The State of Cystic Fibrosis in Turkey

Summary

Cystic fibrosis (CF) represents a major health problem in developed countries. Although its distribution is worldwide, there is little awareness of its prevalance in most developing countries.

In Turkey the infant mortality rate is still very high (59/1000). Respiratory infections and diarrhea are important causes of infant mortality (13%, and 9%, respectively) (1). There are no reliable statistical data available for the incidence of CF in our country. Probably some infant deaths which are listed under respiratory and gastrointestinal problems are due to CF, because of lack of diagnosis.

There is a specific aspect to be considered in Turkey. Consanguineous marriages are very common (21%) (2). Therefore we see a large number of inherited metabolic diseases, for instance phenylketonuria (PKU). The mean incidence of PKU is 1:10 000 all over the world, whereas its incidence in Turkey is 1:6 000 according to our studies (3). CF leads to mortality, but PKU to a sequal, mental retardation. We believe that because of the lack of diagnosis we cannot reach patients with CF in our country, but patients with PKU who survive with mental retardation are more easily diagnosed since newborn screening is not organized nationwide as yet.

Diagnosis of CF is being made primarily in hospitals or specialized centers that can provide subsequent health care. There are no special CF clinics in Turkey. CF patients are followed in a few number of children’s hospitals of medical schools in the metropolitan areas where there are special out- and inpatients departments for CF as in our center. The total number of the CF patients who are followed in these centers does not exceed 250 to our knowledge. It will be necessary to develop new approaches in developing countries, where tertiary care facilities are limited, and to give more emphasis to early treatment in a primary care setting to reach the level of care of the developed countries.