1. NEUROLOGY: Lively's seizures recurred after he was extubated. Pete and I noticed clusters of eye rolling that lasted usually 10 to 30 minutes. These happened 1 or 2 times a day. The neurologists had started Lively on a second anti-seizure medication (Keppra) when he was in the ICU, so they recommended increasing the dose of this new medication. So far it has seemed to help. Meanwhile, Lively still refuses to eat or breastfeed, and Pete and I strongly suspect that the Topamax is behind this. Because it was such a struggle to get Lively to take this particular medication, Pete and I both tasted it, and, even mixed with Hershey's chocolate syrup, it is bitter, horrible stuff. We both decided that we can't force him to take it anymore, and the neurologists agreed. Unfortunately, we have to wean him off the Topamax even more slowly than we increased it, so it will be several weeks before we're done with it.
Pete and I both suspect that the Topamax may be a huge contributor to the problems that are keeping Lively in the hospital: metabolic acidosis, anorexia, vomiting, diarrhea. It's hard to be sure though. He has been on it for quite awhile, although ACTH was on board to counteract some of the side effects.
2. PULMONARY: Lively's breathing has improved significantly. He doesn't require any oxygen at this time. He has a bit of a productive cough, but we think that he's probably still getting rid of the stuff that collected while he was on the ventilator. It hasn't gotten any worse since he was in the ICU. He continues to be tachypneic, but this is likely due to the acidosis. He continues to vomit, however, which is a big concern, because we're not sure that he can protect his airway adequately. See GASTROINTESTINAL, below.
3. CARDIOVASCULAR: Lively's blood pressure is a bit on the high side, but it has been ever since we started the ACTH. It doesn't seem that anyone is terribly concerned about it, but in light of some of his other lab abnormalities, the renal (kidney) team is looking into it more closely. I'm starting to feel that this may be an ongoing issue for Lively, because his blood pressure was a little high before we started the ACTH. Lively did have an echocardiogram in February that was normal.
4. GASTROINTESTINAL: The occupational and speech therapists saw Lively several days ago, and because he coughed and sputtered every time he breastfed or tried to swallow thin liquids, they recommended a swallow study. For this, I fed him some contrast with a spoon, and the radiologist took pictures as he swallowed. We were able to see that not all of the contrast went down his esophagus and into his stomach -- instead, some of it passed into his windpipe.
I don't believe that this was occurring prior to this hospitalization. Lively never had any coughing or choking while drinking prior to his intubation. However, because it is happening now, Lively can no longer drink any thin liquids. Any that we offer him by mouth must be thickened (using a special tasteless gel) to "nectar" consistency. Because Lively continues to refuse to eat or drink anything, right now he is being fed by a tube that goes from his nose, through his stomach, and into the first portion of his intestine. I am pumping milk for him, which is the only nutrition he is tolerating right now. Fortunately, breastmilk does provide him with all of the protein, fat, and carbohydrates he needs in a day. His only nutritional supplement is a multivitamin.
In addition to the poor appetite and vomiting, there is also a question about some reflux that was seen on Lively's swallow study. A follow-up upper GI and pH probe over 24 hours did not suggest abnormal reflux. Because of this, we've been going back and forth about whether he needs to take an acid blocker, whether or not he's a candidate for feeds to his stomach or whether he requires intestinal feeds. Right now, he has a tube going from his nose to the first part of his small intestine.
Here's Lively getting his upper GI series:
Here's Lively with the N-J tube:
I am feeling quite sad that I can't breastfeed Lively right now. One of my main professional (and personal) interests is Breastfeeding Medicine, and it feels wrong to me on so many levels not to be able to breastfeed my baby. Pumping is certainly not the same -- it's much more work, and not nearly as snuggly. There's a chance he may not be able to nurse again. Right now he's not even tolerating the tube feeds well. Ultimately, if he's going to need tube feeds long-term, he may require a gastrostomy tube.
Oh yes, and just when I finally thought we had covered all possible GI issues, a blood test showed that Lively's liver enzymes were fairly significantly elevated. Follow up studies have been negative, so the GI specialists suspect that his transaminitis is somehow related to his acute illness. However, his blood was also noted in the lab to look lipemic (full of lipids), and so this was investigated further, and his triglycerides were found to be very, very elevated. We still don't know why. It may be that Lively is not metabolizing fatty acids appropriately. The specialists are looking into genetic and metabolic causes.
5. RENAL: Due to ongoing electrolyte abnormalities (metabolic acidosis) as well as hypertension, the nephrologists, or kidney specialists, have started following Lively. His kidneys do appear to be functioning normally, thank goodness. They suspect that Lively is acidotic because he is losing so much bicarbonate in his diarrhea. Oops, add diarrhea to the GI list above. Anyway, we arrived at the conclusion that everything is OK with Lively's kidneys only after a brief detour to discussions of hemodialysis due to an extremely (falsely) elevated ammonia level (the very high triglycerides caused a false positive ammonia level).
What else . . . oh, he had a renal ultrasound that was normal.
6. HEMATOLOGY: Due to Lively's history of perinatal stroke and his current DVT (blood clot in his left leg), he is getting Lovenox injections twice daily. Lovenox is low-molecular weight heparin, a blood thinner. Each time we give Lively a shot, he gets a bruise where we inject. You can see that in this photo, especially on his belly.
Lively's platelets are also quite elevated, and have been historically. We don't know why. It may be that the high triglycerides mess up the platelet test. Who knows.
Additionally, Lively will need further thrombophilic evaluation after he has finished this course of Lovenox (in 3-6 months).
7. MUSCULOSKELETAL: After being intubated for 6 days, and sedated for around 8 days, I expected that Lively would be weak when he woke up. He seems much weaker than I had guessed he would be. His neck and trunk are obviously weak. He can't hold his body in a sitting position more than a few seconds, he has trouble lifting his neck and sustaining it when he's on his tummy, and his head sort of flops around like a newborn's. A physical therapist came by to evaluate him a couple days ago, and felt Lively had considerable gross motor skill delay, so that at this time he is functioning as a 4 month old. This is a change from how he was prior to this hospitalization, when he probably functioned at a 6 month level. (Lively will be 10 months old in a few days.) The PT recommended outpatient physical therapy from 3-5 times a week for a month, followed by therapy likely 1-2 times a week, or possibly more. This schedule doesn't include occupational therapy, which he will need to address fine motor issues. Oh, and he'll need speech therapy too.
8. GENETICS: With this very complex medical history and hospital course, there is a concern that there is an underlying genetic disorder that we're not recognizing that is affecting Lively's metabolism, stress response, and possibly even puts him at risk for seizures or stroke. We have quite a few studies pending right now, but it may be 6 weeks or longer until we get the results. It seems like Lively still doesn't fit into a particular picture of a disease process, and actually, genetics seems unimpressed with some of Lively's lab work-up so far. This is good. Most of the metabolic syndromes such as the mitochondrial disorders or the inborn errors of metabolism are not disease processes I would like for Lively to face.
9. SOCIAL: Some days I think I'm at the end of my rope. Some days Pete seems not too far from the end of his. Today was hospital day 21 (remember, we were admitted initially for 23 hours of observation due to dehydration, likely as a result of viral gastroenteritis). We have been told that basically every pediatrician in every subspecialty in the hospital knows about Lively now, and he continues to puzzle all of them.
I have always (half) joked that I want my kids to grow up to be teachers. Many people automatically assume that I want them to be doctors, but I don't. There were too many years of over-the-top stress, sleeplessness, too many holidays missed with my family, too many fun social activities I missed because I either had to work or was too tired. I love my job now -- absolutely love it -- but it took 11 years of training for me to get here. I don't want that for my kids. Pete loves his job too, and he works really, really hard and at times experiences incredible job-related stress. Overall, he is thrilled with what he does each day, and he works from 7 to 5 most days, is home every weekend, every holiday, has a week-long break in February, another in April, and then it's June and he's off for 2 1/2 months. The schedule alone is so compelling to me. So, because I believe that teaching is incredibly valuable, and I want my kids to be happy and have lives that allow them to prioritize family and activities outside of work, I would love to see them become teachers. Lively, at 9 1/2 months, is starting early. With his multiple issues in every organ system, which may or may not be related, he is teaching every doctor that encounters him something new. To my beloved son, I say this: summer is coming. It's time to wind down and take a vacation. No more teaching for now.
Additionally, Lively will need further thrombophilic evaluation after he has finished this course of Lovenox (in 3-6 months).
7. MUSCULOSKELETAL: After being intubated for 6 days, and sedated for around 8 days, I expected that Lively would be weak when he woke up. He seems much weaker than I had guessed he would be. His neck and trunk are obviously weak. He can't hold his body in a sitting position more than a few seconds, he has trouble lifting his neck and sustaining it when he's on his tummy, and his head sort of flops around like a newborn's. A physical therapist came by to evaluate him a couple days ago, and felt Lively had considerable gross motor skill delay, so that at this time he is functioning as a 4 month old. This is a change from how he was prior to this hospitalization, when he probably functioned at a 6 month level. (Lively will be 10 months old in a few days.) The PT recommended outpatient physical therapy from 3-5 times a week for a month, followed by therapy likely 1-2 times a week, or possibly more. This schedule doesn't include occupational therapy, which he will need to address fine motor issues. Oh, and he'll need speech therapy too.
8. GENETICS: With this very complex medical history and hospital course, there is a concern that there is an underlying genetic disorder that we're not recognizing that is affecting Lively's metabolism, stress response, and possibly even puts him at risk for seizures or stroke. We have quite a few studies pending right now, but it may be 6 weeks or longer until we get the results. It seems like Lively still doesn't fit into a particular picture of a disease process, and actually, genetics seems unimpressed with some of Lively's lab work-up so far. This is good. Most of the metabolic syndromes such as the mitochondrial disorders or the inborn errors of metabolism are not disease processes I would like for Lively to face.
9. SOCIAL: Some days I think I'm at the end of my rope. Some days Pete seems not too far from the end of his. Today was hospital day 21 (remember, we were admitted initially for 23 hours of observation due to dehydration, likely as a result of viral gastroenteritis). We have been told that basically every pediatrician in every subspecialty in the hospital knows about Lively now, and he continues to puzzle all of them.
I have always (half) joked that I want my kids to grow up to be teachers. Many people automatically assume that I want them to be doctors, but I don't. There were too many years of over-the-top stress, sleeplessness, too many holidays missed with my family, too many fun social activities I missed because I either had to work or was too tired. I love my job now -- absolutely love it -- but it took 11 years of training for me to get here. I don't want that for my kids. Pete loves his job too, and he works really, really hard and at times experiences incredible job-related stress. Overall, he is thrilled with what he does each day, and he works from 7 to 5 most days, is home every weekend, every holiday, has a week-long break in February, another in April, and then it's June and he's off for 2 1/2 months. The schedule alone is so compelling to me. So, because I believe that teaching is incredibly valuable, and I want my kids to be happy and have lives that allow them to prioritize family and activities outside of work, I would love to see them become teachers. Lively, at 9 1/2 months, is starting early. With his multiple issues in every organ system, which may or may not be related, he is teaching every doctor that encounters him something new. To my beloved son, I say this: summer is coming. It's time to wind down and take a vacation. No more teaching for now.
I am so sorry for all of the mystery and the stress. It does make me happy to see Lively smiling in his martian pajamas, though! I continue to keep you all in my thoughts.
ReplyDeletethanks for keeping us updated. I admire so much that you can do it under the circumstances. I continue to pray for him and your family and I truly hope you will have better days soon. We miss you here. Take care of yourself. Deb
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