MEDICAL INVOLVEMENT
By Ian Yeoh
I have on occasion been criticized for being cynical about the medical profession, and often being critical of physicians and their professional conduct. I have several extended family members around the world who are physicians, and they on occasion tease me about my contempt, noting that I myself had once wanted to be a physician. This article is written with absolutely no disrespect to the medical community or to any of the superb medical facilities and professionals being discussed. Nevertheless, this article shall discuss the importance of ARPKD parents being well informed and vigilant about the care of their children.
It was August 1996 and my now twenty year old son, Mitchell, was then six months old; he was at the high end in terms of ARPKD disease severity. Mitchell and his mom were based at Lucile Packard Children’s Hospital at Stanford University in Palo Alto, California. I was based in Connecticut, working in New York City, in order to provide medical insurance and financial resources for the family to overcome the ordeal of ARPKD. At that time, only two recorded successful very small ARPKD infant kidney transplants with survival were based in the San Francisco area. The team that conducted both of these transplants had recently moved from UC San Francisco to Stanford. Upon deliberation, it was resolved that Mitchell had to be transported from Yale-New Haven, where he had been based for the first month of his life, to Stanford. No hospital on the East Coast, including Boston Children’s and Columbia Presbyterian, or the Midwest, including Mayo and Pittsburgh Children’s, wanted the risk of transplanting an infant. I was blatantly told that there was no hope for Mitchell. Yale could not wait for me to transfer Mitchell out because this was unchartered territory. After speaking to Stanford, it was determined that the objective was to get Mitchell to grow till at least 8 kg, ideally to over 10 kg, before kidney transplantation.
During his infancy, Mitchell suffered from horrible bouts of Grand-Mal seizures. Such seizures are not commonly associated with ARPKD, and the extent of Mitchell’s seizures were rather unusual. Grand-Mal seizures feature a predictable loss of consciousness, violent muscle contractions and abnormal cranial electrical activity. In the general population, such seizures can be triggered by epilepsy, endocrine issues, low blood sugar and many other conditions.
It was a Friday evening and my wife had dressed Mitchell and herself to go to dinner at a friend’s home. Mitchell was doing relatively well. He had completed peritoneal dialysis earlier in the day, and was awake, alert and generally happy. I was coming off a flight from New York City, landing at San Francisco airport. As per usual practice, I was riding in a taxi to Palo Alto. My intentions were to go directly to our friend’s home to meet my family for dinner. Instead, I arrived to a lot of panic without having full details of what had transpired over the past hour since I last spoke to my wife. As per common practice, my cell phone was switched off while at the airport and I had neglected to switch it back on after I left the airport. My wife also had to switch her cell phone off while at the hospital. Mitchell had a series of terrible seizures and had been rushed to the hospital.
After driving a borrowed vehicle from somebody attending the dinner whom I did not know, I arrived at the hospital in total terror, with fears that I had arrived too late and that my son was already gone. I got up to the PICU and was ushered into a very strange area. It was still the ICU but a secluded alcove, almost with hints that this is the area for those children who “are not going to make it.” Code Blue had already been called and there were at least fifty medical personnel in this area. Lights were flashing and panic was written on all the shell shocked faces. I identified myself but two residents and three nurses were preventing me from entering my son’s room. What is this farce? If this were to be the last time I am going to see my son alive, I darn well wanted to be in the room. After about a minute of arguing, I lost my patience and said: “you will just have to arrest me; call security; they can handcuff me.” I barged into the room. My wife was inside and I knew the situation was impossibly tense when she was not crying but was instead praying. They had drawn the blue curtain, preventing my wife from seeing what was really going on.
“What kind of show are you guys running?” I did not realize that nobody inside the room knew me. I was still dressed in my suit and tie coming off the plane. They thought I was a doctor. “How long has he been seizing?” Meanwhile, my wife was still standing by herself in a corner of the room and nobody was paying her any attention. Mitchell had been seizing for almost half an hour. I was witnessing vital signs that I had never realized were humanly possible.
It was clear that these doctors did not have their act together. “Do you not know his anti-seizure medicine?” There was a very quiet response from one of the fellows: “No, we don’t.” Ouch! I yelled “Phenobarbital stat!” From the side of the room, behind the drawn blue curtain, my wife yelled: “100 mg,” and I chimed in “and turn down that IV rate.” My attitude was that doing something was better than doing nothing, and 42 ml/min seemed too aggressive a drip rate for a six month old infant.
Later, I asked my wife why she chose 100 mg and she just replied “mom instincts.” The fact of the matter is that she had memorized the dosage and brand of every medication that Mitchell was receiving. She was attending dialysis, medical procedure and pharmacy classes every morning at the hospital. Every moment of my time away from work and away from my family was spent reading medical literature. That is how we knew which medication and guesstimated the right dosage.
Just ten minutes ago, I was seeing Mitchell slipping away. I was holding his small hand and even whispered that it was alright for my little champ to give in. Mitchell was pale; he was lying in a pool of his own urine and stool, and was absolutely drenched in sweat. He looked like he could not hang on any longer and looked to have given up. Later in life, in his mid-teens, I asked Mitchell if he remembered the episode, and he replied that he had already found peace through meeting the Big Guy, but was told by the Big Guy that his time had not arrived. Within three minutes of receiving the Phenobarbital, Mitchell began normalizing, and after five minutes, Mitchell’s vitals came out of the danger zone. I unilaterally opened the blue curtains in disgust.
In the heat of the moment, it still did not occur to me that nobody by the bedside knew that I was Mitchell’s father. I received a curt “thank you” and “doctor, thank you, you saved the patient’s life.” Out came laughter from two parents in unison, which was so inappropriate considering the gravity of the situation. Note that my wife and I were not standing together, and since we are of different ethnicities, nobody would realize that we are a married couple. “We are not doctors. We are Mitchell’s parents.”
Yet, that was not the end. Husband and wife were ushered into a room and interrogated as to why we spoke up, and how we knew which medication and what dose. I became very insolent. You guys, the doctors, did not know which medication and the loading dose. How could that be possible? In actual fact, the physical medical files were misplaced, and the hospital did not have a decent IT system to maintain electronic notes at that time. Teams of physicians streamed into the conference room to hedge themselves. The neurologists were particularly nervous, warning us parents that our son may never walk or speak, implying that our son is probably now mentally incapable. The head neurologist even explicitly explained that our son “may not do well in school.” Years later, when Mitchell turned 18, Mitchell and I had a laugh about that neurologist’s outrageous statement. Mitchell is a tremendously gifted student.
I hope that this article adequately demonstrates why ARPKD parents should be intimately involved in the healthcare of their chronically ill children, and work as a team. I highly recommend the use of technological peripherals such as touch tablets and other lightweight electronic devices for data storage and note taking. Do not assume that just because physicians are presumably intelligent and competent that they are infallible. Parents know their children best and decision making should be made in unison.
