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Assessment of care of children with sickle cell disease: implications for neonatal screening programmes
1Department of Community Medicine, United Medical School, Guy's Hospital, London.
Insights
Quality of care for young children with sickle cell disease (SCD) is suboptimal. Many children miss appointments and essential blood tests, with inconsistent prophylactic treatment, highlighting a need for better follow-up after neonatal screening.
Area of Science:
- Pediatric Hematology
- Public Health
- Quality Improvement
Background:
- Sickle cell disease (SCD) requires ongoing specialized care.
- Neonatal screening identifies infants with SCD early.
- Effective management relies on consistent outpatient care and family support.
Purpose of the Study:
- To evaluate the quality of hospital care for pediatric patients with sickle cell disease.
- To identify gaps in outpatient management and family support for children with SCD.
- To assess adherence to recommended care standards for young children with SCD.
Main Methods:
- Retrospective survey of 31 children with sickle cell disease treated at a London teaching hospital.
- Data collected via medical record review and semi-structured carer questionnaires.
- Assessment focused on outpatient attendance, blood testing, prophylactic treatment, and family support.
Main Results:
- Irregular clinic attendance was noted, with 13 children attending less than every six months.
- Essential blood tests were infrequent; only eight children received the recommended three tests.
- Prophylactic penicillin and folic acid treatment was inconsistent, with some children not receiving regular penicillin.
Conclusions:
- Current care for young children with sickle cell disease falls short of optimal standards.
- Neonatal screening for SCD must be integrated with robust follow-up protocols.
- Improved adherence to care guidelines is crucial for better patient outcomes.
Objective:
To assess the quality of care provided by hospitals for young children with sickle cell disease.
Design:
Retrospective survey.
Setting:
Teaching hospital in London.
Patients:
31 Children (mean age 4 years 4 months, range 4 months to 7 years 5 months) born with sickle cell disease between 1978 and 1985 identified from Hospital Activity Analysis data, an outpatient diagnostic register, and registers of the haematology department. Eight had been diagnosed on neonatal screening and at least four of these had not been followed up.
Main Outcome Measures:
Aspects of quality of outpatient care (blood testing, clinic attendance, and prophylactic drug treatment) and family care (adequate support and carers' knowledge about the disease) as assessed by reviewing the notes and administering a semistructured questionnaire to the carers, in relation to a devised list of standards deemed necessary to ensure achievement of the aims of screening.
Results:
There were 93 outpatient attendances during the previous 12 months, but 13 children had not attended at least every six months and four not at all for more than a year. Only eight children had had three of the blood tests considered to be necessary for good care; three had had none. Prophylactic treatment with penicillin and folic acid was erratic; three children with sickle cell anaemia were not receiving regular prophylactic penicillin.
Implication:
Diagnosis of sickle cell disease on neonatal screening must be linked with follow up to ensure optimal management.