Survey of parents’ perception and perspective on airway and anesthetic management in their children with Pitt Hopkins syndrome: Mapping out their clinical care odyssey

Abstract

Objective: Pitt-Hopkins Syndrome (PTHS) is characterized by cognitive dysfunction, developmental delays, seizures, and several anatomical abnormalities. These abnormalities may have an impact on airway and anesthetic management. In rare syndromes, many variants exist with potentially different interactions between the children’s medications used for treating their condition and anesthetic agents. The study’s aim was to develop a survey for the parents of patients with PTHS to determine their perception related to airway and anesthetic management. Further, in order to obtain an in depth point of view on their children’s experiences we examined the parents’ perspective.
Methodology: A survey (paper and web-based) consisting of 31 questions was developed and distributed at the Pitt-Hopkins Research Foundation Scientific Symposium and Family Conference in Dallas, Texas from November 3-5, 2016. The survey questions were developed after an extensive literature review of PTHS supplemented with input from certain families of patients with PTHS. The focus of the survey was to obtain the parents’ perception related to airway and anesthetic management and additionally, their perspective of overall care using follow-up telephone calls and interviews to develop narratives.
Results: We received 32 survey responses (31 paper and 1 web). Five were not analyzed since the children had not received either sedation or general anesthesia. The parents’ perceptions related to airway and anesthetic management were as follows: 22 of 27 (81.4%) patients did not report any complications related to airway management. There were no reported episodes of aspiration even though 23 of 27 (85.2%) patients suffered from GI issues: either upper (reflux), lower (constipation), or both. Sixty-three percent (17/27) of respondents reported poor quality of emergence, while 26% (7/27) described delayed emergence. We found there were many different variants of patients with PTHS regarding their verbal, cognitive, and ambulatory abilities, leading to a wide variety of experiences with anesthetics obtained from parents’ interviews and narratives.
Conclusion: Our study found a low incidence of problems with airway management in children with Pitt-Hopkins syndrome. Their problems with anesthetic care were specifically related to poor quality of emergence, though the exact etiology was not clear. Their perspective in the form of narratives offered more insight into their fears, frustrations, and expectations for their children’s care. We obtained valuable information on their experiences after discharge, which is usually not available on the medical records or hospital documentation charts.