Unexpected Break

Charlotte has had a rough week and we are currently deciding whether or not to have a trach placed. I’m sorry for being MIA, but if there is anything I can share about having a preemie, its that sometimes, everything else drops to the side, and you have to focus on their needs for a bit.

Charlotte’s sleep apnea is not under control and she had a severe event last week, which left her grey and cold, and me resuscitating her once again. This is her third event like this since January, and we just don’t know if it will happen again. If it does happen again, and we aren’t able to revive her, or it takes too long to revive her and she has brain damage, will we ever forgive ourselves? On the other hand, what if her apnea is something she will outgrow in a month or two, and we’ve had this trach placed? And the debate rages on.

If we could know whether or not her apnea is a life long condition (due to genetics or permanent brain damage) or something only temporary (related to an immature respiratory system), we would feel so much better about making this decision.

But we can’t know. There’s not a right or wrong answer.

There’s only our answer. Our choice for our child.

We make a final decision tomorrow, when we’ll meet with the ENT surgeon and the pulmonary doctor. Our hearts are heavy that we’ve come to this point, but hopeful that we will find an answer that bring us peace and Charlotte safety.

Thank you for all you do.

Sincerely, thank you.

Update

Charlotte has been doing really well lately. We just had a follow up yesterday and here are her stats:

Weight: 9.8 kilos (21.6 pounds)
{15%tile for actual age/ 50%tile for adjusted age}
Height: 72 cm (28 inches)
{2%tile for actual age/ not on the char for adjusted age}
Head Circumference: 42 cm
{not on chart for actual or adjusted age)

Head: Charlotte has what’s called “microcephaly“, meaning, her head is more than 2 standard deviations from the average head for age and sex. More specifically, Charlotte has micoencephaly, which is a small brain, thus accounting for the small head. {We know this because Charlotte suffered a pretty severe stroke in the cerebellum, leaving very little brain matter in that space} However, luckily for Charlotte, she doesn’t appear to have a small head, so it’s all good. Her pediatrician just wanted it to be an official diagnosis. Yay for more labels. :)

Hearing: A new one, right? Charlotte’s had a bit of a “regression” in the vocal department as of late. Prior to our move, she was making vowel sounds, and the occasional consonant/vowel sound. Lately, it’s only been noises, which is good, because it means she can manipulate the vocal cord, but bad because she’s not making any progress verbally. We want to check her hearing to make sure this is not a factor in her speech delay. Right now, we’re only going for the standard hearing test, but if she still has a vocal delay in say, four to six months, we’ll have to go for a sedated hearing test.

Lungs: Doing well! We’re weaning her from some of her diuretics, and so far, so good {knock on wood}. She’s been consistently off of oxygen for over a month now, and we’re thrilled to see how stable she has been.

GI: Still the same. J-tube feedings 18 hours/day. We’re hoping that soon (in the next few months) we can start working towards G-tube feedings, and then possibly condensing those feedings so that they are at specific times (called bolus feedings) rather than the continuous feeding. The hope is that we can eventually get her to “eat” during regular meal times, and then start weaning those tube feedings so she can be hungry enough to eat by mouth. Basically, we’ve got a lot of therapy in our future. And I’m not just talking OT :)

Developmental: We’ve started with our new therapists here in California. We’re seeing Occupational Therapy twice a week, Physical Therapy twice a week and Speech Therapy once a week (maybe adding another one on? who knows). Charlotte has started to crawl since we’ve moved her, and we’re thrilled with her progress. She still has a problem with rolling over from her back to her front, so if she ends up on her back, she just pushes herself backward around on the floor. The back of her head will never have hair on it :) She is, however, transitioning from crawling to sitting, and from sitting to crawling. It’s not elegant, but it gets the job done, and you can tell how excited she is about all of it.

ENT: The ENT team here has pretty much taken over the role of managing Charlotte’s Sleep Apnea. Right now, that’s probably her biggest struggle. Last night, she had 10 desats, a few into the 70’s. She hasn’t needed to be resuscitated in almost two months, but she does need repositioning and stim to bring her out of some of the more significant desats. She’s starting to tolerate her BiPAP mask less and less, and honestly, I just don’t know what to do for the poor girl. We have an appointment the beginning of August with the Airway clinic (pulmonology and ENT together) so hopefully they will have some better ideas about how to help her sleep. We still don’t have a real reason why she’s having the central apneas, and for that matter, we don’t have a real reason as to why she’s obstructing. We’re mostly hoping at this point that she’ll grow out of it. But that can take years (if it ever happens at all) and kids kinda need to sleep :)

Overall, Charlotte is happy. She’s playing a lot more now that she’s mobile, and its awesome to see her and Caleb play together so well. Basically, I just love this gal. She’s a keeper (in case you were wondering!)

We’re Home!

Phew. The past two weeks have really kicked my trash. CA, on the other hand, has handled things just fine.

So fine, in fact, that she is now 100% oxygen free. Who knew that all she needed was a nice sea breeze and high taxes?

Charlotte’s echo looked great, and the cardiologist is confident that her heart will remain fine without the oxygen. We’ve also gotten the go-ahead to only monitor her sat’s while sleeping. This means that during her “down” time from her tube feeding, she is completely tube and wire free. For three whole hours a day. It’s rather strange. I carried her around the house for the first time today. She’s 16 months old. I carried my baby. It’s rather surreal.

In other news, Charlotte is attempting to crawl as well. So far, I’ve seen her take about 3.5 crawls toward an item. That wore her out, though, and she has refused to perform since then. We’ll get her going though.

We meet with the San Diego Regional Center and a Special Education Teacher today to map out a therapy plan for the little one. We have a follow up appointment with her primary care doctor on Friday, and hopefully then we can know who we still need to see and who we can say “chao” to without looking back.

Of course, all good news has it’s counterpoint and Charlotte’s stay was full of that. It appears that her central apnea is pretty significant. To make things difficult, we can’t quiet figure out why she has central apnea. She’s too “old” for apnea of prematurity to still be a problem, and her MRI revealed that she does not have a malformation of the brain stem, which often causes central apnea. Which leaves CA as a bit of a mystery. Neurology also noted that she has hypotonic CP, although, at this point at least, the diagnosis is more to qualify her for services than it is an actual forecast of her abilities. And finally, her eye doctor told us today that while her eye sight looks great, her field of vision is reduced by about 50%. It’s the question I have avoided asking, but there it is. She has about 80 degrees of vision, and beyond that, her peripheral vision is non-existent. But hey! That’s 80 degrees she in all sense should not have. We’ll take it!

Currently, my two little munchkins are laying on the floor, watching some Go, Diego, Go! It’s been amazing to watch them interact like two “normal” siblings. You know, like Caleb shouting, “Momma, she’s bothering me!”

Sigh. I love it.

Of Course, It’s Always Followed By Bad News

So much for our winning streak.

Charlotte’s sleep study results were less than stellar. She continued to have pretty severe obstructive sleep apnea, accompanied by desaturations of her oxygen levels while on CPAP pressure of 5. When they tried to increase the pressure, she had less Obstructive apneas, but more central apneas. They tried switching her to BiPAP and she went to straight central sleep apneas.

Awe.some.

So we’re kinda taking the, “We’re stuck with this, let’s make the most of it” course of action right now. This sleep study has at least told us that, no, we’re not crazy, her sleep apnea has in fact gotten worse over the past few months. (We kinda thought we were staring to make things up). Currently we are increasing the amount of oxygen going into her CPAP, and maintaining a CPAP pressure of 5. Her pulmonologist figures that if we can keep her oxygen levels constant, regardless of apneas, then at least she isn’t sustaining brain damage while sleeping. (She was consistently maintaining sat’s into the 70’s, which is not the worst she’s ever done, but it’s not recommended.) However, her cardiologist might not like the extra work load that is put on the heart when you fail to, you know, umm, breathe. So we’ll increase the oxygen until her appointment on June 6th, where the pulmonologist warned us the T word might be used.

Tracheostomy.

To be sure, we are a long way off from this procedure. However, Charlotte’s heart is not exactly stellar, and her sleep apnea is only pushing it closer and closer to a rather ugly cliff. Pulmonary would like to see her tonsils and adenoids removed first, and we agree. (Even though ENT has said that a T&A will not do anything, as her tonsils and adenoids are not enlarged. Whatever.) If a trach is placed, it will only be done after we’ve removed all other possibilities. Cardiology, I’m certain, will recommend just watching her heart and making sure that there are not substantial changes to its structure, and/or function as her sleep apnea continues. Should one of those variables change, we’ll try medications to combat the stress placed on the heart by the sleep apnea.

Sigh.

It’s just like her pulmonary doctor said, “Charlotte, well, Charlotte… She’s just one complicated little girl.”

That she is. That she is.

An Update

I haven’t written a post like this in awhile, but for recording keeping sake, here’s Charlotte Amalie’s latest medical run-down:

Weight: a whopping 7.8 kilos (17.5 pounds)

Length: 63.5 cm (25 inches)

Eyes: Her left pupil still dilates differently than her right (scaring nurses who meet her for the first time, and neurologists) but we now know that the eye is related to the paralyzed vocal cord. We have our next follow up in April, and hopefully she’ll still have a clean bill of health.

Vocal Cords: Left is still paralyzed, but she’s making great improvement with how well she is compensating with the right vocal cord. We’ve started actual speech therapy (not just feeding therapy with the speech therapist) and we’re slowly introducing sign language. She will be re-scoped in March

Lungs: Charlotte fluctuates from needing 1/8 to 1/4 O2, depending on her heart rate and her oxygen sats. She needs 1/4 a night about half the time, but can almost always be weaned down to an eighth by morning. She’s still taking her diuretics to keep her lungs dry, and also has a few inhalers that keep her open and breathing. At her last pulmonary appointment they said she is currently better than she ever has been. Awesome! We’re thinking that after RSV season, we might just be able to kick this oxygen. So maybe around May? We sure hope so!

GI: Charlotte’s now on 20 hour continuous feeds. She’s doing so so so SO much better on the continuous feeding than she was on bolus feeding. We have an appointment in two weeks to meet with the Complex Care Center at CHOP and we’ll decide then if we want to start condensing her feeds again or if we’re just going to roll with the continuous feeds. We believe that if she can just make it another 9 months or so, she’ll be developmentally much more capable of handling the reflux and such. So we’ll decide if we just keep plugging along for another 9 months or if we try to handle the reflux now. Handling the reflux now means another “event” is possible and she’s more uncomfortable, but it’s much more physiologically “normal” for her to have bolus feeds than it is for the continuous feeds. The continuous feeds help her reflux, but they stunt her gut and stomach development. So yeah, flip a coin. Any suggestions?

Developmental: Charlotte is capable of rolling over, but I wouldn’t say she’s rolling over. She’s done it a few times, but it’s not a daily occasion.  She’s also capable of sitting for 20 seconds or so. These are our goals to master by HER FIRST BIRTHDAY (holy dang, it’s a month away!).  Cognitively she’s doing well, tracking and interacting and playing a lot more than she used to. It’s amazing how quickly skills develop. We’re also hoping that by her first birthday she’ll be passing toys from one hand to another and that she’ll be able to reach out and grab something more efficiently. Currently she reaches for things, but she has a bit of a hard time actually grasping things in an efficient manner. We’re working on it :)

Medicines: Daily: Aldactone 2xday: Prilosec, Diuril, Flovent, KCL 4xday: Erythromycin PRN: Albuterol, mylecon, tylenol

Notice how I didn’t label anything Oral or Feeding? Yeah, that’s because she’s not doing it. She’ll put her hands in her mouth, and she’s much less sensitive in regards to her gag reflex, but still nothing by mouth. Another goal for her first birthday, I suppose.

And that’s our girl. Medically speaking, of course.