When Ed Wilson was three years old and already diagnosed with hemophilia, doctors told his parents he wouldn’t live past adolescence. “They could have wrapped me up in foam rubber or let me lead as normal a life as possible and accept the consequences,” he says. “People who don’t expect to live past 30 do what they want and have a good time.”
Wilson did just that, “living life to the fullest,” he says, vigorously pursuing action sports like sailing, car racing and flying, and enjoying a full social life. Now age 64, Wilson—who is past president of Hemophilia of North Carolina, one of the National Hemophilia Foundation’s chapters—still possesses that same energized, go-get-’em demeanor.
Wilson, a retired metalworking factory production manager, is not alone in his pursuit of a full life. A growing number of people with bleeding disorders, many of them also infected with HIV and hepatitis C, are living well into their 50s, 60s and beyond. While this is good news for them, many hospitals are underprepared to treat this influx of patients who have bleeding disorders and other disease brought on by age. Scant medical research or formal recommendations are available for men like Wilson, who’ve lived much longer than their doctors had ever expected.
Wilson, who lives in Tryon, North Carolina, remembers that 40 years ago a lot of people with hemophilia had a “live fast, die young, leave a good-looking-corpse attitude.” He recalls several friends who died in high school or in their early 20s.
But Wilson was spared. He jokingly credits the help of a “good fairy,” but also acknowledges the starring role that factor IX has played in his still-evolving lifetime drama. “It’s made a tremendous difference,” he says, and he still uses the product to infuse when he has a bleed.
In addition to hemophilia, Wilson also has hepatitis C. “But it hasn’t gone virulent,” he says. Unlike many of his peers who received contaminated blood products in the 1980s, Wilson was not infected with HIV. But he has had both ankles fused, and both knees and a hip replaced. He faithfully visits the hemophilia treatment center (HTC) at Wake Forest University Baptist Medical Center in Winston-Salem twice a year, “If I have something wrong with me, I want to know. I keep connected by getting a full checkup annually,” he says.
Over time, Wilson became an optimist. “After 30, I decided I might live long enough to make marriage a good idea. After 40, children. After 50, retirement. And after 60, grandchildren. I think it’s worthwhile to hang around for the duration.”
Dodging Multiple Bullets
Some 20 years ago, “hanging around” seemed an unlikely hope for many people with hemophilia. The bleeding disorders community was elated when the first cryoprecipitate and then antihemophilic factor were developed in the 1960s and 1970s. But when the nation’s blood supply became contaminated with HIV, more than half of the 17,000 Americans with hemophilia and 80% of those with severe hemophilia were infected. A large number subsequently died. This devastating loss was particularly tough for the bleeding disorders community that had waited so long for a treatment, only to realize that people were dying from the very medicine that was supposed to help them live longer.
Despite this setback, advances in antiretroviral medications helped the immune systems of many people suppress the HIV virus, allowing them to resume a more normal life. “Treatment is the best it’s ever been,” says Angela Lambing, MSN, RNCS, NP-C, nurse practitioner and coordinator of the Henry Ford Hospital Adult Hemophilia and Thrombosis Treatment Center in Detroit.
Lambing also is pleased with the success rate of treating patients with bleeding disorders with hepatitis C. “Some patients manage to fight off the virus,” she says. “Others carry it and have stable liver enzymes.”
Ray Stanhope is a good example of someone who’s lived with both viruses since the 1980s. This 51-year-old from Sugarland, Texas, and former engineer with Texas Instruments is now vice chair of the NHF Board of Directors. He encourages younger people to “really be the person who drives your care. Hemophilia has made me stand up for myself.”
Stanhope sees aging management as an evolutionary process. “As age-related issues pop up, you have to modify components of your life,” he says. “You start to wonder, ‘What part of my life am I not going to have to change? How much am I going to have to give up and is it all worth it?’” His solution: “I try to do things in moderation.” He carefully monitors his care in conjunction with his primary care provider, hepatologist and pain management specialist.
Stanhope warns others to be prepared to ask for help when they don’t receive the care they need, especially when things change. “When I first started going to my HTC, my orthopedist would automatically see me; now I have to make an appointment,” he says. Plus, he notes that some hematologists are getting away from prescribing pain management drugs, which means he has to find a different doctor with expertise in treating chronic pain.
[Steps for Living: The Pain Facts ]
As people with bleeding disorders and other health conditions get older, many find they must rely on themselves to make decisions. “Take a major role in your care,” advises John Lanzon, age 59, of Southfield, Michigan. “Know your disease and the ins and outs of treatment.”
Lanzon has been proactive under circumstances that would mystify most healthy people. He balances hemophilia, HIV, hypertension, type 2 diabetes, arthritis and cough variant asthma while undergoing dialysis for kidney failure. The HIV and hypertension have stressed his kidneys, he says, “and chronic bleeding probably doesn’t help. I’ve read that older guys with hemophilia are more subject to kidney shutdown.” He’s also had two joints replaced and one fused.
Lanzon’s wellness team includes his internist, hematologist, nurse practitioner, infectious disease specialist and orthopedist. “A lot of caregivers and medical staff still focus on bleeding prevention, as this is the first generation that actually aged with hemophilia,” he says. “Their focus could be more on what can go wrong, in addition to bleeding, as part of normal aging.”
A retired medical laboratory technologist, Lanzon says medical training is not a prerequisite to understanding one’s disease. “There is a lot to be said for being self-taught,” he suggests. For example, he warns that transient hematuria, or blood in the urine, can be common in people with hemophilia from time to time. “If you’re a smoker, it could signal bladder cancer,” he suggests. Stones can cause pain and bleeding into the urine. “The pain caused by a kidney stone can be mistaken for an abdominal bleed,” he says.
Lanzon recommends regular prostate cancer screenings if you’re over 50. “Coughing up blood, bleeding gums, and dark, tarry stools are other concerns,” he says. “It’s easy to write off these happenings as part of having a bleeding disorder while they may be masking another more serious problem.
“People with bleeding disorders face the same aging problems as others, and the bleeding tendency can aggravate these conditions or make them more difficult to deal with,” Lanzon continues. “Diabetics, for example, have problems with wound healing. A bleeding tendency can further complicate this. Coughing and wheezing brought on by asthmatic attacks can cause abdominal bleeding and bleeding in the muscles between the ribs.”
Lanzon warns that “chronic hypertension can make a person with hemophilia more prone to bleed into organs such as the kidneys, eyes (retinal hemorrhage) and the brain. Another frequent occurrence in men with hemophilia is hematospermia (bloody semen). While this is rarely a cancer symptom, it often causes alarm for those experiencing it.”
Finding (and Paying For) Quality Care
With so many potential complications to worry about, getting quality care in a timely manner is of paramount importance for 67-year-old Pete Johnson, who lives in Brigantine, New Jersey, and Harleysville, Pennsylvania. Johnson has hemophilia and HIV; recently he was diagnosed with hepatitis B. He’s also had a heart attack and wears a leg brace. Johnson is a firm believer that with quality insurance and good intervention, patients can lead a normal, long-term existence.
“Access to treatment centers is critical. I’ve always lived in or near a major city. I wouldn’t think of leaving a major market,” he says. He encourages others saying, “Go to a big, multi-disciplinary facility, work with a hematologist as the pitcher and you be the ball.”
Trying to understand your insurance options as you age is another concern, says Dana Kuhn, PhD, president of Patient Services, Inc., a nonprofit organization in Midlothian, Virginia. His company subsidizes the high cost of health insurance premiums and pharmacy co-payments for people with specific chronic illnesses and rare disorders.
“If we can ever straighten out Medicare Parts A, B, and D, that will be a good remedy for patients,” Kuhn says.
US residents become eligible for Medicare benefits when they turn 65. Some people with bleeding disorders whose condition is severe enough to qualify them as disabled under government guidelines can qualify for Medicare much sooner than that.
Within Medicare, Part A pays for hospitalization, Part B for major medical and Part D covers prescription drugs. Clotting factors and a few other self-administered drugs are covered by Part B, not Part D. Co-payments for the program can be high (sometimes costing thousands of dollars), and many chronically ill patients end up buying a secondary insurance policy to cover out-of-pocket costs.
Kuhn also warns that being on Medicare can make it harder to see specialists, especially if patients need drugs for hepatitis C or HIV. “Medicare impedes your access to who can be your provider of service, whether it’s those who deliver your factor concentrate or the physicians you visit.”
The Need For More Research
At the World Federation of Hemophilia’s 2006 World Congress in Vancouver, British Columbia, in May experts noted that the near-total absence of research on the links between hemophilia and the diseases of aging grows more acute as the number of older adults with hemophilia increases due to improved treatment, particularly in developed countries. It’s a double-edged medical sword.
Patients with hemophilia can now expect to live 77.6 years, explains Alice Cohen, MD, director of the Division of Hematology and Oncology at Newark Beth Israel Medical Center in New Jersey. “In the absence of a viral infection, a 1995 study showed the life expectancy of a patient with hemophilia is almost equal to that of the general male population.” Cohen also cites a 2000 Centers for Disease Control and Prevention study that evaluated causes of death in men with hemophilia in the mid-90s: HIV and AIDS were first, followed by stroke and myocardial infarction (heart attack), lung diseases and cancer.
“As HIV goes away because patients do well with new treatments or because their body has developed some mechanism of living with HIV, heart disease will become number one, followed by lung cancer, other cancers, then bleeding disorders,” Cohen predicts.
Ironically, “most people think of hemophilia as protecting people from vascular disease because it prevents blood clotting,” says Bruce Sussex, MD, of Memorial University of Newfoundland, Canada. He wants to see more research on hemophilia and heart disease.
Sussex points to a colleague’s study of a rural Newfoundland community that showed patients with hemophilia had higher average body mass index (a measure of weight in relation to height) and had higher incidences of diabetes, hypertension, and heart disease compared with a control group. People with mild hemophilia seem to exhibit higher rates of heart disease, Sussex says. He predicts this problem will increase as hemophilia management allows people with bleeding disorders to live longer.
Cohen reminds patients that the usual risk factors for heart disease—for people with or without hemophilia—include a family history of heart problems, smoking, high cholesterol, lack of exercise, diabetes and hypertension.
Looking to the Future
In lieu of much-needed research on hemophilia and aging, Lambing at the HTC in Detroit says planning ahead is of utmost importance for individuals. She insists on careful coordination of care, telling her patients, “If you ever go to the emergency department, you call me first.”
She wants to know when her aging patients undergo a recommended routine procedure such as a colonoscopy. “Routine surveillance testing is recommended. But it’s important to coordinate care with the treatment center so factor dosages can be recommended with follow-up monitoring.”
In many cases, this aging population understands more about hemophilia and its treatment than most healthcare providers, observes Barbara Konkle, MD, associate professor of medicine at the University of Pennsylvania and director of the Penn Comprehensive Hemophilia and Thrombosis Program in Philadelphia.
“They’ve been self-educating a long time and we respect that,” Konkle says. “They treat themselves at home and that gives them more ownership of the disease. Still, the deeper you get into the healthcare system, the more you need someone to guide you, especially when undergoing procedures.”
A comprehensive hemophilia team coordinates an older patient’s care with his or her internist and other specialists. “As the hemophilia population lives longer, we want to be sure they receive the needed preventive care that is best provided by an internist,” Konkle says.
All of these efforts are good news for aging patients like Wilson. His advice for younger patients is: “Life isn’t over because you have a permanent, somewhat debilitating disease,” he says. “If we older guys can do it, so can you. Don’t let this get you down.”