Discoveries contributed to national infection control guidelines and improved lab testing

For 30 years, researchers led by Dr. David Speert at the Child & Family Research Institute at BC Children’s Hospital have collected and stored bacteria that cause devastating infections in children and adults with cystic fibrosis (CF). Dr. Speert founded the CF Repository in 1981 and today it contains over 14,000 samples of two types of frozen bacteria.

Understanding the bacteria Pseudomonas aeruginosa and Burkholderia cepacia complex is critical to finding ways of helping children and adults with CF overcome deadly lung infections. Normally harmless to most people, these two bacteria are very dangerous for people with CF.

The most common fatal genetic disease affecting young Canadians, CF primarily affects the lungs and digestive system. In the lungs, a build-up of thick mucus causes severe respiratory problems. This leads to a cycle of recurring lung infections and inflammation, often causing permanent lung damage and lung disease, which is the primary cause of CF-related deaths. One in every 3,600 children born in Canada has CF and there is no known cure.

Over the past 30 years, Dr. Speert’s team has published more than 100 scientific papers based on the CF Repository. Many of these findings have led to major changes to patient care.

Highlights of research discoveries from the Cystic Fibrosis Repository:

  • Determining key features of Pseudomonas bacteria that are unique to CF lung infections. [link to research paper]
  • Discovering some of the science that informed new national infection control guidelines that helped decrease transmission of Burkholderia between patients. [link to research paper] [link to national infection control guidelines]
  • Designing a new method for identifying B. cepacia in the laboratory. This method made it faster to detect the bacteria, which allowed doctors to treat infections in CF patients sooner and to determine whether a patient needed to be isolated from other patients. This method was later recommended by The Manual of Clinical Microbiology to CF clinics worldwide as the most effective way to identify B. cepacia. [link to research paper]
  • Finding a genetic marker for several strains of B. cepacia, which allowed scientists to recognize which strains were more harmful to people with CF, leading to more personalized treatments. [link to research paper]
  • Discovering that using a specific antibiotic to treat patients infected with a specific strain of B. cepacia can increase the bacteria’s resistance to other antibiotics. This could help clinicians determine the type of treatment that would be most effective for CF patients who have this strain of bacteria. [link to research paper]
  • Understanding the biology that explains why Burkholderia can survive some of the most powerful antibiotics. [link to research paper]

In part because of the work of researchers like Dr. Speert and his colleagues, there are now, for the first time, more adult CF patients than there are children CF patients. Forty years ago, most CF patients did not live past age six and today, the median age of survival for Canadians with CF is estimated to be 46 years, according to a 2009 report from Cystic Fibrosis Canada.

Dr. Speert is Head of The Centre for Understanding and Preventing Infection in Children at the Child & Family Research Institute (CFRI); CFRI Scientist; Physician in the Division of Infectious & Immunological Diseases at BC Children’s Hospital; and Sauder Family Professor, Division of Infectious and Immunological Diseases in the Department of Pediatrics at the University of British Columbia.