Hope Abounds at Kit Taylor Lecture

“The New CF Therapies: Who Will Benefit?” was the title of the presentation given by Dr. Michael Boyle at the 32nd Kit Taylor Lecture on Tuesday, May 8, 2012. While some caution is still necessary, Dr. Boyle reported on the promising results from recent drug trials. The topic of the lecture was consistent with Dr. Boyle's experience and expertise. In addition to being the director of the Adult Cystic Fibrosis Program at Johns Hopkins University, he is also affiliated with the Cystic Fibrosis Foundation. He is active in CF related research including his role as a lead investigator for the current trials for the Vertex ivacaftor, VX-809 and VX-661 products. Dr. Boyle provided some background before delving into the new therapies. He started with a summary of statistics that demonstrate the progress that has been made in treating CF. Today, the median survival age is 38 years old – a significant gain over the pre-teen expectancy at the time Kit Taylor was diagnosed in 1958. He also noted that 2012 is projected to be a milestone year when the number of adult CF patients exceeds the number of pediatric patients. Dr. Boyle then presented an overview of the physiology of CF as a basis for understanding the new therapies. CF results from mutations to the cystic fibrosis transmembrane conductance regulator (CFTR) gene. This gene controls production of the CFTR protein which regulates the chloride (and maybe other) channels in epithelial cells that line the passageways of the lungs, pancreas, and other organs. The primary focus is on the lung epithelial cells since reduced lung function is the most critical symptom of CF. Defects in the CTFR gene interfere with the transport of chloride ions through the cells which, in turns disrupts the flow of water to the cell surface. The inadequate supply of water results in thick mucus that impedes the clearing of the lungs and promotes bacterial infections. Over 1,500 mutations have been identified that produce the range of dysfunctions presented as cystic fibrosis. These mutations have been categorized into five general classes that describe the nature of the dysfunction. The classes, dysfunction and rate of occurrence in the CF population are as follows: Most severe | | | Least severe Class I Class II Class III Class IV


New Scholarship for CF Patients

Applications are now being accepted for the Andrew W. Eve Memorial Scholarship. Andrew ("Drew" to his friends) lived a full life in his 33 years until he was overtaken by cystic fibrosis in 2007. Pursuant to Drew's wishes, moneys donated in his memory were used to establish a scholarship to encourage CF patients to pursue education in fields associated with public safety. Drew did not allow his CF to stand in the way of his dream of a career in public safety. After graduating from SUNY Geneseo with a degree in communications, he became a full-time dispatcher for the Livingston County 911 center. Drew also volunteered with the Livonia Fire Department where he attained the rank of Assistant Chief . He was also a certified State Fire Instructor and an Emergency Medical Technician. A highlight in Drew's career was being decorated for his actions in dealing with a fatal bus/tractor trailer accident in January 2009. Any CF patient is eligible to submit an application for an award of $1,000 from the Andrew W. Eve Memorial Scholarship. To be considered for an award, the applicant must attend an accredited college, including any campus of the State University of New York, with a major course of study in one of the following curriculums: Communications Fire Science Criminal Justice Emergency Medicine Police Science Applications will be considered, and awards will be made by, a committee comprised of Andy's family and friends. Click here for a copy of the application form. If you prefer, you can request an application form by completing and submitting the form below. [NOTE: The starred fields are required.] [customcontact form=6]


May is CF Awareness Month

Did you know? May is CF awareness month! Rochester is supporting those who share the CF journey with several notable events! Click on the dates in the list below to link to more information about the event. May 3rd - "65 Red Roses" - movie to be premiered on the Oprah Winfrey Network May 8th - 32nd Annual Kit Taylor Lecture - Come hear what Dr. Boyle, director of the Johns Hopkins adult CF center, has to offer this year! May 12th - The Power Of Two - CFFC is hosting a FREE screening of the beautiful documentary. In addition, local musician Scott Topel will be sharing his gifts with us and Starry Nites will be there providing their goodies as well (sales support CFFC). May 19th - CFF Great Strides - Rochester, Genesee Valley Park May 20th - CFF Great Strides - Geneva, Seneca Lake State Park June 2nd - CFF Great Strides - Hornell, Steuben Trust Company Come on out and enjoy what the Rochester CF Community has to offer!


Opening on CFF Education Committee

Are you passionate about CF education? Do you want to make an impact on the lives of people with CF and their families? If so, apply to be a member of the CF Foundation Education Committee. The CF Foundation Education Committee is a multidisciplinary team consisting of all members of a CF care team, including a parent and adult with CF, for the purpose of reviewing and recommending patient education materials for the cystic fibrosis community. There will be an opening for an adult with CF to serve on the committee as of October 2012 at NACFC. Each member's term is 3 years, which starts and ends at the time of the North American CF Conference (NACFC). The committee meets four times a year – during the NACFC, and then in February, May and August via teleconference. Responsibilities between meetings include: updating content of existing material, drafting and editing new materials, reviewing materials for Education Committee approval, and promoting awareness of materials available. The Committee also looks at areas of educational need for people living with CF and seeks out experts who can help fill in the gaps that have been identified. If you are an adult with CF and are interested in serving on the Committee, click here to apply: Please Note: The application takes approximately 20 minutes to complete. Once you start an application, you must finish it in its entirety, since you will NOT be able to save your answers and revisit your application at a later date. To help you prepare, the application will ask you: Your experience in CF care Specific work you may have done in developing, evaluating, and using educational materials Creative ideas you may have for addressing new material and areas of need Applications are due by FRIDAY, APRIL 20, 2012. If you have any questions, please contact Melissa Chin at This information was provided by Donna Germuga, a Respiratory Therapist at Golisano Children's Hospital.


FDA approves Kalydeco

The first drug that treats the root cause of cystic fibrosis won approval Tuesday (01/31/12), offering a life-changing treatment for a handful of patients with the deadly illness and broader hope for thousands more patients with the inherited disease. The FDA reviewed and approved Kalydeco in approximately three months under the agency’s priority review program that is designed to expedite the review of drugs. The priority review program uses a six-month review, instead of the standard 10 months, for drugs that may offer significant advances in treatment over available therapy. Kalydeco is effective only in patients with CF who have the G551D mutation (about 4% of the CF population). It is not effective in CF patients with two copies of the F508 mutation in the CFTR gene, which is the most common mutation that results in CF. If a patient’s mutation status is not known, an FDA-cleared CF mutation test should be used to determine whether the G551D mutation is present. Read the full FDA press release here. Read an article on the Huffington Post here.  


Highland TV/phone charge gone

Highland Hospital as announced that they have discontinued charges for in-room telephone and television effective January 5, 2012. CFFC has paid these charges for CF patients in the past; however, the hospital's policy change will not only provide consistent coverage but will also free up CFFC funds for other forms of support.