Who will get Cystic Fibrosis?

Cystic fibrosis has been known for over 200 years. Reports from the Cystic Fibrosis Foundation indicate that there are 30,000 Americans, 3,000 Canadians, and 20,000 Europeans with this disease. Every year, about 2,500 babies  in the United States continue to be born with this disease. These babies can only look ahead to a brief 30 year life expectancy. The majority of children are screened for CF at birth and will be diagnosed by age 2, but there are still some that are diagnosed as adults. Apart from those who actually inherit the disease, 1 in 20 Americans contain the abnormal CF gene. This translates to a whopping 12 million people that are carriers, and since carriers have no symptoms of the disease, they usually carry it unwittingly. 

A clinical study in Saudi Arabia sought to gain information on the morbidity and mortality of this disease. They conducted their research at a hospital over 9 years on confirmed CF patients. They found that over the 9 year period, 190 patients were diagnosed with CF. Of these, 164 (86%) patients lived and 26 (14%) died. Fifty-two percent were male and forty-eight percent were female, indicating a relatively even divide in incidence between genders. Factors that related to early mortality were calculated weight/height, low albumin level, high hematocrit, low mean corpuscular volume, and low mean corpuscular hemoglobin concentration.

Since CF is a genetic disease, it cannot be prevented. Babies with two mutated CF genes will have the disease, and at this time gene therapy can only be used to help prevent some of the complications. The hope for the future is that genetic counseling will be able to detect CF carriers, and possibly persuade them to not have children. Every time two carriers of the gene conceive, there is a 25 percent chance that they will pass cystic fibrosis on to their child. However, the problem here is that the tests to determine if one has the defective gene are only 80-85 percent accurate. Parents should always be reassured that it was not their fault that their child has the disease; they could not have prevented it.

Sources used:

http://www.cdc.gov/excite/ScienceAmbassador/ambassador_pgm/lessonplans/high_school/Am%20I%20a%20Carrier%20for%20Cystic%20Fibrosis/Cystic_Fibrosis_Fact_Sheet.pdf

http://www.ncbi.nlm.nih.gov/pubmed/12883603

Search terms:

Cystic Fibrosis epidemiology, Cystic Fibrosis morbidity and mortality, Cystic Fibrosis prevalence