The Acute Phase: Auto-Immune versus Auto-Inflammatory
The Social Worker at Primary Children’s Hospital told us that writing in a journal can be therapeutic. Even more, perhaps sharing our journey these past few weeks may help other families with children who suffer from this rare, devastating disease.
ICU Day #37 (Saturday, August 5th)
7:00 AM: Not much change with Drew overnight, but we have had a few flickers of hope, which means he has started to open his eyes a little bit again. Continues to have frequent seizures (anywhere from 8 to 15 per hour) with a few periods where his eyes are open, but he is unable to follow commands and he has no real recognition of self or his surroundings. He did have a couple of left elbow twitches this morning, some random, spontaneous, muscle twitches, but not consistent or repeatable.
3:00 PM: The fifth plasmapheresis treatment just finished. They are loading him up with some Benadryl and Tylenol in preparation for starting the IVig because it often causes a little bit of a bump in temperature and rash. The doctors also decided to add a fifth anti-seizure medicine named Topirimate in hopes of breaking his seizures, and they are also re-dosing all of his other anti-seizure medicines right now to replenish them in his system after the plasmapheresis washed them out.
10:30 PM: About 30 minutes ago, Drew had a Grand Mal seizure, which is his first grand mal since the very first week. Ironically, it happened while the ICU doctors were doing evening rounds, so I was standing near the doorway to his room when I saw it start. At first I thought he was reaching his right hand up to pull out his breathing tube, but then I realized it was a big seizure. His head was repeatedly turning to the right side and his right arm was jerking up and down. I hollered at the nurses and doctors, and by the time I ran over to his side, the seizure had stopped, so it lasted less than 20 seconds or so. Initially, I was excited to see some movement, but when I realized it was a Grand Mal seizure, I was worried all over again that things were getting worse, not better.
ICU Day #38 (Sunday, August 6th)
10:30 AM: Drew had another Grand Mal seizure this morning (second in the last 12 hours). The Neurology doctors just finished rounds and told us that despite all the medications and treatments, Drew is still nearly constantly having seizures. They’re going to give him one or two big doses of a sixth seizure medicine that he tried previously called Keppra just to see if it will have an effect. The cannabis oil probably won’t be here until the end of the week. Ketogenic diet probably won’t happen for a while because Drew has developed a pretty significant bed sore on his sacrum, but it’s still on the table once all the doctors feel like his body will handle it. With his constant seizures, his mouth is constantly moving and chewing on the “bite block” in his mouth that protects the breathing tube, and as a result, his bottom lip is starting to bleed. The doctors did agree to try a dose of Ativan to see if they can get his clinical movement to stop, but they told us he is still having the seizures on his EEG. We’re basically in the wait and hope the seizures eventually burn out phase again. It sucks.
2:30 PM: Between church meetings, I called up to the hospital to check on things and they had not been going very well since the Neurology doctors rounded. So I left church early, ran home and grabbed Alex and Ashley, and we headed back up to the hospital to comfort and be together as a family.
When I walked into Drew’s room, his mother sat in a chair next to his bed, gloves on as she held Drew’s hand, and her head was resting on her elbow as she sat and cried. I sat down next to her and put my arm around her, but she barely moved other than her shoulders shaking with her sobs. After several minutes I was able to get her to look at me and I asked, “What is the matter? What did the doctors tell you?”
“The doctor looked me right in the eye and told me there is nothing more he can do for Drew, that is the problem,” she explained as tears streamed down her cheeks.
I pulled her close and tried to comfort her. “Surely there has to be something more. They just need to figure out the anti-seizure cocktail and make sure they have the right combination of medicines, including the right doses and timing of the medications. They’ll keep trying,” I tried to reassure her, but tears were rolling down my face now too. It hurt to watch our son in bed having seizure after seizure, and then to see the hopeless look in Dawn’s eyes — it was almost too much to bear.
Eventually, she was able to calm down enough to leave Drew’s bedside and go for a walk. We found the rest of the children in the cafeteria and sat and talked for a while. Erin had been there for the doctors’ rounds and confirmed it had been a rather pessimistic outlook, but more out of frustration on the doctors’ part because even though they were not sure what more to try, at least they agreed to try the Ativan and Keppra today. At the very least, the doctors asked us to have another Care Conference tomorrow at 11:00 to discuss our next treatment options now that the plasmapheresis and IVig had finished. Although, in the back of my mind I was fearful it was a meeting to weigh some type of hospice, or comfort-care discussion, instead of any other possible treatments because based upon their rounds today, it didn’t sound like there were any other different treatment options to try.
10:00 PM: “Did you see that?” I asked the nurse.
“Not sure — what did you see?” The nurse had been busy working on Drew’s breathing tube.
“When you were trying to adjust Drew’s bite block, he moved his right arm and hand.”
“Are you sure it wasn’t just a seizure?” He has been having lots of seizures, mostly just moving his mouth and eyes, and she had heard in report about the grand mal seizures.
“No,” I shook my head. “I saw his Grand Mal seizure last night, and this was not a seizure. Last night, he was flexing his arm and jerking his head over and over again. Just now, I think he was trying to reach his hand up to his mouth.”
Over the next hour or so, Drew moved his right wrist a couple of times back and forth, as well as his 4th and 5th right fingers, his left wrist once, and his right elbow. Unfortunately, I couldn’t get him to do them on command or repeat them, but I was able to video a little bit of the movements to send to the rest of the family. However, the movements became noticeably less as he fatigued quickly.
ICU Day #39 (Monday, August 7th)
10:00 AM: Lots of seizures. When he has seizures, his eyes roll up and cannot track anything, and he is unable to move or do anything. Morning medications were a little late because of pharmacy. Funny thing is that Drew is bigger than some adults, but the medicines here are usually packaged in doses for children, toddlers, and infants, so they often have to send up multiple vials or doses to get an adult equivalent.
He didn’t have a Grand Mal seizure this morning, but he did have a myoclonic seizure in his right leg and abdomen. It started about 7:30 AM as just a fasiculation, or rhythmic spasm, in his right thigh, but then it gradually expanded to include his whole right leg and then his abdomen by about 9:00 AM. Neurology doctors were just in and plan to do a “routine” EEG and then they will give Drew some Ativan to try and stop the seizure. On a more positive note, he did move his right arm a little bit more about midnight or 1:00 in the morning, but since then he has not moved more. We think he may have become fatigued or the medications may have peaked. Anyway, hoping the seizure medicines can kick in shortly, stop the myoclonic seizure, and maybe we can see him start moving more purposefully again.
1:00 PM: The nurse was finally able to give Drew some Ativan and a bolus of phenobarbital by about 10:45 AM and the myoclonic seizure stopped within 5 minutes. Just now, Grandpa had been sitting with Drew during our Care Conference, and he said Drew was moving his right arm a little bit again — he bent his elbow while he coughed and then straightened it back out. Still not really able to follow commands or move on command.
So we had our fourth Care Conference today just before lunchtime, and we went into it resolutely that no matter what, we wanted them to do all they could to get Drew’s seizures to stop, but we weren’t exactly sure what to expect. Once introductions were made around the little conference room (2 ICU doctors, 3 Neurology doctors, 1 Rheumatology doctor, and 3 social workers, along with our family), the ICU attending welcomed everyone and said, “I believe we wanted to discuss with you where we plan to go next with Drew. Who would like to start?”
The Rheumatology doctor immediately sat up straighter in her chair. “I want to go first this time,” she said. “I told you before that we have more options in mind, and here are two research articles that discuss them. Before we jump into the treatment, thought, we need to talk a little bit first about the thoughts behind the treatment.”
With that the Rheumatology doctor stood and grabbed a dry erase marker and started diagramming an overview of the immune system on the white board.
“Our immune system is basically divided into two parts: the Innate immune cells and the Adaptive immune cells. Since not a lot is known about FIRES, there is a debate among practitioners which part of the immune system is more to blame. All of our treatments up to now have focused on treating the Adaptive immune cells, which are essentially the antibodies and lymphocytes. Auto-immune disorders occur when the Adaptive immune system (antibodies) begin attacking specific tissues and receptors. We feel that the plasmapheresis and IVig were good, but not the complete answer. Drew hasn’t run any fevers for 2 days despite his persistent pneumonia. However, even though those treatments are finished, Drew continues to have seizures.”
“Now we are going to shift our thinking and treat Drew’s FIRES as more of an auto-inflammatory disorder, which focuses on the Innate immune system. Auto-inflammatory disorders are more rare and essentially occur higher up in the immune system’s chain of attack. Where the Adaptive immune system is more specific and precise, the Innate immune system is more general and non-specific. The Auto-inflammatory process may or may not be triggered by a virus or pathogen, but the reaction is an over reaction of white blood cells and their respone is not well-controlled.”
“If you agree, we would like to begin a new treatment today that targets Drew’s FIRES as an auto-inflammatory disorder, and the new medicine is called Anakinra. He will receive the Anakinra for at least 10 days, but we should have an idea if it works within the next 48 to 72 hours. Other FIRES children who have used this medication have seen their seizures drop by 75% to 90%.”
After the gloomy outlook yesterday, we happily agreed to proceed. If the Anakinra doesn’t work, next step would include a form of chemotherapy, but we’re really hoping the Anakinra works. Several FIRES children successfully had their seizures become significantly less frequent with Anakinra and Ketogenic diet, so we’re hoping it will work for Drew as well. We also discussed possibly moving forward with a tracheotomy and PEG (feeding tube surgically inserted through the abdomen). Additionally, we plan to try the Ketogenic diet one more time to see if we can get it to work while he is receiving the Anakinra, and some of his anti-seizure medicines were also adjusted to see if the control could be improved.
4:30 PM: First round of Anakrina injections completed. Four shots in total, and with each shot, he moved his left hand. After the first one, his brother Alex held his hand, and although Drew’s eyes remained closed, he squeezed Alex’s hand with each of the subsequent shots.