[This is a refresh of an article originally written in 2011. We wanted to update some of the information about genetic testing.]
Ernest Hemingway, one of America’s greatest writers, died from hereditary hemochromatosis on July 2, 1961. He was one of many Hemingways who succumbed to America’s most prevalent genetic killer, a condition that is treatable if detected early. Yes, you were probably taught that Hemingway died of a self-inflicted gunshot wound, but hereditary hemochromatosis–also known as HH, iron overload, bronze diabetes and Celtic Curse–was undoubtedly the underlying cause of his death. Depression and suicide are closely associated with hemochromatosis, as is the diabetes that afflicted Hemingway, along with his liver problems and high blood pressure.
Tragically, like many people who suffer from hemochromatosis, Hemingway did not know he had the condition until close to his death, too late for treatment to reverse the damage to his health caused by toxic iron accumulation. Indeed, the world did not know of his diagnosis until 1991 when his medical records were discovered (see: Hemingway: the Postwar Years and the Posthumous Novels, by Rose Marie Burwell, 1996, New York: Cambridge UP). Doctors made the diagnosis in January, 1961, six months before his death in July.
Quite by coincidence, July is Hemochromatosis Awareness Month in America, a time to raise awareness of what we now know is the most common genetic killer in America. (This article was originally published in July of 2011). By raising awareness of HH you can quite literally save lives. And if a giant of literature can help raise HH awareness, so be it.
How did hemochromatosis kill Hemingway? By causing toxic levels of iron to accumulate in his joints and organs bringing pain, diabetes, cirrhosis of the liver, heart disease, loss of libido, and depression. That depression is more than just being unhappy because your body is damaged and your health is failing. That toxic iron accumulation plays its own role in affecting mood and brain function. Sadly, suicide is an all-too-common outcome of undiagnosed hemochromatosis.
Back in the 1950s, when Ernest Hemingway’s health really started to deteriorate, doctors did not have direct evidence that hemochromatosis was genetic, although some doctors were sure that it was an inherited condition. The genetic connection was made in 1996 through research on the HFE gene. This discovery led to a genetic test for the condition and doctors quickly discovered that HH was a much more common condition than previously thought.
How common is inherited hemochromatosis? Calculations based on field research vary but 1 in 200 Americans is probably a good estimate. Very few potentially fatal genetic conditions, if any, come close to that level of occurrence. Historically the frequency of HH is higher in populations with an Irish or Celtic connection (the condition is referred to as hereditary hemochromatosis in America, but in Ireland, Scotland, and other parts of Europe, the condition is called genetic haemochromatosis).
Of course, after President Obama’s recent visit with relatives in Ireland, it would be a mistake to characterize HHC as a white or Caucasian or Northern European condition. You can have HH in your genes pretty much regardless of your physical appearance.
Sadly, the Hemingway family history is a classic saga of HH. Ernest Hemingway’s father Clarence committed suicide, as had his father before him. Ernest Hemingway’s sister Ursula, and his brother Leicester, also committed suicide. The father of Hemingway’s first wife, Hadley, committed suicide. From what we now know of genetics and hemochromatosis, all of them would have had hereditary hemochromatosis. Gregory Hemingway, later Gloria Hemingway, died a classic hemochromatosis death from hypertension and cardiovascular disease.
Fortunately, it doesn’t have to be this way. Hemochromatosis can be treated and its effects limited and controlled. Treatment of HH is not only FREE but HH gives back to society in a way no other disease does! That’s because the treatment for excess iron is to take blood (that is, give blood from the perspective of the hemochromatosis patient being treated).
As for detection and diagnosis, a genetic test that can reveal your HFE gene status is available for less than $200. A good example is the 23andme service that provides a lot of medical and ancestral information (getting your HFE status takes a few steps, as described here). Of course, you may be able to get tested for less if you have a sympathetic doctor who will order the test and you have insurance coverage.
Simple blood tests that can detect elevated iron levels cost just a few dollars. If you have any reason to think you might have HH, including a family history of liver problem, you should ask your doctor to order these tests (Fasting Serum Iron, TIBC or UIBC, and Serum Ferritin). If the results are described as “a little high, but nothing to worry about” push for more information and show your doctor this chart. created by doctors specializing in hemochromatosis.
Acceptable reasons include any of the symptoms of HH and/or a family history of liver disease, depression, suicide, joint pain, or diabetes. (And don’t let anyone tell you that cirrhosis of the liver is always caused by alcohol, or that your relatives with liver problems are/were heavy drinkers; this story reported in the Washington Post proves otherwise.)
Ernest Hemingway was a giant of American arts and letters. The Winner of a Pulitzer for his fiction, he is one of just five Americans who were among the first 50 winners of the Nobel Prize in Literature. His life and writings continue to inspire and inform people around the world. Now, in the story of his death, his legacy can include saving lives by spreading the word of this treatable but widely under-diagnosed condition, hereditary hemochromatosis. Don’t let ignorance of this treatable condition rob us of another great author!