DEAR DOCTOR K:
I watched a documentary about President Kennedy around the 50th anniversary of his death last year. The program mentioned that he had something called Addison’s disease. What is that?
Addison’s disease is a rare condition in which the adrenal glands do not function properly. The thumb-sized adrenal glands sit on top of the kidneys. When working properly, they produce several hormones. But with Addison’s disease, the adrenal glands do not make enough hormones.
Hormones are substances made by one of several glands in the body. They travel in the blood to all parts of the body and can affect many types of cells and organs. The adrenal gland makes three main types of hormones:
- Glucocorticoids. These are steroid hormones. One type is cortisol, which helps regulate blood sugar and the body’s response to infection or stress.
- Mineralocorticoids. These help regulate sodium, potassium, blood pressure and blood volume.
- Androgens. These hormones are needed for normal sexual development and ongoing sexual function. Testosterone is the most well-known androgen. Even the adrenal glands of females make some amount of androgens.
Addison’s disease usually results from autoimmunity: an attack on a part of the body by the body’s immune system. This shouldn’t happen. Our immune systems were built to attack invaders, such as invading viruses and bacteria. They were not built to attack us. But in autoimmune diseases such as Addison’s disease, something goes wrong.
Addison’s disease also can result from other conditions that damage the adrenal glands. This includes infections of the glands, bleeding into the glands or cancer that has spread to the glands.
However it is caused, Addison’s disease leads to extreme weakness, weight loss, low blood pressure, gastrointestinal problems and darkening of the skin. People said President Kennedy looked thin, pale and ill when he was young. Fortunately, he was diagnosed before his condition became life-threatening.
Diagnosing Addison’s disease can be challenging. It usually involves measuring the blood level of adrenal hormones (such as cortisol) and of brain hormones that stimulate the adrenal glands to produce adrenal hormones. CT scanning to check the size of the adrenal glands may also be used for diagnosis.
Treatment involves taking hormone pills for life. These pills replace the hormones that the adrenal glands are not making. For example, doctors may prescribe hydrocortisone or prednisone pills for glucocorticoid replacement. Androgens (such as DHEA or testosterone) may need to be taken as well.
It is important to avoid overreplacing these hormones. Doing so may cause side effects. The dose prescribed should be just enough to replace what the adrenal glands should have been making naturally — and no more.
People with Addison’s disease should wear a medical alert bracelet to notify medical professionals of their condition, and carry high doses of their medicine with them. That’s because people who become very sick (such as from an infection or a major injury) suddenly need more adrenal hormones than normal — and more than their usual daily dose. High doses of corticosteroids, in such situations, can save the life of a person with the disease.