Description
What is Cystic Fibrosis? Cystic fibrosis (CF), also called Mucoviscidosis, is a hereditary disease that affects the entire body, causing progressive disability and early death. Formerly known as cystic fibrosis of the pancreas, this entity has increasingly been termed simply 'cystic fibrosis.' Difficulty breathing and insufficient enzyme production in the pancreas are the most common symptoms. Thick mucous production, as well as a low immune system, results in frequent lung infections, which are treated, though not always cured, by oral and intravenous antibiotics, inhalation of mucolytics and hypertonic saline solution, chest physiotherapy and daily exercise. It is vital for CF patients who are pancreatic deficient to use sufficient pancreatic enzymes with every meal and to consume high caloric nutritional foods. A multitude of other symptoms, including sinus infections, poor growth, diarrhea, and potential infertility (mostly in males) result from the effects of CF on other parts of the body. 1 in 2500 children is born with cystic fibrosis, and it is one of the most common fatal inherited diseases. Individuals with cystic fibrosis can be diagnosed prior to birth by genetic testing or in early childhood by a sweat test. There is no cure for CF, and most individuals with cystic fibrosis die young -- many in their 20s and 30s from lung failure although with many new treatments being introduced the life expectancy for people with CF are increasing. Ultimately, lung transplantation is often necessary as CF worsens. While the earliest clear medical descriptions date from the 1930's, CF obviously existed prior to this date but was un-recognized. Its clinical characteristics individually resemble those of other diseases such as pneumonia, bronchiectasis, failure to thrive, and celiac disease. Indeed, where these conditions are prevalent CF goes un-recognized. Moreover, if clinicians believe that CF is absent from their population they will not consider it in a differential diagnosis. A better awareness of and the increasing availability of diagnostic tests- the sweat test and/or DNA tests - frequently leads to the identification of a higher number of affected individuals. In the past it was believed CF only existed in Caucasians, through continued advancements and diagnosis we understand CF is a global disease with many different mutations and symptoms. In the last two decades, CF has been increasingly diagnosed in Latin America, the Middle East, and populations derived from the Indian subcontinent that have emigrated to Western Europe, thus implying the presence of CF in significant numbers among the citizens of India and Pakistan who have remained in their homeland. We now see CF as an emerging disease state throughout the Middle East and Arab populations. Due to the practice of continuity among Arabs, in most cases over 50%, we see CF according in dramatically high numbers. CF is a multi-organ disease and children with CF need access to all necessary medications and multidisciplinary team care in CF centre's regularly. This multidisciplinary team should consist of a CF Nurse, Pediatric Pulmonologists, Pulmonologists, Gastroenterologist/Nutritionist, Nurse, Physiotherapist, Microbiologist and Psychologist/Social worker who have been trained in the specific needs of patient care. Daily home care that requires both parent and patient participation is also needed to ensure the patients experience a better quality of life and life expectancy.
