Fibrosis quística: enfrentando la transición desde el pediatra hacia el internista / Cystic fibrosis: facing patient transition from the pediatrician to the internist

RESUMO

The increased survival of patients with cystic fibrosis (CF) resulted in the appearance of new pulmonary and non-pulmonary complications. Even though subjects with CF present with inflammatory pulmonary changes at birth, several pathogens such as Staphyloccocus aureus, Haemophilus influenzae and Pseudomona aeruginosa contribute to the progression of pulmonary injury. Clinical presentations vary according to patient age; even though mild forms exist, patients with severe forms, develop respiratory insufficiency and end-stage disease at an early stage. Today, new diagnostic and therapeutic tools, increase the possibility of an early diagnosis and of greater survival. Successful management will depend on the timely selection of adequate antimicrobials, the use of pancreatic enzyme supplementation and early institution to respiratory physiotherapy. The transition from pediatric care to adult care must occur according to development of each patient. This timing must be flexible and there should be a constant communication and coordination within the different specialists in internal medicine. Thus, it is crucial to recognize the disease progression as a continuous process, giving the appropriate physiologic support and evaluating the needs of the patient and close relatives.