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!

Changes

So, as y'all know if you read this post, we have been going through a lot of changes lately.

Maisie now has a permanent PICC line, which causes quite a bit of trouble let me tell you. She has a lot more energy lately and her hands have been moving constantly. Particularly, moving to yank on the PICC line. Literally her arm is wrapped in gauze, medical tape, and an ACE bandage to keep her from picking at it. We were sent home with arm restraints (see above), but for heaven's sake the girl has just gotten excited about moving her arms again! I'm sure as heck not going to restrain her. But, the PICC line has already been disrupted badly enough to get us to the ER twice. 

Now, obviously the picture above is not Maisie. But it might as well be, and that's another change I'm making. TONS of people have been asking that I post pictures of Maisie so they can see what she looks like. I'm not willing to do that. I'm not even willing to post pictures of things for fear that something in the most innocent photo will help my ex-husband to track us down. That's the same reason I blog from my mom's account. However, I am willing to seek out photographs of other babes who look like mine so you can see what her button looks like, how skinny she, etc. All the pictures will be linked to their sources for copyright purposes. That girl is Mabel, and I know I've talked about her before, but I never cease to be amazed at how much they look alike. Mabel has Batten Disease and is very hypotonic. She is skinny and floppy and has a mop of fuzzy brown hair just like my doll. They also have the same big eyes and toothy grins. The only difference is that Maisie has great green eyes while Mabel's are brown.

Another big, wonderful change is the preschool down the street. We took Maisie out of public preschool because it was too much stress for her teachers, peers, and mama to have her away from home. Homeschooling a preschooler is weird and chaotic and I am craving some time to breathe, drink some tea, do a little yoga, have a little time to myself. And then the Open Arms Adaptive School opened up. 

This is such a Godsend. Just when I needed it most, the old preschool right down the road closed and was taken over by an amazing, adaptive preschool with a neuromuscular specialist on staff. Other special needs moms will know where I am coming from here. Adaptive education is so hard to come by, and all of a sudden here's one literally a five-minute walk from my house. I cried and praised the Lord for literally two hours when I heard the news. Maisie will be attending preschool with just five other kids. We have already met the other two "special needs" kids, who are Lily and Christian. Lily has Rett Syndrome, which is similar to myasthenia in several ways and is actually something we tested for. Christian has muscular/retinal dystrophy and can no longer walk. He is mostly blind. Maisie will only being going to school for one day a week for now. We may add more days after we see how comfortable she is, I am, and her teachers are with her at the school.

For the other three kids, who are "normal," I was asked to write a letter from Maisie to them, explaining who she is and why she is the way she is, along with a photo and if possible some small bits of equipment, like a spare g-tube or anything that could be shown to the kids so that Maisie's medical equipment isn't so harsh and scary for them. Here's the letter I wrote, and a very similar picture to the one I included. Again, that's Mabel.

Hi! I'm Maisie!

I am so so so so so excited to be going to school with you! I haven't gone to school in months because I need a lot of special care, and it was too hard on me, my teachers, and my mama to be away from home. I hope Open Arms will let me go to school and make new friends. I might not say hello or "Nice to meet you!" but that doesn't mean I don't want you to be my friend. Sometimes, I might lie on the ground or sit in the corner and not speak or move for a while. I might even fall asleep! That's because I get tired very easily. My muscles wear out much faster than yours do, and I need more rest. But that doesn't mean I'm not excited to play with you and having a lot of fun. I just need to rest and then I'll be raring to go again. Also, I am partly blind so if you wave to me and I don't wave back, I probably just didn't see you!

At school with me I will have a stander, which helps me to stand up with the rest of you so my muscles and bones can grow. I can't stand on my own right now, but hopefully I will be able to soon! I will also have a booster seat to help me sit up in chairs and on the floor. Finally, I will sometimes be in a wheelchair-stroller. I am not riding in a stroller because I am a baby or because I'm lazy, but because walking is very hard for me. I'm working on it every day, but right now I have to be carried or pushed in a stroller. Finally, I have a band around my arm and a tube underneath it. This is how I get medicine. I also have a button on my belly. This is how I eat. I can eat by mouth but it is very difficult for me and I can't eat enough that way.

Here's some more stuff about me: I'm four years old. I love love love love Doc McStuffins and Sesame Street. My favorite color is yellow and I love to get dressed up. 

I know I'm small and weak. I'm trying my hardest to grow, but somehow it just isn't happening. Please remember that even though I might not be able to speak or make a lot of noise, I still hear and understand everything you say to me and around me, so be careful!

I might cry a lot. I'm often in pain. I might not participate and I might get frustrated. But I appreciate you learning with me and letting me join your classroom. I know I can be a handful, and I am so grateful for your support.

Hugs,
Maisie

Diagnosis

It has now been almost two weeks since we received our diagnosis.

I'm ready to tell y'all what's been going on.

Maisie was diagnosed with myasthenia gravis, which an autoimmune neuromuscular disease.

Nearly everything we've been struggling with since day one adds up. Muscular weakness, vision problems, impaired speech, difficulty swallowing...it all fits.

A handful of her symptoms are related to brain damage and birth and not myasthenia gravis - seizures, kidney failure, lung problems. But knowing what is causing a plethora of her other disabilities is such a blessing because we have a support group now, other moms who can help and sympathize, and a whole new plan for Maisie's treatment.

It was actually quite a miracle that we got this diagnosis. The pulmonologist had ordered a routine MRI of the lungs to check on Maisie's function, which is something we do three or four times a year, and the radiologist happened to notice that her thymus gland looked funky. We are eternally grateful to him for taking the initiative and showing this to an immunologist, who ordered the blood test that confirmed the diagnosis.

The doctors flew into action immediately. She was admitted to the hospital for three days and given an permanent PICC line through which she received her first dose of intravenous immunoglobulin therapy (IVIg). The past few weeks I took her in three times a week for therapy. Starting on Wednesday we'll be going in on the first Wednesday of every month for her therapy. She is also taking pyridostigmine every three hours. We will continue working with Drill Sergeant June: Maisie's prognosis is good. She might eventually return to regular growth and development.

I want to talk quickly about Maisie's eyes, because I never really discussed them before (I meant to do this the other day when I wrote a post called "honesty and ophthalmology" and then completely failed to talk about her eyes.) No one suspected that her lazy eye and droopy lids were caused by anything more than neuro damage, like a lot of her other symptoms. But since she was around a year old her left eye has been a little wonky. I wish I were willing to show you pictures so you could see how sometimes it tracks with the other one and sometimes it's just off doing it's own thing. Around eighteen months we noticed that her left eyelid was droopy, but at that point she had literally no other symptoms. She didn't start really walking proficiently until she was two and a half, but that was a balance/motor skill thing more than a hypotonia thing, so no one connected the droopy eyelids to muscular weakness. By two she had completely (if only temporarily) outgrown the hypotonia she had as a baby but the bones of her ears were ill-developed. Thankfully she outgrew that without much medical intervention and she started toddling. Her symptoms were then centered around kidney failure, lung inefficacy (that's totally not a medical term), and digestive troubles. If you've been around the blog for a while then you know she didn't really start having hypotonia problems again until after her fourth birthday. In terms of actual vision the left eye does not see anything at all at night. That we know for certain, but that's about it. We don't really know how much peripheral vision she has in either eye or whether or not she has

We still don't know why she won't gain enough weight. So far everything that's been tested has looked totally normal. The good news is she has stayed steady at 19 pounds for the past few weeks. So maybe she won't lose anymore weight. Truthfully, I just don't know. A few people have sent me messages saying she should be tested for diabetes. She has been. The endocrinologist has ordered several different blood tests and all of them have come back, not quite negative, but not really indicative of diabetes. I have been fighting for the endo to treat her as though for diabetes and see what happens. I have a very strong family history of diabetes. One of my cousins, we'll call her Rachel, was not diagnosed until she was fourteen but she had been very, very sick for almost ten years. Her body was ravaged by this disease but her doctor refused to believe that she had diabetes because the blood tests were not strong-positive.

On a happy note, Jeff and I have decided to do away with the white-dress church-wedding. It just is not going to work. So we'll probably get married in the courthouse and have a picnic in my mom's backyard afterward (we don't have a backyard.) Hopefully by June-ish Maisie's health will have stabilized and we'll be able to have a quiet little party.

I'll keep y'all posted!

honesty & opthalmology

Yes, we know.

We know the cause of many of Maisie's problems.

We are making a lot of progress.

But I'm not ready to share with you.

I hate doing this. 

After the scare and false positive we had, I can't bear to find out that this isn't the truth.

I feel as though I have been lying to you since April 23, and I hate this. I know that keeping this information quiet until I have completely and fully dealt with and processed it myself is the right thing to do, and yet I feel guilty. Like I'm lying by omission. Every time someone asks me about Maisie I feel like I'm lying by omission, by not expressly saying "This is what happened."

I want to be honest. But I have to take care of myself.

In brighter news, physical therapy is going extremely well! We started doing twice a week sessions and every in-between day Maisie spends half an hour sitting up and five minutes walking. We have not gotten past five minutes yet, but this is still such a huge step. She is sleeping literally all the time. She wakes up at nine, has a feed, and falls asleep again by ten. She sleeps on and off until noon or so, when she gets lunch, and then is awake for about two hours for physical therapy. After physical therapy, around 3:00, she conks out in front of the television and I often can't get her to stay awake for the whole feed. 

She also has literally zero interest in feeding by mouth. She used to at least take a bit, a couple of graham crackers or two bites of oatmeal, but now she is just so tired. She'll fidget with her food and maybe take a single bite, but literally that's it.

Drill Sergeant June says that none of that is unusual. We're pushing her fast and hard and she's worn completely out. And that's okay.