Cystic fibrosis (CF) is a rare genetic disorder that affects mostly the lungs, but also the pancreas, liver, kidneys, and intestine. Long-term issues include difficulty breathing and coughing up mucus as a result of frequent lung infections. Other signs and symptoms may include sinus infections, poor growth, fatty stool, clubbing of the fingers and toes, and infertility in most males. Different people may have different degrees of symptoms.
Cystic fibrosis transmembrane conductance regulator (CFTR) is a membrane protein and anion channel in vertebrates that is encoded by the CFTR gene.
Benjamin Franklin Deford III was an American sportswriter and novelist. From 1980 until his death in 2017, he was a regular sports commentator on NPR's Morning Edition radio program.
Dorothy Hansine Andersen was an American physician, pediatrician, and pathologist who first identified cystic fibrosis. She was the first to describe the disease, and name it. In 1939, she was awarded the E. Mead Johnson Award for her identification of the disease. In 2002, she was inducted into the National Women's Hall of Fame.
Chest physiotherapy (CPT) are treatments generally performed by physical therapists and respiratory therapists, whereby breathing is improved by the indirect removal of mucus from the breathing passages of a patient. Other terms include respiratory or cardio-thoracic physiotherapy.
Laura Elizabeth Rothenberg was an American author. She wrote a memoir describing her life with cystic fibrosis called Breathing for a Living.
Frankie Jo Abernathy was an American purse designer and reality television personality, known for her time as a cast member on MTV's The Real World: San Diego which was filmed in late 2003 and aired from January to June 2004. Hailing from Kansas City, Abernathy was the elder daughter of Abbie Hunter and Joe Abernathy. She had a younger sister named Mamie, and a stepfather, Perry Hunter. She attended Blue Springs High School in Blue Springs, Missouri.
Mary Ellen Beck Wohl was Chief of the Division of Respiratory Diseases at Children's Hospital Boston, and served as Associate Director of the General Clinical Research Center until 2002. Since the 1962, when she first joined the staff at Children's Hospital, Wohl specialized in the respiratory diseases of children. She was also a leader in the field of clinical research on cystic fibrosis. She developed a number of techniques to evaluate the function of the lungs in young children and is the author of many research papers in this field.
Mucociliary clearance (MCC), mucociliary transport, or the mucociliary escalator, describes the self-clearing mechanism of the airways in the respiratory system. It is one of the two protective processes for the lungs in removing inhaled particles including pathogens before they can reach the delicate tissue of the lungs. The other clearance mechanism is provided by the cough reflex. Mucociliary clearance has a major role in pulmonary hygiene.
65_RedRoses is a 2009 documentary film about Eva Markvoort, a young woman from New Westminster, British Columbia, who suffered from cystic fibrosis. Directed by Philip Lyall and Nimisha Mukerji, it follows Markvoort as she lives her life undaunted by her disease, waiting for a lung transplant while blogging about her experiences.
Eva Markvoort was a woman from New Westminster, British Columbia, Canada who died from cystic fibrosis at the age of 25. She blogged about her life, family and experiences, including undergoing a lung transplant and her subsequent transplant rejection, in her blog "65_Redroses," which is also the name of a documentary film about her, 65 Redroses.
Ann Harrison was the recipient of the world's first successful human double lung transplant. She survived for almost fifteen years after the surgery and died of unrelated causes.
Carolyn Denning was an American pediatrician whose research specialized in learning more about and treating cystic fibrosis. When she began her 40-year career, those diagnosed with cystic fibrosis rarely reached puberty –– today, nearly 50 percent of those diagnosed in the United States are 18 years or older.
Physiotherapists treating patients following uncomplicated coronary artery bypass surgery surgery continue to use interventions such as deep breathing exercises that are not supported by best available evidence. Standardised guidelines may be required to better match clinical practice with current literature.
Emily's Entourage is a nonprofit organization that raises money and awareness to help find a cure for rare ("nonsense") mutations of cystic fibrosis (CF), a genetic disorder that generally affects a person's lungs and digestive system. The foundation has raised over $1 million, largely from viral fundraising through universities and video campaigns. The organization also participates in exploratory research initiatives aimed at understanding and advancing towards a cure.
Five Feet Apart is a 2019 American romantic drama film directed by Justin Baldoni and written by Mikki Daughtry and Tobias Iaconis. The film was inspired by Claire Wineland, who suffered from cystic fibrosis. Haley Lu Richardson and Cole Sprouse play two young patients with cystic fibrosis who try to have a relationship despite being forced to stay six feet apart from each other. The film was released in the United States on March 15, 2019 by CBS Films via Lionsgate. It received mixed reviews from critics and grossed $92 million worldwide.
Margaret Hilda Harper was an Australian pediatrician, medical writer, and radio commentator. Harper was one of the two physicians who described that coeliac disease in the pancreas and cystic fibrosis were "distinct entities" in the 1930s.
Johanna Rommens is a Canadian geneticist who was on the research team which identified and cloned the CFTR gene, which when mutated, is responsible for causing cystic fibrosis (CF). She later discovered the gene responsible for Shwachman-Diamond syndrome, a rare genetic disorder that causes pancreatic and hematologic problems. She is a Senior Scientist Emeritus at SickKids Research Institute and a professor in the Department of Molecular Genetics at the University of Toronto.
Underrepresented populations, especially black and hispanic populations with cystic fibrosis are often not successfully diagnosed. This is in part due to the minimal dissemination of existing data on patients from these underrepresented groups. While white populations do appear to experience a higher frequency of cystic fibrosis, other ethnicities are also affected and not always by the same biological mechanisms. Thus, many healthcare and treatment options are less reliable or unavailable to underrepresented populations. This issue affects the level at which public health needs are being met across the world.
