The BC Children’s Hospital is celebrating a major milestone: 50 years of clinical care for young patients living with cystic fibrosis (CF).
Great strides have taken place since the clinic first opened its doors in 1965. At that time, most children did not live long enough to experience many of life’s precious gifts, including their first day of school. Today, according to Cystic Fibrosis Canada, nearly 60 per cent of people living with the disease in this country are adults and many are expected to live well into their 50s and beyond.
The clinic, its caregivers and the medical researchers who work alongside of them are a huge part of that achievement.
“There have been major advances in treatment,” says Dr. David Speert, Senior Clinician Scientist and Head of theCentre for Understanding and Preventing Infection in Children at the Child and Family Research Institute.
“So many things have changed. It is very hard to identify just which one has been most responsible [for increased life expectancy]. There are better antibiotics than there had been years ago. Dietary care has changed substantially and physiotherapy has improved. Lung transplantation, which was brought in more than 35 years ago, has saved the lives of patients in end stage respiratory disease.”
Cystic fibrosis is the most common fatal genetic disease affecting Canadian children and young adults; approximately one in every 3,600 children is born with it. They inherit a defective gene called cystic fibrosis transmembrane conductance regulator (CFTR). The protein produced by this gene affects cells that produce mucus, sweat and digestive juices. The mucus blocks ducts in the pancreas and creates an ideal breeding ground for bacteria, causing severe damage to the lungs and the digestive system.
Currently, there is no cure for CF and researchers have no idea if one will be found in the future. However, according to Dr. Speert, there is a lot of work going on right now to come up with new drugs to correct defects in patients who possess the more common forms of mutations created by the protein.
“A huge breakthrough would be prevention of infection,” says Dr. Speert. “If there was a way to prevent patients from becoming infected, that would make a tremendous difference. Infections are the leading cause of death of patients with CF.”
Luca Piccolo’s parents learned he had CF when he was just two years old. Today, the 14-year-old manages his disease through a strict regimen of physiotherapy and daily medication.
“I take 45 pills a day, sometimes even more if I have to take antibiotics to fight off an infection in my lungs,” says Luca. “CF is a 24/7 disease. That’s why the caregivers at BC Children’s Hospital keep a close eye on my health.”
Luca is examined by a physician at the CF clinic every few months. The doctors regularly check his Positive Expiratory Mask (PEP) mask and nebulizer to make sure they are doing their job of keeping his airways open and removing the mucus from his lungs.
Once Luca turns 18, he will be transferred to an adult clinic. In the meantime, his mother, Lisa, believes he is receiving the best care possible here in Vancouver.
“BC Children’s Hospital is one of the best centres in the world to treat children with CF,” says Lisa. “We owe Luca’s life to the hospital, so we can’t really say thank you enough.”
For more on the Cystic Fibrosis Clinic at BC Children's Hospital, visit: www.bcchildrens.ca/our-services/clinics/cystic-fibrosis