First Cystic Fibrosis Day to be held Sept. 8
Cystic Fibrosis Worldwide (CFW) will host the first Worldwide Cystic Fibrosis Day on Sept. 8 with the support of their 62 member countries and Pennsylvania Cystic Fibrosis Inc. (PACFI), will join them in celebrating the event.
“We’re glad CFW is hosting this event,” Betty Hollenbach, PACFI president said, “because it helps our organization by making more people aware of cystic fibrosis (CF) and its major impact on children and young adults.” Hollenbach has a grandson with the disease.
CF is a life-threatening genetic disease that primarily affects the lungs and digestive system, and currently the average life span is 37 years. A defective gene causes the body to secrete thick mucus that clogs the lungs and other organs leading to major infections. About 30,000 people in the United States have CF and 10 million people in the nation carry the CF gene.
If both a male and female have the CF gene and decide to have children, the odds are one-in-four that they will have a child with CF. A carrier test is available to determine if a person has the CF gene.
On Sept. 8, 1989, Dr. Lap-Chee Tsiu’s discovery of the CF gene was published. Dr. Tsiu worked in the department of genetics at the Hospital for Sick Children in Toronto where he received a Distinguished Scientific Achievement Award for his discovery. Tsiu’s discovery was lauded as one of the most significant in human genetics and it opened the door to more research into long-term beneficial treatments or a cure for the disease.
“We are really focused on making this first Worldwide CF Day a huge success,” said Christine Noke, executive director of CF Worldwide who is based in Izmir, Turkey. “The major goal is to spread awareness about this life-threatening genetic disease.”
Noke also noted another goal is to offer members an international platform to raise funds, gain volunteers and lobby local officials to support patients and research.
“PACFI was formed in 1985 and we’re dedicated to providing services and supports for Pennsylvania individuals and families affected by the disease,” Bob Derr, founder and public relations person for PACFI, said. “We also allocate funds to CF researchers every year, and we applaud CFW’s effort in hosting Worldwide CF Day.”
Derr’s son, Burke P. Derr, died due to CF in 1997 a few months after two of Burke’s friends convinced The Boyds Collection to name a teddy bear in his honor. PACFI began the Million Dollar Bear campaign and Burke’s Tour in late 1997 as a way to increase awareness and raise funds for research. Co-sponsored by former state representatives Merle Phillips and Russ Fairchild, House Resolution 198 was unanimously passed by the Pennsylvania legislature in 1999 titling Burke P. Bear as “The Pennsylvania Ambassador for Love, Peace, Having Fun and Curing Cystic Fibrosis.” Dubbed the “Globe-Trotting Ambassador of Love” by a British magazine, Burke P. Bear has toured 47 states and 23 world countries in raising more than $450,000 for CF research. The bear hopes to visit Nebraska, South Dakota and Rhode Island by the end of this year to finish his U.S. Tour.
“We’ve also raised over $300,000 in supporting the needs of Pennsylvania citizens affected by the disease,” Derr added.
PACFI provides air conditioners, portable nebulizers, other CF-related equipment, medications and travel expenses not totally covered by insurance, scholarships for students with CF who want to continue their education, advocacy services for families and students, and educational in-services to schools and community organizations.
“CF Worldwide has partnered with PACFI and Burke P. Bear to raise funds and awareness about CF in developing countries,” Noke said. “PACFI is one of the few non-governmental organizations (NGOs) out there that still operates with volunteers only and are totally dedicated to helping people who suffer from cystic fibrosis.”
Derr and his wife, Linda, worked with Noke in having Burke P. Bear create awareness and raise funds for CF clinics in Tblisi, Georgia (of the former Soviet Union), India and other countries. PACFI has sent Burke P. Bears to England, Ireland, Australia and other countries at no cost to the organization.
With an extensive international network of parents, patients, medical professionals and researchers, PACFI sends donated equipment, enzymes and-or vitamins to other countries when the receiver is willing to pay the shipping costs. PACFI is known throughout the worldwide CF community. It is the only organization of its kind in the nation.
PACFI is an independent, nonprofit, all volunteer 501(c)(3) organization that provides services and supports to Pennsylvania citizens affected by CF and funds some of the most progressive CF research in the nation.
Last year, PACFI funded its first international researcher at the University of Rome.
More information is available on the organization’s web site at WEBSITE. Donations to PACFI in honor of Worldwide CF Day can be mailed to PACFI, P.O. Box 29, Mifflinburg, PA 17844.