神経難病における在宅ケアの実際

抄録

We discuss several points to consider for home care for patients with intractable neurological diseases.

In cases of chronic paralytic ileus, patients with neurological diseases may replace percutaneous endoscopic gastrostomy (PEG) buttons with PEG tubes, as they are more useful for gastrointestinal decompression. Also, PEG tubes can be helpful to decompress the gastrointestinal air as aerophagia and abdominal distension, which often occur in neuromuscular diseases, can occur with noninvasive positive pressure ventilation.

It is common to use a suction machine to remove excess saliva. In addition, mechanical insufflation–exsufflation and low pressure aspirators are recommended to be used in combination. A granuloma is a nodule of granulation tissue at the tracheal cannula insertion site. To avoid granuloma growth, the tracheal cannula may be replaced by another cannula without a convex portion, and the surface may be coated by steroid ointment. Surgery to prevent aspiration is often necessary in cases of intractable aspiration to prevent recurrent bronchopulmonary contamination. Even after tracheostomy, this surgery is possible.

Multidisciplinary care improves quality of life in patients with neurological diseases. In amyotrophic lateral sclerosis patients, PEG should be discussed at an early stage. We recommend to perform PEG before respiratory function is impaired or severe weight loss occurs.

Apraxia lid opening, trismus, and dystonic movement disorders in the cranial–cervical region interfere with communication with patients of neurological disease. Botulinum A toxin therapy is recommended for the treatment of these symptoms.