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 :)