Sir Otis: A Better-Late-Than-Never 11-month update

Otis is (way over) 11 months old!

Although it has been a really long time since we’ve posted any updates, so much has happened in the past few months that is worthy of sharing.

Back in late march or early April, we reported to O’s neurologist that he was having some weird spasms that we were pretty sure were NOT seizures, but we couldn’t tell for sure. With the increase in the ratio of his diet up to 4:1 (fat to carb) also came an increase in reflux issues, so we wondered if maybe the new spasms weren’t Sandifer Syndrome, or some other type of GI-related spasms. They were (are) different from his seizures in that the motion is typically backward instead of forward with the GI-spasms, and they tend to occur in very large clusters of anywhere from 50 to 100. The only way tell for sure whether or not these new spasms were seizures was to have Otis undergo a 24-hour video-monitored EEG, so his neuro, Dr. A, went ahead and got that set up for him. In mid-April, we checked into Children’s Mercy South in Overland Park for the EEG. The techs prepped Otis’s scalp and glued 26 (I think) electrodes to his head, then wrapped it up tightly to make sure they all stayed put. We were then instructed to hit a button on a little box each time we noticed an “event” over the course of the 24-hour stay. Also throughout the entire stay, Otis would be monitored by a camera which followed him around the room each time we picked him up and carried him back and forth between the bed and the chair to give him his bottle. He had a whole bunch of wires trailing from his head, which made this trip a little tricky, but we managed.

Otis was having a particularly rough time with seizures in that 24-hour period. Before that day, he had been having 50-75 or so seizures per day, but during that stay, we counted over 150. He really seemed to not be feeling well, and several times during the night, woke up wailing and inconsolable. The next morning when O’s neuro came in to give us the final report, we were given some good news--the spasms we suspected to be GI-related were NOT epileptic seizures, and probably actually were GI-related. We would take Otis to a GI doc in a couple of weeks to follow up on that. We were also given some very bad news. We were told that Otis was having hundreds more “background” seizures per day than we were actually seeing. Over the course of that particular 24-hour period, when we had counted 150 seizures, the neuro and techs had counted well over 300. We were pretty devastated. Otis had already been failed by so many other drugs and treatments leading up to this EEG, and we felt as though we were running out of options. And if his condition was becoming more progressive…we just didn’t know what to think. We felt so helpless.

The neuro assured us that we hadn’t reached our last resort yet—that there were still some options to try to get O’s seizures under control—and told us that he wanted to try another type of steroid on Otis. The ACTH, which was a very powerful steroid, had caused some awful side effects, including ventricular hypertrophy (which has, thank God, gone away). Dr. A said that, out of everything we’d tried so far (Clonazepam, B6, Vigabatrin, Zonegran, Clorazepate, Folinic Acid, Topamax, Ketogenic Diet), the ACTH brought Otis the closest to seizure control. Because of this, he would normally want to try a second round of ACTH, but decided that in Otis’s case, it would just not be worth the risk to his health. So he prescribed a steroid called Prednisolone (not Prednisone, although the spelling is similar). Otis would be started on a large dose and then taper down over the course of the next 6 weeks or so. We were glad to hear that this steroid was a tablet that we could crush and give to Otis with his other medicines, and NOT a daily injection, as the ACTH had been. Before he left the room, Dr. A told us to bring Otis back in two weeks for a repeat EEG so we could see if the Prednisolone was doing any good.

The techs came back in and removed all of the electrodes from O’s head. As we waited for all of the paperwork to be finished up and for Otis to be discharged, we sat in silence, heavy hearted. We wanted to hope that the new steroid would work, but we had watched Otis be failed so many times before that we just couldn’t bear to go through that heartbreak yet another time.

Due to some confusion on the part of our pharmacy, we had to wait a few days before the prescription could be filled. I think we started on a Saturday. During O’s first few days on the steroid, to our surprise, we saw some pretty wonderful things begin to happen. His seizure numbers went down into the 20s, and he became much more alert and active. Still, we were afraid to think that he was actually getting better. I guess we didn’t want to jinx anything. A week and a half later, we found ourselves back at CM for O’s repeat EEG. This was a much shorter one—about an hour once he was all hooked up. It was my turn to stay in the room with Otis and the tech while Ryan waited for us in the waiting room. It was pretty uneventful. Otis had just a few seizures that I noticed, and I dutifully clicked the clicker to report them, as the tech sat in the corner of the room watching the brainwaves and typing up notes. We grabbed some lunch in the hospital cafeteria before heading to CM South for the dreaded follow-up visit with Dr. A. We had both been dreading that visit, afraid of hearing further confirmation that Otis wasn’t getting better. When we got there, we sat in the parking lot for awhile, thinking up questions to ask Dr. A--hard questions that we really didn’t want to know the answers to. We finally found the courage to move out of the car and up to the clinic. Otis was put through all of the usual weight, length, and vitals checks, and then we went to an exam room to talk to the nurse and wait for Dr. A.

When Dr. A (who I swear must be close to 7 feet tall) poked his head in the door, we saw something completely unexpected. He was smiling. BIG. He asked us how Otis had been doing since he saw us last, and we reported the truth—that his seizure numbers were down, and that he seemed to be feeling pretty good. That’s when Dr. A told us that Otis’s EEG had GREATLY improved. That his brain was happier than he’d EVER seen it on any of his previous EEGs. That from what he could see on the EEG and from what we were telling him, it seemed that the Prednisolone was working, that Otis was getting better. We were actually hearing GOOD news. And we had absolutely no idea what to do with it. We just sat there, astonished, both of us completely speechless and in tears. I think we may have startled Dr. A a little bit—it was not really the reaction he’d been expecting. It was just that, after all these months—8 months, to be exact—of hearing nothing but grim news from O’s neurologists, we had both sort of lost hope that we would ever hear anything positive--especially following that 24-hour EEG conversation. Dr. A told us to think of the Prednisolone as a sort of “reset” button for O’s brain, and that if things went as he hoped, it would continue to help heal his brain even as we weaned him off of it. Dr. A told us that although it wouldn’t be safe to keep Otis on that steroid for much longer than 6 weeks at a time, we could do additional rounds of it in the future, if necessary. That was very reassuring, because I know we were thinking “what if”. What if this doesn’t stick? What if the seizures start to come back as we wean him off of this stuff?

We stayed in that exam room for quite awhile after Dr. A left the room, trying to pull ourselves together and wrap our brains around what had just happened.

Now, I want to make sure that I’m not misleading anyone. Otis is by no means cured of his Infantile Spasms. Although he continues to have fewer seizures than ever before, he still does have them, and will probably always have them. But with the numbers down as low as they are, Otis’s brain is freed up to do other things—like develop! Right now we are still working with a GI doc to try to get Otis’s reflux spasm situation figured out, so that stuff has sort of overshadowed the excitement we would probably otherwise be feeling about his seizure numbers being so low. Since the onset of these spasms in March, we have tried replacing his Zantac with Prilosec, then increasing the Prilosec, then introducing a prokinetic called Bethanechol to help his stomach empty a little faster. And so far, none of this has helped—his GI spasms have only gotten worse. It used to be that Otis would be awoken several times per night by seizures, but now that has been replaced by these spasms. Just yesterday, we were able to get an appointment to have him admitted for a 24-hour EGD and pH study (Esophagogastroduodenoscopy, or Gastroscopy) so we can hopefully get to the bottom of what's going on, and do what we can to try to fix it. We already know that the super high fat Keto diet is likely the main culprit here, and we may be faced with the decision of whether or not it’s worth it to keep him on it if it is going to continue to cause such disruption to his poor little GI system. We like to think that the diet is helping to heal his brain and keep his seizures under control, but the only way to know for sure will be to slowly wean him off of it, decreasing the fat-to-carb ratio little by little, to see if his seizure numbers begin to creep back up. Right now, Otis is scheduled to be admitted for the GI procedure on Monday, June 25.

We have also just begun to slowly wean him off of the Topamax, which he has been on since March, and which has never done anything to help control his seizures. The drug has really suppressed his appetite (a common side effect), so our hope is that once he is completely off of it, it won’t be such a struggle for us to get him to take in the calories and fluids he needs. Another side effect of the Topamax which we’re less than thrilled about: it’s taken away his ability to sweat. We can’t wait for him to be done with the stuff!

Otis is continuing to do better developmentally, and has even begun to use his right hand sometimes. He's rolling all over the place, bringing his plug to his mouth with his left hand, holding his head up, helping to bring his spoon to his mouth during snack time, drinking from a sippy cup, using his vision like a champ, and is now cutting a MOLAR (tooth #9)! He is continuing to work super hard on sitting up, getting some weight under his feet while in his ExerSaucer, chewing, putting weight on his hands to help him sit up and prepare for crawling, learning to hold his bottle and toys with both hands, and millions of other fun things.

Here is what April & May looked like for Baby O: