Pain in hereditary neuromuscular disorders and myasthenia gravis: a national survey of frequency, characteristics, and impact.

The aim of our study was to evaluate pain frequency, intensity, and disability levels in a population with neuromuscular disorders (NMD). Of 862 questionnaires mailed to outpatients treated at 10 centers, 511 (300 men and 211 women) responded with answers suitable for analysis (response rate: 59.3%). Patients had Duchenne or Becker muscular dystrophy, type 1 myotonic muscular dystrophy, facioscapulohumeral muscular dystrophy, metabolic myopathy, or myasthenia gravis (MYA). The questionnaire packet included numeric scales for pain intensity and relief, the Brief Pain Inventory, the Saint Antoine Pain Questionnaire, and a scale to assess disability. More than two-thirds of the 331 patients (67.3%) suffered pain during the last three months. The mean number of days with pain was 18.4+/-15.1 days. The mean pain intensity was 4.8+/-2.5. Pain was usually diffuse (153 patients, 44%) and intermittent (228, 71%). Pain intensity varied by the NMD diagnosis; the most severe pain was observed in metabolic myopathy (13/27 patients suffered severe pain, 49%) and in MYA (16/42, 38%). Approximately three-quarters of patients had fewer than 10 days of inactivity due to pain during the last three months, and 98% had fewer than 30 days. Our study indicates that pain is frequent in hereditary muscle disorders and MYA. Mean intensity is moderate. Pain in NMD patients should be systematically assessed.

Forgoing life-sustaining treatments in children: a comparison between Northern and Southern European pediatric intensive care units.

OBJECTIVES: This study was conducted to determine how the decision-making process to forgo life support differs between southern and northern European pediatric intensive care units. DESIGN: Multiple-center, prospective study. SETTING: Thirty-nine pediatric intensive care units: 12 from northern Europe and 27 from southern Europe. PATIENTS: All consecutive deaths were recorded over a 4-month period. Group 1 and group 2 included patients who died in northern and southern pediatric intensive care units, respectively. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Three hundred fifty children were enrolled, 68 in group 1 and 282 in group 2. The decision to forgo life-sustaining treatment was made in 116 children (group 1, n = 32; group 2, n = 84). In both groups, the decision was discussed by caregivers during a formal meeting. The decision to forgo life-sustaining treatment was more often made in northern countries than in southern ones (47% vs. 30%, p =.02). Parents were informed of this decision in 95% of cases in group 1 vs. 68% in group 2 (p =.01). In both groups, the final decision was made by the medical staff. Parents' contributions to the decision-making process did not differ between the two groups according to the practitioners' opinion. The decision was documented in the medical charts in 100% of the cases in group 1 and in 51% of the cases in group 2 (p =.0001). CONCLUSIONS: The decision-making process appears to be similar between northern and southern European countries. The respective contributions of the parents and the medical staff in the final decision itself seem to be identical between northern and southern countries. However, in northern European countries, the level of parents' information about the decision-making process appears higher and the decision is more often documented in the medical chart.