Liam from Aiken in South Carolina, US, asks what good is an emergency treatment if it is difficult to administer. What good is it if EMS crews are not allowed to carry it? An adrenal crisis is a race against time, and the pharmaceutical industry and emergency medical services need to give more support
Living with CAH
Everyone thinks about their own mortality at one point or another. But the thought that we are not invincible and that we could perish at any moment is rather depressing. Oftentimes, it is easier to ignore this idea of our temporality on earth than to contemplate and acknowledge it. For the general population, this ignorance is allowed. However, the rare disease community does not get this blessing in life; they must face the intrusive thought of death, and they must acknowledge and accept it. For instance, a person like me, who lives with classic congenital adrenal hyperplasia, has to confront the idea that they can die in a matter of minutes if they are in adrenal crisis—a medical emergency caused by a lack of cortisol.
Classic congenital adrenal hyperplasia, or Classic CAH, is an autosomal recessive disorder which affects the adrenal glands. Simply put, I do not produce cortisol on my own, nor do I have an easy time maintaining adequate salt levels.
I am grateful that a life-saving intramuscular shot is available to treat people with classic CAH (and rare diseases that cause adrenal insufficiency, such as Addison’s disease). That shot goes everywhere I go. It experiences everything I experience and sees everything I see, almost as if it has a life of its own. But every time I feel the shot in my pocket or open my bag and see it staring right back at me, I am reminded that I do not control my actions—the shot does.
"When illness or injury occurs in adrenally insufficient individuals… an immediate additional dose of glucocorticoids (hydrocortisone) is necessary to avert adrenal crisis. Delay in administration of these medications can lead to shock, heart failure and death.” Source: CARES Foundation
Every decision I make needs permission from that shot—and it does not give me blanket permission for me to live my life as I would wish. There are numerous activities that will never be allowed. Hiking, for example, would be like asking for the strings that attach the puppet to the puppeteer to be cut off, asking for death. If I have an adrenal crisis, I need to be around people who know what to do.
I wish these restrictions were not the case, but I value my one chance at life, so I need to accept them.
Dying with classic CAH?
The problem with the shot available today is that it needs to be done precisely; it takes several steps and several minutes. If someone is in adrenal crisis—the symptoms of which include vomiting, dizziness or loss of consciousness—they will have a hard time administering the shot themselves. Despite that, the EMS (emergency medical services) protocols of many states mean that most ambulances do not carry the shot, nor are ambulance crews allowed to administer the patient’s own shot. So, even if I survive the seven-minute wait for EMS—the average in the United States—their arrival may not improve my chances.
Someone living with classic CAH and other conditions causing adrenal insufficiency could pretend that having the shot with them at all times is enough, but I’d say they are in blatant denial and, quite frankly, delusional. The drug’s design and EMS protocols reduce the odds of a CAH patient surviving an adrenal crisis.
Right now, it seems that people with adrenal insufficiency are on their own in their race against time for survival. We need the pharmaceutical industry to provide a shot that is easily and quickly administered, and we need the medical authorities to improve EMS protocols for adrenal insufficiency, so that ambulance crews are allowed to carry the shot and administer it to patients.
Editor’s note: CARES Foundation plays a leading role in improving the lives of the Congenital Adrenal Hyperplasia community. It seeks to advance quality health care through advocacy, education, research and support. Find out more on its website: caresfoundation.org
The foundation advocates for the inclusion of treatment for adrenal insufficiency in EMS protocols with on-going appeals across the United States. Find out more, here: caresfoundation.org/advocacy-ems-campaign
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