John F. Kennedy suffered from Addison’s disease, in which the cortex of the adrenal glands atrophy, resulting in life-threatening deficiencies of Cortisol and aldosterone. Up until 1940, Addison’s disease was invariably fatal, but it was then discovered that it could be treated with cortisone. Kennedy was diagnosed by a London doctor in 1947, at a time when cortisone was an extremely expensive drug: the Kennedys were said to keep quantities of the drug in safety deposit boxes around the world. Had Kennedy not been a rich man, he would not have survived, because cheaper, synthetic versions of cortisone weren’t widely available until the 1950s. As it was, he was so ill that after the diagnosis he was told that he had only a year to live and was actually given the last rites on his return sea voyage to the States.
Kennedy also suffered from serious back problems and was in so much pain that in 1954, when he was a US Senator, surgery became necessary. This can be an extremely hazardous undertaking in someone with Addison’s disease. He pulled through, but with many complications, and repeat surgery was needed. So unusual was his survival that it was written up as an anonymous case report in the Journal of the American Medical Association in 1955. No one knew that ’37-year-old male’ was in fact John F. Kennedy until 1967, four years after his death. He also looked much healthier than he was because, ironically, another feature of Addison’s disease is a bronzing of the skin caused by abnormal synthesis of melanin pigment in the skin.
When he was standing for President in i960, his campaign team flatly denied that he had Addison’s disease. They relied on a very narrow definition of Addison’s as a disease only caused by tuberculosis of the adrenals, to quash rumours of ill-health. Tuberculosis was about the only disease Kennedy didn’t have. Like Franklin D. Roosevelt before him, Kennedy managed to overcome debilitating health problems to become one of America’s greatest presidents.
The novelist Jane Austen, on the other hand, almost certainly did have TB-induced Addison’s disease. In an article in the British Medical journal in July 1964, Sir Zachary Cope pointed out that Jane Austen had herself identified a key feature of the disease in a letter to her brother of 23 March 1817: ‘Recovering my looks a little, which have been bad enough, black and white and every wrong colour.’ ‘There is no disease’, wrote Sir Zachary, ‘other than Addison’s disease that could present a face that was “black and white” and at the same time give rise to the other symptoms described in her letters.’
You can do without ovaries, thyroid, pancreas – in fact most endocrine organs – provided that you receive the appropriate hormone supplements. But there is one exception. Remove both adrenal glands and you will die very quickly. There is no substitute, no way of mimicking the extraordinary second-by-second adjustments that the stress hormones of the adrenal glands make to crucial body functions. Without these glands, there is no recovery from physical or emotional trauma, no proper control of blood pressure, water balance or heart rate.
Complete loss of an endocrine gland is an extreme cause of having too little of a hormone. There are many other causes of deficiency – or excess. There are many steps involved in the production of a hormone, so insufficiency is not simply about not having enough of the hormone itself. Faults in any part of the chain might be the problem: in the hypothalamus, by there not being enough releasing hormone; in the pituitary, by failure of its hormones; by faulty production in the site of hormone manufacture; by poorly functioning or absent receptors or failure of the feedback systems, and so on, right down the chain.