Table 1

Synthesis of evidence from papers reviewed organised across levels and health system components along with assessments of their coverage and their comprehensiveness

LevelHealth system componentSummaryCovComp
IndividualLife courseRegional and ethnic variations in clinical presentation.21 25 45
Early diagnosis and prophylaxis at different life stages.30 46
High risk at early childhood and pregnancy.46 47
Limited life-course evidence due to lack of long-term follow-up studies.3 17
Health-seeking behavioursDelayed or lack of antenatal check-ups and screening especially in tribal populations could increase vulnerability and adverse outcomes.29
PopulationEpidemiologyCurrent estimates based on 249 surveys from 75 sources in 141 spatially unique sites with fragmented coverage across tribal and other high-risk populations. Surveys conducted in 18 of 36 Indian states/union territories with 61% completed in 4 states—Gujarat, Maharashtra, Odisha and Chhattisgarh with scheduled groups being the most studied (171 surveys). Little or no data available in Haryana, Uttarakhand, Uttar Pradesh, Bihar, Central Karnataka and Andhra Pradesh, and the NE states.18
Wherever medical records (either electronic or paper based) at health centre exist, such estimates possible but limited experience with EMR.29
Community-based interventionOnly two studies in one of an integrated health network with home visits for education and screening linked to care at a primary care clinic26 and another based on volunteer-led screening at village level.32
Health servicesDiagnostics6 studies reported use of a screening test17 25–27 30 48 followed by confirmatory test26 30 typically in cross-sectional population studies with 1 long-term follow-up study with follow-up of every 3 months.31 2 papers have recommended longer term cohort study to understand natural history of SCD in India.3 17
Community-based screening with home visits linked to primary health centre.26
Lack of a systematic SCD screening17 although feasibility of newborn screening and continuous care for SCD established in tribal and rural population31 48; urgent need to establish uniform gold standard for SCD screening.49
Treatment3 studies examining use of combination of hydroxyurea and/or pneumococcal immunisation26 50 51 with one establishing the need for counselling and follow-up51 and 2 studies on the importance of prophylactic treatment for preventing adverse outcomes.27 31 Of these Dave 2019 was an evaluation of a comprehensive SCD programme. Additional treatment with iron supplements in patients with SCD with iron-deficiency anaemia, particularly in tribal adolescent boys and girls.52 53
Use of electronic medical records in improving treatment outcomes.29
Search for anti-sickling agent continues, including potential ethnopharmacology and bioinformatic-driven approaches.
Rehabilitative servicesNo studies.
Quality of careAlthough mentioned in one study, it was not specifically evaluated.26
Healthcare outcomesThree studies in different parts of India demonstrated Improved utilisation, positive clinical and laboratory outcomes (including decrease in hospitalisation episodes) and acceptable costs of care establishing feasibility of community-based SCD programme.26 31 49 54
Health systemFinancingOnly one study28 assessed overall financing implications of not implementing prevention programmes and newborn screening for SCD and haemoglobinopathies. One other study26 analysed cost details of their community-based intervention.
Health information systems3 studies26 29 31 highlight need for health information systems, of which one29 specifically evaluates use of EMR in SCD programmes and reports on improved programme efficiency, management and analysis of data at community level.
Lack of haemoglobinopathy registry for the country and lack of integrated management and diagnostic facilities for haemoglobinopathies identified as a major deficit.19
Giving parents phone for appointment reminders evaluated in one study.17
Knowledge/awarenessCommunity-based health worker involvement crucial.25 26 Parental education important in SCD life course30 and role of counsellor during clinic visits is important for managing complications, improving adherence.31
GovernanceFew studies directly examined health system governance with respect to SCD. One study examined the need for a national prevention programme28 and another briefly discussed programme/governance gaps in terms of ensuring access to medicines and vaccines for SCD programmes under the National Rural Health Mission. Involvement of NGO partner was crucial in achieving programme outcomes.31
Community engagementTwo studies specifically report on community engagement and find positive outcomes in terms of utilisation driven by community engagement.26 32
ContextSocioeconomicSpecific socioeconomic context examined in few studies beyond reporting association with tribal populations, scheduled caste and low socioeconomic groups with high association with illiteracy and poverty.25 One study reported on positive effects of community organisation and integration with the healthcare network in an Adivasi setting.26
PoliticalTwo studies report on the microprocesses around agenda setting for SCD, of which one found community ownership vital to programme success26 and in another the request for screening was a felt need from the village.32
CulturalThe Adivasi Soliga community in southern Karnataka combines traditional medicine and allopathic treatment.33
  • Cov is short for coverage; comp is short for comprehensiveness. The green colour indicates studies in 4–5 regions, with orange indicating studies in 2–3 regions, and red indicating studies in one region or no studies. Comprehensiveness criteria were established per the criteria established in online supplemental table 2

  • EMR, electronic medical record; NGO, non-governmental organisation; SCD, sickle cell disease.