Academic journal article Bulletin of the World Health Organization

Implementation of Cystic Fibrosis Services in Developing Countries: Memorandum from a Joint WHO/ICF(M)A Meeting

Academic journal article Bulletin of the World Health Organization

Implementation of Cystic Fibrosis Services in Developing Countries: Memorandum from a Joint WHO/ICF(M)A Meeting

Article excerpt

Introduction

Cystic fibrosis (CF) was first recognized about 60 years ago in Europe and the USA. In early descriptions, life expectancy was generally less than 2 ears. Diagnosis was based on pancreatic malabsorption accompanied by chronic pulmonary infection. Family histories demonstrated that the inheritance pattern was autosomal recessive. With the development of sweat testing in the 1950s, diagnosis became easier and more accurate, and was made with increasing frequency. CF is now recognized to be the commonest serious single-gene disorder in most Caucasian populations.

With the treatment available in specialized centres, including attention to all major features of the disease (respiratory, digestive, etc.), the prognosis in many countries has improved. Mean actuarial life expectancy is now about 30 years. However, such figures are historical; prospective estimates for young children with CF suggest that they may live for 40 years or more, even without the development of new treatment modalities. Since the identification in 1989 of the gene defect causing CF, there has been unprecedented progress in the understanding of its mechanisms and in new approaches to the development of definitive pharmacological therapy. Such treatments are expected to be available within the lifetime of most patients alive today. Previous joint meetings between WHO and the International Cystic Fibrosis (Mucoviscidosis) Association (ICF(M)A) have considered the geographical distribution of CF and its control, as well as population screening and current and prospective treatment (1-3). Although CF is widespread, there are significant variations in its incidence, with relative sparing of African and Asian races. Recognition of CF, and the provision of appropriate clinical services, are generally good in western Europe, the USA. and Australasia. However, there are other large populations in whom the disease is infrequently recognized and inadequately treated, resulting in avoidable death or suffering in infancy, childhood, and adulthood. The Joint WHO/ ICF(M)A meeting reported here was convened to address these problems and to determine strategies for the implementation and development of CF services in developing countries. In this context, "developing country" refers to a country where CF is known to exist, where it is thought to be much more common than is appreciated by either the medical profession or the general public, and where CF services are either non-existent or in the early stages of development.

Cystic fibrosis in developing countries

Participants at the meeting were asked to complete questionnaires on the overall conditions of and problems regarding the availability of CF care in their countries. The following common problems were identified:

-- underdiagnosis of CF;

-- reduced life expectancy (relative to developed countries) among those diagnosed with CF;

-- a clinical profile that may vary from the "classic" description of CF;

-- poor availability of necessary drugs; and

-- a lack of CF services or basic research on CF.

Against this background, the objectives of CF services in developing countries should be to improve diagnosis and survival and to define the clinical profile of CF in the local population, as well as to explore possibilities for improving the supply of appropriate medications.

The availability of health services and the level of socioeconomic development varied considerably among the countries represented. Although the relative contribution of the public and private health care systems to CF services was not discussed, the principle of the right to appropriate care for all individuals with CF was assumed.

Prerequisites for establishing a cystic fibrosis service

Case-finding. Simple tools must be available for identifying affected individuals. The essential diagnostic tool is the sweat test. …

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