Fostering independence and good health in CF patients
Cystic fibrosis patients are living longer thanks to innovative new therapies, writes Isabelle Mailloux Pulkinghorn.
At 18 years of age Canadian youth treated at pediatric hospitals transition to the adult system. For the staff at the Children’s Hospital of Eastern Ontario (CHEO), that ‘graduation’ is a bittersweet day because they have developed relationships with the patient and their families. But when it comes to patients affected with cystic fibrosis (CF), that transition is an exciting one because only a few decades ago, not many children with CF reached adulthood.
Cystic fibrosis is a genetic disease caused by a defective gene that causes the body to produce thick mucus. It is a multi-system malady that affects mainly the lungs and the digestive tract. In the chest, a build-up of thick phlegm causes severe respiratory problems and in the digestive tract mucus and protein accumulate, making it difficult for CF patients to digest food and absorb nutrients. The accumulation of mucus results in life-threatening lung infections and serious digestive problems, and can also affect growth and the patient’s overall healthy development.
To this day there is no cure, making CF the most common fatal disease affecting Canadian children, youths and adults. On average, one person dies each week from cystic fibrosis.
“It is heartening to see that the adult clinic at The Ottawa Hospital (TOH) is growing and now sees more patients than we do here at CHEO,” says Anne Smith, clinical nurse coordinator at the CF clinic at CHEO. “When I began my career, there were no adult CF clinics because patients didn’t survive childhood.”
Twenty-two-year-old Jessica Forsyth, diagnosed with CF as an infant, was followed at CHEO for 18 years. Today, she is a patient at The Ottawa Hospital’s General Campus CF clinic.
“The transition was made easy because I was gradually taught to manage my illness, and because the staff in both facilities met to discuss my needs,” says Jessica. “They are so invested in you and in making sure you do well, that it fosters your confidence. It felt like an extended circle of care.”
Both CHEO and TOH have CF multidisciplinary teams made of respirologists, a gastroenterologist, a coordinating nurse, a dietician, a pharmacist, a respiratory therapist, a social worker, and a physiotherapist. Treatment plans are built specifically for each patient and take into account various factors such as the patients’ age, the stage of the disease and the organs that are affected. A year prior to the time a young patient needs to transition over to the adult facility, the two hospitals’ CF medical teams begin discussing that patient’s health and specific needs to ensure uninterrupted care.
The medical staff at the CF clinic at CHEO begins to educate and empower patients as young as five years old. At that age they can start to help their parents by squirting medicine in their inhaler mask or by counting their enzymes to take with their meals; then at about age 10 they are taught to make their own snacks and lunches. At age 13 they begin to meet with the medical staff one on one, without mom and dad in the same room so they can learn to communicate their needs, talk about their symptoms and learn to advocate for their needs. Moments later, a second meeting takes place for the staff to discuss the same topics with the parents to see how well the young patient did.
“As children learn to care for their own needs and speak for themselves, it prepares them for life in the real world - like at school - where they still need to take their enzymes prior to lunch, without their parents there to remind them to do so,” explains Anne. “It also prepares the child to gradually gain control of their medication, nutrition, inhalation therapy and the overall communication with their medical team and with family and friends.”
That transition plan is set in place early so patients can learn to play an active role in their care since there is a direct correlation between adherence to care plans and increased knowledge. The fact that CHEO and The Ottawa Hospital have similar multidisciplinary teams in place is a luxury not all hospitals can enjoy; yet it goes a long way to ease the transition so young patients are able to focus on their health and not worry about any changes to their medical team.
“You can tell that both facilities share a same goal – that they want to help us manage our illness so we can live our best life,” adds Jessica. “A year before I turned 18 ,Anne came with me to The Ottawa Hospital to introduce me to the teamover there, and she even came to my first appointment as an adult. I was then able to go on my own and the team at TOH helped me ease into a new routine. It was a really smooth transition.”
Because CF is such a complex disease that not only affects many parts of the body, but also presents itself with varying degrees depending on cell mutations, it requires proper care by a whole team of specialists.
Though Jessica is supported by a stellar team, she is solely responsible for her choices and the way she cares for herself, something which she admits can at times be quite daunting. But she has been well trained to be independent in managing her disease and she can depend on her CF team to support her as she makes big decisions such as deciding to have a lung transplant.
“I feel lucky to see our patients become adults and go on to have careers and families of their own,” says Anne. “I know Jessica and our other patients are in great hand sat The Ottawa Hospital, which makes this transition such a happy and meaningful one for our team.”
Thanks to innovative new treatments, people with CF are now “aging.” Their quality of life has also dramatically improved thanks to early detection and treatment. As newborn screening initiatives spread across the country, so does the outlook for CF patients. The average age of survival in Canada is now 48, one of the highest in the world due to recent developments in drug therapies and advances made in genetic testing.