07:14am Friday 20 October 2017

CF Awareness Month: improving patients’ lives, one step at a time

Madison Phipps (right) with her mother, Shelley.

Madison Phipps (right) with her mother, Shelley

Phipps lives with cystic fibrosis, which makes her feat that much more extraordinary.

Cystic fibrosis is an inherited chronic disease that affects the lungs and digestive system. A defective gene and its protein product cause the body to produce unusually thick, sticky mucus that clings to the lungs and leads to life-threatening lung infections. The mucus in the digestive tract stops natural enzymes from helping the body break down and absorb food.

CF is the most common fatal genetic disease affecting Canadian children and young adults.

Phipps was diagnosed with CF a day after she was born. She wasn’t eating properly and was having difficulty breathing. A bowel obstruction sent her to a Toronto hospital, where a section of her intestines was removed. Phipps spent the first few weeks of her life in the NICU.

Phipps estimates she’s spent 300 days in hospital, having been admitted a number of times with pneumonia.

About three years ago, Phipps started taking Kalydeco, a groundbreaking medication that helps a small number of CF patients breathe easier. Kalydeco is the only drug that treats the underlying cause of CF: a faulty gene and its protein product.

The drug was approved by Health Canada in 2012 for use in people with CF who carry the G551D mutation. Ontario agreed to fund the drug for CF patients last fall. St. Michael’s was the only site in Canada to participate in the early Phase 2 clinical trials for the drug, and had the largest number of patients enrolled in the Phase 3 trial.

Dr. Elizabeth Tullis, director of the hospital’s Adult Cystic Fibrosis Clinic, the largest in North America, was instrumental in helping to ensure Kalydeco was available to Canadians like Phipps.

Dr. Tullis, a respirologist and clinical researcher at St. Michael’s, was recently appointed the first Cystic Fibrosis Canada Chair in Adult Cystic Fibrosis Research. The chair will support research and innovation in care to adults with CF.

“This is an exciting time in CF, where our understanding of the basic problem in CF is leading to novel therapies,” said Dr. Tullis. “I am hopeful that medications like the one that Madison is on will help improve the life expectancy and quality of life of our patients. The CF chair position will help to support this link between our research and the application of new treatments.”

Phipps said both she and her mom have noticed a difference in the young woman’s ability to breathe.

Phipps used to miss dance classes because she found herself gasping for air. Now, she is full of energy to keep up with school.

“I have such a long future to look forward to,” said the health sciences major, who said she was pursuing that field because she wanted to “help other people, just like the doctors helped me.”

About St. Michael’s Hospital

St. Michael’s Hospital provides compassionate care to all who enter its doors. The hospital also provides outstanding medical education to future health care professionals in 27 academic disciplines. Critical care and trauma, heart disease, neurosurgery, diabetes, cancer care, care of the homeless and global health are among the hospital’s recognized areas of expertise. Through the Keenan Research Centre and the Li Ka Shing International Healthcare Education Centre, which make up the Li Ka Shing Knowledge Institute, research and education at St. Michael’s Hospital are recognized and make an impact around the world. Founded in 1892, the hospital is fully affiliated with the University of Toronto.


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