Manual Transmission

It’s been five years since I lost my adrenal glands.  I had no idea when I woke up from anesthesia how dramatically the loss of those two little balls of hormones would change my life.  The endocrinologists originally prescribed hydrocortisone tablets and said to take them two times each day and I’d be fine.  I wasn’t.  My quality of life deteriorated over time.  I would have random days where I suddenly felt terrible, and woke up every single morning with headaches, nausea, pain, and pretty severe confusion while waiting for the pills I took to kick in.  Then, I’d feel better.  The medicine would work.  After four to six hours, hydrocortisone leaves the system, and I’d feel pretty awful again until taking my next dose when the alarm went off on my phone at noon each day.  At 4:30pm, it’d go off again and I’d take another, smaller dose.  Then to bed in the evening and start over again.  It was like being on a daily roller coaster—wake up, feel awful, take pill, feel better, start to feel crappy again, take a pill, feel better, then crappy, then better—all day long, every day.  Waiting for the alarm to go off so I could take another one, wait an hour for it to kick in, and then keep going.

A few months after my surgery, as I started to realize that things were very different, I read about a patient with my exact same condition (even down to the specific genetic mutation which causes our disease), who also has a child with it, and who had lost both glands to surgery to remove pheochromocytoma tumors.  She was using a diabetic insulin pump to deliver liquid hydrocortisone medication (not insulin) so that she had a more consistent, steady stream of medication.  In a normal human body, the adrenal glands release cortisol on a diurnal rhythm based on the sleep-wake cycle.  While you sleep, your adrenal glands begin to produce cortisol between 2:00 and 4:00am, so that by the time you wake, the peak amount of cortisol is in your body and doing its job to keep you healthy and alive.  As the day goes on, that level drops off so that by the time you are sleeping at midnight, it’s almost undetectable (but you still have a small amount in your body).  With an insulin pump, one can tailor the amount of medication infused into subcutaneous tissues and program the machine to increase or decrease the amount during different times of day.  So this woman had programmed it to release hydrocortisone into her body to mimic (as closely as possible) a normal adrenal gland’s daily function.  Genius.

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Omnipod tubeless pump with custom made cover.

I researched it as much as possible, and found others doing the same thing.  There aren’t many, and because it’s not considered a “standard of care,” most endocrinologists are either unfamiliar with it as a form of treatment or are uncomfortable supporting it because of a lack of studies and guidelines.  There is very little research being done on this in large part due to the rare nature of the disease and the lack of financial incentive for the manufacturers of the insulin pumps.  Diabetes is much more lucrative for them.

I had a new endocrinologist and a new hope that maybe I could bring up the idea of using a pump to improve my quality of life and overall health.  My doctor had only had one patient, during her residency years, who was using a pump for adrenal insufficiency.  Most doctors would have immediately said no, but she agreed to let me try to pursue it probably in large part because she knows I have absolutely zero adrenal function in my body.  It took me about a year and a half to convince my insurance company to agree to cover it, and my Omnipod tubeless pump system arrived in May of 2018.  Within a week, the morning headaches were gone.  In the two years using it, I’ve tailored and tweaked the dosing to find an amount that is as close as possible to what my body needs.  It’s still trial and error—anytime I face a stressor or illness that I don’t adjust for properly I suffer the consequences of too much or too little steroid.  Too much means I eat everything in sight, gain weight, and risk long term effects of osteoporosis.  Too little and all my low symptoms kick in and I risk dying.  Your body adjusts the amount of cortisol in tiny increments each day—are you getting a slight cold?  Exceptionally stressed at work?  Car accident?  Minor surgery?  Your body knows exactly how much you’ll need to recover.  Mine asks for more cortisol (sometimes SCREAMS for it), but there is no answer from my absent adrenal glands, so I have to respond once the symptoms show up which is much later than your body would’ve reacted.

Let me be clear—the pump is not a silver bullet.  There is no “set it and forget it.”  Nor is there a way to scientifically assess my body’s need.  Drug companies just don’t have the capability or financial desire to invest in a home test solution for AI patients.  If you’re interested in trying this treatment, be sure to contact your physician to discuss it.  The pump isn’t the right choice for every patient. Having said that, the cortisol pump has dramatically changed my life for the better.  I haven’t been to the ER with crisis symptoms since using the pump and have recovered more quickly from illnesses.

Being diagnosed with AI is like going from driving an automatic transmission car to suddenly driving a manual transmission—without the RPM gauge to tell you when to shift.  Patients have to learn to read their bodies for signs and symptoms of an impending low and adjust steroid intake accordingly.  The best resource is to journal and learn to listen carefully to your body, as well as connecting with other patients who live with this rare condition.

For more information on using the cortisol pump to treat Adrenal Insufficiency, go to www.thecortisolpump.com

One thought on “Manual Transmission

  1. I appreciate receiving your update, Kari; I admire your perseverance; and celebrate your successes. And just so you know I’m not a spammer, I’m copying your Dad, whom I’ve known for only 67 or so years. Although he’s younger, smarter, more handsome, and a better athlete than me. (At least one of these things is true.) Take care! Bruce

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