“YOU MIGHT HAVE a hard time with this case,” said the nurse calling in the referral. “It's for two Amish girls with a rare neurologic disorder. Seeing them is very depressing.”
I'd soon find out how wrong she was. True, Karen and Sally Miller had a terrible disease that left them wheelchair-bound and unable to speak—but what beautiful and special girls they were. As the nursing supervisor of a home health care agency, I got to know them during monthly visits to their school. Betty, one of our nurses, cared for them each weekday from the time they boarded the school bus until they returned home.
Progressive movement disorder
The Miller sisters had Hallervorden-Spatz syndrome (HSS), an inherited movement disorder characterized by progressive degeneration of the nervous system and iron accumulation in the brain. Affected by dystonia, muscle rigidity, and spasticity, both girls had tracheostomy and gastrostomy tubes and attended a special school where they received physical, occupational, visual, and tactile therapy.
Slender Karen, who was 17 when we met, had long, brown hair and a smile that could brighten anyone's day. She had degeneration of the retina, but how much it affected her vision wasn't clear. Unable to speak, she used her right leg to communicate.
Sally, age 12, was considered healthier than Karen at the time. She had long, dark blond hair; rosy cheeks; and eyes with a nonstop twinkle. Because spasms had caused her to bite her tongue, her teeth had been removed, so her grin reached from ear to ear.
As much as possible, the Miller family adhered to Amish traditions. John, the father, wore a white shirt, black pants with suspenders, and a black hat with a wide brim as he left home to operate a lumber mill with relatives. His wife, Mary, wore a pale blue dress and a tidy white bonnet as she cared for her home and family.
The Miller home had an open floor plan to accommodate the girls' needs. It didn't have electricity, so the family used a generator to run Sally's oxygen concentrator. They'd also installed a phone so Mary could keep in touch with the health care team.
Years of friendship
In the 4 years I worked with Karen and Sally, I looked forward to their brilliant smiles and welcoming gestures each time I visited their school. Their middle sister Kathy, who also had HSS, was in the same classroom. She wasn't a client with my agency because she didn't have a tracheostomy, but I came to love her as much as her sisters.
All the students at the school had physical or mental limitations, but the remarkable teacher, nurses, and classroom assistants kept the environment as normal as possible. Excellent care, tender encouragement, and hearty measures of love shone through all their interactions with the students.
When Karen and Sally arrived at school each day, they received range-of-motion sessions to help them loosen up. The therapists used balls, swings, games, and anything else they could think of to sharpen their senses and improve their skills. Thanks to the therapists' efforts, the girls could relax their muscles and better control their limbs. Karen's breathing improved so much that her pale skin became almost rosy and her nail beds went from dusky blue to pink.
A few years after I entered the picture, Sally's health deteriorated. She had trouble getting enough air through her pediatric size 5 tracheostomy tube, and scar tissue building up in her tracheostomy made breathing even more difficult. Surgery to remove the scar tissue improved her breathing, but she had a fever of unknown origin for several weeks afterward and the physicians advised her parents that the scar tissue might return.
Within just months, her condition again began to decline. Her breathing became more labored, the rosiness left her cheeks, and the twinkle in her eyes dimmed. As she needed suctioning ever more frequently, we knew the scar tissue was back.
Having worked as a hospice nurse, I could see that Sally was nearing the end stage of her life. I also recognized that the people closest to her weren't able to talk about it.
Confronting a painful reality
Believing that Sally's friends needed to open up with one another and with her, I volunteered to present a death-and-dying education session for the adults at her school. About a dozen people attended, some who'd never encountered death before. I distributed handouts describing the breathing, color, and temperature changes that can occur in someone who's dying and handed out tissues for their tears.
Relating what I knew about dying, I explained that many terminally ill people choose when to die and reviewed the signs and symptoms of impending death. I told the staff that feelings of sorrow, relief, and guilt before or soon after the death of a loved one are common. Pointing out that many dying people need permission from loved ones to “go,” I explained the benefit of creating memories in their remaining time together. The season was early spring, and we wondered if Sally would return after spring break. The tears flowed freely.
Before parting, I reminded the participants that Sally would choose to die in her own time and place. As they left, I felt they were stronger and better prepared to face the possibility of losing this gentle and loving child.
Because Mr. and Mrs. Miller hadn't attended this meeting, I drove to their home on a day when the children were in class and spoke with them as I had with the school staff. They'd learned so much about their daughters' disease and worked so hard to improve their lives—but they'd never confronted the idea that the girls might die. They had questions about Sally's changing condition and wondered if they should put her through another surgery.
“Will her benefits outweigh the risks?” I asked. “Will surgery result in a significant improvement, or will the scar tissue just grow back in a short time?”
I encouraged them to ask Sally what she wanted and to accept her answer. “Letting nature take its course isn't wrong if it means meeting her wishes,” I said.
Sally continued to lose ground that spring and summer and needed continuous oxygen therapy. In the fall, scar tissue again threatened to occlude her airway, and she and her parents decided to go ahead with the surgery. Afterward, she again had persistent fevers and couldn't regain her strength.
On my next monthly visit to the school, Sally was too ill to attend. I made plans to see her at home the next week but never got the chance. Mr. Miller called Betty to tell her that Sally had died peacefully in her sleep.
The next spring, the Millers invited Sally's teachers and classroom assistants to their home. In the garden, they planted a tree in her memory, sang her favorite songs, and reminisced about her antics at school. I couldn't attend, but the memory of Sally's sweet smile and her friendship will be with me always.
When Karen turned 21, she was “aged out” of the school. Rather than having her parents find another therapeutic setting, she decided to stay at home. Although Karen no longer has access to the therapies she'd had at school, she continues to thrive.
Meanwhile, her sister Kathy is exhibiting more and more signs and symptoms of HSS. If someday she needs nursing care, the nurses who loved and cherished her sisters are ready and willing to help.
Mary Degnan is a case manager for the Medicaid Obstetrical Maternal Services (MOMS) program in conjunction with the federally funded Women, Infants, and Children nutrition program in Jamestown, N.Y.
SELECTED WEB SITE
NBIA Disorders Association http://www.nbiadisorders.org
Last accessed on May 2, 2005.