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A quick update:

We got to bring Lively home on Saturday (May 15). We're very, very thankful to be home. Here he is on his way out:

I've felt pretty overwhelmed since though. Pete had to go back to work on Monday. Also on Monday, Lively had a follow up visit with his primary doctor. He's going to go back next week to make sure he's not losing weight. Lively continues to throw up and to have diarrhea, which I believe is thought to be due to his viral illness; basically, it wiped out the lining of his intestines, and it's going to take awhile for it to come back.

Lively is still getting tube feeds. I pump milk for him and pour it in a bag that is attached to a pump. The milk is pumped into him at a rate of 65 ml/hr for 18 hours a day. To convert that to something that may make more sense: that's 40 ounces a day. I'm not keeping up, and I have about 10 days worth of supplemental bottles in the freezer. If Lively doesn't gain weight, or if he doesn't start eating more solids, I'm going to have to give him some formula. I understand that's not a big deal to most people, but it is to me.

Wednesday Lively had his first appointments with Physical and Occupational Therapy. PT is to strengthen and improve his gross motor skills, and OT is for fine motor. She's also going to work on eating and swallowing with him. Both the PT and the OT saw us together, and they both seemed very positive and encouraging. Lively is so much weaker than he was before the (most recent) hospitalization, but he's doing new things each day. Today he did a lot of "airplaning," where he's on his tummy and he lifts his arms and legs off the ground at the same time. I wonder if he will crawl before he turns 1.

Lively still vomits each time we give him his medications, which is around 4 times a day. He gets 17 doses of medicine each day right now. Fortunately, we give most of them through his tube, so he doesn't have to swallow anything. Unfortunately, no matter how slowly we push in the medications, he still throws up almost every time. The medicines also go directly to his intestines, so it seems like most of what he is losing is the acid in his stomach. It's discouraging.

We've also been working on feeding Lively. I did something I never did with Annie, and I bought baby food. We're just too tired to go buy fresh organic vegetables and steam and puree them like we did when Annie started eating (these are the things about which some of my friends with 2 or 3 kids used to tease me when Annie was a baby). Again, I know this isn't a big deal for most people, but to me, it's the last step before feeding him formula. I find both baby food and formula to be disgusting. Anyway, I bought the really expensive organic, all-natural ingredient baby food without preservatives to try to relieve my guilt, and it's going OK so far. I try to pretend it's all applesauce. Lively will only eat a couple of tiny bites each time we try to feed him, and it takes him an hour to do that. But it's a start. I'm happy to see him eating at all.

I go back to work tomorrow, and I don't feel ready at all. I guess it had to happen sometime. Thankfully, my department has graciously allowed me to be off since Lively was admitted to the hospital, which is one of the many reasons that I love U of M and will work there forever. Go Blue!

In other thank yous, please know that Pete and I appreciate so much all of you who have sent gifts, food, notes, emails, made calls, and visited. I have not written any thank you notes other than a few emails, and I may not do much more. I'm just too exhausted, and I hope all of you will understand how grateful we are without receiving a formal thank you note. (My mother is probably displeased with me for this.) I have been so touched by the thoughtfulness of so many people. This has been a terrible time for us. Coming home from the hospital means, I hope, that we're starting to come out of it. But, well, I know there's still a long road ahead of us in Lively's recovery. Thus, the highway continues.

Hospital Day 25, and counting

A brief update:

One of Lively's blood tests was positive for cytomegalovirus immunoglobulin (CMV IgM, negative for IgG), which indicates an acute infection. This could be what is causing the vomiting, diarrhea, and elevated liver enzymes. This is a very common virus to which the majority of adults are immune due to a past exposure; most people never even know they have been exposed because they don't ever develop any symptoms. However, it may also cause gastritis, colitis, and hepatitis, among other things. The infectious disease specialists do not feel that Lively needs to be treated with big-gun antivirals with lots of scary side-effects, so I'm happy about that. Currently though, Lively feels awful. Two days ago he stopped tolerating his nasogastric feeds (meaning he threw up everything we put in his stomach), so his NG tube was switched back to the NJ tube, which involves a procedure done in radiology. Although the tube now bypasses his stomach and feeds his small intestine directly, he is still vomiting his stomach contents fairly regularly.

What breaks our hearts is that Lively just hasn't been himself the last 2 days. We've hardly seen him smile, which is completely unlike him. Here's a photo of him smiling that I took about 3 days ago. He has on new tractor pajamas I got him because he's outgrown almost all of his other pajamas since he's been in the hospital:

Anyway, we haven't seen much of this guy in 2 days. Instead, he wakes up crying, very irritable, and we're having a hard time consoling him. There's obviously something new going on with him. It's as if someone flipped a switch and something changed around 48 hours ago. I'm worried about him.

The GI specialists, like I mentioned, think that CMV unifies many of his symptoms. They told me today that it's not uncommon for children to develop a post-viral gastroparesis after CMV, which means Lively's stomach is not emptying the way it should. They recommended another medication that may help, reglan, a pro-motility agent. Lively started this one tonight, so I really hope it helps him feel better.

Pete and I are just so ready to have our whole family home together again. We're going to have a lot of work to do once that happens (Lively will be hooked up to a pump for his feeds at home, and will be getting, I don't know, a million medications a day, not to mention lots of physical, occupational, and speech therapies) but we just want to be at home. Living in the hospital is tiring us to the bone. It's time to leave.

Now I just keep on moving because I have to. I try to take care of Annie as best as I can, but she's started to have difficulty with how much Pete and I have been away from her, and so she's acting out big-time. This is difficult for me to handle because I'm so emotionally thin right now. It's easier to take care of Lively, because he just needs me to hold him, rock him, give him baths and change occasional diapers. Pete and I are trying to take care of each other too, but we're both basically in survival mode. Each day, I feel like I'm doing pretty well just by getting through the day, and if I eat something healthy, shower, and maybe return a few work-related emails, well, for that I deserve a gold star.

I know very well that I'm not really processing any of this very deeply right now. My baby was sedated on a ventilator in the ICU for 6 days? Really? I went 6 days without holding him? Huh. He's been in the hospital 25 days now, and no one really understands why he's still sick? How could that be? Must be a mistake. Perhaps when I wake up tomorrow, I'll find out that I dreamed the whole thing.

So goodnight. Hopefully my sweet baby is sleeping as well now as he was when I left him at the hospital with Pete a few hours ago.

Detour

For very sick or very complicated patients, doctors often end their progress notes with an assessment and plan by organ system. I think this is the best way to describe Lively's course over the last week:

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.

Here's what you missed last week on Highway Lively

I've been too tired to update in the last 8 days, so here's a recap of the highlights.

Saturday April 24 (Hospital day 5, Ventilator day 2): Lively was extubated and reintubated, as I described already. He was very puffy, mostly due to the Cushingoid effects of the ACTH. Overnight Friday he lost all of his IVs, and with the help of the vascular access team as well as the anesthesiologists (the IV experts), he finally got a tenuous IV in his left foot. The PICU team spent almost the entire day trying to get a line in him. The PICC nurse came and tried both arms, unsuccessfully. The PICU team tried to get femoral lines. They worked really hard on his left leg. No dice. Finally the attending put a central line in his internal jugular vein. Pete stayed for almost the whole time, but I couldn't watch.

Due to his history of a stroke, as well as the fact that we know that he is heterozygous for Factor V Leiden, the hematology team recommended prophylactic Lovenox to try to prevent him from clotting.

Sunday April 25 (Hospital day 6, Ventilator day 3): This was the worst day and night that we had. Lively became extremely edemnatous because of his IV fluids. He was on the ventilator, but struggling against it and very uncomfortable. His legs, especially his left one, were tense with fluids. His heart rate was in the 200s and his blood pressure was very low. His hematocrit was 20. He received a blood transfusion and we expected him to quickly improve. He didn't. He was still acidotic and obviously uncomfortable, despite the fact that he was very sedated. I started to worry that he wasn't going to get better.

Monday April 26 (Hospital day 7, Ventilator day 4): He got better! New ventilator settings made him more comfortable. A new attending doctor came on service and waved a magic wand. Lively got Lasix and peed off some of the fluid that had accumulated in his skin. He got IV hydrocortisone for adrenal insufficiency (basically, he couldn't mount a normal stress response to his illness because his ability to make steroids was suppressed by all of the ACTH he had been getting). He looked better. His numbers were better. I breathed again.

Lively's left leg still looked much more swollen than his right, and as I had suspected, a Doppler study showed a deep vein thrombosis (DVT). The docs increased his Lovenox to the therapeutic range.

Overall, Lively looked like he was able to rest peacefully all day.

My mom and best friend came to take care of Pete, Annie, and me.

Tuesday April 27 (Hospital day 8, Ventilator day 5): Lively's vitals signs remained stable. His lab values improved. The team taking care of him began to talk about extubating him. This made me very nervous because Lively was reintubated immediately after the last attempted extubation, and we were afraid he may not be ready. At he same time, we obviously wanted him off the ventilator.

Wednesday April 28 (Hospital day 9, Ventilator day 6): Lively was extubated! He did really well. The tube came out, and an hour or so later, I was able to hold him for the first time in 6 days!

He stayed sleepy most of the day, but as he sort of woke up, we realized he was having withdrawal symptoms after being heavily sedated for 6 days. He became jittery, shaking his head and arms. He drooled and had explosive diarrhea. His heart rate rose to over 200. He got small doses of morphine, which seemed to improve his symptoms. The team planned to slowly wean the narcotics.

Thursday April 29 (Hospital day 10): Lively remained sleepy, but was more awake than the day before. I tried to breastfeed him and offered him a bottle, but he wasn't interested, so he continued to receive nutrition (pumped by me) through a tube going from his nose into his stomach. He no longer required oxygen, even through his nose.

The team explained to Pete and me that they were going to get a genetics consult. Lively's lactate levels were intermittently elevated, which seemed unusual. On the night Lively was sickest, one of the PICU fellows asked me if Lively came with a manual, because his doctors couldn't understand exactly why he was acting and reacting the way he was. They were concerned about some underlying metabolic disorder that may explain everything more neatly: his labs, his stroke, his seizures. The genetics team visited us and ordered some baseline labs. My feeling of relief after seeing Lively extubated and recovering morphed into free-flow worry about various genetic conditions that may make all the scary news we've gotten so far seem like a cake-walk.

Also, on this day Pete and I realized that the eye-rolling that we had been trying to ignore when it happened yesterday was, in fact, a breakthrough spasm. So the seizure control we thought we had was, well, lost.

Friday April 30 (Hospital day 11): I spent most of this day holding my boy. He slept in my arms for hours. What a relief to cuddle him again.

Seizures continued. He just had a few clusters of eye-rolling.

A lab that had been suggested by the genetics team returned and was abnormal. Lively's pyruvate level was mildly elevated. The geneticist recommended a screening panel for certain metabolic conditions. It will take, perhaps, several weeks before we get the results.

I feel that we've had enough! He had a stroke, he has seizures, his immune system was suppressed by ACTH so a little bug (gastroenteritis) became a big thing (hospitalization), and that had complications (respiratory failure), and other complications (DVT, blood loss anemia requiring transfusion, adrenal crisis) . . . . must we add a horrible, non-treatable genetic condition that might take his life to this mix? Can't we just deal with what has already happened and have hope that he will someday heal? I still hope for a normal life for my son (whatever that means). Will I lose that hope when we get our test results?


Saturday May 1 (Hospital day 12): Spasms continue. The neurologist (not Lively's neurologist, just the ones who happen to be on call) recommended increasing his second anti-seizure medication. I don't think I mentioned before that when Lively was intubated, the neurologists added Keppra to try to prevent him from having breakthrough seizures. Now they are increasing that medication. He continues to take the Topamax.

Lively is even more awake today. He sat up (with lots of help) and smiled at all the nurses who had taken care of him during his ICU stay. He looked completely adorable.

Pete and I spent a long time just holding him today. He is absolutely the sweetest boy in the world.

We will probably be sent to a regular hospital floor tomorrow.