3 months

Otis is 3 months old today! Our little guy has had a pretty crazy past month, as some of you already know. Instead of trying to sum it all up again here, I've pasted text from e-mails we've sent out over the past week. For those of you who don't want to read this entire post, the short of it is that Otis was diagnosed with Infantile Spasms, a type of epileptic seizure, on September 7th. Since that time, we have been working with some great neurologists at Children's Mercy Hospital in Kansas City who are working hard to help him get better.  

9/8/11

i know that this is the first some of you are hearing of all of this, but we wanted to have a better idea of what was going on before telling everyone. it's a lot of info, but i'll try to sum it all up from the beginning.

a few weeks ago, just over a week after his 2 month checkup and immunizations, otis began having seizures--or, "clusters of spasms" is really a more accurate description--several times a day. at around that same time, he began spitting up huge volumes, and until then, it had been very unusual for him to spit up at all. we took a video of an episode and made an appointment with his ped right away. she saw the video and thought the spasms were being caused by gas pain, and the spitting up by reflux, so she prescribed liquid zantac for him to take twice a day to control it. i was wary of her diagnosis of the spasms, because to me, they didn't look to be gas related at all, but i'm no doctor, so i thought we would just give him the zantac and see what happened. the spitting up stopped almost immediately, but the spasms continued. 

we made another appointment with his ped, and went to the appointment armed with more video footage of the spasms AND a closeup of the weird eye movements that were occurring with them. our good friend kate in nyc told us of a friend of hers whose son had had similar symptoms, and who had been diagnosed at first, like otis, with reflux, but when evaluated more closely, was diagnosed with something called infantile spasms--a type of epileptic seizure. she sent me a video of one of that kiddo's spasms, and it was EXACTLY like what otis was doing. i of course then freaked myself out royally by reading about infantile spasms online--lots of scary information out there--and got myself really worked up. (why is it that it's so much easier to find the scary stuff than it is the success stories??? we know they exist!) i was so scared that his ped wouldn't take my fears seriously, but luckily she referred otis to children's mercy hospital in kansas city so that he could be evaluated by specialists. she admitted that she had very little experience with this type of thing, and ryan and i were both very grateful for her honesty.

i should also mention that there had been some other stuff going on--or i guess i should say, NOT going on--with otis while all of this was happening. just before his 2 month checkup, he had begun smiling at us...but very shortly after that, he just kind of stopped. we haven't seen him smile since august 15th, and the only reason we know that is because we caught it on video. he has also had never really responded to our voices. if we approach him and start talking him when his head is turned away from us, he makes no indication that he knows we're even there. his hearing test upon leaving the hospital the first time was good, so we knew that wasn't it. also, he was not tracking faces, toys, or other objects held or moved across his line of vision, although he would focus on high contrast pictures held in front of his face. our ped acknowledged that she was concerned about those obvious developmental delays, and i was scared because from what i had read, developmental delays like that can be a sign of infantile spasms.

of course, the ped's referral to children's mercy happened at the very end of the week, right before the long holiday weekend. it was the first time either of us has ever dreaded a long weekend! tuesday was to be my first day back at work, having exhausted all of my sick and vacation leave and my 12 weeks of FMLA leave. luckily, children's mercy called me first thing tuesday morning, and before long, otis had been set up with an EEG so they could evaluate his brain activity. they were able to get him in yesterday morning (the 7th) for the EEG. we were told that once the test was done, we would meet with one of the outpatient neurologists to discuss the results, diagnosis, and plan of action.

the EEG yesterday went well. after reviewing the results and evaluating otis, Dr. Kayyali (the outpatient neurologist) diagnosed  him with Infantile Spasms and said that he wanted to have otis admitted right away to begin treatment and to run tests for possible causes for the condition. there can be any number of causes, but the best prognosis for treatment usually occurs when they are unable to find an actual cause. this time, we are able to stay in otis's hospital room with him around the clock, which is a huge blessing.

he had an MRI this morning, which we just found out came back CLEAR (IS sometimes occurs when the baby has a brain tumor or other physical brain abnormality)--such a relief. he also had some blood work done this morning, and we found out just a little bit ago that something got messed up with that initial draw, so we have to make him fast again for another 4 hours so they can draw again. he was started on a medication called clonazepam last night for the seizures, along with pyridoxine, which is vitamin B6 (sometimes a B6 deficiency can contribute to stuff like this). both are pills that are dissolved in a little bit of water and given to him. 

if they don't work, we will have to switch him to something called ACTH, which is a steroid that is administered via daily injection. he would start the treatment here, then they would show us how to administer the injections ourselves. after our insurance approved it (and, of course, insurance companies seem to love to drag their heels on stuff like this), the ACTH would then be shipped directly to our home, as pharmacies do not stock it. the inpatient neurology team decided to start him on the clonazepam first because the ACTH is pretty aggressive for a kiddo his age. usually infantile spasms don't show up until around 4-6 months of age. otis isn't even 3 months yet. the ACTH is a steroid, and would come with a number of side effects--weight gain, skin rash, increase in blood pressure--that would have to be monitored pretty closely.

no matter what the treatment, they will monitor his progress every 2 weeks or so. if it seems that they're working and he stops having spasms, they would then slowly taper off his dosage until he doesn't have to be on it anymore. we're told that there are two possible outcomes for kiddos with Infantile Spasms. Either this condition will completely go away and he won't have symptoms ever again, or they'll go away now, and will be replaced later in life (we wouldn't know how much later) with another type of epileptic seizure, which would be less severe and manageable by anti-seizure medication. not perfect, but MUCH better than Infantile Spasms. we're also told that the outcome is very dependent upon the actual cause of the condition, which is what they're trying to find now. the best case scenario is that they're not able to even FIND a cause, since the possible causes are usually pretty catastrophic in themselves. so far, so good with that! we're very hopeful that the clonazepam and B6 will work their magic and get otis's condition under control. we miss our happy baby, and it's just hard to see him suffer knowing that there's nothing the two of us can do to help him. oh, and the head of the inpatient neurology team assures us that if the treatments are effective, otis may very well be able to catch up developmentally....or at least come close. of course, no matter what happens with that, we love him with all our hearts, forever.

that's pretty much all we know now. we at first thought we might be going home tomorrow, but that will depend on his blood work results and how he responds to the clonazepam and B6. i am working with my boss and with HR to apply for shared leave from work. KU has a bank where people can donate any extra sick or vacation leave they have in their own banks. now that i have exhausted my sick and vacation leave, i have to work at least 20 hours a week to keep our health insurance, even if extension of my FMLA is approved. if approved for shared leave, i would be able to take the extra time without having to worry about losing our insurance.

i should also mention that otis has been in such great hands here at children's mercy. all of the doctors we have worked with have been great, and the nursing staff has been nothing short of amazing.

we ask that you please keep otis in your prayers. (my mantra over the past week has been "please heal otis...please heal otis...please heal otis...") we love all of you so much, and are so grateful to have each of you in our lives.

9/9/11

thanks to all of you so much for your prayers and your kind messages (really, they feel like hugs). it's so hard to not feel angry about all this. sometimes all i want to do is stomp my feet or throw myself on the ground in a temper tantrum over how unfair this all is. then i have to remind myself that there are other kids out there who are much sicker than otis, many of whom don't have access to the great doctors and treatment options that otis does...so...trying to just keep it all in perspective. but even still....GRRR!!! despite the occasional crankiness, we're remaining hopeful and prayerful that one of the proposed treatments works for otis so that he can grow up to lead a healthy and normal life.

otis was discharged from children's mercy today. the plan is for him to keep taking the clonazepam and B6 over the weekend and for us to log his seizure activity. his neurologist will check in with us first thing monday morning to see whether or not there has been any progress. by that time, if the clonazepam is working, he should be having few to no seizures. if it seems like it's working, we'll keep him on it and the neurologist will re-evaluate the dosage every week or two. eventually, he would be tapered completely off of it. if it seems like it's NOT working, he'll be readmitted to the hospital on monday so that they can begin treating him with the hormone ACTH right away. he has already been preadmitted for monday so that he will be guaranteed a bed, just in case he needs it. we hope hope hope that he won't!

there was a wonderful social worker at the hospital who helped expedite my FMLA and shared leave applications (all of the pages that required information from his doctor), and it appears that i'll be able to draw enough shared leave for the time being to keep up our health insurance--such a relief!

9/13/11

Unfortunately, the Clonazepam and B6 didn't do the trick, so Otis was readmitted yesterday morning to begin ACTH (adrenocorticotropic hormone therapy). It sounds bad, but we are actually feeling much more upbeat and positive this week, which I know has much to do with all of the love, support, and prayers from our wonderful friends and family. There is a different inpatient neurologist on rotation this week (Dr. Le Pichon), and we really liked that when he came to Otis's room to meet with us for the first time yesterday, he sat down with us and really made us feel like Otis was his only patient. He didn't leave until he knew that all of our questions (for the moment, anyway--new questions are always popping up) had been answered.

He explained all about the ACTH and its possible side effects, which include rapid weight gain, hypertension, increased blood glucose levels....basically, most steroid-related side effects. We believe we'll be going home with Otis tomorrow. After we leave CM, Ryan and I will be giving Otis an injection once a day. While he is on this treatment, we'll take him to his ped twice a week or so so that they can monitor his blood pressure, blood glucose, and weight very closely. The nurses in our unit have been teaching us how to give the injections using saline solution and oranges. Otis was due for his first dose last night, and Ryan gave it to him in his chunky little thigh under our night nurse's guidance. He did such a great job--Otis slept through the whole thing! I'll get my turn today, so please wish me luck. (Still, I'd still so much rather be sticking oranges.)

A 26-day supply of ACTH has been delivered to our house. Ryan's mom was there to make sure it was signed for and put directly in the refrigerator....which we pray doesn't go out on us! We'll give Otis an injection every day for 2 weeks, and then bring him back to CM for a checkup in the outpatient neurology clinic. We think that he'll probably be on it for about 6 weeks, and some of that time may actually be a tapering-off period. We've been assured that any side effects will subside quickly once he's off of the ACTH, so no worry for lasting hypertension or anything like that. We were told that a pharmacist will be coming up to our room to discuss all of that stuff with us in detail, and to answer our millions of questions about it.

Another interesting--and absolutely shocking--fact that we learned about ACTH yesterday:  it costs $22,000 per vial. I still can't quite get my brain wrapped around that! There is an RN in the neurology department who works very closely with the drug company and with insurance companies as soon as a doctor decides to go with that treatment. She coached us a little bit yesterday and told us that we would be getting a call from our insurance company with a quote on what our copay would be. Her next sentence was "And when they give you that quote, DON'T FREAK OUT." So of course we were expecting some kind of astronomical copay. She told us that whatever the number was, the hospital would work with us to provide assistance--sliding scale based on income. Then we got the call from Blue Cross--they first asked for a credit card number, and then told us that our copay for our first supply, 3 vials, would be..........$75. I have no idea how that's even possible, but I'm not going to question it! All we can do is just thank God and pray that it's not a huge mistake that someone's going to catch later! The subject of health insurance in America usually makes me very angry, but for now, I'm just very grateful for the coverage we've got. I have been approved by KU for 6 weeks of FMLA and shared leave, which means that I can take as much time off as I need during that time while still keeping my health insurance AND will continue to receive full-time paychecks. It is such a huge relief to not have that worry hanging over our heads anymore. I am so grateful to the CM social worker assigned to us, and to the wonderful people in my office and the KU HR department for working so quickly and diligently to make that happen for us.

Back to Otis--Dr. Le Pichon told us yesterday that as long as Otis's Infantile Spasms remain cryptogenic (without a known cause), he strongly believes that he would fall into the 10% of cases that are treated successfully with ACTH with no lasting disabilities or developmental problems. The MRI was clean which is a GREAT sign, and we're still waiting on blood work results to tell us if there are any metabolic causes. Otis also had to have a spinal tap this morning (the second one in his short life, since he had that one in the NICU when he was 3 days old to rule out meningitis) so they can rule out any possible genetic causes. Those results could take up to a few weeks to come back, but our hope is that they find absolutely nothing at all. Any possible cause found on any of those tests would be a big game changer as far as Otis's prognosis goes, so we're praying praying praying that everything comes back clean and clear. Dr. Le Pichon says that he hopes we leave here thinking that he's an idiot for not being able to find a cause :)

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TODAY, 9/14:
We're going home today! Otis had his 3rd ACTH injection this morning, each one given to him by either Ryan or me. Believe it or not, it actually has gotten a little easier, and Otis has done so well with them. He actually throws a MUCH bigger fit when we try to get the blood pressure cuff around his arm or stick a thermometer in his armpit! He is still having episodes, but Dr. Le Pichon said that it could be about a week before we notice a significant decrease. 

Thanks again to everyone for all of your love, support, and prayers. We'll continue to keep everyone posted on Otis's progress! For example...we've already begun to see a few hints of smiles over the past few days, and even caught him grinning and laughing in his sleep once this morning! Also, we're been able to get him to make eye contact with us now and then, which is wonderful. Oh, and there's the other stuff, like...

His hair? Still awesome.

 

post-bath mohawk (nothing faux about it!)

His favorite pastime is still napping with Daddy.

 

He's still the sweetest little guy ever.