Facing Crohn's disease together
Many people living with chronic illness for a significant amount of time become experts about their disease through experience. Shannon Cushman, 36, of Strathroy Ontario, has been living with Crohn’s disease since she was nine years old; 27 years of understanding her symptoms, and managing her disease. When her daughter, Ava Hilton, 12, become inexplicably ill, she could not have envisioned Ava would be diagnosed with the same disease in the same hospital; nor could she envision the unique bond that they would forge.
Crohn's disease is a type of Inflammatory Bowel Disease that causes inflammation of the digestive tract. “Crohn’s disease is considered to be a “gum to bum” disease because the inflammation can present anywhere in the digestive tract. It is an autoimmune disease with no known cause, and no cure, however we have very effective treatment options,” says Melanie Watson, Nurse Practitioner, Paediatric Medical Day Unit (PMDU), Children’s Hospital, London Health Sciences Centre.
Shannon and Ava have very different presentations of Crohn’s. “Before my diagnosis, I was in constant pain, had severe gastrointestinal symptoms, and certain foods would trigger me to feel worse. Ava showed none of these symptoms,” explains Shannon. “Ava was not growing, she was fatigued, looked either pale or too flush, and experienced regular fevers.”
Ava, then 11, had not grown for over a year, and was small for her age, especially compared to her classmates and siblings. “Even though I was sick, I didn’t miss school. I was very tired sometimes. I preferred staying inside and drawing or reading.”
In their search for answers, the family were referred to endocrinology and later hematology at Children’s Hospital at London Health Sciences Centre. Because Ava experienced no gastrointestinal symptoms, but showed very low hemoglobin and iron levels, she was treated with a high dose iron supplement. Unfortunately, she did not respond to the treatment and with normal growth hormone production and no genetic abnormalities she was referred to gastroenterology for further testing. A colonoscopy and endoscopy under general anaesthesia revealed so much active disease that Ava was diagnosed with Crohn’s disease immediately.
For Shannon, the diagnosis came with a wave of emotion, “worry, fear, guilt, relief.” Shannon did not want her daughter to experience the same invasive diagnostic and surgical procedures that she endured. “Within two years of my own diagnosis, I had undergone a bowel resection leaving me a six-inch scar across my abdomen, I missed a full term of school, and was unrecognizable due to bloating as a result of medication,” Shannon recalls. She would also be humbled in her own knowledge of Crohn’s and relieved in the medical progress made since her childhood. “From the less invasive diagnostics, to the treatment options presented to us, it’s like a whole new world of medicine.”
“Ava’s case is an example of how Crohn’s disease can cause various signs and symptoms in different people. For many people, abdominal pain and bloody diarrhea can be the first clue to a diagnosis of an Inflammatory Bowel Disease. However, when patients present with subtler signs, like growth failure, it can make the diagnosis more challenging. Ava’s disease was located in the part of her bowel that absorbs nutrients, which made it harder for her to grow and thrive,” says Watson.
As a Nurse Practitioner, Watson is part of a multidisciplinary team that cares for children diagnosed with Inflammatory Bowel Disease. She supports patients and their families by offering treatment options, education, health maintenance strategies, and monitors patients for disease recurrence and medication side effects. “When speaking with Shannon and Ava, we demystified the current treatment options for Shannon as she came with her own experiences, and spent time educating them about how current practices will benefit Ava.”
Ava was presented with treatment options including a biologic infusion treatment in hospital, which would start with every two weeks and eventually decrease to once every eight weeks, or injections, done at home initially every other day. Ava opted to avoid regular injections and receive infusions at the PMDU once a month for approximately five and a half hours. She also takes medication once a week at home, as well as vitamin supplements.
“Ava was given a voice in her care,” says Shannon. “We are grateful for the specialists who helped diagnose Ava, for the wonderful staff and nurses at Children’s Hospital, including the PMDU, and for the timely, personal care she receives.”
In treatment, Ava’s experience also differs from Shannon’s. Shannon was lonely and isolated because incidents of Crohn’s disease in children were rarer. Ava sees other kids and is kept busy, “There are lots of kids who receive treatment when I do,” explains Ava. “I read, watch something on my iPad, or play video games in the hospital.” Ava also sometimes plays board games or does a craft with a Child Life Specialist, who helps children feel at ease while in hospital or receiving treatment. Shannon relied more heavily on her physician, Dr. John Howard, now retired, who was a “light in the darkness,” and who provided care to her for more than two decades.
Shannon, and Ava’s father, Darryl Hilton, alternate bringing her to treatment to balance their time away from work and try to lighten the day for Ava. Mom and daughter have created a routine for the days Ava receives her infusion. “We try to make it into a special day. We’ll have lunch out, have a movie night, or maybe go shopping if I’m feeling ok,” says Ava. In her mom, she also has someone who can answer her questions and truly empathize with her feelings, as well as compassionate siblings in Emma, 11, Kate, 8 Blake, 5, and Tyler, 3.
In the year since Ava began her infusions, she has grown two clothing sizes, can run around outside, and has outgrown many pairs of shoes.
“Ava is resilient. She keeps moving forward without complaint. Her personality shines through,” says Shannon proudly. Seeing how far the care and management of Crohn’s has come has also given hope to Shannon for a bright future for Ava.
Whatever that future holds, Shannon and Ava will tackle it together.