Alberta Cancer Foundation

New Challenges

When Paula Holman of Sherwood Park was diagnosed with Down syndrome 23 years ago, her family doctor gave her mother and father the same advice he gave all new parents: “She just needs your love. She needs to be fed. She needs to be played with. She’s just like any other child.”

That was true – with a few important exceptions, which Jane and Ken discovered when they did some research. In addition to developmental and cognitive delays, children with Down syndrome have slightly higher incidences of heart defects, leukemia, diabetes, thyroid dysfunction, and problems with hearing and vision.

By the time Paula was two and a half, she was a veteran of the operating room, having undergone major surgeries to fix two large holes in her heart, and numerous day surgeries for less serious ailments. But for the next dozen years she was relatively healthy. The only time Jane and Ken thought about cancer was when Jane’s mother died of leukemia and their older daughter was successfully treated for a benign brain tumor.

When Paula, then 15, developed a stomachache that wouldn’t go away, Jane and Ken thought it may have been stress related, caused by problems at school. Paula assured them school was fine. They began sending her to sleep with a heating pad, which seemed to help.

After a few weeks of doctors’ visits and worry, spots appeared under one of Paula’s arms. Jane brought her to the family doctor, who treated her for a staph infection. Three days later, when Paula developed another stomachache, Jane brought her back to the doctor. When Paula lay on the exam table, it was clear that her abdomen was larger than normal. An ultrasound, blood tests, and CT scan revealed that she had a dysgerminoma, a fast-growing tumor. A biopsy led to a diagnosis of stage-III ovarian cancer.

Dysgerminomas are most common to people in their teens and 20s, but not to people with Down syndrome, says Dr. Beverly Wilson, a pediatric oncologist and director of the Northern Alberta Children’s Cancer Program. But leukemia affects children with Down syndrome at 10 to 20 times the rate of children in the general population.

The good news is that studies show that children with Down syndrome generally respond to leukemia treatment better than children in the general population. The bad news is, like many people being treated for any form of cancer (including people who do not have Down syndrome), complications are not unusual.

The Holmans learned about complications within minutes of Paula’s first chemotherapy treatment at the Stollery Children’s Hospital in Edmonton, early in the summer of 2007, when her throat began to close. Nurses wheeled in a crash cart and pumped her with Benadryl, which made it possible for her to handle the treatment when they restarted it a few hours later.

In the four months during which she underwent chemotherapy, Paula lost her hair, suffered kidney damage, developed marks on her arms, gained weight, and experienced nausea, vomiting, heartburn, constipation and extreme fatigue. She also encountered significant setbacks in her intellectual development, which is typical for cancer patients; studies have shown that the chemotherapy they receive via their spinal fluid can cause problems with learning, Dr. Wilson says. But dealing with any kind of cancer and Down syndrome poses unique problems both for the medical team and the patient.

Children with Down syndrome often have pre-existing health conditions that require alterations to their treatment, Dr. Wilson says. They also have problems with their immune systems, which puts them at an increased risk for infection – and death from infection. Developmental delays mean they often have trouble communicating. That, especially, was a problem for the Holmans.

“Instead of addressing Paula, sometimes staff would look to us to interpret for her,” Jane says. “It affected Paula’s self esteem negatively and she became very quiet and introverted, and at times unsure of herself and her situation.”

New Times: Paula Holman has learned to embrace the possibilities of a future after cancer.

Paula became profoundly depressed. Even though her prognosis was good, “we were all waiting for the second shoe to drop,” her mother recalls. “Most significantly, Paula didn’t believe she was going to get better, which was devastating to a previously outgoing and gregarious 15-year-old.”

Jane credits the team at the Stollery with helping the family get through the experience, particularly the neuropsychologist who worked one-on-one with Paula. “She gave her a platform to express herself completely free of her parents,” Jane says. “It really helped her to open up and talk about her fears with someone who understood, but who wasn’t a family member. It helped her deal with her cancer experience and learn how to look forward to the future and to put her cancer diagnosis behind her.”

Ayden Gamble had a harder time explaining his fears. The Sherwood Park pre-schooler was four years old when he was diagnosed with acute lymphoblastic leukemia five years ago. Like many children with Down syndrome, his speech was delayed. He and his family communicated with sign language and picture cues. To soothe himself at home, he would often lay on his stomach and bang his chin on his hands.

Three weeks into a three-month stay at the Stollery for chemotherapy, Ayden had a neurotoxic reaction to one of the drugs. His vocal chords were paralyzed open. His breathing turned hoarse, his oxygen saturation dropped, his heart rate soared, and a tube was inserted up his nose to feed him. The tube made it harder for him to breathe.

Unable to articulate his frustrations, Ayden yelled and banged his head. Hospital staff padded his crib so he wouldn’t hurt himself. The pediatric psychiatrist gave him antipsychotic medications. “He seemed even more agitated with the drugs,” recalls his mother, Angela Samis. “There was no calming him at all.”

Ayden spent five nights in the pediatric intensive care unit at the Stollery Children’s Hospital. Back on the cancer unit, his home for three and a half months, Ayden continued with the chemotherapy – and the side effects. In addition to hair loss and vomiting, he developed ulcers in his mouth and through to his digestive tract. His skin chapped and peeled off and became easily infected. Yet once he recovered from the initial unpleasantness that led to his stay in the PICU, Ayden remained cheerful.

“Nurses would poke him, prod him, do everything to him, and he would hug them afterwards,” his mother recalls. “He is very forgiving.”

Ayden was on chemotherapy for three and a half years and, while he went back and forth to the hospital, he took most of his treatment at home. His post-treatment tests have shown that he is cancer-free.

Paula, who is studying at MacEwan University to become a special needs educational assistant, also received the all-clear at her post-treatment checkups. However, she continued suffering from exhaustion and stomach pain even as post-treatment blood tests showed she no longer had cancer.

Three years ago Paula was tested for celiac disease and found she had an advanced case. It’s another complication common to people with Down syndrome. And while Jane wonders whether it was triggered by the cancer or the treatment, she’s mostly relieved that Paula has been able to treat it with a change in diet.

Paula is also dealing with the early onset of menopause that is the result of her surgery and chemotherapy. But in typical fashion, she remains optimistic and enthusiastic, not letting anything stop her from achieving her goals. She wants to get a job when she graduates and move into her own apartment when she is 25 years old. “I want to find a good man to marry,” she says.

Still, the cancer diagnosis isn’t ever far from Ken and Jane’s thoughts. “You’d think we’d have accepted her developmental disabilities by the time she was 15, but a cancer diagnosis really put it into perspective for us, that life is here to cherish,” Jane says. “Every day we have together and each accomplishment Paula has is truly a blessing.”

Illustration by Lisa Rebnord

Ups & Downs: The Calgary Down Syndrome Association, aims to enrich “the lives of individuals with Down syndrome and their families by providing a network of resources, sharing common experiences, learning together and creating awareness.”, (403) 289-4394

Edmonton Down Syndrome Society aims to “benefit persons with Down syndrome and their families by providing information and support, facilitating services and promoting inclusion through public awareness and advocacy.”, (780) 944-4224

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