Long day, lots of updates

Pete, Lively and I spent an exhausting day at U of M hospital today. Lively had an EEG this morning followed by an appointment with his neurologist. His EEG showed some improvements -- the hypsarrhymia wasn't apparent any longer, which is an encouraging finding, and the abnormal activity in his brain was decreased overall. However, the EEG still had some abnormalities. This fits with what Pete and I have seen at home: Lively still has seizures, but not as often and not as severe as a few weeks ago. We also learned that according to the EEG, the seizure activity appears to be coming from both sides of his brain, despite the fact that the stroke only occurred in one area.

The plan right now is to continue the ACTH at the current (recently increased) dose for another week. If all goes well, we will likely wean that down over the next 7 or 8 weeks. If the seizures get worse as we wean the medication, we may have to stay on it longer. In the meantime, Lively is still at risk for opportunistic infections due to the immunosuppression. We will also add a second anti-seizure medication as soon as we can find a compounding pharmacy that will make it for us. The new medicine is called Topamax, and we will titrate it to effect as we wean the ACTH.

Here's Pete holding Lively at the neurologist's office today.

At this point, we'd been at the hospital for 4 hours, and had several more to go.

The last few days I've noticed that Lively seems to be clenching his left fist. He can certainly open his left hand, and even prefers to suck his left thumb, but sometimes it looks like he requires the help of his right hand to make his left hand do what he wants it to do. This may be an early sign of some motor problems. He is definitely at risk of cerebral palsy, especially affecting his left hand and arm, and possibly affecting the whole left side of his body. When his immune system recovers, we will start physical and occupational therapy right away to try to minimize the effects of his stroke.

Lively also had some blood drawn today for some labs, and that is what is worrying me right now. He is quite anemic (appears to be a hemolytic anemia, for those of you that know about these things), and I'm not sure what might cause that. Sometimes medications can cause anemia, but I don't know that any that he's taking now would. I'm afraid we're going to have to go back to see the hematologists again.

I realized today how very many hours we will be spending seeing doctors and therapists because of Lively's health problems. Pete and I are on vacation this week and able to go to these appointments, but next week when we both have to work, it's going to become more difficult. Soon we will likely have several PT/OT kinds of appointments every week, as well as follow up with neurology, laboratory draws, consultations with lots of other doctors (ophthalmology, audiology, physical medicine and rehabilitation, likely some specialties I've never heard of) -- I'm not sure how we're going to do all this. We just will, somehow.

I suppose that the news today was mostly positive and encouraging, although I'm feeling a bit overwhelmed and worried and not entirely positive at the moment. I still keep thinking I'll wake up in the morning and this whole thing will have been a nightmare.

Thanks to all of you who have called, written, visited, and emailed. We feel a bit isolated right now since we can't really take Lively anywhere, so we're always thrilled to hear from friends and family.

Seizures continue

Lively's neurologist called us Thursday night for updates, which I really appreciated. At that point, Lively had been taking the ACTH for 10 days. Pete and I are certain that it has made a difference -- Lively's seizures were worsening by the hour for the first day or two we were in the hospital, but after 2 or 3 days on the ACTH, they definitely became less intense and less frequent. However, he still noticeably rolled his eyes up and to the left, which we felt was persistent seizure activity.

The neurologist was encouraged that the ACTH was making a difference, and she told us to increase the dose (to the maximum dose) to see if we can get the seizures to resolve. Today is day 3 of that dose. Although the intensity and number of the seizure continue to decrease, Lively is still having them. Neither Pete nor I noticed any yesterday, so we had hoped that they were gone, but Pete saw Lively rolling his eyes again this morning. We go back to the neurologist on Wednesday for another EEG, and if Lively is still having seizures at that point, or if the hypsarrhythmia is still present, we may have to add another anti-seizure medication. As with all his medications, there is the potential for serious side effects, so we are hoping not to have to do this.


Speaking of side effects, one of the big effects of ACTH is increased appetite. Although we started trying to add solid foods to Lively's diet when he was 6 months old, due to his respiratory illness, the seizure development, and the hospitalization, this obviously wasn't something we pushed. Now I think Lively suspects I'm trying to sneak him more medicine when I offer mashed bananas, so he generally refuses anything other than milk. His appetite is steadily increasing, to the point that he now drinks about 2 liters a day. I can't keep up! Thankfully I have quite a lot stored in the freezer, but I'm worried that we may even run out of that. Each time I offer him a bottle, I try to dissolve as much rice cereal as possible in it first, but even with the extra calories, he's insatiable.


Here's a photo of Lively I took this morning as he was laughing and playing with his toys. Each time I see him reach out to grab something, especially with his left hand, I feel both relieved that he has this ability, and terribly frightened that it might not be there tomorrow.

His hands look blurry because he's moving them so quickly as part of his happy dance!

A Better Day

First of all, I want to post another photo of Lively from the hospital. Pete took this with his phone, and Lively looks so sweet in it. This was taken last Wednesday (one week ago) after his MRI.

Isn't he the cutest thing you've ever seen?

Yesterday was a better day. Lively had started the antibiotics for his ear infection, and a tooth that had been bothering him pushed its way through. So he smiled for us, laughed a lot, did his happy dance. These little things make Pete and me feel like a million bucks.

Since his hospitalization, Lively has seemed quite a bit weaker to me, and I have been worried that he is going backwards in his development. Although he had not been sitting before the seizures started, he held himself up like he wanted to sit. Sometimes when we put him on his tummy, he would push himself up a bit and look around. Since the seizures started, he hasn't pushed up on his arms at all, and he seemed not to be able to maintain a sitting position, even with help. However, yesterday, perhaps because he's feeling better, he held himself up quite well in his bumbo.

You can see in this picture how he holds his head turned to the right, which is due to the muscle weakness in his neck. Now we know that was probably caused by his stroke.

He also reached with both arms for a toy and pulled it to his mouth to chew on, several times. You can't imagine how I hang on these accomplishments.

I took Annie to music class yesterday as well, for the first time in four weeks. Here's my little percussionist!

Add another med to the mix

Today I thought I was ready to do something I hadn't done yet -- I did a literature search on infantile spasms and stroke. I knew I wouldn't like what I found, although of course I was hoping to find something reassuring. I've been telling myself that Lively is disqualified from the Symptomatic IS category because his stroke occurred so long ago, likely at the time of his birth, or shortly after, and he has developed normally until this point. I told myself that any major motor deficits would be obvious already. The fact that he interacts normally with us must be a great sign, right? Well, it turns out, this isn't the case. He's still very much at risk for epilepsy, for motor delays, even for hemiplegia. Here's one way to summarize (West syndrome is a triad of spasms, the EEG finding that Lively has, and mental retardation):

Infants with idiopathic West syndrome have a better prognosis than do infants with symptomatic West syndrome. Only 14% of infants with symptomatic West syndrome have normal or borderline normal cognitive development compared with 28-50% of infants with idiopathic West syndrome. Mental retardation is severe in 70% of patients, often with psychiatric problems such as autistic features or hyperactivity. Infrequently spasms may persist in adulthood. 50-70% of patients develop other seizure types and 18-50% of patients will develop Lennox Gastaut syndrome.

Today I read some of the studies that generated these numbers. I probably won't do that again for awhile.

Meanwhile, Lively is still having seizures. They're subtler than they were when we were in the hospital, but they're still there. We hope that what looks like an improvement to us is truly an improvement. Meanwhile, we continue the ACTH, which is making Lively fat, cranky, and at risk of serious infection. I plan to write in more detail about ACTH soon, but I haven't felt ready to tackle it yet.

Speaking of infection, we were told when Lively was discharged from the hospital to call Lively's pediatrician if he had any signs of a cold. The last few days, Lively has developed a new cough and a runny nose, not something for which I would normally call his doctor, but I called today. The doctor had us come in, and Lively has an ear infection. Again. This is his third in two or three months. So now Lively is taking:

1. Omnicef for his ear infection (has side effects like diarrhea)
2. ACTH for the seizures (billions of side effects, don't want to think about them right now)
3. Zantac because he's at risk of GI bleed due to the ACTH
4. Bactrim for pneumocystis pneumonia (PCP) prophylaxis. PCP is an opportunistic infection commonly found in AIDS patients. That's how suppressed his immune system is right now.

So that's 3 oral meds, and 1 shot. My poor little guy.

Here's a picture of Lively today. I like this one because he's doing his happy dance in it. We don't see that so much these days.

You can really see how chunky he's getting in this one. I still believe that there's no such thing as a too-fat baby!

Today was rough.

Lively woke up smiling and seemed to be in good spirits. Then later in the morning, he had what we now refer to as "big seizures". I didn't notice any more, and felt encouraged, but toward dinner time, Pete pointed out to me a repetitive eye-rolling thing Lively had evidently done many times throughout the day ("little seizures"). I don't know if it's a good sign that he is now having almost all little seizures, but I'm ready for them to stop altogether. I've been ready since I saw the first one 8 days ago.

I took Annie to music class today. Music class is a fun thing Annie and I do together every week -- lots of singing, dancing, banging on drums, rolling balls around, very fun 2-year-old stuff.

She doesn't look happy in this picture, but this is one of her favorite things to do. She asks, "Is it music class day?" at least once a day.

There's a baby in this class who I actually delivered around 7 days before I had Lively, and of course, I couldn't help but notice today that he sits, rolls over, pushes his upper body off the ground and maneuvers himself into a crawling position. Lively doesn't do any of these things. I know it's always dangerous to compare kids, because different kids just do things at different times. But it really struck me how much more this little boy did than Lively does. And it made me really sad. I think that Lively will probably sit and crawl and walk, but I'm not sure that he will. The relief that we felt when we found out that what was causing the seizures was not the most terrible thing in the world is being replaced with nagging worry. 

I have felt lucky to have healthy kids since they were born. Right this minute though, I don't feel lucky.

Part of the reason I'm feeling so discouraged at this moment is that Lively had a terrible day. He is always a happy, smiley guy. He has always been an easy baby. He started sleeping through the night at 10 weeks. He rarely cries, and just has the sweetest disposition. Now that he's getting the ACTH medication, he's very unhappy and uncomfortable all the time. Thankfully, so far he's also been very sleepy. I would love for him to sleep through the entire course of medication and wake up when it's over. When he is awake, he's distressed. He cries in a way that breaks my heart, and there's almost nothing that comforts him. It's terrible. I miss his beautiful smile.

The one thing that sometimes comforts him is nursing, which he does both because he's ravenous and to console himself. He's substantially puffier and heavier than he was even one or two days ago, and his appetite is enormous. I'm working hard to keep up with him, and thankful that I have gallons of milk in the freezer in case I can't. I'm actually setting my alarm for 2 am so that I can get up and pump to try to stimulate my milk supply.

Lively's personality has changed, probably because he feels terrible. I'm trying to have faith that my sweet baby will come back when we've finished this treatment. I don't want this to change him. I'm overwhelmed with worry that his development seems to have come to a screetching halt. Pete says that he probably isn't trying to sit or roll over or lift his head because he feels awful, and I hope that's the reason and that he will catch up after we finish this treatment.

The neurologist told us that we will celebrate one milestone and almost immediately start to worry about the next one. I'm starting to understand what he meant.

Here's hoping for a better day tomorrow.

Paczki

This is a photo of Lively that Pete took tonight.

I was introduced to paczki 6 Fat Tuesdays ago. We don't have them in Kentucky. For my friends not living in an area with a large population of people of Polish heritage, paczki (pronounced poonch-key) are delicious, fattening, sinful jelly or cream-filled doughnuts, only available around Fat Tuesday. Since becoming a Michigander, I have embraced Paczki Day whole-heartedly. Although Pete and I no longer look forward to a special delivery from Hamtramck, MI from our friend Jamie, I have had to look elsewhere -- thank goodness they're in every grocery store, coffee cart, deli, and bakery right now. As you can see, I got the big box.

In this photo, Lively is doing what he has done most of the day, which is to sleep soundly. He's so exhausted from the hospitalization, which involved many uncomfortable tests, and very little sleep. His medication is supposed to make him irritable, which he was when he was awake today. The good news: his seizures seem to have decreased today. I second-guess myself when I say that (do I see fewer spasms because I want to so badly? am I missing them because he's sleeping so much?) but Pete definitely agrees, and he has been the most sensitive in picking them up. The first 22 hours of the hospitalization, Lively was hooked up to a video EEG, and we were supposed to push a button when we noticed seizures so the neurologist could go back and look at those moments both on video and on the EEG. Oftentimes when Pete pushed the button, the neurologist could not see any noticeable seizures on the video, but when they looked on the EEG, Lively was having a seizure. On the day we left, Pete pointed out one of the subtler episodes to the attending neurologist; the doctor remarked, "Most parents wouldn't notice that. In fact, if you hadn't called that to my attention, I wouldn't have noticed that." So I guess if Pete feels sure there are fewer seizures, then it must be true.

Here's one of my favorite hospital photos of Lively, when he was hooked up to the EEG. I love how he's smiling. My mother says he reminds her of my grandfather (his namesake), who was always cheerful and optimistic in the face of adversity.

Home Sweet Home

Lively had his echocardiogram today, and it was normal, which is great news. Sometimes babies have congenital heart defects that make it more likely that they will throw clots up to their brains, causing embolic strokes. This is scary because as long as the heart defect is there, there is a chance for more strokes. Lively's neurologist suspects that the stroke was caused by a hypotensive episode because of the distribution of the damage, and not an embolism, but in cases like this, it's important to make sure his heart is normal and that he doesn't have a blood clotting disorder, which can also cause an embolic stroke. Unfortunately we weren't able to see the hematology team in the hospital today (to run tests for clotting issues), so we'll have to see them in several weeks.

So we got to go home! Annie, who has been having a blast eating valentine cookies at grandma's house, is home too. Here's a picture of her when Pete picked her up this evening.

We're finally all home together, but of course there are a million things to do. Pete has to pick up Lively's medicines at the drug store, Annie needs dinner, bathtime, Lively is eating like a pig due to the side effects of one of the medications, which is taking lots of my energy (quite literally -- he still breastfeeds almost exclusively, so my body has to ramp things up so that he can get enough to eat, which makes me ravenously hungry much of the time). Finally it's time to read a book and put Annie in bed. Halfway through she tells us, "Annie's tummy hurts," and then throws up all over herself, Pete, me, the couch, the floor, her special bunny and blanket (beedah) that she must sleep with in order to be happy . . . let's just say it's now 3 episodes and several loads of laundry later, and Annie has learned a new word (barf), and Pete has made another trip to the drugstore for some pedialyte. Still, we're thankful to be home.

Tomorrow we start all the home care stuff -- giving Lively daily injections of ACTH, monitoring his blood pressure (which is already a bit high due to the medicine). Here's hoping for fewer seizures.

A Stroke of Luck

Infantile spasms are divided into two categories: cryptogenic IS, which means there is no identifiable cause of the seizures on MRI or metabolically, and symptomatic IS, which means there is a known underlying cause, like a tumor, or a metabolic or genetic disease. We had been hoping for cryptogenic IS, because the overall prognosis is better.

Lively had an MRI yesterday, and we found a cause, so he has symptomatic IS. At some point during his short life, he has had a stroke. We don't know when it happened -- it could have been before he was born, or during delivery, or 2 or 3 months ago. Based on the MRI findings, it didn't happen recently. As a consequence, there has been damage to the right side of his brain, most concentrated in the parieto-temporal area. He also has some diffuse atrophy on the right side of his brain. Here are some of the pictures:

On MRI, the left and right are switched, so the right side of his brain is shown on the left. When you compare the two sides, you can see some obvious differences caused by the stroke.

Believe it or not, "Your baby has had a stroke" was the best news we'd heard all day. The fact that this happened quite awhile ago, and Lively has continued to develop normally, is good news. We don't think the damage to the brain will get worse. Despite the significant damage, Lively moves both arms and legs, laughs, smiles, coos, babbles. He doesn't sit yet, and he has some muscle weakness in his neck that we've known about for awhile, which we now realize may have been caused by the stroke. He does have some incontinence, but the doctor feels that is age-appropriate. :)

So we don't know exactly what this means. He might have some permanent motor deficits, which would mean that he has cerebral palsy. He may have some developmental disorders, learning disabilities, ongoing seizures, we just don't know.

This morning Lively is still having seizures, so the medicine he's getting hasn't worked yet. The seizures are getting bigger, and Lively is more upset when they happen. However, we are hopeful this medication will work for him, and that he won't have to be on it very long (lots of scary side effects, I'll get to that later).

We have some more tests today (an echocardiogram, a hematology consult and some blood work), and if everything goes OK, we might get to go home tonight.

This is Lively, insisting that he suck his thumb despite his unfortunate IV placement.

Hospital Day #2

I haven't followed many blogs. I've certainly never written one before. The ones I have read tend to recount hard times in friends' lives -- a wife's cancer course, postpartum depression, a prolonged and difficult adoption process. And now my husband (Pete) and I are facing what very well may be the most excruciating thing we'll ever have to face: our beautiful baby son's illness. I'm writing this for me, but I'm also writing it for our friends and family who live far away and who may want to share this experience with us.

Five days ago, Lively, now 6 1/2 months old, began having seizures. At first he sort of rolled his eyes back in his head, just a few times, but otherwise was perfectly fine. That was Friday night. I didn't think much of it. On Saturday night, he shrugged his shoulders while he rolled his eyes. This happened a several times, but still he was otherwise acting normally. Pete and I decided to call the doctor first thing Monday morning. On Sunday, a sort of flailing arm movement accompanied the eye rolling and shoulder shrugging. By Monday, he flexed his legs too. Pete took him to his doctor, who referred us to see neurology. On Tuesday morning, Lively had an EEG (an electroencephalogram, where tiny electrodes are attached to his head and monitor the electrical activity in his brain). This showed hypsarrhythmia, which is a special pattern that is diagnostic of something called infantile spasms.

Infantile spasms sounds fairly benign, I think. A spasm, a tic, seems like no big deal. Well, that's not the case, and Pete and I knew that right away. We have some friends whose daughter has IS, and so we understood how serious this is, how terrible it can be.

When I read about IS (again) yesterday, after Lively received this diagnosis, four words jumped out at me immediately. The prognosis is poor. That has not sunk in yet. This boy and I just met 6 1/2 months ago, and I cannot imagine a life without him. He is so perfect, so sweet, mellow, joyful -- literally the perfect baby -- and he has a disease that might kill him. He's undergoing a dangerous treatment (the only one with a chance of helping, right now) which could kill him too. And if he does make it through, there's a high chance of developmental delays, mental retardation, intractable seizure disorders, other terrible things. How can this be? I'm holding my perfect baby in my arms, nursing him -- you'd never know anything was wrong except that we're in the hospital and he has about a million wires coming out of his head. Oh, and the seizures.

The neurologist said yesterday, the ax just fell, now we have a long road ahead of us. (Gotta love the metaphors.) So welcome to the road. What should I call it? Lively Lane? A lane doesn't seem long enough. Maybe Highway Lively. I'm open to suggestions.

Lively has an MRI today. The prognosis is best if they can't find anything wrong, so I know what I'm hoping for today.

Please send good thoughts, prayers, meditations, love.