Here is part one of the two part summary of our daughter’s major surgery in Philadelphia at Shriner’s Hospital for Children. I decided to be thorough on certain details of this trip since I know of a few people going in for the same (or a similar) surgery, and I wish I had known a bit more of the overall process. So since I don’t have the time to sit down and pour my heart out in one blog post, and my kid is finally sleeping, here is part one.

Night before surgery.

Watching Daddy play Mario. (This is how she likes to stand, wedged between the coffee table and couch.)

Laelia rubbing my back while playing peek-a-boo.

We cuddled and stayed up late the night before our trip for those extra hours of family time. We also took that time to once again talk about where we were going and what we were doing. Yet despite that, Laelia happily announced that we were going to Disneyland while she helped me put her clothes in the suitcase. When I corrected her and talked about surgery, she corrected me and says, “Daddy told me.” Well Daddy got in trouble. Then Daddy spent the rest of the time explaining that we weren’t going to Disneyland until much, much later. “Tomorrow?” Laelia asked. *sigh*

Laelia was a good little girl. She hand-picked one stuffed animal to come with us, her doggie, then comforted her doggie on its upcoming surgery. “There there, doggie. It be okay.” She was a good flyer. During the entire day of flying she only had one melt down. When we got on our second flight after a snow-covered Denver connection, she haggled with us, “Last plane today, okay?” Since it was, we agreed to her demands and the melt down ended. We also found out on our lay over that Ronald McDonald house near Shriners would take us for the night, and possibly for the week.

Then Mama had her own melt down after arriving in Philly when we realized we had missed several calls from the hospital. They were all informing us that our surgery time had changed from 6:30 to 10:00 am. Since I had been told 6:30 am, and that had been the plan for months, and I’m not good with last second changes, and it was a different story than I’d heard from the surgeon’s scheduling person, and and and… let’s just say I wasn’t sure it was correct. And of course even though the PACU was suppose to close at 5:30, it was 5:12 and no one was there. I started to cry, standing there in the middle of the airport, calling people at Shriners. I reached a lone admissions desk person who, after apologizing for laughing when she realized I was freaking out, convinced me not to show up at 6:30 just to wait for hours with a hungry toddler. She wasn’t sure on the time, but she was sure the PACU people would have told me the correct time. But I just didn’t want to risk all the time and effort and set up it takes to fly across the country with your daughter then miss a major, important surgery just because I wasn’t willing to wait a few extra hours in a waiting room. I didn’t know what to do.

Thankfully we arrived at Ronald McDonald house on Erie street to a welcoming, calming environment. Instead of doing orientation they suggested we eat dinner first. In the dinning room a soccer team of girls put on a puppet show and dressed up my daughter and another adorable girl, Sabina, like princesses and sang songs. Sabina’s dad, Michael, started talking to us about what our daughters would be doing tomorrow. It turned out he had an early appointment, 6:30 am, with Dr. Van Bosse, our doctor! Well since Dr. vB can’t be at two places at once, I was finally at peace that our appointment was indeed at 10:00 and we’d all get to sleep in. He was at the right place at the right time to calm all my fears.

I guess I should explain a bit about the Ronald McDonald house. They don’t take reservations, so the only stressful thing about them is not getting to know where you’ll be staying until the day of your trip. That said, Ronald McDonald house is like Grandma’s: there is plenty of food, lots of toys, friendly people and you can go through the cupboards and pantries like you own the place. And you’re expected to clean up after yourself before you leave! It’s also a place to can be alone, or you can meet other people in your situation. It is your home away from home, and a lifeline to a lot of families who can’t be with their hospitalized children without paying through the nose for a hotel otherwise.

We had a large room with two beds. But Laelia, in usual Laelia fashion, was being a big turkey and refusing to sleep. So we locked her in the closet! Haha, I’m not kidding! We put a blanket bed in there and when she immediately sat up and worked the door open, we put her back and closed the door on her!  So she had a tiny little bedroom, Harry Potter style! I felt bad about it so as soon as she quieted down (about ten minutes) I opened the door. Then Charley took this picture of her sound asleep. So cute!  That dirty bottom was from scooting around in the airport on those dirty floors. I’m thankful she started the hard day ahead well rested. She woke up cheerful and full of life. I started the long day ahead with dread.

It’s a funny thing. I would think I was handling everything fine and then I would throw up with nothing to blame it on except stress and worry. This happened twice–once Thursday night and again Sunday night–and both times I was surprised by it. Since I mostly felt very peaceful, I mean for the circumstances. In fact, I’ve felt more peace about this surgery than any of the other three, even though this will be the most major. Besides being terrible at internally handling these situations, I do handle them well outwardly. Then I throw up apparently. I truly believe I did better this time because of the number of people praying for us. Sure I was stressed and worried, but I didn’t have to deal with the depression or morbid thoughts that I have before. I felt lots of peace flowing my way. And it was something outside myself. The other several differences between this surgery and the former ones were that I was more prepared for this one, I had more say, I had more questions answered by the doctor (who I have a crush on) and I had more hope. And, people were sending us their love, positive thoughts and praying lots and lots of prayers to God on our daughter’s behalf.

One stresser was transportation. The rental car companies at the airport wanted to charge us $500 for those days we would be in Philly, for their smallest cars! Our budget was $200 total. We tried to do the whole thing with cabs, but we had to go between the hospital and RMD house too often. Then while looking up prices online I stumbled across something a whole lot cheaper, but I had accidentally found something in Philly but outside the airport. But that place closed too early and we’d miss it. So Charley found another place and flat rates for cabs online to get there. So we ended up taking a cab to another rental place, then the rental car to Ronald McDonald house. It was a round-about way to do things, but budget friendly! I suggest it for anyone needing to have a substancial stay in Philly. We used Enterprise in Downtown. (Tell the cab driver their address–36th S 19th Street–or it’s hard to find.) Cab there from airport: $26. 

Our hospital is surrounded by the ghetto. I’ve said this before, but I was hyper aware of it having to make a lot of late-night drives through it. Plus having to deal with the non-stop sirens at night or crazy drivers/pedestrians during the day… bleh. At least this time with Charley with us, we transversed those terrible, confusing signs and streets that make up north Philly, and actually found the Shriners entrance/parking lot this time around!

Shriners’ Hospital’s Waiting Room Vrrrrrroooooooom!

We arrived at Philadelphia at 10:00 am and were still waiting for surgery at 12:45 pm. So glad we didn’t show up at 6:00 am! Every time a person came to check Laelia’s measurements, blood pressure, temperature or simply to take out the trash in the room, Laelia freaked out. She was scared. The people at Shriners are nicer than any other hospital we’ve dealt with. They care. Period. That’s the difference most of the parents I’ve talked with notice first. These people know how to distract a child, talk to them and work quickly through their screams of protest. But despite this skill, Lali was still pretty freaked. When we walked into the PACU and saw the nurse, the first thing Laelia did was cry and shake her head, “No no no no no!” The nurse put a hand on her and said, “I have a very important job for you to do, okay? Are you ready?” Laelia nodded quietly. Then the nurse showed her a wall of bears and asked her to pick out her special surgery friend. Laelia started to relax a little.

Finally they gave her something that left her inebriated. She went from completely rigid to totally relaxed. She kept sliding off my lap even! Then my drunken baby girl began to sing silly songs while slurring her words, and with that reddish hair it really brought out the Irish in her. When the man in scrubs came to pick her up and carry her into surgery, instead of freaking out, Laelia went complacently with him. I watched them walk down the hall as her head swung limply, like a sack of potatoes, in time with his steps.

And she was gone.

My arms, that she’d been in since we walked into this scary place, were unnaturally empty. I was clutching her blanket really hard to remedy that empty feeling. When we stepped into the elevator to head back to our room I caught a glimpse of myself on the elevator’s reflective metal wall and I looked like some melodramatic lifetime movie reject. I made a conscious effort to relax my body and facial features to look, what Charley refers to as, ”not crazy.”  We went to the cafeteria first off and forced down some fried food that sat in our stomachs like rocks all day. Bad move. A nurse came in while we ate to let us know they got the epidural and IV in (a potential issue with arthrogryposis patients). We thanked her for the news, but it became harder to swallow after that. We called family, watched Man Men, hung out with Sabina and her daddy for a bit and paced. Well I paced, while Charley kept trying to grab me as my course sent me within a foot of him.

I called the PACU around 2:40 pm. They had just finished with her first leg and were just about to break her second leg. Waiting after that became harder. I paced some more. The last half hour was the worst. My stomach hurt, I felt sick and every minute felt like it had been too long and something had to have gone wrong. I was on the seventh floor in the waiting room that had large windows looking out over the ghetto. I’m afraid of heights and was going slightly mad. My mind played this trick where I could imagine myself falling as the floor melted away, and then for a second every so often it felt like it was actually happening. I started to think about worst case scenarios. Just as I started to get really morbid and my stomach lurched,  just under five hours after handing my child to a stranger, the doctor we’d traveled across the country to see walked into the room.

The surgey was done and we could see her in about 20 minutes. The sites looked good, but her right leg gave him a bit of trouble. She wasn’t as straight as we’d hoped, but this was definitely a great first step towards standing up straight. But because of her epidural, her recovery would be in the ICU until Thursday. And hopefully we weren’t too set on flying back Friday.

My skin was crawling because I wanted to see her so badly. A woman led us to Lali’s room at a pace much too slow for me. Laelia started crying once she saw us. I looked at her little face, so sad and angry and drugged, and my mommy instincts kicked in with all the comforting I had within me. I blotted at her sweaty face and told her it was all over. She always looks so little after surgery, so tiny. She was hooked up to so many different machines. Her breathing was labored and she sounded really congested because of the liquid in her lungs. She didn’t talk or smile–so unlike her. Then I looked behind me to see my husband, my rock, completely lose it–big tears falling down his cheeks. He excused himself from the room and sobbed.

It’s days like this that I realize all over again that my baby girl has arthrogryposis, type amyoplasia, that she did nothing to deserve, and what that means for her life–a life that is a total blessing to me.

We are leaving in three days to fly all day to Philadelphia. Then on Tuesday, November 16th at around 6:30 AM, Laelia will have her most major surgery yet.

The surgery is five words long: bilateral (both sides) proximal (nearer to the body) femoral (thigh bone) reorientation (change position) osteotomies (bone cuts).

What they do is cut all the way through the bones extremely close to the hips, removing them and then clamping and pinning those bones in a slightly different location facing downward. Since Lali has joint contractures (which is when the joints harden and don’t move) in her hips, and doing surgery on the joints themselves will cause her to be immobile, we are hoping to move her bones around her contractures so she can one day stand. We don’t yet know if she has the muscle strength to stand or walk (because of the muscle weakness of amyoplasia), but she will never be able to even have the chance without this surgery. Right now her legs are externally rotated so that her knees turn out and she can’t bring her legs together. With this surgery the thigh bones will still be facing outwards, leaving her legs externally rotated, but the bone will be positioned downward so she will, for the first time in her life, bring both her legs together.

Think of a ballerina doing first position. We also call this surgery the sitting-like-a-lady surgery since right now she sits with legs wide apart.

The surgery will take many hours and require weeks of recovery as well as six weeks in a lower body cast connected by a bar at her feet.

A year from now they will do another surgery (in Philadelphia) to remove the pins and clamps that will be inserted internally into her body and at that time they will be doing another major surgery on her knees and left foot as well.

I’m having a bit of stress today as we are still trying to get a rental car worked out, we don’t know if Ronald McDonald house will take us (we don’t find out until the day of) and we’re not packed. Also if any troubles come up during surgery and we’re unable to fly back on time then that becomes another expense and headache to work out. And sickness is going around town, Charley is on antibiotics, and we can’t risk Laelia getting sick right now.

Some wonderful friends are dropping by Sunday to help me clean so we can bring Little Miss Post Op home to a clean environment. We’re looking at some hard weeks ahead. We’d appreciate everyone’s positive thoughts and prayers for success as we proceed. Thanks.

When my husband mentioned to a friend that Laelia’s surgery was next month, November 16th, Laelia exclaimed, “I gonna have surgery!”  Which she pronounces more like, “ser-gy.”

This led to a big conversation about surgery.

She then said, a little less excited, “I gonna be sad on my sergy.” I wondered if that was a question, even though it was presented as a statement. I asked if she knew it was going to hurt. She parrotted my words back to me, “Is sergy gonna hurt?”  I said it was, but that it would be temporary. She began to get upset and cried, “I don’t want sergy!”  She then scooted pretty fast into my arms. I held her and explained what the surgery would do and where it would be. When I touched her legs to show her where it would be, she flinched. Poor frightened little thing. I wish we were having this talk the day before surgery instead of a month before so it would be over with quicker. But to spare her I demonstrated on my own leg what they would do in surgery. I drew a line with my finger over my hip and down my thigh a little ways. She was wide-eyed.

I asked her to bring her knees together and she did a good job. Then I explained that after surgery she would be able to bring them together all the way. “Like this?!” she brightened and swung her body back and forth on the carpet pushing her legs closer. “Even closer! Surgery is important to help you do that.”

“My surgery will hurt me.” Now I knew these statements were questions since her eyes were pleading with me. I could almost follow her little mind as it processed how something good could hurt her. But I assured her that she would be asleep when they did it and feel nothing. And I would be with her.

Then we did pretend surgery. I put my cupped hand over her mouth and she breathed deeply and then went to pretend sleep, snoring loudly. Then I said, “Okay surgery is over now, time to wake up!”

She blinked at me and grinned, “I awake!” then added more sadly, “Now my sergy hurt me.”  I told her she would still be sleepy and not feel much, but then much later she would feel sore. “I no want my surgery.” She said.

Then I asked her if she’d ever had surgery before. She hesitated and said, “Yeah.” Another question! She wasn’t sure! How did I not get before that my little girl wasn’t sure how to ask questions about this. So we pulled up her pants leg and I showed her the scars. I asked her if they still hurt. “Nooooooo,” she said mocking. Did the scars on her feet still hurt? Her thighs farther up? Nope. How about on the other leg. Nope. Well this next surgery wouldn’t hurt after a while too. She was just too little to remember all the surgeries she’s had.

She still looked uncertain so I decided to show her my surgery scar that stretched across my lower abdomen. I explained it was a surgery to help Lali get out of my tummy safely.  I said it hurt for a few days afterwards, but it wasn’t as bad after that. Then I punched my stomach to show it didn’t hurt anymore. Then we talked about Chelsea’s foot surgery and looked online at two kids who had had the same surgery Laelia will have. One of those kids is standing unassisted now.

“I can stand like this,” Laelia boasted as she showed off her one knee balancing act. I asked if she would like to stand on both legs and she said in a quiet voice, “yes.”

But… And there was a “but” coming.

“Mama, I no like surgery.”

Me either, little girl. Me either.

Come get us!

Not really helping…

We asked her to go backwards… Use your legs, child! Not your head! Haha!

Ram into Adam! Good girl!

We are very thankful that we get to semi-permanently borrow a walker for free practice with it at home! From what I understand the company who loaned it to us are hoping we get our power wheelchair through them and make them lots of money.   And if we can only get one big piece of hardware with the help of insurance, they want it to be the expensive power chair and not the semi-expensive walker. So it’s in their best interest to let us borrow a walker for as long as we need it. Cool, eh?

Best of all, our friends have helped encourage Lali to use it even though it’s hard! Lali saw Grandma Deb and Grandpa Gary on Friday and had some walker fun on their back porch. Chelsea offered to lug it with her when she watched Lali that morning. Then we went to a double birthday party on Saturday and Lali got to use it around all of the party guests who cheered her on.

I think we’ve figured out the perfect walker combination: hand grips facing up, one knee immobilizer on her right leg (since it has the worse contracture), the left side of her seat hitched up (where her hip has the worse contracture), the back wheels locked so they can’t turn, but so they can go backwards, the front wheels able to turn all directions and a nice level surface that’s not carpet. Even still she moves very slowly if at all. Still, it’s neat to see her upright!

While we were at PT and OT we met our friends: Rosa and her mom, Leslie! So how do we “meet” friends? Well we became friends online, but had never met in person before! (Um, I have a LOT of these kinds of friends.) Here is a cute picture of Laelia and Rosa. Rosa has arthrogryposis too. For whatever reason it’s the only picture that came out. They were giving each other hugs and kisses. So cute! Lali wouldn’t let go of her PT slip that she has to hand to her therapist so it was hard to give hugs.

We also signed some hospital paperwork saying that Children’s Hospital can use Laelia’s pictures for anything and everything. I can’t help it if they think my daughter is super beautiful! I guess they end up using her pictures a lot. Apparently they have a good one of her at four months old in splints that they used at some official function and needed to make sure I was still cool with that. Laelia’s pictures also go with our OT who is a hand specialist to her lectures in other parts of the country.  So in exchange for Lali’s picture, I got a couple pictures of Lali and Jill (our OT) doing therapy.

Therapy is fun!… You know, for Lali too.

So all in all a good day at Children’s Hospital. An aide helped me into my car with the walker. The darn thing doesn’t fit in the trunk so we had to load it into the front passenger seat. I drove home with one of its front wheels in my face the whole time. I managed to get all the way into my apartment complex parking lot before bursting into tears. Thankfully Lali was sound asleep after such an eventful day.

I don’t know why I was so upset to the point of balling. I can’t tell you if it was gratefulness at getting to use a walker or if it was pain at the reality that my daughter needs a walker and looks ”more disabled” with it. Or maybe it was a combination of both. I figured I’d get all my tears out in the parking lot instead of bombarding Charley with them, especially when he’d likely ask what was wrong and I DIDN’T KNOW, but my car was not as private as I thought. The neighbors, a family of three, caught me.  The dad helped me carry the walker inside while I carried my sleeping beauty.  They didn’t mention the fact that I had a walker (even though I don’t know for sure that they know about Laelia’s condition), and they didn’t ask questions. They just smiled and helped me carry stuff through our shared gate. For some reason that was comforting. It made having the walker less awkward. It helped my mind to make the connection that the walker is a good thing–a good, helpful thing–and an everyday thing. It helped me feel normal. I think for someone to achieve that act of magic–making me of all people feel normal–then God must have been helping to quell these emotions before I could even put a name to them.

Very thankful.

We survived! Four hours of sleep. Four planes in two days. Air sickness. CONSTANT Laelia chatter/attention grubbing! Five partial viewings of Finding Nemo. Sore arms that ache. Bruises from carrying the car seat. Ripped our bag open. Got lost three times. Stress. Got lectured by a flight attendant because my daughter was sitting instead of standing. Cried twice. Had to advocate several times. It was hellish. Travel. Ugh.

And there was a point, after getting lost (again!) while driving around Philly, then being two hours late because we went to Temple Hospital instead of Shriners Hospital, when I wished the doctors at Shriners would be mediocre so I’d never have to do this trip again. Never again!!!

Unfortunately for me and fortunately for Laelia, Dr. van Bosse is now our orthopedic doctor!

And we’ll be back to Philly three times in the next 14 months if things play out right. Yes, that means surgery. I didn’t schedule it yet since I need to plan the time off work and to talk it out with Charley, but it most likely will happen as soon as we’re available. ASAP. Probably after this summer. I’ve already met with the anesthesiologist, Dave, and gotten the details. Now it’s a matter of logistics, and processing.

And since we live so far away, they’re going to treat us like an international patient (since people with AMC come here from all over the world), and try to limit our trips back and forth. Maybe we’ll do a few follow up appointments in San Diego then forward the results to Philly. They’ll also be in contact via email and phone.

We’re not doing the derotational osteotomies on her knees we’d heard about in Seattle, not yet anyway.  We’re doing a surgery that makes much more sense for right now (a whole methodology that makes much more sense), that will allow Laelia to put her legs together where the IT band releases (surgeries from September and October) could not.

So far our surgeries have focused on soft tissue issues.  But Dr. van Bosse is going to focus on her overall body shape and do a surgery to cut and reposition the bones in her hips to fit what her contractured body is already doing. And the result will allow her to do more and look more like a typical kid. (Notice how I didn’t say ‘normal’ kid? See, I’m learning. ). It’s called a reorientation. (It’s also called a big, long four-part name that my sleep-deprived brain could not retain.) We’ll get everything in writing first then contact them to set up the appointment. It puts us in Philly about 9 days, then flying home, then flying back six weeks later for the cast removal (and they may give us KAFOs at this point since they’ll be casting her for them before surgery). Then flying home. Then flying back in a year to get pins, clamps, etc, surgically removed and start a new surgery on her foot, etc etc. Our future may be tied to Philly for quiet some time.

Since her hips are in the sockets, but they’re externally rotated and contractured, this would be the best surgery for her. But I didn’t have to just take Dr. van Bosse’s word for it, as so many doctors expect, but instead he pulled up a few other patient files (children with arthrogryposis who had been through this surgery) and he showed me their progress. We had taken Laelia’s x-rays while we were there. (She’s less scared of the machine if she can be a ham. “Smile for your picture, Lali!” did the trick. ) This allowed us to compare her x-rays with other kids with arthrogryposis until the doctor found one with the same shaped hips and legs in their x-rays. Then I could see that child’s x-rays after they had had this surgery and know exactly what to expect Laelia’s surgery to look like. It was exciting.

Dr. van Bosse wasn’t just showing me what he wanted to do, he was showing me what had worked for other kids! An amazing experience! A new experience.

I have met with doctors who didn’t even know what arthrogryposis was, or had trouble pronouncing it, and some of them even gave us advice on surgery or therapy. Even our expert surgeon in San Diego who does have experience with arthrogryposis seemed to pale in comparison with Dr. van Bosse’s experience. It felt like we’d arrived at arthrogryposis mecca!

We also met with Dr. Kozin. It’s fun to get looks of wonder from your doctor instead of horror that your child has so many degrees of passive range in her elbows. Doctors who are familiar with arthrogryposis know Laelia is lucky in this regard. Where other people would bemoan the fact she can’t lift her arms (including me at times), these guys are in awe of the fact that she can reach her mouth and doesn’t need surgery! Oh and Laelia had not known Dr. Kozin more than 30 seconds before falling in love.

I have several more pictures like this. If Dr. Kozin was the cuddly doctor, Dr. van Bosse was the playful doctor. Lali just giggled as he measured every joint, instead of fussing like she usually does.  She seemed to like everybody. Ooh she liked Mimi too!

Mimi too!

Her “airplane hair” afro for the trip. You can’t see it, but on the other side I stuck her comb in her hair and it stayed.

So glad they put these racing cars in every room! Vaaarooooom!

Laelia was such a good little girlie for this trip! I was specifically worried about this. I mean, sure, she was demanding, but mostly in a sweet way. She doesn’t sleep on airplanes so she had to be constantly entertained (constantly) on all four flights. I felt like Super Hero Mommy by the fourth flight. Well she did finally pass out on the last flight home at around 11:00 PM. And she fell asleep for almost 40 minutes in between flights, including sleeping through a shuttle ride.

Another huge blessing was that we got to stay at the Erie Ronald McDonald house!!! I called as soon as our plane landed in Houston (the day of) and got the okay! (They don’t make reservations so you call the day of.) That cut out having to search for a hotel or make other plans last minute. It was an amazing house! One of the roads on my Google directions was closed so it took an extra 45 minutes to find it (d’oh), but once we got in there they were so nice and friendly. I had just been on a plane with a mean person who made us feel terrible that Laelia couldn’t stand or walk. Long story. So when I put Lali on the floor to scoot around and saw a Ronald McDonald volunteer approach her, I inwardly tensed. But they just wanted to play! The volunteers were all amazing! There was always food around even though we never made it to a single meal on time and there were plenty of relaxing areas to unwind. Laelia loved the therapy dog, Boss, and they gave her a free toy doggy that she adores too. We couldn’t enjoy all they had to offer since our schedule was so tight, but I really hope they are available to house us again when we go back for a little over a week.

Only bad thing about Ronald McDonald house is that it had this magical ability to keep Lali up all night. She slept five hours. I got four. We tried her on the floor where she screamed bloody murder for twenty minutes. Then we tried her in my bed with two chairs with large backs against the side of the bed so she wouldn’t fall off. She played, she sang, she screamed. It was enough to drive me crazy. She even removed her splints, DB bar brace and was starting on her AFOs before I stopped her. She started hitting me in the face at one point. And kicking the bed over and over and over. Then she got really upset that Pooh Bear didn’t close his eyes to sleep. She kept trying to close them and telling him to go to sleep already! Haha! Every time Laelia gets in trouble, it’s not long before Pooh Bear is in the SAME trouble! At Tim and Nicole’s wedding last week, Laelia lectured Pooh Bear about keeping quiet during the ceremony. SO STINKIN CUTE!

So when I woke her up at 8:00 AM to leave, she was so out of it. She gave me the same look she had given me the day before when I woke her up for an early flight. That “Are you kidding me?” look.

Exact words, “No Mama. I asleep. Bed pweese.”

I mentioned I got lost… yeah, just about every time I got behind the wheel.  Maybe it was the lack of sleep or the noisy kid in the back seat, but I’m pretty sure Philly is just a ridiculous place to drive in. Not only that, but I got lost in a not-so-great part of town. It’s funny because this great hospital is surrounded by ghetto neighborhoods. And if the jay walking, lack of clothes, yelling, kids drinking of a broken fire hydrant or constant sirens weren’t clue enough, we were warned it was “ghetto” by hospital staff, other people who have been there, the car rental guy and several patients. So getting lost at night down one way streets was not fun.

Okay so I’ve hinted that we had a bad experience with yet another airline person. But after four flights in two days it could have been worse. We got our tickets for Continental flights through Mercy Medical Airlift, and appreciated it so much! That said, there were a few things I would have done differently if I had been booking my own flight. Not having to get up super early or get home super late would have been one thing. Getting window seats for the car seat would have been another thing. Getting wheelchair or special assistance set up before-hand would have been a third thing. But Continental employees were helpful. When we first arrived I had two of them cooing over my kid. Always the quickest way to my heart. Then we got wheelchair assistance, and knowing how to request it now, had it properly set up for our connecting flight in Houston. They even gave us a ride to our connecting gate, which would have been difficult to make otherwise. All in all they were very accommodating and I don’t know how I would have lugged everything I had to lug without them.

My only two complaints have to do with arbitrary rules and one flight attendant’s enforcement of these rules.

Seriously having a personality that HATES breaking rules or getting lectured on breaking rules AND doing four different flights in two days gives me perspective on how arbitrary these rules are. Flight #1 required we put a car seat in a wheel chair and have an employee wheel her down the ramp backwards. I couldn’t touch her until we got to the plane’s door. Flight #2 was similar, but they insisted I check the car seat, which didn’t end up happening, more on that later. Flight #3 I was informed that under no circumstances could she go down in a wheelchair. It was unsafe and against airline policy! Seriously? I’d just been on two flights with them yesterday and the story was different. Flight #4 they made a big deal about it. I was given options. Did I want her to go in a chair? Or be carried? And how could they help? (I felt like they were saying, “Why are you putting us in this situation?”) I just wanted to say, “Just tell me the exact policy and stick to it! I’ll follow whatever the rule is!”

Not just entering and exiting the plane, but on the plane the rules tended to change. It wasn’t until flight #3 that we were told our portable DVD player was against the rules. It had to have head phones. Flight #2 all the flight attendant said was, “What movie is that?” And Laelia said, “NEMO!!!” And that led to a couple minutes of Laelia explaining who every character in the movie was. But one flight later it’s against the rules. Okay…

Oh and placing the car seat was a pain. We had a window seat on Flight #1, but on the rest of the flights we had an aisle seat for the car seat. On Southwest, the week before, we were told putting a car seat in an aisle seat was against FAA rules and that this applied to every airline. But here it was okay I guess. It all depends on the day, and the whim of those enforcing whatever rules.

Okay so back to Flight #2 when the incident happened. They demanded I check the car seat. Since Laelia can’t sit in a regular seat and WILL NOT keep her seat belt on for any length of time, which leads to battles the whole flight, I had to insist that I didn’t lug this thing around for fun’s sake. The flight attendant then wouldn’t let me board for several minutes while I stood at the front of the plane and she inspected my car seat while grumbling loudly. I must have heard, “You need to check this,” several times. Telling her I’d been on five flights with it in the last two weeks, one just a few minutes ago, and it was just fine, only led her to speaking to me in a less friendly way. A few long minutes later they found out the seat was approved (which is what I’d said all along) and let me on. It was at this point that I took the opportunity to explain why Lali needed the car seat, and I informed them that Laelia had disabilities, and without working arms to break her fall, turbulence could knock her around in a big seat. The only reason we have to buy a seat for her is because of airline rules. The only reason I bring a car seat is for her personal safety. I was apparently ignored.

Later on in the flight the seat belt light went off and I was able to get up with my kiddo. I knew I wouldn’t be able to do her daily stretches here, let alone some PT, but I did need to get her joints moving somehow. I went to the back of the plane by the restrooms after I’d seen two other parents of small children take them back there. I put Laelia down on her bottom and she stretched out a bit. This is when the incident happened.

A lady who was not nice to begin with, let’s call her Cindy because that’s her name, lectured me on how dirty the floor was. I’ve heard this from so many “concerned” people that I was tempted to ignore her entirely, but I did respond that I didn’t mind about the dirty floor. It’s funny how people don’t care about the bottoms of kids’ shoes getting dirty, but the bottom of their pants is a different story. But, no, that wasn’t good enough. She insisted that I should immediately get my child off that dirty floor. And that being there was “terrible.” I had seen other children stand just where we were and they were not told about how filthy it was, so I then restated that I knew it was dirty, but it was fine for her to be dirty. She then ordered me (not joking) to get my child off the floor right now! I got my advocate hat on and asked if this was a written policy (since I hate breaking even stupid rules, it’s against my personality) and she said it was! No being disabled on the plane, folks! I asked for her name, intending to introduce her to Laelia and get Laelia involved in the discussion. I’d heard that works really well and is a nice way of informing people without ostracizing Laelia. But I got so much fire back from that lady that it sent me to tears and I found my voice, along with my advocating powers, diminish under her anger. In fact I was so distracted by the way she spoke to me that I didn’t notice a guy behind me who needed by, which sent Cindy into a rant about how my child was blocking the aisle! I moved, Laelia didn’t, and he got by fine. At this point Laelia started to fuss, which led to tears. Cindy ordered me in a nasty tone of voice to pick up that child right now! I pulled her in my arms and she screamed, “NO NO I STRECHING! MAMA NO!” How terrible for a girl with contractured joints! I put her in the bathroom stall to get away from Cindy and sat there stunned until the fasten seat belt sign came on.  Not being able to stay in my shelter after that, I moved back to my seat, but on the way I tearfully explained why we were even back there to the fellow employee who had witnessed the whole thing. He apologized for Cindy, said she was wrong, but he seemed slightly afraid of his lead flight attendant. We took our seats and tears wouldn’t stop, but I hid them well. I heard Cindy complaining about us to the guy we’d just spoken to and he told her about Laelia being disabled and not able to stand up, which sent her into a rant about how right she was despite the circumstances. We were in row 34 of 37 so I could hear her in the back. Not every word, but I heard her. I was trying to gather my thoughts. Were we really just discriminated against for having special needs, because we couldn’t stand like the other children? Or was there actually a rule we broke? That kids who don’t have disabilities don’t break? And if so, is that a good rule to have, Continental? As I thought about it, I could hear her still going off behind me. Her tone of voice was like a knife to my insides, from three rows away.

If she indeed had been working for Continental as long as she says and was a lead flight attendant, what kind of training had she not been receiving for those many years?

Thankfully, and I could have kissed her, Cindy showed back up at my seat to tell me off some more! (Or maybe to apologize? But you can’t apology if you’re “not wrong” so it came across badly.) Yay!!! Now the tears had cleared, my thoughts had cleared, and we were both well aware of her discrimination against my daughter. Mama Bear was back in action! I said things like, “I appreciate being warned about unsanitary conditions, but I do not appreciate being ordered to pick up a child when other children were allowed in the same area under the same conditions!” She tried to tell me that the seat belt sign was on during this, and I interrupted and informed her that that wasn’t the case, and it only came on after we entered the restroom. And, after hearing in the pseudo-sweet voice that I should really ask to be seated in row 7 next time, (grrrrrrrrrr), because they can put down a blanket or something for her, I responded, “Look, I am the expert in my child’s care. And we don’t appreciate being treated this way.” BOOYAH! Thank you thank you thank you so much for coming back and not letting this end with me in tears and my daughter undefended! Finally Cindy and I could not come to an understanding and she left. (In other words, we were both “right” in our own eyes.)

So okay that was hard. I was already so stressed about what we’d learn the next morning, and part of me forgets my kid is disabled until she’s treated differently than other children, like she was on that flight. But nothing could be funnier than when for Flight #3, the very next day, I get on the plane and who do I see? Cindy! Again! She was as surprised as I was, “Wow, you again. That was a fast trip.” She said. I responded, “Yep.” Then Cindy jumps over herself to get my bag, ask if I’d gotten to switch to row seven for this trip (no) and snaps at fellow co-workers to help Laelia and me. Hahahahaha! I texted Charley, “Guess who our flight attendant is again?” And he texted back, “NOOOOOOOOOOOOOOOOOOOOOOO!”

At one point in the flight, Laelia said “hi” to Cindy and Cindy was like, “Does she want something? Water? Can I get her water? Does she need something?” Wow, I think she had a day to dwell on our experience and found herself in the wrong. Or at least very much in trouble if I wrote a letter… which I am *that* person who rights letters, so she’s rightfully freaked. I asked Laelia if she wanted anything and she says, “SOCKLET!” (Chocolate) So Cindy came back with Hersey bars for Laelia who gobbled them all up! Spoiled kid!

I really have to consider my own actions and reactions in these situations. I feel like I’m getting stronger as a mother, but I’m still awkward in these situations. How terrible it would have felt to have ended with me crying in the bathroom? I know I don’t like authority much because I’m a pleaser and authority can take advantage of that, but I do want what I want, and I want to do what I think is best for my child. Of course I don’t want to push or bully, and often have difficulty finding a balance between nice and pushover. But in this case I believe we were 100% in the right. Which is a position I’m most comfortable with. And I’m so oo oo writing a letter.

There were lots of times I felt discouraged on this trip and was almost immediately reminded that people were there for me. At Flight #3 when all the rules for entering the plane changed (overnight, I might add) I got a text from Lauren saying she was praying for us RIGHT NOW. I also got a couple similar texts from Chelsea at just the right times. And my dad even called and left a voice mail message that encouraged me as I was absorbing the news that we’d be back for another major surgery or two.

All in all I felt this strength and peace that isn’t something I naturally carry around with me. I felt God’s presence during these really  hard times. I’d be in a situation I couldn’t handle, praying for help, and nothing around me would change, but I would change. I’d feel the strength to read Laelia yet another story. And for those of you who get motion sickness (or what I call air sickness), you know how awful reading can be during those times. But we did it. And Laelia doesn’t seem worse for wear. I’m exceedingly thankful to God for his provisions when I was all alone and responsible for my daughter’s care.

Okay I blogged about it. And once again informed my spell check that “Laelia” and “Arthrogryposis” are words. Now I’m adding “Kozin” and “van Bosse” to the list. *sigh* Now I’m going back to bed.

WE HAVE FLIGHTS TO PHILLY!!!!!!!! THANK YOU MERCY MEDICAL AIRLIFT!!!

We’ll be taking off for the airport at 4:30 in the morning on the 17th and arriving home from our trip close to midnight on the 18th.  Now I just have to figure out how to entertain a bad, cranky flyer for two straight days of flying across country.  And maybe entertain Laelia too.

Today I’m working out a place to stay–a much easier task now that we’re certain we’re going.  And I have to figure out how little me is going to lug around a baby who doesn’t walk (or stand so you can’t always put her down), our giant book of medical records, our personal stuff and a car seat! We’ll probably need a rental car.  And we (and by “we” I mean YOU CHARLEY ) still need to fill out all that paperwork that Shriners mailed us.  And we’ll maybe borrow a portable DVD player and Elmo DVDs. And pack. And write out questions. And and and…  And I hope you’re reading the excitement in my voice/words at the prospect of these tasks. It’s an exciting time!

Well, exciting and scary. Now we see what the expert says. This is our last hope in a lot of ways. Our Obi Wan. I’m nervous about it, and burying myself in the planning stages lets me avoid thinking about it.  I start to wonder if those other doctors are right–that Laelia is the worst of the worst when it comes to her muscle weakness and won’t be able to move around much on her own.  :-/

I don’t *need* an ambulatory daughter (despite what Disneyland rides that limits her to), but I do *need* hope that maybe one day she’ll be ambulatory. If that makes any sense. I think I’m okay now with Laelia’s disabilities because she’s still young, and I’m nowhere near done fighting for her. I guess I just pray that I don’t come away from this exhausting two days a depressed mess.

And it’s funny to actually be hoping for giant, major surgeries across the country. Ones that don’t come with a 100% guarantee.  If it was me I’d want to be made straight and take that chance, however slim, at walking. But it’s not me; it’s someone much more precious. And there’s a risk that, because of the  severity of the joint contractures in her hips and knees, we end up surgically getting her in a position to stand (because she physically can’t do it now) then find out she won’t be walking or standing because of muscle loss. (And surgeries also diminish her muscle strength!) So you can imagine how heartbreaking it would be to go forward in hope, then end up with a straight, scarred, non-ambulatory baby girl who now has the added disadvantage of being stuck in a “bad” position for comfortable wheelchair sitting.   Because with arthrogryposis you are stuck in one position with limited range of motion, like a flimsy cloth doll with joints made of metal. So you pick: standing or sitting, legs bent or legs straight. Then you cut and solder back together as necessary and hope it stands up under it’s own weight.

So what to pick?  Dear God, help!

I think we’ll mostly rely on Dr. van Bosse’s extensive experience with arthrogryposis cases.  We only get something like 45 minutes with him, so I pray he sees what he needs to see and has the wisdom to know what to do. And if we get an answer that depresses me, I’ll trust that to be our best advice for now and determine to have peace about it.

Or forget Obi Wan, I’ll go find Luke.

[Want to see some videos of my daughter doing amazing new things? Scroll down to the bottom of this post! But if you want to build up the suspense, which is way more fun, please continue reading!]

For a quick update on our Philly plans, we submitted our application to Shriners yesterday morning. Now we wait a month to see if we’re accepted. Her doctor will be Dr. van Bosse. He’s emailed me a couple times and seems optimistic. In fact, to quote him, “I’m certainly interested. Am in Peru at the moment [...]. One important point that experience has taught me, even at 4 years old, it can be difficult to figure out who can walk. These kids keep surprising me! Don’t give up hope easily.”

Then after I gave him links to her website with pictures he wrote, “I’m very encouraged by what is see in the pictures [...]. I certainly see every reason for working towards maximizing her potential.”

!!!

He continues, “[...]  Unfortunately, Laelia’s treatment will need some planning of the stages, therefore I would have to see her first, independent of any surgery dates, but we will try to limit your back and forth travels as much as possible.”

So now I’m saving up for yet another long trip with my kiddo, and hoping for a side order of derotational osteotomies. We’re thinking that Laelia and I will just take a couple days off work/school for a long weekend. We’re trying to decide if we should do it now or wait the six months other doctors are recommending. Lots to think about. I guess it will mostly depend on when we’re accepted and what we can afford. I’m just hoping Dr. van Bosse is as encouraging and optimistic in person as he is over email! And from all accounts and testimonials, he is! 

We had an appointment with Laelia’s pediatric orthopedic surgeon this morning to go over what the Seattle doctor talked to us about. In the waiting room Laelia said, “Look a baby!”  The only other kid in the room looked to be four so we asked where the baby was. She responded, “The baby’s over there in the picture.” Whoa. Full sentence, clearly stated and masterfully communicated!  Sure enough, behind us was a blown up and framed picture of the doctor we were about to see holding and examining a baby. We hadn’t really noticed it. My kid is SO FREAKIN SMART!!!

At the time of the appointment we had to admit that Laelia couldn’t sit up by herself, even with assistance, or do a lot of other things. This was also written in the Seattle doctor’s letter to me. So I had no idea what what coming later this same day!! (Oh but getting ahead of myself.)

Once again it seemed the doctor had one foot out the door from the moment he entered our room. After the initial greeting and exam, I turned to grab my list of questions off the table and when I turned back I noticed that most of the doctor’s back was to me and he was inching towards the door. That automatically stressed me out and would usually lead to me skipping questions or lumping them together. But this time I just calmly and defiantly wasted his time. We walked out with FOUR prescriptions: wheelchair, knee immobilizers, pool therapy and hippo therapy. Then my husband added, “See if you can get a prescription for a pony!” So when the wonderful surgeon’s fellow returned with more prescription paper I added, “Also Laelia would like a pony.” He responded, “So do I!”  Haha!  Now where can I find a pharmacy willing to fulfill that order?

I don’t know why these appointments get me so down. The surgeon is always cracking some joke and then lightly scolding me for having no sense of humor. In fact it’s kind of formulaic: he cracks the joke, I put on a small smile, he puts his hand on my shoulder then looks around the room as if to say, “Everybody else thought that was funny, right?” then the other doctors/fellows smile and nod, then I get some small lecture on how I need to relax.

He doesn’t get it.

But at least he’s skilled at what he does get. I mean her feet look great after he got to them. Not perfect, but excellently fixed. (Plantigrade is the word.) I think part of the reason I feel so down after these appointments is that I’m being faced with the harsh realities of her disabilities. Day-to-day life doesn’t bring that up. In fact both her father and I forget she’s disabled most of the time until certain events bring it up. In lots of ways she’s the typical happy-go-lucky kid. Then we go to this kind of doctor’s appointment and realize she’s not so lucky. And often we’re given a rough pessimistic guess of how she’ll be in the future from a doctor who probably couldn’t recognize her face in a picture line up the next day. (Well that probably isn’t true. They do know who she is. In fact one of the doctor’s fellows rode with us in the elevator and out of nowhere she said, “Oh this is Laelia!” I didn’t really recognize her right away (we’ve seen this doctor only a few times), but she was one of the doctors who observes Lali’s appointments. So because she’s such a rare case, maybe Lali is popular after all. :)) 

I felt pretty down afterwards. I found myself praying she would prove them all wrong in the six months they’ve given her to magically build enough muscle so they can operate. Dear Lord please!

But I haven’t gotten to the amazing part yet!

An orthopedic wedge costs around $250 depending on what you get. I found a 30 degree orthopedic bed wedge for adults (different use, same idea) for $60 so I ordered it. The Seattle PT recommended a 45 degree wedge, but I just couldn’t find one that was cheaper than $200 so I went with the 30 degrees which is much more difficult to use. The Seattle PT expressed some doubt that she could sit up with just 30 degrees, but Lali doesn’t need to do a straight sit up; she can use her arms.

Laelia sat up by herself (using her wedge) for the first time I’ve ever seen! And the best part: I got it on camera!!!

Laelia also can now do a push up and lift her pelvis so that her weight is more on one knee!!!

So needless to say I’m no longer feeling down from my morning appointment. I’m blessed! I’m thrilled! I’m drowning in answered prayer! I’m singing songs and dancing! I realize I’ve been trying to ”fix things” and I’ve had to let go and realize I can’t fix this. I can do exercises that help her, sure, but I can’t make her not hypotonic anymore.  But my God is so big, so strong and so mighty, there’s nothing my God cannot do!

(Since this got long, I went ahead and put all the pertinent information in bold so you less-hardcore Lali fans could skim through this much easier.)

I don’t really know where to begin. We got back last night from our Washington trip with Kiersten and Ryan to Seattle Children’s Hospital. We haven’t seen Ryan since he was itty bitty, so it was really neat to see him and Lali be bad influences on each other. You know those seagulls from Finding Nemo who say, “Mine mine mine!” Well these two do that way better… constantly!

From the very first, they were being little monkeys in the back seat. It’s so neat to see them play little copy cat games together. Usually little kids leave Lali behind with copy cat games since she can’t lift her arms, but Ryan and Lali can do the same things. And they are so goofy! And this is another picture of them playing together throwing blocks off the bed while saying “Hi-ya!”.

And here’s another picture of dinner that first night after long plane rides.

Hehe. Aren’t they precious? Seattle has some GREAT restaurants and I admit it’s been hard to going back to eating like a real person again. And on that happy note, I guess I’ll start with the good stuff.

~

Dr. Hall

We met with Dr. Hall and (drum roll) Laelia clearly has amyoplasia. I even got to brown nose a bit and mentioned all the side effects of amyoplasia that I’d read about in Dr. Hall’s book that Laelia clearly has (her hair whorl and underdeveloped labia). Then she showed me some things I’d missed from Laelia’s newborn pictures (a bone protrusion in her forehead hidden under her bright stork bite, and her face was asymmetrical for a while after birth). So even though getting that diagnosis should be devastating, in our case it was just confirming what we already knew.  The whole exam took around 40 minutes. Dr. Hall studied pictures of Laelia, then looked her over and also got her genetic history–it was funny when I was giving her my family history because she got every family connection, she even corrected me once when I got confused in my notes. She had practically summed up by whole large, crazy family tree using a single sheet of white paper. Hmmm, I think someone should be a geneticist when she grows up.

As far as myself or ANYONE in my gene pool (hello family!) having another kid with this condition, the odds are 10,000 to 1!!! This means that in the future I’m as guaranteed to have a normal, healthy pregnancy as the next normal, healthy person. The odds of me having two kids with this would be like winning the lottery twice with the same numbers. In fact Dr. Hall (who was completely personable) seemed to be encouraging it:”These kids do well with siblings!” “I’ve overseen over 2000 families with amyoplasia have other unaffected kids!” “You are clear to get pregnant.” So okay then. Um, not so fast now. Hey! :)

Then she said seriously, “You need to tell your family that this wasn’t your fault. This has no known cause. You didn’t do anything wrong. And you can tell them that the expert said so.”  So since I said I’d put that out there, there it is. But don’t worry, family, I defended your awesomeness.  I guess lots of her patients have had families or friends who were too embarrassed to ask the mom if she did something. And really I have felt guilty and wondered if something I did caused this. I got to run several things by Dr. Hall that I thought were awful and could have caused it: I didn’t always take my vitamins because I was sick, I never exercised, I had three drinks before I knew I was pregnant although it was on three different days and I was never drunk… oh and I drove over bad, bumpy roads to get home! Surely one of those things caused this? Nope.  All my parts work and should have produced a typical baby girl, but instead I got an extraordinary baby girl.  

A small percentage of amyoplasia cases show evidence of a vascular accident (something that was mentioned the day after Lali was born), but Laelia doesn’t show any evidence of that. (That would be like the tip of a finger being dead.) It can’t be a virus because they’ve seen it in one twin and not the other. The only other thing they think causes it is somehow the anterior horn cells (for muscle) never develop. No reason why. When the researcher, who was sitting quietly in the background, mentioned that they were still trying to figure out what caused this, Dr. Hall said, “And you won’t find it!”  Then they grinned at each other. (A bet maybe?) But there you have it. Totally random!

The only bad thing from Dr. Hall’s visit was that she let me know that Laelia would get arthritis in her 20s. I knew arthritis was in her future, but was not aware it was this early. I know people with arthritis, and I’m sad she’ll have to suffer with it for so long.

~

Children’s Hospital

It’s an amazing place. It’s set up like a giant aquarium. There are fish everywhere and Laelia LOVES fish! And since Kiersten brought Finding Nemo for the kids, Lali could point out all the “Nemos” in the tanks the next day.

I decided to buy Laelia an Elmo balloon after she grabbed a tiny Elmo doll from a low table display in the gift shop and it turned out to be $16.95. (No, it was not made of gold. Nice guess.) So $3 Elmo balloon it was! She carried that thing to all appointments and it was a general bother. Both her and Ryan yelled “Elmo Elmo Elmo!” whenever it was near.

Our experience in this amazing place was really great, but it’s about to get not so great.

~

PT

After a get-together with a few other parents there, I got an escort to our next appointment by a person who turned out to be an OT! And since we had not been able to afford seeing an OT, and I mentioned we didn’t have an appointment with one, she agreed to do a little impromptu visit with Lali right then and there. She mostly just praised the OT I have now and did a little exam. But she was very positive and I appreciated that. Then came time for our meeting with the PT. That wasn’t as positive. Our PT was very nice, but kinda negative. Instead of saying, “Good job Lali!” when she tried to kick using a muscle she didn’t have, the PT instead said, “So she can’t really do that.” And one time she was holding a ball out for Lali and trying to get her to do something with her leg, so of course Lali reaches out for the ball instead of kicking with her leg and the PT seemed frustrated and grabbed the ball away. She also seemed tired. Oh and I brought with me a list of questions to ask her that my PT back home had written out for this very reason, but she only wrote down the questions into her notebook and she never answered any of those questions! Is she going to write out her answers? And when? And who will she send the answers to? She said she needed a consult first. So… Anyway, this is all looking back on things, because she was perfectly nice, but I have one more gripe. At one point she noticed how Lali said, “help please” when she couldn’t do something and I mentioned that she says that a lot when she wants help. She then said that Lali was giving up too easily and that “personality determines ability”. What’s that suppose to mean?!

We did get some good ideas after PT. We’re going to get Lali used to the scary water (aka real baths) to prepare her for some possible pool therapy. We’re also going to get a 30 degree wedge to help her sit up herself. And we’re also going to try to get knee immobilizers. So that’s good.

Kiersten and Ryan had an appointment with the same PT the next day, but instead they got another one last minute. When Kiersten mentioned how Ryan had done well on his knees (and Lali too after hearing about Ryan’s progress), the PT asked where she’d learned to start knee weight bearing. Kiersten mentioned the AMC Atlas (arthrogryposis book that Dr. Hall edited) and that PT said, “Oh I wrote that article!” Kiersten took a video of all this so I could reap the benefit of her PT appointment. I kept thinking, “Why couldn’t we have gotten that PT?!”

~

Dr. Song

Dr. Song is a bone doctor/surgeon. After looking Lali over he was very optimistic and mentioned doing surgery to her knees to release them and internally rotate them and leave the hips externally rotated to help her stand. I was getting hopeful that there was something else to try, and scared that we were looking for a completely new surgery coming up very soon. He also mentioned knee casting to try to straighten them first. I was ready to do something since stretching hasn’t been gaining range of motion in her lower extremities. In my mind if the casting worked and the surgery moved her in the right position so she could stand (since she can’t physically stand now), then maybe with leg braces and a walker she may be able to get around the house and achieve independence! Actually looking back I was getting a lot of hopes up. Dr. Song then decided to take a break from our appointment to confer with the PT that Laelia had just seen.

He went out to talk with her. When he came back his countenance had changed. His optimism seemed more guarded, his words more politically correct. He said she wasn’t a great candidate for this surgery, and to wait six months to a year to see if we should do anything with her. He then brought up that kids with her personality (asking for help, or giving up easily as the PT had mentioned) didn’t do as well as other kids with go-get-’em attitudes. He said that 90% of kids with amyoplasia were ambulatory (able to transfer and stand), but it wasn’t looking like Laelia was going to be one of those kids.

When he left the room I asked the blond who stayed behind, “It’s not just me, right? He did start out very optimistic about all this surgery stuff and then came back from a meeting with a PT we had seen one time and is now pessimistic.” She responded in between niceties, “He wasn’t that optimistic in the beginning.”  Okay that was NOT my impression. She then printed me out a Return to Clinic paper to schedule a follow up (in that six months to a year we’re ignoring her), and I instead used the paper to write out my feelings. I was emotional and in tears, and these feeling are few and far between nowadays, but I have learned that it’s important to work through them. And even though my rational self disregards these feelings now, they were nevertheless honest (if a little disjointed), and I think a lot of parents in my position might feel the same way so I’m sharing them here. So these are my notes scribbled on the back of a paper minutes after leaving my appointment:

“10% of amyoplasia cases aren’t ambulatory at all and Laelia is one of those cases? I’m feeling depressed. I look over and Laelia is happily looking at some fish. If only she knew what he said in there about her future.

Sometimes when she’s bratty I worry that she shouldn’t be because she has to be “pretty on the inside” to make up for this.

I’m having wrong feelings about somehow having more kids to make up for this. I’m having feelings of failure–maybe because I’ve put more energy, patience and work into this one project (raising, caring for Laelia) than anything in my entire life. In fact I get how parents would not feel worth living if their child died. It’s not all about how much you love them–it’s also about how much of your own life you put into them.

I see kids who are worse off than Lali–I think there’s another clinic going on because I’ve seen lots of kids with breathing and feeding tubes–and that makes me feel better. Wow. That’s sick. And I wonder what it is about me that has to compare my child’s (and my own) worth to other people. But this lets me down when I leave the clinic area since out in the real world away from Children’s Hospital most kids are better off than mine. Most. And it’s taken for granted.

This sucks that my faith isn’t stronger. Right now I’d love the reassurance that God has a plan for my daughter and she’ll have a new body one day in Heaven. Something is keeping me from doing that. It feels like false hope.

Her lack of trying… does she get that from me? I didn’t really try at anything long term until I was in college.

I saw the PT’s back as she walked through the hall after my appointment with Dr. Song. And I felt the urge to run after her and ask “Really?” Or ask why. Like my kid was applying for a job and didn’t do well on the interview. I wanted to fix it. “

That’s all I wrote before I started to feel better and didn’t need to journal it anymore. I wiped my tears away and took my daughter on a well-deserved trip around the fish tanks that she’d been begging for. Seattle Children’s even let me borrow a bright yellow taxi car for her to “drive” around while I pushed.

~

Sleep

My Laelia is allergic to sleep sometimes and I didn’t get more than a few hours of sleep that first night.  She cried for a while before going to sleep. Then she woke up in the middle of the night screaming. I tried moving her entire hotel-borrowed crib into the small bathroom at one point just to let other people in the room sleep. It didn’t fit. At one point someone started banging on the wall. It was probably directed at us. Then finally Lali was so wide awake that we both played quiet games in my bed. I tried really hard for those not to be singing games, but she does those best of all so unfortunately she would belt out a tune and I would cringe and see if she’d woken up the other baby. Then she would look AT ME and say, “Shhh Mama. Baby sleeping!” !!! She fell asleep at 5:33 AM. The hotel alarm clock that no one set went off about an hour later. At first since it was playing music I thought it was Kiersten’s cell phone and wondered why she wasn’t turning it off. Then I realized that it was the alarm clock on my side of the bed, and it was too complicated to turn off. Even though I found my glasses in the darkness and hit every button on that thing I didn’t do it right apparently because it went off again nine minutes later! I couldn’t unplug it because the cord was behind the big immovable bed. So I was a bit tired for all our first appointments. Thankfully Kiersten woke us up an hour before our Dr. Hall appointment (because among other things that stupid alarm had the wrong stinkin’ time!) and we only ended up being 15 minutes late after check in.

But the second and third nights were absolutely wonderful.  We had a plan, Lali and I, for her to try to sleep in her crib, but if she woke up in the middle of the night then I would put her in bed with me. So she would go to bed around 10:30 PM and then wake up at 3:30 AM crying. I would grab her and put her in bed with me and instead of playing games she would cuddle up and go right back to sleep! She had not slept with me since she was six months old! If I had known that would work I would have done it in the first place! I used to get her when she’d wake up at night and she NEVER could sleep with me around. I tried not to take it personally. But this time Lali snuggled up under my chin, pushing all that crazy hair in my face, and slept like an angel. I fell in love all over again! She’s so precious when she’s asleep. She even does that high pitched sigh in her sleep like I did when I was younger. Besides being a total bed hog, she’s just a dream! And we got a bit more sleep that way.

~

Dr. Hanel

The next day (Thursday) we had an appointment with Dr. Hanel, the upper extremity doctor. It reminded me of my visit with Dr. Hall–it was a joy! Dr. Hanel took to Lali right away. He checked out her arms and shoulders and then said, “Can I tell you something, Laelia?” She looked in his eyes and nodded her head. And he said, “Don’t you let them ever operate on you pretty girl!” And she said, “Yeah!”  Then he looked at me and told me not to do any surgeries on her upper body. No releases and no muscle transfers; she doesn’t need the release because of her elbow range, and she’s not a good candidate for the transfer. Muscle transfers were one hope of getting her to maybe lift her own arms one day, but after hearing the down sides, I’m almost convinced it’s not worth the gamble. Especially not with her weak muscles, and the fact that we’re borrowing from Peter to pay Paul (in this case her back or peck muscle that she needs). But we’ll do more research and come back to discuss it in five years or so. Then Dr. Hanel gave me advice on parenting and teaching Lali words because she was going to be the smartest and prettiest baby in the world. Yep, this was much better than some of my other appointments.

~

Fun

We went to the Space Needle Thursday night for dinner. Lali passed me her french fries by putting them on my plate and then I’d share my pasta and we’d wink at each other. The view was amazing! And I think the waitress would have taken Ryan home with her. It was a fun trip. The next day we went to the Children’s Museum for ages 0-3 and played until it was time to fly home. It was a great trip and I had lots of fun with Kiersten (aka “Gigi”) and Ryan. I have some great memories of little kids chasing each other around, great food (sometimes too spicy for wimpy me) and one very funny run-in with Kiersten at 3:00 AM.

Oh and we ended our trip just like we started it: being harassed by the TSA over Laelia’s orthotics. We got a sticker out of it. It says, “I was detained for questioning by the TSA for looking suspicious!” No, it doesn’t say that. I asked. They don’t have a sticker like that. But, no, really they were very nice to the baby girl because she demands it with her sweet nature. She held out her shoes for them to wipe. They also needed her to turn her palms up, and after I explained that she couldn’t do that (she’s missing the muscles) she announced cheerfully, “I do it!” and held her hands up as far as they’d go which worked just as well to wipe the undersides. Nothing this little girl can’t do! 

Honorary TSA

And a special thanks to my husband who posted my April Fools joke while we were away. Love you!

Physical Therapy

This is the year for mobility! We’re working hard to see Laelia sit up, stand and scoot before she turns three! We found out at our last trip to the PT that her body is still not in the position for standing. That’s what the surgery was for so we’re bummed as you can imagine. But she is much straighter! We have some new hip stretches for her (that she hates) and some new weight bearing positions for her (that she hates more), so we’re hoping all this hard work will pay off before next October. If not, then we’ll see if another surgery can’t do the trick. I’m pretty optimistic though.

It’s weird, sometimes she can’t do something that I would think she could do, like get out of a kneeling position on the couch, and then other times she does something I didn’t think she could do, like scoot across the living room!

Here’s the video of the scooting!

We found out some interesting things this week and I’m not sure what to make of them. Apparently how her hair all seems to come from that whorl in the back and then sweep to the side (she doesn’t have a straight part like me) is due to her arthrogryposis and the position her skull was in while in the womb. Also her labia didn’t fully form since her legs were so wide spread in the womb. It probably won’t affect anything, but who knows. Just random things for Mommy to worry about.

Anyway, one thing the surgery really helped was her feet!

This is before her bath so there’s still crusties on her feet from the casts.

Happy girl!

~~~

Progression of the left foot:

Left foot at birth.

Left foot after minor surgery AND serial casting.

Left foot after major surgery!

Go lefty!

Third set of casts in two weeks. At least they’re cute, short and of course PINK!

Laelia’s casts slipped. AGAIN!!! Once again I find myself heading to the emergency room at Children’s Hospital, getting redirected to the clinic casting area and getting an emergency cast removal. (Fine. I didn’t want to work today anyway. *sigh*) The pin in her left foot had come up again too. The difference this time was that Laelia’s surgeon was in the building at that moment (being followed by his posse of three other doctors) and willing to see me! Fifteen minutes later (which is like a split second in doctor time!) he was checking out her feet and giving the okay for removal of the casts. He asked the tech to try to save the pin, but then later decided that the pin was just going to keep popping out so it was removed instead of screwed back down. (So much for Daddy doing the second pin removal. *sighs again*) All over again the baby girl gave a noise like she had been shot by a gun, then came the blood… and they asked if  I  was okay!?! I was thinking, “Forget me! Just take care of the my baby!”  Well that was followed by thoughts of, “Don’t pass out don’t pass out don’t pass out don’t pass out...”  Anyway, she was then recasted in short casts without a bar to avoid future slipping. New casts. Oh joy. At least there’s no more pins in her feet!

The casting tech asked me what color I wanted for her new casts… um, is there any other color?  

Laelia screamed as soon as she saw the surgeon all the way until we were almost home. She screamed for the entire cast removal and even louder after the pin was removed and then pretty darn loud as they recasted her. She was also shaking like crazy, bright red in the face and drowning in her own sweat. She HATES this! Then she cried through the check out process, getting my parking validated, the looooooooooooooong walk back to the car on the second floor of the parking garage and most of the way home. I got all sorts of looks and stares and pointing fingers from people. That’s unusual for this little girl–I mean that’s a little over two solid hours of screaming. Laelia had a miserable time.

So we decided when we got home that the rest of the day today will henceforth be known as Laelia Day! I promised her that she could have whatever she wanted for lunch and dinner. Any snacks she wanted. Any nap time she wanted. And we’ll even watch cartoons! When we got home she demanded I read her every book we owned! That took a while. Then she wanted some time with Puppy (the name of our kitty), who just had to put up with being pinned down by me while Laelia happily tugged her fur. Now I’m all scratched up. Then Laelia got to play in my purse and she got my cell phone out and started to dial numbers. Sorry strangers! What can I do? It’s Laelia Day!

Hooray!! Happy Laelia Day!