The weight of a 26-year-old man with Emery-Dreifuss muscular dystrophy fell in one year from over 23.8 kg to 22.5 kg, in connection with a worsening of his dysphagia to solid foods over the last six months. He was consuming 500 to 600 kcal/day and 150 to 200ml of water.
- He was put on total parenteral nutrition with 986ml/day of Smokfkabiven® combined with enteral nutrition based on natural foods for less than 200 kcal/day.
- Non-invasive ventilation (NIV) was maintained during the month he was in hospital, after which his general condition improved, enabling him to return home on NIV and enteral nutrition.
- Within a year, his weight had risen to 31kg and remained stable, even though he had been on this diet for six years, with good psychological and biological tolerance.
- During this period, the superior vena cava catheter was replaced twice: the first time because it appeared blocked, and the second time because it had been accidentally withdrawn during dressing.
