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. 2016 Oct 6;10(10):CD011175.
doi: 10.1002/14651858.CD011175.pub2.

Interventions for patients and caregivers to improve knowledge of sickle cell disease and recognition of its related complications

Monika R Asnani  1 , Kim R Quimby, Nadia R Bennett, Damian K Francis
Affiliations

Interventions for patients and caregivers to improve knowledge of sickle cell disease and recognition of its related complications

Monika R Asnani et al. Cochrane Database Syst Rev. .

Abstract

Background: Sickle cell disease is a group of genetic diseases which is especially prevalent in tropical and subtropical regions; however, forced migration and ongoing population movement have spread it throughout the world, with estimated birth rates reaching 0.49 per 1000 in the Americas, 0.07 per 1000 in Europe, 0.68 per 1000 in South and Southeast Asia, and 10.68 per 1000 in Africa. Life for individuals with sickle cell disease can be affected by repeated acute complications and compounded by progressive organ damage. Studies reveal that when people with chronic illness learn self-management, their clinical outcomes and quality of life improves; and they show lower dependence on healthcare services. There are, however, no reviews identifying which interventions improve knowledge and little is known about the impact of patient or care-giver knowledge on clinical and psychosocial outcomes in people with sickle cell disease.

Objectives: 1. To determine the effectiveness of patient- and caregiver-centred educational interventions for changing knowledge and understanding of sickle cell disease among patients as well as caregivers of people with the disease.2. To assess the effectiveness and safety of patient- and caregiver-centred educational interventions and programs for the recognition of signs and symptoms of disease-related morbidity, adherence to treatment and healthcare utilization in patients with sickle cell disease.

Search methods: The authors searched the Cochrane Cystic Fibrosis and Genetic Disorders Group Haemoglobinopathies Trials Register, compiled from electronic database searches and handsearching of journals and conference abstract books. Additional trials were sought from the reference lists of the trials and reviews identified by the search strategy.Date of last search: 11 April 2016.

Selection criteria: Randomized and quasi-randomized controlled trials which evaluate the effectiveness of individual- and group-based interventions for either the patient with sickle cell disease or their caregivers, or both. Eligible interventions will aim to change knowledge, attitudes or skills, improve psychosocial aspects of the disease as well as treatment adherence and healthcare utilization. Trials evaluating the intervention versus no program, comparing two interventions and those which are part of a multi-faceted intervention to improve a range of sickle cell-related health outcomes are all eligible for inclusion.

Data collection and analysis: Two review authors independently selected trials based on stated inclusion criteria and thereafter examined each selected report to extract data using a prepared, piloted, data collection form. A third author assisted in reaching consensus if there were any discrepancies. Similarly, risk of bias was assessed by two authors and verified by a third author.

Main results: A total of 12 trials (11 randomized controlled trials and one quasi-randomized trial) of 563 people with HbSS, HbSC or HbSβthal, aged six to 35 years old, were included in the review; the majority of participants were African-American. Interventions ranged from a total of one hour to weekly sessions for eight weeks and the post-intervention assessments ranged from the end of the intervention period to 12 months after completion. The heterogeneity of the included trials, which encompasses setting, inclusion and exclusion criteria, interventional method and time of assessment, ranged from 'not important' to 'moderate to substantial' for different review outcomes. The overall risk of bias was low for selective reporting, unclear for random sequence generation, allocation concealment, blinding of participants and blinding of outcome assessment. Incomplete outcome reporting and blinding of personnel showed mixed bias representations.Patient knowledge was assessed by four trials (160 participants) with moderate to substantial heterogeneity. There was evidence that educational programs improved patient knowledge, standardised mean difference 0.87 points (95% confidence interval 0.28 to 1.45, moderate quality evidence), which improved further when a trial with high bias was removed in a sensitivity analysis. Caregiver knowledge, reported in a single trial of 20 families, also showed an improvement, standardised mean difference 0.52 points (95% confidence interval 0.03 to 1.00, moderate quality evidence). The effect on patient knowledge was sustained at longer follow-up periods, whereas the effect on caregiver knowledge was not sustained.There were two primary outcomes related to the effectiveness of educational programs on the recognition of signs and symptoms of disease-related morbidity. No comparative data were reported for patients or caregivers (or both) recognising signs and symptoms leading to self-management. Data from two trials were analysed for the utilization of health services and showed no evidence of an effect, mean difference 0.33 (95% confidence interval -0.57 to 1.23, moderate quality evidence).With regard to the review's secondary outcomes, depression showed a statistically significant decline in intervention groups, standardised mean difference -0.66 points (95% confidence interval -1.18, to -0.14, moderate quality evidence). Adherence to treatment was not assessed in any of the identified trials. No effects of interventions were seen on coping, family relationships or health-related quality of life of patients.The quality of evidence was low for positive coping and moderate for child knowledge, healthcare utilization and depression. This suggests that further research is likely to have an important impact on our confidence in the estimate of effect and may change the estimates.

Authors' conclusions: This review identifies important positive effects of educational interventions on improving patient knowledge of sickle cell disease and depression. Effects on patients' knowledge were maintained for longer than for caregivers. The effect on knowledge was significant but small and whether it offers any clinical benefit is uncertain. Significant factors limiting these effects could be trials being under powered as well as attrition rates. Effects were not statistically significant in assessments of secondary outcomes, possibly due to the paucity of the number of trials and patients and caregivers. Trials showed moderate to high heterogeneity which might impact the results. To better study effects on outcomes, further controlled trials are needed with rigorous attention given to improve recruitment and retention and to decrease bias. Predetermined protocols using similar measurements should be used across multiple sites.

PubMed Disclaimer

Conflict of interest statement

Monika Asnani: none known.

Kim Quimby: none known.

Nadia Bennett: none known.

Damian Francis: none known.

Figures

1
1
Study flow diagram.
2
2
Risk of bias graph: review authors' judgements about each risk of bias item presented as percentages across all included studies.
3
3
Risk of bias summary: review authors' judgements about each risk of bias item for each included study.
1.1
1.1. Analysis
Comparison 1 Psycho‐education vs control ‐ patients, Outcome 1 SCD knowledge: patients.
1.2
1.2. Analysis
Comparison 1 Psycho‐education vs control ‐ patients, Outcome 2 Sensitivity analysis risk of bias: SCD knowledge: patients.
1.3
1.3. Analysis
Comparison 1 Psycho‐education vs control ‐ patients, Outcome 3 Healthcare utilization.
1.4
1.4. Analysis
Comparison 1 Psycho‐education vs control ‐ patients, Outcome 4 Change in caregiver/patient skills.
1.5
1.5. Analysis
Comparison 1 Psycho‐education vs control ‐ patients, Outcome 5 Family relationship.
1.6
1.6. Analysis
Comparison 1 Psycho‐education vs control ‐ patients, Outcome 6 Depression.
1.7
1.7. Analysis
Comparison 1 Psycho‐education vs control ‐ patients, Outcome 7 Disease self‐efficacy.
1.8
1.8. Analysis
Comparison 1 Psycho‐education vs control ‐ patients, Outcome 8 Positive coping.
1.9
1.9. Analysis
Comparison 1 Psycho‐education vs control ‐ patients, Outcome 9 Negative coping.
1.10
1.10. Analysis
Comparison 1 Psycho‐education vs control ‐ patients, Outcome 10 Emotional & behavioural difficulties.
1.11
1.11. Analysis
Comparison 1 Psycho‐education vs control ‐ patients, Outcome 11 Health‐related QOL.
2.1
2.1. Analysis
Comparison 2 Psycho‐education vs control ‐ caregiver, Outcome 1 SCD knowledge: parent.
2.2
2.2. Analysis
Comparison 2 Psycho‐education vs control ‐ caregiver, Outcome 2 Family relationship.
See this image and copyright information in PMC

Update of

  • doi: 10.1002/14651858.CD011175

References

References to studies included in this review

Barakat 2010 {published data only}
    1. Barakat LP, Schwartz L, Simon K, Radcliffe J. Coping mediates the association of pain with depression in adolescents with sickle cell disease [abstract]. Proceedings of the 29th Annual Meeting of the National Sickle Cell Disease Program. 2006:187.
    1. Barakat LP, Schwartz LA, Salamon KS, Radcliffe J. A family‐based randomized controlled trial of pain intervention for adolescents with sickle cell disease. Journal of Pediatric Hematology/Oncology 2010;32(7):540. - PMC - PubMed
    1. Bennett KY, Simon K, Barakat LP, Radcliffe J. Factors associated with disease knowledge in adolescence [abstract]. Proceedings of the 29th Annual Meeting of the National Sickle Cell Disease Program. 2006:190.
    1. Boyd RC, Barakat LP, Thigpen NR, Radcliffe J. The development of a brief psychoeducational intervention for adolescents with sickle cell [abstract]. Proceedings of the 27th Annual Meeting of the National Sickle Cell Disease Program. 2004:84.
    1. Radcliffe J, Barakat LP, Boyd R, Thigpen N. Sickle cell disease knowledge and self‐efficacy are associated among adolescents and caregivers [abstract]. Proceedings of the 28th Annual Meeting of the National Sickle Cell Disease Program. 2005:119.
Broome 2001 {published data only}
    1. Broome ME, Maikler V, Kelber S, Bailey P, Lea G. An intervention to increase coping and reduce health care utilization for school‐age children and adolescents with sickle cell disease. Journal of National Black Nurses' Association: JNBNA 2001;12(2):6‐14. - PubMed
Daniel 2015 {published data only}
    1. Daniel LC, Li Y, Smith K, Tarazi R, Robinson MR, Patterson CA, et al. Lessons learned from a randomized controlled trial of a family‐based intervention to promote school functioning for school‐age children with sickle cell disease. Journal of Pediatric Psychology 2015;40(10):1085‐94. [CENTRAL: 1102193; CRS: 5500135000001413; PUBMED: 26136404] - PMC - PubMed
Gil 1996 {published data only}
    1. Gil KM, Carson JW, Sedway JA, Porter LS, Schaeffer JJW, Orringer E. Follow‐up of coping skills training in adults with sickle cell disease: analysis of daily pain and coping practice diaries. Health Psychology 2000;19(1):85‐90. - PubMed
    1. Gil KM, Wilson JJ, Abrams MA, Orringer E, Clark WC, Janal MN. Coping skills training in adults with sickle cell disease: Post‐intervention and 3‐month follow‐up results [abstract]. Proceedings of the National Sickle Cell Disease Program Annual Meeting Conference. 1996:8.
    1. Gil KM, Wilson JJ, Edens JL, Webster DA, Abrams MA, Orringer E, et al. Effects of cognitive coping skills training on coping strategies and experimental pain sensitivity in African American adults with sickle cell disease. Health Psychology 1996;15(1):3. - PubMed
Gil 1997 {published data only}
    1. Gil KM, Anthony KK, Carson JW, Redding‐Lallinger R, Daeschner CW, Ware RE. Daily coping practice predicts treatment effects in children with sickle cell disease. Journal of Pediatric Psychology 2001;26(3):163‐73. - PubMed
    1. Gil KM, Wilson JJ, Edens JL, Workman E, Ready J, Sedway J, et al. Cognitive coping skills training in children with sickle cell disease pain. International Journal of Behavioral Medicine 1997;4(4):364‐77. - PubMed
Hazzard 2002 {published data only}
    1. Hazzard A, Celano M, Collins M. Effects of STARBRIGHT World on knowledge, social support, and coping in hospitalized children with sickle cell disease and asthma. Children's Health Care 2002;31(1):69‐86.
Kaslow 2000 {published data only}
    1. Kaslow NJ, Collins MH, Brown F, Baskin M, Griffith J, Hollins LD, et al. Psychoeducational intervention for pediatric sickle cell disease (SCD) increases disease knowledge [abstract]. Proceedings of the National Sickle Cell Disease Program Annual Meeting Conference. 1997:225.
    1. Kaslow NJ, Collins MH, Rashid FL, Baskin ML, Griffith JR, Hollins L, et al. The efficacy of a pilot family psychoeducational intervention for pediatric sickle cell disease (SCD). Families, Systems, & Health 2000;18(4):381.
Ketchen 2006 {published data only}
    1. Ketchen B, Hazzard A, Lassiter S, Barber N, Armistead L, Mentz R, et al. STARBRIGHT World: A pilot study of a home‐based sickle cell psychoeducational intervention. Children's Health Care 2006;35(4):321‐38.
    1. Kinlow M, Hazzard A, Adams F, Batts B. Computer based interactive education programs for hospitalized children with asthma and sickle cell disease [abstract]. Respiratory Care. 2001; Vol. 46:1112.
Koontz 2004 {published data only}
    1. Koontz K, Short AD, Kalinyak K, Noll RB. A randomized, controlled pilot trial of a school intervention for children with sickle cell anemia. Journal of Pediatric Psychology 2004;29(1):7‐17. - PubMed
Lemanek 2006 {published and unpublished data}
    1. Lemanek KL, Ranalli M, Parsons E, DeJacimo L. Effectiveness of auxiliary sickle cell disease management guidelines of children hospitalized with pain [abstract]. Proceedings of the 29th Annual Meeting of the National Sickle Cell Disease Program. 2006:134.
Schatz 2015 {published data only}
    1. Schatz J, Schlenz AM, McClellan CB, Puffer ES, Hardy S, Pfeiffer M, et al. Changes in coping, pain, and activity after cognitive‐behavioral training: a randomized clinical trial for pediatric sickle cell disease using smartphones. Clinical Journal of Pain 2015;31(6):536‐47. [CENTRAL: 1068102; CRS: 5500133000000033; GR:: F31 HL108582/HL/NHLBI NIH HHS/United States; JID:: 8507389; NLM:: PMC4424076 [Available on 06/01/16]; OID: [Other ID]: NLM: NIHMS639485 [Available on 06/01/16]; PMCID:: PMC4424076; PUBMED: 25503599; R21: HL092336/HL/NHLBI NIH HHS/United States; T32: GM081740/GM/NIGMS NIH HHS/United States] - PMC - PubMed
Thomas 1999 {published data only}
    1. Thomas V. Cognitive behaviour therapy for the management of sickle cell disease pain: an evaluation of a community based intervention [abstract]. Proceedings of the National Sickle Cell Disease Program Annual Meeting Conference. 1999:230.
    1. Thomas V. Cognitive behavioural therapy for the management of pain in sickle cell disease [abstract]. Proceedings of the National Sickle Cell Disease Program Annual Meeting Conference. 1997:38.
    1. Thomas V. Cognitive behavioural therapy in pain management for sickle cell disease. International Journal of Palliative Nursing 2000;6(9):434‐42. - PubMed
    1. Thomas V. The longitudinal effects of cognitive behaviour therapy for the management of sick cell disease pain [abstract]. Proceedings of the National Sickle Cell Disease Program Annual Meeting Conference. 2000:15.
    1. Thomas V, Dixon A, Milligan P, Thomas N. Cognitive‐behaviour therapy for the management of sickle cell disease pain: An evaluation of a community‐based intervention. British Journal of Health Psychology 1999;4(3):209‐29.

References to studies excluded from this review

Applegate 2003 {published data only}
    1. Applegate H, Webb PM, Elkin TD, Neul SKT, Drabman RS. Improving parent participation at pediatric diabetes and sickle cell appointments using a brief intervention. Children's Health Care 2003;32(2):125‐36.
Barakat 2007 {published data only}
    1. Barakat LP, Schwartz LA, Simon K, Radcliffe J. Negative thinking as a coping strategy mediator of pain and internalizing symptoms in adolescents with sickle cell disease. Journal of Behavioral Medicine 2007;30(3):540‐7. - PubMed
Boroffice 1991 {published data only}
    1. Boroffice OB. Self‐help in sickle‐cell disease in Nigeria. Health Promotion International 1991;6(4):241‐6.
Carpenter 2007 {published data only}
    1. Carpenter S, Barakat LP, Radcliffe J. Health education in pediatric sickle cell disease: facilitating increased knowledge by using games [abstract]. Proceedings of the 35th Anniversary Convention of the National Sickle Cell Disease Program. 2007:347.
Chernoff 2002 {published data only}
    1. Chernoff RG, Ireys HT, DeVet KA, Kim YJ. A randomized controlled trial of a community‐based support program for families of children with chronic illnesses: pediatric outcomes. Archies of Pediatrics and Adolescents Medicine 2002;156(6):533‐9. - PubMed
Daly 2012 {published data only}
    1. Daly B, Kral MC, Brown RT, Elkin D, Madan‐Swain A, Mitchell M, et al. Ameliorating attention problems in children with sickle cell disease: a pilot study of methylphenidate. Journal of Developmental and Behavioral Pediatrics 2012;33(3):244‐51. [Record 14: 6125 ] - PMC - PubMed
Ireys 2001 {published data only}
    1. Ireys HT, Chernoff R, DeVet KA, Kim Y. Maternal outcomes of a randomized controlled Trial of a community‐based support Program for families of children with chronic illnesses. Archies of Pediatrics and Adolescents Medicine 2001;155(7):771‐7. - PubMed
Jenerette 2014 {published data only}
    1. Jenerette CM, Brewer CA, Edwards LJ, Mishel MH, Gil KM. An intervention to decrease stigma in young adults with sickle cell disease. Western Journal of Nursing Research 2014;36(5):599‐619. [CENTRAL: 1068104; CRS: 5500133000000035; GR:: K23NR011061/NR/NINR NIH HHS/United States; JID:: 7905435; PUBMED: 24309381; UL1TR000083/TR/NCATS: NIH HHS/United States] - PubMed
Kaslow 1997 {published data only}
    1. Kaslow NJ, Collins MH, Loundy MR, Brown F, Hollins LD, Eckman J. Empirically validated family interventions for pediatric psychology: sickle cell disease as an exemplar. Journal of Pediatric Psychology 1997;22(2):213‐27. - PubMed
King 2006 {published data only}
    1. King AA, Whit A, Armstrong M, McKinstry R, Noetzel M, DeBaun M. An educational remediation program for children with sickle cell disease and cerebral infarcts is feasible and effective [abstract]. Proceedings of the 29th Annual Meeting of the National Sickle Cell Disease Programme. 2006.
King 2007 {published data only}
    1. King AA, White DA, McKinstry RC, Noetzel M, Debaun MR. A pilot randomized education rehabilitation trial is feasible in sickle cell and strokes. Neurology 2007;68(23):2008‐11. [CENTRAL: 700281; CRS: 5500050000000263; PUBMED: 17548550] - PubMed
Lemanek 2009 {published data only}
    1. Lemanek KL, Ranalli M, Lukens C. A randomized controlled trial of massage therapy in children with sickle cell disease. Journal of Pediatric Psychology 2009;34(10):1091–6. - PubMed
Mendolia 1993 {published data only}
    1. Mendolia SM, Freed N. Sickle Cell Disease ‐ a comprehensive education for the adult patient [abstract]. Proceedings of the 18th Annual Meeting of the National Sickle Cell Disease Programme. 1993.
Oyeku 2005 {published data only}
    1. Oyeku SO, Feldman HA, Ryan K, Muret‐Wagstaff S, Neufeld EJ. Clinician knowledge and confidence about newborn screening for sickle cell disease: randomized assessment of educational strategies [abstract]. Proceedings of the 28th Annual Meeting of the National Sickle Cell Disease Program. 2005. - PMC - PubMed
Oyeku 2005 b {published data only}
    1. Oyeku SO, Feldman HA, Ryan K, Muret‐Wagstaff S, Neufeld EJ. Primary care provider knowledge and confidence about newborn screening for sickle cell disease [abstract]. Proceedings of the Pediatric Academic Societies Annual Meeting. 2005.
Oyeku 2010 {published data only}
    1. Oyeku SO, Feldman HA, Ryan K, Muret‐Wagstaff S, Neufeld EJ. Primary care clinicians' knowledge and confidence about newborn screening for sickle cell disease: randomized assessment of educational strategies. Journal of the National Medical Association 2010;102(8):676‐82. - PMC - PubMed
Radcliffe 2007 {published data only}
    1. Radcliffe J, Barakat L, Simon K, Schwartz LA, Ohene‐Frempong K. Attitude counts: self‐efficacy, negative thinking and health care costs in adolescent sickle cell disease within the teens taking control study [abstract]. Proceedings of the 35th Anniversary Convention of the National Sickle Cell Disease Program and the Sickle Cell Disease Association of America; 2007 Sep 17‐22; Washington DC, USA . 2007:93, Abstract No: 79. [CRS: 5500125000000683]
Robinson 1990 {published data only}
    1. Robinson GA, Devane J. Impact of home health care on sickle cell disease patients [abstract]. Proceedings of the National Sickle Cell Disease Program Annual Meeting Conference. 1990:113.
Schatz 2004 {published data only}
    1. Schatz J, Robinson J, Puffer E. Promoting school success following childhood stroke: standard school advocacy versus a neuropsychologically‐informed team planning approach [abstract]. Proceedings of the 27th Annual Meeting of the National Sickle Cell Disease Program. 2004:180.
Thomas 2013 {published data only}
    1. Thomas LS, Stephenson N, Swanson M, Jesse DE, Brown S. A pilot study: the effect of healing touch on anxiety, stress, pain, pain medication usage, and physiological measures in hospitalized sickle cell disease adults experiencing a vaso‐occlusive pain episode. Journal of Holistic Nursing 2013;31(4):234‐47. [CENTRAL: 1068103; CRS: 5500133000000034; JID:: 8506709; PUBMED: 23817144] - PubMed
Wilkie 2013 {published data only}
    1. Wilkie DJ, Gallo AM, Yao Y, Molokie RE, Stahl C, Hershberger PE, et al. Reproductive Health CHOICES for young adults with sickle cell disease or trait: randomized controlled trial immediate post test effects. Nursing Research 2013;62(5):352‐61. - PMC - PubMed
Yang 2000 {published data only}
    1. Yang YM, Andrews S, Peterson R, Shah A, Cepeda M. Prenatal sickle cell screening education effect on the follow‐up rates of infants with sickle cell trait. Patient Education and Counselling 2000;39(2‐3):185‐9. - PubMed
Yerys 2003 {published data only}
    1. Yerys BE, White DA, Salorio CF, McKinstry R, Moinuddin A, DeBaun M. Memory strategy training in children with cerebral infarcts related to sickle cell disease. Journal of Pediatic Hematology/Oncology 2003;25(6):495‐8. - PubMed

References to studies awaiting assessment

Bhogte 2013 {published data only}
    1. Bhogte C, Nickel R, Hsu L, Meier E. Pilot intervention to improve sickle cell disease transition with a DVD and website shows education of adolescents should occur in the outpatient clinic [abstract]. Pediatric Blood & Cancer 2013;60:S27‐8. [CENTRAL: 1027515; CRS: 5500050000000264; EMBASE: 71047861]

Additional references

Abidin 1995
    1. Abidin RR. Parenting Stress Index (PSI). Lutz, Florida, USA: Psychological Assessment Resources Inc, 1995.
Altman 1996
    1. Altman DG, Bland JM. Statistics Notes: Detecting skewness from summary information. BMJ 1996;313(7066):1200. - PMC - PubMed
Anie 2005
    1. Anie KA. Psychological complications in sickle cell disease. British Journal of Haematology 2005;129(6):723–9. - PubMed
Anie 2015
    1. Anie KA, Green J. Psychological therapies for sickle cell disease and pain. Cochrane Database of Systematic Reviews 2015, Issue 5. [DOI: 10.1002/14651858.CD001916.pub3] - DOI - PMC - PubMed
Armstrong 1993
    1. Armstrong FD, Lemanek KL. Sickle Cell Knowledge Questionnaire. University of Miami School of Medicine 1993.
Ataga 2000
    1. Ataga KI, Orringer EP. Renal abnormalities in sickle cell disease. American Journal of Hematology 2000;63(4):205–11. - PubMed
Barlow 2010
    1. Barlow J, Smailagic N, Ferriter M, Bennett C, Jones H. Group‐based parent‐training programmes for improving emotional and behavioural adjustment in children from birth to three years old. Cochrane Database of Systematic Reviews 2010, Issue 3. [DOI: 10.1002/14651858.CD003680.pub2] - DOI - PMC - PubMed
Begg 1996
    1. Begg C, Cho M, Eastwood S, et al. Improving the quality of reporting of randomized controlled trials: The consort statement. JAMA 1996;276(8):637‐9. - PubMed
Bhatt 2011
    1. Bhatt K, Reid ME, Lewis NA, Asnani MR. Knowledge and health beliefs of Jamaican adolescents with sickle cell disease. Pediatric Blood & Cancer 2011;57(6):1044‐8. - PubMed
Bodenheimer 2002
    1. Bodenheimer T, Wagner EH, Grumbach K. Improving primary care for patients with chronic illness: the chronic care model, Part 2. JAMA 2002;288(15):1909‐14. - PubMed
Brousse 2012
    1. Brousse V, Elie C, Benkerrou M, Odièvre MH, Lesprit E, Bernaudin F, et al. Acute splenic sequestration crisis in sickle cell disease: cohort study of 190 paediatric patients. British Journal of Haematology 2012;156(5):643‐8. - PubMed
Brown 1993
    1. Brown RT, Armstrong FD, Eckman JR. Neurocognitive aspects of pediatric sickle cell disease. Journal of Learning Disabilities 1993;26(1):33–45. - PubMed
Burlew 1989
    1. Burlew AK, Evans R, Oler C. The impact of a child with sickle cell disease on family dynamics. Annals of the New York Academy of Sciences 1989;565:161‐71. - PubMed
Deeks 2011
    1. Deeks J, Higgins J, Altman D. Chapter 9 Analysing data and undertaking meta‐analysis. In: Higgins JPT, Green S editor(s). Cochrane Handbook for Systematic Reviews of Interventions. Version 5.1.0. Available from www.cochrane‐handbook.org. Wiley, 2011.
Delis 1988
    1. Delis DC, Freeland J, Kramer JH, Kaplan E. Integrating clinical assessment with cognitive neuroscience: construct validation of the California Verbal Learning Test. Journal of Consulting and Clinical Psychology 1988;56(1):123‐30. [PUBMED: 3346437] - PubMed
Di Sabatino 2011
    1. Sabatino A, Carsetti R, Corazza GR. Post‐splenectomy and hyposplenic states. Lancet 2011;378(9785):86–97. - PubMed
Ebert 2010
    1. Ebert EC, Nagar M, Hagspiel KD. Gastrointestinal and hepatic complications of sickle cell disease. Clinical Gastroenterology and Hepatology 2010;8(6):483–9. - PubMed
Guyatt 2011
    1. Guyatt GH, Oxman AD, Schunemann HJ, Tugwell P, Knotterus A. GRADE guidelines: a new series of articles in the Journal of Clinical Epidemiology. Journal of Clinical Epidemiology 2011;64(4):380‐2. - PubMed
Higgins 2003
    1. Higgins JP, Thompson SG, Deeks JJ, Altman DG. Measuring inconsistency in meta‐analyses. BMJ (Clinical research ed.) 2003;327(7414):557‐60. [PUBMED: 12958120] - PMC - PubMed
Higgins 2011a
    1. Higgins JPT, Altman DG (editors). Chapter 8: Assessing risk of bias in included studies. In: Higgins JPT, Green S (editors). Cochrane Handbook for Systematic Reviews of Interventions Version 5.1.0 [updated March 2011]. The Cochrane Collaboration, 2011. Available from www.cochrane‐handbook.org.
Higgins 2011b
    1. Higgins JPT, Green S (editors). Cochrane Handbook for Systematic Reviews of Interventions Version 5.1.0 [updated March 2011]. The Cochrane Collaboration, 2011. Available from www.cochrane‐handbook.org 2011.
Hill 2008
    1. Hill C, Maskowitz K, Danis B, Wakschlag L. Validation of a clinically sensitive, observational coding system for parental behaviors: The Parenting Clinical Observation Schedule. Parenting: Science and Practice 2008;8(2):153‐85. - PMC - PubMed
Hurtig 1994
    1. Hurtig AL. Relationships in families of children and adolescents with sickle cell disease. Journal of Health & Social Policy 1994;5(3‐4):161‐83. - PubMed
Imdad 2011
    1. Imdad A, Yakoob MY, Bhutta ZA. Impact of maternal education about complementary feeding and provision of complementary foods on child growth in developing countries. BMC Public Health 2011;11 Suppl 3:S25. - PMC - PubMed
Ingram 1959
    1. Ingram VM. Abnormal human haemoglobins. III. The chemical difference between normal and sickle cell haemoglobins. Biochimica et Biophysica Acta 1959;36:402–11. - PubMed
Kral 2001
    1. Kral MC, Brown RT, Hynd GW. Neuropsychological aspects of pediatric sickle cell disease. Neuropsychology Review 2001;11(4):179–96. - PubMed
Landgraf 1998
    1. Landgraf JM, Maunsell E, Speechley KN, Bullinger M, Campbell S, Abetz L, et al. Canadian‐French, German and UK versions of the Child Health Questionnaire: methodology and preliminary item scaling results. Quality of Life Research: An International Journal of Quality of Life Aspects of Treatment, Care and Rehabilitation 1998;7(5):433‐45. [PUBMED: 9691723] - PubMed
Leikin 1989
    1. Leikin SL, Gallagher D, Kinney TR, Sloane D, Klug P, Rida W. Mortality in children and adolescents with sickle cell disease. Cooperative Study of Sickle Cell Disease. Pediatrics 1989;84(3):500‐9. - PubMed
Maxwell 1999
    1. Maxwell K, Streetly A, Bevan D. Experiences of hospital care and treatment‐seeking behavior for pain from sickle cell disease: qualitative study. Western Journal of Medicine 1999;171(5‐6):306‐13. - PMC - PubMed
Miller 2011
    1. Miller ST. How I treat acute chest syndrome in children with sickle cell disease. Blood 2011;117(20):5297‐305. - PubMed
Modell 2008
    1. Modell B, Darlison M. Global epidemiology of haemoglobin disorders and derived service indicators. Bulletin of the World Health Organization 2008;86(6):480–7. - PMC - PubMed
NICE 2007
    1. National Institute for Clinical Excellence (NICE), Social Care Institute for Excellence (SCIE). Parent‐training/education programmes in the management of children with conduct disorders. NICE Technology Appraisal Guidance 102. www.nice.org.uk/TA102 (accessed 13 Feb 2014).
Ohene‐Frempong 1998
    1. Ohene‐Frempong K, Weiner SJ, Sleeper LA, Miller ST, Embury S, Moohr JW, et al. Cerebrovascular accidents in sickle cell disease: Rates and risk factors. Blood 1998;91(1):288–94. - PubMed
Pauling 1964
    1. Pauling L. Molecular disease and evolution. Bulletin of the New York Academy of Medicine 1964;40(5):334–42. - PMC - PubMed
Pham 2000
    1. Pham PT, Pham PC, Wilkinson AH, Lew SQ. Renal abnormalities in sickle cell disease. Kidney International 2000;57(1):1–8. - PubMed
Quinn 2004
    1. Quinn CT, Rogers ZR, Buchanan GR. Survival of children with sickle cell disease. Blood 2004;103(11):4023‐7. - PMC - PubMed
Rees 2010
    1. Rees DC, Williams TN, Gladwin MT. Sickle‐cell disease. Lancet 2010;376(9757):2018–31. - PubMed
Robin 1990
    1. Robin AL, Koepke T, Moye A. Multidimensional assessment of parent‐adolescent relations. Psychological Assessment 1990;2(4):451‐9.
Sawyer 2005
    1. Sawyer SM, Aroni RA. Self‐management in adolescents with chronic illness. What does it mean and how can it be achieved?. Medical Journal of Australia 2005;183(8):405‐9. - PubMed
Schünemann 2008
    1. Schünemann HJ, Oxman AD, Higgins JPT, Vist GE, Glasziou P, Guyatt GH. Chapter 11: Presenting results and ‘Summary of findings’ tables. In: Higgins JPT, Green S editor(s). Cochrane Handbook for Systematic Reviews of Interventions. Chichester: John Wiley & Sons, Ltd, 2008:335‐57.
Schünemann 2008a
    1. Schünemann HJ, Oxman AD, Vist GE, Higgins JPT, Deeks JJ, Glasziou P, et al. Chapter 12: Interpreting results and drawing conclusions. In: Higgins JPT, Green S editor(s). Cochrane Handbook for Systematic Reviews of Interventions. Chichester: John Wiley & Sons, Ltd, 2008:359‐87.
Steen 2003
    1. Steen RG, Miles MA, Helton KJ, Strawn S, Wang W, Xiong X, et al. Cognitive impairment in children with hemoglobin SS sickle cell disease: relationship to MR imaging findings and hematocrit. American Journal of Neuroradiology 2003;24(3):382–9. - PMC - PubMed
Streisand 2001
    1. Streisand R, Braniecki S, Tercyak KP, Kazak AE. Childhood illness‐related parenting stress: the pediatric inventory for parents. Journal of Pediatric Psychology 2001;26(3):155‐62. [PUBMED: 11259517] - PubMed
Stuart 2004
    1. Stuart MJ, Nagel RL. Sickle‐cell disease. Lancet 2004;364(9442):1343–60. - PubMed
Tanabe 2010
    1. Tanabe P, Porter J, Creary M, Kirkwood E, Miller S, Ahmed‐Williams E, et al. A qualitative analysis of best self‐management practices: sickle cell disease. Journal of the National Medical Association 2010;102(11):1033‐41. - PubMed
Tyrer 2005
    1. Tyrer P, Nur U, Crawford M, Karlsen S, McLean C, Rao B, et al. The Social Functioning Questionnaire: a rapid and robust measure of perceived functioning. International Journal of Social Psychiatry 2005;51(3):265‐75. [PUBMED: 16252794] - PubMed
Weatherall 2001
    1. Weatherall DJ, Clegg JB. Inherited haemoglobin disorders: an increasing global health problem [Inherited haemoglobin disorders: an increasing global health problem]. Bulletin of the World Health Organization 2001;79(8):704‐12. [S0042‐96862001000800005 [pii]] - PMC - PubMed
Welch 2012
    1. Welch V, Petticrew M, Tugwell P, Moher D, O'Neill J, Waters E, et al. PRISMA‐Equity 2012 extension: reporting guidelines for systematic reviews with a focus on health equity. PLoS Medicine 2012;9(10):e1001333. - PMC - PubMed

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