Adrenal Insufficiency or Addison’s Disease

 

90% of boys with ALD will also have adrenal insufficiency or addison’s disease, which occurs when the adrenal glands do not produce enough of certain hormones. The adrenal glands are located just above the kidneys. Adrenal hormones, such as cortisol and aldosterone, play key roles in the functioning of the human body, such as regulating blood pressure; metabolism, the way the body uses digested food for energy; and the body’s response to stress.

While ALD usually does not present before the age of 3, Adrenal insufficiency can present within the first year of life and therefore it is extremely important to test blood ACTH and cortisol levels. Adrenal insufficiency can be treated easily by replacing or substituting the hormones the adrenal glands are not making with daily steroids. The dose of each medication is adjusted to meet the needs of the patient.

Problems can occur in people with adrenal insufficiency, who have an illness, suffer an injury, or are undergoing surgery or sedation for a medical test. To prevent an adrenal crisis, which can lead to death, the dosage is increased to allow the body to handle the additional stress. People with adrenal insufficiency should always carry identification stating their condition, “adrenal insufficiency,” in case of an emergency, as well as the supplies necessary to administer an emergency corticosteroid injection.