Dialysis

I've been meaning to do this for a while, but I keep forgetting.

A good friend of mine, S, has a daughter K who is on dialysis three times a week. She has almost no kidney function left and so her blood must be cleaned by a machine, always. She can't do it herself. I've posted a few things about Maisie's experiences with dialysis over the past few months, and a few weeks ago I got a call from S, who was outraged to hear how different Maisie's experiences with dialysis are from K's. I explained to her what I'm going to explain now, for all of you who have experience with dialysis and know how difficult it can be.

First of all, Maisie has 70% function in her kidneys (basically she can filter our 70% of the gunk in her blood on her own, while most people can filter out 100% of the gunk). Her kidneys can clean her blood on their own without help, but they are weak. They are easily exhausted. Sometimes when she gets sick she can't get better because her blood is not as clean as it should be. Whenever she is in the hospital long-term for some other reason, she is also on dialysis a lot.  Most kids with kidney failure go in three times a week for three to four hours. There have been stretches of time where we have done this with Maisie for various reasons, but it is exhausting. It's strenuous on the child and the family. I have heard S's horror stories and am filled with awe for those moms who take their child to the hospital for nine hours or more EVERY SINGLE WEEK. These kids have tubing in their arms constantly. They can't take showers or swim. Changing the bandage is a nightmare because the environment must be completely sterile. Without dialysis, these children will die.

This is not the case with Maisie. She is in a unique situation where she is capable of cleaning her own blood, but it is unusually taxing for her. Whenever her body is compromised by illness or something else, it is often helpful for her to be on dialysis for 24 hours. We find that a full day of cleaning once in a while works better for her and for our family than coming in every week did. It was too much pain for Maisie when she could survive pretty well without it.

I also know that most hospitals don't have enough dialysis machines to allow one child to use it for twenty-four hours periods. I'm sorry for that. The only thing I can say about that is we go to a small, private hospital with enough machines to allow Maisie one for twenty-four hours. It's that simple. If she were taking it away from a child who needed to survive, I would never allow her to use it for so long. Rest assured that I have checked many times that no one else needs her machine more urgently. And several times the machine has been taken away because someone else needs it. And that's fine. They need it more.

Potty Training

Can't sleep, so I thought I'd talk about our experience potty-training Maisie. I remember vividly my own potty-training experience, which is probably not good. It was traumatizing. I was almost two when my mom, pressured by her friends whose children were already potty-trained, decided she had to buckle down and do it. But I wasn't ready. I couldn't control my bowels. I just could not do it. I was so frustrated. I felt like a failure. I was two. It was awful. I have, as you can imagine, very defiant beliefs about potty-training. When your kid is ready, it will happen. When he's not, don't bother trying. So I planned to wait however long it took for Maisie to be ready, be it two years or even five.

A few months before her third birthday, a dear family friend of ours was getting married. This was a girl two years younger than me who I grew up next door to along with her two sisters. When she got married, I was asked to be a bridesmaid. I was delighted. Two weeks before the wedding we flew out to Colorado to help her get last-minute things ready. Well, it so happened that her older sister had a two-year-old girl who was potty training. Maisie was fascinated by her plastic potty (we didn't have one) and how she used it. She decided after three days that she wanted a potty to use. She was very excited. My dear friends were happy to let Maisie potty train then and there. Unfortunately, it didn't work. Maisie could not seem to do it. She would announce she had to go to the bathroom, rush to the potty, get her pull-up and everything off, plop down on the potty, sit for like ten minutes, and then finally say "Never mind," and get up. The moment her pull-up was in place she'd pee and burst into tears. "Didn't do potty! Didn't do potty!" She was so upset, it was awful. She was mentally ready. She wanted to use the potty, but somehow she couldn't.

Well, we convinced her to give it up until we got home. But it was no better there. Finally we decided to take her to the pediatrician, afraid that this was a sign of some new health issue. Thankfully, the doctor found nothing. Nothing in her blood or urine or even in the MRI we had done a month later (for another reason but they checked it for any urinary/digestive obstructions anyway). We were relieved. And a month later, Maisie ran into the living room shouting, "Mama! I did it!"

The front of her pants was soaked. She hadn't taken them off before she sat down.

But close enough.

Vaccines

Hello everyone!

We've been having a very fun, restful week, but unfortunately it ends tomorrow. Maisie has to go back to the hospital to continue testing and treatment tomorrow morning. 

Today, however, I want to talk about vaccines.

There are two reasons I'm bringing this up

1. A dear friend of ours had a baby a few weeks ago and recently announced that she would not be vaccinating the infant. 

2. One of the girls Maisie roomed with during our last hospital stay died last night of meningitis.

It is true that Maisie was not vaccinated against anything until she was eleven months old (except whooping cough - Charlotte got the vaccine during her pregnancy). This was only because she was too weak to defeat the weak/dead pathogens in the vaccines until that point. We were afraid that, far from protecting her, she would get very sick. She has now been vaccinated against all the usual things (hepatitis, chickenpox, diptheria, meningitis, measles, mumps, flu, polio, pertussis, rubella, pneumonococcus...I'm missing a few). She did contract some interpretation of measles and pneumonia very briefly, but she was hospitalized for two days, given some assistance in fighting the weak pathogens, and was fine. 

My friend, who will remain nameless, is choosing not to vaccinate because her older son was vaccinated and now has autism. She believes, as so many do, that the immunizations cause ASD (autism spectrum disorder). This is not true. There is a wealth of scientific information to back this up, as you can read here, and, if you need any more convincing, my niece was not vaccinated against anything until the age of two but was born with autism. There is no connection between vaccinations and autism. Furthermore, as this beautiful and eloquent young lady says, autism is not a death sentence. These vaccine-preventable disease could be.

To my darling second-time mother L, please listen to me. I cannot respect your decision because it means the end of our contact. I cannot risk exposing my weak granddaughter, who already has a compromised immune system, to the germs and bacteria that could be carried by your precious baby. By not vaccinating sweet little D, you are not only risking the health of your baby, which is one thing, but also the health of all children who could contract these hideous diseases from D. Please listen to reason and take that bundle of joy for her vaccines.

I do not mean to be disrespectful or rude. I understand there are health complications that can delay or prevent vaccines. But if you have a healthy child, or even an unhealthy one, vaccinate as soon as you can.

Thank you.

Eleanor.

Merry Christmas!!!

Today

there is no illness in my house

Today

there is no pain

no needles

no dialysis

no diagnostics

Today there is no suffering

Today there is a four year old

Who is four

Not four-going-on-twenty

But four

Not suffering four

Not sad four

Not scared four

Not damaged four

But four

Just

Four

About a half an hour ago, Maisie keeled over and fell asleep, facefirst, into a pile of used wrapping paper and new socks on an armchair, clutching three new stuffed animals. Jeff just carried her upstairs and tucked her into bed. I think this scene speaks to the success of our Christmas Day. She about wet herself when she started unwrapping presents, she was so excited. Mom, Dad, Jeff, and I just sat around with cups of coffee for hours watching her play. She is the best. This is a season of hope, but sometimes in the fluorescent light of hospitals and doctor's offices, I forget about the hope. I see doctors and surgeries and medicine and pain in the future, but I don't see hope.

But she does. She always does. Maisie is smart. She knows that her future hold pain, sorrow, suffering. She knows that her future is undetermined, she knows that her life is not the same as it is for her classmates and friends (except Kaitlynn) but she also knows that there is always hope. She never loses sight of the good things and the happy things. She sees them, always and everywhere, even during intense suffering. She sees hope. She is hope.

The Eye of the Storm

As y'all probably know, Christmas is in (gasp!) three days! We're taking Maisie home today so she can celebrate Christmas with us. We'll spend the next three days decorating, baking cookies, shopping, wrapping presents, unwrapping presents, etc. In light of her recent struggles, I am reminded how important it is to celebrate every moment of Maisie's life. Any Christmas could be her last. Any birthday could be her last. It's worth it to make sure she spends her days, even if they are numbered, full of joy and glitter and sparkling lights.

This morning Jeff and I went Christmas shopping for Maisie while Mom helped her get ready to go home. She's had a huge Doc McStuffins thing going on for a while now so we got her a pile of Doc stuff.

 

<--Right now this is sitting in the basement waiting to be wrapped (we took it out of the box for ease of playing on Christmas morning. It's worked better that way in the past). Those are her "patients" over there -- I don't have actual pictures but we got all those guys for her. And then there's a Doc McStuffins doctor's outfit and some more tools and a clipboard thing. I know she'll be thrilled. And hopefully these things are small enough that she can take them to the hospital to cheer up the long, dreary hospital visits that I know are coming. I hope this Christmas is a dream come true for Maisie, but I know it's just the calm eye of the storm. But if I have anything to do with it, it's going to be a festive, spirited happy-happy-happy calm eye.

Adam & Maisie Update!

Hey everybody!

I just got a call from Anna about Adam. Tomorrow he will be six weeks old and so far he is doing great. His heart rate has stabilized and in three weeks he's gained over a pound! Yay, Adam! So they'll be heading home soon and hopefully everything will go just smashingly!

Back to Maisie-the-Great: We met with an orthopedist this morning. Although Maisie's problem is not orthopedic, exactly, his expertise in the area of supporting and controlling the leg bones and muscles will help us support Maisie's limbs so she can continue walking and running and jumping and skipping. He is suggesting having ankle-foot orthopedic braces (AFOs) ordered to help her on days (like the other day) when she is particularly weak. Interestingly enough, he does NOT want her to wear them during physical therapy so that the exercises might help her muscles grow stronger.

We also met with a rare disease specialist, a dietician, and an endocrinologist. The endocrinologist and RDS have presented an interesting theory based on the evidence that Maisie has some days where she seems totally fine and other days where she can barely hold her head up (this has always been true, but we always attributed it to lethargy rather than hypotonia). They think it's possible that the heart of this problem is that Maisie is not regularly producing the proper proteins and wish to continue to study and test her (God I hate how gross and evil that sounds) to see if there is enough evidence to support this theory. The good news is that if this condition is not excessively severe, her problems might be solved with a high-protein diet and possibly some medication. Sorry this post is so scientific and medical, but I want to keep all my far-away friends and relatives appraised. I'll get some more fun stuff up later.

Rag Doll

So a few hours ago we met with a rare disease specialist and a neurologist, both of whom had thoroughly examined Maisie and conferred over their findings. They both agree that what Maisie is suffering from is acquired hypotonia, which means very low muscle tone not from an inherited genetic disease but from an outside influence. Normally the term "acquired" means it happened after birth, but this is just another example of how rare and unique Maisie is. She probably did not inherit these conditions through a genetic mutation but instead was affected in utero. By an outside source.

Maisie is still young. Her brain is still growing and developing, and (though, as you'll see if you read posts from 2009, we didn't realize it at the time) has been damaged by the attack on me during her gestation. There is every chance that she could develop further complications as she grows and changes, and it's possible that some of these could be headed off, prevented or diminished if we know more about them. For this reason the neurologist suggested, and I agreed, that we go ahead with several different diagnostic tests, such as fluoroscopy, X-rays, neuro exam, and possibly a CT and brain biopsy. Unfortunately this means Maisie will be spending quite a bit of time in the hospital over the next few days, but it might also save her life.

Now, back to the original reason we came to the hospital. It would appear that her the muscles in her limbs are not growing stronger. In fact, they may be weakening. We are hopeful that a change in the physical therapy regimen and her diet will help her grow stronger, but if the root source is a damaged nerve in her brain, it's hard to know what the future holds. We have an appointment with an orthopedist in a few days to talk about bracing and other support devices. For now, she's the same little girl she's always been.

There have been times in the past where developments in Maisie's health have pushed me over the edge, completely overwhelming and frightening me. I have had episodes of extreme guilt, depression, panic, and terror, but this is not one of them. I have been remarkably calm and collected through this whole process. I am well aware of the risks and challenges ahead, but they do not frighten me as I thought they would. Maisie has proved herself to be strong and determined to survive. I am determined not to dwell on the seriousness of her condition. I will absolutely fight and continue to fight for the best care for Maisie, but at the same time I want to enjoy her for who she is right now, today, even if she is a limp, sick rag doll baby. She is mine and I am hers, and nothing, not even death, can take that away from me.

A Little Background

I just met with a lovely man named Dr. G, who specializes in rare diseases. But before I tell you about his findings and theories, I realized I need to give you a little background.

You know already that Maisie has low-functioning kidneys and half of her left lung just doesn't work. She suffered from severe calcium deficiency as a baby because she could not digest lactose and thus has some funky deformed bones (bowed legs, crooked fingers). Some of these things are direct results from the attack, but many of them are side affects of the extensive nerve damage she sustained. I just want to make sure I've listed all the relatively little things that she deals with on a regular basis.

1. Vision. While she is not blind, we don't think Maisie's eyes work quite the way they should. She doesn't have the ability to see in the dark, for instance. Her eyes simply don't adjust to low light. She will also need glasses as she gets older because her cornea (the flexible lens over her eye that controls focus) is doing what everyone's does as they age: stiffening. Most people don't have an issue with stiff corneas until they are elderly, but Maisie's, for whatever reason, is growing stiffer and stiffer and in the next year or so she will start wearing glasses.

2. Seizures. Her whole life Maisie has had "simple partial seizures," which basically means one of her hands or feet will suddenly start shaking violently for about five minutes, randomly. There is nothing we can do about this. A wide variety of drugs have been tested on Maisie but she has responded to nothing. We believe that these are caused by a damaged nerve in Maisie's brain.

3. Hypotonia. Maisie was what is called a "rag doll baby," which means she had very low muscle tone for a long time. This is partly the reason she did not walk for three years, and she has been in Early Intervention (a baby physical therapy program) since she was just three months old to help strengthen her. Today Maisie has low muscle tone but not to the point that it impairs her. She can walk and run and jump and skip. Her arms are weak but strengthening, and she tires easily.

4. Digestion. The best way to describe the problems Maisie has with her digestive track, and indeed the way her doctors describe it, is "gastroenterological dysfunction." She suffers/has suffered from a variety of ailments that are random and sometimes go away on their own and sometimes require intervention, including high acid production, weak/thin stomach tissue, weak smooth (involuntary organ) muscles, enzyme imbalance, and inappropriate smooth muscle contraction. All of these things cause stomach aches, vomiting, constipation...basically any digestive issue you can think of, Maisie's had it. We are constantly adjusting her medication to suit her current needs and always on the lookout for issues. Any of these things could prove life-threatening. Indeed, her stomach is the scariest thing Maisie has to deal with.

5. Tetanus. When Maisie and I were attacked, I was stabbed by some kind of metal blade. Luckily I had been recently inoculated against tetanus, but Maisie, being a fetus, never had. She contracted neonatal tetanus and has been treated for it twice. We have no way to know whether this will continue to be a problem in later years or if it will eventually go away completely. But we are always watching for muscle stiffness, difficulty swallowing, fevers, accelerated heart rate, all symptoms of tetanus, all very frightening.

Not all of these little things are a problem all the time. Many, like the hypotonia, tetanus, and calcium deficiency, have been dealt with and (hopefully) are completely behind us. But, as you've seen if you've been around a while, Maisie is growing and changing constantly. Her rapid development from a baby to a girl coupled with her highly unique situation, means we can hardly begin to guess what the future holds for her.

Update from the ER

Hello hello hello!

Sorry it's been so long! My last post apparently didn't go up (Baby Back Ribs -- I just posted it). We've had such a wonderful few weeks that I just wanted to cuddle with my baby, make cookies, paint the office, and relax. Jeff and I have been apartment hunting but I don't know if we'll move anytime soon. I've felt so content, I don't want to change anything.

Maisie has been doing SO WELL! She's been eating, not puking, no seizures (did I talk about seizures? I'll get there), no fevers, nothing. It's been simply wonderful.

And then, about two week ago, she started walking funny. Normally she walks just a little weird, hardly noticeable unless you watch her for a while and really pay attention. Her left leg is misaligned ever so slightly. It's a problem we're working on in physical therapy, and slowly, slowly, s l o w l y it's been improving. But this was something new. It was almost like her right leg kept collapsing underneath her. I called her GP and she thought Maisie might have just strained a muscle and to call her back if it didn't go away in a week or so. It didn't, and then something else. Jeff's mom came to visit from Maine and when I brought Maisie to meet her she asked if she was blind. I was totally taken aback. As far as we knew her vision was virtually unaffected. But Martha pointed out that her eyes were not "tracking" or following people and things as well as they should. I called the GP again and this time made an appointment for a few days later but two hours or so ago she collapsed when I set her on the ground after taking her out of the crib (she still sleeps in a crib for safety -- I'll explain about that later). It actually took a few tries for her to stand up. And then she collapsed again on the kitchen floor. She wasn't fainting, but simply falling straight down. Her legs weren't supporting her. It was really scary. Of course, this is the week we don't have PT because the therapist is away with her sick ma.

I was home alone. My daughter was wailing and could not seem to stand up. I decided to take her to the ER. So here we are. Right now they're running diagnostics and performing tests on her muscles and eyes, trying to figure out what's going on. If it is a muscular failure, which is the working theory, her muscular organs could be in danger, and, in turn, her life.

It's funny. I'm not afraid. In the past I have sat in this very chair in the ER waiting room and panicked about my little daughter. But I feel weirdly calm. I am under control. I know that I am doing the very best thing for my Maisie, and I know that she is being taken care of. I have no false sense of security -- I know that she may not be okay. But I have enjoyed her fully and loved her to the bottom of my heart and so if this is it, though it would break my heart, I know that I loved her as much as I could. And she knows that.

Baby's Back Ribs

It's been a long, boring, rainy day and I just remembered that I promised I'd post about Maisie's past. So I think I'll do that right now, starting 10 months in when she had open-back surgery. She's had six major surgeries over the course of her life, but the first one was when she was ten months old. Up until then she was breathing with a CPAP machine (box thing with a face mask that uses air pressure to keep her airways open). The pulmonologist couldn't take her off it because every time he did her lungs collapsed. For ten months she breathed on a CPAP, and then one day I got a call from Dr. Livesay.

"Hi, Charlotte. I think I figured it out. Feel Maisie's back. It's kind of concave, right?"

And just like that, we had a diagnosis. Bowed Ribs by way of Calcium Deficiency. Not very scientific, but it worked for me and it worked for Dr. Livesay. I'm sure the orthopedist had some fancier word, but that's what we called it. Bowed Ribs by Way of Calcium Deficiency.

I should probably mention the calcium thing. I think I forgot to. Maisie was for a very long time, for lack of a better word, lactose intolerant. She was never nursed on my milk (even though we tried) because she could not digest it. She didn't react to it, exactly, but she didn't do anything else with it either. She drank it, then it was awkward for a little bit while it sat in her stomach, and then she puked it all up. At around 8 months we discovered that she was producing about half as much lactase (the enzyme that digests lactose) as she should be and the problem was remedied by injecting her every morning with motivation for lactase production. Some kind of medicine. I forget specifically what it's called (in case you haven't noticed by now, I have a terrible memory. I can't keep all these scientific names straight in my head. I have a notebook with all of them written down because I just can't remember them).

Anyway, she had some weird-ass bone deformation for a while there. The bones in her ankles were a little wacky (kind of didn't fit together properly and her feet were sort of sideways-ish) and a couple of her fingers have very confusing bends and twists. She's also a little adorably bow-legged. A lot of these things we sorted out with minor surgery and bracing when she was a baby. Her legs are still bowed (no need to undergo that major surgery - it won't impair her) and two fingers are twisty still because the orthopedist was afraid to fix them because the nerves and ligaments were all twisted around the bent bones. Her fingers worked, he pointed out, and if he tampered with them they'd be pretty but they may not function anymore. So we nixed that idea. Her feet had to be fixed quickly or she would not start walking. As it was she didn't walk until she didn't start standing until she was almost three. Oh and she has three implanted teeth and one just awkwardly missing because the calcium and vitamin D deficiency prevented them from coming in.

We'd thought that, by the time she was 10 months old, we had identified and started the process of fixing all her little bone problems. Apparently we overlooked one, because her back ribs curved in the wrong direction. And they were squishing her lungs. That was why she couldn't breathe!

It was so exciting - but also really scary. Because how were we going to fix that? The orthopedist called in two other doctors before suggesting that they take each rib out and turn it around. Just flip it. It was so simple, so elegant, and it worked like a charm.