“Now, I think that I should have known that he was magic all along. I did know it-but I should have guessed that it would have been too much to ask to grow old with and see our children grow up together. So now, he is a legend, when he would have preferred to be a man.” -Jackie Kennedy
Addison’s disease is a rare but known disorder (John Fitzgerald Kennedy suffered from it) and it is also referred to as adrenal insufficiency. Adrenal insufficiency develops when adrenal glands do not produce enough of the hormone cortisol. This can happen due to a problem within the adrenals (called Addison’s disease or primary adrenal insufficiency) or due to a problem with ACTH, the signal the brain sends to the adrenals instructing them to make cortisol (called secondary adrenal insufficiency).
An important distinction for patients is that people with primary adrenal insufficiency (Addison’s disease) usually don’t make enough of the hormone aldosterone too; so in addition to taking cortisol replacement, they also need aldosterone replacement. Also, ACTH levels are elevated. People with secondary adrenal insufficiency are only low on cortisol.
Causes: Primary adrenal insufficiency - the outer layer of the adrenal glands is called the adrenal cortex. If the cortex is damaged, it may not be able to produce enough cortisol. A common cause of primary adrenal insufficiency is an autoimmune disease that causes the immune system to attack the adrenal gland(s). There are some very rare syndromes (several diseases that occur together) that can cause autoimmune adrenal insufficiency, such as Autoimmune Polyendocrine Syndrome (type 1 or type 2). Other causes include: amyloidosis; bleeding into the adrenal glands; cancer that spreads to the adrenal glands; infections (bacterial, fungal, tuberculosis) of the adrenal glands; surgical removal of the adrenal glands. Secondary adrenal insufficiency - it is caused by a lack of ACTH, made in the pituitary gland. By far the most common cause of this type of adrenal insufficiency is chronic steroid use.
Signs and symptoms:
Low blood pressure
Increased pigmentation of the skin, including areas not exposed to the sun (due to high levels of ACTH in primary adrenal insufficiency. This occurs because melanocyte-stimulating hormone (MSH) and ACTH share the same precursor molecule, pro-opiomelanocortin (POMC))
Salt craving (due to low levels of aldosterone in primary adrenal insufficiency)
Treatment: it involves replacing the cortisol and/or aldosterone that the body is not able to produce, or that it secretes in an insufficient quantity. Cortisol is replaced with an oral synthetic glucocorticoid. The drug is taken one or two times each day. Aldosterone is replaced with an oral mineralcorticoid.
“My brother really was smitten with her in the beginning when he first met her at dinner. Members of the family knew straight away that she was very special to him, and saw the developing of their relationship. I remember her coming up to Cape Cod at that time and involving herself in the life of the family. He was fascinated by her intelligence: they read together, painted together, enjoyed good conversation together and walks together.”