Objective To research the therapeutic effect of BIPAP machine-assisted breathing combined with Jianpi Yifei Formula（spleen-fortifying lung-boosting formula）for amyotrophic lateral sclerosis（ALS）with respiratory failure.Methods Nine patients were orally taken Jianpi Yifei Formula granules 1package and twice daily for 1month on the basis of nutritional support

symptomatic treatment and BIPAP machine-assisted breathing.The pH value

PCO2（mmHg）and PO2（mmHg）were analyzed with electrode method before and after treatment.The operating time of BIPAP breathing machine and ALS self-assessment questionnaire（ALSAQ-40）score were recorded.Results The levels of PCO2and PO2were significantly improved after treatment（P<0.05）.The operating time of BIPAP breathing machine was significantly shortened after treatment（P< 0.05）.The emotional response score of ALSAQ-40was significantly improved after treatment（P<0.05）.There was no significant improvement in other scores of ALSAQ-40after treatment（P>0.05）.Conclusion The combo therapy of BIPAP breathing machine and Jianpi Yifei Formula for ALS can effectively alleviate the respiratory muscle fatigue

improve the respiratory function

shorten the operating time of BIPAP breathing machine and relieve the symptoms.