Nöromusküler hastalıklardaki solunumsal problemlere güncel yaklaşım

Nöromusküler hastalıklar solunum kaslarında ilerleyici bir güçsüzlüğe neden oldukları için solunum yetmezliğine yol aç- maktadır. Yapılan çalışmalar, noninvaziv mekanik ventilasyonun bu hastalarda, semptomları, gaz alışverişini, yaşam kali- tesini ve sağkalımı düzelttiğini göstermiştir. Noktürnal hipoventilasyon başladığında noninvaziv mekanik ventilasyon da kullanılmalıdır. Hava yolu klerens tedavisi bu hastalarda çok önemlidir. Noninvaziv mekanik ventilasyon ile öksürüğe yar- dımcı teknikler birlikte kullanıldıklarında pulmoner morbidite ve hastane başvuruları azalmaktadır. Noninvaziv mekanik ventilasyonun kontrendike olanlarda veya birkaç günden daha uzun süre mekanik ventilasyon gereken hastalarda inva- ziv mekanik ventilasyon endikasyonu vardır. Yüksek seviye spinal kord hasarı olan hastalarda diyafragmatik pacemaker bir tedavi seçeneği olabilir. Solunum yolu infeksiyonlarının tetiklediği akut solunum yetmezliği atakları nöromusküler has- talıklarda görülebilir. Akut solunum yetmezliği ataklarının tedavisinde endotrakeal entübasyon gerekebilir.

Current approach to respiratory problems in neuromuscular diseases

Neuromuscular diseases lead to respiratory failure as they cause progressive weakness of the muscle of respiration. Studi- es show that noninvasive mechanical ventilation improves symptoms, gas exchange, quality of life, and survival in these patients. Noninvasive mechanical ventilation should be use at the onset of nocturnal hypoventilation. Airway clearance therapy is very important in these patients. The combination of noninvasive mechanical ventilation with cough-assist tech- niques decreases pulmonary morbidity and hospital admissions. Invasive mechanical ventilation is indicated for patients who require continuous mechanical ventilation for longer than a few days, or those who have contraindications to nonin- vasive mechanical ventilation. Diaphragmatic pacemakers may be a treatment option in patients with high spinal cord in juries. Episodes of acute respiratory failure in neuromuscular diseases are precipitated by respiratory infections. Manage- ment of episodes of acute respiratory failure may require endotracheal intubation.

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