A clinical case of long-term successful treatment of chronic respiratory failure in a patient with congenital kyphoscoliosis

Abstract

A clinical case of a female patient of 44 years old with chronic respiratory failure (CRF) and alveolar hypoventilation caused by congenital kyphoscoliosis is described in this article. Noninvasive ventilation was initiated using bilevel positive air pressure (BiPAP) with the controlled minimal respiratory rate (the ST mode). The respiratory support was preformed around-the-clock with short breaks for eating and taking hygienic procedures; later, the respiratory support was administered at nighttime only combined with low-flow oxygen 3 to 4 L followed by the decrease of the oxygen flow to 2 L. Such treatment allowed active lifestyle including travelling and teaching. The patient was followed-up for 4 years. Nighttime oxygenation disorders are typical in severe kyphoscoliosis and precede the development of CRF at daytime. Noninvasive respiratory support can improve pulmonary ventilation during night sleep and gas exchange at waking time. Unfortunately, respiratory support is used very rarely in patients with stable CRF, as some physicians consider this treatment only in critical and terminal conditions.