Milestones

While I was away, we marked a lot of milestones for Charlotte. Her first time home from the hospital was August 26th. It’s been a year since I watch my daughter leave the NICU at Chester County Hospital. The NICU was incredibly hectic that day, and despite spending six months there, we left without much commotion. Our good friend Mr. Bobby was throwing a fit and the entire staff was working on re-intubating him. We felt it was only right that someone else should be getting the attention Charlotte so often stole during her long stay there at Chester County. Walking down the hallway, towards the elevators that had so often been full of balloons and presents for another baby, a celebration to mark the end of some other woman’s pregnancy, was now full of monitors and oxygen and carseats and a whole lot of junk that we had lugged into the NICU over many, many months. A yet, despite the lack of balloons and the plethora of electronic equipment, I would be hardpressed to say that any other family was as overjoyed as we were to bring our child home.

Charlotte comes home from the NICU
All ready for her ride home.

The first few days at home were beautiful and awful. You might remember. Maybe you’ve joined us since then, so let me inform you. The first night, we forgot to hook up Charlotte’s oxygen. Funny enough, she actually needed it. In a panic, we called the NICU and were planning on bringing her back to the ER when we realized that the tubing actually had to be connected to the M tank for the oxygen to do anything. Details, details.

Two days later, Charlotte started getting sick. She just seemed off to me and in my paranoia, I took her into the ER and she got a chest X-ray. Which, of course, looked alright. Two days later, however, she was admitted to CHOP with aspiration pneumonia and some virus which undoubtedly came from her loving and affectionate older brother.

That’s when things started getting dicey. Peter left for San Diego for an away rotation the day Charlotte was admitted. My parents were coming into town to visit Miss Charlotte Amalie at home. So much for that. My paternal grandparents came as well, planning on meeting Charlotte in a less “sterile” environment. Despite their fears of never knowing this great-granddaughter, my grandmother and grandfather came to CHOP to see their little miracle. They weren’t going to let a little hospitalization stop them. We spent Labor Day weekend playing tourists in Philadelphia, and literally as I was hugging them goodbye on that Monday afternoon, I got the phone call. Charlotte had spiked a fever, and her temperature was not under control, despite ice baths and medication. I don’t even remember what I did with Caleb at that point, maybe my mother stayed behind? I don’t know, it’s all a blur.

For the next week, I watched as my child slipped closer and closer to death. There was something ravaging inside her body, destroying her lungs, her heart, her kidneys, and blood test after blood test came back without answers. She maintained temperatures of close to 106 degrees Fahrenheit for nearly seven days. She was receiving the strongest antibiotics, the strongest fever reducers, the strongest antivirals. Nothing was working.

Finally, we were sent to another floor; a floor for chronically ill patients. Sometime in the middle of the night the nurse came in and asked how we were doing. I mentioned that Charlotte just seemed “off” to me, even though it appeared earlier that she was starting to turn the corner, even just a little bit. Charlotte had slept all day, and I couldn’t seem to wake her, even when I was changing a diaper or giving her meds. The nurse calmly started taking vitals, and her serene nature quickly turned panicked. Within moments, the PICU team was rushing to our floor. Charlotte had no discernible pulse, and virtually no blood pressure. Despite her body temperature rising from the fever, her limbs were turning blue. As I exited the room to make space for the PICU team, I heard words I’ll never, ever forget,

“Theady rhythm. No pressures. 3, 2, 1, CLEAR. Again! This baby isn’t waiting for us! 3, 2, 1, CLEAR.”

This happened several times over the next eternity. Ok, maybe a minute. I heard those doctors and nurses who had never even met my child give her everything they possibly had. They were so invested in her survival. So determined to keep her alive. When an aide came over and walked me to the family lounge I didn’t argue. I gave up. I had sat by her bed so many, many times, and I knew I couldn’t sit by it alone as she died. I didn’t have it in me to say goodbye to her all alone, in that dark room we had only come to stay in hours before. Not like this. This isn’t how it was supposed to happen.

I thought about calling Peter. How would I tell him I had failed? I had failed to keep our daughter alive. How would I tell him he could never hold her, never rock her to sleep again? How would he forgive me for allowing her to slip away when he was unable to tell her one last time that he loved her?

I couldn’t even bring myself to write about any of it on the blog. Even now, I sit in Starbucks, crying as the customers around me avoid looking at me. I couldn’t share my darkest moments, my greatest fears. My moment of complete and absolute destruction.

I don’t remember walking to the lounge, only that suddenly I was there, sitting in my pajamas at three AM, wondering who I needed to call first. I’m not sure how long I sat there, somewhere around 20 minutes. When the PICU nurse came in, I barely heard her speaking, until I noticed she was telling me that they were transferring Charlotte Amalie to the PICU and I could see her there. Somehow, in the back of my mind I realized that they wouldn’t transfer a child who had already died.

I rushed to the PICU, several floors away, and saw a baby so close to death it took my breath away. She had an IV in her leg bone, since her veins didn’t have enough blood pressure in them to get an IV in. She was back on the ventilator and her chest was bright red, while her hands remained blue. She didn’t look real. But there she was, alive. Alive as you can be when you’ve come that close to death.

The next day was a long one. The doctors told me we had about 12 hours left with Charlotte before they would recommend removing care. Charlotte continued to code, even while on the ventilator. Three more episodes she went through in a matter of hours. Her chest was raw. I asked when I should call Peter, when should I tell him to come home, and they told me to let him be until we came to the point of deciding to remove assistance. No need in worrying him until we absolutely had to. I was pretty sure we had arrived at that moment, but the doctors knew better. I didn’t know how to tell anyone– those words just wouldn’t come to me, so I sat in her room for hours. Telling her all about her family, the people who loved her, the amazing influence she had been on all of us. I sang her the songs my mother sang to me. I told her that Prom wasn’t much fun anyway, so she didn’t need to worry about missing out on it. My heart broke as the minutes ticked away.

And then, miracle upon miracle occurred. The med student ran in about 11 hours into our deadline. A culture had grown something. Tears welled up in his eyes as he told me. They had identified the bacteria and were setting up her medicine. The attending came in and hugged me. She couldn’t make me any promises, but her experience told her, Charlotte would live. The resident came in and sat by my side over the next three hours. After watching Peter’s residency schedule, I don’t know how she had time to do it, but she did. She cried and I cried as we watched Charlotte’s vital signs improve, literally right before our eyes. Her shift ended, and she stayed. She stayed the entire night with me, telling me stories of residency and medical school and her own family.

She came back the next day so she could be there while they extubated Charlotte. A few days later, when Miss CA was finally stable enough to move, she came in and held my baby. This resident whispered to my little girl that she would forever remember her, forever remember to never count a baby out. To never, ever give up on a family. To never, ever forget that each child is loved and needs love.

Days later, as soon as Charlotte was stable, they pushed to do her nissen and g-tube surgery. To risk another aspiration pneumonia was clearly not an option. And so, only days after our daughter died and was brought back to life, she underwent surgery. September 15, 2010. A mere nine days from the phone call I got while saying goodbye to my family in a hotel parking lot.

Days after her g-tube surgery

Miss Charlotte, days after her g-tube/ nissen surgery

A year later, this is the first time I’ve been able to sit down and tell the whole story. The fear of that week still lives with me; still paralyzes me every time I find Charlotte’s hand is cold, or she starts to run a fever. I still brace myself each morning that she may not have survived the night. I’m not sure it will ever go away.

And yet, we are the lucky ones. Too many families never get that 11th hour miracle. Too many families have to make those phone calls. They have to find the words I’ve still not found.

Hug your babies. Hold them tight. Sing to them the songs your mother sang. It all happens so quickly. And those moments that happen the fastest, will linger with you the longest. Make those moments worthy of lasting a lifetime.

Because a lifetime is undefined.

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!)

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.

Milk Scan

We got the results from the milk scan this morning and they told us, well, pretty much nothing. Her gastric emptying time is within normal limits and it showed several episodes of reflux. No big announcements there.

So we’re waiting to hear back from the GI department to see if they want us to go for a scope to check out her esophagus or if we’ll just wait and see how things play out once she has her sleep study.

Hurry up and wait: the story of Charlotte’s life.

Literally.

GI and Pulmonary Appointments

Charlotte has had a busy week. Yesterday she meet with the GI department at CHOP and today she had a follow up with the Pulmonologist, had a milk scan, and was evaluated for a sleep study. Phew. I don’t know how she keeps up with herself… did that make sense? Must be the lack of sleep.

Anyway.

The GI suggestions depend a lot on the results from the milk scan. Charlotte’s been having some problems with her feedings, and has a difficult time keeping her oxygen saturations up while sleeping.

I’m going to ramble here with a bit of an explanation, so those who want to can skip it.

When Charlotte had her g-tube placed, she also had a nissen fundoplication done as well. The nissen procedure takes the top of the stomach and wraps it around the esophagus, creating a valve that only allows things to come from the mouth into the stomach, and not allowing things from the stomach to flow up to the mouth. Vomiting, burping, spitting up, etc are not “possible” for Charlotte. {the quotes indicate that anything is possible in real life}. Since part of Charlotte’s stomach is wrapped around the esophagus, there isn’t as much space in her belly for food. So she can only take about 2 ounces of formula at once. (A 10 month old typically takes about 6 ounces at a time). So Charlotte gets 2 ounces 4 times a day, and then, to make up for the missing calories and fluid, she is fed continuously at night for ten hours. Charlotte has been having an increasingly difficult time with these nighttime feedings. She’s been gagging and retching so much that she often turns blue, and cannot breathe, which tends to be an important thing to do. Luckily we have nursing and they have fancy things like ambu bags to keep her breathing.

So far, we’ve changed her fluid volume, spread her feedings out so they take longer (she’s fed via a pump that feeds at a certain rate since she can’t stretch her stomach fast enough to take all the volume at once), and added special bags that should help her vent (she can’t burp, so the air has to be vented from her belly). So far we’ve seen some improvement, but not enough to be thrilled. Actually, not even enough to be comfortable with the current status quo.

The surgery team (who placed her g-tube and performed the fundoplication) and the GI team are currently suggesting that we change Charlotte to a GJ tube. Her current feeding tube feeds right into the stomach, while the GJ tube would feed into her stomach during the day, and into her intestines during her night time feedings. We’re not totally on board with this suggestion, as we feel it takes care of her symptoms and doesn’t address the problem behind it. Of course, that’s a problem in and of itself, since we have NO idea what that problem actually is.

So we’re waiting it out. We’ll get the results from the milk maybe tomorrow, but probably next week. From this scan we’ll find out if her stomach is emptying too fast (called dumping syndrome) or if it is emptying too slowly (called delayed gastric emptying). Either of these problems could causing the gagging/retching episodes that we have been witnessing. We’d treat dumping with fiber (tricky, huh?) and delayed gastric emptying would be treated with a motility agent (usually an antibiotic).

If it isn’t one of those, it could be that her esophagus is narrowed. At that point, we’ll work on treatments for that.

Clear as mud, eh?

Pulmonary wrap up will have to come in another post. But you knew that was going to happen, right?