Congenital Adrenal Hyperplasia Treatment: Medication, Surgery, and More
The treatments for both classic and nonclassic congenital adrenal hyperplasia (CAH) include medication, surgery, and psychological support, or a combination of all three. Some people who have CAH, including men with nonclassic CAH, may not need treatment at all, but this depends on the severity of the condition.
If you have classic CAH or nonclassic CAH with symptoms, medications are available to help fix your body’s imbalance of hormone levels — namely, a lack of cortisol and aldosterone and excess androgen. People who have nonclassic CAH without symptoms won’t need treatment.
Fludrocortisone (Florinef Acetate) is a steroid medication that replaces the hormone aldosterone. This helps boost development and growth, counter sodium loss, and prevent adrenal crises (a life-threatening emergency that occurs when cortisol and aldosterone levels are too low).
Glucocorticoids (aka corticosteroids or simply steroids) replace the hormone cortisol. This helps manage adrenal insufficiency, control androgen levels, and aid in development and growth. Options include:
In children, glucocorticoids may also cause growth delays, says Phyllis Speiser, MD, a pediatric endocrinologist at Cohen Children’s Medical Center of New York and an associate investigator at the Feinstein Institutes for Medical Research at Northwell Health in Manhasset, New York.
Because of these side effects, “We usually recommend the least long-acting or least potent glucocorticoid, which is hydrocortisone, for daily maintenance,” says Dr. Speiser. “For children, we are especially vigilant about maintaining the lowest possible dose to achieve effective adrenal control.”
The other drugs are used “for situations in which a patient doesn’t respond as expected to the hydrocortisone regimen,” she says.
A medication called crinecerfont (Crenessity) is a corticotropin-releasing factor type 1 receptor antagonist that can also be used with glucocorticoids to better control androgen levels in adults and children over age 4 who have classic CAH. This allows for a reduced daily glucocorticoid dose while maintaining control of androgen levels.
People who take medication for CAH will be monitored regularly, says Speiser. For example, follow-up visits may be:
In people with CAH, those with the classic type will need to take medication for life, says Speiser, whereas those with the nonclassic type may not need medication forever — if at all.
If you have CAH, you may need to take more glucocorticoids during times of physical stress, such as after an injury or when sick. This is called “stress dosing.” Your doctor will give you instructions about using higher and more frequent hydrocortisone doses. Or, if you’re unable to take oral medications, they may prescribe an injectable form of glucocorticoids, along with intravenous fluids, if you go to urgent care, says Speiser.
Some women who have nonclassic CAH may also be given other treatments, including:
In classic CAH, infants born with ambiguous genitalia may be candidates for surgery one day. People who are born female and raised as girls, for example, may choose to have surgery to improve urine flow, make intercourse more comfortable, or reduce an enlarged clitoris.
That said, there are also benefits to waiting. “In the past, people would say that surgery for atypical female genitalia must be done right away,” says Speiser. “But now the thinking has changed so that it’s a matter of shared decision-making. Some families forgo reconstructive surgery in childhood and reserve decisions for when the patient can participate in the decision.”
It’s best to consult a variety of healthcare providers about the pros and cons of surgery and its timing, including a pediatric endocrinologist, surgeon, and urologist. You can find a healthcare provider who specializes in CAH by visiting the Endocrine Society’s physician referral directory.
Any surgery for CAH should be performed at a center that specializes in genitoplasty.
“Mental health counseling is important for the family as a whole and for the patients themselves,” says Spieser. Some support groups for people with CAH include:
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