Challenges in screening for sickle cell disease among newborns from the tribal region of Palghar, Maharashtra during the COVID-19 pandemic

Research on health of tribal newborns or children is quite intricate as it involves participation of parents and families as well1. Familial and sociocultural concerns are integral while planning research in the tribal population2. Despite associated challenges, it is however crucial to sustain research having high public health relevance among tribal population such as sickle cell disease (SCD). The sickle gene is reportedly widespread among many tribal groups in India with the prevalence of heterozygotes varying from one to 40 per cent; more than 5000 cases of SCD have been identified in Gadchiroli, Chandrapur and Nandurbar in Maharashtra3,4. Newborn screening, early intervention and comprehensive integrated care are important management aspects of SCD5-8. However, newborn screening and testing facilities are lacking at sub-district level. Since sustainability of positive outcomes remain critical following research conducted among tribal population for their uptake at programmatic levels9,10, the objectives of this multi-centric study were to establish a newborn screening programme for SCD in tribes for early detection to understand the barriers for undertaking such a programme and to measure the benefits of early comprehensive care in affected babies. The Model Rural Health Research Unit (MRHRU), Dahanu was the participating centre in Maharashtra for implementation of this study.

The COVID-19 pandemic imposed several adversities in the implementation of research worldwide resulting in a significant decline in enrolment and interrupted follow ups11. The aim of this study was to identify the challenges faced in implementing newborn screening and follow up of babies with SCD or trait during the COVID-19 pandemic and to document the measures and strategies, which were developed to overcome them in the tribal region of Palghar district, Maharashtra.

Material & Methods

This study was part of a large multicentric initiative under the aegis of the Indian Council of Medical Research-National Institute of Immuno Haematology (ICMR-NIIH), Mumbai, Maharashtra. This initiative included seven tribal centres (Maharashtra – Chandrapur and Dahanu, Gujrat – Jhagadia, Tamil Nadu – Kotagiri, Odisha – Bhubaneswar, Madhya Pradesh – Jabalpur and Rajasthan – Jodhpur). The study was initiated after obtaining necessary administrative and ethical approvals in December 2019. Initially, meetings were held with the District Health Officials, gatekeepers of the community (Sarpanch, local leaders) and community awareness activities were conducted for project sensitization.

The newborns delivered at the Sub District hospital, Dahanu in Maharashtra from December 2019 to September 2021 were screened for SCD. The still births, and intrauterine fetal demise (IUFDs) were excluded. Parents were approached in labour ward before delivery and cord blood (2 ml)/heel prick (0.5 ml) blood was collected after obtaining written informed consent from either of the parents. The initial screening was performed by high performance liquid chromatography (HPLC) using the variant Hb testing system (Bio-Rad Labs, USA) to diagnose haemoglobin phenotypes as sickle cell homozygous (SS), sickle cell trait (AS), other haemoglobin variants and normal babies (AA). The newborns having SS, AS or other haemoglobin variants were followed up at six weeks for confirmation of diagnosis, along with the screening of parents and siblings. Genetic counselling of the parents was done for future pregnancies. The babies having HBSS or HbS-β thalassaemia were provided with early intervention in the form of folic acid, prophylactic antibiotics, immunization and were followed up further till 36 months of age through MRHRU.

During the COVID-19 pandemic from April 2020 to September 2021, challenges associated with implementation of the project, were documented by the study team based on its interactions with the healthcare providers and study participants.

Results

The challenges identified with respect to newborn screening and follow up, as depicted in Figure 1 are enlisted below.

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Fig. 1

Flow chart describing the planned strategy. Measure 1: Community awareness activities on SCD in anganwadis and PHCs following social distancing norms; Measure 2: Restricting up to three families per day with adequate sanitization measures and social distancing norms; Measure 3: Virtual genetic counselling started with the help of geneticists for sickle cell trait as well as SCD trait at MRHRU; Measure 4: Facilitating telemedicine follow up at tertiary care centre follow up at PHCs, subcentres and anganwadis, facilitating evaluation of affected children at tertiary healthcare facility having expertise in hemoglobinopathies babies at MRHRU; Measure 5: Follow up was adjusted in accordance to work schedule of parents, facilitated at PHCs, subcentres through ASHAs, ANMs, AWW, CHV. SDH, sub district hospital; MRHRU, model rural health research unit; PHCs, primary health centres; ANM, auxiliary nurse midwife; AWW, anganwadi workers; CHV, community health worker; ASHAs, accredited social health activist.

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Operational issues posed as difficulties after the nationwide lockdown in April 2020, travel was forbidden and the local transport mechanisms came to a halt. Due to geographical diversities, parents required travelling greater distances (30-75 km) to reach healthcare facilities. The number of institutional deliveries was reduced as the study site was turned into a COVID-19 facility, diverting delivery load to other facilities and also due to frequent closure of labour room for fumigation due to COVID-19 cases.

Fear of contracting COVID-19 among parents caused reluctance for follow up at healthcare facilities. The COVID-19 cases in the family as well as the vicinity also affected follow up to some extent. Shortage of staff due to COVID-19 infection and posting for COVID-19 duties also hampered project work such as cord blood collection and sample processing for SCD. Other miscellaneous non-COVID-19 reasons existed before the initiation of study and continued to be a challenge during COVID-19 period as well.

Poor awareness regarding health, mainly due to low level of literacy and seemingly healthy looking babies increased the reluctance of parents for further follow up regarding SCD and trait. Non-availability of parents was major concern as majority of fathers/parents were staying away on boat for fishing or migrated to the nearby districts for work. They also avoided follow up during sowing season, harvesting and festivals. Poor network issues, loss of wages incurred for follow up visits and high cost of transport were other barriers. Lack of specialist care and referral centre in the district also affected routine follow ups and management. Many parents were not comfortable to seek care at tertiary care facilities in view of their earlier negative experiences. Measures and strategies were evolved while facing these challenges (Fig. 1).

During the initial phase, babies were followed up telephonically by medical officers (MO) with the involvement of accredited social health activists (ASHAs) of nearby primary health centres (PHCs). Newborn screening was resumed in July 2020 with all necessary precautions. Follow up was initiated at MRHRU, Dahanu, keeping the number restricted up to three families per day with maintenance of adequate sanitization measures and social distancing norms. The follow up, prophylactic treatment and immunization were initiated through PHCs, and sub-centres as per the feasibility of the concerned families. Speciality care was integrated into the study by facilitating follow ups at the tertiary care facility, importantly using telephonic consultation during the lockdown.

Various measures for counselling of mothers and fathers, involvement of healthcare workers (91 ASHA and 63 Auxiliary nurse midwife; ANM), virtual platforms and flexibility of facilitating visits at nearby PHCs improved enrolment and follow up of newborns (Table).

During the study, a total of eight babies were identified with SS, 163 with AS and 141 with indeterminate results. From December 2019 to March 2020, a total of 696 newborns were screened, out of which follow up was needed with 75 newborns (SCD, traits or indeterminate results) up to February 2020. The follow up was possible in 26.7 per cent (20/75) babies from February 2020 to 23^rd March, 2020. During the lockdown imposed in the first wave (23^rd March - June 31, 2020), babies were followed up only telephonically. Despite various challenges, as mentioned above, with the implementation strategies we adopted, follow up rate increased to 47.5 per cent (66/139) from July 2020 to April 2021. The follow up rate further increased to 66 per cent (65/98) of babies during the second wave of COVID-19 (May-August 2021). Figure 2 depicts the trend of follow up at various time points.

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Fig. 2

Follow up rates at different time points (Baseline, First wave, Second wave).

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Discussion

This investigation describes challenges in the recruitment and follow up of newborns for SCD in tribal regions of Maharashtra during the COVID-19 pandemic. The pandemic had a huge impact on research affecting enrolment of participants, and follow ups including systematic monitoring of haemaglobinopathies12-14. Our study required long-term follow up in tribal settings, which imposed further challenges due to travel restrictions, fear of COVID-19 and shortage of staff during implementation of the project.

The study revealed that adopting new approaches such as switching to virtual follow ups and telemedicine resulted in significant improvement similar to a few earlier studies15. Telemedicine has shown above-average usability and high satisfaction for SCD patients by improving access to appropriate medical care16,17. Nevertheless, certain limitations of telemedicine, such as lack of physical examination for clinical assessment, should be kept in consideration, as highlighted by some researchers17.

Our study showed the three-dimensional impact of the aforementioned new approaches on SCD management with respect to confirmation of diagnosis and family screening, timely initiation of preventive measures and finally, genetic counselling for parents of the affected babies. This was achieved through amicable strategies as well as engagement and capacity building of healthcare workers, community gatekeepers and by conducting outreach programmes18,19.

The results of this study highlight the importance of building up of provider network to ensure optimal medical care, psychosocial support and genetic counselling to reduce morbidity and mortality20. Operationalizing the study at all health facility levels proved to have positive impact on screening and receptiveness by the community21.

Genetic counselling is an integral and important pillar of the preventive strategy of SCD, enabling opportunity for prenatal diagnosis and avoiding detrimental outcomes of future pregnancies22. The use of virtual platforms for counselling in pandemic settings proved beneficial, as reported earlier23. Virtual counselling in the middle of sociocultural diversities in tribal settings also helped in preventing unplanned pregnancies. The need for adopting specific approaches in conducting research in tribal areas is recommended because of diverse cultural, traditional, religious and socio-economic factors, along with enhanced communication and networking strategies to improve follow up during the pandemic16,24-26.

The limitation of this study was that it depicted challenges with respect to one tribal setting, which might not represent the situation at other six centres. Despite this shortcoming, the findings of this study throw light on the various challenges and avenues for sustaining research in tribal settings, such as by introducing virtual platforms for addressing health needs, and establishing telemedicine facilities in tribal setup through engagement of community health workers. The findings have the potential to pave a path for further research involving specific interventions to improve research outcomes in tribal settings.