Clinical Pearl What the case involved: 16 month old female presented to local emergency department with short sudden episodes of floppiness and head dropping, followed by immediate return to normal self. Referred to regional centre for neurology review, 24 hour ECG noted marked bradycardia during episodes of head dropping. On cardiology review there were no abnormalities detected on examination, echo or 12 lead ECG. Multi-disciplinary team approach required to determine management. 24 hour ECG evidenced abrupt onset of complete atrio-ventricular block, ventricular standstill of up to 7 seconds with spontaneous recovery of atrio-ventricular conduction.
Pharmacist contribution: Literature reviewed and treatment options determined by multi-disciplinary team. Evidence strongly supportive of theophylline.1–3 Issues with prescribing and use of unlicensed medicines considered and authorisation obtained. Treatment commenced and 24hour ECG performed and analysed daily to determine effect of theophylline on number of atrio-ventricular block episodes. Pharmacokinetic knowledge applied to determine appropriate dose, dosage interval, interpretation of levels and response to treatment. A family centred care approach was taken throughout and parents understood and engaged with the treatment plan. A response to treatment was observed as demonstrated by reduced clinical symptoms, confirmed by ECG findings which showed a small reduction in the number of atrio-ventricular block episodes. However, parents and nursing staff reported notable drug side effects of sweating, agitation and bad behaviour two hours post dose, despite achieving therapeutic concentratrions. Parents also felt the frequency of drug administration required at home would be difficult to manage and they had a level of anxiety regarding the unpredictable frequency of the episodes and feeling the need to be in attendance at all times. In the long-term this could have significant implications for family life. On review the multi-disciplinary team and parents agreed the response to theophylline was not adequate enough for a long-term option and the patient proceeded to pacemaker insertion. All were in agreement that this was the correct decision.
Outcome Permanent pacemaker inserted, programmed to VVI mode. This mode will prevent bradycardia by pacing the ventricle if there is a loss of atrio-ventricular synchrony and inhibit ventricular pacing in response to intrinsic ventricular rhythm. Most recent pacing check: ventricular pacing 1.8% of the time = approx. 2.7hours/week
Lessons Learned Advantage of multi-disciplinary team approach to care. The benefits of engaging parents in discussions and treatment plans were highlighted and improved the patient and family journey. The multi-disciplinary team acknowledged this and will endeavour to apply this approach in the future. The multi-disciplinary team improved their knowledge of the processes involved when using unlicensed medicines and the complex issues around this. The processes followed in this scenario confirmed the importance of evidence and research to plan future novel treatment options.
Dai AI, Demiryurek AT. Effectiveness oral theophylline, piracetam and iron treatments in children with simple breath-holding spells. Journal of Child Neurology 2020;35:25-30.
Garg M, Goraya JS. Treatment of cyanotic breath-holding spells with oral theophylline in a 10 year old boy. Journal of Child Neurology 2015;30:919-921.
Carano N, Bo I, Zanetti E, et al. Glycopyrrolate and theophylline for the treatment of severe pallid breath-holding spells. Pediatrics 2013;131:1280-1283.
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