Laelia Sky

As for an update on Laelie, her casts were redone yesterday. I dragged myself to the clinic since I had my own doctor’s appointment anyway and was already up. Bad idea. I’m an idiot. Made everything worse. But I did get to be there to comfort baby.. before almost passing out. Long story. Anyway…

More bad news: her right wrist/hand looks bad. Actually I haven’t seen any progress for the last few castings. It doesn’t even go out straight let alone bend upwards. The fingers are bent to the left and there are deep creases as well as dead skin and all that just to add to the effect. We made the tough choice to stop casting it. The casts are the most extreme way of dealing with those stubborn wrists, but medical science has its limits. It just really doesn’t look good and I hate this. I kinda wanted to do enough casting so she didn’t look like an arthrogryposis kid from a distance or when you walked by her on the street. She has an appointment next week to get her splint for it. The splint’s job is to keep things where they’re at (not letting them go back), and to stretch out those tendons in the fingers that get neglected when fixing a wrist.

Now don’t get me wrong, the wrist looks much improved. Just far from normal. Actually we don’t say “normal” anymore, the new term is “typical.” I guess by saying “not normal” I’ve been stigmatizing my daughter into later years of therapy. Oops.

Also we have our first surgery coming this Monday. Both little feet/ankles. They’re not even putting her to sleep, but just giving her a local since she’s a baby. They told me two scary things: 1. Yes, she will cry and be in pain. 2. No, parents aren’t allowed in the surgery room with her.

The casts don’t let up though. They will do the surgery and then cast the legs up. They warned me that the next time they remove the casts (three weeks later) there will be blood in them and not to worry.

They waste their breath. All I do is worry.

Charles here.

We got Laelia’s casts changed again today. My wife asked the doctor how far the knees could get straightened out. We hadn’t seen a ton of progress, and we were wondering if her legs would be straight enough for her to walk.

“Oh,” the doctor replied, “we haven’t been trying to straighten out her knees. We just need to anchor the casts there while we straighten out her club feet. The legs tend to stay in whatever position we move them to without a lot of flex room, and the question right now is whether she’s going to be spending most of her time on her feet or in a wheelchair. Right now I’m thinking that she’ll be wheelchair-bound, so her knees are at about the perfect bend to accommodate that. But we can straighten them out with surgery later if it looks like she’s ready to walk.”

Alexis and I both had understood from other sources that the general treatment for these kinds of legs was to get them totally straightened out, and that’s what we thought we had been doing. How did we miss this?

And is the doctor right? Is my daughter never going to be able to walk, even with braces or crutches?  She (the doctor) tossed off the comment so casually.My wife and I were both just blindsided by it.

I feel like I can’t give up on my daughter so easily without even giving her a chance to prove herself. If doctors can straighten the knees, then they can bend them again if necessary. I’ll do research, but it looks like we may need to prescribe a new course of action for our orthopedic doctor.

The doctor might be right for all I know; our daughter could be bound to a wheelchair for the rest of her life. But I can’t believe that–not yet, anyway. God, protect my daughter. I need to know that whatever happens to her happens because You allow it to happen, and not because You fell asleep at the wheel or didn’t love my daughter very much. Heal her, if You are willing.

As of yesterday we were given our second set of arm casts and third set of leg casts. Her right thumb got caught and moved in her cast and it is shoved more against her palm as a result. We’re fixing it with the new casts, but I’m unhappy about it. The doctor was surprised the casts stayed on since wiggly newborns usually don’t keep them on well when they aren’t anchored above the elbow. There was another baby there who was a bit younger and very wiggly and I can see what they mean. I understand that not all babies are like mine, but can’t people understand that Laelie does not move? She has amyoplasia which is a=no myo=muscle plasia=growth, so by definition she won’t be able to move much with the limited muscle mass and then add to that the arthrogryposis (joint contractures) and extra tissue and I don’t know why they’re surprised that she doesn’t move and the casts stay on. Ah, an old back and forth between this doctor and myself.

I told the tech to be careful with her arms since they don’t bend the way normal arms do and the lady told me that she doesn’t mess with that, the doctors do. Then she started to remove the casts and turned her arm (the wrong way) to do it! Maybe there was a miscommunication, but I wish people would listen without me having to be Mama Bear.

Baby did better with her casts this time. She was very fussy and demanded food every hour for the next 24 hours. She also not only screamed during the casting but during the prep (unlike last time) because she knew what was coming. Despite all that, something about this visit made me think I can actually do this. Maybe she was a bit better or we knew what was coming. Maybe it was because she calmed down once in her car seat. (Actually when we can’t get her to stop fussing we just stick her in her car seat and pretend we’re going somewhere and two seconds later she’s unconscious. She’s in her car seat right now in fact…) She just seemed to handle it better, although I admit it could be that emotionally I handled it better and am projecting my mood onto her.

We also finally got occupational therapy authorized. So now we have all our therapies authorized. It turns out that it is possible to get our insurance to authorize this stuff sooner since her case desperately warrants it. We just didn’t know how to force them to do it or who to talk to when we needed PT authorized and they weren’t going to help us! (groan) Charley kept calling the insurance people and checking on the status and telling them how important it was. It turns out that all those stupid idiots (for lack of a better description) did was check our name on a computer screen for the status we kept requesting, brainlessly nod their heads through our story about our situation and then hang up and forget about us. They never got in touch with people who could help us. Actually when we asked to speak to other people like supervisors, they just did the same &@#&* thing! Well this time by luck (and not a little prayer from friends and family) Charley managed to find the right people to talk to who sped the process along. We had our first authorized appointment today. The bad news is that she may have missed opportunities for greater range of motion (ROM).

With casts Monday and therapy Tuesday Laelia is exhausted and fussy. Hopefully we’ll be able to schedule things better in the future, but we couldn’t pass up an opening with PT and OT.

When they took her out of the old casts for the five minutes of pictures before the new went on, we held her and she weighed nothing! She was so light and felt so wonderful. We touched her arms and legs all over. I tried to hold her hand, but her fingers won’t bend from being in the casts. The tech told me that they would be sore if I tried to move them. I did my best not to cry and just focused on the improvements in her wrists. They are still at a negative number when it comes to ROM (meaning they cannot go straight out) but I think the casts are slowly working. I’ll be so much happier when I can hold her hand. I’ll be happiest when she can lift her arms high enough to put them around my neck (that is a long way off and may never happen though).

I drew flowers on her leg casts this time and hearts on her arms which got lots of comments. A girl’s got to accessorize! I drew little hands with thumbs facing the right way too. For some reason her left arm cast is a lot longer because of how they wrapped it. So I drew a hand of cards on that cast to make up for the length.

I also drew some aces up her sleeve on the underside.

Laelie got her casts Monday night. The next 24 hours were the predicted hell. Now we’re getting use to them a little more. I drew on them so I could tell which way her hands went. I didn’t realize that her arms curled in the wrong way until we had the casts. (Just one more difference, *sigh*.) Instead of resting at her sides with palms facing towards the body, they face away. Drawing little hands on the casts help us not to hurt her when positioning her.

After getting the casts she would wake up in the middle of a deep sleep to scream thinking we are doing PT on her, but it was just the constant pull of the casts. She cried for hours and then whimpered after she was exhausted. It wasn’t until Tuesday night that she started to act normally again.

I’ve been sick. This is my first time out of bed for any length of time in a couple days. We thought I had a breast infection, but it turned out to be a bruise that made breast feeding painful. The lactation consultant said to try massage and pumping and now the blockage is gone. I was also sick to my stomach for a long time. I just needed sleep, but baby was hungry and doesn’t take a bottle very well yet. We think she either had another growth spurt or she just needed comfort on Monday and Tuesday because she ate every hour… which was painful.

I miss holding her hand. We would hold hands and she would try to grip my finger or thumb evey time we breast fed. I miss it so much I cry.

When doing the casting the doctor and assistant just kept asking if we were sure this is what we wanted. I hated that. No, we’re not sure. We don’t know anything. The occupational therapist, who has studied arthrogryposis more than any other medical professional we know, said this was best. They didn’t use to do it this way, but saw better results with extreme cases. The doctor just kept saying how she had never casted all four limbs before and poor little baby. The assistant said he had done it once before, but not on a baby. They offered to just do one arm and not the other for no reason other than “poor baby with four limbs in casts.” Then they explained that she wouldn’t be able to move her arms if we did this. (Duh!) But she doesn’t move her arms anyway! Ever! Just her fingers. And it’s not like I don’t miss holding her hand! I didn’t choose this! I didn’t wake up one day and decide to be mean and put my daughter in four tons of plaster! We’re just trying to do what’s best.

After listening to all the input, I decided (at the last second) to only cast up to the elbows. The doctor casting her seemed happy about that. But now we have the possibility that these casts will slip down. Which would suck and we’d have to start over. I think I made the right choice since it is a little bit easier to get clothes on her since her elbows have a small passive range of motion. That said, she is still wearing clothes for kids four times older than she is in order to get them on her.

I just hope this works.

Before we even had the casts on them, a little boy at church asked me if her hands were broken. I didn’t know what to say, but someone else was there to field the question. Other kids were asking why she was that way. I have been thinking about it and have decided finally how I would respond. I would say that God didn’t make her this way. He made her perfect. She had an accident. Just like people have car accidents and need fixed by doctors. Only this accident happened when she was still in her mommy’s tummy. Now this accident was no surprise to God, but he allowed it. Now we’re trying to fix her, but she’ll never be like other kids. And that’s okay. Because being different is okay. And being different doesn’t mean you did anything wrong. That’s just the way it is.

Question: I found out my baby has AMC when she was born. How do I take care of her?

Answer: Your child is like any other child. You may have to feed her by tube for a while, because sometimes babies with AMC have weak sucking muscles. You MUST begin stretching and massage right away—in the hospital if possible. If the doctor wants to wait a while, find someone else who will teach you how to do it. Surgeries and splints can wait, but gentle massage and stretching doesn’t need to unless your child is exceptionally fragile. Your child will need to receive physical therapy for years, as well as occupational therapy and possible speech therapy. Developmental delays are usually caused directly by their physical limitations, not by any problem with their brains. You will still cuddle them, read to them, make funny faces for them, and show them off proudly. I promise.

This is one of many helpful things I have learned from the AMC (Arthrogryposis Multiplex Congenita) support group website (third link down in the links section). I am now a junior member and have already been in contact with other moms with kids like Laelie. Some are better than her and some are worse off, but all the moms seem to have the same things in common. Many felt just like me when I found out about Laelia’s condition even though now they are strong, well adjusted and totally in love with their kids. There is a forum so we can share what worked in the way of surgeries and casts and physical therapy too.

Based on some of the information and emails I have gotten from other moms, we have decided to back off of the hard physical and occupational therapy and go for a more mild, gentle approach. We’re still doing physical therapy everyday, but now we stop if she cries. If she fusses we’ll keep going, but more gently. Then we will request more aggressive splints and casts for the greater improvements. Also I am massaging her every time I breast feed her.

In effect, this is making her more “touchable” and less flinchy. I always want touches to be a good thing. Now we need prayer more than ever that we get regular PT and OT appointments (especially OT) to do the more aggressive therapy. That way the doctor’s office is associated with pain as opposed to our home.

I’m so glad the other moms with AMC kids were able to help us out in this area. We were doing really bad emotionally with doing her PT. It was killing us. It was really bad. It was the worst thing I’ve ever had to do in my life. We couldn’t go on like that. It’s being adjusted for our sanity’s sake as well as Laelia’s sake.

Laelia will have her casts on and off for a long time. This first batch of casts on her legs will last around three months with recasting happening every Monday. When the casts are finally off, then physical therapy will start on her legs until recasting is needed.

Her arm casts are more tricky. Her wrists are bent in such a way that they cannot even make it to a straight (0) position. When noting her mobility in a chart, her wrists had a negative number (which the intern taking the measurements didn’t even know how to write down). So with a -45, her wrists need major work. Casting will happen Monday and recasting every Monday as well, but we don’t know for how long. After the casting, we will do splints which will be more long-term but removable. By high school we are hoping she can just wear them at night and even put them on herself if all goes well. By high school.. geez. All our hopes are on a functional adult, but her childhood will be so hard. The doctor who does the casting was very hesitant about casting her upper body and warned that it will impair bonding with her. But after the other doctor talked to her and told her that Laelie doesn’t move her arms at all anyway, it was agreed that she would get casts.

We are not meeting our five-a-day goal for PT at home. (Her therapist wanted us to do it after *every* diaper change.) So far we manage two a day. For about five to seven minutes Charley will stretch her joints out (20 to 30 seconds each joint) as she screams. She screams so much she can’t breathe. I constantly blow on her but she still gets so red in the face that I don’t recognize her so we have to take brakes to get her oxygen. Then it takes about 30 minutes afterwards to calm her down completely. After which she will fall so deeply asleep from all the screaming that she will sleep through her next feeding. And even after we get regular PT and OT appointments we will still be expected to do this everyday. It’s during these times that I pray God will just take her to Heaven right now and spare her a lifetime of this.

Today we tried hot towels and massages to loosen her up before PT. It didn’t seem to work at all and she still screamed just as much. Next time we are going to try wrapping her in lots of blankets to warm her up. We would try hydrotherapy (putting her in warm water), but we can’t yet because of the casts and her stubborn umbilical cord.

We’re not doing good emotionally. Charley is near tears every time he does PT. He told me he feels like a Nazi torturer when he can’t get the baby sweat smell off his hands afterward. I can’t do it at all. After PT we both hold her and rock as she whimpers with these sad, hurt little eyes for quite a while afterwards. It’s getting where she will start to cry when we put her down on the couch (where her PT takes place) or touch her at all.

She no longer curls her fingers around my thumb when she breast feeds. If my thumb touches her hand, she flinches.

So we showed up today at the physical therapy building in person and determined to be obnoxious until we got an appointment. We came with enough baby supplies to last a while. We were all prepared to go to war and bug people and stand there in people’s faces and all that, but in reality it was all niceties and the whole business was over in 20 minutes. We got her physical therapy and her first appointment is tomorrow! Of course insurance is only letting us schedule her through January (with three weeks off for Christmas) and they swear they couldn’t have done this faster.

We also had our occupational therapy appointment today (same building). As we feared, it was just an evaluation to see if we qualified for future therapy after which our insurance will take two to three weeks to authorize it. However, after explaining our situation, the therapist spent a ton of time showing us exactly what to do at home so as to not waste valuable time. She said that our insurance will just take its three weeks of dragging its feet and there’s nothing we can do about it. It will be exactly like it was with physical therapy, except our OT appointment was a much better experience. The therapist told me what she was doing, had experience with many other arthrogryposis patients and made sure we weren’t losing opportunities.

So for the bad news.

Laelia has some problems I didn’t even know about. Her shoulder is deformed in a way that makes her pull to the right and one arm becomes shorter than the other. The therapist has no idea what exactly is wrong and we have an appointment with orthopedics to look into it next week.

Her wrists are also worse than I feared. She will get casts on her hands and arms now to try and make any difference. If you read a previous journal entry, you’ll know that casts were not an option we wanted due to lack of mobility. Things change. The casts are coming next week and I just pray she will still be able to type someday.

Now we know what to do as far as therapy at home. Five times a day (a goal we have set for ourselves) we hurt Laelie for about five solid minutes (that seem like an eternity). She screams and it hurts her.

The therapist told me that it goes against instinct to do what she expects me to do to my baby. But I have to do it anyway. I don’t want to hurt her, but if I don’t then she’ll be completely crippled for life for sure. She may be anyway. This is hell.

In a week she will have all four limbs in casts. The casts are a constant pull and very uncomfortable. But at least then we won’t have to do as much therapy since we won’t be able to get to her elbows, wrists or thumbs.

Charley has dutifully taken the brunt of the therapy while I go upstairs and rock and cry.

Laelia also has a cold that makes it hard to breathe and nurse. She wakes up several times during sleep in a panic, wheezing and gagging. Her poop has changed color because of all the mucus and she’s coughing and sneezing. I have the most miserable baby. I hate life.

We had our orthopedics appointment! We called ahead of time to see if they were closed for the fires. Then we covered the baby, held our breaths, brushed the ash from our car and drove through what looked like Armageddon. What felt like little drops of rain turned out to be tiny bits of ash from unusually dark skies.

I was so nervous in the waiting room. The other kids had broken arms, but they were jumping around and happy. I don’t know why I thought other people in the waiting room would have Arthrogryposis. They were all soccer injuries or whatnot.

These people really seemed to care. They kept asking questions like, “Has she gotten a hip ultrasound?” No. “Has she gotten x-rays to see what’s going on?” No. “Has she had a genetic exam?” Not really. “Has PT told you how much mobility she has in which limbs?” No. This was actually the most thorough exam she had received since the diagnosis, and we told them as much. The physician’s assistant just shook his head and scheduled a hip ultrasound that was there in less than ten minutes. We gave her a pacifier for that one since it was mostly just uncomfortable and the gel was cold. When the lady first put the cold ultrasound on her tush, Laelie’s eyes got really wide and that pacifier got sucked pretty furiously. That’s cold, Mommy!

They also moved her body parts and wrote down how much movement she has in each limb. (Not as much in some, more than we thought in others.) They also explained EXACTLY what they were doing instead of just moving my baby and taking notes like they did in PT. Now I know that I have total rights to make doctors explain exactly what they’re doing and stop immediately if I don’t like it. Some doctors just move all her little joints to see how far they can go for no reason other than curiosity (at least as far as they explain to me). These people were great and I appreciated them.

We were getting casts that day. No waiting seven more appointments. No having to call and harrass anyone. No need for ambiguous “approvals.” We were told that these casts are usually done at one week of age! We don’t think waiting this long cost her much, but it’s infuriating that our insurance dragged their feet so much even when we were calling twice a day everyday.

The doctor came in and we talked options (finally!). They decided not to put her arms in casts and to try splints instead at our occupational therapy visit (on the 29th). It’s discouraging that her arms have not once moved on their own. Hopefully we’ll know more at OT… unless it is like our PT appointment… grrr.

We decided to try and fix the club feet with the casts and stretch out the legs since leg movement is less important. If we ever want her to walk, she may just need the legs straight and do a kind of stilts-walk. Whereas the doctor put a lot of emphasis on her learning to type so we’re keeping casts off her wrists. She also wanted to put off surgeries and x-rays until Laelie was a little older. I like how the doctor said, “Let’s get her typing!” and “We’re gonna get these little wrists going this way so she can type!” It was better than the usual, “Well maybe if we do this she may gain a small amount of mobility bla bla bla,” that we get from other doctors. I felt hopeful.

While they were casting her, she cried and cried. The doctor kept telling me how necessary it was and explained exactly what the discomfort was and that she wasn’t in terrible pain. The physician’s assistant started singing to Laelia when she wouldn’t stop crying. He had a nice voice and babied it up a bit. Laelia was still crying, but she stared right into his eyes and I bet she had her first crush right then and there.

We hate our casts. It’s like the discomfort of having braces on your teeth. It’s not a sharp pain, but it is a big discomfort. She cries more now and is having trouble eating. She went 12 hours with me barely getting anything in her mouth. They think it’s just because she’s uncomfortable. We also have to check her little toes all the time to make sure they stay pink. The bad thing about club feet in Arthrogryposis patients is that it goes back after the casts are off. So she could be in and out of casts since it’s not a true clubbed foot situation.

Working with a baby who for all intents and purposes had no legs (since they were so curled up and tight) to having these long, awkwardly wide, heavy and hard-as-a-rock legs is hard to navigate. Picking her up and having her feel the weight of the casts makes her cry. Breast feeding is so impossibly awkward now! The casts are hot and the temperature is so hot now that we were warned she would get heat rashes. The casts are going to be taken off and replaced every week. It’s looking like this will be hard.

But it is so nice to look down at my baby in her box (as you can see in her photo) and have this baby who has legs. She looks more like a person now instead of a sack of potatoes.

No, we’re not in the fires. We haven’t evacuated. Actually we’re in a good spot next to Qualcomm Stadium which is the evacuation site so people are actually coming to us. A few friends of friends have lost their homes and we had some visitors that couldn’t come see us today since they are leaving the highways for evacuations. We have a bag packed for baby, but I’m not worried at all since the fires are east and north of us. Yes, the fires are kinda close, but they are also far enough away to give us plenty of time to get to the beach if we need to. We’re trying to stay off the cell phones since everybody is trying to contact relocating friends and family. So if you’re calling, call the home phone. For more information about the evacuations: http://www.sandiego.gov/newsflash/firealert.shtml.

Also for those of you who have asked about Laelie’s booboo on her knee, no, I didn’t drop her. She actually had that when she was born. I don’t know how, but she wasn’t moving and she was rubbing up against my ribs or something and it broke her knee open. So she was bleeding inside me which is a nasty thought. You can actually see the injured knee in the photos section. It’s her Playboy naked picture. It’s healing well and we have special medicine for it.

Also for those that have asked we FINALLY have an occupational therapy appointment! When we thought it was taking forever to authorize the appointments, it was *actually* taking them forever to *tell* us they authorized the appointment. If Charley wasn’t calling these jokers everyday, we would still be waiting for them to remember us and schedule an appointment. Just shows that no one is looking out for Laelia except those who love her. Since every day we wait on this stuff means she gets less help and less mobility for the future, it really makes us angry. But we have the appointment set for the 29th.

According to the specialists, we are putting her in the best care at the best hospitals. I’ve been assured by other parents that once you get past the red tape, bureaucracy and people who *don’t* care, then you get regular appointments with doctors and therapists who know you and have a relationship with you and *do* care. (Except for the occasional one who sees her as a pile of body parts.) Just got to jump this hurdle first.

Laelia’s physical therapy session was today.

When they told me that there was only 500 cases of Amyoplasia Congenita Disruption Sequence reported in the last hundred years, I still thought there would be more children with Arthrogryposis in San Diego and the doctors and physical therapists would know what to do.

We went into Children’s hospital and our physical therapist looked like a teenager. She moved baby Laelie’s arms/legs/feet/hands/hips while she screamed. I was balling so badly. I was shaking. Dr. Teenager didn’t give us any hope or any long term plan. She talked of small minor improvements at best and surgeries. She had *never* worked with Arthrogryposis patients and pronounced it “gryposis” instead of “groposis” which is how it’s spelled but shows a lack of familiarity with it. It was like she read it out of a textbook right before seeing us. They only had two other children come through with Arthrogryposis and only one could walk. His (the walking kid’s) physical therapist said it was nothing she did either, he just could. It turns out that babies with Arthrogryposis that have the leg joints stiff in a straight, outward way are more able to walk. Of course our baby girl has the wide open legs and hope seems dim.

I’m so depressed.

I can’t eat. I have diarrhea and I’m throwing up. I try to eat so I can breast feed but it just makes me more sick. On top of the hormones in my body going crazy, my emotional state at a constant flux, grief over my daughter’s body, 2 and 1/2 hours of sleep last night (literally) and stress to feed and take care of the baby, I also have to work her little sore spots. She just cries and cries. I feel like I can’t do this—I can’t go on.

Then there’s the natural mother instincts. It keeps me in-tune with all her needs. I hate it, but it’s so necessary. When she can’t sleep I can’t sleep. I can’t get over how intense this feeling is. Actually the cat will cry and I’ll lose it.

I naturally act like a mom. I rock her instinctively and I burp her when she’s unhappy. Actually Charley passed gas in his sleep and I already had a hand on his back and my hand began to burp him in my sleep. He thought it was funny and just let me do it.

The mom instinct is what made me ask the doctor how old she was (26!!), her qualifications (one year out of school) and then I asked for another doctor. I made a stink about getting someone who knew Arthrogryposis patients and what they’re capable of. I have never had to be someone’s advocate before. I did all this like a real champ… while balling.

Later today Charley called our pediatrician (Dr. Schwartz… “May the Swartz be with you”)and he is going to try and talk to the parents of another Arthrogryposis kid in his care so they can recommend doctors they liked to us.

Dad and Christina (Grandpa and Grandma) are leaving tonight. I tell myself I’m going to be able to live like this. I need help but I need a break from help too. I need to be able to do this myself with Charley. We just don’t know what we’re doing. I need people mostly to tell me her crying is normal and make light of it. That keeps me from panicing. All newborns are suppose to cry, right? Mine just also cries when you move things on her that hurt. It’s so hard to know the difference. For instance, burping her upset her and she cried every time. It turned out that I still had My Breast Friend on (a little shelf to keep her at breast level) and she would be on my shoulder but her feet would be on the shelf and it was hurting her. Accccc! But then there are times when she is just fussy and I think she’s dying.

Right now Charley is working her little arms. She has a little pout on her face that makes her look like a bulldog. Charley tells her that she’s fine and not to cry and so far she’s not. When she fusses then I start to cry. Dad says he prays she never learns the power she has over me or she’ll be eating cookies for breakfast everyday.

I love my sweet little Laelie Bug. God, just make her whole.