It All Started With A Fever, Part 15

The Acute Phase: The Darkness Before the Dawn

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 syndrome (F.I.R.E.S. = Febrile Infection-Related Epileptic Syndrome).

ICU Day #40 (Tuesday, August 8th) — A Decent Day

4:30 AM: The nurse just finished giving Drew his second round of Anakinra shots (4 shots each time). I held his hand because the shots seem to be pretty painful and sting even though they are only subcutaneous injections. The first shot, Drew didn’t really flinch, because I think he was sleeping, but then he woke up and the last 3 shots he was moving his left arm and hand quite a bit, reacting and trying to withdraw from the pain. Respiratory Therapy had to come re-tape his breathing tube again a couple of hours ago, and it looks better now. It is strange to think that this 12-inch, plastic tube dips all the way down into Drew’s lungs to help him breath, and the primary anchor is simply a couple of long pieces of tape along Drew’s upper lip and  cheeks. It is no wonder they need to re-secure the tape every few days.

10:00 AM: ICU and Neurology doctors both finished rounding, and they agreed starting today to try the Ketogenic diet one more time. The Ketogenic diet is high fat, low carb and low protein, but for reasons unknown, about 1/3 of kids on this diet see a 50% reduction in their number of seizures. For Drew, he will be on a ratio of 4.5 fats for every 1 carbohydrate and protein combined (it is all liquid in his feeding tube anyway). However, because he has developed a pressure sore on his sacrum (the bottom of his back), they are only going to try for 7 days and if the diet doesn’t kick in and help, they will switch him back to a regular diet to get more protein and heal his pressure sore more quickly. At this point, the concern to stop the seizures outweighs the concern over the pressure sore, and they will try to keep Drew on his sides more than his back to keep pressure off his wound.

One other development is that it looks like he got a little too much phenobarbital yesterday, so Drew may be really sleepy most of the day today. His phenobarbital level this morning was 93, which is pretty high. They were hoping to get him between 60 and 70 the next couple of days while the Anakinra shots had a chance to take effect, but they overshot a little and will have to hold his phenobarbital dose for today. His blood pressure and breathing and labs are all good this morning. In fact, he just looks like he is resting peacefully for the first time since coming out of the last Pentobarbital coma. The Respiratory Therapists are even giving him a break from the bite block to let his lips get a rest. The Neurology doctors also said as they were looking at his EEG from yesterday morning, they were hesitantly optimistic and the EEG seems to look pretty good today so far, but it could be the phenobarbital overdose. To their credit, the Neurology doctors seem to be a little more positive in their assessments and recommendations.

3:30 PM: Occupational therapy worked with Drew this afternoon, and because of his pressure sore on his sacrum, she wasn’t able to get him up to the cardiac chair again, but she did stretch both of his arms. Interestingly, when the therapist was stretching his right arm above his head, he actively tried to push it down she said. And then later today he was feeling the breathing tube with his tongue then reached up his right hand to his chest. It is nice to see that he is starting to move his arms and hands a tiny bit more today.

ICU Day #41 (Wednesday, August 9th) — A Not-so-decent Day

8:00 AM: Drew seems about the same this morning. I helped the nurses roll him and reposition him at 2:00 AM and 7:30 AM, and I got a good look at the wound on his sacrum. I can see why they are worried because there is one spot that the Wound Nurses said may go all the way to his bone. Each time we rolled Drew and changed his position in bed, the nurses carefully cleaned the wound and applied new bandages. He also has small pressure sores starting to develop at the base of his left toe and along the crest of his left shoulder blade, so they put bandages on those spots as well.

He has been moving his arms and hands a little bit more today, but not on command and he still can’t really follow any directions. I also woke up and helped comfort Drew while the nurses gave him his Anakinra shots at 4:00 AM. Reading the numbers again on that study about the Anakinra and they are a little misleading about the response rate, but the Rheumatology doctor said that given the significant seizure activity Drew has been having, they thought it would take at least 3 to 5 days on the Anakinra to hopefully start seeing some improvement.

4:00 PM: After a lot of thought, research, discussion, and prayer, we have agreed to proceed with tracheostomy surgery for Drew. He will have been intubated 5 weeks tomorrow, and it looks like he will need ventilator support for at least another 2 weeks or more. For pediatric patients, they really don’t like going beyond a month with the breathing tube in their mouth and they try to switch them to a tracheostomy so the breathing tube comes out of the base of their throat. As we watch him have seizure after seizure, he continues to chew and gnaw on the breathing tube and is causing sores on his lips. We are also worried about the impact on his teeth, tongue, and vocal cords as the plastic breathing tube rubs and irritates and starts to cause inflammation and damage. After reading up on it and discussing it, Dawn and I agreed that it will be best to have the surgery and switch him to the tracheostomy as he continues to battle these seizures. However, Drew’s brother and sisters are not entirely convinced yet and are a little worried about seeing their brother with a tube sticking out of his throat.

6:00 PM:This afternoon, he really seems to be having a ton more seizures, most likely as his Phenobarbital levels come back down into a safer range. The nurses gave him Ativan twice this afternoon to try and break up some myoclonic-type seizures, and they seemed to work temporarily, but only after a delayed response (one shot took 45 minutes before it seemed his seizures slowed down). His EEG has remained about the same since Monday, with usually 10 to 12 bursts per screen interspersed between frequent seizures every 5 to 10 minutes. He hasn’t been able to track with his eyes much at all today. On a positive note, they should have the CBD oil here tomorrow to start with Drew. We are just barely 49 hours into the Anakinra, but we’re sure hoping it will have an effect soon, because so far it doesn’t seem to be making a dent in the seizures.

7:00 PM: Drew 2, Nurses 0. He just peed all over his nurse twice. At first, he was peeing past the Foley catheter — weird because he still had the catheter, but he was peeing around it. So she took the catheter out because the doctors wanted to discontinue it anyway, and a couple of minutes later, we were surprised when Niagara Falls broke loose all over the poor nurse’s scrubs. In seriousness, this is the first time his bladder has been able to relax enough to urinate since he first arrived at the hospital over 5 weeks ago, so we’re hoping this is a really good sign that his body is slowly returning to normal.

10:00 PM: So even though his EEG is still a mess, and he has had a lot more seizure activity today, Drew has been reacting and moving the most tonight than he has all day. Just now as we turned him onto his right side, we tried to cross his left leg over, and he slowly pulled it back straight — his leg!! He also moved his right arm twice to cover his abdomen as his nurse was trying to give him his Lovenox shot (for blood clots). He seemed to scowl at the nurse when she washed his face. The nurse also noticed he has been flaring his nostrils and seems to be moving his eyebrows more. Hoping and praying for more tomorrow, but a few more little victories tonight after a long, hard day full of seizures.

11:00 PM: I just finished putting lotion on Drew’s hands and feet, and talking to him the whole time. He had one big seizure for sure, but the rest of the time he seemed very calm and quiet, and I swear a couple of times he tried to squeeze my fingers when I asked him. I told him good night and gave him a kiss on his forehead.

ICU Day #42 (Thursday, August 10th) — A Bad Day

8:30 AM: Drew’s EEG looks pretty bad this morning. As I look at him in bed, he seems to be sleeping and all the seizure activity would seem to be sub-clinical and not readily apparent. However, the Neurology team has a doctor who remotely monitors all of the EEG patterns around the clock, and Drew’s EEG was bad enough this morning that the Neurology doctor telephoned in to the nurses and ordered a 4 mg dose of Ativan this morning to try and quiet his EEG activity down.

1:30 PM: Drew loves Music Therapy. Today was the third day that the therapist came to his bedside with her guitar and she gently sang to him. You can tell he enjoys it because his heart rate and breathing will slow down, and during the session today, it seemed to be the first time he has rested all day with all of his seizures.

3:30 PM: “This is so stupid,” Drew’s brother says as he reads through the research article on the Anakinra. “Why is it not working on Drew?”

“I think it is working, but we need to give it more time,” I try to calm him down.

“It’s been three days now, so why is it not working?”

“You have to remember how bad his seizures have been and they said it will take a little longer than the girl in the case study,” I answered. “She was only having about 6 to 8 seizures per DAY, while Drew has been having at least 6 to 8 seizures per HOUR.”

“Well, it’s stupid that it is taking so long,” his brother answered. “The Sage 547 — it’s supposed to work on 77% of patients, but it didn’t on Drew. Ketogenic diet — supposed to help decrease 50% of the seizures, but not on Drew. All this plasmapheresis and medicines — nothing is working on Drew. Why is nothing working? Why is he not getting better?”

“He is getting better, but they just need more time,” I tried to reassure him. “We all want Drew to get better, and he will. You need to hang in there just as much as Drew.”

He just shook his head and threw the research article back down on the bedside table. “I don’t think I want to go to the Family Conference this afternoon,” Drew’s brother said. “I’m not in the mood.”

I turned and looked him in the eye, “Of anyone in the family right now, you need the Family Conference meeting the most, and you need to be there.”

5:30 PM: We just finished the Family Conference with the Rainbow Kids Team at Primary Children’s Hospital. They help families to cope with illness and prolonged hospitalizations, and the last several days the team of doctors, social workers, nurses, and chaplains have been visiting our family and helping us. They suggested the conference prior to Drew’s tracheostomy surgery, and I quickly agreed as the family certainly needed to talk through some of our feelings and worries.

For most of the conference, Drew’s brother and sisters sat silently contemplating the comments and feelings being expressed mostly by Dawn and myself. I tried to be quiet and give them a chance to answer as much as I could, but I couldn’t help it with what was probably the hardest question.

“What is the one thing you fear most about Drew’s illness?” One of the Rainbow Team members asked.

Silence. I sat there patiently waiting for someone to speak until I could not take it any more, so I answered, “I hope it is OK, but I think as we have been talking as a family that the biggest fear we have is that Drew will never come back to us… that he will never get better and never be the same again….”

The Rainbow Team looked at each of the family and asked, “Is this how you feel also?”

The kids each nodded yes, but Dawn added with tears in her eyes, “One of the most frustrating thing about all of this is that nobody can tell us if he will get better or not. Nobody knows how much he will recover. It’s just a big guessing game, and it’s frustrating.”

“That can be a hard thing — the unknown,” the Rainbow Team Fellow said. Silence settled again around the table as everyone reflected on this hard thing, but I felt instead that we did know some of the possible outcomes.

“I think we do have a little better idea now than we did six weeks ago,” I said. “At the very least, Drew comes home to us just like he is now. He might always have seizures and never be able to walk or talk again, but we would still love him and care for him and help him to be as happy as possible. However, I am not yet ready in the slightest to give up hope that these new medicines and treatments will have an impact on his seizures. I believe he will make some more improvement, and even if he is not exactly the same as before, at least we will still have him with us.”

8:30 PM: I asked Alex to help me anoint Drew and give him a blessing ahead of his tracheostomy surgery that is scheduled for tomorrow at Noon.

11:00 PM: Despite my enthusiastic speech in the Family Conference this afternoon, a feeling of dread has enveloped me. All of the frustrations and fears have descended upon me like a lead weight, and I am having difficulty as I sit and watch his EEG screen tonight. Drew seems to have been a lot worse today, having nearly constant seizures, despite all the new medications. Just tonight, he seems to have had a seizure that lasted for 90 minutes or more, both on his EEG as well as with his eyes and mouth. The ICU doctors rounded just now and agreed to try a shot of Ativan even though they aren’t sure it will help. CBD oil started today and now 72 hours into the Anakinra, but still nothing helping. He is scheduled to have surgery tomorrow at Noon for placement of a tracheostomy, and just in time as he started getting another sore on his lip today. As I lay there on the recliner next to his bed, I listen to the hum of the ventilator, the clicking of his feeding pump, and the many sounds from his SCD pump, the air mattress motor, and the IV machines. But one thing I don’t hear is any sound from Drew, who continues to have seizure after seizure after seizure. I hope something helps soon, but I feel my hope slowly slipping away.

Drew loves movies, and some of his favorites are the Lord of the Rings movies. My favorite of all the great quotes from those movies happens near the end of second movie, The Two Towers, as the two hobbits, Frodo and Sam, despair after narrowly escaping capture by Sauron’s minions. Frodo has been tasked with traveling to Mordor to destroy the One Ring, and Sam is his best friend and trusty companion who volunteered to help him. However, in light of the seemingly impossible quest still stretching out before them, Frodo begins to lose hope…

Frodo: I can’t do this, Sam.
Sam: I know. It’s all wrong. By rights we shouldn’t even be here. But we are. It’s like in the great stories, Mr. Frodo. The ones that really mattered. Full of darkness and danger, they were. And sometimes you didn’t want to know the end. Because how could the end be happy? How could the world go back to the way it was when so much bad had happened? But in the end, it’s only a passing thing, this shadow. Even darkness must pass. A new day will come. And when the sun shines it will shine out the clearer. Those were the stories that stayed with you. That meant something, even if you were too small to understand why. But I think, Mr. Frodo, I do understand. I know now. Folk in those stories had lots of chances of turning back, only they didn’t. They kept going. Because they were holding on to something.
Frodo: What are we holding onto, Sam?
Sam: That there’s some good in this world, Mr. Frodo… and it’s worth fighting for.

I suppose that at this very moment, I feel just like Frodo. After all of the dissapointments and hopes and emotions the last few weeks, it seems like we will never get out from under the FIRES shadow. How can our family ever go back to the way it was when so much bad has happened? And do I want to know the end, because what if it isn’t happy? I don’t really think we can turn back with Drew, but it just seems like this darkness will stretch on forever.

© Copyright 2017 Jeff, All rights Reserved. Written For: Jeffrey Olsen