I know. It's been too long. I've had many people ask how Johnny has been doing and it's made me realize I've been lazy about updating this blog. I'm not sure if there are still followers out there but if so, thanks for sticking with us and thanks for caring!

So how is Johnny doing? Johnny is doing great. He's been doing so well lately it's hard to believe how sick he was 9 months ago. Johnny has transformed from an infant that was so sick his only sustenance came through an IV to a toddler that is so full of energy he can't be contained.  He can be restrained temporarily, but containment for Johnny is a temporary condition.

Johnny is the type of kid that continually looks for trouble. Stairs are irresistable. He wants to climb up or down, doesn't matter. He basically wants to climb up, over, in or through anything he can. The boy is an intrepid explorer. One of his favorite games is opening cabinets or trash cans and pulling everything inside out. He doesn't even bother cleaning up after himself; the nerve of that child! 

Needless to say, Myra and I are pleased with the outcome of the treatment Johnny has been receiving from Cincinnati Children's Hospital and our local pediatrician, Dr. DaSilva. He continues to take the same medication prescribed by Dr. Kumar in January of this year, Trametinib (nib). We dissolve ½ a pill in 5 MLs of water and administer 4 MLs of the solution to Johnny via a syringe orally.

Johnny isn’t allowed to eat 2 hours before or an hour after taking the nib. We typically try and give it to him first thing in the morning. This generally works well but as you might imagine Johnny isn’t always wild about having nib for breakfast when he wakes up. That’s an understatement really, he wants to eat breakfast like everyone else in the morning but he has to wait to eat. Maybe it’s teaching him patience or maybe he just thinks we’re being mean? Anyway, administering the nib is a pain but certainly not something you’ll ever hear me complain about given how effective it is at keeping the symptoms of his disease (LCH) dormant.

One of the most gratifying things to see over the last 6 months is the relationship that’s developed between Johnny and his older sisters. Johnny is now 1 and his older sisters are 7 (Rachel) and 9 (Penny). Penny watches over Johnny like a 2nd mom. Rachel loves Johnny but her relationship is more playful whereas Penny is maternal. Johnny got so sick so early in his life I think it was hard for his sisters to develop a bond. Fortunately we’ve all been given the opportunity to see Johnny grow and mature. Johnny gets lots of love and attention from his older sisters. I doubt Penny and Rachel would trade their little brother for anything in the world (except maybe for a dog).

Johnny and I have settled into a nightly routine. Ever since Myra had a tonsillectomy I have been in charge of getting Johnny to bed. Myra wasn’t feeling great for some time after her surgery so I took over everything I could with Johnny. Myra is now an adult tonsillectomy survivor (seriously, that surgery as an adult is no joke) but the bedtime routine has stuck.

It starts with a bath. He likes to splash around in the water awhile. Then I get him out and the fun begins. Johnny knows it’s almost time for bed but he has no interest in actually going to sleep. After getting out of the bath I dry him off and do my best to get a diaper and pajamas on him. In theory, this sounds easy, but the boy has no interest in cooperating. I’ve found the most effective way to get him into pajamas is to hold him down with my leg on the floor while I use both hands to force the pajamas over his head, and then get his arms and legs in the appropriate holes. All the while he screams, kicks, bites and hits to try and prevent the success of the operation. Next comes the brushing of teeth. Brushing the teeth of infants is not something I remember as necessary but it seems to be the standard today so I am committed to it. As you might imagine, Johnny has no interest in cooperating. The only way to accomplish this is to hold him in my lap, hold his hands and wait till he opens his mouth to cry so I can get the toothbrush in his mouth.

The next step in the bedtime routine is what I affectionately refer to as infant bedtime foreplay. Our bedtime routine has become an elaborate ritual which ultimately leads to Johnny falling asleep. I feed Johnny a bottle before bed each night. In an ideal world I’d hold Johnny in my lap, wrapped up in a blanket, and he’d drink his bottle and gently doze off to sleep. Then I’d carefully lie him down in his crib while doing my best not to wake him. That never happens. The boy doesn’t ever sit still. I give him his bottle and do my best to hold him in my lap while he drinks. That usually doesn’t last more than 30 seconds. Rather than sitting comfortably in a blanket on my lap he prefers to slide out of my lap and crawl or waddle around the room in the dark. He continues drinking the bottle while he crawls around the room occasionally falling down and losing his bottle. Sometimes he’ll slide down from my lap, walk around the chair I’m sitting in then expect me to pick him back up again. Invariably he wants to get back down from my lap after no more than a minute. This process continues for 15-20 minutes. It feels like a weird mating ritual when he is circling the chair I’m sitting in while I am trying to get him to sleep. I don’t remember going to these lengths with my daughters to get them to sleep, but maybe this is just the difference between boys and girls?

So what’s next with Johnny’s treatment? Johnny is scheduled to return to Cincinnati Children’s Hospital in January. Dr. Kumar wants to see Johnny 1-2 times a year to ensure the symptoms of disease remain dormant. Johnny will also undergo a bone marrow biopsy and additional blood tests to ensure the disease remains under control with the current treatment plan.

The big question for Myra and I and Dr. Kumar is when it’s safe to stop giving Johnny nib. One of the most frequent questions I get about Johnny is how long he will have to take the drug he’s on. The answer is we don’t know. Dr. Kumar doesn’t know (at least he didn’t the last time we spoke). For now the expectation is Johnny will need to remain on nib till he is about 5 years old. Once he’s 5 we will consider removing him from the drug to see if the symptoms of LCH return. Dr. Kumar shared with us the experience of another child who was taken off the drug too soon. This child’s LCH symptoms returned so quickly the treatment team was not able to restart the nib in time to prevent the onset of diabetes insipidus. That child will now have to deal with diabetes insipidus for life. Needless to say we don’t want that for Johnny but the nib is not without risk either. 

Nib is not FDA approved for infants. It is a drug normally used to treat melanoma in adults. Some loyal readers of this blog will remember the difficulty we had getting Johnny on nib. In adults, side effects include vision issues, cardiac issues (decreased left ventricular ejection, cardiomyopathy) skin rash and others. At this point the benefit of nib clearly outweigh the risks. However if Johnny doesn’t need the drug we don’t want to give it to him unnecessarily. All we can do is continue to work with Johnny’s doctors and pray that it will be obvious to me and Myra when it’s appropriate for Johnny to stop taking nib. In the meantime we hope and pray the worst possible side effects of the drug do not develop in our son and that the drug continues to “cure” LCH.

In past posts I’ve mentioned my frustration in dealing with health insurance. Overall, I can’t complain about our insurer’s response to the demands of Johnny’s medical care. LCH is a rare disease and the treatment protocols are uncertain and evolving. Despite that, our insurer, Anthem, approved transfer to an out-of-network hospital and they continue to authorize Johnny to receive treatment on an in-network basis in Cincinnati. While I am thankful for our insurance I will say it’s frustrating trying to decipher the medical bills that come as the result of treatment for a serious illness like LCH. I’ve had numerous conversations with my insurer arguing over the classification (or misclassification) of charges being in-network vs. out-of-network. Charges are misclassified fairly regularly most likely because of the non-routine nature of Johnny’s treatment. 

We reached the deductible and out-of-pocket max pretty early on in this year. For the latter half of 2020, after reaching our out-of-pocket max, most of Johnny’s bills were covered. However, as of 10/1 our insurer’s plan year reset. That means a new deductible which we hit in the 1st month thanks to the cost of nib. These are the realities of paying for a chronic illness on a high deductible insurance plan. I’m thankful that we have the means to pay the bills as they come in. This experience has helped me appreciate how hard it can be to pay for medical care in this country. No wonder the number one reason people file for bankruptcy is due to medical expenses.

In past posts I’ve mentioned my desire to share Johnny’s story. My hope is the treatment Johnny received for LCH will eventually become the standard care for kids with the diagnosis. Myra and I have taken any opportunity that comes our way to share our story with others that are dealing with LCH. This past week Cincinnati Children’s Hospital asked us if we would mind sharing Johnny’s story on their website. Myra and I discussed it and agreed it makes sense to share Johnny’s story. We both hope that when Johnny grows up he won’t mind his parents sharing details about his medical history. Hopefully he will understand our goal is to help other parents who are dealing with children sick with LCH and that by sharing his story it may save another sick kid’s life. I interviewed with the hospital staff this past week. The person I spoke with thinks the local media may pick up the story. Once the hospital publishes our story I’ll write another post on the blog so you all can see it.

2020 has been a pretty terrible year. We’ve had LCH, then a pandemic to deal with. Despite all that has gone wrong, I am very thankful for all the help we’ve received. Myra and I have had an incredible amount of support from friends, neighbors, co-workers and family members. I can’t express enough gratitude to thank all those who helped us get through a difficult time. THANK YOU!