Impact of pulmonary rehabilitation pre- and post-lung transplantation in a child with cystic fibrosis: a case report

ABSTRACT Cystic Fibrosis (CF) is a genetic disease that reduces quality of life. Lung transplantation (LTx) is a strategy for end-stage lung disease treatment in CF. Pulmonary rehabilitation (PR) in LTx is effective, however, only one study has determined its effectiveness in children, and most studies have not included CF exclusively. Thus, reports showing components for PR protocols and outcomes not considered in previous studies of PR in LTx due to CF in children are still needed. To report this case, written informed assent and consent of patient and parent were obtained. Ethical Requirement was formally waived by the institution. A 12-year-old patient with CF was referred to PR due to LTx. A general and respiratory training was conducted daily for six months (pre) and two years (post) the transplantation, with the parents’ full support. General training included treadmill and cycle ergometer use and upper limbs exercises. Respiratory protocol included inspiratory training and respiratory physical therapy. We observed improvements in pulmonary function, exercise capacity, inspiratory muscle strength, and quality of life, including school functioning, with progress maintenance after 2.5 years of continuous intervention. This case presents a PR protocol pre- and post-LTx with good long-term results. These components for treatment protocols and outcomes may be useful to consider in clinical interventions or future investigations. RESUMEN La fibrosis quística (FQ) es una enfermedad genética que reduce la calidad de vida de los afectados. El trasplante de pulmón (LTx) es una estrategia para el tratamiento de enfermedades pulmonares en fase terminal en FQ. La rehabilitación pulmonar (RP) en el LTx es eficaz, aunque solo un estudio evaluó su eficacia en niños, y otros estudios no han tratado exclusivamente de FQ. Por lo tanto, se necesitan informes que contengan elementos de los protocolos y de los resultados de RP y que no habían sido considerados en los estudios anteriores sobre RP en LTx por FQ pediátrica. Así se describe el caso del paciente de 12 años de edad con FQ que fue remitido a RP debido a LTx. Para ello, se obtuvieron el consentimiento informado y el consentimiento por escrito del paciente y sus padres. La institución renunció formalmente a los requisitos éticos. Se realizó entrenamiento general y respiratorio durante seis meses antes de LTx y dos años después de LTx, diariamente, con apoyo de los padres. El entrenamiento general incluyó cinta de