Merry Trach-less New Year!!!

Yes, ladies and gentlemen, there has been no tracheostomy for Maisie the Great!

It's been a psychotic few days.

If you read my last post you know that she was scheduled for a tracheostomy on December 26.

This, friends, did not happen.

There was a scheduling mix-up (this happens WAY TOO OFTEN. Hospital people, GET IT TOGETHER!) and we were bumped back two days.

And then, by some crazy miracle, Maisie started breathing over the vent. Breathing on her own. Up until this point she had been riding the vent almost completely (allowing it to do all her breathing for her) and suddenly, she was working hard and struggling to breathe on her own.

It's obvious that it's very, very hard for her.

And she is still intubated.

BUT! The surgeon is going away for two weeks to celebrate the new year, and he and her pulmonologist want to give her another chance. So we're going to be in the hospital for the next few weeks. On January 14th, she will be re-evaluated.

Truth is, she will probably still get a trach.

But somehow, it means a lot to me that 2014 will not end with a tracheostomy.

2014 will end with hope.

Unfounded, ridiculous, illogical hope.

But hope.

Happy New Year :)

Christmas on the closed ward

Merry Christmas!!

I didn't actually take that picture but that's pretty much what Christmas in pediatrics looks like. 

There are baubles and IV poles, stockings and wheelchairs...it hasn't been very merry, to be honest.

I'm exhausted. I haven't been home in three days. One of the best Christmas presents I got was a shower in our next door neighbor's wheelchair-accessible shower, which is bigger and nicer than the cramped little thing in Maisie's room.

It was very sweet, it really was. They had a Santa come around just after the kids were woken up for middle-of-the-night blood draws and vitals checks, so they would hear a rustling and see Santa Claus delivering presents.

They all got stockings hung in their rooms that he filled, and the nurses had festive scrubs and there was even a Christmas tree.

And of course Maisie got some lovely presents. Jeff made sure of that.

It's all been overshadowed by the news we got this morning.

Yep.

After dilly-dallying for days, the doctors have finally decided that the best thing to do is trach Maisie so she can come home. It's become very clear that she can no longer breathe on her own.

The surgery is scheduled for midday tomorrow so she'll probably go back around dinnertime. 

*laughs* Hospital time.

I'm sorry this has been such a mleh post. I'm just feeling like a Debby Downer right now.

I want to go home. I want Maisie's lungs to do their fucking job. I don't want a trach.

....stiiiiiiilllll in the picu

Yep.

We're still in the PICU.

(this is going to be a long post, I've been trying to write it for a week. More stuff keeps happening.)

When I posted four days ago I thought this would be over-and-out in a matter of days. At the very least I was sure we would be out of the intensive care unit in a few hours.

I was very wrong.

Maisie is one sick little girl. They've confirmed pneumonia/pleurisy/pleural effusion. All that on top of her already-weak lungs is why we're still here. She isn't breathing on her own.

So she's still on the ventilator. She had to be intubated again around eleven Monday morning so she's still on the vent, but weaning. Slowly.

(I think I made a mistake in my earlier post; she was intubated in the ambulance, switched to a BiPAP around 8:30 in the morning, made it about two hours crashed, intubated. Looking back the timing in that post makes almost no sense. Forgive me, the ER is not the best place to get a good night's sleep.)

The pneumonia/pleurisy is being treated with two different antibiotics and Prednisone, which finally started making a difference today. She has a chest tube draining the pleural cavity and is on albuterol every four hours.

The vent is doing I think like seventeen breaths per minute, which is better than the thirty she was originally on, but still not great. Her regular breathing rate is 22 breaths per minute, so she's doing maybe five on her own. 

At this point she is resting comfortably and breathing fairly easily, if not by herself.

Ick, that sounds like she's dying.

She's not dying, folks. 

Even if her lungs fail her completely and she can no longer breathe on her own at all, she's not going to die.

That's the miracle of modern medicine.

My daughter is alive only because of the advances we have made in technology.

There's even this thing they can do where they cut a hole in your throat so a ventilator can breathe through your neck for you.

Yeah, I heard the word "tracheostomy" for the first time today.

We might be going there. I don't know for sure yet. Our pulmonologist is kind of young and has never prescribed a trach before, so she asked two other pulms who happened to be in the building today to pop in and evaluate Maisie. The three of them will pow-wow and then I'll get to say my piece, and we'll have a whole meeting with her "sick-team" which consists of the pulm(s), the pedi, child psychologist, and like four other doctors. 

^^^That meeting just happened (this post has been now seven days in the works). 

The consensus is no trach. Yet.

Her lungs have cleared up and she is still relying on the ventilator only because her muscles are too weak to move air into and out of her lungs on their own. At this point (three days after I posted about her breaths/minute) the vent is doing fifteen breaths per minute, and Maisie is taking seven on her own. Which is progress. I don't know if it's enough progress, but progress is progress.

Actually it might not be progress at all. On Sunday when she was intubated she was on thirty breaths per minute and not breathing on her own at all. On Monday afternoon we were able to take her down to seventeen breaths per minute (her regular respiratory rate is 22 breaths p/m) with her doing about five on her own. Now she's down to fifteen breaths per minute from the vent, but she might have been able to do seven breaths on her own from the beginning. Ah well.

The thought is to try to take her down as much as possible today. If she can get to eleven breaths from the vent and eleven breaths on her own, the trach will be completely off the table.

That's not to say if she can't make that much progress she'll be trached tomorrow. We'll re-assess tomorrow if that's the case. 

*le sigh*

OH! She is out of the PICU as of yesterday night though. Her lungs are clear, her vitals are good, and she's totally stable. So that's good news.

My new babe is seventeen weeks! Everything seems pretty good. I had an ultrasound while we are here and so far, so good. The tech was a little worried about her right arm, but she's pretty confident that she couldn't see it because of the position the baby was in.

Either way, I have a level 2 ultrasound scheduled for next week just as a precaution, given the complications around Maisie's pregnancy and birth, but I'm not worried. Too much worry is spent on my Maisie the Great, I guess.

PICU

UGH.

I am so sick of getting kicked in the butt every time we get into a groove. Things start going well, Maisie's making progress, yada yada yada, and then

BOOM

Respiratory arrest.

The chest x-ray showed a lot of fluid in the lungs and inflammation of the bronchial tree, but nothing too serious. The pulmonologist put her on twice a day Advair (which is a NIGHTMARE to administer btw because she doesn't inhale on command) and albuterol by nebulizer every four hours. For a couple of days we were doing that and all was going as well as could be expected, and then all of a sudden on Sunday night she was coughing and choking. She went into respiratory arrest and was admitted to the PICU through the ER at 10:00 on Sunday night.

It was a rough and scary night. She was intubated in the ambulance (which was TERRIFYING) and didn't get extubated until around eight this morning. They tried to take it out at one in the morning but she crashed almost immediately. Finally this morning they switched her to a ventilator breathing room air but with oxygen through the nasal cannula.

She still has a lot of fluid in and around her lungs. An hour ago they put in a chest tube to drain fluid from the pleural cavity and take a biopsy. They're also biopsying the mucus she's coughing up.

I will update again as soon as I have more info.

Tubes

Somebody has an oxygen tube.

WAAAHHH.

Maisie had a "breathing episode" yesterday that culminated in a trip to the ER. As you might know she had a lobectomy when she was a two and a half to remove the bottommost lobe of her right lung because it was nonfunctional and at risk for causing infections. Anyway, yesterday her sats were in the 60s and refused to go up after two nebulizer treatments and then they suddenly dropped into the 40s. I called 911 (she was BLUE). The only thing that got her oxygen sats back to the mid-80s was the oxygen tube in her nose. So that's where we are right now. Kind of exactly like this poor baby:

No idea how long the oxygen tube will be with us. We have a lung x-ray and a follow-up with the pulmonologist scheduled for tomorrow afternoon, and hopefully then we'll get some clarity.

UGH.

These feet were made for walkin'

WE HAVE WALKAGE!

Well, not exactly.

Princess Maisie spent the morning gripping the edge of the coffee table in her little Christmas footie pajamas, lifting one hand at a time and putting it back down. At first I thought she just liked the noise, but I realized that she was trying to stand so I put her little AFOs on, which look pretty much like this:

And fifteen minutes later she stood up! All by herself!!

And then she fell down.

And then stood up again after trying for like half an hour!

AND THEN SHE TOOK A STEP BEFORE SHE FELL DOWN!

It's the little things, guys.

Look ma, no hands!

I have felt energy from this little girl like nothing I've seen in years.

She was up and down, in and out, playing with everything and making a mess.

Maisie has been "walking" in a pediatric walker for weeks now. 

It looks a bit like this and gives her A LOT of support.

She needs it.

Her gross motor skills are pretty limited. She can stand holding the edge of a table or a hand or something. She can sit up. In the walker, she can move. But I've never seen her stand unassisted.

We started putting Christmas decorations up today.

Maisie was "helping" by using the various boxes to practice her standing.

I tossed a stuffed snowman out of a box.

She saw it, and turned toward it.

She let go of her box.

For maybe six seconds, she stood all by herself.

And then she was so surprised by what she had achieved that she fell over.

In sickness and in health

Last Friday I called my mom to let her know that I was in the hospital, and she immediately wanted to know what's wrong with Maisie. But nothing was wrong with Maisie, it was me! I was hospitalized after I spiked a 105 degree fever at around 2:00 on Friday morning. I woke up in the middle of the night freezing, shaking, and delirious. Then I vomited six or seven times and passed out and Jeff called 911. I'm fine now, they gave me an antibiotic and IV fluids and something for the fever and kept me overnight. Turns out I just have a very bad flu. I came home on Saturday night and I'm feeling worlds better today.

This post is about Jeff. My hero.

When I woke up on Saturday morning I was thinking clearly for the first time in 24 hours and I immediately freaked about Maisie. Had she gotten her meds? Her enzymes? Her meals? Her boluses? Did someone check and clean her stoma and change her button buddy? Had she been weighed?

The answer is yes.

Jeff was amazing. For twenty-four hours he took care of all of Maisie's many needs without me. He sent the ambulance to the hospital with our neighbor, a dear friend, to take care of me and stayed behind with Maisie. When she'd had all her medications and could just chill he switched places with Stacy, and stayed with me for a while.

This man. Jeff is amazing. He did not have to take care of Maisie as much as he has. He made himself her father because she did not have one. What a guy.

Thank you.

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.

Freedom! Except not

After a whole, wonderful, glorious five days out of the hospital, WE ARE BACK.

Not back to New York, but back to NJ children's.

G-tubes need to be changed every four to six months and usually you can do it at home and it takes like fifteen minutes. Unfortunately we are not so lucky as Maisie has been having problems with her stoma. She had a reaction to one of the antibiotics they put her on for HUS and it caused her skin to get swollen and rashy. Her stoma got so tight it popped the bubble that keeps it from falling out and the button fell out. And immediately the stoma started closing. After they took her off the antibiotics the reaction went down but the stoma remained almost closed. They sent us home with a new button and the high hope that the swelling would go down within the next couple of days. It did not. Poor Maisie was swollen, hungry, hurting, and losing weight every day. I called the doctors and we decided that the best thing to do is to have a surgeon at NJ children's drain and reshape her stoma and replace her button.

So here we are. They came in to take her about fifteen minutes ago, and until that point she was weepy and moaning and poking her belly and begging me to take her home. It broke my heart, but she'll be out of surgery soon and with a little luck we'll be home for dinner tonight.

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.

Steps forward

Whoa.

It's been like three weeks since I've been around the blogosphere.

But what a whirlwind those weeks were.


Ironically, right after I posted this Maisie developed a minor cough. We started all her regular treatments and maintenance meds for breathing and she seemed fine enough. On June sixth she went into the craziest coughing fit I've ever seen. Nothing -- not the nebulizer, not the inhaler, not patting her on the back or holding her upside-down (yes, really) -- calmed her down. She was coughing so hard she threw up twice and gave herself a seizure. After the seizure I rushed her to ER where she was given a steroidal anti-inflammatory. Turns out she had a pretty sever case of bronchitis, the symptoms of which we had kept under wraps with her breathing treatments. So she spent a few days in the hospital recovering from bronchitis and then a few more days sprawled on the couch looking a bit like that.

Since we were just at the pediatrician for a follow-up to the hospitalization, I want to give you a quick description update. She weighed in at 26 pounds and 34 inches. So she's growing!! She's still the size of a small two year old, but she's growing! She's been back to herself for the past week or so, and otherwise she's doing really really well.

So well that she took three steps on Sunday.

As you may know, she's been in physical therapy focusing on two things: walking with a walker and sitting up. For months she could not sit or walk at all, and after we started working with Drill Sergeant June she still could not sit up unsupported for more than a few seconds at a time, both because her balance and muscle tone were very poor. She could walk for three to five minutes in the walker. The longest she ever went before going completely limp was seven minutes. Over the past month, since she started treatment for myasthenia gravis, Maisie has been able to sit up on her own, with no swaying or toppling over, for up to half an hour at a time. She has worked up to ten to fifteen minute walker-walks and finally, on Sunday, we challenged her to walk. She looked so strong standing by herself, holding the edge of the coffee table and nothing more for support, in huge baggy shorts and nothing else because it was darn hot out. My princess, my angel, my Maisie the Great walked on Sunday. June is going to be so proud.

giving words

Oh, this little girl.

We have had a wonderful few days, quiet and peaceful

Her pain seems to have finally subsided with the addition of a new pain medicine

Her lungs are clear

Her body is still (ish)

Her eyes are bright

Her ears are open

Preschool is so amazing for her

She is seeing children her own age doing things she remembers being able to do

Walking, talking, eating, laughing, singing, playing

She's trying to find these skills inside of herself again.

Her cry has subsided but she is making more noise, trying to talk

She is incoherent still, like a babbling infant, but I know she has something to say and something to tell me, so every time I hear her tiny voice I respond. Alone in my house except for my little girl, I am always talking to my baby. I sing and chant and describe what I am doing. In my mind, I'm giving her words. Material to work with. Sounds to form with her weak tongue, lips, jaw, and I know one day she will talk back in words recognized by Merriam-Webster. 

She is so amazing, isn't she?

Day in the Life of a Breathing Episode

Hello!

So if you're paying attention you might have noticed that Monday was Maisie's SECOND DAY OF SCHOOOOOOOOL!!!!

She still loves it, she still came home and conked out for like three hours before dinner, she was still exhilarated and exhausted.

Nothing much to talk about there.

What I really need to talk about is lungs!

I realized when writing my post last week, I realized that I have completely stopped telling y'all about lungs!

Her breathing maintenance has been part of our lives since day one. I have become so used to nebulizers and CPAP machines and inhalers that I literally forgot that most people don't deal with that. I did a "Day in the Life" post a while back in which I failed to mention any breathing equipment. Because I guess I thought talking about that was like talking about brushing her teeth. Or my teeth. Boring. Whoops.

So today I'm going to a Day in the Life of a Breathing Episode.

That's literally what we call them. Maisie has no technical diagnosis except that the bottom half of her left lung does not communicate with her brain. So it doesn't do anything. There are no capillaries to that part of her lung, no air enters the alveoli, nothing happens. Normally, it's not a problem. When she was more active she would get out of breath or dizzy faster than her playmates, but the bigger problem right now (since she's not running and jumping) is that whenever she gets the slightest little cough or chest cold, we have to spring into action right away so that she can get enough oxygen.

Here's what happens when Maisie is experiencing any kind of cough, chest cold, wheezing, whatever. We call it a breathing episode and this is how we deal:

7:00

As soon as I'm downstairs I check the CPAP machine. I usually wake up three or four times during the night and check on her when she's on the CPAP machine. I don't really have a rational explanation, because usually her life is not relying on CPAP, but I feel like I have to make sure she's still hooked up (this is what a CPAP looks like. The tube is connected to just a regular pump kind of a thing. Nothing exciting. Looks a bit like a nebulizer or a feeding pump.)

8:00

Jeff wakes up and usually he checks on Maisie too :)

9:00

I get Maisie up, which means unhooking her from the CPAP machine and turning it off. Then I check her blood oxygen levels with the pulse oximeter. If her levels are low, I then listen to her breathing. If she's wheezing, I will usually hook her up to the nebulizer while she feeds, but if she's not then I hook her up to the oxygen pump.

               



10:00

Feeding is probably done, maybe not. I check her oxygen levels again and hopefully they're fine now. By the way, "fine" for Maisie means anywhere above 80-85. For most people, anywhere above 90 is fine, but Maisie has a little bit of allowance there. She doesn't get fed again until 1:00 so until then we often go out and do something fun. Museums, beach, park, something like that. When she's in the middle of an episode, though, this means taking with us her inhaler, nebulizer, pulse oximeter, and mini oxygen tank.
                               


1:00

Feeding time! I've been checking her oxygen levels on the hour and if they dipped below 85 (or 80 but she wasn't experiencing symptoms), I listened to her breathing with my trusty stethoscope. If she's wheezing she takes the nebulizer (assuming it's been at least four hours since her first nebulization of the day) but if not I hook her up to the oxygen tank and go on our merry way.
We almost always come home for feeds just because it's such a hassle to bring her feeding supplies with us. So, home for lunch and basically the day continues in the same way until bedtime.

9:00

Maisie always sleeps with a CPAP machine when she's having an episode. It gives her overworked lungs a much-needed break and usually means she gets over whatever her cold or cough or infection or whatever much faster. So brush her teeth, bath time if necessary, and strap on her CPAP mask. For the next few hours we check on her every hour or so until we fall asleep, and like I said before we get up a lot in the night to check on her and take her blood oxygen level.

It might seem like a lot of work for a minor problem, but all these precautions are just that, precautions. If we don't take them and aren't super careful, a minor cough could turn into a hospital stay. She has 75% lung function as it is, and she cannot afford to lose any more. So when she has any kind of respiratory distress, we give it our all to make sure a little bit doesn't go a long way.

One last thing: we have a lot of equipment, and a lot of it isn't completely necessary 100% of the time. Don't get me wrong: we use every piece of equipment we have. But not always, not all the time. And this stuff is very expensive. I frequently get angry Facebook messages and comments with the same theme: aren't you afraid you're taking away equipment from other kids for your own convenience? Yes. We are. We are very very lucky to have been given many things  -- from standers to oxygen tanks -- and to be in a place financially where we can purchase equipment for our convenience. But when Maisie doesn't need her CPAP machine or her nebulizer, I often loan them out to friends who do need them and can't afford them. Right now, a boy we met through the mito circle is using Maisie's nebulizer until his parents can purchase or rent one of their own. If Maisie goes into respiratory distress and needs to have a nebulizer, we might rush her to the hospital, rent one, or find another way so Joshua can have what he needs. We are lucky, and we are so happy to pay it forward. Rest assured that no one is suffering for our convenience.

That's all for now, I think I still have to update about neurology so I'll do that later, but the muffins are ready!

First day of school! First day of school! (part 2)

Sorry it took so long to update! We had a crazy couple of days, which I'll explain later. Here's the blow-by-blow of Maisie's first day!

9:00

Up and raring to go! I give Maisie her morning feed along with some banana slices which as usual she has no interest in. Jeff stays home for a few hours to help me get her ready and get her off to school. We decided to just carry her in today and not worry about the stroller. Her classmates are going to have a lot of equipment to get used to and if we can eliminate one thing today they may be less overwhelmed.

10:00


Her school day will start at 11:00 and we need to block out fifteen minutes to get her in the car, drive there, and unload the car. By 10:00 she was done with her feed and playing in the living room. Before we leave, I have to pack up her pump. To make life easier on Miss Stevens (the teacher's assistant who will be in charge of Maisie's feeds) I'm filling the pump bag and connecting it to the tube so that all Miss Stevens has to do is attach her tube to the connector and the connector to the tube and start the pump. I forgot to bring in the bean bag chair she will also be using the other day and her special toilet seat (just a Bumbo toilet training seat with a supportive back attached), so I get all that ready. The bean bag is awesome for Maisie because she can control how much muscle she wants to use. She can fall asleep in it, she can sit up and play, it provides as much or as little support as she feels she needs.



Next I check her not-a-diaper-bag. She needs to have a change or two of clothes, her feeding backpack, and all of her meds. I plan on doing a medicine post soon, but just to give you an idea she takes about a dozen different medications every day, including stool softeners, laxatives, digestive enzymes, immunosuppressants, anti-seizure medication and just now we're adding tranquilizers to the mix. That doesn't include her monthly maintenance dose of IVIg or the over-the-counter antihistamines and pain medication she takes to manage her severe environmental allergies and unexplained pain. So anyway Maisie needs a bag with her feeding backpack, a change or two of clothes, antihistamines, pain meds, pull-ups (even though she is potty-trained she occasionally loses control) and her occupational therapy tools. Because her grip is weak and poorly coordinated Maisie can't grip a marker or colored pencil very easily. She's very much like a baby just learning how to color. She uses these knobby crayons and we made a couple of those tennis balls down below for holding various things -- glue sticks, markers, colored pencils, etc. So all those things needed to be in her bag too. And then (I keep thinking of more things she needs) we bought a second pulse oximeter and inhaler -- jeez I don't even talk about her lungs anymore do I? -- in case of emergencies. There's a little binder with instructions for her care, both routine and in emergencies, with doctor's numbers and emergency contact numbers.

10:30

I'm scared.
I keep myself busy packing and fussing.

10:40

Now I'm freaking out.
I am seriously freaking out.
What if she has a seizure I've never seen before?
What if her lungs collapse (I really don't update about her lungs ever)?
What if she can't breathe?
What if her inhaler doesn't work?
What if she can't eat because her pump broke or Miss Stevens can't figure out how to feed her?
What if she has a random reaction to some medication? What if she turns blue? What if she's in pain and I'm not there to hold her and dry her tears?

10:45

Loading the car. Loading the Maisie.
Scared, scared, scared.
What if what if what if.

10:50

Arriving at Open Arms Adaptive Preschool.
Check in with school nursing staff.
Jeff carries in the bean bag and the not-a-diaper-bag.
I carry in Maisie.
Door opens.
Miss Becky greets us.
Maisie is introduced (again) to other kids.
The other two kids with disabilities are already there and have already had their equipment and such explained. Immediately a little girl cries "What's wrong with you Maisie?"
Talk about feeding tube.
Talk about muscles.
Talk about breathing.
Talk about seizing.
Talk about a hundred other things.
Miss Becky announces it's circle time. Maisie's Baby-Snug has already been set up in the circle and so I get her set there and back up.
She panics.
I calm her down.
Miss Becky distracts.
I back up.
Maisie is not sure about the whole thing but there are colorful balls happening now so she's distracted.

11:20

After about twenty minutes I sneak out. I'm told Maisie had a complete breakdown when she realized I was gone, but it only lasted a few minutes. Totally normal. She had fun, the feeding and medicining went fine, no emergencies.

Of course, that's Miss Stevens's version.
I wasn't there.
Guys, Maisie can't talk. She can't sit up. She can't really play the way the other kids do.
She can't sit on the potty by herself.
She can't eat.
She is wonderful, beautiful, sweet, and darling just the way she is, but she isn't like her classmates. I cannot imagine that's going to be easy to deal with, both for her and for her classmates. How will she make friends if she can't talk? How will she play if she can't sit up? How will she be acceptable if she is so different.

I don't know.
And it scares me.
But this is a big step.
I have to let her do this.
I know she enjoyed her first day of school.
I have to let her do this.
She might get hurt.
But anyone could get hurt.
I'm really scared.
But I have to let her do this.

First day of school! First day of school!

Maisie's first day is tomorrow!

She is so excited. Her speech is very limited by her disease but every time I ask she smiles and burbles and I just know she can't wait.

I had a meeting with her teacher, Becky, who is young and energetic and surprisingly knowledgable about neuro diseases. Maisie will need to have a feeding while she's at school tomorrow, so I bought a little bag to hold all of her feeding supplies and brought it to the school to show Becky and her assistant how to use it.

 I also brought in the stander she'll be using at school, this adorable little Leckey Totstander, which we've actually had forever but couldn't use very much because she was too weak.

She'll also have a Mamas & Papas Baby Snug seat (yes, she still fits in a baby seat) and the Mamas & Papas booster seat. These things are literally our best friends at home and hopefully will be just as useful and effective at school. We have at home a very nice (very expensive) Special Tomato supportive stroller-wheelchair thing (below right), but it was a huge monetary investment and I didn't want to buy another one for school, especially if it doesn't work out, so instead we got a very simple wheelchair and added a supportive liner.

I won't pretend I'm not super nervous to be sending Maisie off tomorrow. Recently she has been doing so well. Her medication is giving her so much strength and energy, it's amazing to see her crawling and playing with toys.

Some hours and days are still very hard. Her seizure activity is still very bad and some days she starts shaking and crying every few hours. Seizing is so distressing for her. She doesn't lose consciousness so she is aware that she's lost control of her body, and it terrifies her. All I can do is hold her and rock her and try to convince her that everything is okay. 

Most of her seizures are clonic, which means her whole body jerks, but she also has myoclonic seizures, where just her hand or arm will shake. She usually zones out when this happens, focusing on a point in the distance and dropping whatever toy she's playing with. Her last EEG was in April and the results are just the same as they've ever been. She's on seizure medication, but it doesn't seem to be helping. We're meeting with neurology after school tomorrow. Hopefully we'll get some answers or some help.

So! Hopefully she has a great day tomorrow and we'll get some relief from the neurologist tomorrow!