This entry marks part five in a series on Hemochromatosis.
When I look back over the years, I sometimes shudder to think of the effrontery of some of my actions. Sitting in a motor home at Vancouver Airport years ago, I read a book called Bouvier: The Life and Times of Jackie O's Father, a paperback by Kathleen Bouvier. I was already riveted by the mention of some very provocative symptoms (one of which had given rise to the nickname Black Jack), but when I reached the end of the story and found that he had had liver problems, I was so concerned that –- then and there in the motor home — I wrote a letter to Mrs. Bouvier, care of the publisher, begging her to persuade Mrs. Kennedy to go for tests, and posted the letter, right there at the airport!
While researching this I came across a note on the Internet, which tells us that, “John Vernon Bouvier III (May 19, 1891, Easthampton, Massachusetts – August 3, 1957, New York, New York) was an American socialite and Wall Street stockbroker. He was the father of former First Lady Jacqueline Kennedy Onassis and Princess Lee Radziwill. His nickname was Black Jack, referring to his omnipresent dark tan and his flamboyant lifestyle.” As I did not receive a response to my letter, I have no way of knowing whether my concern was warranted or not!
Steve (Terrence Steven) McQueen
Another celebrity over whom I agonized was this blue-eyed, cool on the outside, but rebellious turmoil on the inside actor whom I had first seen in The Great Escape, a World War II epic, together with James Garner. His steely image had, according to the Internet, made him a box-office phenomenon the 1960s and early '70s. I was shocked to learn of his illness and, although we did not yet have television in South Africa at that stage, I followed his progress with great interest on the radio.
By the time he died of cardiac arrest at a clinic in Mexico in 1980, one day after undergoing surgery for cancer, he had gone from pillar to post trying to find a cure for what ailed him. By then he had tried various non-medical treatments, including the use of laetrile, a derivative of apricot kernels, and other procedures involving, among others, the injection of live cells from cows and sheep, coffee enemas, frequent shampoos and massage. I could never find confirmation of my conjectures and, before his death, he had given a medical interview in which he blamed his disease on exposure to asbestos. Only later would the cause of death be specified as Hemochromatosis.
That was another family about whom I became anxious, and because Margaux was exhibiting some very suspicious signs, I once more had the audacity to write to someone and, to this day, I wonder whether her erratic behaviour might have been caused by HH. She later committed suicide and I was to discover that other members of the immediate family including the father of Ernest, Clarence Hemingway, his siblings, Ursula and Leicester, and his granddaughter Margaux Hemingway had also done so. “Some believe,” we have since been informed, "that certain members of Hemingway's paternal line had a hereditary disease known as Hemochromatosis (bronze diabetes), in which an excess of iron concentration in the blood causes damage to the pancreas and also causes depression or instability in the cerebrum." (By the way, it can also cause impotence.)
We now know that Hemingway's father developed hemochromatosis in the years prior to his suicide at age fifty-nine, and it was common knowledge that this author, whom I studied closely, was a heavy drinker, succumbing to alcoholism in his later years. It was, however, the behaviour so well portrayed in two films that rang alarm bells for me. Take for example the behaviour of young Hemingway in Love and War with Sandra Bullock, and later in Hemingway vs. Callaghan, a gripping mini-series based on the true story of the friendship between Ernest Hemingway and Morley Callaghan between 1923 and 1929. Either I am paranoid or I what witnessed was behaviour very typical of some Hemochromatosis patients I have known.
Why, I would wonder, after seeing the James Dean movie East of Eden, did the mother wear gloves? Did her hands hurt? I knew from experience that novelists frequently base characters on people they have known personally, so could the writer’s own mother possibly have known the agony of the "painful handshake"? Of course there is no way I shall ever know, but since reading the moving book, The Other Side of Eden: Life With John Steinbeck by Nancy Steinbeck, there is no longer any need for speculation about the possibility of hemochromatosis. This book refers both to the famous American novelist as seen by his son, and to Nancy Steinbeck's life with the son, her late husband, John Steinbeck IV. In the introduction she explains that Steinbeck IV commenced his autobiography in 1990, and after his untimely death in 1991, she "needed to finish his manuscript for [their] family." Both the book and her Web site graciously provide links to the Canadian Hemochromatosis Society, with a reciprocal link on the society’s Web site.
She makes it very clear that her husband, John Steinbeck IV, son of the American author, died of "complications related to HH during back surgery." She provides the information that the first noticeable sign of his disease was that his skin turned a peculiar shade of green. “We were living in Kathmandu at the time, and often Tibetans would stop him in the street to compliment him on his bronze tan,” she says, adding, "'You look like one of us,' they would tease."
I have learned that her husband was tired all the time and that his joints ached. In due course he was diagnosed with cirrhosis of the liver. His gall bladder was removed due to severe abdominal pain, “but that”, as she has put it, “too can be a symptom of HH. Later, his doctor suspected Addison's disease. Only when the HH was full-blown did an internist diagnose John correctly. By then, it was too late to save his ravaged organs.”
She tells a heart-rending story. “From the onset, and as the disease progressed, our children and I struggled with John's mood swings, violent outbursts, severe depression and anger. We were never told that those symptoms, which mimic mental illness, were part of the disease, as was his inability to communicate, sleeplessness, loss of memory and emotional withdrawal.”
These symptoms, she reveals, have had profound and long lasting effects on their psyches, and that it would have been so much easier to work with his mental and emotional states had they known about the psychological effects of HH. What she found most confusing of all was the way these mood swings appeared after periods of relative calm.
Her husband developed diabetes in 1990 and died in 1991 during a back operation “because the doctor neglected to take his complicated medical history into account,” she maintains. “Due to the toll HH had taken on his body, John was never an appropriate candidate for surgery.” In her opinion the doctors chose to ignore the radiologist’s recommendation for further testing, and John Steinbeck 1V died on the operating table from a pulmonary embolism. To his wife, the final injustice was “a doctor who didn't have a clue about the severity of HH's effects.”
Fused Joints and Crippled Hands
How tragic it is that the careers of some hockey stars and other sportsmen I have known have come to an end because they had had their ankles and, in some cases, even their wrists fused, and this because the agony caused by HH was thought to have been the result of sports injuries.
For me, the memory of a consummate musician's hands, crippled by HH, is sometimes more than I can bear.