Bump in the Road

Thursday night and Friday morning, Lively's breathing became more of a problem. He had a chest X-ray, which evidently looked pretty normal. His oxygen saturation was normal. Around 3 am Friday morning I fell asleep next to him. At 5 am I woke up to the sound of his rapid breathing and found his nurse, the resident on call, and the respiratory therapist standing over us. They had already given him a breathing treatment (which had not woken me up) and it hadn't helped. I sat in his bed and held him and tried to help him change positions to make him more comfortable. Nothing worked. I called Pete to come in.

Things quickly got worse. His respiratory rate was in the 40s, then the 50s. I could tell that he was working really hard to breathe. Pete arrived and the room filled up with people as the rapid response team arrived. Pete traded places with me and held Lively as the team wheeled the bed to the pediatric intensive care unit (PICU). When we arrived, Lively was breathing 60 times per minute and he was clearly getting tired. He had stopped crying and looked very sleepy.

The doctors intubated Lively to protect his airway. This means that he has a tube in his throat and a ventilator helping him to breathe. Friday afternoon, the ENT surgeons (ear, nose, and throat) took him to the operating room to evaluate his airway. They saw that he has tracheomalacia, which is a narrowing of his upper airway. From what I understand, this isn't a problem for him unless he gets an infection of the airway or some other injury that causes swelling; a small amount of swelling can become a big problem for him.

We don't really know what triggered this event for Lively. He may have contracted a virus or bacterial infection. The ENT doctors think that when he vomited Thursday evening, he aspirated some of the stomach secretions, which can cause a chemical injury to the airway. I tend to think this is what happened also, because the onset of his respiratory symptoms coincides with an episode of vomiting.

This morning, the team tried to extubate him. His lungs sounded clear, and he had been on minimal ventilator settings all night. He must have still had too much swelling in his upper airway though, because he immediately had distressed breathing again when the tube was removed. So he was quickly reintubated. They're not going to try to take him off the ventilator again for at least 2 days.

Here's a picture of him that I took yesterday.

The plan for the next few days is for him to rest and heal. He is stable right now. The doctors here say he's going to be OK. I have to believe them.

I'll write about his IV dramas later.

Hospital Day, um, 3

It's hard for me to remember what day it is when we're in the hospital. We arrived at the hospital Tuesday night and were admitted Wednesday. Fortunately we were able to see Lively's neurologist Wednesday afternoon. He was feeling quite a bit sicker by that point, and had developed some GI symptoms, so he had a planned admission for continued hydration for a likely gastroenteritis.

Here's a photo of Pete holding Lively when we got to his hospital room.

Doesn't he look like a shar pei?

Because Lively's neurologist had wanted to repeat an extended EEG, she arranged for him to have it now since he had to be here anyway. Here's some video of him looking very cute in his EEG hat. (They cover the EEG leads with gauze to keep the kiddos from pulling them off.)

We don't have an official read on the EEG yet, but we were told (by a fairly noncommunicative team of doctors, not Lively's usual neurologist) that they do not see hypsarrhythmia, which is related to the infantile spasms. We also were told that the EEG looks "good" and "improved," but we don't know what that means yet.

Just a few minutes ago, like flipping a switch, Lively's cough changed from infrequent and dry to productive with wheezes and stridor. He's not having distressed breathing, which would mean that he had to increase his effort to breathe using accessory muscles, and his oxygen saturation is fine, but he sounds like crap. He just had a breathing treatment which didn't really help. His chest x-ray evidently looked fine. So right now, another thing to watch.

ED, Part 2

Lively and I have been in the ED close to 13 hours. He's getting IV hydration, so he's starting to have wet diapers again. I managed to get him to take about 2 or 3 ounces of breast milk from a bottle, but he still refuses to nurse, and for a boy who usually drinks about 5 ounces every 4 hours when I'm at work, the small amount he drank is a drop in the bucket. He's probably had less than 5 ounces by mouth in the last 24 hours.

Lively had a chest X-ray this morning. It looks like this:

To me, the most interesting part of this picture is the massive amount of soft tissue around his face. He continues to lose weight since stopping the ACTH, but this shows how far he has to go. Anyway, I don't read chest X-rays very often (and never pediatric ones), but the radiologist read it as "diffuse haziness in the lungs bilaterally . . . no focal opacities to suggest pneumonia." Basically, a wishy-washy call, for which our radiologists are famous. Lively has a very mild dry cough that he has had for the last 3 days or so, and it didn't worry me at all at home, but now, in combination with the radiologist reading (which is likely an overcall of a normal X-ray), I'm worried about PCP pneumonia. This is probably a case of me knowing just enough to worry myself needlessly about something that is unlikely. To my non-medical blog readers, please refrain from googling this pneumonia. I'm happier alone in my worry-boat right now, and I'd prefer that no one else join me. (This is meant especially for my 4 parents who are opening browser windows to read more about PCP as they are reading this post-- close down the search engine!)

I emailed Lively's neurologist early this morning (did I say that in my last post? I haven't slept much, so I can't remember). This is one of the big advantages of my job -- I can email Lively's doctors who work for the U, and they usually either call or page me back. I'm hoping she'll see us today if we get admitted to the hospital and have to miss the appointment with her. I'm really not interested in opinions from other neurologists (except for one, who happens not to be on service in the hospital this month).

Here's a photo of Lively right now, resting quite comfortably.

Back in the ED again

Last Tuesday (April 13), Lively got his last dose of ACTH. He was miserable with this, so we were thrilled to move on. On Friday (April 16), we increased Lively's topamax to what should be his maintenance dose. We knew that the ACTH was stimulating his appetite, and that the topamax may decrease his appetite. Lively had gotten huge though, and I looked forward to his slimming down a bit.

As soon as we stopped the ACTH, Lively not only seemed so much more active, and happier, but his appetite decreased immediately. When we increased the topamax, his appetite hit an all-time low, so that Lively transitioned from starving, insatiable baby to one who pretty much refused all food and drink within a few days.

I let things get away from me. Lively nursed a few minutes yesterday, and ate a couple bites of pureed peas at lunch, and fell asleep. When he got up from his nap around 5 pm, I realized he hadn't really had a wet diaper all day. And in looking back over the previous 2 days, I realized he'd only had 1 or 2 wet diapers a day even then. Still, he refused to eat or drink. I decided to try to rehydrate him orally at home with Pedialyte and breast milk from a 5 ml syringe. I got about 4 ounces in him (that's 24 squirts with the syringe) and he threw up all over me. So I thought, some must have stayed in there, we'll give it one more try. I cleaned him up, cleaned me up, force-fed him 1 ounce, and, as Annie would say, BOF!

Here's a photo I took of Lively a couple of hours ago:

He doesn't seem to have any kind of underlying infection. His white blood cell count is normal, and thankfully, my refusal to acknowledge that he was becoming dehydrated has not seemed to affect his kidneys (normal BUN and creatinine). The ED resident (that's emergency department; we don't call it an ER anymore) spoke to someone in pediatric neurology (not Lively's doctor, she's not here) who thinks the problem is unlikely to be related to the topamax. I'm not sure I agree. I trust Lively's neurologist and we have an appointment with her tomorrow, so I'll believe whatever she tells me.

Lively has had 2 IV boluses and hasn't peed yet. He nursed a little, which is good, but by a little I mean 2 or 3 minutes tops. If he doesn't pee in the next 2 minutes, he's going to get his third bolus, and we can't go home until the hunger strike is over; otherwise, we'll just have to come right back here. We haven't been admitted to the hospital yet, but I think that's probably coming soon.

Got my baby back

Last week, on Wednesday, we decreased Lively's ACTH from 0.2 ml/day to 0.1 ml/day. Each week we decrease by 0.1 ml and our maximum dose was 0.7 ml, so we've been doing this for quite awhile (due to some unexpected adjustments, Lively has been on ACTH for almost 9 long weeks now). ACTH has been a mixed blessing. Lively's seizures have certainly improved, which I understand is very important. However, the side effects have been very difficult for our family.

We're on the lowest dose of ACTH now, and there was an instant difference when we switched. Lively immediately slimmed down considerably. So much of his weight must have been related to edema that when we lowered his dose, his cheeks and chins shrunk within hours. Literally. He went down for a nap, and when he woke up, he was less puffy.

Lively still doesn't look like himself entirely, but I can see him when I look at him now -- and this is something I haven't been able to do in weeks, even months. Better yet, his personality is coming back. Pete and I really didn't know how bad things had gotten until we realized, when we heard Lively laugh again, that we hadn't heard him laugh in weeks. He's cooing again, and babbling, which hadn't happened since before the seizures started. He rolled over today, which he also hadn't done with any regularity since before the seizures. And then he rolled over again. And again! Best of all, he does his happy dance while squealing with delight. It's the best sound in the world.

Pete told me a few days ago, I feel like we're getting our baby back. I had been thinking the same thing.

I haven't seen any seizures in several days -- at least, I don't think I have. Sometimes I see little things and wonder, but certainly there's nothing obvious. We're increasing Lively's topamax each week, so we have new side effects to contend with, but they're not nearly as bad as the ACTH. By way of trade off, the topamax must taste disgusting, because Lively fights every dose (three each day) and Pete and I are at wit's end trying to find a way to get him to take it. We've tried a suspension in a syringe, sprinkles mixed with jam (and every other food imaginable), but nothing works. Suggestions, anyone?

I'm trying to be optimistic, and I'm working hard to enjoy my precious baby all the more now that he is happy and more comfortable. However, now that I know that anything can happen (meteors falling out of the sky, or my tiny baby having a stroke), I can't help but worry that this new progress may not mean what we think, that there are new manifestations of the stroke we haven't seen yet. I continue to struggle with living in the moment.

I also continue to be grateful for the love and support from friends and family. Pete's parents recently took care of Annie and Lively on a Saturday so we could see a movie! Pete's sister visited us for a few days and really took care of all of us while she was here. My mother came to take care of us this last week while Pete and I were both on vacation, and we appreciated that so much. My grandmother and stepfather joined my mom over the weekend and we had a nice visit -- and Annie and Lively enjoyed some great-grandparent and grandparent time. Some friends from work recently arranged for dinner to be delivered to us weekly, and I can't think of a single thing that could be more helpful. So thank you, thank you, thank you, to all of you for reading Lively's updates and for your kind support.

Here's a photo of Lively that I took today. He fell asleep while I was getting his lunch ready for him (this never would have happend a couple weeks ago -- food came first). I couldn't wake him up enough to eat, so I eventually just put him down for a nap. I especially like this photo because you can see Pete sleeping on the couch in the background.

Seizures return

Two nights ago, I noticed more spasms when I was feeding Lively. He did his eye-rolling thing, which I haven't seen in around three weeks. My heart just sank. I asked Pete to come over and watch, and he confirmed that he was, in fact, having spasms.

So here we go again. It's amazing how quickly we became complacent. Despite all we had been through, 3 seizure-free weeks made us feel like we were done with them.

I talked with Lively's neurologist on the phone the next day. We're lucky to have her. She lets me email her between visits, calls to check up on us, and is happy to talk on the phone if we have questions. We're going to increase his topamax over the next 3 weeks and see how he does, but if he continues to have spasms, we're likely going to end up in the hospital again for another 24 hour EEG.

Pete says he's noticed a couple more clusters of spasms over the last 2 days, and our babysitter evidently noticed some also. We've only been on the new dose of the topamax for the last day, so I'm going to give it a bit longer before I (1) report the ongoing spasms to his neurologist or (2) freak out.

Meanwhile, it's spring and feels almost like summer, which is both good and bad. It's great for Annie, who needs to run around and play outside, and who loves going to the park with all her heart. Here's a picture of her on her bike at the park this week.

Spring is bad because all around us, other people's kids are acting normally, playing and running around, talking and walking. And it reminds me that I don't know if my little boy will be able to do those things. I don't begrudge the other kids their running and play time, but sometimes it's hard to see how far we're going to have to go.

We took Lively to the park on the same day. Here's the evidence.

While we were at the park, we met a woman with her 14 month-old little girl. She was very nice and her daughter was cute, walking and playing normally. Her daughter was swinging next to Annie, so we struck up a conversation:

"How old is your little boy?"
"Eight and a half months now."
"Is he crawling yet?"
"No. Not yet."
"Well he will be soon. Crawling around everywhere, getting into everything."
"Hmmm. Yeah."
"Wow, his cheeks are really fat!"
"Um, yeah."
"He must be a good eater."
"Yes, he has quite an appetite."

OK, I've probably instigated conversations like this myself with strangers in the past. She was being friendly and nice, and of course there was no way she could know there was anything wrong with Lively. But it was painful.

She kept asking nice, friendly questions until it came to the point that I either had to lie about Lively's ability to meet some milestone, or I had to tell her what was going on. I think eventually she asked me if Lively sleeps through the night, and I told her no, but it's because he's taking a medicine right now that makes him very hungry, so he wakes up to eat every few hours. "What's the medicine he has to take?" "Why does he have to take that?" "Why is he having seizures?" "Why did he have a stroke?" And so on. I think she may have told me a story about some kid she knows who has seizures or who has had a stroke.This did not help me feel better.

It's hard to know what to do in these situations. Pete thinks I should just say yes, he sleeps through the night, and avoid the rest. But I don't want to paint a picture of Lively that is anything but who he is exactly. I'll let you know when I figure this one out.

Finally, I'm including a photo from a few nights ago. I was working in the basement, and it was late and Pete wanted to go to bed, so he brought Lively upstairs to give him a bottle before bed. We've been trying to fill up his tummy with milk mixed with cereal before he falls asleep so that hopefully all of us will sleep for longer stretches. So far, it's not working. Anyway, Pete put Lively in bed with him and promptly fell asleep. I thought this little scene was so cute.

My boys!