It All Started With A Fever, Part 6

The Acute Phase: Into the F.I.R.E.S.

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 #9 (Saturday, July 8th)

5:30 AM: Drew’s seizures continue to spiral out of control over night, and for most of the seizures, the nurses are having to help him breathe. At one point around 3 AM, he has 4 seizures in one hour, and the Ativan and other medicines they are giving him to stop the seizures no longer seem to be helping. The nurses are working frantically and diligently to maintain his oxygen saturation through kind of a pseudo-CPR using a combination of jaw thrusts and a breathing bag. There are three nurses taking turns through the multiple seizures, rotating as their arms and hands fatigue.

When the ICU doctors arrive and see the gravity of the situation, they quickly discuss with the nurses to determine a course of action, after which they nod their heads in agreement. One of residents breaks from the huddle and comes back out of Drew’s room to where I am standing in the hallway. As she approaches me, she says, “It has come to the point that we need to insert a breathing tube into Drew’s throat and lungs so that he can maintain an open airway during these multiple seizures.”

I nod and respond, “Yes, I understand — the nurses have been bagging him most of the night, and the seizures certainly seem to be worsening.”

In order to intubate Drew, the doctors have to sedate him and they hope that the sedation will also help to stop the seizures. I stand out in the hallway and watch nervously as two Respiratory Therapists race into the room with a ventilator and other equipment, and then the 10 to 12 nurses and doctors in the room all converge around Drew’s bed as they quickly work to insert Drew’s breathing tube. Between the breathing tube and the sedation, it seems like Drew’s body stops shaking for the first time all night.

1:30 PM: Our family retreats to the chapel on the third floor of the hospital. We have the little room to ourselves, which seems subdued and calm from the hustle and bustle of the ICU. As light softly pours through the stained glass window along one of the walls, we sit and talk about Drew and his diagnosis of FIRES and the worsening seizures. We cry and hug each other as we let our emotions pour out. Again, there was talk from the ICU doctors about palliative care and preparing for the worst, as children with FIRES generally have poor outcomes (10% to 30% mortality rate).

The possibility that we might lose Drew seems to be creeping ever closer. We talk about Drew fondly and what losing him would mean to our family, and with that uncertain future facing us, we feel hopeless and somewhat despondent. As we talk about the Savior, and temple blessings, and placing our trust in the Lord, we start to feel a sense of reassurance that all will be well in the end. We remind ourselves that Drew is still here with us and we resolve to act as such. Despite some of the grim news from some of the doctors, and their reports of poor outcomes and likely having to let “nature take its course,” we decide we need to focus on the positive side of the statistics and try to stay upbeat and encouraged, especially as we visit with Drew in his room. At the end of the afternoon, we emerge from the chapel feeling a sense of peace and a resolution to keep fighting alongside Drew to overcome this tribulation.

6:00 PM: Because FIRES is such a rare disorder, there is relatively little information on the World Wide Web about the prognosis or outcomes. However, that doesn’t stop Drew’s older sister from poring over the internet for the past 24 hours to learn everything she can about FIRES, and she shares some of the research with us. Many of the research articles reference two landmark studies from 2011 and 2014, and in those studies, they found that older children do better (average ages were 2 to 15 years old). Additionally, they found that 10% to 15% of survivors of the Acute Phase will have a full recovery, while another 18% to 20% will recover with only minimal impairment. Drew was very healthy and strong previously, he had no underlying health conditions, and we rushed Drew to the hospital within two hours of onset of his symptoms, so hopefully all of that will add up to a full recovery for Drew. Only time will tell, but first, he has to get through the Acute phase (the constant seizure phase) of the syndrome.

ICU Day #10 (Sunday, July 9th)

8:00 AM: The nurses spent the night walking a tightrope trying to balance Drew’s sedation, seizures, and blood pressure. As his blood pressure would drop, they would have to lighten his sedation, and then he would have seizures. Finally, about 1:00 AM, the doctors agreed to order a PICC line to improve his IV access and allow more medicines to run simultaneously. When the IV Team finishes the PICC line about 2:15 AM, the nurses then have two additional lines through which they can start some medications to better control his blood pressure in the hopes that the sedation can be a little stronger and improve the seizure suppression. In all, he ended up having 7 seizures last night, and 2 more this morning despite the sedation and other seizure medicines.

10:30 AM: While Alex and I drive home to shower, grab fresh clothes, and attend church, Dawn and Erin and Ashley stay at the hospital and have an in-depth discussion with the Neurology doctors. Erin has written a list of questions on the white board in the room, and the attending doctor patiently discusses each one, with the bulk of the questions simply asking to clarify the treatment plan for Drew going forward.

With the decision to start treating the FIRES last Friday, they started him on a high dose of IV steroids. When the final dose of steroids concludes on Monday (tomorrow), then they can begin a round of IV Immunoglobulin therapy over 3 days, as well as starting Drew on a Ketogenic diet. They also have him on 3 seizure medicines, to which they plan to add a fourth and continue to monitor their effectiveness. Finally, they plan to keep Drew in a medically induced coma for 2 to 3 days with the hopes of achieving “burst suppression,” or eliminating all seizure activity and allowing his brain to rest while all the medications catch up.

If nothing else gained from the long meeting with the Neurology doctors, we feel like there may be some hope after all. The Neurology doctors assure us that they plan to aggressively fight Drew’s seizures, and if these first few treatments fail, there are several others that they can use.

9:30 PM: Doctors still not happy with his “burst suppression” because he is still having seizures despite the sedation. The doctors make more adjustments to try and fully suppress the seizure activity and get his brain to rest.

ICU Day #11 (Monday, July 10th)

8:30 AM: He had 8 seizures overnight despite the sedation with Propofol and Versed, so about 6:00 AM this morning, the doctors decided to switch his sedation medication to Pentobarbital with hopes of achieving true burst suppression (no seizures) for at least 48 hours. The Pentobarbital is a much stronger medication for sedation, but it also carries greater risk for damage to Drew’s heart, kidneys, and lungs, so they do not want to keep him on the Pentobarbital for more than a few days. Given the persistent seizure activity overnight, and the lack of full burst suppression, the doctors decide to keep him sedated until Wednesday morning. They want a full 48 hours on the Pentobarbital and then bring him out of the coma to see if his seizures have stopped.

8:30 PM: No seizures since switching to the heavy sedation under Pentobarbital this morning.

ICU Day #12 (Tuesday, July 11th)

8:30 AM: Drew had a quiet night with no seizures. More IV Immunoglobulin and more seizure medications today, including a new one called Onfi (Carboxapem). Hopefully no seizures when they bring him out of the coma tomorrow.

9:00 PM: Drew’s mother and I nervously pace the ICU hallway outside his room. We are hoping and praying so very hard that tomorrow will be full of good news and the seizures will have stopped. No word on when they will start lifting the sedation, but Drew has gone for about 38 hours without a seizure now. The doctors did warn us that if he starts having “a lot” of seizures again, they will have to put him back under the sedation for an additional 2 to 3 days as they continue to load him with the IV immunoglobulin and the several seizure medications. When we ask the Neurology doctors to clarify what they mean by “a lot,” they have difficulty specifying the exact threshold that would make them return Drew to the coma. They do finally say that ideally, Drew will have no more than 1 seizure per hour, and even 2 per hour would be considered a success.

In a way, it is like Christmas in July as we anxiously hope and pray to get Drew back. We know the medicines require time to work, but we are so hopeful that he will be past the seizure stage tomorrow and on his way to recovery.

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