Combined tetrahydrocannabinol and cannabidiol to treat pain in epidermolysis bullosa: a report of three cases.

Epidermolysis bullosa (EB) is a genetic blistering disorder characterized by intense pain related to disease pathology and care-based interventions. Opioid-based therapies underpin pain care in EB; however, they are unable to provide adequate analgesia in a significant proportion of patients. Cannabinoid-based medicines (CBMs) have been studied increasingly for pain conditions of various aetiologies and pose as a novel dimension for pain care in EB. We present three patients with EB who were prescribed pharmaceutical-grade sublingually administered CBMs comprising tetrahydrocannabinol and cannabidiol. All three patients reported improved pain scores, reduced pruritus and reduction in overall analgesic drug intake.

Long-term follow-up of patients with Herlitz-type junctional epidermolysis bullosa.

BACKGROUND: Junctional epidermolysis bullosa, type Herlitz (JEB-H) is a rare, autosomal recessive disease caused by absence of the epidermal basement membrane adhesion protein laminin-332. It is characterized by extensive and devastating blistering of the skin and mucous membranes, leading to death in early childhood. OBJECTIVES: To present the results of the long-term follow-up of a cohort of patients with JEB-H, and to provide guidelines for prognosis, treatment and care. METHODS: All patients with JEB-H included in the Dutch Epidermolysis Bullosa (EB) Registry between 1988 and 2011 were followed longitudinally by our EB team. Diagnosis was established using immunofluorescence antigen mapping, electron microscopy and DNA analysis. RESULTS: In total, we included 22 patients with JEB-H over a 23-year period. Their average age at death was 5.8 months (range 0.5-32.6 months). The causes of death were, in order of frequency: failure to thrive, respiratory failure, pneumonia, dehydration, anaemia, sepsis and euthanasia. The pattern of initial weight gain was a predictor of lifespan in these patients. Invasive treatments to extend life did not promote survival in our patients. CONCLUSIONS: It is important to diagnose JEB-H as soon as possible after birth so that the management can be shifted from life-saving to comfort care. The palliative end-of-life care can take place in hospital, but is also safe in the home setting. Suffering in patients with JEB-H can become so unbearable that in some patients who do not respond to adequate analgesic and sedative treatment, newborn euthanasia, performed according to the Groningen protocol, is legally permitted in the Netherlands.

Epidural anaesthesia in a child with possible spinal muscular atrophy.

Spinal muscular atrophy (SMA) is a rare lower motor neurone disease in which anaesthetic management is often difficult as a result of muscle weakness and hypersensitivity to neuromuscular blocking agents. Neuraxial anaesthesia is controversial in these patients; however, some cases have been reported in which neuraxial anaesthesia has been used without neurological sequelae. We describe a 7-year-old patient with possible SMA scheduled for a Grice-arthrodesis. Because of previous prolonged postoperative drowsiness and poor oral intake, we decided to use an epidural technique with sevoflurane sedation and spontaneous ventilation to avoid the use of muscle relaxants and systemic opioids and consequently admission to the intensive care unit. After 3 days, the epidural analgesia was stopped and the patient regained her preoperative motor function within 5 h. Despite the controversy surrounding the use of neuraxial techniques in neuromuscular disease, we found no well-founded basis for this in patients with SMA in the literature.

Comparison of vecuronium with ORG 9487 and their interaction.

PURPOSE: To compare the pharmacodynamic behaviour of vecuronium with that of ORG 9487, we measured the time-course of action of equipotent doses of ORG 9487 and vecuronium and investigated their mutual interaction when given in succession. METHODS: Sixty ASA I-II patients were anaesthetized with thiopentone, fentanyl, halothane and nitrous oxide and assigned randomly to four groups. Each patient received an initial dose (ID) of either vecuronium (V) or ORG 9487 (O) followed by maintenance doses (MDn) of either V or O (ID/MD: O/O, V/O, O/V, and V/V). The time course of action was measured mechanomyographically, determining the duration until 25% recovery of the single twitch (DUR25). RESULTS: The onset time of an ID of ORG 9487 was shorter than that of an ID of vecuronium (96 vs 203 sec, P < 0.001). The DUR25 of the ID of ORG 9487 was less than half that of vecuronium (10.7 +/- 2.8 vs 28.8 +/- 6.1 min, P < 0.001). The DUR25 of MD1 and MD2 of ORG 9487 were shorter than those of vecuronium (O/O: 7.3 +/- 2.8 and 8.5 +/- 2.4 min; V/O: 12.7 +/- 3.3 and 11.5 +/- 3.5 min, vs O/V: 16.4 +/- 4.5 and 20.6 +/- 4.7 min; V/V: 18.8 +/- 3.0 and 20.1 +/- 3.8 min, respectively, P < 0.05). AN ID of vecuronium prolonged the DUR25 of MD1 and MD2 of ORG 9487 (P < 0.05). CONCLUSION: ORG 9487 is a muscle relaxant with a shorter duration of action than vecuronium. Maintenance doses of ORG 9487 are also shorter acting than roughly equipotent maintenance doses of vecuronium, irrespective of which relaxant is given initially.