Wednesday, August 23, 2000

She eases the pain of a child dying young

StarShine hospice program at Children's Hospital helps families see their way through one of life's toughest moments

By Peggy O'Farrell
The Cincinnati Enquirer

        Lynda Tucker does what any good nurse does: She asks her patient how he's feeling. She checks his blood pressure and temperature. She fishes her stethoscope out of a purple and teal totebag and listens to his heartbeat.

[photo] Linda Tucker checks the blood pressure of Danny Breuer, 15.
(Steven M. Herppich photo)
| ZOOM |
        “Is he eating?” she asks in her distinctive Manhattan accent. “Getting enough fluids?”

        “Yeah, he's doing really well,” replies his mother, Carla Fitzgerald. On this home visit, in fact, Danny Breuer is sitting up and eating vanilla sandwich cookies in the hospital bed his folks have set up in the living room of their Butler County mobile home.

        Like all of Lynda's patients, Danny's condition changes from day to day. And, like all of Lynda's patients, Danny is dying.

        Danny is in the final stages of Batten disease, a rare neurological disorder that kills most patients before they reach their 20s. Danny is 15. Lynda is the hospice nurse who will care for him until he dies.
    StarShine is the home-based hospice program of Children's Hospital Medical Center in Cincinnati. Treatment is geared toward children who, if their disease follows its normal progression, are within six months of dying. But because outcomes are difficult to predict, the six-month period can be extended.
    The focus is on providing home-based supportive and palliative care for the child, and psychological and spiritual support for the child and family.
    Each child's hospice team includes a nurse, social worker and chaplain, as well as occupational and physical therapists, massage therapists and pain-control management specialists as needed.
    Services include in-home care; spiritual support; occupational, speech, physical and infusion therapy and follow-up bereavement care. Support is highly individualized and based on each family's needs. Volunteers can assist by reading or doing crafts with patients or their siblings, taking the children on special outings and helping with funeral arrangements.
    For information on hospice care, becoming a volunteer or making a donation, call the StarShine office, 636-4663.
• National Institute of Neurological Disorders and Stroke, (800) 352-9424
• Batten Disease Support and Research Association ( or (800) 448-4570 for information and support or to make a donation to aid research.
• National Tay-Sachs and Allied Diseases Organization ( or (800) 9ONTSAD

Years of experience

               Lynda has worked for StarShine, the hospice program at Children's Hospital Medical Center since it began in 1995. StarShine is one of only a dozen or so pediatric hospices in the United States. Danny is one of about 35 children whose pain will be eased by StarShine staff this year. More pediatric hospices are needed, medical experts say, to meet the needs of children such as Danny.

        The outcome of this scenario is inevitable. Despite her years at StarShine, 20 years as an adult hospice nurse and previous work with cancer patients, Lynda can't make Danny better. On Danny's behalf, she coordinates a team of StarShine experts — a social worker, chaplain, occupational and physical therapists, massage therapist and pain-control management specialist — to do everything they can to make the 15-year-old comfortable and pain-free.

        But Danny's days are numbered, and losing a patient — especially a child — is never easy.

        “There are times when we're not able to reconcile ourselves to a situation. I think we've just accepted that,” Lynda says. “You have to establish a certain equilibrium so you can function the next day. We still need to get up in the morning if we've gone out and done a postmortem visit on a child the night before.

        “I can think of several times when I never thought I could get up the next morning and go to work. But we try to do our private thing privately. I really don't want these families to feel that our nurses are so needy, and I think our gratification really comes from helping these families out.”

        Lynda's young patients make her think of her own four children, all now grown, and three grandchildren. “I'm a mother and I'm a grandmother, and sometimes it's difficult to deal with,” she says.

An unfair blow

               The 56-year-old takes the 45-minute drive to Seven Mile in Butler County at least once a week to see Danny. On this summer day, she stops at a Walgreens to buy a bag of Gummy Worms for another patient's older brother before climbing into her green Land Rover and heading up to Danny's house.

        In some ways, every visit is the same: Lynda checks Danny's vital signs, makes sure he's eating, teases his 12-year-old sister, Katie, about the glitter eye shadow she's wearing and makes sure Mrs. Fitzgerald has enough anti-seizure medicine and supplies to see Danny through the week until her next visit.

        And every visit is different. Some days Danny is sitting up and talking and she can tease him about the buzz cut his 17-year-old stepbrother, Jarrod, has given him. Some days he is awake, but not alert. Some days he is sleeping.

        Danny's illness, she says, is “heartbreaking.”

        Batten disease is progressive. Danny was diagnosed in 1996. Now blind and bed-ridden, he suffers spasms of the legs that can last as long as 15 minutes. He has entered the dementia phase.

        “He's not going to look old. He's going to look like a teen-ager. But he's aging as he deteriorates. The debilitating neurological process is just going to continue and continue to happen.”

"Am I dying?'

               Lynda is fairly sure he still recognizes her.

        “I think he pretty much knows who I am. He kind of floats in and out of some of the dementia.”

        And Danny understands how sick he is.

        In January, he was hospitalized after a particularly severe round of seizures.

        “He woke up from a seizure and said, "Am I dying?'” Mrs. Fitzgerald says. Not long after, he was enrolled in StarShine.

        Every now and then — more often in recent weeks as his seizures have worsened — Danny talks about joining his late grandfather.

        “He'll tell you, "I'm going to go to heaven and play football with Poppy,'” Mrs. Fitzgerald says.

        Lynda says her young patients understand much more than their parents think they do. And many are ready to let go before their parents are, so they hang on a little longer.

        “Sometimes kids don't let go because they think the parents can't handle it,” she says. “Sometimes a family has to give permission, to say it's all right to let go.”

        She remembers an 8-year-old girl with leukemia whose parents weren't quite ready to say goodbye. “She said to me, "Lynda, I'm so tired.' And I said, "Honey, go to sleep.' And she knew what I meant.”

        And she remembers the first child she cared for after she became a hospice nurse, a 10-year-old named Richard who died of leukemia in 1984.

        “That took a lot out of me,” Lynda says quietly.

No other options

               The decision to place Danny in StarShine was a hard one, Mrs. Fitzgerald says. Choosing hospice care means acknowledging there are no other options.

    Question: What is Batten disease?
Batten disease is a fatal inherited neurological disorder, the most common of a group of diseases called neuronal ceroid lipofuscinoses (NCLs).
    Symptoms begin between ages 5 and 10, when parents or physicians notice a child experiencing vision problems or seizures. Signs may be as subtle as personality and behavior changes, problems learning, clumsiness or stumbling. The disease is progressive, and children eventually become blind and bed-ridden and develop dementia.
    Batten's is fatal by the late teens or 20s. It cannot be cured or prevented at this time.
    Q: How common are NCLs?
The diseases are fairly rare, occurring in two to four of every 100,000 live births in the United States. Batten disease and other NCLs are more common in Finland, Sweden and other parts of northern Europe and in Newfoundland. They may strike more than one person in families that carry the defective genes.
    Q: How are NCLs inherited?
Childhood NCLs like Batten disease are autosomal recessive disorders: A child has to inherit the defective gene from both parents in order to be at risk for developing the disease. Individuals who inherit only one copy of the gene are carriers and don't develop the disease but can pass the gene onto their own children. There is no test yet to identify carriers of NCLs.
    Q: What causes the degenerative symptoms of NCLs?
Substances called lipopigments, which are made up of fats and proteins, build up in the cells of the brain and eyes, along with the skin, muscles and other tissues. Scientists aren't yet sure whether people with NCLs can't produce enough of certain kinds of enzymes to break down the lipopigments or produce too much of the substance. But as the lipopigments accumulate, they cause the tissues to deteriorate. The lipopigments have distinctive shapes that can be seen under an electron microscope, and, when found in skin samples, are used to diagnose Batten's.
    Q: Is there any treatment?
    A: No treatment is available to halt or reverse the symptoms of Batten's or other NCLs. Seizures can be controlled with drugs like dilantin or Valium, and other symptoms be treated as they arise. Physical and occupational therapy can also help patients function better.
        Few children end up in hospice programs — probably because doctors and parents are reluctant to give up if there's a chance that a treatment might work.

        Lynda understands that. “If it were my child, I don't think I could give up,” she says.

        But sometimes there are no treatments.

        For Danny's family, hospice means no more trips to the emergency room when his seizures start. And it means he's home with them, able to make the occasional jaunt to Paramount's Kings Island or some other fun place, and not being stuck in a hospital.

        Thankfully, StarShine is small, director Lisa Richter Beck says. The highest enrollment has been seven children at a time. Each child is assigned to one of the seven nurses in the program. Nurses also fill in on each other's days off and take turns being “on call.” StarShine takes children who, if their disease follows its normal progression, are within six months of dying.

"That terrible silence'

               Danny's sickness is hard on Jarrod and Katie in many ways. Sometimes they're reluctant to ask for things or to go places with their friends.

        StarShine helps its families deal with the stress and grief of a terminal illness while coping with day-to-day life.

        “Sometimes the siblings' regular needs get overlooked,” says Bonnie Crawford, StarShine's social worker. “It's easy to do.”

        StarShine's child life therapist helps siblings understand and cope with the illness. The therapist might do crafts or one-on-one counseling with a sibling. Or the therapist might go to school to talk to classmates and teachers about what's happening.

        StarShine volunteers drive siblings to to soccer practice, scout meetings, after-school activities and help arrange outings to amusement parks or events.

        The death of a child leaves a huge gap in a family, Lynda says.

        She knows how quiet the Fitzgerald home will be once Danny is gone.

        “There's such a fine line,” Lynda says. “One minute the child is there and breathing, and maybe not doing anything, but he's alive and there's a heartbeat. And the next, the child is gone, and there's that terrible silence the family has to deal with.”

        From time to time Lynda worries that some of the parents won't be able to cope with having their child die at home, rather than at the hospital.

        She praises how Danny's family is coping, calling Mrs. Fitzgerald “a real trouper.”

        “She's coping very well.”

        Mrs. Fitzgerald says she's just doing what she has to do.

        “People ask, how do you do this?” she says. “Well, you just do it.” She gave up her job to stay home with Danny. Her husband, Charlie, changed jobs to be closer to home.

        Because she can't make Danny well, Mrs. Fitzgerald wants to make his disease better understood. “I want to be Michael J. Fox,” she says, referring to the actor's plea for Parkinson's Disease funding. “I want to stand up and ask Congress for money for research.”

Setting boundaries

               Lynda also has to think about herself.

        Working closely with patients and their families under such emotional stress pushes objectivity to the limit. It's too easy to get too close.

        Lynda works hard to maintain a professional boundary between compassionate care and emotional involvement so she can function after a patient dies.

        “We all run around reminding each other, "boundaries,'” Lynda says.

        She knows she's too close to a family when she micromanages the nurses assigned to her patients on her days off and when she can't sleep.

        Exercise is her remedy of choice. She goes for a walk in the Indian Hill neighborhood where she lives with her husband, David, an ophthalmologist. She takes aerobics. She visits her grandkids.

        Hospice work demands a different mindset, and Lynda knew that when she chose it.

        Hospice nurses need “compassion, dedication and a grounded sense of personal spirituality,” says program director Beck. Lynda's experience is an asset. “She brings a great deal to the program with her many years of hospice background, and she's a very giving individual,” Ms. Beck says.

        When Lynda was working with cancer patients, there wasn't much a nurse could do for a patient if chemo and radiation didn't work.

        Hospice allows her to ease the pain and suffering of the patient. And that, she says, is a privilege.

        She has plenty of thank-you notes from families, but that's not the point.

        “It's not the idea of getting the gratitude back,” she says. “It's the feeling that if we're extending their lives, we're extending the quality of their lives as well.”


Batten Disease is progressive


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