Elijah's Journey

Please pray for Elijah at Elijah's Journey. He is in the late stages of late infantile Batten's disease and he is a very, very sick little boy. Pray for his family and that they make it through this tough time together.

Speech Therapy, Physical Therapy, AFOs, Enzymes, Updates!

The last time I made a real post about Maisie was over a month ago. October 1. So much happens in a month in this household, so this might be a long one.

First, Maisie has been cleared by her many doctors to start therapy again. She also had a follow-up with the endocrinologist, who cleared her to return to "normal" feeding with the addition a number of digestive enzymes, which she takes through the tube before every meal/snack. She is still tiny, weighing just about 25 pounds. Which is great and means she has gained weight, but I mean I've know five-month-old babies who weigh about that. A lot of people hear that and wonder why she isn't failure to thrive. She's only 29 inches front head to heel. She's just tiny in every sense of the word. She's not even on the charts, but her pediatrician isn't concerned because she gains about two ounces a week. So she's growing. Completely at her own pace, on her own chart. But she's growing.

Anyway, now that her health has stabilized finally, I'm really concerned about getting her on track developmentally. Two weeks ago we met with a child developmental specialist who put her at about 18 months old. She catalogued Maisie's entire vocabulary (thirteen words), assessed her motor skills, gave us some vague goals for each category, and recommended speech/language therapy and physical therapy as well as preschool three days a week. Maisie returned to the Open Arms school last Monday, enrolled in the three-year-olds' program. She's going on Mondays, Wednesdays, and Fridays for four hours a day. On Sundays and Thursdays she has speech and language therapy, and Tuesdays she is returning to physical therapy with the much-beloved Drill Sergeant June.

All of this happened so fast. The specialist did not want to waste a second in getting Maisie out of the sedentary sick-girl lifestyle that she's been forced into with all of her health issues. She's five, and she should be active and social and laughing. I know this is going to be good for her, but it's been so hard. Maisie has to take enzymes before every meal and has to have a 100mg bolus feed through the tube every two hours. She is taking Keppra, a diuretic, an antidepressant (for anxiety-induced seizures), a beta-blocker (for kidney function), 24-hour Zyrtec, and Levalbuteral via nebulizer every day. So all of that has to get done before we walk to the preschool at 9:00 in the morning three times a week. Therapy days are easier because nothing starts before 11:00. Still, fitting in all this medication and finding time to clean the house and blend food for her and yada yada yada...I'm tired. But it seems to be a good fit if a rigorous routine.

What else is there...she's back in AFOs for 6 hours a day and doing an hour of stander time every day. I'm suffering from some serious morning sickness which does not go well with administering morning meds...I guess that's all. Hoping that we get into a routine and have an entirely unremarkable winter.

Halloween Strangeness

I KNOW! I keep saying I will update more regularly and then it doesn't happen and I'M SO SORRY. I'm a terrible blogger. But it's a hard life! I've got a perpetually-sick little one to take care of on top of being pregnant and miserable. Maisie is just starting speech therapy and physical therapy and a couple of new meds (more about all that later). It's been a crazy couple of weeks. Anyway. I think I can safely say that on Friday, I experienced one of the strangest events of mommyhood ever.

It should surprise no one that Friday was Halloween. It may, however, surprise some of my neighbors that, when you trick-or-treat, the "treats" are candy. Maisie did a little trick-or-treating in her little elephant costume and it was adorable, but we came home after only twenty minutes or so because she was exhausted. So from 6:30 to eight we were fielding trick-or-treaters of our own. And around 7:00, a group of five kids between the ages of three and eleven showed up. They all cried "Trick or treat!" and I pulled out the candy bowl and the oldest kid looked me in the eye and politely asked for the non-food options. That is exactly what he said. "Can you bring out the non-food options?"

Um.

No?

Was I supposed to prepare a bowl of toys too?

I know some moms will have little plastic teeth and stuff in with the candy, but I always hated cheap, useless knick-knacky things like that. "I don't have anything but candy!" I said with a big smile.

And then. The sanctimommy. Oh. My. God.

This women march up and give me the full lecture on the toxicity of store-bought candy, the obesity rates in the US and the reasons behind them, the nutritional needs of kids, and the inconsideration and narrow-mindedness of not providing something other than comestibles for trick-or-treaters. What about the kids with allergies? What about the mothers who actually care about what their children consume instead of shoving over-processed, nutritionally-poor crap "food" into their kids? Have I ever stopped to think that not all kids are as lucky as mine must be, and that not all kids can eat whatever they damn well please?

At this point I was FURIOUS. I looked her square in the eye and said "What about the children who are tube fed? What about the children who can't eat anything at all?" And then I called for Maisie. I lifted up her pajama top and showed these women the g-tube that keeps her alive. Yes. I know about the kids who can't eat whatever they want. I know about the kids with allergies. I know about the kids with dietary restrictions. I HAVE ONE.

She shut up and hustled her kids away. But it left me thinking. This was the first Halloween that Maisie was actually able to participate in. She had very strict dietary restrictions up until about ten months ago. There have been stretches of time where she couldn't eat or drink ANYTHING because her kidneys couldn't take it. There have been times where she just refused to eat. There have been times where she could only eat very short list of things. And we celebrated Halloween in other ways. With decorations and pumpkins and stuff that was safe for her to eat or drink. We didn't freaking take her trick or treating where she would have to walk away from almost every house empty-handed because she couldn't eat sugar or chocolate or dairy or dye or nuts or whatever. That's just cruel.

I'm over it now, obviously, and the whole thing was just weird. But I thought my fellow special needs mamas would like to hear that story. Anything like that happen on your Halloween? Or any other holiday? Or anything at all? I want to hear from my readers, I don't get any comments!!

Noelle part two

Sorry this took so long to get up! To make up for it, I have a great Halloween story. Here's part two of Noelle's story. If you didn't catch the last one, you can read that here. At the end of that post, her mom had just talked about the realization that at two years old Noelle was not ready to learn how to walk, despite being technically physically capable with a prosthetic.

As it became clear that Noelle was not going to learn to walk anytime soon, we struggled to give her the freedom of mobility that a limb-normal child would have. After an MRI and a handful of physical therapy and orthopedist appointments, she stopped wearing a brace on that knee. It was clear that the alignment was corrected and it was developing normally, so we freed her from her brace and quickly discovered that she relied on the weight and length of the brace for much of her mobility. The old brace, a complicated contraption that was irritating to put on, wear, clean, etc., was replaced with something similar to the brace she had as a baby, a soft log weighted to make up the difference between her little leg and her normal leg. Instead of being attached to a brace, the log was glued to the sole of a shoe. Noelle loved having her knee free and immediately began developing new ways of exploring her surroundings. She quickly figured out how climb into chairs and onto the couch. After just a few months she was able to stand up without any kind of assistance by crouching and straightening her right leg as quickly as possible while lifting her left leg so the prosthetic didn't drag her down. Then she would slowly straighten her left leg so the prosthetic could hang straight down and she could put her weight on the flat end of the prosthetic. Because the "prosthetic" was really just a cylinder of some kind of foam or gel or something in a purple Spandex sleeve, it collapsed if she applied too much pressure, so she learned to put very little weight on the leg and really just use it for balance. When I saw her standing up like this, I sewed a rubber patch on the bottom of the cylinder to keep her from slipping on wood floors. Once she was able to stand up quickly and fluidly in this way, Noelle was able to open doorknobs and pull all kinds of things off tables. We learned quickly to keep everything at least six inches from the edge, further as she grew.

Up until this point we had hardly paid any attention to the way Noelle was using her arms. Her left arm is amputated just above the elbow and her right arm is about four inches shorter than it should be, and the hand on that arm is deformed. The fingers are fused and sort of squished together and overlapping. The thumb joint is rolled in, forming a lump on the right side of her palm (when the palm is turned toward her face.) So she doesn't have any real hands, but she does have a kind of a scoop on the right. When we started paying attention, we noticed that she had figured out on her own how to pick objects up by pinching them between her short arm and her hand. She also was able to scoop small things like blocks into her hand and carry them around. Almost the second we gave her some dry Cheerios to experiment with, she was able to scoop them up and pour them into her mouth. That gave us the idea to glue the bowl of a rubber spoon to an elastic band, which we could slip over her hand so she could eat liquid-y things like cereal and soup. This worked so well that we became extremely skilled in the art of glueing rubber bands to toothbrushes, spoons, forks, toys, etc. so that Noelle could use her little hand to it's greatest potential.

Today, Noelle is a bright and beautiful four-year-old. About a year ago she saw a girl walking through the mall on a prosthetic leg and asked why she didn't have one. We talked it over (she didn't remember how much she hated the pseudo-prosthetics she had as a two-year-old) and she said she wanted to try a prosthesis. So today, Noelle has a prosthetic leg that attached to her left foot with a special shoe. She continues to use the soft support, and alternates between the two, depending on what she wants to do. Sometimes she doesn't wear anything at all. She can even take the prosthetic off and put the soft support on, although she hasn't quite managed to get the prosthetic on herself. She does not wear a prosthetic arm at all because she can already do so much. She also does a lot with her right foot, like drawing and painting. She has developed extraordinarily prehensile toes, and uses that foot to put on clothes and her prosthetics, and she's trying to figure out how to tie her shoes with it. She never ceases to amaze us with her dexterity, innovation, and endless optimism. I am unable to think of a single thing any other four-year-old could do that Noelle can't.

In terms of cystic fibrosis, she has been very healthy. Almost bizarrely healthy. We are absolutely RELIGIOUS about her treatments and make sure every beating-vest session and nebulizer treatment are used to their full capacity. In fact, she doesn't even seem sick most of the time, and since we moved and started seeing another pediatrician, we are questioning whether or not she really has cystic fibrosis. I will let Char know and update if I have any news on that front!

Thank you so much for giving me this opportunity, Charlotte! It's been fun (and heartbreaking) reliving and remembering our journey with Noelle, and I hope our experiences will be educational for new moms of kids with limb-differences.

Noelle part One

This is part one of two posts about a little girl Maisie and I met while she was hospitalized for g-tube care. When you spend a large amount of time in a pediatric ward or children's hospital, you meet a lot of moms who are a lot like you. It's a unique place, one of the few places you can have candid conversations about g-tube care or dressing changes. At our last visit to NJ children's hospital, Maisie's roommate was a four-year-old named Noelle who, aside from having cystic fibrosis, was born with limb differences affected three limbs. Her left leg is significantly shorter than it should be (though it is jointed) and the toes on the foot are short and fused. Her right leg is normal. Her left arm is amputated just below the elbow, and her right arm is slightly shorter than it should be, with a deformed hand.

All these things aside, Noelleis a beautiful, bright, and functional little girl. I asked her mom to write a little something for the blog, so here it is.


Hi Charlotte! Thank you so much for featuring my sweet Noelle on your blog! We are both so honored. Our story begins almost exactly two months before Noelle's due date. We didn't want to find out the gender until the baby was born, so we had only planned on two ultrasounds, one at the beginning of the pregnancy and one at the beginning of the third trimester, just to make sure everything was working the way it should. So we didn't find out that there was anything special about Noelle until she was at 32 weeks. The ultrasound tech told us to stay put and the doctor would be right with us. She raced out of the room and my husband and I just kind of looked at each other. This was our first baby so neither of us had any experience, although this hadn't happened at the first ultrasound. The OB/GYN sat us down and explained that our baby was developing differently. Because of the position she was in, he didn't really know what was going on other than that she didn't look like she should. So he wanted us to come in next week for another ultrasound. I was in shock, and assuming the worst, and he was so kind. He said, "it could be nothing, it could be something very minor, but there is a small chance we have a big problem. We're being cautious. At this time, there's nothing to worry about."

I was so scared. Over the next few weeks we learned a little more about her arms, and we came to a conclusion that she had limb differences isolated to her arms. You can imagine our shock when she was born with her little leg, too! We loved her completely and instantly, and her limb differences meant nothing to us.

She was a very small baby despite being full-term, weighing only 4 lbs 9 oz at birth. She spent four days in the NICU to make sure she had no other complications which often come with limb differences. We found out that she has fibular hemimelia and congenital short femur. What that means is she has no fibula in her left leg and that femur is very short. When we brought her in for her 1 month well-baby visit, the doctor commented on the strange shape of her left leg, not just it's length but that the knee didn't connected properly. She sent us to an orthopedist, who prescribed her first brace. She wore that brace for 22 hours a day for four months to correct the placement and development of her leg.

At this point our bigger concern was that she wasn't growing. Though she was four months old, she weighed barely 6 pounds. The pediatrician had insisted for the first few months of her life that she because she wasn't growing in height, her lack of weight gain wasn't all that concerning. She was a healthy weight for a newborn. But she wasn't a newborn. At four months the pediatrician finally agreed that something needed to be done. She prescribed a high-calorie formula to be mixed with equal parts breast milk and given to Noelle every two hours. It did very little. At five months, she weighed in at 6 lb, 5 oz. We were referred to a geneticist, but before our appointment Noelle developed a horrible cough. She was hacking up mucous and struggling to breathe. That's when the pieces clicked together and we received the CF diagnosis.

Noelle began breathing treatments and taking 12 different pills every day. Cystic fibrosis was a devastating diagnosis, and my husband and I grieved the loss of a healthy-if-physically-different child. However, she has a relatively mild form of CF and it was brought under control fairly easily. In the end her limb differences became the bigger problem. When she outgrew her first brace, the orthotist designed a brace with a soft, weighted log like a sack of rice on the end that he hoped would mimic the balancing action of a baby's leg and allow her to learn to sit and crawl properly, and hopefully even encourage walking. I don't know if she would have learned to do those things without the brace, but she was crawling, sitting, and doing a kind of one-legged balancing act to reach higher objects by seven or eight months old. At ten months she was fitted for a combination brace/lifted shoe and told not to intervene until she showed signs of being ready to walk.

For months she crawled and crab-walked on three legs. Around sixteen or seventeen months she figured out that if she could balance on the right leg, she could use the brace/shoe to balance herself. At first we thought this was a good sign, an indicator of good things to come. But months went by and she didn't develop any new skills. At her second birthday party I watched as she stood to reach a toy on the shelf and then plopped onto her butt and crabwalked across the room. She was very mobile, but she wasn't walking. It was unfathomable to me. She had two legs, right? Lots of kids walk with a prosthetic and this was wayy simpler than that. Why wasn't she walking?

I am not proud of this next part of her story. I was angry and frustrated. I had let myself believe she would learn to walk just like every other kid, and that walking was the only solution, the only goal. After that party, I dragged her back to the orthopedist. I demanded to know why she wasn't walking, I insisted that she must have another limitation we didn't know about. He told me simply that she wasn't ready, and if it was meant to be, it would happen when it happened.

So I took her to a child psychologist, and basically screamed "MAKE MY KID WALK."

I am embarrassed to write that, Charlotte. It is now clear to me that, at two years old, Noelle was not ready to learn how to walk. She was navigating her world beautifully and developing normally in every other way. I hadn't realized just how much she had struggled to get to where she was then in terms of crawling and standing. I hadn't realized how hard she was already working. At that time, I refused to take no for an answer. After talking with Noelle, she asked to speak to me by myself. I had to come back a week later. That conversation opened my eyes. I finally accepted that Noelle was not walking now, and maybe not ever. If and when she was ready, she would figure it out.

Back in Action

Oh fall, as always you have brought us such joy, such healing.

I apologize for my extended absence; we have had a hard few months. Weeks ago I tried to write this post but I just couldn't get through it. I decided to publish it as-is so maybe you can understand why I was gone so long. When we finally got done with treatment for HUS and getting her tube fixed, she was sick and miserable I figured she was just healing and recovering and exhausted. When she developed a 101 fever, I figured she had picked up a virus. After a week, I was worried. After eight days, she had a seizure like I have never seen before. Normally her seizures are myoclonic, isolated to one hand or foot or facial muscle. I was standing in the kitchen making dinner or something when she suddenly toppled sideways on the couch from a sitting position. Then she collapsed off the couch and shook violently on the floor, groaning and and drooling. I rushed to her; she was unresponsive with open eyes. I called 911, but by the time the paramedics arrived she was herself again. They advised that it was probably a febrile (fever) seizure and left. After the second seizure of this magnitude, I made an appointment with the neurologist for a few days later. She had two more seizures before the appointment.

Neurology switched her to Keppra, which stopped the seizures but made her quiet and nauseous. She threw up a couple of times, she had a fever and her chest and throat were rubbed raw from coughing. And she often complained of "hurting, Mama." After looking up the side effects of Keppra I called the neurologist about twelve times before I finally got them to switch her to something else. The nasty side effects went away, but she was still complaining of a vague and occasionally excruciating pain.

We are masking her pain with a powerful prescription and trying to pin down a more definite source so we can get her a proper treatment. The long and the short of it is that we've had a rough summer, but it is now fall and as the blistering heat has faded away, so has Maisie's many woes, at least a bit. On the first day of fall, just a week ago, my doctor confirmed wonderful news.

I'm pregnant.

So that's nice.

A rough month

August has not been kind to my Maisie the Great. She's been getting progressively sicker since they we first came home from the hospital (kind of ironic.) After the nephrologist took her off all her medicine and she had to be rushed to the PICU for emergency respiratory failure, uncontrollable seizures, and severe pain. At first, we were able to control her seizures and her breathing long enough for her to get over HUS. She was doing really well for a few days, we took her home and then had the feeding tube fiasco and finally got home and were resting for, oh, five hours before she started seizing violently. These were not her normal seizures, just a few seconds long and/or isolated to a single hand/foot/facial muscle. These were full-body, tonic clonic or grand mal seizures. It was terrifying. She would pitch forward and shake from her head to her foot, foam at the mouth, her eyes would roll, and all I could do was hold her. This went on for four days. I called the epilepsy neurologist as soon as it became clear this wasn't some random, one-off thing, and she changed her medicine. We picked up the new prescription the next day, and it reduced the grand mal seizures but only to the extent that she is now constantly seizing in one hand, foot, eye, lip, cheek, something. The neurologist cannot see her because another patient is a state of emergency. His life is threatened, and our doctor needs to throw her whole weight behind this little boy. That's okay.

Maisie is in a lot of obvious pain. She cries almost constantly and has stopped speaking for the most part. As I write this my poor baby is slumped over my lap, wailing. I can do nothing for her. We don't know what causes her pain.