(Note: Shortly after her death in 1984, this story was written partly in memorial, partly to aid in the emerging efforts of family and friends to turn this somber loss into a gesture of hope and support to others affected by cystic fibrosis. In the 16 years that have since passed many victories have been realized in seeking causes, therapies, treatments and cures for this still-deadly disease. The details given here were as they were in 1985. This is the way it was.)
On the 19th of December, 1984, six days before Christmas, Karen died. She was 25 years old. Every day of those 25 years was both a gift and a struggle. A gift of life itself and a struggle with pain, with daily therapy and with the constant coughing required to raise the deadly mucus produced in her lungs. Karen Larlee Grant was born, lived with and died of Cystic Fibrosis. CF is a disease from which, like the Devil's Island of yore, few, if any have escaped. Alive.
Cystic Fibrosis is one of the truly terrible diseases. Caused by a genetic defect in both parents, five percent of the Caucasian population of this country are undetectable carriers of this disease. Two parental carriers have a twenty-five percent chance of producing a child with CF. There is an equal twenty-five percent chance the child will escape any trace of the disease. And a 2-to-1 chance of the child itself being a carrier. CF is a genetic timebomb whose primary victims are the babies; it saps their strength, subjects them to constant, daily, painful therapy, deprives them of most of life's taken-for-granted pleasures and wreaks havoc on all of the body's vital organs until, in time, there is no support system with which to struggle for breath.
The secondary victims are the parents, who must alter the normal course of their lives in response to the distorted ebb and flow of the disease's demands. Also victims are the innocent siblings fortunate enough to escape the disease but entrapped in the guilt of health.
Most CF victims succumb early in life, often before their teens. Some get through high school. Of those diagnosed and treated early, some 50% reach the age of 21. Because it is a respiratory disease, the result of excessive or too thick mucus, being a "normal", active energetic child is impossible. Daily therapy, involving a hoofbeat-sounding, cupping, pounding on the back and ribcage is required to dislodge the thick matter, which is then raised and expectorated, as long as the lungs are strong enough to withstand the demands of coughing. And even then, in the midst of relative strength, the slightest activity brings on bouts of coughing. Racking, painful, debilitating coughing.
Blonde, blue-eyed Karen would not bow to her burden. From childhood, she did what she wanted. She swan, rode her bicycle, shot pool, fought with her brother and sister, laughed a lot, cried some and insisted on living. Karen's philosophy of survival was based on goal setting: no matter how unlikely to succeed, she constantly set new goals and drove herself towards them in the unshakable belief that she
would indeed be alive to reach them. A major goal was reached when, at the age of 15, she climbed Mt. Katahdin, highest peak in Maine. One step at a time, stopping only when she had to, she got up above the timberline, out across the plateau and up to the very top on the Mountain where, on a clear day, with even clearer vision, one can see all the way to the ocean.
Many was the time she sat beside a bedridden and discouraged CF patient and admonished them to "Just keep trying, just think of something you really want to do. Let's set an objective for you..let's set a goal…". And she was so hard to resist, they often did it. And thus moved ahead a little more, for a little longer.
A Celtic heritage, leading backward through time to Wales and Ireland, gave Karen two special gifts: music and laughter. Born with a natural ear for music, she needed no lessons on the piano. She heard the song, then played it. She loved music. Foot tapping, eyes shining, bright-smiling music. Any kind, all kinds. Among friends and family, her laughter is legendary. Anything but ladylike in volume and style, it rolled out in great gusts of infectious delight, totally impossible to ignore, engulfing all in its path. She loved to laugh. And smile. Impish grin, with finger-waving, eye-gleaming merriment.
In an odyssey that took her through every major hospital in New England, from Bangor's Maine Medical Center to Boston's Children's Hospital, year after year she made the rounds. No-one seems to know how many days Karen spent in hospitals. As the years rolled by, days in began to match days out, the time between hospitalizations becoming shorter and shorter. With operations buying time and constant medication a finger in the dike, her normal life became in, not out.
As 1983 began, the medications became less effective and more difficult to administer. Especially trying were the antibiotics, given intravenously each day through steadily shrinking veins, until it became an ordeal just to insert the needle. In desperation, agreement was reached to try a Hickman catheter. Used with some success on cancer patients, the Hickman catheter is a thin tube inserted below the collarbone into the jugular vein and then directly into the heart. Through a special external opening the medication can be pumped directly to the bloodstream, to do its magic without the added stress of a needle…but not without danger: meticulous care must be taken to ensure absolute sterility of the device, demanding frequent cleansing, to prevent introduction of germs and bacteria. Equally threatening is the possibility of introducing an air bubble along with the medication, which could result in a coronary embolism. And sudden death.
In an operation that nearly took her life, Karen became the first CF patient in the State of Maine to receive a Hickman catheter. The date was February 14th, 1983. Valentine's Day.
The catheter changed her life. Suddenly she took on new vigor as the medications gave more than they took. Full of energy, she was up and down stairs, swimming again and playing frisbee. As in all other aspects of her disease, she studied every facet of the catheter. How to clean it, how to use it, and what the dangers and possibilities were.
Visits to the hospital now invariably resulted in Karen holding clinics for the staff in proper procedure for the device. Her store of knowledge about CF was thorough. Having learned all there was to know about the disease, she shared her knowledge willingly and openly. Doctors on their rounds with a group of medical students scheduled stops at her bedside, where Karen instructed them about CF. Many a student returned on their own time, in the evenings and weekends, to continue their education from a teacher who really knew her subject. Only too well.
And she tried everything she could. Even marriage for a time. A beautiful picture-book wedding; white gown, champagne toasts. Music and dancing. Proud and doting parents. Dozens and dozens of well-wishing and wishful-thinking friends and relatives. Eventually she moved back home. Not many can live gracefully with the daily torment and pain of such a disease.
So she picked up the pieces, adjusted to the empty space inside and moved on.
CF is a patient killer. Depriving the body of sufficient oxygen, it gradually destroys vital organs. It wrecks the pancreas, and weakens the lungs, making them eventually unable to provide enough force to cough, thus making it impossible to, on their own, raise the deadly phlegm gathering in those delicate sacs. As time goes by, the threat of drowning on one's bodily fluids becomes nightmarishly real.
The imminent ending was not to be ignored. Back into Manchester, NH's Elliot Hospital for what should have been just another few days of medication, rest, intravenous nourishment and building up of strength for the holidays, Karen encountered a new torment. Severe bouts of coughing left her with two painfully cracked ribs, a weakened body and no way to cough up the fluids building in her lungs.
Despite her protestations, she was put onto a combination respirator/ventilator, a wonderfully complex machine that took over the task of regularly filling her lungs with life-giving air. Periodic suctioning by more machinery removed the liquids, a process so painful that medication could only begin to mask the discomfort. From the beginning of the end, these measures were limited to, at most, days.
Although she was adamant about not being supported by machines, Karen took brief comfort, of a sort, from them. With pure oxygen flowing into her system, and pain dulled by medication, she had an irrepressible urge to communicate.
Tubes in her nose and mouth prevented her from talking but she was not to be denied. With a pad of paper and pencil, Karen kept up a stream of dialogue with whoever was at her side at the moment. Quips and questions, serious thoughts and silly, her waking moments were filled with "talking". Many times, as she drifted off to sleep, notepad at her side, she would convey one last message by lifting a thin hand and writing, one letter at a time, in the air, while the recipient read it aloud. Until, with a loved one holding her hand she would drift off to sleep, gathering enough strength to endure a few more hours.
The machines could only do so much. Beyond a certain point, more complications are introduced than problems are solved. The inevitable moment came when a decision had to be reached: not if, but when, to disconnect.
On the morning of the 18th of December, Karen made that decision. Out came the tubes. With a winning smile and a gleam in her eye, she make the classic motions of playing an "air guitar", asking for music.
By evening, lungs barely functioning, she was slipping away. Despite being heavily medicated, she roused herself to listen to music provided by a small band of guitar pickers and singers. Christmas carols intermingled with John Prine and Neal Young tunes, room crowded with hospital staff, doctors, nurses, relatives and friends. A warm and comforting priest who commented on the love present in the room.
Through the long night, she struggled for every breath, forcing her lungs to bring in just a little more…Just a little more…Air.
Two am and she drifted off into a coma. Still working to breathe, still clinging, still climbing. On and on through the night.
At 9:45 am, with family around her, her mother reached out and rubbed Karen's foot, as she had done so many times throughout this twenty-five year ordeal. Then she said, "It's all right, Honey, we are all here with you."
Karen relaxed and let out a breath. Then she flew away.
Karen is gone. What remains is an implacable disease that can only be cured by constant and continuing research, a genuine genetic timebomb, Cystic Fibrosis. It's detection, control or cure are goals that Karen would have set. And worked toward. Until reached.
© March 19, 1985 David Larlee