May 31, 2016
The thought that Ethan might sleep apnea was first brought up to us by a neurologist in December 2015 and was verified in February 2016 through a sleep study. On Tuesday, May 24th, Ethan finally had his tonsils and adenoids removed. The hope is that their removal will help decrease the sleep apnea which in turn should reduce the seizures, since almost all of Ethan's seizures are breaking through while he is sleeping.
On Tuesday morning we arrived at the hospital at about 5:15 am for the surgery that was scheduled for 8:00 am. We were taken up to the pediatric floor and from there we were taken down to the operating room. The surgery went smoothly and afterwards we were able to see him in recovery. Ethan was in recovery much longer than what is usual simply because there were no beds available for him on the Pediatrics Intensive Care Unit / Pediatrics Intermediate Care floor.
It was after noon before we were taken to a room and things seemed to be going okay for several hours. Jeff's parents came to visit and so did two friends from church. Unfortunately a little after 3pm Ethan woke up seizing. It was just one short 30 second seizure, and the nurse was actually in the room to see it. I told her that I was worried because his seizures tend to cluster, but there was only the one seizure and then he settled back to sleep.
About 20 minutes later he had another seizure and they began to cluster one on top of the other. His oxygen level dropped and he began to turn blue. Alarms were going off and the room filled with nurses and doctors. Ethan's oxygen level dropped into the 40s --- it should be between 98 and 100 at all time. He was struggling to breath and they "bagged" him (see picture) to help force air into him and to help get him breathing again. They also administered medication through his IV that works even quicker than the rectal diastat that we give him here at home to "rescue" him.
I was a complete wreck! Thankfully, Jeff was my rock and he allowed me to fall apart. I was truly afraid that this was it. His oxygen levels were slow to rise, they kept using the bag to force him to breath and then once he was breathing they started giving him oxygen through his nose (see picture). He was so out of it that it took him a few minutes before he realized that it was bothering him. By then his oxygen levels were up and they were able to wean him off and back to room air fairly quickly. I just couldn't believe what was happening to my little boy.
The doctors believe that Ethan was having trouble breathing and therefore had the seizure because of post surgical swelling, which can last up to two weeks! Ethan was given IV steroids before the surgery, but they gave him a dose of a different stronger steroid to reduce the swelling.
After Ethan was off the oxygen and stabilized the room began clearing out. The whole ordeal lasted less than 20 minutes, but it seemed like an eternity. I'm not sure that I've ever felt so scared, except perhaps on the day Ethan was diagnosed with PBD-ZSD. I know that ultimately Ethan, like all of us, will die, but I'm not ready.
The rest of the afternoon and evening were pretty uneventful and Ethan rested comfortably. The youth pastor from our church, who we became friends with shortly after we moved here, came by to visit and pray with us. Eventually, Jeff's parents took him back to our house, so that he could get his car. Later he brought back dinner for me and once Ethan was asleep for the night he headed back home to sleep. The night went by pretty smoothly and I was able to get a little bit of rest. Sleeping at the hospital isn't fun for anyone.
Jeff came back early the next morning, followed later by his parents. One of the doctors came by and shared the good news that he thought that Ethan was doing well enough to go home later in the day. As anyone knows who has waited to be discharged from the hospital, it isn't a quick process. After rounds were completed and after calls to the pharmacy so we could get scripts for additional pain medication and steroids we headed home.
Ethan rested the majority of the day, and I decided to sleep in his room with him, which helped but my mind at ease. I also set an alarm so that I would wake up to give him another dose of pain medication at 4:00am. The ENT (surgeon) warned us, when he spoke to us after the surgery, that it was important to keep on an every 4 to 5 hour pain medication schedule for a couple of days if needed. He also said that days 5-10 after surgery can sometimes be worse than the first couple of days and that "normal" recovery time can be anywhere from a week to two weeks.
Since we know that there is very little about Ethan that is "normal" we aren't going to be surprised if it takes him a lot longer to recovery. Tomorrow will be one week since surgery. He continues to be very lethargic and not his typical happy self. There are moments that we get a little glimmer of his personality, which is nice. We are hoping and praying that once he is fully recovered from the surgery that his sleep apnea will be resolved and therefore he will be less likely to have seizures while he is sleeping.
We are also hoping and praying that then we will be able to work with his Epileptologist to see if we can reduce and/or change any of his anti-seizure medications. While, minus the episode at the hospital, he hasn't had any visible seizures since the latest increase in the Banzel, all of the medications that he is on have really affected his energy level, his stability/mobility, mood, and his overall personality. He's is currently on the highest doses possible for his size of four different anti-seizure medications (Banzel, Topamax, Keppra and Phenobarbital) all of which are taking a toll on our little man and haven't really done a very good job at keeping him seizure free because almost every week he has a really bad seizure episode that requires the use of his rescue medication Diazepam/Diastat. His "sparkle" is simply not there like it was before the seizures came back this fall and we have had to add to and increase his anti-seizure medications. Here's a look at the common side effects of the anti-seizure medications that Ethan takes:
Common side effects of Banzel:
headache, dizziness, fatigue, nausea/vomiting, sleepiness, double vision, trouble with balance
Common side effects of Topamax:
fatigue or drowsiness, difficulty with concentration, confusion, dizziness, unsteadiness, a feeling of pins and needles in the tips of the fingers and toes, nervousness, loss of appetite, depression, difficulty with memory
Common side effects of Keppra:
sleepiness, loss of strength and energy, dizziness, anxiety, nervousness, irritability, depression, other mood changes.
Common side effects of Phenobarbital:
sleepiness/fatigue, depression, trouble paying attention, dizziness, memory problems, upset stomach, anemia, rash, fever, low calcium levels/bone loss.
Common side effects of Diazepam/Diastat:
poor coordination, behavior changes, unsteadiness, sleepiness, fatigue, drowsiness, blurred or double-vision, dizziness.
As you might be able to imagine that when you combine all of these medications together, the side effects can be magnified. We absolutely want to do all that we can to reduce the number of seizures that Ethan has, however we also do not want to continue to have him be so drugged out that his quality of life is impacted. Everyone who knows Ethan has seen how these medications have changed our little boy, and we have to try to do something to get our boy back. We would love to be able to try the medication Epidiolex with Ethan. Epidiolex is a liquid formulation of pure plant-derived Cannabidiol (CBD), but since it has not yet been granted full FDA approval at this time is only available to a limited number of patients participating in clinical trials. So, we are going to be asking our Epileptologist his thoughts on the hemp oil extract known as Charlotte's Web, which contains naturally high levels of non-psychoactive cannabinoids.
Please pray for us as Ethan continues to recovery from surgery and we seek to work with his medical team to try to find treatment options that reduce his seizures with minimal side effects. We really miss the little boy that Ethan was back in September and October. It has been a long fall, winter, and spring battling seizures and sleep apnea. We hope and pray that this summer will be different and that we could get our little boy back.
May 19, 2016
We may hate PBD-ZSD but we love our GFPD family. The Global Foundation for Peroxisomal Disorders has truly become our second family. Check out this short video to meet some of the other families who, like us, have had their lives forever changed because of PBD-ZSD.
As of today, the GFPD only knows of 89 children in the U.S. who are living with PBD-ZSD. It is estimated that approximately 80 children are born with PBD-ZSD each year in the U.S. and approximately half of these children will not survive past their first birthday.
We are passionate about raising awareness and funds for research because we pray that a breakthrough will be discovered in Ethan's lifetime, but even if that doesn't happen we will keep fighting because this cause is so much bigger than just our family. In the next couple of weeks we will be sharing details about how you can get involved in our bowling and silent auction fundraising event that will be in September. Even if you aren't able to attend there will be ways for you to help. More information coming soon.