What are the treatments for congenital adrenal hyperplasia (CAH)?

Treatments for CAH include medication and surgery as well as psychological support.^1,2,3,4

Medication

• Classic CAH 
  
  Newborns with classic CAH should start treatment very soon after birth to reduce the effects of CAH. Classic CAH is treated with steroids that replace the low hormones.
  • Infants and children usually take a form of cortisol called hydrocortisone.
  • Adults take hydrocortisone, prednisone, or dexamethasone, which also replace cortisol.
  • Patients with classic CAH also take another medicine, fludrocortisone, to replace aldosterone.
  • Eating salty foods or taking salt pills may also help salt-wasters retain salt.⁵

The body needs more cortisol when it is under physical stress. Adults and children with classic CAH need close medical attention and may need to take more of their medication during these times. They may also need more medication if they:

• Have an illness with a high fever.
• Undergo surgery.
• Sustain a major injury.

People who have classic CAH need to wear a medical alert identification bracelet or necklace. To alert medical professionals in case of an emergency, the bracelet or necklace should read: "adrenal insufficiency, requires hydrocortisone." Adults or parents also need to learn how to give an injection of hydrocortisone if there is an emergency.

Patients with classic CAH need to take medication daily for their entire lives. If a patient stops taking his or her medication, symptoms will return.

The body makes different amounts of cortisol at different times in life, so sometimes a patient's dose of medication may be too high or too low. Taking too much medication to replace cortisol can cause symptoms of Cushing's syndrome. These include:

• Weight gain
• Slowed growth
• Stretch marks on the skin
• Rounded face
• High blood pressure
• Bone loss
• High blood sugar

It is important to alert the health care provider if these symptoms appear so that he or she can adjust the medication dose.

• Nonclassic CAH 
  
  People with nonclassic CAH may not need treatment if they do not have symptoms. Individuals with symptoms are given low doses of the same cortisol replacing medication taken by people with classic CAH.
  
  Symptoms of nonclassic CAH that signal that the patient may need treatment are:
  • Early puberty
  • Excess body hair
  • Irregular menstrual periods (females)
  • Infertility

It may be possible for patients with nonclassic CAH to stop medication as adults if their symptoms go away.

Surgery

• Classic CAH
  
  Girls who are born with ambiguous external genitalia may need surgery. For example, surgery is necessary if changes to the genitals have affected urine flow.
  
  Surgery for treatment of classic CAH should be done by an experienced surgeon who has expertise with this specific type of surgery. Parents may want to consider surgery for their child during infancy, or they may want to delay until later in childhood. Parents should work with their child's health care providers to determine the best timing of treatments.
  
  The Endocrine Society provides care recommendations on classic CAH , and can help parents find a health care provider who specializes in CAH . The American Association of Clinical Endocrinologists also offers a way to find an Association endocrinologist .
  
  Parents may also want to find a psychologist, social worker, or other mental health professional to support them in their decision making. 
• Nonclassic CAH
  
  CAH Girls with nonclassic CAH have typical genitals, so they do not need surgery.