A Texas pre-teen's medically induced obesity and subsequent slim-down has led her parents to a broader mission: Ensure that people with dangerous adrenal insufficiency get the right treatment in emergencies.
Alexis Shapiro's condition, caused by removal of a benign brain tumor three years ago, prompted nonstop hunger that pushed her weight above 200 pounds. Life-saving surgery in March has left her 50-plus pounds trimmer.
Alexis' condition must be painstakingly managed via oral steroids and 24/7 access to a needle and the drug Solu Cortef, which must be injected within 30 minutes after serious symptoms arise. Yet the vast majority of ambulances don't carry the drug.
Shapiro is banding with a network of"friends and family" impacted by AI to lobby for all EMS units to carry Solu Cortef — and to urge that all first responders learn more about the illness.
People with AI lack vital hormones produced by the adrenal glands. The paired organs sit atop the kidneys, flooding the body with chemicals that, among other things, maintain blood sugar, blood pressure and cardiac output during stress, said Dr. Michael Freemark, chief of pediatric endocrinology and diabetes at Duke University Medical Center.
Some people are born with AI. But far more — possibly "several million," said Freemark, who supports the push for EMS units to carry Solu Cortef — have secondary adrenal insufficiency caused by brain tumors (and their removals), strokes or long-term use of administered drugs like prednisone and hydrocortisone.
"I told them she has adrenal insufficiency and was going to need her shot at the hospital. She was white as a sheet, shaking, going into crisis."
Two close calls with AI have stricken Alexis — the second aboard a jet as sheflew to Cincinnati last spring for the gastric-sleeve operation that curbed her ravenous appetite. (NBC News has followed Alexis' journey since December.)
But it was the first terrifying episode at her kitchen table about a year ago that launched Jenny Shapiro's latest fight for her daughter, who wears a medic-alert bracelet revealing she has AI. A chair accidentally landed on and fractured Alexis' pinky toe — and the injury triggered an adrenal crisis.
"Before the ambulance got there, she said everything was going dark," Sharpiro said.
The mother gave Alexis five steroid pills to swallow — what's known as a "stress dose." (She normally takes one pill in the morning, one at night.) She hoped her daughter would keep the meds down. Vomiting is a common symptom of adrenal crisis. Sharpiro also agonized about giving Alexis an emergency Solu Cortef shot in case her stomach heaved up the tablets. As the EMS team arrived, Shapiro urged them to rush Alexis to the hospital. They urged her to "calm down."
"A lot of paramedics don't know what it is. When they came to my house when Alexis was in crisis, they kind of blew me off when I kept asking if I should inject her. They didn't know what I was talking about," Shapiro said. "That, plus not having the medication on ambulances, creates a dangerous situation."
Many people likely wouldn't learn they have AI until they lapse into a disorienting adrenal crisis, said Eric Hart. He lost his 22-year-old son, Philip, to an adrenal crisis caused by undiagnosed Addison's disease. Philip's adrenal crisis was triggered by a bout of flu.
His death spurred Hart to start 4Philip.org, a nonprofit in the Raleigh, North Carolina, area that has persuaded a local EMS training school to list adrenal shock as an illness paramedics must consider when assessing patients.
"Like Alexis' parents discovered, Solu Cortef is not carried on ambulances — something we seek to change," Hart said. "This needs to go nationwide."
Adrenal Insufficiency United is spearheading the drive to raise awareness among medics and, perhaps eventually, equip every ambulance with a $7 vial of Solu Cortef, a corticosteroid that restores the body's crashing hormonal chemistry and helps people in adrenal crisis to start recovering.
The nonprofit, based in Eugene, Oregon, was formed by Jenny Knapp, whose 18-year-old daughter was born with AI (Knapp's daughter, who wears a medic-alert necklace, prefers not to be named).
Knapp's daughter has a horror story similar to Shapiro's. The triggering incident was a car accident.
"I did not have the Solu Cortef injection with me because my (daughter's doctor) told me EMS squads carry it and would give it to her," Knapp recalls. "The ambulance got there. They didn't have it. I told them she has adrenal insufficiency and was going to need her shot at the hospital. She was white as a sheet, shaking, going into crisis. They barely looked at her."
On Tuesday, Knapp's group launched anonline petition to establish new emergency policies to treat adrenal crises, emphasizing "timely treatment prevents needless disability and death." By Friday, more than 3,000 people had signed the petition.
"You have a $7 injection versus someone becoming permanently disabled or dying from this," Knapp said. "Is this a third-world country where people are dying because of (the lack of) a $7 injection on ambulances?
"I don't blame EMS (emergency medical services) for this," Knapp said. "What they hear in training about adrenal insufficiency is maybe a couple sentences. They want to help. I'd love all EMS to carry Solu Cortef. However, that's not going to work for (all EMS units). Our solution is: 'Why don't you (paramedics and EMTs) just inject the patient-carried meds?'"
"If they want the ambulance squads to give their kids Solu Cortef that someone pulls out of the refrigerator, that's not fair to an EMS squad," Sacchetti said. "The paramedics would think: 'What's really in this vial? How much do they get?' The whole thing is very suspect."
The better choice, Sacchetti asserts, is to treat people in adrenal crisis with Solu Medrol, a glucocorticoid carried by every EMS responder. That drug will "buy you time," stabilizing the patient until they reach a hospital, he added.
"I've talked to some of these parents at length: 'I understand, you've got a child with a special problem and I sympathize.' But I can't say to every EMS squad: 'You must carry all of these very unusual drugs for the three people in the state who have this disease.' You couldn't justify the cost," Sacchetti said.
Freemark, the AI specialist from Duke University Medical Center, disagrees with that stance — and with the number of people perhaps afflicted. He believes all EMS units should carry Solu Cortef — and should include AI among the assessment protocols used to evaluate patients in distress.
"It is a potentially life-threatening condition," Freemark said.