Managing multiple medications for treating CAH

Congenital adrenal hyperplasia (CAH) is a group of inherited genetic disorders that interfere with the body’s ability to produce the hormones cortisol and aldosterone.

Cortisol is often called “the stress hormone” because it regulates the body’s stress response. It also plays an essential role in metabolism, maintaining blood pressure, and immune activity. Low levels can interfere with all of these processes.

Aldosterone is critical for regulating the body’s sodium and fluid balance. Low levels of this hormone can result in “salt wasting,” with potential complications like dehydration, dangerously low blood pressure, and toxically high levels of potassium.

Some forms of CAH also cause an overproduction of another type of hormone, androgens. Androgens play an important role in growth, development, and maturation. Excess levels in the body can cause early puberty and other potential health issues.

However, CAH can vary significantly from person to person. People with mild forms of the disease may only require monitoring or low doses of medications, while those with more severe forms of the disease are at risk for significant complications. For severe CAH, continuous treatment with medications is needed to prevent complications related to the body’s inability to maintain hydration and adequate blood pressure.

Treatment plans can require multiple medications

Medications are a mainstay of treatment for CAH, and many people require multiple medications. This can include:

• Glucocorticoids (also called corticosteroids) to replace missing cortisol.
• Mineralocorticoids that help replace missing aldosterone. These help the body maintain salt and water balance.
• Salt supplements to help maintain electrolyte levels (primarily used to treat salt-wasting forms of CAH during infancy).
• A glucocorticoid emergency injection kit to be used in case of an adrenal crisis, a life-threatening complication where adrenal hormones are critically low.
• Medications that help reduce excess androgen production. In some people, these medications can reduce the glucocorticoid dose needed to manage CAH.

Not everyone with CAH will require all of these medications or require these medications all the time. Additionally, treating CAH may require stress dosing at certain times. Stress dosing is a temporary increase in glucocorticoid medications prescribed during periods when the body has an increased need for cortisol, such as illness, fever, injury, dental procedures, and surgery.

Monitoring for medication side effects is also an important aspect of treatment. Though a medical necessity, long-term use of glucocorticoid medications has been associated with weight gain, diabetes, and osteoporosis.

Strategies for managing multiple medications

Following a medication schedule that includes multiple medications can be challenging. If you or a loved one is prescribed multiple medications to manage CAH, the following strategies may help:

Have clear instructions and a schedule

• Have clear instructions on how and when to take every medication. In order for a medication to work as intended, each dose should be taken as directed and on schedule.
• Write down the full medication schedule, including dosages, timing, and what each medication is for. Keep this list in a visible location and have copies for family members and anyone sharing caregiving responsibilities.
• Also know what to do if you miss a dose of a medication.

Stay organized and set reminders

• Keep medications, supplies, and schedules in one place that is organized and clearly labeled.
• Use a pill organizer that separates oral medications by day, and by time of day if needed. Set a reminder to refill the organizer on the same day each week.
• Set alarms or reminders on your phone for each dose. Have a backup reminder in a visible spot in case an electronic reminder is missed.
• Pair medication times with an existing daily habit, such as breakfast or brushing teeth.

Plan ahead and be prepared for interruptions

• Keep extra doses on hand in case you will not be home at your normal time.
• Tell your healthcare provider about upcoming travel or any other changes to your routine that could affect a treatment schedule or appointments.
• When traveling, bring more doses than you think you will need. Also have a list of all medications and dosages, a copy of the dosing schedule, and emergency contact information.
• Tell your healthcare provider about anything that could require stress dosing.

Each of your healthcare providers should have an up-to-date list of all your medications, including prescription drugs, over-the-counter drugs, and supplements (including herbal products). This helps avoid unintended drug interactions. A pharmacist is also an excellent source of information.

If you or a loved one is having difficulty following a medication routine for any reason, talk about it with a healthcare provider.