When Jeff turned 6 years old he weighed just 30 pounds. His mother had worried about her little boy's health for years through the continual coughs, colds, and his overall failure to thrive. Jeff's pediatrician had suggested, much to his mother's dismay, that perhaps she wasn't feeding the boy correctly.
When Jeff's one-year-old sister nearly died of dehydration, however, and the girl was rushed to a hospital in a nearby city, doctors diagnosed both children with cystic fibrosis. It was 1969, and the average life expectancy of children with CF was a short 12 years.
The average life expectancy for people with cystic fibrosis grew right along with Jeff. Today it hovers close to 38 years, the same age as Jeff; his health has kept pace with and benefited from the medical advancements made in the treatment of CF over the years. They are advancements that have resulted in a growing adult CF population.
Dr. Bruce Marshall, the vice president of clinical affairs at the national office of the Cystic Fibrosis Foundation, says that stories like Jeff's have driven the creation of a new adult-care initiative at the CFF.
“About 45 percent of the U.S. population is currently18 and above,” says the pulmonologist, who joined the foundation in 2002. “Almost all of our patients are now making it through childhood and into adult care.”
The healthcare needs of that burgeoning adult CF population are putting a strain on existing adult care programs, and the CFF has responded with the Program for Adult Care Excellence, or PACE.
Launched in April 2008, and designed to populate adult clinics with more providers, PACE designates funds to recruit and train more CF doctors and fellows. The foundation hopes to add 40 new adult-care providers to centers nationwide through initiative efforts, a step Marshall hopes will provide important back up to thinly-staffed adult clinics.
“We did a survey and asked adult-care providers, 'If you were suddenly recruited away and you could no longer deliver adult care at your center, would there be a major disruption in service?' Over half of those who responded said 'Yes.'”
Marshall says the foundation is identifying regions in the country where there is an acute need for additional adult providers In the long run, it is the ever-increasing adult CF population that will be the real winners.
In 1955, when the CFF was first established, children rarely lived past the age of 6. Improved treatments for cystic fibrosis over the last 50-plus years, however, have resulted in adults, like Jeff, who are living far beyond their childhood prognosis.
“It's still a devastating disease, and we have a long way to go,” says Marshall, “but it's not like it was 30 years ago. Our dream is for people to die with CF, not of CF.”