Adrenal insufficiency (Addison’s disease) occurs as a result of permanent injury to the adrenal glands from elevated VLCFA levels for reasons we do not understand. 80-90% of males with ALD will have adrenal insufficiency. In about 10% of ALD cases, this is the only manifestation of the disorder.
The adrenal glands are located on top of the kidneys and produce steroid hormones that control levels of sugar, sodium, and potassium in the body, and help the body respond to stress such as an illness or injury. In Addison’s disease, the body produces insufficient levels of these steroids, which can be life threatening. Fortunately, this aspect of ALD is treated simply by taking a steroid replacement pill daily (and adjusting the dose in times of stress or illness).
Because of the high rates of adrenal insufficiency in the male population, testing for insufficiency is extremely important, and an endocrinologist should be consulted. Although it is treatable with oral medications, adrenal insufficiency can be life-threatening if it is not recognized quickly (adrenal crisis). An adrenal crisis occurs when there a complete collapse of the adrenal function characterized by low blood pressure, a fainting spell, altered mental status, and seizure – all of which can be life threatening. Patients and caretakers should educate themselves about the importance of stress-dosing steroids with illnesses and major injuries.