Hemingway's Death and Hemochromatosis Awareness

Image of Ernest Hemingway
Ernest Hemingway, one of America's greatest writers, died from hereditary hemochromatosis on July 2, 1961. He was one of a number of Hemingways who succumbed to America's most prevalent genetic killer, a condition that is remarkably cheap and easy to treat, and may do very little damage if detected early enough.

If you're like me, you were taught in school that Hemingway died of a self-inflicted gunshot wound. However, it is clear to many that the underlying cause of his death was iron overload due to hereditary hemochromatosis or HHC, also known as bronze diabetes and Celtic Curse. Why? Hemingway suffered from classic iron overload issues, like liver problems, heart disease, diabetes. Depression and suicide are both associated with hemochromatosis (numerous people on his family tree committed suicide).

[The first version of this article was written by Stephen Cobb in 2011. It was revised in 2016 and refreshed more recently to mark World Haemochromatosis Week 2020, June 1-7. Note that in some parts of the world hemochromatosis is spelled with an extra "a" in it.]

Like so many people afflicted by iron overload due to hemochromatosis, Ernest Hemingway did not learn that he had the condition until close to his death. By then it was too late for treatment to reverse the damage to his health caused by toxic iron accumulation.

Indeed, the world did not know of Hemingway's hemochromatosis diagnosis until 1991 when his medical records were discovered. Doctors made the diagnosis in January, 1961, six months before his death in July (see: Hemingway: the Postwar Years and the Posthumous Novels, by Rose Marie Burwell, 1996, New York: Cambridge UP).

I know from my partner's experience that getting a diagnosis of iron overload due to hereditary hemochromatosis can be life-changing, for patients and their loved ones (I wrote about what it meant to me in this 2008 article: What Am I Thankful For? A diagnosis of hemochromatosis).

First of all, the diagnosis may explain a lot of the patient's current symptoms, a welcome relief to someone who has experienced a string of doctors intimating that some or all of these symptoms were "in your head" (a refrain all too familiar to female patients).

Secondly, the medical history of the patient's family starts to make a lot more sense. For example, iron overload can damage the liver in ways that appear similar to the effects of excessive alcohol consumption. After her father was hospitalized with liver problems, my wife was told that her father must be a secret drinker (he was not, but he did have hereditary hemochromatosis).

Thirdly, the patient can inform relatives who are at risk of developing iron overload due to this inherited genetic defect. Steps can then be taken, such as blood tests to check for elevated iron levels, phlebotomies if needed to reduce iron levels, and adjustments to diet and lifestyle that will help stave off ill effects. 

Fortunately, there is a way to reduce the number of people who suffer and die due to hemochromatosis: make sure the world knows about this problem. Raising awareness of HHC does actually save lives. And if a giant of literature can help raise HHC awareness, so be it.

So 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 HHC 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 HHC 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 purely white or Caucasian or Northern European condition. You can have HHC in your genes pretty much regardless of your physical appearance.

Sadly, the Hemingway family history is a classic saga of HHC. 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 HHC is not only FREE but HHC 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. One example is the 23andme service that provides a lot of medical and ancestral information. 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 HHC, 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 for getting tested include any of the symptoms of HHC 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!

Comments

Popular posts from this blog

Blood Variance and Hemochromatosis: Iron News from the Iron Disorders Institute

HFE testing: an open letter to the American College of Medical Genetics and Genomics