Hemingway’s Death and Hemochromatosis Awareness

by Stephen Cobb on January 10, 2016

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[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!

{ 29 comments… read them below or add one }

Aggie Baird July 2, 2011 at 4:58 pm

I am so grateful to my daughters Doctor in Vancouver…..she moved to North Van in 2009 and was dealing with very low energy and joint pain…..Cristi Doctor tested and continued to check when he found her iron levels to be a bit high he then took it a step further…..yes HHC was found after iron saturation, iron binding and

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Jeff Williams July 2, 2011 at 5:54 pm

Here’s my story: http://www.katu.com/news/medicalalert/124634899.html
It’s… the same old story..

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Pamela November 9, 2011 at 12:26 am

Please keep educating others about HH . I was diagnosed almost two years ago at age 42 while in iron overload with almost a 100% saturation level. I am grateful to a dear aunt, an R.N., who insisted I be tested for HH as her sister was diagnosed in her 60’s with non-alcohol related liver damage and HH. While my physicians at the time of my illness just seemed puzzled by my symptoms, this aunt’s intuition and insistence on testing prevented me from having any known organ damage. More importantly, I had my eldest son, age 13, tested (who has c/o joint issues since preschool) and he as well has HH. I know because of this early diagnosis that both of my sons will have the opportunity to lead a healthier life.

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Brenda Harper April 25, 2012 at 11:55 pm

I have a radio program on health and wellness. I am wondering if some from your organization would like to come on my program in July and discuss Hemochromatosis, to help educate the folks in Southern Idaho, the State where Mr. Hemingway now rests.

Brenda Harper

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Isabelle Bedell August 8, 2016 at 9:53 pm

Dear Ms Harper: I live in Maryland, where are your located. I would love to speak about Iron Overload. Perhaps we can talk via telephone and have it transmitted. Regards,
Isabelle Bedell.,MPH
Online Volunteer Coordinator for The IDI ExcessIron Support Group

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Mickie Moore August 5, 2012 at 6:46 am

My story unfortunately does not end well. My husband of 27 years passed away May 11, 2011 of what I firmly believe was complications of hemochromatosis. It was only after the fact that it dawned on me what could have been causing his years of symptoms. I remembered that when he was in his 20s, he donated blood regularly, until one time when he was told his blood was not acceptable anymore. I seem to remember that it was because his liver enzymes were up – hepatitis was mentioned (which he did not have) and he was told to be tested for hemochromatosis. He went to the doctor and passed the info along. The doc basically blew off the suggestion that it could be hemochromatosis saying that it was so rare he couldn’t possibly have it. Even said that the blood donation centers were know for making errors. I know now that he didn’t test for it. Instead, he began lecturing how my husband should quit drinking because he was probably an alcoholic. Fast forward 15 years – Rick began suffering bouts of depression and anxiety. He even had heart palpitations and started having joint pain. Alcohol was used to self medicate because he hated taking pills and was very sensitive to side effects. He went to numerous doctors and was told simply that he needed to quit drinking. He did quit for many months at a time. He would try to convince himself that he felt better, when in reality he was getting worse. He had full blood work done annually, but the test for hemochromatosis was never done. In the year prior to his death, the depression was almost unbearable. He now had painfully swollen toe and finger joints (diagnosed as gout or arthritis) and his toenails and fingernails turned yellow and crumbled. He had skin problems and vision problems. Again, he quit drinking for months but felt worse and worse. Finally alcohol was the only thing that relieved his pain. I believe his liver had begun failing up to a year prior to his death. His stomach was swollen and distended and he could not lose weight. He had been to a psychologist and psychiatrist for counseling, also to our family doctor and an internal medicine specialist. No one picked up on the disorder. The weekend before his death, I went out of town to watch our daughter play in the state golf tournament. He was feeling terrible, but convinced me that he would be fine until I got back. We kept in touch through texts and phone conversations. Of course, I was worried, but still believed the primary problem was the depression. He had never contemplated suicide and insisted he would never take his own life. He loved me and our three daughters too much. When we returned home, it was clear he had been drinking heavily for several days. He was relieved to see me, and said that now that I was home we could figure this thing out and get him well. I was angry but not surprised about the drinking, knowing that was the only relief he could get. That night Rick went to sleep – I was hopeful because I know he had not been able to sleep well, if at all, for days. The following morning, he was still sleeping (he had been up in the night to go to the bathroom). I left for an hour to run a few errands, and when I returned, I found him unresponsive. I had no idea how sick he was – the doctors had me convinced he was bipolar and depressed. There was no autopsy, only a toxicology study (wish I had insisted on an autopsy). The final determination on the death certificate was alcohol induced cirrhosis. It has been over a year. I am still angry with myself for not making this connection when we could have helped him. Incidentally, Rick’s younger sister was diagnosed with hemochromatosis a couple of months after his death, after the “light came on” so to speak, and I remembered his experience with the blood center. She is doing well, but donating blood frequently to keep it under control. I have also since found out from my own doctor that the condition can be masked and undetectable in a normal blood test. I have know others suffering with depression or other psychological disorders – my advice is always to exhaust all possible medical causes before taking antidepressants. I firmly believe that most depressive disorders are probably caused by an underlying condition. Just seems that doctors are quick to treat the symptoms and not so willing to truly make the extra effort it takes to discover the root of the problem.

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jana skinner February 12, 2013 at 9:48 am

I am gratful for this web-sight, living in Sun Valley ID I new Margo, Muffet, Mierriel, Jack…Ernist son and his wive Angela. Remember Margo saying she was hearing foices and was going to become ph
ycic before she moved to Santa Monica…..I dont know why this is such ahard thing for dr’s to dignost. If you know sonthing is wrong..keep searching untill they give you a ferritin count.

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Barbara February 19, 2013 at 12:17 pm

Well I have hemochromotosis as well. My twin sister has it with the diabetes so I wanted to be tested. Have been so exhausted lately and can sleep all day. Funny part is…my iron levels are very LOW!! Doctor thinks from colits attacks I get now and again. I have been looking up on computer and found as they put it the “Other Celtic Curse”. Its called celiac…..same symptoms as colitis…but cause low iron, vitamin 12, C etc. Going to call doctor to add this test to my next blood work..Freaky right?? Making alot of sense to me now.

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peter cope May 4, 2013 at 4:50 am

HH sufferers should also be aware, most doctors aren’t, that HH is strongly associated with stroke, as I found out to my cost in2006. I was fanatically fit, a marathon runer, but I stil suffered a massive stroke. My sister who is also HH takes precautionary aspirin daily. I don’t know why this isn’t standardpractice, the cause may be more specific than the atherosclerosis. There is some evidence that the HH gene interferes with the structure on th carotid arteries, If you want refererences email me.

peter cope

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rena beattie June 16, 2014 at 6:33 pm

My husband and i both have hh we discovered because our daughter was tested her ferrinton level was very high we both went to be tested and fould out we had it . our doctor knows this but never checks our iron overload. so much for us knowing. good luck to all rena

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Steve September 23, 2014 at 12:46 am

All I can say is HH sucks. My entire body is racked because of it – from my toes to my head. I get phlebotomies every 6 weeks now BUT the damage done by excess iron being stored in my body – over 55 years is done. My endcocrine system is screwed up, my liver, my kidneys, my pancreas, my spleen – name a body part and I can guarantee you IT IS EFFECTED. I have arthritis, Gout, Crohns, Diabetes, Hypertension, Hypothyroidism, several Aneurysms, spinal stenosis, Carpal Tunnel in BOTH wrists, peripheral neuropathy in hands, fingers and balls of my feet including all of my toes, severe hearing loss, mood swings, blood tests that go everywhere and other symptoms that make me appear physically fit and most days I am literally screaming inside.

I have more doctors taking care of me than carter has liver pills. I have a Neurologist, a Neruosurgeon, a Gastroenterologist, an Endocrinologist, a Hemotologist, a Podiatrist, a Vascular Surgeon, an Orthopedist, a Dermatologist, a Nephrologist, an ENT, an Opthamologist and lastly I believe – my family physician – lol. I also use my local hospital when I get my 6 week phlebotomies.

So far about the only thing I have not needed – a Psychiatrist.

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Kathleen Marsh October 7, 2014 at 3:58 am

I have just read this with great interest. I will pass this on to my family and others as I think HH is a very neglected illness here in the UK. Not many people have heard of it or the associated problems that go with it and I having been diagnosed with the same is grateful to all who raise awareness of this horrible disease.

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Jean November 5, 2014 at 1:01 pm

My husband died 09/08/2014 original diagnosed with liver disease due to alcohol which then the VA told us to following up with his gastroenterology – I fought to the bitter end – My husband was adopted and we had no medical history on him so I insisted that they run genetic testing and if they tell me is was liver disease due to alcohol then ok – after several months back and forth the results came back as having Hemochromatosis by that time it was to late we had one treatment of pulling blood every 2 weeks on the second treatment 08/05/2014 he ended up in the hospital and died of sepsis – liver disease due to hemochromatosis

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Stephen Cobb June 27, 2015 at 6:11 pm

Thank you so much for sharing your story. So sorry to hear of your loss…tragically, there are too many cases like this. There seem to be very few funds for research into the condition and an appalling lack of education in the medical community. (I also apologize that this comment did not appear before – we had a problem with the software).

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Susan hartman July 27, 2015 at 11:26 am

Is HHC related to porphyria? Sounds like some same symptoms. My mother has Porphyria and first thing they did was drain massave amounts of blood, her uncle had it and went nuts. Please let me know if I should talk to my doc about HHC

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Merna July 27, 2015 at 11:08 pm

My entire family has suffered with depression, Diabeties, suicide and congestive heart failure. I was diagnosed with genetic Hemachromatosis 15 yrs. ago. I researched foods to avoid and food to add to diet that get rid of iron i.e. yellow peppers I also gave up all alcohol, cigarettes and meat. So far, so good!

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Sally July 30, 2015 at 11:02 pm

I was diagnosed 3 years ago age 36 by sheer luck! After getting neck, shoulder and associated hand pains I insisted on seeing a neurologist who was so very thorough she picked it up. My ferritin was 4500! I owe her my liver it seems, haematologist told me I’d of likely needed a new one by 50 otherwise. My brother has since been diagnosed too. Our mother passed away very suddenly aged 45 in early 1990s of heart attack…haematologist seems to think it highly likely (although we’ll never know) it was down to the Haemachromotosis.

So glad I insisted on seeing a specialist otherwise I’d be none the wiser! They should screen for it in my view.

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Larry May 14, 2016 at 4:32 pm

I have hemachromatosis and discovered it by an astute doctor about 1998. I would be dead now if he hadn’t tested me for high iron. I am sure that is what killed my grandfather and father almost no doubt in my mind. Mine seemed to be in submission for about seven years as I have it checked at least every 4 months. Now it seems to be back as it has been creeping up now for the last 2 tests. Probably going to go back to phelbs now hopefully will knock the ole iron level down again.

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Teresa July 15, 2016 at 11:08 pm

It sounds crazy, but I am hoping I have Hemochromatosis as I have been suffering for 20 years miserably with all kinds of health problems. Actually since childhood. Recently my doctor found very high Ferrin levels which is very suspicious cause they have always been just barely average. I am waiting for results from the 23 and me to come back. 7 more weeks. All four of my grandparents died of heart related problems, heart failure, congestive heart failure, a heart attack and stroke. My grandfather that died from heart failure was waiting for exploratory surgery and always had had a super large stomach. I don’t remember anyone having liver problems though. Wish me luck.

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merna July 28, 2016 at 10:43 am

Does anyone one with HH have recurring bladder infections?

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Megan McCarthy September 12, 2016 at 1:22 pm

Hello. I have HH and have had recurring UTIs for years. I had a number of surgeries to correct problems. My uro/gyn dr at UW told me about a supplement called Ellura. It is the only bladder product with testing behind it. It is expensive but nothing compared to the pain, misery, and expense of UTIs. It is only available online and you get a 50% discount on your first order. I have not had a recurrence in the 8 months that I have been taking it.
Good luck!

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kent September 1, 2016 at 3:34 pm

My wife has hereditary hemochromatosis…. She nearly died in 2007. Her doctor called me at 7:00 one evening to tell me her diagnosis. My wife was at a point she said she didn’t want anymore hospital/medical test….. she just wanted to visit her Mom. I got the call when she was out of state…. she started treatment then. I think.. we caught it just in time… Thank you Dr. Nutt. And…… thanks for the call that night. Heck, that’s the only time a doctor ever called me back…..and at 7:00 pm. Dang. My wife gives blood every 6 weeks. Strange… I had my Red Cross Gallon Pin years ago… she was squeamish.. and said she could never give blood. I love her…. :)

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Connie September 2, 2016 at 4:02 pm

After years of high ferratin levels I was tested to have the H63D gene mutation in 2011. After rising ferratin levels, my doctor recently referred me to a Hematologist who told me that the H63D rarely causes iron overload and that I need to change my diet..which is extremely healthy..mostly fresh fruit and veg with very minimal gluten, grains, sugar and dairy. Where can I find more information about the H63D gene mutation? My maternal grandmother had liver issues though never drank alcohol ever.

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Stephen Cobb March 11, 2017 at 6:22 pm

Connie – well yes, hemochromatosis-induced iron overload is rarer in H63D folks, but ask your doctor “Since when did rare=never?” They have to accept that if you have this defect it can mess with your iron processing. Doctors spent 20 years telling me I should eat more bananas and less salt to lower my blood pressure when all along it was an adrenal malfunction, which no amount of diet and exercise would have fixed. Meanwhile my heart was getting increasingly damaged. Anyway, here is an H63D study that might help, but you are fighting against ignorance about HH in general https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4071918/. Here is another http://www.irondisorders.org/Websites/idi/files/Content/854268/ST%20H63D%20APR%202010.pdf.

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Floyd Longwell November 30, 2016 at 4:39 pm

http://celticcurse.org/hemingways-death-and-hemochromatosis-awareness/

I just watched the new movie “Papa” about Hemingway and it got me thinkin about their family history of suicide which brought me here.

You forgot to mention his daughter, Margaux, who also killed herself.

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Pat December 15, 2016 at 12:18 pm

It’s pretty close to criminal that there is not more awareness about a disease that affects such a large percentage of the population. I have it – the full blown homozygous C282Y mutation – only found out because my brother got tested based on his high iron levels. My sister has it, too.
One pathetic thing about hereditary hemochromatosis is that it’s so easy to treat when caught early.
The other pathetic thing is the lack of awareness….my primary care physician didn’t even know about hemochromatosis or the C282Y mutation and was afraid to order tests for it. I went to a gastroenterologist to get the test.
That worked okau for me since I have a PPO – but what about someone who needs a referral? There really needs to be more public – but more importantly, professional awareness.

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Helen December 23, 2016 at 6:10 am

merna , my daughter is 29 was diagnosed about 5 years ago, and she has problems with recurring bladder infections also. I hadn’t thought about the connection with her HH and that issue. If you learn any more, please let me know!

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James January 19, 2017 at 5:29 am

After nearly sixteen years. Suffering from depression, joint pain, impotence, low moods, unable to sleep. (Level nearly at 5000 when they are only meant to be 50).

I was finally diagnosed with Hemachromatosis.

My question to all the good Doctors and Consultants out there is this:-

Why did it take sixteen years before I was tested for the above?

Are there no regulations in place to check people with depression and other long lasting illness for the above disease?

How many people are walking round this world with the disease above who have been wrongly diagnosed with some other illness?

My levels are now down to 2000 after being vi-asection once a week for the last 10 months.

I am already feeling a lot better and no longer have to take anti-depressants which in my opinion were wrongly subscribed to me.

Looking forward to your reply with interest.

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Stephen Cobb March 11, 2017 at 6:00 pm

James – many thanks for sharing, and so glad to hear things are improving in some areas. Apologies for delayed response but I find it hard to get the time for everything I am doing these days. You raise excellent questions. In your case the 16 years is longer than the average diagnosis time, but that average is still a shocking 9 years if i am remembering the stats correctly. According to a fairly typical primary care doctor I spoke with, medical students get very little education on hemochromatosis and are told it is very rare. So, naturally, they rarely suspect it. And it rarely gets diagnosed and that feeds the FALSE notion that this is rare. It is not rare if 1 in 250 white Americans have the genetic defect. Personally, I think anyone with Irish or northern European roots would do well to get the HFE test to at least know if they are a carrier (I know people fear asking their doctor for genetic tests, but you can get the test without a prescription). Then if they get the symptoms they can say to the doctor – what about this Hereditary Hemochromatosis? Ironically, at that point many doctors get excited because – hey, I diagnosed a rare disease AND we have treatment! So, please hang in there, and keep asking questions.

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