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People Who Have Died from Cystic Fibrosis

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  • Alan Michael Dressler (1980 - 2007)
  • Kacy Michelle Harris (1985 - 2004)
    M. Harris Kacy Michelle Harris, 19, of Mount Zion passed away Sept. 14, 2004, at Ruby Memorial Hospital, Morgantown after a lifelong battle with Cystic Fibrosis. She is survived by her parents, Bernard...
  • Graham C Lay (1960 - 2016)
    Graham Lay of Antiques Roadshow= From Wikipedia: Graham Lay (1960-2016) was an antiques expert, specialising in arms and militaria, and military history, probably best known for his many appearances on...

Cystic Fibrosis

Cystic fibrosis (CF) is a 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 include sinus infections, poor growth, fatty stool, clubbing of the fingers and toes, and infertility in males, among others. Different people may have different degrees of symptoms.

No cure for cystic fibrosis is known. Lung infections are treated with antibiotics which may be given intravenously, inhaled, or by mouth. Sometimes, the antibiotic azithromycin is used long term. Inhaled hypertonic saline and salbutamol may also be useful. Lung transplantation may be an option if lung function continues to worsen. Pancreatic enzyme replacement and fat-soluble vitamin supplementation are important, especially in the young. Airway clearance techniques such as chest physiotherapy have some short-term benefit, but long-term effects are unclear. The average life expectancy is between 42 and 50 years in the developed world. Lung problems are responsible for death in 80% of people with cystic fibrosis.

CF is most common among people of Northern European ancestry and affects about one out of every 3,000 newborns. About one in 25 people is a carrier.[3] It is least common in Africans and Asians. It was first recognized as a specific disease by Dorothy Andersen in 1938, with descriptions that fit the condition occurring at least as far back as 1595. The name 'cystic fibrosis' refers to the characteristic fibrosis and cysts that form within the pancreas.

The prognosis for cystic fibrosis has improved due to earlier diagnosis through screening and better treatment and access to health care. In 1959, the median age of survival of children with CF in the United States was six months. In 2010, survival is estimated to be 37 years for women and 40 for men. In Canada, median survival increased from 24 years in 1982 to 47.7 in 2007.

Of those with CF who are more than 18 years old as of 2009, 92% had graduated from high school, 67% had at least some college education, 15% were disabled, 9% were unemployed, 56% were single, and 39% were married or living with a partner.

History

CF is supposed to have appeared about 3,000 BC because of migration of peoples, gene mutations, and new conditions in nourishment. Although the entire clinical spectrum of CF was not recognized until the 1930s, certain aspects of CF were identified much earlier. Indeed, literature from Germany and Switzerland in the 18th century warned "Wehe dem Kind, das beim Kuß auf die Stirn salzig schmeckt, es ist verhext und muss bald sterben" or "Woe to the child who tastes salty from a kiss on the brow, for he is cursed and soon must die", recognizing the association between the salt loss in CF and illness.

In the 19th century, Carl von Rokitansky described a case of fetal death with meconium peritonitis, a complication of meconium ileus associated with CF. Meconium ileus was first described in 1905 by Karl Landsteiner. In 1936, Guido Fanconi described a connection between celiac disease, cystic fibrosis of the pancreas, and bronchiectasis.

In 1938, Dorothy Hansine Andersen published an article, "Cystic Fibrosis of the Pancreas and Its Relation to Celiac Disease: a Clinical and Pathological Study", in the American Journal of Diseases of Children. She was the first to describe the characteristic cystic fibrosis of the pancreas and to correlate it with the lung and intestinal disease prominent in CF.[9] She also first hypothesized that CF was a recessive disease and first used pancreatic enzyme replacement to treat affected children. In 1952, Paul di Sant’Agnese discovered abnormalities in sweat electrolytes; a sweat test was developed and improved over the next decade.

The first linkage between CF and another marker (Paroxonase) was found in 1985 by Hans Eiberg, indicating that only one locus exists for CF. In 1988, the first mutation for CF, ΔF508 was discovered by Francis Collins, Lap-Chee Tsui, and John R. Riordan on the seventh chromosome. Subsequent research has found over 1,000 different mutations that cause CF.

Because mutations in the CFTR gene are typically small, classical genetics techniques had been unable to accurately pinpoint the mutated gene. Using protein markers, gene-linkage studies were able to map the mutation to chromosome 7. Chromosome-walking and -jumping techniques were then used to identify and sequence the gene. In 1989, Lap-Chee Tsui led a team of researchers at the Hospital for Sick Children in Toronto that discovered the gene responsible for CF. CF represents a classic example of how a human genetic disorder was elucidated strictly by the process of forward genetics.

According to the Cystic Fibrosis Foundation Patient Registry, in the United States:

  • More than 30,000 people are living with cystic fibrosis (more than 70,000 worldwide).
  • Approximately 1,000 new cases of CF are diagnosed each year.
  • More than 75 percent of people with CF are diagnosed by age 2.
  • More than half of the CF population is age 18 or older. 

The symptoms and severity of the disease vary according to the genetic defect that caused it, as well as patients’ age and other existent conditions. Similarly, the symptoms are not constant and differ over time. There is currently no cure for CF, but research leads to new medications that helps patients improve their quality of life and life expectancy. According to the National Institutes of Health (NIH), today the current average life expectancy for CF patients in the United States and United Kingdom who live past childhood, is 35 to 40 years. Some people live well beyond that. Life expectancy is significantly lower in certain countries, including El Salvador, India, and Bulgaria, where it’s less than 15 years.

The majority of pediatric CF patients are able to manage symptoms and maintain quality of life such as taking part in school and other activities. In fact, many young adults with cystic fibrosis manage to finish college and find jobs. Despite the tendency for life expectancy in CF patients to increase, lung disease continues to be common and gets worse over time, which eventually disables the patient. Death is most often caused by lung complications. Though life expectancy continues to be globally uneven, with the U.S. where CF patients tend to live longer, the NIH believes that current research will lead to improvements in treatment and life expectancy worldwide.

Life expectancy is significantly lower in certain countries, including El Salvador, India, and Bulgaria, where it’s less than 15 years.

Famous People with or died from Cystic Fibrosis

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