Reprinted with permission from NHPCO’s Children’s Project on Palliative/Hospice Services, Issue #47
When our first grandchild was born, I wrote her a letter quoting words I had heard the day she was born: “May the bright morning star rise in your heart.” Her Grandfather Charlie and I subsequently celebrated the births of six more babies. I included these words in the welcome I sent to each new child. Our first grandchild is now in college; the fourth and fifth born are now in high school, and the seventh, born to our daughter and her partner, is now in grade three.
The second and third in the order of our grandchildren are a boy, Hayden, born in April 1998, and a girl, Cameron, born in May 1999, each born to one of our identical twin boys and his wife. These babies were born with Tay-Sachs disease. “The bright morning star” was certainly a part of their short lives because of the care and love they received from their parents, their Aunt Deirdre (the boys’ younger sister), and three sets of grandparents. It is their lives, and their effect on me and my husband, that inspires my story. Our twin sons and their wives, unknown to any one of the four, each carried the defective Hex-A Tay-Sachs gene. Our sons carried a mutation of the gene which was unknown at the time, as did our daughter-in-law and her mother. I am the carrier in our family and one of the twins’ mothers-in-law also carried an unknown strain of the disease (not the Irish, Cajun, French Canadian, or Ashkenazi). When both parents are carriers (one wife carried the Ashkenazi strain), each child has a 25% chance of having Tay-Sachs and a 50% chance of being a carrier of the disease.
Hayden Lord has a complicated medical history because he received two different diagnoses before the doctors, with significant help from his physical and practical therapists, arrived at the final dread decision. At Hayden’s 12 month physical exam he was said to be “developmentally delayed.” He had a beautiful celebratory birthday, with other children he had played with over the months. It was so typical of his adoring parents to give him a “normal” celebration. It did not matter that he could not sit up yet or crawl. He could still have his first bite of chocolate cake and share his inimitable giggle with the other children. Several months later, the neurologist diagnosed Hayden as having cerebral palsy, mainly affecting his limbs. While Grandfather and I were saddened by this news we felt encouraged when we read every word that lay people could find, and learned that CP does not actually become “worse” with age. The limitations a person receives in the beginning do not change. Nonetheless we were affected by the news. We cried and tried to comfort each other, wondering what his actual “limitations” would be? Every time I passed a school playground I peered into the groups of children, hoping to spot a “disabled” child who was happily playing with other children. How badly affected would he be? Would he be able to catch a ball or go trout fishing with his father and mother using the fly rod he had gotten at his baptism?
After the CP diagnosis, the doctors recommended that Hayden have physical and occupational therapy. Twice a week he had sessions with therapists who worked with rolling balls and stretching, holding different objects, blowing bubbles. Alison, Hayden’s mother, made a beautiful chart to record a detailed account of the exercises, and the numbers of repetitions he accomplished. We tried to learn these routines. Usually we were not able to be helpful because Tim worked so diligently, getting up especially early before work, then working again when he came home, and Hayden got too tired to work more than a couple of times a day. In the few weeks after the PT began, his parents thought Hayden seemed more active and able to move his limbs. I thought this was proven when I watched this child, who could not sit or crawl, determinedly pull himself, crabwise, towards two handles he could see on a chest. He then tried to pull himself up repeatedly until, exhausted, he fell back to the floor. Several months later, Hayden’s parents assembled the four therapists who had been working with him to assess his progress. It was the therapists who gave the opinion, by stating that the little boy was actually 40 40 regressing, that there was a need for another visit to the neurologist. The neurologist sent the family to an eye specialist, who discovered the dread ”scarlet red spot” in his eye, a defining mark for the diagnosis of Tay-Sachs disease.
Shortly after the diagnosis, Tim and Alison invited both families to be with them for the weekend. Tim’s brother, Charlie was there. We realized, more than ever, in spite of this devastating news, that Hayden’s life would be as comfortable, as joyful, and as fully loved—and lived—as it could possibly be. As grandparents, all four of us were committed to helping achieve this potential. After our dinner on that night in November 1999, Grandfather Charlie and I walked son Charlie home to the place where he was staying. As we turned to go, I reached up to give our son a hug. I said: “I hope you never have to see your adult child suffer.” It was a terrible realization that we could no longer be the “fixers” of children’s problems. The feeling of that power embedded in parental spirits was gone. I had no idea how tragically prophetic my words to our son would be.
Immediately, knowing that, as an identical twin, Charlie would be a carrier of the disease, Blyth went for the test. Only a short time after having heard about Hayden, when Blyth found that she too tested positive for Tay-Sachs, Cameron was taken to be tested. Not long after Hayden’s parents heard the news in New York, Charlie and Blyth were in their pediatrician’s office in Cambridge. They learned then that Cameron, who had been developing perfectly normally as most Tay-Sachs babies do in the first 6 months, was also afflicted. Later, in a video made for distribution to pediatric departments in medical schools, this doctor expressed the anguish he felt having to tell parents that “a perfect 6 month old” would regress steadily and not live to be more than a few years of age.
It was at this point that Grandfather Charlie and I finally found a therapist. We had been interviewing people for a couple of months and were pleased to have found the wonderful woman who would be with us throughout this journey of anguish, as we saw two babies and their parents move on through life and towards the inevitable deaths of the little children. We now had someone who helped us cope with the times of anguish and the questions of how to help and how to care for ourselves and each other.
In the next weeks, all four parents decided that they would care for the children at home, in the most comfortable, pain-free environment possible. All four wanted to avoid hospital care completely. Tim and Alison had experienced the hospital only once when Hayden had pneumonia. The noises of bells and alarms and cleaning implements and other distractions and unfamiliar hands and movements cause TaySachs babies, who never lose their “infant startle reaction,” to be miserable. When cared for quietly by people who are familiar to them, babies with the disease are generally calm and gently responsive, rarely screaming or agitated. Cameron’s father said that he wished for her “an arc of life, with a beginning, a middle, and end, all filled with music, “play,” sun, and nature, using every palliative measure possible. Both families agreed that, when the swallowing reflex began to fail, as happens with Tay-Sachs infants, there would be no feeding tubes, only home-made healthy food, given with the most natural feeding devices available while being held in a parent’s arms. Both families put ample queen beds in the babies’ rooms so that a parent or grandparent, brother or sister could spend the night with them, for warmth and companionship, to calm a seizure or, perhaps, to suction them if needed. Cameron’s uncle made a special low chair with a table attached so that Cameron could easily reach and play with her stuffed animals toys and books. This was the first destination for three-year-old Taylor when she ran in after school to be with her sister. It is hard to say enough about Charlie and Tim’s sister, Deirdre who was there in Boston and New York whenever most needed. She coped with crises of needed medication and sudden emergencies. And she took from the rest of us some of the most painful end-of life burdens, which she would not let the parents or grandparents bear.
Tim and Aliey had a group of friends who wanted to help and were enlisted to take turns on Thursday nights to be with baby while he slept, so that the parents could have a “date night” alone.
Our children found ways, often with the help of therapists, to do the most for these children. Now, what could the grandparents do to be helpful to these families?
We began by saying that we would come to them any time they needed extra hands. All four parents were fortunate enough to be able to continue with their jobs in both Massachusetts and New York, where insurance paid for child care. However they did need respite from the intense involvement with the care of children who needed constant care, attention, and whose complex needs were ever changing. So we did go to stay. We went to help with feeding, to go for walks in good weather in the complex carriages designed for babies with these special needs. We read aloud and sometimes slept in those big beds next to the little ones. At first I felt insecure with Cameron at night, fearing that I might disturb her sleep or be unable to do the small things needed during the night: suctioning to remove mucus, calm her after a seizure (which we had been taught to do), or turn her to a more comfortable position. These nights became an important part of the wonder of being in the loving world of these families.
We tried to find ways that the parents could get away for time both with, and occasionally without, the babies. On weekends when they were away, we stayed in the apartment and helped the baby sitter for a couple of days. One winter we found a friend’s house in Florida where each family could go for a week. The house had a heated pool where Hayden spent many hours in his enormous “treatment” pool. For Cameron’s family that cold Florida winter did not allow for swimming though watching the waves and escaping New England winter gave the parents a break.
Each year the National Tay-Sachs organization hosted a conference in a different part of the country for families with children affected with the disease, and their siblings. Available child care gave families time to compare issues and treatments with others, and to learn about current research into possible treatments and eventual cures for the disease. At meetings for the attending grandparents, we learned that not all grandparents were willing to be as involved as we were. Some were hesitant and afraid, a few were resentful of the disease to the extent that they lost contact with their children. These children suffered the loss of both the emotional and practical help that grandparents can give. How wonderful it was that, in our case, all three pairs of the grandparents were quite close. We have them to thank for wonderful times together
Another way to help the parents was to accompany them to doctor’s appointments. One visit was particularly relevant for us. We were with Dr. Gold, an elderly man with a shock of silver hair and large hands that were both strong and soothing. He had been a pioneer in the care of Tay-Sachs babies soon after the disease was named for the two doctors by whom it was identified. When he came back into the room, after examining Hayden, where we four were waiting, he turned to Grandfather Charlie and me. “How do you two grandparents feel?” Our answer: “We are proud of our children.”
Hayden died in December 2000, with family coming quietly in and out of the apartment to be with his parents. Cameron died in May 2001, four days after a dancing 2nd birthday party, having been surrounded with family, friends, and flowers. We thought that the most important things we could do for our children, and for ourselves, were to find all the occasions we could to comfort, hold, and care for the babies, to know their love, and to recognize them as individuals. These little beings, like all humans, were quite different from each other. Hayden was strong, solid, determined, and funny. His father once said that when he looked at Hayden, he saw his brother. Cameron was alert from birth, never taking her eyes away from the person she was with; and she was gentle, delicate, peaceful, and aware. I thought she would grow up to be kind, as well as competent like her mother. These children gave us so much more than we could give them.
Wordsworth said this in “Ode to Immortality”
“But trailing clouds of glory do we come From god, who is our home? Heaven lies about us in our infancy! “