Keekaroo-whoo-hoo!

We're still in the hospital and not at all happy about it. The doctors told us five days max and it has now been twice as long. It's not really their fault even though I can't help but blame them. Maisie had to have 24 hours off all medication except for the drugs for myasthenia, seizures, and hemodialysis. That, apparently, wasn't good enough for the nephrologist (this was a new guy, the doctor we usually work with is away) who insisted upon 24 hours without ANY medication. I fought, I argued, I yelled, I tried and tried but he insisted and finally I gave in. She didn't get her evening meds and by two in the morning she was seizing constantly, unable to breathe, and in obvious pain. She was rushed to the PICU and two hours later was stabilizing, thank GOD. I had a private conference with her pediatrician, neurologist, and pulmonologist to make sure I had my facts straight and I wasn't just crazy and terrified before I fought to get her another nephrologist, which thankfully we did. So then she needed a full days in the PICU to recover and get her levels consistent and normal before they let her go back to the regular pediatric ward. Now we'd lost three days, four if you count the fourth day when they took her off just a couple of meds to evaluate her symptoms. Grr!

They finally got her started on treatment on Wednesday and by Thursday her blood and urine tests were coming back even worse. I'm not surprised, I mean, I'm sure all that panic and stress on her body helped her kidneys heal. So they started her on an antibiotic to which she promptly had an allergic reaction. And then they had to wait for her rash to go away before they tried something new, and now we are here, and she's finally giving better blood/urine tests. This has all been so traumatic and confusing for her because she wasn't feeling ill before. The only good thing I can say about this fiasco is that we caught the HUS early so she is doing really well now and we might get out of here tomorrow.

What I really wanted this post to be about is the wonderful, serendipitous thing that Jeff's sister Emily found. Emily lives here in the city and she's been visiting us a lot, which is especially wonderful because I'm not alone all the time while Jeff is at work. I was telling her that we're struggling to find a booster seat/high chair/something that Maisie can sit in at the table. We have been using the combination of a booster-seat and a bathtub headrest, but it is just so not working. When Maisie is exhausted, the seat doesn't hold her up. The seat is uncomfortable on her bony behind and there is just not enough support for her head. So Emily was walking home one day and stopped into a thrift store and there, in the middle of the floor, was a brand-new Keekaroo Height Right Kid's Chair. It was fifteen dollars so she bought it on a whim, brought it to the hospital to show me and we plopped Maisie in it and holy cow.

This.

Thing.

Rocks.

It's very comfortable and very easy to clean. We needed to order the cushion set to the right for her head and the blue "infant insert" on the left fits her beautiful and gives her alignment and padding and support. I AM IN LOVE. Finding adaptive equipment that is not unbelievably pricey is so hard because the market is so small. This thing is amazing, supportive, wonderful.

Keekaroo-whoo-hoo!!!

Sick girl

Okay, so the biopsy results came back at 3:00 in the morning and the news is that Maisie has a disease called hemolytic-uremic syndrome. Normally kids get very, very sick with this condition before they are diagnosed. Unfortunately, because Maisie is really sick all the time, we didn't notice her symptoms -- fatigue, diarrhea, pain, fever, seizures, low urine production, etc -- are symptoms she displays constantly. So the minor increase in all these things was not alarming. We are very lucky that she had this checkup, and this is exactly why we have these checkups.

We're going to stay in the hospital for a couple of days so they can keep watch on Maisie. She is going to be off stool softeners, physical therapy, and pain meds for 24 hours so we can see what her symptoms are unaffected by medicine (except for seizure meds, hemodialysis, and her drugs for myasthenia). Then she'll go back on normal medication. Our hope is that this will resolve itself after a few days, but if it doesn't things will complicate quite a bit because everyone reacts differently to the various kinds of antibiotics and stuff that are used to treat it.

Maisie feels okay, mostly because she feels as ill as she always does. She's mostly just really tired and really not happy about the cancellation of physical therapy. Therapy is really hard work for Maisie, but she also really loves it because she loves experimenting with walking and sitting and standing and mobility in general. So there has been lots of struggling to get out of bed and wearing herself out trying to get onto the floor to get some hardcore sitting done and lots of crying when I have to put her back in bed. It is wonderful to see her having so much enthusiasm and excitement to get moving, and just as heartbreaking to have to tell her no.

A lot of people have asked what Maisie's status is mentally,  developmentally, speech-ally, etc. Maisie is not, as far as we can tell, mentally delayed in any way. She struggles with a lot of anger, frustration, and anxiety because she remembers being able to walk and run and talk and now she can't do any of those things with as much grace and efficiency as in the past. Because she has not lost any memory or mental abilities, Maisie is still technically potty trained, but she often is not able to communicate her need to use the bathroom to me quickly enough, and so she wears pull-ups most of the time as a precaution. She has lost a lot of speech because talking using an extraordinary amount of muscles and coordination, which Maisie simply lacks. We're working on it, and in fact she will be starting speech therapy in the fall. For now she communicates much like a 1.5-year-old, in incoherent stuttering phrases and crying. It's hard to watch her go through this. But I am so proud of her and all she has achieved and all she is working towards.

A checkup gone very wrong

I have been dragging my feet to update because everything is going so swimmingly here in the kingdom of Maisie the Great. She's been feeling great and doing really well in physical therapy. As always, however, the fun must end. We headed back to the hospital Monday night -- by the way, we have two regular hospitals: the emergency one in New Jersey which shall henceforth be known as the NJ children's hospital (not it's formal name) and the one in New York City where her more specialized care happens, referred to as the NYC pediatric specialty care or NYPSC or PediCare or something (again, not its formal name. I'm making these up for privacy reasons) -- so we headed down to the  for two days of routine evaluations, follow-ups, and a formal sleep study, which her GP wanted done as a follow-up to one we did a year ago (sleeping, another thing I should give details on. I swear I will post a detailed explanation of her anxiety and sleep struggles soon.) So first stop was the sleep center at NYPSC, where she would stay overnight to do a polysomnogram while she sleeps. She was really scared when the nurses started sticking the sensors all over her. She had this done before and it was only a year ago, she had a bad case of pneumonia at the time and was already in the hospital when the GP had the realization that she might not be getting better because she wasn't sleeping well, so we did the sleep study and discovered that, yes, she was getting only about an hour of real sleep each night despite being in bed for hours. Anyway! The GP wanted to do a followup to the sleep study to make sure that the Melatonin and Lunesta were still doing their job. Then, this morning, she had renal tests and an electroretinogram done to test her eye function, which has been a major concern. Then we had lunch, and then this afternoon she had a long series of x-rays on her past trouble areas (ribs, hands, legs).

We were supposed to have a meeting with her major care providers and then either be sent home or schedule follow-ups. That didn't happen. Her renal tests came back so bad that the nephrologist thought her blood or urine might have been contaminated. And so they drew more blood and took another urine sample and while the results were better, they weren't enough better. She was placed in pediatric ICU on dialysis to clean her blood, which is apparently still full of creatinine (the crap that kidneys are supposed to filter out). Meanwhile, her doctors had a tense conversation out in the hall. The anxiety was palpable. Finally, the nephrologist looked at me with that looked that doctors have mastered so well, that look of poorly disguised fear. After three hours of dialysis they would test her blood again. If the creatinine levels were down enough, she would remain in the hospital overnight for observation, and then be tested again in the morning to see if she maintained healthy levels. But (of course there's a but) her levels weren't low enough. A nurse took her temperature and more blood and asked me a zillion questions about her seizures, her energy levels, her bowel movements, her skin color! At ten o'clock they took her in and took a kidney biopsy. Now we're waiting. It is almost 12:30 in the morning. Maisie woke up after the biopsy and fell promptly back to sleep. I have been told that I am free to fall asleep until they bring the biopsy results back, but I can't. I am freaking the fuck out. This was supposed to be a routine overnight stay, just a couple of quick tests to monitor her progress and suddenly this stifling PICU room is thick with panic, uncertainty. Jeff is on his way with clothes and toys and some other things. I'm trying to stay calm. I will update when we have news.

A Day in the Life #2

Hello!

Happy Fourth of July!!! I forgot to say it yesterday because we had a storm over here in New Jersey and our fireworks were postponed to today.

Two posts in two days! Wow! Is this for real??

I decided to do another "day in the life" because a lot of things have changed since March when I did the last one. These posts are really good for me to look back and remember how we were doing something and how we've changed.

7:00 - 9:00
Jeff and I get up, have coffee and bagels or muffins or something. It's nice, peaceful, quiet time to ourselves.

9:30ish
Maisie wakes up. She's been sleeping later which is awesome because she has always had slept so badly. I don't know if we talked about it very much but she struggled with apnea a lot. She actually had a blockage in her trachea that we discovered when she was about six months old and -- you know what, I'll post about that later. But anyway, she has never slept well and the fact that she is wanting to sleep later and later is wonderful, because she's always so tired, the poor thing.

10:00
By ten Maisie is up and has had all her morning meds. Right now she's taking Prozac twice a day for anxiety (something she struggles with a lot and I realize I haven't really talked about. We'll get there.) an oral liquid management dose for asthma (the real problem with her lungs is a neurological disconnect, but this medication seems to help keep things moving); Mestinon (pyridostigmine bromine) four times a day for myasthenia; a stool softener so she can have bowel movements on her own with limited muscle tone down there; diuretics to control blood pressure and limit the stress on her kidneys; Nasonex for her chronic environmental allergies; Ciprodex drops in her ears to reduce inflammation; and antibiotics for an ear infection, the fourth one she's had this year. Most of these medications go through her g-tube, but the Nasonex goes through her nose, and obviously the Ciprodox is ear drops. We also have prescriptions for topical antihistamines, her rescue inhalers and her nebulizer, which she takes in the afternoon.

10:30
At this point Maisie is set up with PediaSure through the tube and oatmeal/graham crackers/apple sauce/bagel/eggs/toast/yougethepicture for breakfast. I have to make sure she gets 300 calories in her breakfast (at least) and since she'll only reliably eat about 100, I give her 250 calories of PediaSure to give her a little cushion. And also so I don't have to force-feed her graham crackers and peanut butter.

11:00
Breakfast is over, playtime commences. (on school days, which are Mondays and Fridays, everything happens half an hour earlier and from 11 to 2 she is at school.) This is when I get work done and Maisie plays in the living room, which we have set up with a bean bag chair, TV, and a number of toys. Some of her toys are there to encourage her to work on skills like standing, grasping, sitting, etc, but some, like her beloved Doc McStuffins gear, are just for fun. She deserves some fun.

1:00
Lunchtime and afternoon meds, which are much less complicated than morning meds. She takes the Nasonex and the Ciprodex again, and takes the Mestinon again. Then she enjoys of a lunch of PediaSure and turkey, ham, chicken, sweet potatoes, applesauce, bananas, etc. She has to have half her caloric intake (total is 1800, so 900) by now and so she's probably already had a snack or PediaSure, so she's now had about 600 calories and is getting her final 300 for lunch.

2:00
We start physical therapy with sitting. She's gotten pretty good at this and is now able to sit up unassisted for at least thirteen minutes. After that she starts swaying a little, but she pushes through to fifteen or twenty minutes, usually falling a couple of times. Then she rests for ten minutes before we start standing. First we do as long as possible (about a minute) unassisted and then at least five minutes assisted. Then she rests for ten to fifteen minutes, and then I load her into the walker. The walker is by far Maisie's favorite part of physical therapy. The ability to move under her own steam is the best gift I've ever been able to give her. Her face lights up when she feels the floor beneath her feet. I have to drag her out of the walker, even though she's shaking from muscle fatigue.

3:00
Third dose of pyridostigmine is administered, usually right at the end of physical therapy and right before she takes a nap.

4:00/4:30
Up until this point Maisie is probably asleep. We finish physical therapy at around 3:00 and she usually naps until 4:00 or 4:30. Therapy wipes her out. It seems like silly stuff, but it really is hard work. When she wakes up, as close to 4:30 as I can get it, she does a breathing treatment on the nebulizer. After her treatment we just hang out, watch Doc McStuffins or Sid the Science Kid, and play. I get dinner ready.

5:00
This is when Maisie has to have her evening meds, which means her second dose of Prozac, Mestinon, Deltasone (prednisone), Nasonex, Ciprodex, Melatonin and Lunesta for sleep, and (if it's Saturday which it happens to be) Otrexup (methotrexate). Otrexup is injected into her thigh, but Mestinon and Deltasone are tablets that have to be dissolved in PediaSure and taken through her tube, which means that even if Jeff is not here she gets about 100 calories at 5:00 without fail. I try to wait to feed her until Jeff is home so we can all eat together but sometimes it doesn't happen. As soon as she sees the pump and the PediaSure she's like NEED FOOD NOW. Obviously by dinnertime she has to have had her last 900 calories. She usually gets a snack after therapy and after her nap, so dinnertime means about 400 calories in homemade chicken nuggets, spinach, broccoli, noodles, meatballs, etc. And then probably PediaSure.

After dinner (whenever that is) I give her a bath and pajamas and then we hang out and watch a movie or play or something until 8:30, which is when we start reading books and singing songs and sleeping.

9:00
Lights out, Maisie out. Jeff and I are usually asleep by eleven, and then the whole process starts over again.

Summertime update, and happy happy news

By now anyone who checks regularly probably stopped checking for a new post. I just couldn't bring myself to take my eyes off my beautiful girl, and my hunky husband, and the flowers in my garden. Life is good.

Yes! Jeff and I got married THREE DAYS AGO. The first of July! It was wonderful! Mom, Dad, Maisie, Jeff, Jeff's sister, Jeff's parents, my sister Miranda (who says hi) and a couple of our very close friends all went to the church for a very simple ceremony. Afterwards we had a picnic in my mom's huge backyard. Basically everyone I know was there at some point. My friend Stella, who I think has posted on the blog, made us a four-tier cake with layers of vanilla and chocolate and hazelnut and caramel frosting and real flowers. There were ribs and hamburgers and hot dogs and little children who ran through sprinklers and got ketchup and cake and frosting everywhere.


And Maisie had a wonderful time. We set up a blanket and her favorite bouncy chair, which looks like a ladybug (without the bar though). She sat in the chair and watched the activity and laughed. We couldn't get her to take any steps on her own, but she walked holding someone else's hands. Most of the time, though, she lay sprawled on her blanket or in her ladybug, her fingers in her mouth and her big eyes surveying her kingdom. When the time came, I fed her like it was any other day. A lot of small cousins and grand-cousins (second cousins?) asked a lot of strange and probably rude questions, and I answered all of them.

It's hard to remember sometimes that most people feed their five-year-old peanut butter and banana sandwiches and carrot sticks and juice boxes, that most five year olds run and squeal and play and have long since outgrown ladybug bouncy chairs. In the past few days, surrounded by love and friends and healthy little five year olds, I have remembered how extraordinary my daughter is, how far from usual she is. My cousin Sarah has a five-year-old boy named Hank and my cousin Lily has a two-year-old boy named Joey. And despite being so much closer to Hank's age, Maisie looks so much more like Joey. Even he leaves her in the dust in terms of running and shouting. She could barely keep up with the one-year-old baby with whom she shared her blanket until he discovered her g-tube and became determined to pull it out. When I want to show you pictures of my girl, I show you pictures like this, which are heart-breakingly close to reality (except without the enough-confidence-in-standing-abilities-to-take-a-picture-and-not-constantly-hover-behind-her-in-case-she-falls). I'm trying to find words to convey the sense of confusion and failure that come with holding your tiny, frail little girl up while her contemporaries run and scream. When I hold Maisie she clings to my shirt or my arm with weak fingers. Her legs don't grip my waist like most babies' would, and she often won't hold her head up, instead letting it droop against my shoulder. And then I hold Hank or Jessie or Rachel and realize that they, while being within one year of her age, are such completely different entities to pick up. For one thing, You would almost never hold a four, five, or six year old on your hip. But when you do they are heavy but not as heavy as they should be. Their strong legs and trunks and arms support themselves but not Maisie.

That's not to say she isn't getting stronger, oh so much stronger! Every week we meet with Drill Sergeant June. Physical therapy these days is not so much the therapy itself but a kind of weekly progress assessment. The actual therapy happens during the week when Maisie and I practice her three skills: sitting, standing, and walking. When we first started, she could barely stand to practice sitting up for five minutes. And she would fall over every couple of seconds and I'd have to prop her back up. Today, however, she can sit unassisted for a whopping thirteen to fifteen minutes at a time! Standing is somehow harder than walking in the walker, but we're up to a minute or so unassisted, and five to seven minutes assisted (holding hands or the table or in the walker). In the walker she can walk for five solid minutes without stopping. Unassisted she's taken probably a total of a two dozen steps in three-to-four step increments. But holy cow. How far my angel has come.

Overall I am happy. I am at peace. I am relieved. Our life is not where we thought it would be, but it is stable. Maisie is not as big and strong as we had hoped, but she's not losing weight and not losing skills. My tendency is to have such incredibly high standards for her and myself that I struggle so much to breathe and remember how wonderful this summer has been.

It's a good life. It is.