Quality of life among caregivers of sickle cell disease patients: a cross sectional study.

PURPOSE: To assess the quality of life (QoL) of caregivers of children with sickle cell disease (SCD) and to determine the risk factors associated with poor QoL. METHOD: A cross sectional study was conducted between 01 and 30 June 2015, in a tertiary care center in western Saudi Arabia to assess various dimensions of QoL by using TNO-AZL Questionnaire for Adult's Health-related Quality of Life (TAAQOL). A total 164 adult caregivers (aged 16 years or more) of children with SCD, who were regularly visiting the department were enrolled (refusal rate = 61.6%). The questionnaire scores were transformed into 0-100 scale; with higher scores indicating less difficulty and better QoL Demographic, socioeconomic data and a satisfaction questionnaire regarding participants' lifestyle were collected and analyzed as risk factors for impaired QoL, by comparing different QoL dimensions' scores using independent t-test, Oneway ANOVA, or linear regression, as appropriate. RESULTS: Sixty-three caregivers were included; 79.4% were mothers, age range 21-71 years, 64.5% were from low social class receiving insufficient support and financial needs were unmet for considerable number of families. Analysis of QoL using TAAQOL showed that emotions (median [75th centile] = 44.44 [66.67] for negative and 61.11 [72.22] for positive emotions), sleep quality (66.67 [91.67]) and sexual life (50.00 [83.33]) were the most affected dimensions. Professional achievement (91.67 [100]), cognitive skills (83.33 [100]), and social contact (100 [100]) were relatively preserved. Negative emotions were more marked in mothers and mostly predicted by satisfaction with social relations notably with partner (B = 3.14, p = 0.016), friends (B = 2.51, p = 0.015) and relatives (B = 2.69, p = 0.016). Positive emotions were predicted by the levels of satisfaction of the caregiver with his/her health (B = 2.56, p = 0.001), job achievement (B = 4.54, p = 0.001), living conditions (B = 2.60, p = 0.034) and the condition of the diseased child (B = 2.55, p = 0.011). A strong correlation was found between sleep quality and cognitive skills. CONCLUSION: There are notable financial and emotional burdens on the caregivers of children with SCD affecting various aspects of their QoL, which are likely to be impacted by the individual levels of social and professional achievement. Physicians and health authorities should give particular attention to the QoL of caregivers and families of children with SCD, to help them cope up with the disease and overcome its related psychological and financial impacts.

In vitro performance of prefilled CO2 absorbers with the Zeus®.

Low fresh gas flows (FGFs) decrease the use of anesthetic gases, but increase CO2 absorbent usage. CO2 absorbent usage remains poorly quantified. The goal of this study is to determine canister life of 8 commercially available CO2 absorbent prepacks with the Zeus®. Pre-packed CO2 canisters of 8 different brands were tested in vitro: Amsorb Plus, Spherasorb, LoFloSorb, LithoLyme, SpiraLith, SpheraSorb, Drägersorb 800+, Drägersorb Free, and CO2ntrol. CO2 (160 mL min- 1) flowed into the tip of a 2 L breathing bag that was ventilated with a tidal volume of 500 mL, a respiratory rate of 10/min, and an I:E ratio of 1:1 using the controlled mechanical ventilation mode of the Zeus® (Dräger, Lubeck, Germany). In part I, canister life of 5 canisters each of 2 different lots of each brand was determined with a 350 mL min- 1 FGF. Canister life is the time it takes for the inspired CO2 concentration (FICO2) to rise to 0.5%. In part II, canister life was measured accross a FGF range of 0.25 to 4 L min- 1 for Drägersorb 800+ (2 lots) and SpiraLith (1 lot). In part III, the calculated canister life per 100 g fresh granule content of the different brands was compared between the Zeus and (previously published data for) the Aisys. In vitro canister life of prefilled CO2 absorber canisters differed between brands, and depended on the amount of CO2 absorbent and chemical composition. Canister life expressed as FCU0.5 (the fraction of the canister used per hour) was proportional to FGF over 0.2-2 L min-1 range only, but was non-linear with higher FGF: FCU0.5 was larger than expected with FGF > 2 L min-1, and even with FGF > minute ventilation FCU0.5 did not become zero, indicating some CO2 was being absorbed. Canister life on a per weight basis of the same brand is higher with the Zeus than the Aisys. Canister life of prefilled CO2 absorber canisters differs between brands. The FCU0.5-FGF relationship is not linear across the entire FGF range. Canister life of prepacks of the same brand for the Zeus and Aisys differs, the exact etiology of which is probably multifactorial, and may include differences in the absolute amount of absorbent and different rebreathing characteristics of the machines.

Sphincter sparing resection of a large obstructive distal rectal gastrointestinal stromal tumour after neoadjuvant therapy with imatinib (Glivec).

Gastrointestinal stromal tumours (GISTs) are generally rare but are known to be the most common mesenchymal tumours of the gastrointestinal tract. We present a case of a patient who presented with persistent obstructive gastrointestinal and urological symptoms, a sense of incomplete evacuation and bleeding per rectum. A colonoscopy was performed and a biopsy was taken of a submucosal mass in the distal rectum that revealed a GIST with positive KIT immunostaining. A positron emission tomography (PET)/CT scan showed a large metabolically active distal rectal tumour of 8 cm with macroscopic invasion of surrounding structures. Neoadjuvant therapy with receptor tyrosine kinase inhibitor imatinib (400 mg orally daily) was initiated and an excellent partial response consisting of a significant decrease in the size of the tumour with complete metabolic resolution was observed within 3 months. Six months after initiation of the neoadjuvant therapy a rectum resection with manual side-to-end coloanal anastomosis was performed. Pathology showed a GIST of 5 cm located 0.1 cm from the distal section plane. Our case shows that in patients with a large invasive distal rectal GIST, neoadjuvant imatinib therapy can facilitate anal sphincter sparing surgery.