mommy, if you die can i go with you? The holiday season of 1979 was less than merry for Steve andCharlotte Harkness. On Thanksgiving, Charlotte nursed an extremely soreneck gland that had failed to return to normal size after herlate-summer bout with the “mumps.’ She attributed herstiffness, fatigue and discomfort to the demands of mothering a livelypair of two-year-olds– her natural son, Keith, and her adopteddaughter, Jill. The week before Christmas, all personal concerns were droppedcompletely. A late-night phone call brought the news that Steve’sbrother-in-law had been killed in a coal-mining accident in southernIndiana. By dawn, the two Harkness toddlers had been shuttled off tothe homes of friends, and Steve and Charlotte were speeding towardOakland City.
On Christmas Day, Charlotte, back home with the two children, feltcertain she was “coming down’ with the flu. Steve was stillhelping his relatives adjust, so Charlotte, hoping to ward off thevirus, went to bed early. She awakened during the night, however, andwas so faint that she could barely wobble from the bed to the bathroomand back. When Charlotte Harkness reopened her eyes early on the morning ofDecember 26, she knew that something was terribly wrong, but she couldnot have guessed that the events of the next few hours would sodramatically alter her life. Charlotte was experiencing the firstsymptoms 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,’ Charlotterecalls, “and the nausea was incapacitating. Luckily, the phonewas within reach, so I frantically called a friend to come pick up thekids.’ Charlotte had settled back in bed for less than ten minuteswhen she got the scare of a lifetime. “There’s no delicateway to describe what happened,’ she says.
“I began,literally, to heave blood all over the sheets– huge, dark puddles ofblood.’ Fortunately, Charlotte was able to call her family doctor. He toldher to call an ambulance immediately. Fearful that she wouldn’t beable to get to the door to let in an ambulance crew, she instead dialeda 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. Hourslater, I remember opening my eyes in intensive care and seeing Stevestanding over me.’ Charlotte Harkness’ nightmarish holiday was over, but herordeal as a victim of liver disease was just beginning.
At only 31, shewas one of the youngest women ever diagnosed with the disease known inmedical circles simply as PBC. “Looking back,’ Charlotte says today, “I sometimeswonder if I could go through it all again. Yet I know there must behundreds of people just like me– many with diseases with differentnames–who need to know that they can continue to enjoy life despitenecessary medications and treatments. They need to know that lifeneedn’t come to a halt just because of an unexpecteddiagnosis.
‘ For Charlotte, the eventual diagnosis actually came as a relief.”From that bleak December of 1979 until the following June, weendured so much,’ she explains. “All of the uncertainty wasalmost more scary than the ultimate knowledge that I had PBC.’ One night a week after being dismissed from the hospital, forinstance, Charlotte woke up, pained by the same enlarged neck gland thathad plagued her the previous autumn. “In the wee hours of thatparticular night, I finally cried,’ Charlotte confesses, “butstill more out of frustration than fear.’ Early the next morning, the gastroenterologist monitoringCharlotte’s liver condition arranged for her to see an ENT specialist; the following day, Charlotte was in surgery as doctorslooked 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, inand out of the hospital, with a husband and two little kids at home, andno one seemingly able to tell me what was wrong. Meanwhile, my thirdmiscarriage and a neighbor’s fatal accident only added to ourmisery.’ Finally, in June 1980 Charlotte got some long awaited answers.”My gastroenterologist called Steve and me in together and told usthat he was almost certain that I had PBC. Once we had bombarded himwith questions about the nature of the disease, we knew the newswasn’t good,’ she admits, “but he did tell us to considera new drug being studied at Mayo Clinic that might slow the progressionof the illness.’ After careful thought, Charlotte decided the drug-study program atMayo meant she could play a role in her struggle for survival. InAugust 1981, therefore, Charlotte Harkness made her first trip to theMayo Clinic in Rochester, Minnesota.
She underwent four days ofrigorous examinations and learned a great deal about primary biliarycirrhosis. A chronic disease most frequently afflicting postmenopausal women,PBC gradually destroys certain bile ducts within the liver. Thesymptoms 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 andleads to cirrhosis, a condition that prevents the liver from functioningnormally and inhibits blood flow. Too often, people think of cirrhosisas an alcoholic’s disease, but many liver disorders can lead tothis serious deterioration.’ Charlotte was diagnosed at Mayo as entering the third stage of thefourstage disease. She became a participant in a federally fundeddrug-study program of D-penicillamine, a medication used to reduce thebody’s level of copper.
(PBC patients can’t adequatelymetabolize 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-monthcheckup. Doctors assured her she was holding her own.
By her Novembervisit, however, her difficulties were again multiplying. Since summer,she had experienced occasional upper-gastrointestinal bleeding, twomajor gall-bladder attacks and daily nausea and cramping. At Mayo,doctors theorized that the enlarged liver had caused the gall bladder tobecome disengaged, a theory that explained why they couldn’t locateit even with ultrasound. Since travel was out of the question, a Mayo surgeon removedCharlotte’s gall bladder. Shriveled and knotted with stones, itwas the obvious culprit in her most recent discomfort. Her primarydisease had also progressed, however–Charlotte was teetering betweenthe third and fourth stages of PBC.
The following month, Charlotte’s local gastroenterologistdecided to schedule her for plasmapheresis, a plasma-exchange processthat 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 costsapproximately $1,000 per treatment. It relieved Charlotte’schronic itching but caused her additional concern about “a new andunwelcome feeling of dependency on a machine.’ In November 1983, Charlotte again returned to Mayo, her skinblistered from a reaction to the D-penicillamine and her itching morepersistent than ever because she had weaned herself from the pheresismachine. Doctors told her that her routine liver biopsy was no longernecessary now that she was an advanced PBC patient. Because of her sideeffects, she was also retired from the D-penicillamine study program. Bouts with mild internal bleeding marred Charlotte’s springseason, and by late summer 1984, she was openly worried about thegeneral state of her health.
Her liverfunction scores had been climbingfor several months and the stubborn seepage problem had kept herhemoglobin count low. As she feared, her visit to Mayo in August wasnot a pleasant one. “My doctor confirmed that I’m now in the fourth stage ofPBC,’ Charlotte explains, “and he admitted that he was”alarmed’ by my rate of progression. For the first time, theneed for an eventual liver transplant was seriously discussed–and as anindication of the gravity of things, when my life expectancy without atransplant was predicted, my husband literally darted from theroom.’ Charlotte can certainly relate to Steve’s need for escape.
“When I first saw a PBC patient in end stage, I wanted torun,’ she declares. “Maybe I’m just vain, but I rememberthinking, No way am I ever going to look like that.’ Right now at least, she doesn’t.
Her skin does not appearjaundiced, and her eyes show only traces of yellow. Charlotte’slife is presently so good, in fact, that the diminutive blonde hates toconsider the uncertainties of a transplant. “I worry,’ sheconfesses, “that if I wait too long, my health will decline enoughto harm the transplant’s chances. Yet who wants to dwell on therisks of a liver transplant when a halfwaynormal lifestyle is stillpossible?’ For PBC patients, depression, understandably, is also a problem.”Psychologically,’ muses Charlotte, “what really hurts isknowing that your condition is only going to get worse. Yet I try notto dwell on how or why I got PBC.
‘ Slight enough to pass as a Churchill Downs jockey, Charlotte morethan compensates for her small stature with her gritty determination totake all her problems in stride. “Maybe I’ve never reallyaccepted my disease,’ Charlotte contends, “but I have beenrealistic enough to reckon with it. Otherwise, I couldn’t functionnormally and continue to do the things I enjoy–working on houseprojects with Steve, going to movies with my children, reading, doingneedlework, playing golf and visiting amusement parks–I loverides!’ Charlotte’s priority is motherhood. She has been especiallyquick to reassure the children. “Keith, for example,’Charlotte explains, “is very suspicious about any trips I make tothe 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 Jillhave accompanied their mother to the lab, and Keith has “satin’ on an office appointment. Last August, the entire Harknessfamily made the trek to Mayo. “Children that age are like sponges,’ Charlotte observes.”You can talk all you want in whispers, communicate inpostkindergarten code– and then they still reveal some amazingawareness that they’ve soaked up.’ She sighs.
“I thinkI finally realized that we were all sharing the same wavelength whenKeith looked up at me one day and solemnly asked, “Mommy, if youdie, can I go with you?” Charlotte says that Keith has since found great reassurance inwitnessing 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.
‘ Charlotteadds: “Friends sometimes wonder how he can appear so indifferent tomy symptoms, but I personally think that he refuses to give the diseaseany credibility. Maybe his insistence on normalcy is even to mayadvantage. 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. “Stevedoesn’t have PBC, so I can’t expect him to relate to all of myfeelings,’ she explains. “When my closest fellow liverpatient died more than a year ago, I really felt an agonizing void. Asthe president of Indiana’s chapter of the American LiverFoundation, Anne Mullendore had worked tirelessly on behalf of localliver 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 thelack of an available support network,’ Charlotte continues,”the lack of ready access to other victims, other people who canpersonally identify with your doubts and fears.’ Yet Charlottequickly credits the American Liver Foundation in Cedar Grove, NewJersey, for its help to victims of unusual liver diseases. “The foundation goes all-out,’ she explains, “toprovide educational literature, leads about doctors and treatmentcenters or just a sympathetic ear when needed.’ Charlotte adds:”I gratefully acknowledge Thelma Thiel, the executive director ofthe foundation and the mother of a son lost to liver disease, forkeeping me on my toes with regard to my own illness. A great believerin preparedness, Thelma has been urging me for two years to go toPittsburgh to get my transplant evaluation. “Do it whileyou’re healthy,’ she insists.’ “Pittsburgh,’ or the University of Pittsburgh School ofMedicine, is the premier American facility for liver transplants and thehome address of the world-renowned liver-transplant surgeon Dr.
ThomasE. Starzl–“The best,’ says Charlotte Harkness. Yet thedistance is great, and the faces there unfamiliar. Charlotte hopes atransplant program at Mayo will be in place by the time she needs it. “Steve, I think, believes that the transplant is still waydown the road somewhere,’ says Charlotte. “I believed that itwas at least five years away, but after my August visit to Mayo,I’ve had to revise my calculations.
Right now, I’m waiting tobegin antirejection therapy with a controversial drug calledCyclosporin, which some doctors think may also slow the progression ofthe disease. “The liver is the largest organ in the body,’ notesCharlotte, “and it performs a thousand vital functions. Itstransplantation is more complicated than that of a heart or of akidney–and here I sit, wondering if I can handle it.’ She pauses,adding: “I really don’t dread the pain so much.
I franklyworry more about the effects of the drugs that have to be taken afterthe transplant.’ She also worries about the costs: “It’s truly outrageous.How many American families have $200,000 or more stashed away for aliver transplant? And how many American families have an insurancepolicy that will cover it? Right now, most victims are left dangling.
The medical community has fought to prove that liver transplants aretherapeutic, but the insurance lobbies insist that they are stillexperimental.’ Charlotte manages a wry grin: “If I were a sad-faced7-year-old, hearts would go out to me. I can’t expect the samereaction for a 36-year-old housewife.
Yet somehow I know that peoplewill do what they can.’ Neighbors, awed by her fortitude, don’t hesitate to call hercourageous. “She seldom complains,’ observes one.”Despite everything, she keeps coping,’ says another.
But Charlotte is fearful. “I have my apprehensions,’ sheadmits. “After all, I know more or less what is ahead ofme–PBC’s progression, both systemically and cosmetically. I knowthat I’ll need the best of both my faith and my will.
“Meanwhile,’ Charlotte reflects, “I think of thepossible good I can do if I help educate other people about a diseaselike PBC. Unfortunately, most victims don’t even realize they haveit until it’s fairly well advanced. The other day, for instance, Iwas 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 surrendereasily. “If I’m feeling low and Steve’s away, I’llfix 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 herchildren.
“If my life is interrupted, I want my thoughts andfeelings to survive me. I want to have my say.’ A heroine in her own neighborhood, Charlotte Harkness has beennonetheless just another unknown fighter. Those who cheer her wonderhow she motivates herself for each new round. Her response is simple: “I remind myself that depression cando nothing to enhance my life. I’m like anyone else; I stillthink, sometimes, that it’s all a big mistake–you know,someone’s blood-test results mixed up with mine. But when I’mbeing honest with myself, I know that I can waste time on self-pity–orI can enjoy my family in the best way I know how.
“Because of my priorities, I can rest easy. No matter whathappens, no one will ever replace me, and my family will always becertain of how much they were loved.’ Photo: Charlotte and Steve Harkness and their two-year-olds, Keithand Jill, are just one of thousands of U.S. families living with liverdisease. Charlotte was diagnosed 4 1/2 years ago with primary biliarycirrhosis.
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 tolet the future swallow me up.’ Photo: Charlotte’s lifestyle is presently so good (she lovesplaying golf and going on amusementpark rides) that she hates toconsider the uncertainties of a transplant–yet she worries about thepossible consequences of waiting too long. Photo: Dr. Thomas Starzl gave new hope to liver patients byperforming the world’s first liver transplant in 1963. Since then568 patients (in North America) have received transplants. More thanhalf are still alive.