Patient suspected susceptibility to malignant hyperthermia: impact of the disease
O impacto da doença em pacientes com suspeita de suscetibilidade à hipertermia maligna: estudo observacional
Gislene Rodrigues, Pamela Vieira Andrade, Joilson Moura dos Santos, Isac de Castro, José Luiz Gomes do Amaral, Helga Cristina Almeida da Silva
Abstract
Introduction
Malignant Hyperthermia (MH) is an inherited hypermetabolic syndrome triggered by exposure to halogenated anesthetics/succinylcholine. The lack of knowledge regarding this condition might be associated with the rare occurrence of MH reaction and symptoms.
Methods
This observational study evaluated 68 patients from 48 families with confirmed or suspected MH susceptibility due to medical history of MH reaction or idiopathic increase of creatine kinase or MH-related myopathies. Participants were assessed by a standardized questionnaire and submitted to physical/neurological examination to assess the characteristics of patients with MH, their knowledge about the disease, and the impact suspected MH had on their daily lives.
Results
Suspected MH impacted the daily life of 50% of patients, creating difficulties in performing surgical/clinical/dental treatment and problems related to their family life/working/practicing sports. The questionnaire on MH revealed a correct answer score of 62.1 ± 20.8 (mean ± standard deviation) on a scale 0 to 100. Abnormal physical/neurological examination findings were detected in 92.6% of susceptible patients.
Conclusions
Suspected MH had impacted the daily lives of most patients, with patients reporting problems even before MH investigation with IVCT. Patients showed a moderate level of knowledge about MH, suggesting the need to implement continuing education programs. MH susceptible patients require regular follow-up by a health team to detect abnormalities during physical and neurological examination.
Keywords
Resumo
Introdução: Hipertermia Maligna (HM) é síndrome hereditária hipermetabólica desencadeada por anestésicos halogenados/succinilcolina. Raridade da crise e sintomas poderia associar-se ao desconhecimento sobre doença.
Métodos: Estudo observacional feito com 68 pacientes de 48 famílias com suscetibilidade conhecida ou suspeita a HM, por história de crise de HM, aumento idiopático de creatinoquinase ou miopatias relacionadas à HM. Os participantes foram avaliados com questionário padronizado e exame físico/neurológico com o objetivo de analisar características de pacientes com HM, seu conhecimento sobre doença e impacto da suspeita no cotidiano.
Resultados: Suspeita de HM impactou a vida diária de 50% dos pacientes com dificuldades para realizar tratamento cirúrgico/clínico/odontológico e problemas na vida familiar/trabalho/esporte. Questionário sobre HM revelou média+desvio padrão do escore de acerto de 62,1+20,8 (escala 0–100). Anormalidades no exame foram detectadas em 92,6% dos pacientes suscetíveis.
Conclusões: Suspeita de HM impactou vida da maioria dos pacientes, com problemas relatados pelo paciente antes mesmo de realizar investigação com IVCT. Pacientes apresentaram grau moderado de conhecimento sobre HM, sugerindo necessidade de implementação de programas de educação continuada. Pacientes suscetíveis necessitam de seguimento periódico pela equipe de saúde, em função de anormalidades detectadas pelo exame físico e neurológico.
Palavras-chave
References
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Submitted date:
07/12/2021
Accepted date:
10/24/2021
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