Congenital Adrenal Hyperplasia Treatment
Congenital adrenal hyperplasia (CAH) occurs when your child’s adrenal glands (glands that make hormones) don’t work properly. In most forms of CAH, your child’s body does not produce cortisol (stress hormone), resulting in too much testosterone (male sex hormone).
There are two types of CAH:
- Classical CAH is present at birth. People who have classical CAH need lifelong treatment.
- Non-classical CAH usually presents later in life and causes milder symptoms overall. Children with non-classical CAH may only need treatment if they develop unwanted or severe symptoms.
Pediatric CAH Treatment
At Children’s Hospital Los Angeles, our specialists are experts in treating congenital adrenal hyperplasia. We offer extensive support to ensure that children and their families living with CAH know how to care for themselves. With every treatment, our goal is to help your child have the highest quality of life possible.
CAH treatment may include:
Medication
Children with classical CAH need to take medicine every day for the rest of their lives. Babies begin treatment by taking a steroid called hydrocortisone to replace cortisol, one of the hormones produced in the adrenal glands. Your child may also need another medication called fludrocortisone to replace aldosterone (a hormone that regulates salt and water in the body).
Your child also needs to wear a medical bracelet or necklace to alert medical professionals in case of an emergency. It should say, “adrenal insufficiency, requires hydrocortisone.” Caregivers also need to know how and when to give emergency treatment for adrenal insufficiency.
Emergency treatment for adrenal insufficiency
Adrenal insufficiency can lead to acute adrenal crisis, a medical emergency that occurs when cortisol levels are dangerously low. Signs of acute adrenal crisis include:
- Abdominal pain, nausea or vomiting
- Dehydration
- Excessive or unusual sweating
- Fatigue or extreme weakness
- Fever or chills
- Rapid heart rate
- Sudden confusion or loss of consciousness
When acute adrenal crisis occurs, children must receive an immediate injection of hydrocortisone. If your child has primary adrenal insufficiency, you will receive an emergency kit with an injection of this emergency medication. A specialized nurse will train you on when and how to administer the injection.
Surgery
Classical CAH can cause atypical external genitalia in females. The Division of Urology at Children’s Hospital is a designated Medical and Surgical Center of Excellence for CAH by the CARES Foundation (Congenital Adrenal Hyperplasia Research, Education and Support). Our team works closely with each family to create a personalized surgical plan.
Depending on your child’s symptoms and age, some caregivers choose to delay surgery. The care team will explain all risks and benefits to you and help you make the most appropriate decision for your child.
Multidisciplinary CAH Care
Your child may receive care from other specialists, including:
- Dermatologists for skin issues, such as acne
- Dietitians for weight management
- Genetic counselors for guidance on how genetic disorders may affect your family
- Psychologists for care of living with a chronic condition, in addition to screening for co-existing conditions like depression, anxiety or ADHD
- Radiologists for state-of-the-art imaging such as X-rays, ultrasounds or MRIs
- Social workers for support with psychosocial needs, such as identifying financial resources, finding transportation or coordinating time away from school
Expert Care for Adrenal Disorders
When your child receives a classical CAH diagnosis, our specialists spend extensive time educating your family on how to care for the condition. As your child grows, we continue to offer robust support. When you choose Children's Hospital Los Angeles for CAH treatment, you have easy access to highly trained experts.
We run multiple support groups to help caregivers and children, teens and young adults adjust to a CAH diagnosis and connect with others. We also host educational conferences for the CAH community.
When it’s time for teenagers to begin transitioning to adult care, we facilitate a smooth hand-off to ensure young adults experience no gaps in care. Learn more about the Congenital Adrenal Hyperplasia Clinic.
