Collaboration between two CF centers; one in USA and one in Turkey before and during CoV2 pandemic

Nasr, Samya Z.; Gökdemir, Yasemin; Erdem, Ela; Karakoç, Fazilet; Ergenekon, Pınar; Tapley, Christopher; Dagher, Sharyn; Bouma, Sandra; Keniş-Coşkun, Özge; Kocamaz, Damla; Karadağ, Bülent

doi:10.1002/ppul.26041

Cited by 3 publications

(2 citation statements)

References 5 publications

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“…Twinning schemes are recognized to positively address discrepancies in CF outcomes between international centers 17,18 . Extension of the scope of such schemes to diagnostic infrastructure, alongside the expansion of initiatives across regions such as the Indian subcontinent, Latin America, and the Middle East, could play an important role in improving rates and age of diagnosis.…”

Section: Discussionmentioning

confidence: 99%

International disparities in diagnosis and treatment access for cystic fibrosis

Guo,

King,

Hill

2024

Pediatric Pulmonology

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BackgroundElexacaftor/tezacaftor/ivacaftor (ETI) has revolutionized cystic fibrosis (CF) treatment. However, previous research has demonstrated profound global disparities in diagnosis and treatment access. If unaddressed, these threaten to widen existing health inequities. Therefore, in this analysis we aimed to reappraise gaps and evaluate progress in diagnosis and treatment equity in high‐income (HIC) versus low‐ and middle‐income countries (LMICs).MethodsEstimates of the global CF population were made in 158 countries using patient registries, systematic literature searches, and an international survey of 14 CF experts. Estimates of the global burden of undiagnosed CF were made using epidemiological studies identified in literature searches and registry coverage data. The proportion of people receiving ETI was estimated using publicly available revenue data and a survey of 23 national drug pricing databases.Results188,336 (163,421–209,204) people are estimated to have CF in 96 countries. Of these, 112,955 (60%) were diagnosed and 51,322 (27%) received ETI. The undiagnosed patient burden is estimated to be 75,381 people, with 82% in LMICs. ETI is reimbursed in 35 HICs, but only one LMIC. Four years after approval, there are 14,911 people diagnosed with CF who live in a country where ETI is inaccessible. This increases to 76,199 when including the estimated undiagnosed population.ConclusionsEquitable access to CFTR modulators must become a top priority for the international CF community. ETI costs up to $322,000 per year but could be manufactured for $5000 to allow access under a voluntary license. Given the extent of disparities, other mechanisms to improve access that circumvent the manufacturer should also be considered.

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Section: Discussionmentioning

confidence: 99%

International disparities in diagnosis and treatment access for cystic fibrosis

Guo,

King,

Hill

2024

Pediatric Pulmonology

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“…During 2020 and 2021 physical visits were impossible because of travel restrictions due to the coronavirus pandemic, but online meetings between the two centers took place regularly. Areas for improvement were identified, and viable solutions were proposed by using QI techniques including fishbone diagrams, flow charts, and Plan Do Study Act (PDSA) cycles 13 (Table 1).…”

Section: Methodsmentioning

confidence: 99%

Improvements in body mass index of children with cystic fibrosis following implementation of a standardized nutritional algorithm: A quality improvement project

Gökdemir

Eralp

Ergenekon

et al. 2023

Pediatric Pulmonology

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Background: A collaboration between the University of Michigan (UM) Cystic Fibrosis Center (CFC) and Marmara University (MU) CFC was initiated in MU through conducting Quality Improvement projects (QIP). The global aim was to improve nutritional status of children with CF (cwCF), with a specific aim to increase the mean BMI percentile (BMIp) for cwCF by 10 percentile points in 12 months.Methods: Body mass index (BMI) percentiles of cwCF were categorized as: nutritionally adequate (BMIp ≥ 50%); at risk (BMIp 25%-49%); urgently at risk (BMIp 10%-25%); critically at risk (BMIp < 10%). Appropriate interventions were made according to BMIp category every three months. Forced expiratory volume in one-second percent predicted (FEV1pp), and health-related quality of life (HRQoL) were evaluated.Results: One hundred and eight-two cwCF with a mean age of 9.1 ± 4.3 years were included in the project. Baseline BMIp increased from 25.6 to 37.2 at the 12th month (p < 0.001). In the critically at-risk group BMIp increased from 3.6 to 20.5 (p < 0.001), in the urgently at risk group from 15.9 to 30.8 (p < 0.001), in the at risk group from 37.0 to 44.2 (p < 0.079) and in the nutritionally adequate group the increase was from 66.8 to 69.5 (p < 0.301). FEV1pp also improved significantly, from 81.3 ± 20.6 to 85.9 ± 20.8 (p < 0.001). Physical functioning, eating problems, and respiratory symptoms domains of the HRQoL evaluation improved (p < 0.05). Conclusion:This project has led to significant improvements in BMIp, FEV1pp and HRQoL of cwCF; similar projects could easily be implemented by centers in other developing countries.

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