Safety and efficacy of high-dose enteral, intravenous, and transdermal clonidine for the acute management of severe intractable childhood dystonia and status dystonicus: An illustrative case-series.

OBJECTIVE: Acute dystonia in children is distressing, painful and can progress to life-threatening status dystonicus. Typical management involves benzodiazepines which can result in respiratory depression requiring PICU admission. Clonidine is less respiratory-depressant, and by facilitating sleep, switches dystonia off. It can also be administered via enteral, continuous intravenous infusion, and transdermal slow release routes. We describe the dose range and safety profile of clonidine management in a case-series of children with severe acute exacerbation of dystonia in a tertiary hospital setting. METHODS: The management of 5 children (3 female, age range 8-14 years) suffering from an acute exacerbation of secondary dystonia requiring hospital admission at the Evelina London Children's Hospital was reviewed. The average and maximum dose of clonidine in mcg/kg/h and routes of administration were recorded for each day of hospital admission. Co-administration of any other medical treatments for dystonia and their route of administration were also recorded. Cardiovascular and respiratory clinical status were measured by recording the daily mean and maximum Paediatric Early Warning Scores (PEWS). RESULTS: Clonidine was administered via enteral, intravenous, and transdermal routes at a median dose of 2.5 mcg/kg/h (range 0.1-9 mcg/kg/h). Administration of high dose clonidine was associated with decreased use of benzodiazepines, morphine, and propofol: avoiding invasive respiratory support for ¾ cases during admission. Clonidine doses via all routes of administration did not correlate with poorer PEWS scores (p = 0.839). Both high dose intravenous and transdermal clonidine where found to be effective. CONCLUSIONS: High dose clonidine administered via different routes can be used in the acute management of severe exacerbations of dystonia. Its use in our cohort was not associated with significant cardio-respiratory depression even at doses as high as 9 mcg/kg/h.

INHALER COUNSELLING, THE REAL DEAL OR JUST FRESH AIR?

AIM: To evaluate whether healthcare professionals within the local community are able to counsel paediatric patients on the essential steps required for drug delivery with multi-dose inhalers (MDI), MDIs with a spacer and turbohalers. METHOD: An expert panel produced and piloted checklists for essential and good practice counselling steps. Eligible participants included healthcare professionals regularly counselling children on inhaler devices, including doctors, nurses and pharmacy team members. Participants were recruited through purposive sampling on a general paediatric ward in a major children's hospital over 2 months and local community pharmacies. Participants counselled on each technique through simulated paediatric scenarios and were assessed by a trained researcher. RESULTS: The audit captured 92 healthcare professionals, comprising 43 nurses, 9 doctors, 13 hospital pharmacy staff and 27 community pharmacies team members.Overall 13% (12/92) of participants counselled on all the essential criteria for an MDI inhaler. Pharmacy teams within the hospital and community saw the highest competency levels with 31% (4/13) and 30% (8/27) of staff able to discuss the essential steps respectively, no doctors or nurses were able to indentify all steps.10% (9/92) of participants were able to counsel on all essential steps for a MDI with a spacer device, with no nurse nor doctor achieving all steps. Hospital pharmacy staff were most likely to discuss all the essential steps with 6/13 staff competent and 3/27 community pharmacy team members counselled on the required steps. Commonly omitted steps included shaking the inhaler and leaving sufficient time between doses.Competency levels for turbohaler counselling were low, only 5 participants were able to counsel on the essential steps required. Commonly omitted or incorrect descriptions surrounded the priming of the device and incorrect inhalation technique. CONCLUSION: Our findings mirror those of previously published studies with an adult focused counselling set,these concluded that less than 10% of healthcare professionals were competent with an MDI.1 Though respiratory conditions represent a large proportion of paediatric consultants, healthcare staff are failing to equip patients and carers with the knowledge and skills to utilise even the most common inhaler devices. With the NHS spending £900 million on inhalers2 and hospital admissions increasing dramatically3 more needs to be done to improve inhaler literacy within the local healthcare community.A comprehensive review of training methods needs to be undertaken to ensure these meet the requirements of the local healthcare community. A renewed emphasis on asthma care is required including standard methods for counselling on inhalers.A competency document should be created to assess and validate healthcare professionals to ensure the delivery of accurate and high quality inhaler counselling.

Use of oral clonidine for sedation in ventilated paediatric intensive care patients.

OBJECTIVES: We aimed to document our experience with oral clonidine when used as a sedative in combination with intravenous morphine and lorazepam in a group of mechanically ventilated children with single-organ, respiratory failure. In particular, our objectives were to establish the relationship between oral dose, plasma concentration, and sedative effect, and second, to document the side-effect profile. DESIGN: Prospective, cohort study over a 72-h period. SETTING: Regional paediatric intensive care unit. PATIENTS AND PARTICIPANTS: Twenty-four children were enrolled (median age 3 months) of whom ten were excluded (six due to extubation before 72 h, three sedation failures, one protocol violation). MEASUREMENTS AND RESULTS: Plasma clonidine was measured using gas chromatography mass spectrometry, and sedation assessed using the COMFORT score. Using a dose of 3-5 microg/kg every 8 h, plasma concentrations appeared to plateau at approximately 41 h giving a mean value of 1.38 ng/ml (95% confidence interval 1.0-1.8). Adequate sedation was achieved during 82% (837/1022 h) of the study period; however, this decreased to 70.3% when analysed on an intention-to-treat basis. There was a concomitant overall decrease in the average hourly requirements for both morphine ( P = 0.02) and lorazepam ( P = 0.003). There were no documented episodes of bradycardia, hypotension or hyperglycaemia. CONCLUSIONS: Oral clonidine may be a safe and effective sedative in combination with morphine and lorazepam for young children with single-organ, respiratory failure. This agent may also exhibit opioid and benzodiazepine sparing effects in this patient group. A full pharmacokinetic study is warranted.