Management of severe faecal impaction in an adolescent with Duchenne muscular dystrophy (DMD) receiving palliative care.

Abstract

Slow colonic transit is common in Duchenne muscular dystrophy (DMD) patients.1 Immobility, weakness of abdominal wall muscles and smooth muscle involvement all contribute to ineffective bowel emptying, producing chronic constipation that can impact systemically leading to poor quality of life. A 17-year-old non-ambulant adolescent male with late-stage DMD, requiring full assistance with activities of daily living under a palliative care plan, with slow-transit constipation, cardiomyopathy, respiratory insufficiency, scoliosis and depression was admitted to our tertiary children's hospital. He had acute pain in the legs, with a possible diagnosis of a deep vein thrombosis or a fracture of the lumbosacral spine and hips. A lumbar spine X-ray revealed severe faecal impaction with no bony abnormalities, suggesting the leg pain could be due to pressure of the faecal mass on the sacral plexus (Fig. 1a). (a) X-ray taken on admission to determine if there was fracture of lumbosacral spine or hips. The rectum is enlarged and full of faeces with hard faecal mass at the bottom of the image. (b) X-ray taken on day 4 – Pelvic space is dark indicating an empty rectum. DMD patients are non-ambulant from 9 to 10 years old, often resulting in stool accumulation due to poor toileting. Stool volume in the rectosigmoid was estimated from the X-ray to be 3600 mL. There was also extensive faecal loading throughout the colon. His prognosis was very poor with acute deterioration of his DMD, and previous bowel interventions had resulted in poor outcomes and quality of life. Because of the severe impaction, he was referred to us for disimpaction. He was given polyethylene glycol (PEG + E, Movicol; Norgine B.V., Amsterdam Zuid-Oost, The Netherlands) plus sodium picosulphate (SP, Dulcolax drops; Boehringer Ingelheim GmbH, Ingelheim am Rhein, Germany) orally, (PEG + E 109.6 g, SP 11.25–15 mg, 2 litres fluid/daily), for 4 days.2 In addition, each day, transcutaneous electrical stimulation (TES) was applied over the abdomen using interferential current (IFC), four electrodes, two front, two back, T9-L2, carrier freq 4 kHz, beat frequency 80–160 Hz, as described previously,2, 3 as this has been shown to be effective in improving colonic motility. Because of the crisis presentation, TES-IFC was used for 6 h/day, 2 h on, 2 h off. The patient produced 5–6 L of faeces over days 2–4, all soft formed stools with no diarrhoea. An X-ray on day 4 showed that the large faecaloma was gone (Fig. 1b). Loss of the large stool mass from rectum and faecal load in the colon removed pressure from the lumbosacral plexus and femoral nerves. All hip and leg pain resolved with substantial improvements in physical and psychological state. He was discharged home on day five and recommended to continue on the oral medication and TES. This is the first report of the use of combined disimpaction with oral medication and TES treatment in a DMD patient and we suggest that used earlier in DMD patients it might have improved bowel activity and reduced constipation during palliation, reducing crisis. This treatment may assist the treatment of other serious, deteriorating bowel conditions previously thought to be refractory.