Will
Introduction
This is the fifth of a series of educational vignettes addressing the serious risks associated with adrenal insufficiency and adrenal crisis. These fictional stories reflect real-life situations that many patients and families face. Because this particular vignette has a tragic outcome, it may be emotionally difficult, especially for those who have experienced a similar trauma or loss. Readers are advised to decide whether to read it based on their own comfort level.
NADF’s goal is for no one to go into crisis, and for no one to die from crisis. However, sometimes low cortisol’s symptoms, like confusion and brain fog, prevent recognition of an impending or ongoing crisis. Additionally, some crises come on suddenly, leaving little to no time to intervene. Most crucially, no cortisol-equivalent version of an EpiPen-like emergency treatment currently exists, leaving people in crisis and those around them without any expedient emergency treatment. Will’s story describes the tragic results of lack of availability of emergency treatment.
– By Susan Majka, NADF Secretary
Will’s Story
Will was excited to attend his best friend Logan’s all-day 12th birthday party. Eight of the guys were meeting at a local pancake restaurant for breakfast at 7 am, and then Logan’s dad was driving them in a van to the biggest amusement park in the state, which was located in the middle of farmland and far from the nearest town. They had dared each other to ride the Devil’s Drop, a ride that would plunge them 60 feet before coming to a sudden stop.
Will’s mother had carefully instructed Logan’s dad about Will’s need for hydrocortisone, specifically that it should be taken between noon and 1 pm. Additionally, she had increased his daily dose by ½ to account for the excitement of the day. Will’s adrenal insufficiency (AI) had been fairly well controlled for the three years since diagnosis.
The group arrived at the amusement park at 10 am. Entry to the park was $75 for those under 13, but Logan’s dad would have had to pay $100 to get in, and he did not plan to go on any rides, so he decided to wait in the van and listen to the ball game on the radio. He completely forgot about Will’s medication.
The boys rode on the state’s tallest roller coaster, the tumbling cages that held them upside-down for minutes at a time, and the space themed rocket in the dark. They enjoyed turkey legs, funnel cakes, milkshakes, and burgers. Now it was 6 pm, almost time for them to leave. They had one last ride: the Devil’s Drop. Will complained about feeling nauseated and dizzy, and his friends teased him that he was trying to get out of the challenging ride. Will even threw up in a trash bin and was leaning against Logan in the line for the ride. But he did not want to be considered a sissy, so he was determined to get on the ride. He and Logan were strapped in side-by-side, and the seats rose slowly to the top of the tower, then plunged. At the bottom of the ride, they noticed that Will’s head was lolling against his chest and his shirt was covered in vomit. The park’s staff immediately called 911 and began emergency treatment, but no one understood what the cortisol called for on Will’s emergency alert bracelet was or where to get it.
By the time the rural ambulance service got Will to the nearest emergency room, he could not be revived. He was pronounced dead.
Questions
How many cortisol-consuming stress events can you recognize in this scenario?
ANSWER: the excitement of the birthday party, the change in routine with a breakfast out and trip to the amusement park, the unusual foods, the various thrill rides.
How many symptoms of low cortisol can you identify in this scenario?
ANSWER: nausea and vomiting, dizziness, weakness.
What actions might Will have taken when he began feeling bad?
ANSWER: Remember to take his cortisol or prednisone. Find a responsible adult and inform them about his condition and need for immediate cortisol. Ask Logan to call his dad and bring his cortisol to the gate of the park. Alternatively, Will also should have been carrying cortisol in his pocket to take.
What might have prevented Will’s death if the pharmaceutical companies had developed it?
ANSWER: If Will had been able to have an auto injection device with cortisol in it, he would have been able to inject himself when he first became nauseated and lightheaded. Realistically, it is impractical to expect a person to be able to carry a cortisol ACT-O-VIAL, a syringe, and needles, and to be able to mix the solution and draw it up during a crisis, particularly in the setting of an amusement park.