RESUMEN
BACKGROUND: Amyotrophic lateral sclerosis (ALS), as a consequence of the progressive failure of respiratory muscles, often causes chronic ventilatory failure (CVF), indicated by hypercapnia. This study analyses the physiological parameters of breathing in patients with ALS over time and the variables which influence survival time. METHODS: In this observational study we analysed the data of physiological parameters (respiratory function, blood gas levels and breathing during sleep), as well as survival rate (according to Kaplan-Meier) of all 85 ALS patients who stayed in our hospital during the period of 1st January 2003 until 31st December 2007. After ALS had been diagnosed, all patients ran through standardised pneumological diagnostics during the observation period, this procedure was repeated every 3-6 months. If hypercapnia (carbon dioxide tension pCO(2) > 45 mm Hg) was detected, non-invasive ventilation (NIV) was indicated and offered to the respective patients. RESULTS: In the course of the observation, the parameters of respiratory function IVC (inspiratory vital capacity) and FEV1 (forced expiratory volume after 1 second) have shown a significant reduction by 14-15% per year. Half of the patients died within 3.1 years after ALS had been diagnosed. IVC and FEV1 had no impact on the survival time. In contrast, pCO(2) correlates negatively with the survival time. The period between diagnosis of ALS and manifestation of hypercapnia is about 1.9 +/- 2.4 years. In spite of a clear indication, some of the patients did not comply with NIV or did not accept it (19 patients, 22%). Twenty-eight patients (33%) started NIV with a good compliance. The survival rate of patients with NIV was 1.27 years on average--after the initial measurement of hypercapnia. The survival time of hypercapnic patients without NIV was only 0.12 years. CONCLUSIONS: Hypercapnia has a significant impact on the prognosis for ALS patients. In the case of CVF (hypercapnia), the survival time of ALS patients is significantly reduced. NIV is able to significantly increase the survival time of ALS patients with hypercapnia.
Asunto(s)
Esclerosis Amiotrófica Lateral/mortalidad , Esclerosis Amiotrófica Lateral/rehabilitación , Hipercapnia/mortalidad , Hipercapnia/prevención & control , Mecánica Respiratoria , Esclerosis Amiotrófica Lateral/fisiopatología , Comorbilidad , Femenino , Alemania/epidemiología , Humanos , Hipercapnia/fisiopatología , Masculino , Persona de Mediana Edad , Prevalencia , Medición de Riesgo/métodos , Factores de Riesgo , Análisis de Supervivencia , Tasa de SupervivenciaRESUMEN
When the sanatorium "Heidehaus" was founded on June 1, 1907 in the northern countryside of Hannover with Dr. Otto Ziegler as head about 120 beds for patients with tuberculosis were available. By 1914 about 200 patients were being treated by 4 physicians and 10 nurses. An operating theatre and a modern radiology unit were added in 1927. Shortly after the 2nd World War 400 patients with tuberculosis were hospitalised simultaneously. With the introduction of antituberculous triple drug treatment the number of patients dropped significantly. During this period many traditional facilities, used to care for patients with tuberculosis lost their financial basis and closed. However in the 1960s Prof. Schindler, the head of Heidehaus, widened the spectrum of the hospital into a modern chest hospital, focused on lung and airway diseases. In particular in the 1980s and 1990s this trend continued and 2 independent departments, i. e., pneumology and thoracic surgery were founded. In 2005 due to restructuring by the community of Hannover the "Heidehaus" moved completely and merged with another traditional hospital to become the new "Oststadt-Heidehaus". In its new surroundings both departments for pulmonary medicine and thoracic surgery offer a broad spectrum of modern thoracic medicine in cooperation with other disciplines.