it's been WAY too long!

Hello!

Fancy meeting you here!

I'm so sorry things have been so dull on the blogosphere. We've had a big change...

Please welcome to the world my darling Matilda Mackenzie!

(not actually Tilly. But I thought a baby announcement should come with a picture of a cute baby)

She was born almost six weeks early, but she's doing well. When Maisie was born, I had an emergency c-section to save her life. My uterus was pretty traumatized by the experience and has some pretty heavy duty scar tissue. It got to a point where my OB/GYN didn't think the uterus would be willing to stretch much further. Rather than test the limits and possibly risk rupturing my uterus (nothankyou) I had acupuncture to induce labor. Guys, that was INSANE. I had one session. I was in labor fourteen hours later. Labor normally starts within 48 hours after acupuncture, so that was a bit fast. But anyway, it worked. Matilda was born at home on May 19th and taken right to the NICU, where she spent five days before coming home. She weighed four pounds and fifteen ounces. At four weeks, she now weighs five pounds, four ounces.

And, of course, she has her limb differences. I talked about this a long time ago when we first found out, but honestly it hasn't bothered me. Tilly will not struggle with the hundreds of other things that Maisie copes with every day. She will probably be able to walk and run and write and play. She'll be fine. She'll adapt.

Basically, Tilly's right foot is permanently curled in, sort of like a club but far worse and less fixable. She's missing a number of bones in that foot and ankle as well as two toes. Her left leg is completely normal. Her right arm is amputated at the elbow and her left hand is sort of triangular, like her thumb is tucked into her palm and her fingers are all squished together. Imagine the "quiet coyote" hand signal but without the pinky and index fingers up. And again, she's missing a lot of bones in her hands. An orthopedist tried to talk to me about corrective surgery but honestly all I wanted to hear was that there were some options and then I asked to him leave. I just delivered this baby! I don't want you cutting her up just yet!

So anyway, enough about Tilly. I'm sure you're all dying to know about Maisie the Great, my beautiful almost-SIX-year-old girl.

Maisie, my friends, is doing A-MAISIE-ING. She was decannulated just last week -- that means her trach is GONE! Gone! Whoo-hoo! She is so big and strong. She is FINALLY over 30 pounds -- yes, I did throw a party when she weighed over 30 for the first time at a checkup. In fact, she weighs 34 pounds and is almost 40 inches tall. Which puts her in the 5th percentile for weight (which is actually AMAZING because she's hardly ever even on the chart, she still isn't for height.) She's still pretty skinny and no one believes she's almost six years old, but she is making progress and that's all we can ask for.

I can't begin to describe my joy at Maisie's progress. Since I last posted in April, she has advanced by leaps and bounds in walking. She is very very very very close to completely unassisted toddling. As it is, she is using the Yogi Anterior walker by Ottobock in this adorable shade of purple. That's right, folks, there are no straps, no buckles, no harnesses, just a little extra balance support. Her balance is improving a little every day, but it still isn't where we want it to be.

A big part of Maisie's therapy lately has been stretching. I discovered a few weeks ago that her muscles were pretty seized up from lack of use, so I started stretching her arms and legs to regain mobility. I think it's helped a lot, and her physical therapist agrees with me. I think it's also increased her strength, because she will push her legs against me or engage her abs when I bring her legs up.

What else? She started group occupational therapy a few weeks ago at the recommendation of a special needs mom friend of mine, and I think she's really enjoying it. There are four kids in the class and they all practice drinking from cups, washing hands, blowing noses, etc. Maisie is the only one who isn't a great conversationalist, and I think it's been great for her to listen to other kids talking.

I think the last time I talked about her speech was in February, when she only had about a dozen words. She's up to I think 44 now, I have to check. We recount once a month so that number is from her May physical, which was I think May 7.

All in all, this royal disaster is doing really well. She's growing, developing, walkin' and talkin'. And I have a hunky husband and a cute new beanie to boot. Life is good.

Hi everybody!

Long time, no see! Seriously! Where have I been?

The kingdom of Maisie the Great has been a pretty chaotic place lately. Our little medical mess is, as always, keeping us on our toes. Other than a little 36-hour scare, we've stayed out of the hospital. We have finally worked up the courage to take Maisie out of the house (quite the feat with all her equipment) and we went to a park nearby. A little boy coughing all over the place was a little too friendly with her, and when I talked to his mother she said he's getting over some kind of respiratory infection.

UM.

We took her straight to the ER, which may seem like a major overreaction until you realize that she has a severely compromised respiratory system and and her immune system is pretty tattered. What is a week of discomfort for that little boy could be a death sentence for Maisie. We took her to the hospital, she was admitted overnight and given a broad-spectrum IV antibiotic. They took cultures and sent us home a day and a half later with an oral antibiotic. They took more cultures a week later, but nothing has developed so hopefully we dodged a bullet.

Now, for some good news! And there is plenty of it!

First, Maisie has gained eight pounds since I last posted her weight, which puts her at 29 pounds! I think we'll throw an honest-to-goodness party when she hits the big three-oh. She also, believe it or not, grown A QUARTER OF AN INCH. In like, a month. My little munchkin is getting to be such a big girl.

Maisie is doing so well in therapy -- physical, speech, and feeding. We have actually almost eliminated the stander hour because she is upright in her walker so much. She is still having a lot of problems with fatigue, which we don't really understand, but she is in the walker (actually a gait trainer) 2-3 hours every day at least. She loves the walker. She loves having her freedom, and it's really unfortunate that the thing is so big it makes it hard for her to approach anything, like a table to play at, and it's impossible for her to pick anything up. She has a Comet Anterior Gait Trainer, which is the thing to the left and is quite the little speedster in it. The only problem is that she is in the upright and locked position -- she can't really relax. So we're hoping to get her a "pull" gait trainer, something more like a posterior walker. That would at least allow her to bend down and walk right up to tables and things.

She is doing so well with the trach. The incision site healed well and we haven't had any trouble with infections, etc. At this point we have experimented with a few minutes off the ventilator several times a day. I unhook her to carry her upstairs, change her clothes, etc. She tolerates it very well and is greatly pleased to be freed from her tubes. We've met with a respiratory therapist several times and are slowly increasing her to ten minute increments off the vent. Yayayayay! She hates the ventilator, and fights to breathe "over" it whenever possible already. We discovered at a recent session with the therapist that she starts to panic when she's off the vent, because at this point she is so used to having breaths taken for her that it is disturbing to be breathing entirely on her own. So for five minutes every day, while she's off the vent, we practice taking big, deep, slow, calm breaths.

The trach is, and always was, a temporary solution. Maisie hates it. I hate it. It's frustrating to be hooked up to a machine and connected to yards of cording all the time with an active five year old who is trying very hard to walk and talk. This is all really good news, but she is so hindered by the vent. She regularly disconnects herself from the vent (yay fine motor skills!) and then grins like a maniac as she struggles to breathe and play at the same time.

That's all for now, I guess! We are just making slow progress, taking it one day at a time. We are looking into a school program for Maisie, but with her increasingly complex medical needs it may not be possible.

Love,
Charlotte

One Day

It's hard to believe we have been home for five days.

Five beautiful, glorious, messy days.

It's been preetty crazy around here.

I thought that if I just did everything when it needed to happen -- in terms of administering medicine, trach care, feedings, physical therapy, etc.

But I was wrong. I needed to create a set-in-stone schedule and map out everything, including my own meal times, laundry days, cleaning time, sleeping time.

We found while we were in the hospital that her Keppra was best administered in the evening, before bed, with the Prednisone. In the morning she needs to take Acetylcholine, Zyrtec, Prozac, and Metolazone. She should be having five small meals a day and taking enzymes half an hour before each meal. She needs 300 calories at each meal and whatever she doesn't eat orally has to go through the tube. And she will also have an overnight feed of 10 oz of high-calorie prescription formula. Oh yeah, and every day she needs to have an injection of HGH. All of this needs to be better coordinated with my life and the nap that Maisie really needs to take every day and all the regular hygiene and playtime and therapy stuff.

Okay, so:

8:00 -- Wake up, change overnight diaper, administer enzymes through the tube. Trach care, suctioning, tube change if necessary. Get dressed, check the tube site and change button buddy, brush hair & teeth.

8:30 -- Breakfast! Banana, pear, oatmeal, pancakes, eggs, bacon, etc; all fortified with powdered milk, butter, whipped cream, etc.

9:30 -- Administer Acetylcholine, Zyrtec, Prozac, and Metolazone. Whatever she didn't eat at breakfast goes through the tube as formula.

10:00 -- Therapy. Monday, Wednesday, and Friday Maisie has physical therapy. Tuesday and Thursday she has speech therapy.

12:00 -- Enzymes through the tube

12:30 -- Lunchtime! Chicken nuggets, yogurt, avocado, graham crackers and peanut butter, peanut butter and banana sandwich, hummus, any kind of beans, etc. Once again I fortify whatever I can with powdered milk, butter, whatever works.

1:30 -- HGH injection and nap time. While Maisie naps, I get housework done. Laundry, prepping meds and feeds, reading, cleaning, etc. If she stays asleep for an hour and a half, I can check off maybe half of my to-do list.

2:30-3:00 -- When Maisie wakes up I set her up in the living room with toys, books, and Doc McStuffins, PawPatrol, Bob the Builder, or one of her favorite movies (Finding Nemo, Frozen, Tangled, Wreck-It Ralph). This is her time.

4:00 -- More therapy. If it was a speech therapy day, we do as much PT as possible, including stander hour and fifteen minutes in the gait trainer. If it was a PT day, I do what speech therapy I can on my own at this time.

5:00 -- Enzymes!

5:30 -- Dinner time!

7:00 -- A little Daddy play time and then we start the bedtime process. Prednisone and Keppra through the tube, bath, teeth brushing, jammies, set up the overnight feed, and then several hundred books before she conks out.

9:00 -- Maisie is usually asleep by now and I'm exhausted and not far behind her.

So this is a pretty good schedule, except for the fact that she also has a trach. And that usually throws a monkey wrench in our days. And once a week we return to the hospital for plasmapheresis, and once every three weeks that hospital trip includes IVIg.

Whew! I won't pretend it wasn't easier having her at the hospital, having someone else worry about her, but it's worth it to be home.

HOME!!!

Quick update to let you all know that we. are. HOME!

HOME.

Oh my gosh, I love that sound.

Home.

<3

:)

Friday

This morning Maisie's nurses were barely containing their excited squealing.

We didn't know why every glance at Miss Maisie made them so happy.

They weren't allowed to tell us.

We figured it out at rounds when the ENT told us that Maisie will be discharged today.

I couldn't believe it. 

The nurses were basically partying.

There were cupcakes.

I am not kidding. Apparently the nurses asked for a bit of advanced warning so they could bring cupcakes and have a little celebration.

Maisie's is the only bed that has remained occupied for the past two and a half months. Every other patient has come and gone, but Maisie has been here.

I mean, this has been a long stay. And now -- well, the day after tomorrow -- it's over. We're going home. I have been living at the hospital as sure as Maisie has been. I have been out and home a few times, but for the most part I have needed to be here. She's been in pain, afraid, in surgery, or in some kind of arrest or distress for nearly our whole stay. And when she wasn't sick, I was training to give Maisie injections, to suction, change, and monitor her trach, and to use all the new equipment that is in our home, waiting for Maisie.

Over the past few days Jeff and I were able to get the house ready for Maisie. She has been in the first floor bedroom and even though it's really a very small room, we decided that she should stay there. The stationary vent will sit by the bed along with the feeding pole. and above it is a little shelf for the apnea, pulse-ox, and heart rate monitors. She has a nice new storage hutch with lots of shelves and cabinets that we have moved all of her new medical stuff into. The emergency oxygen tank and cannula are tucked into her closet, and on the flat top of the hutch we set up a trach-care station with a place for her to sit/lie while her trach is taken care of. The only open floor space is the three-foot wide path down the middle of the room, but that's okay because her playing can be done in the living room.

The great thing about having her vent downstairs is that her circuit (the tubing from the trach to the vent and back to the trach) can reach from the stationary vent almost anywhere in the kitchen, bathroom, and living room. This means that her stroller can remain pre-loaded with the portable vent and humidifier, the secondary apnea/heart rate/pulse ox monitors, and some emergency supplies and I don't have to pack up every time we want to go outside.

Most of her feeding equipment is in the kitchen. She will get her feeds while Jeff and I eat our meals, so that hopefully she makes the association between eating and the pump and the time and feeling full. Her physical therapy equipment is all in the living room, mostly put away and organized in the cabinets and shelves along the wall. Her stander and gait trainer are tucked behind the couch.

Maisie is coming home with a lot of support equipment, and there's a lot that I need to do. She is by no means "healthy." Aside from her regular g-tube feedings and physical therapy that were happening before the hospitalization, Maisie now has a huge list of daily/weekly/monthly needs.

Every day I will have to clean the trach site, and once a week the tube will have to be changed. The trach needs to be suctioned to get the mucous out (you and I would just cough secretions out, but this doesn't work with a trach.) She will receive daily HGH injections. Once every three weeks we will return to NJ Children's Hospital where Maisie will be weighed and measured precisely, and she will recieve IVIg. Once a week we'll go back for plasmapheresis.

The physical therapist will be by three times a week, but Maisie and I will do our own physical therapy every day -- besides an hour of stander time, she will be in the gait trainer for at least fifteen minutes every day, and she will have lots of little therapy activities throughout her day.

We have a long list of goals for Maisie, things we want to see her accomplish by her sixth birthday. I will blog more about that later, but right now I have discharge things to do!!

In pictures

I follow a number of blogs written by mommies of medically complex children, like my Maisie the Great, and once in a while I see a photo and I just think 'yep, that's Maisie.'

So a few weeks back I came across these pictures on the blog Life with Lily, and my breath caught at the stunning similarities between Lily and Maisie.

Although today she was a bit more like this:

(the third picture is from ABC news. the last two are of Piper Breinholt, who has CCMS. I have always been amazed by the physical similarities between Piper and Maisie. Maisie is hardly any bigger than Piper, and between those skinny little legs and the trach now, they look a lot alike.)

When she woke up this morning she was so perky and happy, it was wonderful. She was sitting up and playing and babbling on and on. What a little fighter. She's even gained a little weight, so she's around 21 pounds. Yay! She is still getting daily growth hormone shots, which is one more thing Jeff or I will have to do at home. I'm not sure if she's grown height-wise at all, they only measure that once a month but she gets weighed once a week. I feel like I'm getting my sweet little girl back, and I can't wait.

Miranda's plane landed this morning and she arrived at my parents' place at 8:00 or so. She's been here, hanging out with Maisie, who is having a grand old time. Tomorrow I will leave Maisie in Miranda's capable hands and head back to NJ to get the house set up for Maisie's imminent arrival home. 

Home. It sounds so wonderful. We're going home.

WE'RE GOING HOME!

AHHH

Provided that Jeff and I can everything organized by then, Maisie can probably be discharged Friday or Saturday, which is even sooner than originally planned.

God is good.

Post Surgery & Such

Hello!

I am SO SORRY I didn't update through the surgery. I completely intended to and even had a post written but it got kind of crazy and I just forgot to post anything.

So here's the blow-by-blow:

7:30 -- Maisie FINALLY gets wheeled back. She is TERRIFIED. I don't think she's really been aware of having surgery before, so this was a "new" experience for her.

8:50ish -- A nurse came to the waiting room and asked that I scrub in and come into operating room. Um, WHAT?

9:00 -- I go back to find out that Maisie has fought the anesthesiologist so hard she wants me to help calm Maisie down.

9:30 -- Maisie is unconscious. I leave the operating room. Yes, it took half an hour.

10:00 -- Another nurse comes back and tells me the surgeon opened for the thymectomy. I probably won't hear anything until after noon.

12:30 -- The thymectomy is over and the surgeons are switching. A different surgeon performs each part of the operation (thymus, Broviac, port.)

1:00 -- This is where it got weird. Maisie had a dialysis catheter as an infant in the same place her Broviac was to be placed. The surgeon began the procedure and when he went to insert the actual catheter, found that he could not slide it as far through the vein as was necessary. After a fluoroscopy they found that the catheter was coiling inside her superior vena cava.

EXCUSE ME?

They assumed she had a blood clot in her SVC that was preventing the catheter from going through.

UM.

It was possible that the clot had formed during the thymectomy -- this seemed weird to me but I'm not the doctor. They immediately went into panic mode and sent for an emergency ultrasound and for a nurse to come tell me what was going on.

A little after 1 -- I get informed that Maisie probably has a blood clot in her heart.

1:30ish -- I get informed that she does NOT have a blood clot in her heart. Turns out the catheter tubing was faulty. Honestly I think the ER was bored was hoping for a cardiac arrest patient.

HOWEVER her pleura is torn and she has chylothorax. Which means she needs a second chest tube (I already knew she would probably have one to drain her thymus cavity.)

2:00 -- Broviac is in, all is well but for an elevated heart rate, time for the port.

2:30 -- Port is done, she's in recovery.

3:00 -- I can go see her.

4:00 -- She is vomiting. And vomiting. And vomiting. Pretty soon she's on IV fluids and anti-nausea meds. Her feed is stopped and her stomach is pumped through the g-tube so there isn't anything for her to throw up. But she is STILL TRYING TO VOMIT.

At this point I was exasperated. I nearly had a heart attack, she got TWO nasty chest tubes, and then she had a horrible reaction to the anesthesia, which is so not like her.


Anyway, all is well at this point. Both chest tubes were pulled this morning, they finally found an anti-nausea med that handled the vomiting, and she was mostly back to normal today.

The port and Broviac look great. No infections or anything. The process itself was crazy, but the end result looks good. She'll be having an ultrasound tomorrow to check everything out, make sure her lines are all placed correctly and the cavity where her thymus was is looking good.

In other news, Squishy is doing well. She is 26 weeks old, and apparently she is the size of a head of cauliflower. Um, okay. According to the doctor Squishy is pushing three pounds already. Which means I'm growing me a big baby! And no, we don't know for sure her gender. But I'm certain she's a girl.

This is considered a high-risk pregnancy because of my extensive medical history, but honestly everything looks great and I have no worries about my little Squishy.

I think that's all for today. We are still planning on going home on the 1st -- possibly sooner! Jeff has been fielding deliveries of oxygen tanks and ventilators and all kinds of stuff. This week I will probably be spending a lot of time at home getting everything ready. My sister Miranda is going to be here for the week and Maisie will love the attention. She won't miss me at all. Miranda dotes on her worse than my mother. Not that I'm complaining :)

Up late, can't sleep, surgery tomorrow!

The title says it all, ugh.

Forgive the nonsensical nature of this post. I'm tired.

Maisie's surgery is scheduled to start bright and early at 7:00 tomorrow morning. She will be in surgery until about 1:00 in the afternoon.

The thymectomy will happen first (to be clear, she is having her THYMUS taken out. I guess autocorrect changed it to thyroid in a bunch of my other posts. Either than or I typed wrong :) She'll be have a transcervical thymectomy, which means they'll be taking her thymus out through an incision in her neck instead of opening her chest the same way they would for open-heart surgery.

Then the Broviac line will be place and the port implanted. Hopefully, if all goes well with the surgery and we get all our supplies and equipment set up at home, we'll be going home on March 1st!

Hopefully our supplier will come through for us by Monday and I will be able to make a trip home to get her equipment set up. We will have to rearrange our house a bit to accommodate everything, but it's all so worth it.

It's hard to believe that after being in the hospital for two months, we are finally going home.

I have been flopping around tonight, trying so hard to fall asleep so I will be alert and awake when
they take Maisie back to put her under anesthesia.

Facing surgery for your child is terrifying, even when it is one that should dramatically increase their quality of life.

There is just so much that could go wrong.

She is doing so well finally, with speech, physical, and occupational therapy. She is already fighting to breath over the ventilator, and she shocked everyone when she was jabbering away just HOURS after trach surgery.

My beautiful girl is smiling, giggling, chattering, and even walking (okay, in a walker) again. I feel like I have my Maisie the Great back, and there is a part of me that is terrified something will go terribly wrong with this surgery and I'll lose my sweet girl again.

Please pray for us that the surgery goes beautifully and the supplier gets us everything we need in time for our home date!

Oh, also, tomorrow we break our record for the longest hospital stay! 68 days! *unenthused woo-hoo-ing*

Quick little update

I only have three seconds to post this because Maisie is being taken for an ultrasound, but I wanted to let you all know that Maisie will definitely be having her Broviac surgery at the same time she has her thymectomy.

We're not sure whether or not the port will be implanted at the same time. They have to find a different doctor to do the port because the doctor doing the Broviac has a busy schedule.

I have to run, I will update as soon as I know more!

Medical Update: Day 64

There's good news on the Maisie front!

She is responding very well to a combination of low-dose prednisone and another drug called Acetylcholine. She is also doing plasmapheresis and IVIg (intravenous immunoglobulin.) This combination of medication and therapy seems to be working well. Unfortunately, she will have to have a Broviac line and an implanted port placed.

Once we leave the hospital, we will return for IVIg once every three weeks. This will go through the port. She will have plasmapheresis (a procedure similar to hemodialysis in which her blood plasma will have antibodies filtered out) once a week through the Broviac.

Right now she has a PICC line and a regular IV. Hopefully the surgery for the Broviac and the port will happen this week.

She will also FINALLY be having her thyroid gland taken out on Thursday. Whoopee!!

I know it seems odd to be so excited about your child having surgery, but this is something that I have been pushing for since July. Every other person with myasthenia has described dramatic improvements after their thymectomy. Obviously everyone is different and it might do nothing for Maisie, but it has always seemed crazy that we didn't try.

My hope is the thymectomy, the Broviac, and the port will all happen on Thursday. It will mean a four or five hour long procedure, but I want it all done at once.

The really great news is that -- drumroll, please -- it seems we might finally be going home soon!!

I completed all of my trach training. Check!

We've found a good combination of drugs for Maisie. Check!

Maisie is feeling and doing FANTASTIC. She has more energy than I've seen in a long time. Speech, physical, and occupational therapy are going extremely well. Check!

I am working with a social worker to get insurance and home nursing care sorted out. While Maisie has the trach she will have 24-hour nursing for the first week we're home, and then 8-hour night nursing as long as the trach is here.

The ENT gave us a tentative home date of March 1st. That gives us two weeks to get everything sorted out.

That means getting

1. Home nursing care
2. Insurance to get with the program
3. An arrangement with a supplier for all of Maisie's new vent and trach supplies, which include
               - a stationary vent
               - a portable vent
               - a suction machine
               - an apnea monitor
               - a pulse-ox machine
               - emergency oxygen tanks & nasal cannula
               - all the bells and whistles that go along with the trach (trach ties, all the different parts of
                 the trach tubing itself, and a billion other little things.)
4. Permanent IVIg and plasmapheresis appointments
5. Broviac surgery
6. Thymectomy
7. Port implantation

There's SO MUCH to worry about and SO MUCH to organize, but honestly, it's all worth it to have Maisie home again.

I'm a little bit overwhelmed and a little bit SUPER EXCITED.

I have neglected someone very important!

Someone very special came to the hospital to visit Maisie today.

Even though she has remained a large part of Maisie's life, I have neglected to mention her in the blog.

I feel terrible about this, because she has played such a major role in Maisie's life. Without her, I have little doubt that Maisie would suffer from some of the same medical PTSD that so many kids who spend too much time in hospitals struggle with.

And especially because the work she's been doing is so incredibly selfless and, at eleven years old, this special kid is changing oncology and maybe even saving lives.

I owe her a lot. We owe her a lot.

Can you guess who I'm talking about?

I'll give you a hint.

She lives in Baltimore, Maryland.

She's been fighting bone cancer for six years, but has been in remission for a year and a half.

Can you guess yet?

Okay, I'll just tell you!

It's Kaitlynn!

Wow! I can't believe we haven't seen her in so long!!

Maisie is PSYCHED!

These girls have known each other since Maisie was a year old and Kaitlynn was seven. Kaitlynn looks fabulous. She's put some meat on her bones and grown like three feet since I last saw her. She's all grown-up and gorgeous.

When Kate's mom called me over the weekend to ask if they could visit, I was worried that it might be hard for Maisie to see Kaitlynn so strong and healthy while she herself was so sick.

I needn't have worried. Once again, Maisie has proven that she is above such petty things as illness and outward appearance.

Kaitlynn has been doing exceptionally well. After her initial diagnosis with bone cancer in her left foot at five, she developed mets in her left leg and through her intestines. She's come through eight rounds of chemo, fourteen surgeries, and come dangerously close to having her foot amputated.

She's a miracle girl.

She's been in Tennessee working with St. Jude's Children's Research Hospital, where her body and her story are being used to study oncology and figure out what it is about this particular kid that beat cancer in a way that no one else ever has. I don't have any more details than that, unfortunately, but I know that Kaitlynn has sacrificed a lot to partake in the study, as have her parents.

I don't know how long she'll be around, her schedule is dictated pretty much completely by the hospital. She was given roughly two weeks off from "working," but she might be called back to Tennessee sooner or later. Right now what matters is that she is here, and Maisie has her best friend back.

(Speaking of Maisie, I will post a medical update soon.)

Failure to Thrive

Maisie has received this diagnosis on and off for years.

She was the size of a 6-8 month old from 10 months to over a year. Failure to thrive.

She had a sudden growth spurt and became a chubby, toddling two-year-old. Thriving.

At three and a half, she developed tetanus again and lost fifteen pounds. She only weighed about thirty-five. Failure to thrive.

By her fourth birthday, she had improved by leaps and bounds. She was eating and growing and learning how to walk and talk again. Thriving.

Once a month she gets weighed and measured. That day was yesterday.

At 30 inches tall and 21 pounds, she's the size of a two-year-old.

A little two year old.

There it was again: failure to thrive.

She's been switched from her blenderized tube feedings to a super-high-calorie prescription formula. It smells gross.

On Friday, she will start receiving growth hormone shots.

The doctors want to see kids her age grow three to four inches a year. In the past two years, she's grown like three inches.

It's time to take action.

She will be having weekly weigh-ins and measurements. The goal is 1/4 inch a month and at least two ounces per week.

She's never grown that much or gained that much weight in her whole life.

(obviously I'm exaggerating.)

But still.

Cocktail Party in Pediatrics: An Autobiography

I apologize for the lack of posts. I'm in vent training and have been spending most of my time either in conversation or meetings with doctors or learning how to care for a vent-dependent child.

I won't be allowed to take Maisie home until I pass a bunch of tests.

So I'm taking this pretty seriously.

Meanwhile, Miss Maisie the Great has been throwing a cocktail party in pediatrics. The doctors are trying to find the magic combination or "cocktail" of drugs that will give her or help her get the strength to breathe on her own.

That's what her autobiography should be called.

Cocktail Party in Pediatrics. An endless trial to find the right combination of meds.

I won't bore you with the details, at this they're switching up her meds, running trials, giving tests, poor thing's had more PFTs, chest x-rays, and neuro workups in the past two weeks than she'd ever had before.

In other news, she is doing FANTASTIC in speech therapy. We got her passy-muir speech valve in last week and she has been working so hard with it. She is now saying about a dozen words consistently and clearly.

1. Mama
2. No
3. Yes
4. Ow-wow
5. Ball
6. Pay (play)
7. Hi
8. Bye-bye
9. Dolly
10. Teddy
11. Don't
12. Want
13. Top (stop)

Okay, so thirteen. I might be missing a few. Since November I have carefully recorded every word that comes out of her mouth, and she maxed out at 62. We're a long way from there, but it's encouraging to see that all or nearly all of the words she is saying now are words she already knew, which tells us that she is not forgetting the words. She is just losing her ability to enunciate them.

You might also notice that like half of them are complain-y words. No, ow, don't, stop, etc. This all stems from her hatred of the ventilator and the trach. We are happy to let her complain, yell, kick, and scream as much as she wants. She's using her muscles and her vocal cords, and she is completely at liberty to hate the stupid trach. Hopefully the more she complains about it, the sooner it will be gone.

She also started physical therapy and has been doing laps of the hallway outside her room in a walker or a gait trainer. Jeff brought her stander here a while ago and she does her hour of stander time every day. We met with orthopedics a few days ago, and after an x-ray and an assessment the doctor declared that everything looks good. Her bones are structured well and in the right place. A calcium supplement has been added to her daily medications to ensure that her bones stay strong.

To the untrained eye, Maisie looks good. She definitely seems to be getting stronger. She is happier, peppier, and much more awake, lucid, and interactive.

We still don't have any estimate when we'll go home. The doctors want her under their supervision as much as possible until they find a good cocktail of medicines, so it seems we might be here for a while. Alternately, we might move into the nearby Ronald McDonald house and she will continue seeing the doctors as an outpatient. But first, I have to pass my trach care tests.

(today is day 57, btw)

Day 45

Dear Diary,

Today is my forty-fifth day on this strange planet. The natives call it "the Hospital."

The food here is not unlike that from my home, but it is strangely flavorless.

Despite having access to a variety of dishes, many of those who live here choose a diet made mostly of pudding, jello, orange juice, and ice chips.

Some of the natives do not eat at all but are fed homogenous liquids through holes in their stomachs or a needle in their arm.

I have observed that the people here seem to belong to several different tribes, identifiable by their distinctive clothing.

The men and women in ill-fitting, matched shirt-and-pants sets are in tribe called "Nurses."

Some people wear similar attire to the Nurses but with the addition of a sort of stiff, white coat. These are Doctors. Some Doctors wear suits or dress shirts and slacks under their coats.

There are a small number of adults with a constant look of worry on their face. They are typically in comfortable, soft clothing and do not bathe or change their attire as frequently as they probably should. They wash their hands far more than I would deem "normal." These are Parents.

Members of all three tribes work together to serve small beings of a different tribe: the Patients. They spend much of their time in bed, often wearing simple dresses.

I fear that I am succumbing to Stockholm Syndrome, dear Diary, for though I hate it here I will not leave until my captors, members of the Doctor tribe, tell me I can go. I am free to leave, Diary, and yet I will not. I am afraid even to leave this room long enough to shower or eat food. I am going crazy, Diary. Why am I doing this? Why am I behaving so strangely?

Oh, I know.

It's because I'm in love.

I'm in love with a little girl with gigantic eyes and a beautiful, rare smile.

My Maisie girl.

Screaming therapy

Today is our forty-second day in the hospital.

I'm going kind of crazy.

Don't get me wrong, I love this hospital. Our doctors and nurses are fantastic, the facilities are beautiful, and the care here is absolutely top-notch. But it's a hospital. If you ever meet someone who says they enjoy the hospital, they've never spent sixty-seven consecutive days in one (and that's just my record. That's nothing in the medical community. A NICU friend was hospitalized for almost a full year.)

Anyway, despite my cabin fever, today was a good day. Maisie is adjusting to the trach as well as could be expected. Her first day and a half after surgery were pretty rough. She historically does well with anesthesia, but she just had a hard time coming out of it this time around and there was a lot of vomiting and general loopiness. (Loopidity? Loopy-ness? I have no idea.)

The third day was really spectacular. She vomited up her dinner, spiked a fever around 8:00, sweated it out at 104 for two hours, slept for nearly twelve hours, and woke up a totally different girl. She found her voice and complained loudly and relatively coherently about the ventilator for the next hour. 

Speech therapy started immediately.

I should make it clear that Maisie is not mentally impaired. She is as intelligent and aware of her surroundings as any other five-year-old. She sometimes appears mentally disabled because she struggles with speech quite a bit. She was quite chatty as a two-to-four-year-old, in that sweet spot between recovering from her traumatic infancy and the onset of myasthenia, but her speech tapered off dramatically a few months after she turned four because she was losing the muscle tone necessary to coordinate speaking and breathing and walking or moving at all. After she started treatment, her speech improved a little bit. 

Up until November of 2014, Maisie had a vocabulary of about two hundred words. Her speech dwindled and now she has mastery of maybe fifty words, none of which she employs with any regularity. We have all been delighted to hear her complain loudly and verbosely about the trach and the vent and encourage her to yell and protest and even kick and scream as much as she wants. It's all good therapy XD

Update

(this should have been posted on January 17.)

A lot's been going on, my friends.

I don't even know where to begin.

Maisie was re-evaluated by a whole team of doctors -- a pulmonologist, a thoracic surgeon, an ENT, a child psychologist, a general pediatrician, a respiratory therapist, a Myasthenia specialist...I don't even remember who all -- on Wednesday. They all agreed that she is struggling to breathe over the vent, and the nurses who stopped in testified to how much she hates being on a ventilator. She works very hard and very deliberately to knock it over, disconnect it, even kink the tubing. 

The CP didn't really have much to say. She was there because there is a psychological component here since Maisie clearly hates the vent, but seems very confident that if Maisie becomes vent-dependent, she'll get over it. She also said that if Maisie would probably work hard to be off the vent and if the deciding factor is whether or not Maisie will do the work to breathe on her own, then she should be learning to breathe on her own again.

The thoracic surgeon had previously ordered an MRI on her lungs and after examining the images declared that her lungs are still healed nicely from the lobectomy three years ago. He was confident that her breathing troubles today have little or nothing to do with the missing lobe and doesn't think any more surgery is necessary.

The pulm agreed that what is left of her lungs seems healthy, i.e. nothing else has died. She has had a chest x-ray, a dynamic MRI, and a billion PFTs (pulmonary function tests) all of which showed clear lungs that are expanding less and less the longer the test goes on. Her dynamic MRI actually showed her diaphragm starting to shake with fatigue after five minutes.

The respiratory therapist and the Myasthenia specialist conferred for a long time over whether or not Maisie will gain the strength back to breathe. And the answer is still up in the air. The therapist and the ENT were sure about one thing: Maisie cannot be orally intubated any longer. Her trachea and lungs are torn up and bleeding and she will start to have other problems if we don't make a decision.

So here it is: Maisie got her trach early yesterday morning. The surgery went well. We are very hopeful that the trach is a temporary solution while Maisie's meds are adjusted and wiggled and we try to get her diaphragm's strength up again. The MG specialist has seen cases like this go both ways, and there's really no way to know what will happen until it happens.

I AM EXCITED

GUYS

I got to go home for the first time in like forever today!!!!

Maisie has been extremely on-again, off-again this hospital stay and even Jeff was more than willing to stay with her (and oftentimes both of us were there) I was afraid to leave Maisie lest something happen to her (she's at NYPSC -- the pediatric specialty care hospital in NYC -- so she's like two hours away from home.) BUT she's been really stable for the last few and I really needed to shower in my own bathroom. Like really really really. GOD I missed my shower gel! And my loofah! And my mirror!

(obviously Jeff brought me stuff like soap and towels and a change of clothes but I mean there's only so much a man can do.)

This was the first time since Maisie's hospitalization that I actually went home. I left the hospital to go get food and some stuff from CVS a few times, but other than that I really haven't left. So it was AMAZING to be in my own house again.

I packed like a freaking sherpa while I was home. Ever since we heard rumors of a trach, I've been obsessively reading this blog. And I laughed like a hyena when I read this post. It's so true!

I brought a suitcase for me with jammies, fresh clothes, dryer sheets to keep everything smelling nice, my favorite lotions and and soaps and washcloths and towels and food and just...a lot of stuff.

Then I packed a suitcase for Maisie full of fresh clean clothes so she could get out of the hospital gowns. I packed literally almost every toy and movie and book we have. I brought her huge collection of button buddies so we could stop relying on the itchy gauze pads. And I brought her "Maisie the Great" sign that my mom made :)

Maisie herself has been doing okay. She's still working very VERY hard to breathe over the vent, even though it is obviously very hard for her. It's very clear she hates the vent and wants off of it.

Exhibit A: Four times Maisie has clenched the tube in her little fist and yanked. She has very nearly completely extubated herself twice.

Exhibit B: Maisie figured out about a week ago how to disconnect the endotracheal tube from the ventilator and has done so several times a day. Now she's got No-Nos. I don't know why she's surprised.

Exhibit C: The ventilator got moved a little closer to Maisie than it usually is so that a nurse could get to the wall, and Maisie managed to kick the vent over and break it. Luckily it was fixable and the portable vent was sufficient until she got hooked back up to a stationary.

Maisie the Great Troublemaker.

Bodies

I had my third ultrasound this morning and it's been confirmed that our baby has some limb differences.

Her right arm is amputated below the elbow and her right foot is weird. Hard to tell from the ultrasound what "weird" means, but it's pretty obvious that it's not normal.

And you know what?

I don't really care.

I know that some people might be really devastated by this news, but the thing is, our little squish has no abnormalities in her lungs, heart, brain, eyes...none of the stuff that really matters.

And that stuff is what really matters. In the long run, kids who have limb differences lead much more "normal" lives than kids with complex medical problems -- kids like Maisie.

I've been perusing blogs like This Little Miggy, whose daughter "Lamp" has limb differences affecting all four limbs.

And she's just fine.

We have no reason to believe that Squishy will be any different. (yeah. Squishy. That's her nickname. We don't know what her gender is, but I feel certain Squishy's a girl :)

Seventeen days

That's how long we've been in the hospital.

Seems like a lot long than two weeks, but that's all it's been.

Maisie is fighting hard to breath over the vent and while she's not doing a great job right now, it's obvious that she's trying.

She's perked up a bit in the last day or so and was even alert and happy enough to dance to Taylor Swift and ring in the New Year.

She's off of antibiotics and anything other than what she takes normally (Mestinon, Imuran, Zyrtec, Levalbuterol, Keppra, diuretics, Prozac, digestive enzymes.) Her chest tube was finally pulled two days ago and she officially no longer has pneumonia or pleurisy/pleural effusion. So that's good. Her breathing problems are completely due to hypotonia.

I did have a level-two ultrasound a few days ago and it seems that my baby might have limb differences. I have another scan day after tomorrow to get some clarity, but the consensus seems to be that nothing besides her appendages are affected -- normal brain, heart, lungs, etc. Which is awesome. After everything Maisie went through, I honestly can't complain over a missing hand.

<3 Charlotte