Archive for the 'medical science' Category

Arm Surgery Results! (rotational humeral osteotomies)

Saturday, May 31st, 2014

Once upon a time there was a little girl named Laelia who was born with internally rotated arms due to amyoplasia, one of the many types of arthrogryposis multiplex congenita (AMC).

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It meant her little arms faced the wrong way. Go ahead and try it! Stand with your arms hanging at your sides. Notice how your palms face your body?

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Now turn your arms around until your palms face away from your body.

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Now imagine living life stuck like that. It would affect your…

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Cooking…

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Writing…

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Pushing a stroller…

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Pushing a wheelchair…

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Holding a basket…

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Holding a hand…

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Even holding a magic wand!

This is what Laelia’s arms just did as they hung at her sides.

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On February 12th we had surgery in Philadelphia at Shriners Hospital for Children to remove hardware in Laelia’s legs and do some releases in her thighs. (Our lower limb doctor is the amazing Dr. van Bosse!) We realized we hadn’t seen an “arm doctor” in a while since Laelia was so functional. We asked if we could see Dr. Z (Zlotolow). He agreed to see us last minute before he left for the day since we flew there from California. (People from some foreign countries fly less of a distance to come here.) He showed us how Laelia’s arms were rotated. Duh, we know, that’s why we do all those stretches! Then he showed us how surgically rotating her arms would make her more functional.

So we discussed what the surgery would mean for Laelia and if she was cool with it.

She had some different ideas for this surgery. She wanted something to make muscles go into her arms.

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Laelia’s vision

We explained that this would not do that, that she didn’t qualify for surgery that could do that since she’s missing the nerve endings and enough healthy muscle for a transfer. Well, at least where medical science stands now. But this would rotate her arms so she could eat lunch easier. She agreed that was something she wanted.

Six weeks after Laelia’s “last surgery for a while” we were once again traveling 3,000 miles back to Philadelphia to undergo surgery. Dr. Kozin and Dr. Zlotolow each took an arm and like synchronized swimmers they gracefully rotated them at the same time. (This was the first time they had EVER done both arms at the same time; and only because we were a special case.)

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“You take that one; I’ll take this one.”

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“Okay, go!”

And she got out of surgery looking like this:

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This picture marked the first time in Laelia’s entire life that she had arms facing the right direction. *getting emotional here* And instead of actively forcing them into this position she was unconscious. Unconscious! I’d never seen her arms like this while she was asleep before!

(They could have rotated them even more, but then she wouldn’t have been able to reach her bottom to use the potty independently. She’s 100% independent in the bathroom now, depending on the outfit. ;))

Four weeks of casts later…

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Casts.

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Bored.

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Hi, casts still.

Then…

Back we flew to Philadelphia! (Making it TWO trips to Philadelphia we hadn’t budgeted for. :))

And casts finally came off!

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LOOK AT THOSE ARMS!!!

And that affected her ability to…

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Hold Fuzzy Bear…

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Color her Scarman coloring book

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Play a game of cards…

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And even fold her hands together!

Funny story: Charles and I were complementing Laelia’s new arms while she sat there and beamed. Finally we looked at her for her response and without missing a beat she put her fingers together (a feat that’s new since surgery) and said in the most perfect Mr. Burns voice, “Excellent.”

We died laughing.

Oh and her IEP (Individual Education Plan, required for special needs kids to go to school) happened around six weeks after surgery and her adaptive PE teacher, occupational therapist, physical therapist, the school principal, her teacher and aides all spoke about how she can write better, feed herself better, hold things better, etc. She had missed a lot of school for this, but she came back a totally new girl with different abilities! And they were amazed!

So to recap: We walked into the hospital like this:

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And walked out like this:

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faith healing (because again?! people!)

Friday, May 30th, 2014

Trigger warning: Sarcasm, faith healing, more sarcasm

*ahem*

There’s a reason we don’t pray for someone’s amputated leg to regrow. Even those with the gift of healing usually draw the line there. I have never seen a group of amputees line up outside a faith healing tent and come out with arms and legs. I’ve known three faith healers (family friend, friend of friend, family member) and all three have never even offered to regrow people’s limbs. (Rude!) We humans reason (correctly) that there’s a natural order to things. Could God regrow limbs? Sure. So why don’t we regrow them through faith? Because we are not lizards (yet).

So why do people insist that God can regrow my daughter’s muscles that she literally does not have? Literally! The anterior horn cells in lots of places were disrupted during fetal development and those body parts did. not. form. And (I’m sad I have to even make this point *sigh*) that fact does not change just because you can’t see it!! (Science!)

If I was born without a liver or foot or brain could prayer create one out of thin air for me? Does God bend the laws of physics in that way any other time? (Imagine playing Monopoly and then the creator of Monopoly is all, “Randomly all properties will now be used as tarot cards. Oh you have Park Place? That means you’ll be unlucky in love. But hey you’re winning! How? No clue!”) Because that’s what you’re saying. Just realize that. That right there. You’re saying that!

If you pray, pray normal things. (Talking to unfathomable cosmic creator of all things with your mind = normal!) Okay, like I pray she works hard in physical therapy and that her goals are worked toward with patience and with grace. I pray she learns how to navigate the world with the incredible body she does have! (Because she’s different, not tragic.) I pray for wisdom for us as we make medical decisions on her behalf. I pray for technology to catch up to need. (But I’ve given up on insurance catching up to need a long time ago.) I pray for her to have this light that shines in the dark spots of our culture. I pray she grows up to help the poor and fight injustice. And today I prayed for the poor unaware bully who got her lip! (Note to bullies, don’t call my daughter “bossy.” It somehow gives her cosmic authority to take charge of the correction of your mistake, known to you as “bossing you more.”)

Heck I can’t tell you what to pray, go nuts! Pray she grows wings! Because wings!!!!

Just, I don’t know, please see arthrogryposis logically and don’t get stinking mad at us or God when the laws of physics don’t go topsy turvy at your prayer missives. My kids will do amazing things and “prove doctors wrong” but it will likely happen within the realm of reality and follow the rules of the universe.

PS: My son is also missing muscle, but not as notably so he has received considerably less offers of miraculous healings. Don’t worry, he’s not feeling left out. Unless you start offering my family faith healings *and* cookies. Which is only polite.

PPS: But but but! What about Jesus and the dude’s ear or part of St. Augustine’s leg or stories like that? Most faith traditions have miracles or healings that held some purpose. I think we could *at least* agree these are exceptions to the rule.  And I am referring to modern, has-access-to-Internet faith healers in this post.

PPPS: Chocolate chip cookies with chunks of brownies in them. No store bought crap.

Laura’s kidney (kidney saga continues!)

Monday, November 18th, 2013

(For a link to the story with text click here.)

I want to thank Ashlee DeMartino from 10News for airing the original story (seen here) about the cancelled surgery. James is a great advocate and because of his fight and the exposure from the news report a new surgery date was given that’s only two days from the original date instead of two or more months! This last week has been a roller coaster ride for the Rivera family. Thanks for all your prayers and support. My aunt, uncle and cousin fly in day after tomorrow! Exciting!

(The above video contains Roland being a cute little annoyance who wouldn’t let us do an interview. He was really cute. He talked the whole time I did. :))

There’s so much background to this that is incredible. One of my good friends, Meg (who is responsible for the veggie lasagna that kept my newly adopted and very stubborn son alive for the first month home), really cared about her coworker, James. In fact one day when she was over she pulled me aside to talk about him. She told me all about his family and his little girls and his circumstances. She encouraged me to find out my blood type and consider donating to him since she was one of the few who knew I was starting that process anyway. I contacted my kidney coordinator and, after talking to Janine I got James’ coordinator too, but long story short he didn’t fit in my chain unless we wanted to kick several people off the chain who had been waiting a long time. Plus our two hospitals weren’t really compatible, although Sharp was willing to try to work it out if there were no other options. (Sharp Memorial and the NKR pretty much would have done whatever I asked even if it was in no one’s best interests but mine. They take care of their donors.) Then Laura said she would see if she could help. (So in a way Laura gets credit for the six additional people in my chain who got their kidneys since James was now taken care of.) After the testing Laura found out that not only was she a match for the oh-so-hard-to-match James, but she was a VERY close match! The kind of match they usually only see with siblings! I texted Meg the good news about Laura and she was texting me back while in labor and I didn’t even know. Haha! Her son was born sometime soon after that! This is the most random amazing story. James’ and Laura’s surgeries are this Friday!

Video of Laelia

Monday, September 16th, 2013

I created this video of Laelia learning to walk as a present for her 6th birthday. I used all of the videos I had of her where she is moving her lower limbs. And let me just say that there were not many videos of her even moving her lower half for the whole first year there, despite daily physical therapy and stretching. Wow, it’s amazing how far she’s come and how hard she worked. Laelia’s birthday party isn’t for a couple of weeks yet, but tonight she caught me editing, saw it was videos of her and then squealed and demanded to see it. (By the way this is my first video editing job EVER and it was really hard and I cussed a lot and now I hate technology.) So the cat’s out of the bag and I’m just going to make it public.

Enjoy.

New diagnosis

Friday, August 2nd, 2013

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my hubby and my baby

I haven’t wanted to sit down and open up on the blog lately. I have my fingers in too many pies, and a lot of my work is done online so when I have a minute to myself I don’t exactly want to bang out more copy at a computer. I’ll just make this brief, or as brief as I’m capable of. Last Friday Roland had a great day. He went to preschool. He wrestled with his sister. He got into the tickle fight to end all tickle fights that ended with all of us on the ground gasping for air. He threw some blocks and crashed his walker into everything. He grinned and laughed and spun in circles. Then it was dinner time. And he didn’t want any. Then he threw up. He’s thrown up before without incidence so we waited it out with a cup of apple juice. But he threw up everything that touched his lips until he cried. I put some glucose gel into his mouth and rubbed it into his gums when he wouldn’t swallow it, but it was too late. An hour after the first gag he was seizing. And we ran out the door to the ER.

We were in the hospital from Friday night until Sunday night. It’s thrown off our whole week. (Sorry for the 200 emails I’m ignoring and the two orphan care blogs for Aug 1st that posted on Aug 2nd.) The ER is only three and a half minutes down the road and he was rushed in and given some glucose after they recorded his blood sugar at a 14. (The lowest it should have been was in the high 70s!) The doctor said this level was low enough to cause brain damage and wondered aloud why the hell our insurance had not covered the life-saving glucagon gun the last time he did this?!

[For our new readers, about 8 months ago at the tail end of December Rolly had a similar seizure fit and a ridiculous blood sugar score of 19. I blogged about it here. The doctors guessed since it was an isolated incident and probably wouldn’t repeat after he was getting good nutrition. We chalked it up to a lack of “reserves” from poor nutrition and orphanage life in general. Our insurance even refused to cover the glucagon gun making it almost $500 out of pocket and outside our budget. So we watched him like a hawk for three months and then shrugged it off.]

Now that he’s had his second episode, despite excellent nutrition for almost a year, it’s clear our son has an unknown metabolic disorder and a temporary (until we can figure out more) diagnosis of “seizures due to low blood sugar.” It’s like he’s hypoglycemic, but just sometimes. And both times his crashing has had a clear progression of lack of appetite, fasting, lethargy, vomiting and then, after lots of vomiting, seizures. And even though this process takes a couple hours, that’s still really fast! Thankfully after this second life-threatening episode our insurance finally covered (in full) the glucogon gun. Two of them actually. And a new monitor.

Rady Children’s hospital is excellent when it comes to parent training. We were re-trained on our glucose monitor that has been doing nothing but collecting dust for eight months. And I used the glucogon gun on my son to test if it worked while he was still in the hospital. It worked great, but dang. The needle is the size of the inkwell in a pen… just really big. Roland was pretty mad at me after that.

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yeah I hadn’t given him the shot yet

Then I took his blood every 15 minutes after that for an hour. Yeah. *Me.* The girl who passes out watching other people give blood and has passed out twice from giving blood herself was taught to take blood and test the sugar content. And I can do needles now. I didn’t even pass out a little bit!

I even practiced shooting Mr. Bear full of insulin. Roland looked so protective of Mr. Bear after that.

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(check out the countries represented)

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Laelia cried the whole first night, refused to sleep without Roland there (they share a room) and said in a pathetic voice, “He’s gotta be okay. He’s my best brother in the world.”

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Best brother got an IV in each arm. One to take blood and one to give sugar.

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Then best brother tried to pull out both IVs like a million times until they were forced to put arm immobilizers on him.

So we’re home now. We’re doing okay. Roland is his old self again. We’re just all in a daze figuring out our new normal.

And we have appreciated all your prayers so much. And those of you who just wrote to ask how we were doing and say you were thinking of us, and those who offered meals or help, or those who visited… it just meant the world. It was very sustaining and I know I never thank people enough in the moment. I just suck every last drop of love or energy or help they’re offering and then a week later when our heads are above water I stop and think, “Wow that was amazing and they meant so much to us. I hope they know that!” So thank you! You’re appreciated. I hope you know that.

Alright I’m tired and the kids need a bedtime story so I’ll wrap this up.

Right now our three biggest issues are:

1). Getting an appointment with the metabolic doctor. I’ve called and finally I was told a WEEK after our hospitalization that the doctors would “talk about it” and give me a call “in a week.” I verified they actually meant I had to just wait around for 7 days and couldn’t make an appointment in the meantime. Yes that’s exactly what they meant. But, hey, maybe they can get us in next month! Yay? :-/

2). We’re new to this and right now we’re constantly worrying about what he’s eating, how much he’s eating and how often he’s eating. It’s stressful. Like right now he won’t eat dinner and that won’t fly these days! And peanut butter on whole grain bread is served for like every meal. So eventually we’ll figure this out, but right now it’s stressful.

And 3). Roland’s new diagnosis disqualifies him for Head Start. (Where he would go next year for preschool after aging out of Early Head Start.) We were counting on that. And I doubt anyone else will take him either.

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Husband’s one year donation anniversary

Wednesday, March 20th, 2013

Today, March 20th 2013, marks the one year anniversary since my husband saved the lives of several people. He didn’t jump into a burning building, throw himself on a land mine or even wrestle a bear. He just laid there. :)

He donated his kidney to a stranger, setting off a kidney donation chain. The stranger, a lady in her 50s, will now live to see her grandchildren. And her husband who wanted to donate to her, but wasn’t a match, was able, in exchange for my husband’s kidney, to “pay it forward” and give another person a life-saving kidney. And so on.

I created a little PR cartoon to mark the occasion.

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Click to enlarge.

 

#1. What if something happens to your other kidney? Altruistic donors  (people who give to strangers) go to the top of the kidney transplant list if anything goes wrong with their remaining kidney. They are given top priority. They rarely need it. (Says our medical team.)

#2. Will that shorten your life? No. Actually altruistic donors live longer than the average person. (Says our donation coordinator.) Having one or two kidneys doesn’t matter at all. If your kidney is going to go, usually they both go at once, and people who can donate are healthy to begin with. So it’s a lot like saying, “Healthy people live longer than the average person.” And your surgical risk is the same as any other surgery that you would subject yourself to at one point in your life or another. (Our daughter is looking at her seventh surgery later this year for pin removal in her knees. The risk is 0.0032%.)

#3. I would but I don’t want to give up my glass of wine or drinking on my birthday or… you know… my friend Mr. Alcohol. You don’t have to stop drinking, or make any major lifestyle adjustments with one kidney. They did suggest no cage fighting. I shot a look at hubby at that point in the discussion and laughed my head off. But of course binge drinking is bad for you in lots of ways.

#4. But what if someday his kids need a kidney? Well kids don’t wait for kidneys as we learned. Adults do. Kids are at the top of the list. “They’re covered,” said our social worker when we were asking questions. It was the top question on my list even though only one of our children is biologically related to us. It turned out not to be an issue.

#5. What if a family member needed that kidney and you wasted it on a stranger? Waiting for a family member to maybe need a kidney when lots of people are right now dying is like saving your fire extinguisher for a possible home fire while watching your neighbor’s house burn. Actually it’s worse than that because you have a much greater statistical chance of having a fire than ever needing a kidney. There were around 17,000 people who needed a kidney in 2009 and around 362,000 fires (source and source). Let’s put it this way, there are 1,000 cases of lightening striking people a year. (Source.) Say every time lightening struck you, 17 of your neighbors needed kidneys. That’s significant but not a ton of people.  In fact I called up the National Kidney Disease Education Program and was told 1.9% of the population are diagnosed with kidney disease, but of those people the number who will benefit from a donation is even smaller but they do not have the statistic for it.  They sent me to the Center for Disease Control and Prevention’s Dada and Statistics branch at which point I realized I don’t need an exact number for some research paper and was happy to just say on my personal blog “it’s not likely your family will need a kidney that only you can provide.” Let’s speculate that you have less than a 1% chance of needing to save that kidney for a rainy day. Math was a major factor in my husband’s decision to donate.

#6. What did it cost? It was free. Everything from the hospital stay, recovery, doctor’s visits, parking, etc. Hubster’s work even had accommodations for altruistic donors and we didn’t need to dip into his vacation time for recovery.

#7. Any regrets? One. My husband was disappointed that he didn’t have a cool scar. Since it’s a laparoscopic surgery the recovery time is easy and the scarring minimal. They squeeze that little sucker out your belly button so my C-section surgery totally beats up any of my poor husband’s surgery stories. Hubbers went shirtless to a pool party afterwards and no one noticed. But if I lifted up my shirt you would all wonder how all of my guts did not fall out of this giant incision.

Hubby: “My tummy was sore for a while there.” Me: “Oh were you sore? Oh poor thing! Were you unable to climb stairs? Stuck in the hospital for days? BREASTFEEDING??? No?????!”  (Owned.)

#8. I want to help, but isn’t it enough to let them just harvest my organs after I die? They need live donors. Desperately. Becoming a donor after death is wonderful, don’t get me wrong, but to donate your kidney you have to die in a very specific way (I heard a doctor lament, “Can I get a good drowning case?”) and even then the kidney of a deceased person only lasts half as long as a live person. (Source.) A kidney from a live donor, according to Nicholas Crace’s doctor, “performs better, works quicker and lasts longer”. (Source.) Who is Nicholas Crace? He’s an 83 year old altruistic kidney donor. “Within a week I was bicycling and mowing the lawn,” he claims. To which I want to say, “Really?! My husband was playing video games and watching movies!” He was mowing the lawn, hubsters! (Owned again.)

People are suffering and dying and need someone with no benefit to themselves to step forward. Lots and lots of those people actually. And we’d love to help you do it!

Roland’s knees and legs

Saturday, February 16th, 2013

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I’ve already done a blog on my daughter’s knees and their progression from bendy to straight so you all already know how amazing this little girl’s story is. We went from doctors absolutely flatly refusing to cast her knees five years ago (and denying us for stretching knee splints because Laelia was too “disabled” to utilize straight legs) to commuting to Philadelphia so Dr. van Bosse could get this little lady’s legs super straight and walking everywhere. She’s even begun to climb small steps!

Five years later and one of the original doctors who didn’t want to cast Laelia’s legs straight was all too willing to cast Roland’s legs straight. (Saving us weekly flights to Philadelphia.) Medical practice evolves, thankfully, and I want to believe my daughter was a pioneer in that regard, at least in a small way. I do know her pictures were used in many occupational therapy presentations across the country, and I have met people who have seen this blog and changed their expectations for their AMCers based on it. Her story was made stronger by the dim predictions of some of the best doctors in orthopedics early on. And I’d like to think there was a reason we did not have instant access to the best advice and therapy techniques. Maybe it was so people could learn from our mistakes. Maybe it was so we could travel to Seattle and Philly and Kansas City and meet many people. Maybe so others who had also missed the boat could get hope that if Laelia could start walking at age 4 and independently at age 5 then their kids could too.

Well, I know one AMCer who has had his life changed by Laelia’s experiences: her brother. Someone who was not believed to ever walk himself and started doing that very thing after six months* of Ponseti casting, bracing and stretching.

(*Now Laelia’s experiences seem to be at one end of the spectrum  and Roland’s at the other. Roland responded really well to casting. He avoided tibial subluxation (a complication of casting knees) and he had enough of the right kinds of leg muscles to walk without needing years of PT intervention first. What has taken Laelia years has taken Roland months. We know we got off easy with him. So I guess I’m not saying, “Roland achieved ambulation in six months and so can you!” The same exact therapies would not have worked as quickly on Laelia’s body. Every AMCer is different. But I am saying even the most severe cases of amyoplasia can be ambulatory. I know two adults around my age who have decided to start the process of ambulation or easier ambulation later in life. And Dr. van Bosse has seen many many adopted older kids with AMC and increased their functionality greatly!)

Okay now on to the Roland pictures. Here’s what’s been going on for the last several months.

Roland’s knees

Roland's knees

(Click on any picture to enlarge it.)

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Those knees were stuck around 100 degrees for one leg and 90 degrees for the other.

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Stretching and gravity didn’t straighten them much.

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Roland’s little legs during tummy time.

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Combined with his little curved feet we knew Ponseti casting was in his future.

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After a month home we had stretched his arms to around 15 degrees of bend, but his knees only gained a few degrees of range from the stretches.

Time for casting!

Casting montage!

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Dinosaurs from Philly. Dr. van Bosse started us off with the first set of casts. The following were all done locally.

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Red

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Green

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Black

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Purple

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Blue

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Right after blue were removed. Look at those straighter feet too!

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From the side

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Red, green, blue and purple

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Orange was our favorite!

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Blue again. This is Roland playing at the Polar Bear exhibit at the zoo.

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Right after second set of blue casts were removed.

They gave him little stockinette gloves to keep him from scratching at that poor skin.

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Look how straight those knees are getting!

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PINK!!!!!!!!!!!!!!!!!!!! Just for kicks.

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Standing practice! (Hiding the pink casts under yellow socks. ;))

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Some skin breakdown meant we needed to take a break from casting. But his feet were very close to perfect!

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This is how far we got his knees. It’s easier to tell in x-rays.

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FIRST REAL BATH IN MONTHS!!!!!!!!!!!!!!!!!!!!

(The casts smelled so bad.)

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The casts were bivalved (cut in two) and became removable.

We kept them on with ace bandages while his leg braces were being made.

Those ace bandages had to be burned afterwards. STINK!

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Finally new KAFOs! (Knee Ankle Foot Orthotics) The left leg needed more time to heal at this point.

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In Payless getting his VERY FIRST PAIR OF SHOES! He’s really thrilled.

Once they healed enough (and we put a ton of liquid skin on the sore areas) someone could wear his KAFOs again!

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And he’s standing independently for the first time!!!!!!!!!!!!!!!!!!!!!!!

(And he’s thrilled about it.) :)

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You’ve come a long way little guy.

:)

New Feats for Rolly Man!

Monday, January 14th, 2013

I’ll keep this brief. Roland started out with super straight arms and super bent knees. And they were stuck that way. And I mean STUCK.

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Roland before any medical intervention.

 

After therapy, stretching and casting  for the last five months, Roland’s arms can bend enough to suck his thumb (!!!) and his legs are straight enough to walk across the living room without a walker.

WITHOUT A WALKER!

(Those were links up there. That’s right. Videos. Clicky clicky!)

Oh yeah and the doctors in his birth country didn’t think he could do either one.

Take THAT arthrogryposis!!!

Oh…

You may all commence screaming now.

YAY YAY YAY YAY YAY YAY YAY YAY YAY YAY YAY

!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!!

Roland don’t ever scare me like that again, you hear me!

Tuesday, January 1st, 2013

It went like this.

Super happy crazy boy one minute…

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(Click here for video.)

And seizures and vomiting the next minute.

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We had a super fun time with pizza and friends. Everything went great. I put two hyper, pizza-covered kids into bed around 9:30pm. The next morning around 8:00 Roland woke up covered in vomit.

After that he stayed in my arms for the next three and a half hours. I admit I enjoyed it. Baby in one arm and Facebook in the other. We took naps together and he snuggled under my chin. Then he would make a noise, I would get out the bucket and he would puke and then go right back to sleep in my arms. I just purred over him. He is usually so crazy rambunctious so this was nice. We even took a picture to remember this.

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But the poor guy kept throwing up which worried me. He threw up small amounts eleven times in those three or so hours. I had a cup of apple juice ready for him but he wouldn’t stop puking or sleeping long enough to get any fluids. Then right before noon he started his first seizure. I ran him out to the living room to show my husband. It was the tail end, but Charley took a video. When he started another one we ran out the door. I jumped in the driver’s seat while Charley loaded him into his car seat. He didn’t look good. The emergency room is only about four or five minutes using back roads so I took off instead of calling 911. Roland had five (maybe more) seizures after that. He was seizing in my arms as I ran him into the ER, and he continued seizing as the RN took him from me. The RN’s name was Steve and he was a big guy. My little guy looked *so* little in Steve’s arms as they quickly made their way through the double doors. Whereas I was a panicky mess, Steve calmly described the seizure as it was happening and that made me feel like maybe this wasn’t so bad. Maybe everything was fine and they see this every day and it turns out okay. So I calmed down a lot.

Then I answered about 800 questions about arthrogryposis and adoption, none of which were helpful for a seizure, but the doctors needed his history. One doctor asked if I was his mother and then demanded to know why I left him in an orphanage for almost two years. I explained I adopted him and he’s been home four months. She replied incredulous, “Wait, but you said you were his mother!”

The truth is shocking. He has no medical history. I don’t know what he weighed at birth or if he came early. I don’t know how healthy his mom was or what surprises are in his genes. I don’t really know what they gave him at the orphanage. I don’t know what drugs he’s been exposed to. I know he has a lead count of 2 (at 3 they do medical intervention) and he has had to catch up on vaccinations, but other than that I’m no help at all. I kept being asked, “Has he ever done this before?” And kept having to answer, “Not in the last four months!”

And in the back of my mind was the knowledge that he had been misdiagnosed with convulsive disorder in Ukraine. That diagnosis turned out to be false. (The “doctor” had mistook a jerking arm for a convulsion because she didn’t realize AMC came with muscle loss and he would need to swing his body or bounce his arm to move it. After meeting Laelia and seeing how she moved the doctor said Yegorka [Rolly] did not have convulsion disorder after all.) But what if it was true? It was scary to think about.

I was worried Roland would be scared of the hospital or strangers, but at this point Roland was completely unresponsive–no eye contact, couldn’t close his mouth, non verbal, limp, eyes rolled back. The nurse was checking out his head and shoulder for veins, but finally it was decided that he needed an emergency cast removal in order to place the IV. I was glad Laura (from Dr. W’s clinic) was there to do it since we know her and she is super fast. Roland had a startled response during the sawing part, but he was not “there.” His eyes were crossed and downward, the right side of his mouth did not move while the other side twisted in pain and he looked like he was in a coma. I kept crying, “That’s not normal. He never does that.”

Roland was given ativan and glucose. He was hypoglycemic with a blood sugar count of 19! Normal for his age is 70s to 80s! Eventually Roland made a noise. I asked him to say hello but he couldn’t. (I ask him to say hello about a hundred times a day and he happily complies about a hundred times a day.) He was rolling his eyes looking around the room, but unfocused. It was creepy. Eventually he looked right at me and seemed to recognize me (he had not up until this point) and he lifted his arms for me. The nurse said, “That’s the glucose working!” I asked him again to say hello and he tried so hard to speak but couldn’t. A tiny squeak came out, but you could tell he was trying. It took him a while to come out of the seizure. Eventually Roland looked at me, right at me, and said “da.” Then he looked down at his arm which now had no cast and an IV and wires and he just looked baffled.

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Roland’s right arm without a cast and with an IV.

Every finger had to be poked more than once so eventually he was just covered in bandaids.

Charley and Laelia then came through the doors at this point. We were transferred from the ER to the critical care unit as a family. Because they had to move fast and Laelia cannot walk that quickly Steve put her on the bed. She comforted her brother and enjoyed the ride. Roland was still completely out of it and barely noticed his sister.

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We left the ER and arrived at the critical care unit. Roland was connected to more monitors and the doctors came up with a game plan. They wanted to get Roland’s numbers up, keep them up long enough to make sure he was out of danger, and then purposely lower them again in order to run tests. So he went from 19, up to 120, then artificially crashed back to 40 to run the tests. Poor guy was so scared every time someone took his blood pressure or poked his finger or anything. Then when they introduced glucose again after the blood draws his numbers shot up to 250! His poor body just couldn’t regulate.

The hospital had Christmas presents for patients left over so they gave him a dog pillow. Roland hugged and kissed it (he’s really attached to stuffed animals) and then fell asleep with his wittle face pressed against the bars. That was his favorite way to sleep.

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After the tests were run we had to wait overnight to get answers. In the afternoon Roland was sick of the hospital and tried to take a nose dive off the crib and pull his wires out. I ran home with Laelia and we grabbed Roland’s pillow, blankie and teddy bear for comfort. (Also a toothbrush for Daddy.) Roland settled at that point and fell asleep… only to be woken up several times with blood pressure tests and blood draws and finger pokes and monitors beeping.

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Okay here’s what we know. We know the seizures were absolutely caused by low blood sugar. Not an injury (that boy is always bumping his head), not a brain condition (so no MRI needed) or anything like that. Here’s what we don’t know: why vomiting caused his blood sugar counts to crash that much. It shouldn’t have. Also we don’t know if the low blood sugar caused the vomiting or if the vomiting cased the low blood sugar.

Here’s the doctor’s guess after two days in the critical care unit: Roland had very small reserves for glucose from his life in the orphanage, exposure to lead, lack of medical care and poor nutrition. The doctors say that all his medical issues from being deprived early in life will be completely reversed with time and not to worry, but we will keep an eye on his blood sugar from now on. We have been educated about hypoglycemia and what to do in an emergency. (Of course our insurance denied us for the emergency medicine that would have saved our son five seizures if we had had it. Thanks insurance!) We got a chemistry quiz and biology lecture from the endocrinologist who should really be teaching in a classroom somewhere he’s that good. When we got home we had training on this new condition, a glucose monitor that even blood-phobic me can use and an emergency plan in place. After one night’s sleep at home Roland was happy and bouncy again.

 

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Just kidding guys! I’m fine now!

:-/

 When we got home we were informed that we had end of the year adoption reports to do. They ask what Roland’s diet, daily routine and health is like at home with us. It took everything in me not to write, “Like hell you care! F you!” and send it back just like that. But if Russ*a can punish thousands of dying orphans for the mistakes of 19 American families (out of 60,000 over 20 years) then I had better do what’s asked of me and what I’ve promised to do and continue to play nice. Still I’ve been pretty mad at Roland’s birth country, orphanage and birth family as well as those in my church and community who do. not. care.

*deep breath*

But now we’re doing well and everything is slowly getting back to normal. Roland says hello.

 

 

 

 

Raise your Glass foundation

Monday, November 26th, 2012

A.J.’s parents are launching the Raise Your Glass foundation to honor Dr. van Bosse and the work he did to get their son walking. You can read about it here. The following is my contribution.

I get air sick. I just wanted to start with that in case anyone is under any delusion that I enjoy flying 3,000 miles one-way from California to Philadelphia to see one specific doctor. My family lives five minutes away from the hospital that U.S. News ranked #2 in the nation for orthopedics. My daughter, Laelia (LAY-Lee-Uh), was not only correctly diagnosed with arthrogryposis the morning after she was born, but also with the correct type of arthrogryposis, out of more than 400 types. Our pediatrician could not only pronounce arthrogryposis, but upon our first visit he produced a photo of himself and another patient with AMC–a photo affectionately on his desk. After hearing from so many other families I realize our story is rare for how easy we had it. If anyone was blessed with a working knowledge of one of the rarest conditions in the world right from the start, it was us. If we could have had our daughter anywhere in the world, San Diego would have been hand-picked.

Yet doctors gave up hope.

I can’t explain it. Well, it’s true that my daughter did not really move her arms or legs for the first year of her life. She also has extremely low muscle tone that one local doctor described as the lowest muscle tone that he’d ever seen in kids with AMC. Despite hearing that I continued to make appointments and try to get more help for my daughter. I must have been annoying because the head surgeon actually took my shoulders in his hands and told me to accept that my daughter was disabled. Then he left the room without signing the prescription for splints in my hand. It was for knee splints and the doctors were convinced that straightening Laelia’s knees would not allow her to be comfortable in a wheelchair where they predicted she would spend her life. Every doctor’s visit came with a wave of depression as we grieved the loss of hope. The last glimmer of hope to die was when we learned about an arthrogryposis clinic in Seattle from a friend who was willing to pay for us to go there. It was the final nail in the coffin when that orthopedic big wig gave up on our daughter after one visit. He would make the fifth pediatric orthopedic doctor to say, “Nothing to be done. See me in six months.”

We found out about Dr. van Bosse after we had already given up. I hate to admit it. Even when success stories were pouring out of Shriners in Philly, I was still skeptical. It wasn’t until our friend Cheryl and her daughter, Tracey, spoke up about their personal experience with Dr. van Bosse that we agreed to see him.

At this point I felt like an AMC expert. I was familiar with my surgery options, or what little there were. I knew all about splints, walkers, weight-bearing, wheelchairs, casting and stretching. I owned the AMC Atlas textbook. And my daughter had already been through two major surgeries and some tendonotomies before local doctors had told us she was “done.” I felt so certain she had more potential than doctors could see. So we went to Philly.

The trip to Philly was rough. So rough that I was praying the great and powerful doctor was merely just the man behind the curtain.

Instead I got to meet my very first expert in the AMC orthopedic field. I was impressed with a doctor who listens, examines and waits. But I’m not here to write about bedside manner, although I remain super impressed with a doctor who will email me back within 24 hours with answers to my medical questions. Dr. van Bosse introduced us to an entirely different  methodology   So far Laelia had only had soft tissue surgeries. We kept hacking at precious resources my daughter needed to move. Instead Dr. van Bosse introduced us to derotational osteotomies. And unlike the doctor in Seattle who first mentioned them but would only perform that surgery on patients who “deserved” it (his PT said Laelia’s muscles were too weak), Dr. van Bosse left his crystal ball of future predictions at home and focused on the issues at hand. The most important thing was to give her a chance and see what she did with it. And unlike others we’d seen, all surgical suggestions he made were backed up by evidence. I was shown my daughter’s x-rays and then x-rays of the same exact lower body contractures of other anonymous patients before and after the surgeries he was proposing. It wasn’t a “take my word for it” pitch at all. It was a course of treatment over the next few years with known results. Instead of, “Will my daughter walk?” my questions became, “When will my daughter walk?”

Hips before.

Right after surgery.

Hips after.

Knees before.

Knees right after surgery.

Knees now. Perfectly straight and aligned.

My daughter walks. (Now I’m crying as I write this.) Everywhere. Up and down inclines, around the grocery store, all over the house. Without a gait trainer. Without a walker. Without pediatric crutches. And recently she took a few steps without even her leg braces on. Having correct alignment allowed her to exercise and grow muscles in her legs we didn’t even know she had. I can’t begin to describe how her life has improved dramatically since she gained the ability to stand and walk, something she never would have done without Dr. van Bosse. My daughter was almost five when I had my first conversation with her about walking in the street, opening the bathroom door when I’m in there and running with scissors–conversations I was told we would never have. If not for Dr. van Bosse Laelia would be sitting on wide, abducted legs right now.

Doctors should never give up on children. Children are the very definition of potential. Dr. van Bosse is the very definition of excellence in care. And all my parent friends will tell you, he never gives up.

And I joyfully get air sick to see him.