مجید کیوانفر

Cystic fibrosis (CF) is a deadly genetic disease that causes various symptoms and complications. This study aimed to evaluate the demographic, clinical and para clinical characteristics among patients with CF.

This cross-sectional study was performed in Imam Hossein (AS) Children's Hospital for one year, from February 1, 2017 to February 1, 2018. About 100 patients referred to Imam Hossein (AS) Children's Hospital in Isfahan diagnosed of cystic fibrosis were included in the study, and all demographic, clinical and laboratory information of the patients was recorded.

Among the CF patients in this study 58% were boys and 42% girls. The average age of the patients was 102 months and within the age range of 5-15 years. The average age of children with CF at the time of diagnosis was 15 months. Around 62% of patients in whom expiratory volume was measured by pressure in one second showed mild involvement, 28% moderate involvement and 10% severe involvement. Gastroesophageal reflux disease (GERD) with 26%, nasal polyp and asthma with relative frequency of 13% and 10%, respectively, were the most common complications among CF patients. Only 18% of CF patients had done genetic testing. Only one patient in the present study used Dornaz alfa. In terms of longevity, only 5% of patients were over 18 years old.

Compared to developed countries that have newborn screening, patients with CF in this study were diagnosed late. Also, CF patients benefited less from the primary drugs that control the disease, and had a shorter lifespan.

Pain assessment and control are essential parts of the treatment of children admitted to the intensive care unit. Moreover, the connection between pain and its physiological/ psychological effects is important. This study aims to evaluate the pain caused by endotracheal tube suctioning in mechanically ventilated children admitted to the intensive care unit.

This study was performed on 50 hospitalized children who were under mechanical ventilation. In these patients, the pain was assessed according to FLACC pain criteria and physiologic indices before and after endotracheal tube suctioning following the standard suction protocol. Patients were divided based on the overall pain score as no pain, mild pain, moderate pain, and severe pain.   

In summary, the average age of the patients was approximately 34 months, and 70% (n = 35) were male. Respiratory diseases (30%, n =15) were the most common initial diagnosis. According to FLACC pain criteria, the frequency and severity of the pain increased after endotracheal tube suctioning (p-value <0.05). The relation between increasing pulse rate, respiration rate, and systolic/diastolic blood pressure with enhancing distribution and pain level after suction is meaningful (P-value <0.001). Also, the patients with higher pain scores received lower doses of analgesic drugs by continuous infusion (P-value <0.05).    

Endotracheal tube suctioning is a painful intervention and patients are distressed following it. Therefore, pain assessment methods and pharmacological and non-pharmacological methods to control the resulting pain are recommended.