Pain in Youths With Neuromuscular Disease

Abstract

To examine the prevalence and characteristics of pain in children with neuromuscular disease (NMD), 42 youths with NMD underwent a comprehensive evaluation including a detailed intake interview and structured questionnaire that included demographic and functional data. Youths who reported chronic pain were further queried about pain characteristics, locations, and intensity using an 11-point numerical rating scale and a modified Brief Pain Inventory (BPI). The sample consisted of 24 males (57%) and 18 females (43%), ages ranging from 9 to 20 years (M = 14.8, SD = 2.96). Participants included 14 (37%) with Duchenne-muscular dystrophy, 6 (14%) with myotonic dystrophy, 2 (5%) with Becker dystrophy, 2 (5%) with limb-girdle dystrophy, 2 (5%) with congenital muscular dystrophy, 1 (2%) facioscapulohumeral, and 15 (36%) were classified as "other NMD." Twenty-one (50%) were ambulatory; 26 (62%) used power wheelchairs/scooters, 9 (2%) used manual wheelchairs, 3 (.07%) used crutches/canes, and 1 (2%) used a walker. A total of 23 (55%) of the youths reported having chronic pain. Current pain intensity was 1.30(range=0-6), mean pain intensity over the past week was 2.39 (range = 0-7), mean pain duration was 8.75 hours (SD=12.84). Pain in the legs was most commonly reported and 83% reported using pain medications. This study indicates that chronic pain is a significant problem in youths with NMD. These data strongly support making comprehensive pain assessment and management an integral part of the standard of care for youths with NMD.

Pain characterization in Duchenne muscular dystrophy

“…Engel DMD: Duchenne muscular dystrophy; NDM: neuromuscular disease; BMD: Becker muscular disease; SMA: spinal muscular atrophy; BPI: brief pain inventory; VAS: visual analogue scales; *the paper had no information about each group separately; **the paper had no information about minimum and maximum age. et al 6 analyzed patients with neuromuscular diseases in general. Bray et al investigated pain in DMD patients according to their parents' reports 5 .…”

“…A variety of instruments was used in the reviewed studies to qualify and quantify pain. The most common instruments used in these studies were the Visual Analogue Scales (VAS) 1,5,15 and the Brief Pain Inventory (BPI) 1,6,7 . Other important factors are pain frequency and duration.…”

“…Historically, pain has not been considered to be a major part of the symptom manifestation of DMD. However, a growing number of studies indicate that chronic pain is a common symptom for people with neuromuscular diseases (NMD) 1,5,6 . Chronic pain may be common, even though it is not the primary concern of patients or doctors 7 .…”

“…Pain is described as "an unpleasant sensory and emotional experience that is associated with actual or potential tissue damage or described in such terms". Causes of pain in NMD are multifactorial, due to muscular overuse, severe contractures, vertebral fractures (caused by osteoporosis) and/or orthopedic procedures 1,5,6 . Pain in NMD may be under-assessed and underestimated because of its nature and because of the context of a disorder that affects mobility.…”

“…The youths and adults with DMD experience significant problems with pain that negatively affect their lives. Clinicians caring for these patients should investigate the nature of pain and its extent 6 . Although pain is a common occurrence in boys with DMD, it may still be under-recognized 5 .…”