Global disparities in cystic fibrosis outcomes prior to CFTR modulators: A CF registries cohort study in South Africa and Canada.

BACKGROUND: Outcomes of cystic fibrosis (CF) differ between low-middle income and high-income countries, but comparative data are lacking. We compared South African (SA) and Canadian CF outcomes to explore what disparities existed prior to access of CFTR modulators in Canada. METHODS: A cross-sectional study of SA and Canadian CF registries data for period 1 January to 31 December 2018. CF registry data were harmonised between countries to compare lung function and nutrition outcomes. Poor nutrition was defined as BMIz-score < -1 in children and < 18.5 kg/m2 in adults. Standardised mean difference (SMD) >10 was considered significant. RESULTS: After excluding Canadians on CFTR modulators and lung transplant recipients, data on 4049 Canadian and 446 SA people was analysed. Compared to Canada, people in SA were younger (median age 15.8 years vs. 24.1 years: SMD 52) with fewer males (47.8% vs 54.2%; SMD 12.5) and White (70.9% vs. 93.3%; SMD 61.3). Class I-III CFTR mutation frequency was similar in SA (n = 384, 86.1%) and Canada (n = 3426, 84.9%). After adjusting for age, gender, diagnosis age, genotype, P.aeruginosa infection and pulmonary treatments, FEV1pp was 8.9% lower (95% CI 6.3% to 11.4%) and poor nutrition 1.7-fold more common (OR 1.70; 95% CI 1.19-2.41) in SA compared to Canada. CONCLUSION: Lung function and nutrition was significantly lower in SA compared to Canada. Global disparities in CF outcomes between high and low-middle income countries are likely to widen as CFTR modulators are rapidly scaled up in only high-income countries.

The African Pediatric Fellowship Training Program in Pediatric Pulmonology: A Model for Growing African Capacity in Child Lung Health.

Childhood respiratory diseases are the major cause of mortality and morbidity in African children. However, there is limited expertise in pediatric pulmonology in Africa. The African Pediatric Fellowship Program (APFP) was developed in the Department of Paediatrics and Child Health at the University of Cape Town in partnership with African academic institutions beyond South Africa to promote training of African child health professionals and to build capacity. From 2008 to 2016, 11 fellows have completed APFP training in pediatric pulmonology. Fellows have come from Kenya, Nigeria, Ghana, and Uganda. All but one returned to their home institutions, where they are building academic departments, improving clinical service delivery, growing research capacity, and advancing advocacy and policies to improve child lung health. In parallel, training of South African pediatric pulmonologists has been strengthened with a further nine South African fellows trained during this period. The African Pediatric Pulmonology program provides a highly successful model, with high retention of graduates in their home countries. The long-term goal is to grow African clinical capacity and strengthen services, research, training, and advocacy for child lung health in Africa.

Home Ventilation in South African Children: Do Socioeconomic Factors Matter?

Poor socioeconomic circumstances and poverty are perceived to be barriers to successful home ventilation. Pediatric home ventilation has escalated rapidly in high-income countries but is underreported and underfunded in low-middle income countries. A retrospective chart review covering the past 20 years was carried out at the Red Cross War Memorial Children's Hospital in Cape Town, South Africa, a low-middle income country. Data collection included demographics, socioeconomic and family factors, clinical information, and ventilation-related information. Fifty-five children received home ventilation between 1994 and December 2015 from a median age of 3.5 years (range 0.4-17.6). Thirty-nine (71%) children received invasive ventilation and 16 (29%) children received mask-assisted ventilation. Most common primary diagnosis was a neuromuscular disease (60%). Twenty-six children (47%) were still on home ventilation in December 2015, 8 (15%) had been weaned off ventilation, and 21 (38%) had died. Median time between initiation of ventilation and discharge was 15 days (range 1-52) for mask-assisted ventilation and 88 days (8-991) for tracheostomy-assisted ventilation. Of the total 40 readmissions in the first year of home ventilation, 34 (85%) were emergency readmissions mainly necessitated by respiratory infections (n = 26; 65%). Despite a high prevalence of socioeconomic challenges, 89% of the children were successfully discharged on home ventilation. Main cause of death was acute infections (n = 11; 52%). Pediatric home ventilation in South Africa is feasible despite difficult socioeconomic circumstances. Survival outcome was comparable with that of high-income countries. However, a high level of psychosocial support and interventions is needed.

Paediatric tracheostomy and ventilation home care with challenging socio-economic circumstances in South Africa.

BACKGROUND: Children discharged home with a tracheostomy need a safe home environment and access to health care. We described the indications, clinical characteristics, socio-economic circumstances and outcomes of children enroled in a tracheostomy home care programme in South Africa. METHODS: We performed a retrospective chart review of children receiving a tracheostomy and enroled in the Breatheasy programme at the Red Cross War Memorial Children's Hospital, Cape Town. Medical and background characteristics were recorded. Influences of socio-economic variables and underlying medical conditions on length of hospital stay, unplanned readmissions and mortality in the first year after discharge were evaluated. RESULTS: In the period 2008-2012, 157 patients were discharged home with a tracheostomy. Median hospital stay after tracheostomy insertion was significantly longer when parents had incomplete schooling compared to completed secondary school or higher education; 30 days (IQR 21-53) versus 23 days (IQR 16-33), respectively. Unplanned readmissions in the first year were documented for 72 patients (45.9%). The risk for unplanned readmission was 2.6 times higher in families with substance abuse the risk of respiratory infections was two-fold in case of household cigarette smoke exposure (OR 2.3.) Tracheostomy-related mortality was low (1.2%). An underlying medical condition was the only independent significant risk factor for mortality (OR 5.1, 95% CI 1.8-14.3). CONCLUSION: This study demonstrates that despite difficult socio-economic circumstances, home ventilation of children with a tracheostomy is safe, provided caregivers are adequately trained and supported.