It All Started With A Fever, Part 8

The Acute Phase: The Pavilion

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.

During the frigid, Missouri winter of 1839, after spending several months imprisoned in Liberty Jail, the prophet Joseph Smith cried out in anguish, “O God, where art thou? And where is the pavilion that covereth thy hiding place?” It seems that despite our pleas, and all the prayers and fasting on behalf of Drew, perhaps God has hidden himself far from us the last several days. How long will God allow Drew to be sick?

ICU Day #17 (Sunday, July 16th)

1:00 AM: The hospital can feel like such a cold, lonely place in the middle of the night. I think they purposely program the thermostat to be colder at night to conserve energy, but the night staff seem to just expect and accept the icy temperatures as most of them wear jackets and sweatshirts. Sometimes I take a break and walk the hospital corridors and lobbies in the wee hours of the morning, and I am absolutely certain that I am not alone, even though I cannot see anyone beside me.

We are more than halfway through the slow removal of the deep sedation, and already we are starting to see increased brain waves. However, we just had a major scare as they rolled Drew onto his side to clean his back, his oxygen saturation dropped to near 50%. Respiratory therapy and the nurse both tried to get it back up, but it wouldn’t climb above 70% (normal is over 90%). They quickly rolled him onto his back and started manually bagging and suctioning his lungs repeatedly, but his oxygen wouldn’t come up. Two of the doctors and the charge nurse were summoned and they all worked together for several minutes until finally his oxygen slowly climbed back to 90%. A stat chest X-ray revealed that his left lung was not fully inflated and both lungs were really full of fluid and secretions. Respiratory Therapy readjusted his breathing tube and the doctors ordered breathing treatments with Albuterol to see if they could improve his air flow. Although things turned out OK, the doctors and staff were pretty intense and concerned for several minutes as they worked to keep Drew breathing.

10:00 AM: While Erin and I attend church at home, Dawn, Alex, and Ashley stay with Drew and plan to attend the Sacrament meeting held at the hospital. Just before they leave for the church service, the Neurology doctors round on Drew. The deep sedation is turned off now, but it will again take several hours for the Pentobarbital to metabolize out of his system. Despite his several seizures already, they try to explain that his brain wave activity on the EEG is showing some improvement with fewer “superimposed seizures” on top of the scrambled background activity, and part of this may also be attributable to his emergence from the deep sedation. Plan is to increase the ratio on his Ketogenic diet and addition of another seizure medicine (Zonisamide) this afternoon.

2:00 PM: The more the Pentobarbital dissipates from his system, the worse the seizures are getting. Fortunately, almost all of the seizures are just focal and not Grand Mal. Still, the longer his seizures keep going, the higher his blood pressure and fever creep. The ICU doctors call the neurology doctors and they don’t want to give him Propofol or any other sedative yet. All of the sedation has definitely started to take a toll on Drew’s body, and the Neurology doctors strongly feel that they want Drew to ride the seizures out for at least 6 to 8 hours after the Pentobarbital stopped and just see if the seizures persist or not.

11:00 PM: Drew’s limp body is pale and clammy. He still has not regained consciousness and his pillow and bedding are damp with his sweat from numerous seizures all afternoon and evening. Lots of people in his room right now checking his labs, running an EKG for the study drug (he is participating in a double-blind study for Sage 547, but we think he got the placebo), getting ready to start him on some Propofol for a few hours. He has had lots of seizures and his blood pressure has been slowly rising all day. They’re checking his electrolytes to see if correction is needed with all the fluid they’ve been diuresing off today, and then the ICU doctors just talked to Neurology and they agreed to temporarily place Drew back on a Propofol and Ketamine drip to give his body a rest until morning. Once the sedation enters, his body seems to relax for the first time all day. The ICU doctors say they will discuss more in the AM with Neurology, but it seems to our family like the two groups of doctors are not entirely on the same page.

ICU Day #18 (Monday, July 17th)

8:00 AM: Drew looks good this morning, but he is back under the sedation again. All night, the nurses returned to the balancing act between his blood pressure, sedation, and seizures. The ICU doctor said we’re playing it “hour by hour.”

10:30 AM: The Neurology doctors plan to continue making adjustments to Drew’s seizure medications, but the ICU doctors are talking about taking Drew to Radiology for another type of scan to pinpoint where the seizures are occurring in his brain. The Neurology doctors are not convinced we need this scan unless we plan to do surgery, which really upset the family that surgical intervention was even being discussed. The Neurology doctors assure us that there are still many other options to try before we get to a surgical option. Again, the doctors don’t seem to be on the same page. Either way, the Neurology doctors add a Ketamine drip with the Propofol to try and improve the seizure control, because Drew is still having seizures with just the Propofol by itself.

9:30 PM: Chest X-ray from tonight is improved over the one yesterday, but still has some phlegm and a little fluid. Left lung looks much better and is more fully inflated. With the EEG screen in Drew’s room, we have been able to watch his brain waves ourselves. In a way it is reassuring to see how things are progressing with his seizures, but it also makes us more acutely aware when the seizures are occurring. Interestingly, the doctors started increasing Drew’s Ketamine drip this evening to try and bring the seizures more under control, and since that time, I noticed that his seizures are no longer constant but have developed a rhythmic pattern with a little crescendo before each seizure. I started tracking the frequency to see if the seizures are improving or worsening, and right now they are occurring every 8 minutes and lasting about 30 to 50 seconds each. We’ll see which way they trend.

Elder Henry B. Eyring said, “God is never hidden, yet sometimes we are covered by a pavilion of [our own] motivations that draw us away from God and make Him seem distant and inaccessible. Our own desires, rather than a feeling of “Thy will be done,” create the feeling of a pavilion blocking God.” In every priesthood blessing given to Drew, and every prayer uttered by our family, we always ask “thy will be done,” but perhaps in our hearts we aren’t fully committed? We want our son/brother back so desperately, but Drew is asleep right now, so I guess it is up to the rest of us to put our full faith and trust in the Lord and His will, but it sure is easier said than done.

ICU Day #19 (Tuesday, July 18th)

8:30 AM: Well, the dam burst last night. Drew had not had a bowel movement in nearly 10 days, so they have been pumping him full of laxatives and medicines to help get things moving again. Finally, he had multiple bowel movements last night from 11:00 until about 5:00 or so this morning. So we were pretty busy with that much of the night. Also, last night his seizures were occurring every 7 to 8 minutes, but since about 6:30 this morning, they have been occurring about every 4 minutes — these are just the little, sub-clinical seizures. Unfortunately, the cultures came back positive for C-Diff (Clostridium Difficile), which is an infection in his intestines and a reason for his diarrhea last night. The doctors say this is common when a patient has been in the ICU and receiving antibiotics for a prolonged period of time, so they need to add another antibiotic to treat this new infection.

I have been talking this morning through Facebook with the Gibson family from Colorado, whose daughter had FIRES last year, and they have been sharing some of the many milestones and concerns that are too numerous to mention in a blog. Their words of advice have been comforting, but the toughest comment they shared was that we are truly engaged in a marathon with FIRES, and not a sprint. Today is the last day of the double-blind portion of the Sage 547 study, and then regardless of whether he had the placebo or not, we will likely start re-treatment with a higher dose of the Sage 547 starting tomorrow night or Thursday morning. We are praying he had the placebo at this point, because the study drug did not seem to have much of an effect otherwise.

8:30 PM: The ICU doctor again came to us this afternoon to ask for consent for a SPECT scan for Drew’s brain to see where the seizures are occurring. The whole discussion is upsetting to the family because this really wouldn’t impact his current treatments, but would be preparatory for possible surgical intervention, which we are nowhere prepared to discuss for Drew yet. We ask to leave it for discussion for our first Care Conference, which is scheduled for 11:00 tomorrow morning and will allow us to meet with all the doctors together, ask questions, and agree on a solitary treatment plan going forward.

Elder Eyring continued, “We can’t insist on our timetable when the Lord has His own…. Sometimes our insistence on acting according to our own timetable can obscure His will for us.” I firmly believe the Lord knows what is best for us, and we must rely on His wisdom, but His timetable is even harder to accept sometimes. We want blessings now. We want a miracle for Drew now. How much more does the Lord want us to learn from this Groundhog-Day of a nightmare we have been repeating  for nearly 3 weeks now?

ICU Day #20 (Wednesday, July 19th)

9:00 AM: Drew finally came off all of the sedation medicines last night about 8:00 PM, and now he just has the several seizure medications on board, but he is still not conscious yet. Continued seizures most of the night, but there were some periods of calm at times, usually for about 30 to 45 minutes after receiving some of his medications (the doctors believe the Phenobarbital is having the biggest impact). Then the seizures would gradually return. Most of the seizures are focal and limited to his eyes and mouth watering, lips moving, coughing, rising blood pressure as he has his seizures. Overall, his seizures seem to be getting less intense and shorter duration, but at times they are still occurring every 3 to 4 minutes. His pupils are more reactive this morning, and ventilator settings are minimal. Still getting antibiotics for both pneumonia and C-Diff.

11:30 AM: Family Care Conference this morning. We meet with the Social Workers, ICU doctor, Neurology doctor, and the Rheumatology doctor. As we discuss the options and care plan, it seems like everyone is finally on the same page. One question from Drew’s brother punctuates the main concerns of the family — are the multiple seizures causing damage to Drew’s brain? The doctors explain that the types of seizures Drew has been experiencing will not have a direct impact on Drew’s brain, but that these are simply symptoms of the underlying illness. Whatever is causing Drew to have seizures would be the same cause of any possible damage to Drew’s brain, not the seizures themselves. The plan for this coming week is to continue with the study drug and attempting to control/stop the seizures with the cocktail of medicines. If things persist into next week, we can then discuss the next round of options — and, most importantly, the SPECT scan and any surgical options are way down the road and won’t be discussed again for a few weeks.

9:00 PM: We have gone through many nurses and aides in the ICU now, and almost all of them have been exceptional and above average, but there were two nurses who were not, and today was one of those nurses. This afternoon, following the Care Conference, Drew started having more and more seizures, and it was difficult to find the nurse let alone ask her about anything to stop the seizures. She seemed to be more interested in chatting with her friends at the nurses station then attending to Drew, and several times the kids would run out to ask her to help suction Drew, and she was gone, nowhere to be found. At one point, the kids timed her and she did not step into Drew’s room for nearly two hours. As the day wore on and the lack of attentive nursing care persisted, Drew’s blood pressure and fever also began to climb. After shift change and the new nurse started in the evening, Dawn went and talked to the charge nurse and asked to never have that nurse assigned to Drew again.

11:30 PM: The seizures continued to build until this evening and the night nurse was trying to catch up from the moment she started her shift. Drew’s fever climbed up to 39.8, his blood pressure was sky high at about 180/100, and he was coughing and coughing like crazy. Dawn was so angry with the day nurse as it seemed everything started spiraling out of control during the afternoon and was finally reaching a crescendo tonight. The worst part was when Drew was coughing so hard and so frequently that he vomited all over everything. The doctors had to come into his room and help manage Drew while the nurse could give him some medications. In the end, they had to re-sedate Drew on Propofol and Versed so that he could get some rest and bring his fever and blood pressure back down. They are getting worried about using the Propofol much longer because his labs have been all over the place with his electrolytes. Still, after a terrible day like today, we’re not sure it could get much worse. Multiple, nearly-constant seizures, infections in his lungs and intestine, vomiting, coughing, and still Drew remains unconscious and unresponsive.

As I contemplate God’s will and timing, I am reminded of the story in the book of Daniel of Shadrach, Meshach, and Abed-nego, three young men who refused to worship King Nebuchadnezzar’s idol. The king warned them that they would be cast into a burning fiery furnace if they didn’t comply. They refused and said:

“If it be so, our God whom we serve is able to deliver us from the burning fiery furnace. …

“But if not, be it known unto thee, O king, that we will not serve thy gods.” (Daniel 3:16-18)

Perhaps it is only fitting that as Drew passes through his own furnace of FIRES, whatever and whenever the outcome may be, we will continue to do our part and obediently rely on He who is mighty to save.

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