Mommy, if you die can I go with you?
The holiday season of 1979 was less than merry for Steve and Charlotte Harkness. On Thanksgiving, Charlotte nursed an extremely sore neck gland that had failed to return to normal size after her late-summer bout with the "mumps.' She attributed her stiffness, fatigue and discomfort to the demands of mothering a lively pair of two-year-olds-- her natural son, Keith, and her adopted daughter, Jill.
The week before Christmas, all personal concerns were dropped completely. A late-night phone call brought the news that Steve's brother-in-law had been killed in a coal-mining accident in southern Indiana. By dawn, the two Harkness toddlers had been shuttled off to the homes of friends, and Steve and Charlotte were speeding toward Oakland City.
On Christmas Day, Charlotte, back home with the two children, felt certain she was "coming down' with the flu. Steve was still helping his relatives adjust, so Charlotte, hoping to ward off the virus, went to bed early. She awakened during the night, however, and was so faint that she could barely wobble from the bed to the bathroom and back.
When Charlotte Harkness reopened her eyes early on the morning of December 26, she knew that something was terribly wrong, but she could not have guessed that the events of the next few hours would so dramatically alter her life. Charlotte was experiencing the first symptoms of an insidious liver ailment called primary biliary cirrhosis, and her often-to-be-called-upon courage was getting its first big test.
"I was so weak that I couldn't sit up,' Charlotte recalls, "and the nausea was incapacitating. Luckily, the phone was within reach, so I frantically called a friend to come pick up the kids.' Charlotte had settled back in bed for less than ten minutes when she got the scare of a lifetime. "There's no delicate way to describe what happened,' she says. "I began, literally, to heave blood all over the sheets-- huge, dark puddles of blood.'
Fortunately, Charlotte was able to call her family doctor. He told her to call an ambulance immediately. Fearful that she wouldn't be able to get to the door to let in an ambulance crew, she instead dialed a neighbor who had a house key. "After that,' she recalls, "all I remember is being carried in a blanket to my neighbors' van. The next thing I knew, I was in a hospital trauma room. Hours later, I remember opening my eyes in intensive care and seeing Steve standing over me.'
Charlotte Harkness' nightmarish holiday was over, but her ordeal as a victim of liver disease was just beginning. At only 31, she was one of the youngest women ever diagnosed with the disease known in medical circles simply as PBC.
"Looking back,' Charlotte says today, "I sometimes wonder if I could go through it all again. Yet I know there must be hundreds of people just like me-- many with diseases with different names--who need to know that they can continue to enjoy life despite necessary medications and treatments. They need to know that life needn't come to a halt just because of an unexpected diagnosis.'
For Charlotte, the eventual diagnosis actually came as a relief. "From that bleak December of 1979 until the following June, we endured so much,' she explains. "All of the uncertainty was almost more scary than the ultimate knowledge that I had PBC.'
One night a week after being dismissed from the hospital, for instance, Charlotte woke up, pained by the same enlarged neck gland that had plagued her the previous autumn. "In the wee hours of that particular night, I finally cried,' Charlotte confesses, "but still more out of frustration than fear.'
Early the next morning, the gastroenterologist monitoring Charlotte's liver condition arranged for her to see an ENT specialist; the following day, Charlotte was in surgery as doctors looked for a cancer called Hodgkin's disease.
"I felt such relief when the tests came back negative,' Charlotte says, "but my life was still in shambles. Here I was, in and out of the hospital, with a husband and two little kids at home, and no one seemingly able to tell me what was wrong. Meanwhile, my third miscarriage and a neighbor's fatal accident only added to our misery.'
Finally, in June 1980 Charlotte got some long awaited answers. "My gastroenterologist called Steve and me in together and told us that he was almost certain that I had PBC. Once we had bombarded him with questions about the nature of the disease, we knew the news wasn't good,' she admits, "but he did tell us to consider a new drug being studied at Mayo Clinic that might slow the progression of the illness.'
After careful thought, Charlotte decided the drug-study program at Mayo meant she could play a role in her struggle for survival. In August 1981, therefore, Charlotte Harkness made her first trip to the Mayo Clinic in Rochester, Minnesota. She underwent four days of rigorous examinations and learned a great deal about primary biliary cirrhosis.
A chronic disease most frequently afflicting postmenopausal women, PBC gradually destroys certain bile ducts within the liver. The symptoms are varied and often late to appear; severe itching (pruritis) and fatigue are among the most common. "Eventually,' Charlotte explains, "the scarring affects the liver itself and leads to cirrhosis, a condition that prevents the liver from functioning normally and inhibits blood flow. Too often, people think of cirrhosis as an alcoholic's disease, but many liver disorders can lead to this serious deterioration.'
Charlotte was diagnosed at Mayo as entering the third stage of the fourstage disease. She became a participant in a federally funded drug-study program of D-penicillamine, a medication used to reduce the body's level of copper. (PBC patients can't adequately metabolize copper.) Charlotte was also placed on a disciplined, low-copper diet, which denied her such common delicacies as chocolate, tea, syrup, nuts, beans, shellfish, mushrooms and coconut.
In April 1982, Charlotte returned to Mayo for her first six-month checkup. Doctors assured her she was holding her own. By her November visit, however, her difficulties were again multiplying. Since summer, she had experienced occasional upper-gastrointestinal bleeding, two major gall-bladder attacks and daily nausea and cramping. At Mayo, doctors theorized that the enlarged liver had caused the gall bladder to become disengaged, a theory that explained why they couldn't locate it even with ultrasound.
Since travel was out of the question, a Mayo surgeon removed Charlotte's gall bladder. Shriveled and knotted with stones, it was the obvious culprit in her most recent discomfort. Her primary disease had also progressed, however--Charlotte was teetering between the third and fourth stages of PBC.
The following month, Charlotte's local gastroenterologist decided to schedule her for plasmapheresis, a plasma-exchange process that separates red blood cells and plasma, filters out the green, bileinfected plasma of the liver patient and replaces it with clear, normal plasma. The pheresis procedure takes about two hours and costs approximately $1,000 per treatment. It relieved Charlotte's chronic itching but caused her additional concern about "a new and unwelcome feeling of dependency on a machine.'
In November 1983, Charlotte again returned to Mayo, her skin blistered from a reaction to the D-penicillamine and her itching more persistent than ever because she had weaned herself from the pheresis machine. Doctors told her that her routine liver biopsy was no longer necessary now that she was an advanced PBC patient. Because of her side effects, she was also retired from the D-penicillamine study program.
Bouts with mild internal bleeding marred Charlotte's spring season, and by late summer 1984, she was openly worried about the general state of her health. Her liverfunction scores had been climbing for several months and the stubborn seepage problem had kept her hemoglobin count low. As she feared, her visit to Mayo in August was not a pleasant one.
"My doctor confirmed that I'm now in the fourth stage of PBC,' Charlotte explains, "and he admitted that he was "alarmed' by my rate of progression. For the first time, the need for an eventual liver transplant was seriously discussed--and as an indication of the gravity of things, when my life expectancy without a transplant was predicted, my husband literally darted from the room.'
Charlotte can certainly relate to Steve's need for escape. "When I first saw a PBC patient in end stage, I wanted to run,' she declares. "Maybe I'm just vain, but I remember thinking, No way am I ever going to look like that.'
Right now at least, she doesn't. Her skin does not appear jaundiced, and her eyes show only traces of yellow. Charlotte's life is presently so good, in fact, that the diminutive blonde hates to consider the uncertainties of a transplant. "I worry,' she confesses, "that if I wait too long, my health will decline enough to harm the transplant's chances. Yet who wants to dwell on the risks of a liver transplant when a halfwaynormal lifestyle is still possible?'
For PBC patients, depression, understandably, is also a problem. "Psychologically,' muses Charlotte, "what really hurts is knowing that your condition is only going to get worse. Yet I try not to dwell on how or why I got PBC.'
Slight enough to pass as a Churchill Downs jockey, Charlotte more than compensates for her small stature with her gritty determination to take all her problems in stride. "Maybe I've never really accepted my disease,' Charlotte contends, "but I have been realistic enough to reckon with it. Otherwise, I couldn't function normally and continue to do the things I enjoy--working on house projects with Steve, going to movies with my children, reading, doing needlework, playing golf and visiting amusement parks--I love rides!'
Charlotte's priority is motherhood. She has been especially quick to reassure the children. "Keith, for example,' Charlotte explains, "is very suspicious about any trips I make to the doctor or the hospital. He remembers my earlier hospitalizations, and he associates any absence with those prolonged stays away.' Charlotte now includes them in her medical routine. Both Keith and Jill have accompanied their mother to the lab, and Keith has "sat in' on an office appointment. Last August, the entire Harkness family made the trek to Mayo.
"Children that age are like sponges,' Charlotte observes. "You can talk all you want in whispers, communicate in postkindergarten code-- and then they still reveal some amazing awareness that they've soaked up.' She sighs. "I think I finally realized that we were all sharing the same wavelength when Keith looked up at me one day and solemnly asked, "Mommy, if you die, can I go with you?''
Charlotte says that Keith has since found great reassurance in witnessing firsthand that the doctors do nothing to hurt his mother.
Steve, however, is still reticent about Charlotte's illness. "He won't admit that he doesn't accept my sickness,' Charlotte notes, "but I can sense his difficulty.' Charlotte adds: "Friends sometimes wonder how he can appear so indifferent to my symptoms, but I personally think that he refuses to give the disease any credibility. Maybe his insistence on normalcy is even to may advantage. If he coddled or pitied me, I'd probably tend to wallow more in my own self-pity.'
Yet Charlotte does admit to some loneliness. "Steve doesn't have PBC, so I can't expect him to relate to all of my feelings,' she explains. "When my closest fellow liver patient died more than a year ago, I really felt an agonizing void. As the president of Indiana's chapter of the American Liver Foundation, Anne Mullendore had worked tirelessly on behalf of local liver patients and, despite her own illness, provided a sturdy shoulder. When you told Anne you were tired, she knew exactly what tired meant.
"One of the worst aspects of the more uncommon diseases is the lack of an available support network,' Charlotte continues, "the lack of ready access to other victims, other people who can personally identify with your doubts and fears.' Yet Charlotte quickly credits the American Liver Foundation in Cedar Grove, New Jersey, for its help to victims of unusual liver diseases.
"The foundation goes all-out,' she explains, "to provide educational literature, leads about doctors and treatment centers or just a sympathetic ear when needed.' Charlotte adds: "I gratefully acknowledge Thelma Thiel, the executive director of the foundation and the mother of a son lost to liver disease, for keeping me on my toes with regard to my own illness. A great believer in preparedness, Thelma has been urging me for two years to go to Pittsburgh to get my transplant evaluation. "Do it while you're healthy,' she insists.'
"Pittsburgh,' or the University of Pittsburgh School of Medicine, is the premier American facility for liver transplants and the home address of the world-renowned liver-transplant surgeon Dr. Thomas E. Starzl--"The best,' says Charlotte Harkness. Yet the distance is great, and the faces there unfamiliar. Charlotte hopes a transplant program at Mayo will be in place by the time she needs it.
"Steve, I think, believes that the transplant is still way down the road somewhere,' says Charlotte. "I believed that it was at least five years away, but after my August visit to Mayo, I've had to revise my calculations. Right now, I'm waiting to begin antirejection therapy with a controversial drug called Cyclosporin, which some doctors think may also slow the progression of the disease.
"The liver is the largest organ in the body,' notes Charlotte, "and it performs a thousand vital functions. Its transplantation is more complicated than that of a heart or of a kidney--and here I sit, wondering if I can handle it.' She pauses, adding: "I really don't dread the pain so much. I frankly worry more about the effects of the drugs that have to be taken after the transplant.'
She also worries about the costs: "It's truly outrageous. How many American families have $200,000 or more stashed away for a liver transplant? And how many American families have an insurance policy that will cover it? Right now, most victims are left dangling. The medical community has fought to prove that liver transplants are therapeutic, but the insurance lobbies insist that they are still experimental.'
Charlotte manages a wry grin: "If I were a sad-faced 7-year-old, hearts would go out to me. I can't expect the same reaction for a 36-year-old housewife. Yet somehow I know that people will do what they can.'
Neighbors, awed by her fortitude, don't hesitate to call her courageous. "She seldom complains,' observes one. "Despite everything, she keeps coping,' says another.
But Charlotte is fearful. "I have my apprehensions,' she admits. "After all, I know more or less what is ahead of me--PBC's progression, both systemically and cosmetically. I know that I'll need the best of both my faith and my will.
"Meanwhile,' Charlotte reflects, "I think of the possible good I can do if I help educate other people about a disease like PBC. Unfortunately, most victims don't even realize they have it until it's fairly well advanced. The other day, for instance, I was transfixed by a cashier's yellow, almost orange yellow hands. I wondered if a liver problem was at work in that woman.'
Her spirit indicates she's not ever likely to surrender easily. "If I'm feeling low and Steve's away, I'll fix dinner by candlelight for myself and the kids,' she relates. "I try not to miss any opportunity for special moments.' Charlotte also keeps a journal and enters very personal comments to her children. "If my life is interrupted, I want my thoughts and feelings to survive me. I want to have my say.'
A heroine in her own neighborhood, Charlotte Harkness has been nonetheless just another unknown fighter. Those who cheer her wonder how she motivates herself for each new round.
Her response is simple: "I remind myself that depression can do nothing to enhance my life. I'm like anyone else; I still think, sometimes, that it's all a big mistake--you know, someone's blood-test results mixed up with mine. But when I'm being honest with myself, I know that I can waste time on self-pity--or I can enjoy my family in the best way I know how.
"Because of my priorities, I can rest easy. No matter what happens, no one will ever replace me, and my family will always be certain of how much they were loved.'
Photo: Charlotte and Steve Harkness and their two-year-olds, Keith and Jill, are just one of thousands of U.S. families living with liver disease. Charlotte was diagnosed 4 1/2 years ago with primary biliary cirrhosis.
Photo: "I did let the depression get to me for a while,' says Charlotte. "Now, I just take one day at a time and try not to let the future swallow me up.'
Photo: Charlotte's lifestyle is presently so good (she loves playing golf and going on amusementpark rides) that she hates to consider the uncertainties of a transplant--yet she worries about the possible consequences of waiting too long.
Photo: Dr. Thomas Starzl gave new hope to liver patients by performing the world's first liver transplant in 1963. Since then 568 patients (in North America) have received transplants. More than half are still alive.
|Printer friendly Cite/link Email Feedback|
|Title Annotation:||Charlotte Harkness' liver illness|
|Author:||Giffin, Marjie G.|
|Publication:||Saturday Evening Post|
|Date:||Jan 1, 1985|
|Previous Article:||My life in the gutter.|
|Next Article:||Getting older, growing younger.|