Jan 10 2017

An injection of optimism into biomedical research – Part 3: From deadly to chronic

by at 11:09 am under General

The first two parts of my Injection of Optimism into Biomedical Research series can be found here and here.

This part concerns a disease that was once considered deadly and is now considered generally chronic, Addision’s disease. Though somewhat obscure, this disease has affected many, including several noteworthy individuals. A more recent example of the deadly to chronic improvement process is HIV/AIDS, as increasing evidence is accumulating that the life expectancy for infected individuals in the developed world is approaching that of uninfected individuals – for example, see here and here.

Addison’s disease, first discovered in 1849, refers to reduced levels of adrenal hormones – such as cortisol – due to damage of the adrenal glands, usually of autoimmune origin, but also possibly due to a variety of other ailments such as tuberculosis and cancer. It is estimated to affect slightly more than 1 in 10,000 individuals. It can lead to low blood pressure, fatigue, weakness, and pain, with loss of consciousness and death possibly occurring during the severe “adrenal crises.” Before the introduction of glucocorticoid replacement therapy in 1938, this disease had a 2 year mortality rate of 80% and a 5 year mortality rate of 100% from the time of diagnosis. After the synthesis of hydrocortisone in the late 1940s, outcomes truly started turning around, although morbidity and mortality are still higher than in the general population, partly due to the side effects of chronic glucocorticoid use.

As an example of how the course of this once deadly disease was changed, consider two famous sufferers: Elizabeth Catez, a young French Carmelite nun and mystic, recently canonized as Saint Elizabeth of the Trinity, and President John F. Kennedy. Elizabeth died in 1906 at the age of 26 after being diagnosed with Addison’s three years prior. Her experience with the disease, especially the later stages, was so excruciating that it led her to develop a “mystical theology of suffering” and to write that Christ “wishes me to be the surrogate human being in whom he can suffer again for the glory of the Father and the salvation of the Church. This thought makes me so happy.”

The improvements made in disease management in the decades after Elizabeth’s death enabled JFK to keep his disease under control via the use of glucocorticoid treatment. There was much speculation during his life and his physicians always failed to either confirm or deny that he suffered from Addison’s disease. In 1967, after the president’s tragic 1963 assassination, Dr. John Nichols of the University of Kansas Medical Center provided evidence for the conclusion that JFK not only probably suffered from Addison’s, but had in fact also been case No. 3 in a 1955 study on the management of Addison’s disease during surgery. This was confirmed in 1992 when JAMA was able to establish this on the record with both hospital officials and the lead author of the 1955 paper.

Current treatment for Addison’s still relies on replacing the missing hormones, for example by substituting cortisol by hydrocortisone, prednisone, or dexamethasone, along with possible dietary interventions and the management of potential side effects of corticosteroid treatment, such as osteoporosis. Adrenal crises require immediate intervention and often involve giving the hormones as injections. Guidelines also exist for managing specific problems, such as surgery, illness or injury, and pregnancy, enabling those affected to live a much fuller life than was ever possible before.

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