terms, it’s weed-whacking. The blood vessels grow as Addy grows, so we hope to weed-whack those suckers faster than they grow with her. That’s why we attacked them early (while they were young, easily-killable weeds), rather than waiting until she was older and the vessels were larger, tougher, and more numerous.

Results? Awesome. The stain is smaller and lighter. But because Addy’s happens to be more resistant (how appropriate for my stubborn eldest), we’re still weed-whacking after 30-some treatments, instead of the 12 to 20 they originally told us she might need. (It’s always hard to estimate, because each stain has different size & depth) But the progress so far has been fantastic. People mistake her stain, which previously lived up to its ‘port wine’ nomenclature, for a sunburn. Yessssss.

Each treatment requires Addy going under general anesthesia, since powerful lasers pointed at a squirmy child’s face would otherwise be cause for concern. I know some dermatologists just use local anesthetic or numbing cream (particularly for older kids or smaller stains), but as far as I can tell, Dr. Z. puts all of his kids under general anesthesia as a precaution.

In other news, I’ve heard that there might be some application for newly-improved cancer treatments to port wine stains: new cancer treatments cutting off blood supply to tumors (depriving them of their lifeblood) could potentially be applied to wreak equal havoc on the blood vessels of port wine stains. I shall stay tuned…

What is It?

For those of you who wonder what exactly a port wine stain is (and either haven’t Googled it or are overwhelmed by the search results), it’s basically a wild proliferation of blood vessels – they never got the signal from their nerve to stop growing. 

Normally (and this is totally “Port Wine Stain 101” in layman’s terms, so double-check all this and any questions with a legit M.D.), the nerve sends a signal to its associated blood vessels to stop growing while the human is developing in utero.   Now, look at a human face from the side, and imagine three branches of the facial nerve running from the ear to the center of the face: one high (up along the forehead), one middle (straight to the nose), one low (along the jaw).  Each of these branches has blood vessels associated with it.  In Fetus Addy’s case, the middle branch of the facial nerve was the delinquent one:  somewhere along the way in utero, that nerve never gave the “okay, stop growing now” signal to its associated blood vessels, and they just kept growing.. .and growing… and growing, until they were a huge tangle (TONS of them) and huge themselves (with diameters MUCH bigger than a normal blood vessel). And, voila, a port wine stain.

These are not hemangiomas; these are not strawberries; they WILL NOT FADE with time.  In fact, as long as the body is growing… the blood vessels will keep growing, too.  So, in Newborn Addy’s case, we could expect that red port wine stain to turn purple.  And, eventually, thick and nodular as the blood vessels grew and tangled and grew some more.  Hence, our choice to zap it – and to start zapping it as soon as possible (before those blood vessels grew thicker and hardier).  For Addy, that was at 5 weeks old.

So this, hopefully, answers the basic “what is it?” question.  Soon, I’ll go into more detail about variations (because not all port wine stains are the same), recent research discoveries, and treatment (yay for pulse-dye lasers!).

“A Painting”

(It’s been a while – sorry.)

Siblings. They’re as honest as every other kid out there.

Addy (who is now 5) just had a laser surgery, which makes her port wine stain darker and a bit blotchy with bruising. Clarence, her 3-year old brother, just noticed it – he pointed to the stain and said “That’s blood!” Addy corrected him nicely: “No, it’s not.” “Yes, it blood.” I interrupted: “No, buddy, Addy’s not bleeding.” “Yes, it blood.”

So Addy explained, “It’s my port wine stain from Dr. Zelickson.” (Close enough.)

Then she added (repeating what she had told me a few days earlier when we talked about going in for another treatment), smiling and touching her cheek delicately, “I pretend that this is a painting.”

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