Diagnosing and managing cystic fibrosis in the developing world

Abstract

Cystic fibrosis (CF), earlier believed to be non existent in non Caucasians, is now a pan ethnic disease, having being reported from various regions of the world over last one decade. Apart from limited resources, the major problems in diagnosis and management of CF in developing countries include: lack of awareness among pediatricians, absence of facilities for diagnosis (sweat chloride estimation and genetic studies), lack of trained manpower for care of specific problems, and non availability of appropriate drugs. Care of children with CF may not be a priority for governments in countries where childhood mortality rates are high, predominantly due to acute infections. An indigenously developed and relatively inexpensive method of sweat collection and chloride estimation using pilocarpine iontophoresis and titration, respectively, may be an alternative to the commercially available costly equipment. Having a team of trained nurse, physiotherapist, and dietician for optimal care of CF patients may not be feasible due to inadequate resources. Training a single person (e.g. nurse) to deliver comprehensive CF care may be a feasible alternative. To overcome problems of non availability of appropriate drugs (enzymes, inhaled antibiotics, DNAse, etc), locally available drugs may be used. Examples include use of hypertonic saline in place of DNAse, enteric coated enzyme tablets in place of enteric coated spherules, etc. Factors that are associated with decreased survival in CF patients from developing countries are age of onset of symptoms <2 months, severe malnutrition at the time of diagnosis, colonization with Pseudomonas at the time of diagnosis and frequency of pneumonia >4 episodes/year. All these factors can be modified except onset of symptoms before 2 months of age, by early diagnosis and appropriate treatment. Many of the above mentioned hurdles have been successfully overcome by us to establish CF services in a resource-limited setting. We conclude that education of pediatricians about the disease, early diagnosis using indigenous technology and aggressive physiotherapy with nutritional management and judicious use of antibiotics can improve the quality of life and survival in CF patients in resource-limited settings.