The Acute Phase: Hushing Our Fears
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.
Everyday we make choices. Some are trivial, such as the color of your socks or what to eat for lunch, but some decisions are many times more significant with impacts not only in this world, but also in the next life to come. These monumental decisions that come at the crossroads of life are incredibly difficult and emotionally draining, but a choice must be made. At some point, each of us may eventually be faced with deciding whether to continue aggressive and uncertain treatment for a loved one, or simply to pull back and help them to be comfortable as the illness runs its course. Our family was given this choice for Drew this week.
“Stonewall Jackson was a general in the Confederate Army during the American Civil War. Many military historians consider General Jackson to be one of the most gifted tactical commanders in U.S. history. Once as General Jackson was discussing strategic options with his associates, a commander began his recommendation with the statement, ‘I fear we will not find our wagons tonight.’
“Stonewall responded to the words, ‘I fear,’ in his colleague’s observation with a penetrating truth that ultimately became the general’s trademark: ‘Never take counsel from your fears’” (Mary Anna Jackson, Memoirs of “Stonewall” Jackson, 1895, 264, excerpt taken from a BYU-Hawaii Devotional given by Elder David A. Bednar, December 2012).
ICU Day #27 (Wednesday, July 26th)
10:30 AM: All night long, Drew’s seizures continued to worsen, not only in frequency, but also in duration and clustering. The seizures would crescendo in intensity, at times with clusters of 7 or 8 seizures in less than 30 minutes. This cycle repeated over and over again throughout the night despite the large doses of medication that Drew has been receiving. Finally, starting about 5:45 this morning, he had a continuous seizure that lasted for more than 4 hours. I spent about 90 minutes suctioning his mouth as he was constantly hypersalivating with the sustained seizure. Finally, the 8:00 AM medications slowly started calming things down, but they took nearly 2 hours to finally break the seizure. Drew’s trend is ominous and disheartening and rapidly progressing despite all of the medications, treatments, and best efforts of the hospital staff.
Noon: We had a meeting this past hour or so with all of the doctors (ICU, Neurology, Rheumatology, about 8 doctors in total).
One of the Neurology doctors started off by saying, “I think it is fair to say that despite all of our efforts, Drew’s seizures are the worst now than they have ever been, and they continue to progressively worsen.”
Another doctor added, “At this point, we cannot be certain that the seizures are no longer taking a toll. During a seizure, blood flow decreases in the brain, and if the seizure lasts less than 30 or 45 seconds, it does not necessarily do significant damage. However, when you start having clusters of seizures really close together, or sustained seizures lasting more than 5 minutes, then we typically start to see some damage.”
From the corner of the room, another one of the Neurologists, the director of the Epilepsy Program at Primary Children’s, noted that, “there is still a small chance that Drew could emerge from this illness with near-normal function, but probably only about 10%. There is also a 10% chance that he could still succumb to the illness. Most likely at this stage, though, is that he will fall somewhere in between those bookends, but exactly where he ends up is the big unknown.”
“With the study drug concluding, and after consulting with each of the other teams of doctors in the room, as well as discussions we have had with some of our colleagues around the country,” one of the ICU doctors said as he looked around the room at the other doctors, who were all nodding in agreement. “We feel that it is critical for us to take a much more aggressive approach to curbing his seizure activity.”
The Neurology doctor resumed, “We feel that Drew’s best chance now is to return him to a medically-induced coma for about 7 days, and truly shut his brain down to get some rest and protection from the seizures.”
“While he is sedated,” the Rheumatology doctor added, “We will start Drew on plasmapharesis in an effort to reboot his immune system so that it will quit attacking his brain. If you think about your body’s immune system as an army, when a virus or infectious agent attacks us, the army will fire missiles to destroy the infection. For Drew, however, some of his missiles are attacking himself, and up until now, all of our efforts to stop the bad missiles has been unsuccessful. So the plasmapharesis will essentially wash all of the missiles, good and bad, out of his immune system and his army will be essentially wiped out and need to be rebuilt from scratch — but hopefully this time with no bad missiles.”
Our daughter raised her hand, “We expected the plasmapharesis next, but why would you put him back in the coma when he required a high dose to maintain burst suppression last time?”
“Because the problem with plasmapharesis,” the Neurology doctor answered, “is that it not only washes the immune system, but it also washes out much of the anti-seizure medicines at the same time, which often results in an increase in seizure activity shortly after the plasmapharesis treatment. We feel the sedation will better protect Drew’s brain while we can try and stop his immune system from further attacks.”
“So how will the plasmapharesis work?” Our daughter asked. “And why will he be in the coma for 7 days this time instead of just 3 days?”
“Those are good questions,” the Rheumatology doctor said. “The plasmapharesis will consist of 5 sessions that will be spaced out every other day over the next 10 days. Additionally, we will plan to give him IVig immediately after each plasma treatment so that he may have some added protection from infections and also additional treatment to help suppress his antibodies causing the damage.”
The Neurology doctor added, “Because of the long half-life of Pentobarbital, we will be able to bring Drew out of the sedation prior to the last plasmapharesis treatment. Often with autoimmune disorders, patients will note improvement after only 2 or 3 treatments, but we plan to give Drew 5 treatments.”
The ICU doctors then warned, “With the more aggressive treatment also comes a higher risk of complications. In fact, we will be lucky if Drew doesn’t get an infection while he is immunosuppressed. Granted, we will do everything we can to prevent it, but after more than 7 days of intubation, and prolonged insertion of other lines and catheters, the risk of infection rises even without the immunosuppression.”
“Further, after putting Drew through so much without any success at stopping the seizures, and not knowing the extent of any possible functional or cognitive impairment, you do have the option to not take any aggressive measures and stop all treatments, essentially just making him comfortable until he succumbs to the illness,” another ICU doctor explained. “Some families choose this, and we would respect that if this was your choice as well.”
We were not prepared for that choice. We knew Drew’s situation was very critical, but we thought he was stable enough that death would not be an option at this point. Tears started to spring from my eyes, and I looked at Drew’s mother and sister, and they were also crying. Once the impact of their explanation sunk in, it didn’t take very long for us to say, “We want Drew treated. Please let’s try the plasmapharesis.”
“Very well,” the ICU doctors nodded their heads, “we will start this afternoon to get Drew into burst supperssion.”
In the Book of Mormon, there is a story that identifies the key to dispel fear and provide peace even in the face of great adversity. We read in Mosiah that in the land of Helam, Alma’s people were frightened by an advancing Lamanite army.
“But Alma went forth and stood among them, and exhorted them that they should not be frightened, but … should remember the Lord their God and he would deliver them.
“Therefore they hushed their fears” (Mosiah 23:27–28).
Elder David A. Bednar explains, “Notice Alma did not hush the people’s fears. Rather, Alma counseled the believers to remember the Lord and the deliverance only He could bestow (see 2 Nephi 2:8). And knowledge of the Savior’s protecting watchcare enabled the people to hush their own fears.
“Correct knowledge of and faith in the Lord empower us to hush our fears because Jesus Christ is the only source of enduring peace.”
4:00 PM: The family was bereft as we continued to try and process the dire situation and critical nature of Drew’s condition. Even though Alex had been sick earlier in the morning and Ashley had gone out to lunch with our neighbor, we felt after the Care Conference discussion it would be best to have all of the family come to the hospital to talk about Drew.
“I am not ready to see Drew in a coffin,” his brother shouted as tears welled up in his eyes. “What good will any of these treatments have been for if we just give up now?”
“We’re not giving up on Drew now,” I assured him.
“But Drew still has a chance to be completely normal,” his brother continued. “They said he still has a chance. Why would they ask if we wanted to stop treatment now?”
“They said it was because some families don’t want to continue after so long and with a chance of brain damage,” I said as I shook my head. “We are not one of those families.”
Dawn and the children were so upset, I decided I had better skip going into work this afternoon and spend the time talking with them as we try to figure out the serious implications and possible outcomes. Granted, we were all disappointed that the Sage 547 drug and earlier treatments didn’t work for Drew, but the thought that some families might give up at this relatively early stage of the acute phase (which on average lasts 3 to 4 weeks anyway) just boggles the mind. There are also still several other options that have not been attempted beyond the plasmapharesis, so we vow to never give up on Drew, and that seems to help his brother and sisters calm down somewhat. We are definitely at a critical juncture and it is imperative that we can get the seizures stop, but in the end, we are still hoping for the best and agree that this treatment will give Drew the best chance to come back to us.
8:30 PM: As family continued to gather in the front lobby, we sat around and cried and hugged and tried to encourage each other that it would all work out for the best. To think that we were closer now to possibly losing Drew, even if it was still remote, had rattled the family. However, we reassured everyone that we were not giving up on Drew and would continue to battle these seizures. After all, we still had hope for our beloved son and brother.
The key to truly overcoming our fears is to have our focus firmly fixed upon the Savior at all times and in all places. Another example comes from Matthew 14: 27-29 when the Apostles were in a ship, tossed by a storm in the midst of the sea. The Savior went to them, walking on the water, but they did not recognize Him and they cried out in fear.
“Jesus spake unto them, saying, Be of good cheer; it is I; be not afraid.
“And Peter answered him and said, Lord, if it be thou, bid me come unto thee on the water.
“And he said, Come.”
Peter then walked on the water to Jesus.
“But when he saw the wind boisterous, he was afraid,” began to sink, and cried out, “Lord, save me.
“And immediately Jesus stretched forth his hand, and caught him, and said unto him, O thou of little faith, wherefore didst thou doubt?” (Matthew 14:30–31).
From Peter we can learn that we will be blessed as we respond immediately with faith in the Savior to His invitation. With his eyes fixed squarely upon Jesus, Peter stepped out of the boat and miraculously walked toward him on the water. However, as soon as his gaze was diverted by the wind and the waves, then fear crept into his heart, pushing out his faith, and Peter became afraid and began to sink. I tried to keep these examples in mind as I talked with the family. It has been a difficult day to maintain hope, but with continual reliance on the Savior and His teachings, we made it through this very hard day.
ICU Day #28 (Thursday, July 27th)
9:00 AM: Overnight, they had to re secure Drew’s breathing tube, and as they did that, the feeding tube got pulled out a little bit. Breathing tube looks good, but they will have to replace the feeding tube (the tip is in his stomach and they want it in his small intestine). His burst suppression is down to about 3 to 6 bursts per screen, and they want it at 1 to 3, so they will up his pentobarbital a little bit above the 5.0 it is currently at. His blood pressure has been OK most of the night as nursing has balanced the epinephrine and norepinephrine with the sedation all night. Lastly, he has been running a pretty high fever all night and is currently at 39.5 Celsius. We have been putting on ice packs and they have been giving him Tylenol and Ibuprofen, but his fever has been resistant (cultures are still negative).
11:30 AM: ICU rounds just finished. Labs and cultures all negative, so they will treat the fever but still do not believe he has an active infection. However, the ICU attending, Dr. Woods, said we are on high alert for infection prevention now and he will be a germ-Nazi going forward. As Drew becomes immunosuppressed, everyone needs to be very strict with hand washing and cleaning of everything going into his room, especially if we come anywhere near Drew. Occupational Therapy came and stretched his arms, but since Drew is sedated again with fluctuating blood pressures, they will hold off on getting him up to a chair for a while and just do ROM on his arms and legs again for now. One of the nurses also came and placed a new feeding tube through Drew’s nose down to his stomach, and hopefully into his small intestine.
3:00 PM: Dawn and Drew just went with the nurses down to Interventional Radiology for placement of the central line that will enable them to perform the first round of plasmapharesis tomorrow. Drew’s Pentobarbital level is at 6 on his sedation, but he is still not quite fully suppressed. He continues to run a fever this afternoon and we just helped the nurse put some ice packs on him so hopefully those will help bring body temperature back down. Blood pressure good, everything else good so far. Hoping for a quiet night and starting the next round of treatments tomorrow.
If you couldn’t tell, I love the words of Elder David A. Bednar, who discussed hushing our fears during at least three of his talks. He promised us:
“To not take counsel from our fears simply means that we do not permit fear and uncertainty to determine our course in life, to affect negatively our attitudes and behavior, to influence improperly our important decisions, or to divert or distract us from all in this world that is virtuous, lovely, or of good report. To not take counsel from our fears means that faith in the Lord Jesus Christ overrules our fears and that we press forward with a steadfastness in Him. To not take counsel from our fears means that we trust in God’s guidance, assurance, and timing in our lives….
“As we exercise faith in Christ and trust in His promises, we can walk into the dark with the absolute assurance that our pathway will be illuminated—at least far enough to take the next step—and then the next step—and the next step…. I recall listening to President Hinckley on several occasions counsel doubters, cynics, and critics that ‘everything will work out.‘ And he was right. Everything worked out—even though we did not know at the time all of the details and specifics….”
I believe everything will work out, and as we maintain our faith in the Savior, we can hush our fears and allow peace and hope to enter into our hearts. At a minimum, Drew needs that from us now more than ever.