Sickle cell disease (SCD) is progressive and continues to affect patients throughout their lives1,2

People live with the consequences of SCD throughout their lives. Explore the physical and psychosocial impact at each stage below.

The physical effects of SCD in childhood

The devastating organ damage characteristic of SCD begins early in life.3 Patients may experience a range of clinical manifestations, some of which can be life-threatening.1,3

Stroke

Stroke risk in children with SCD is 300 times greater than in children without SCD or other pathology associated with increased stroke risk (e.g. heart disease)1
  • The risk of stroke is highest during the first decade of life, being most significant at age 2–5 years1
SCD, sickle cell disease.
1. Verduzco LA, Nathan DG. Blood. 2009;114:5117–5125.

Silent Cerebral Infarct and Neurocognitive Impairment

Anaemia is associated with silent cerebral infarct,1 which is present in a quarter of patients before 6 years of age, with the proportion rising to a third of children age 14 years2
  • Neurocognitive impairments often occur in children with silent cerebral infarct2
1. Ataga KI, et al. PLoS One. 2020;15(4):e0229959; 2. DeBaun MR, et al. Blood. 2012;119:4587–4596.

Retinal Artery Occlusion/Retinopathy

Retinal artery occlusion/retinopathy may emerge in early childhood1
1. Kanter J, Kruse-Jarres R. Blood Rev. 2013;27(6):279–287.

Acute Chest Syndrome

Acute chest syndrome is among the most common causes of death after the age of 2 years1
  • ~1% of children with SCD died from acute chest syndrome in one analysis; approximately two-thirds of whom were aged 3 years or under2
SCD, sickle cell disease.
1. Serjeant GR. Cold Spring Harb Perspect Med. 2013;3:a011783; 2. Vichinsky EP, et al. Blood. 1997;89:1787–1792.

Splenic Injury

Hyposplenism increases susceptibility to infection1
  • Hyposplenism is present in most children before age 12 months, and in general splenic injury is silently progressive, although acute and life-threatening splenic sequestration of RBCs can occur in infants1
RBC, red blood cell.
1. Brousse V, et al. Br J Haematol. 2014;166:165–176.

Glomerular Damage

Glomerular damage occurs as early as the first decade of life in SCD and is characterised by high renal flow, glomerular hyperfiltration and hypertrophy1
SCD, sickle cell disease.
1. McPherson Yee M, et al. Clin J Am Soc Nephrol. 2011;6:2628–2633.

Psychosocial manifestations of SCD in childhood

SCD in childhood is characterised by disruption to both life and learning. The psychosocial impact can affect school, home and social life.4–6

SCD and school

Children often struggle to build friendships and succeed academically because of:

  • Frequent school absence1–3
  • Hospitalisations1
  • Impaired intellectual functioning (a potential consequence of SCI4) and social functioning1–3
Adapted from Osunkwo I, et al. 2021.5
  • The impact of SCD on education in childhood can follow through to adult life, affecting employment prospects and lifetime earnings5,6
SCD, sickle cell disease; SCI, silent cerebral infarction.
1. Jenerette CM, Brewer C. J Natl Med Assoc. 2010;102:1050–1055; 2. Bhagat VM, et al. Indian J Palliat Care. 2014;20:107–111; 3. Salih KMA. Family Med Prim Care. 2019;8:468–471; 4. Houwing ME, et al. BMC Med. 2020;18:393; 5. Osunkwo I, et al. Am J Hematol. 2021;96:404–417; 6. Lubeck D, et al. JAMA Network Open. 2019;2:e1915374.

Quality of life

Children with SCD have comparable quality of life to children on cancer treatment.1

Adapted from Kato GJ, et al. 2018.1
  • In addition, looking after children with SCD and worrying about their future also has a significant impact on caregivers’ quality of life2–4
HRQoL, health-related quality of life.
1. Kato GJ, et al. Nat Rev Dis Primers. 2018;4:18010; 2. Caprini FR, Motta AB. Estudos de Psicologia (Campinas). 2021;38:e190168; 3. Karadağ G, et al. J Caring Sci. 2018;7:125–129; 4. Madani BM, et al. Health Qual Life Outcomes. 2018;16:176.
Abbreviations: SCD, sickle cell disease.
References: 1. Kanter J, Kruse-Jarres R. Blood Rev. 2013;27(6):279–287; 2. Cançado RD. Rev Bras Hematol Hemoter. 2012;34:175–187; 3. Meier ER, Miller JL. Drugs. 2012;72(7):895–906; 4. Jenerette CM, Brewer C. J Natl Med Assoc. 2010;102:1050–1055; 5. Bhagat VM, et al. Indian J Palliat Care. 2014;20:107–111; 6. Salih KMA. Family Med Prim Care. 2019;8:468–471.

People live with the consequences of SCD throughout their lives. Explore the physical and psychosocial impact at each stage below.

The physical effects of SCD in adolescence

In addition to facing progressive worsening of disease symptoms with age and progressive multiorgan damage,1 adolescents with SCD may experience low self-esteem, embarrassment and other psychosocial issues.3 This happens at a time when they must contend with the challenges that face all adolescents: forming an identity and concerns about self-image.4

Stroke

Stroke occurs before age 20 years in approximately 11% of patients with SCD1
SCD, sickle cell disease.
1. Verduzco LA, Nathan DG. Blood. 2009;114:5117–5125.

Debilitating Acute Pain Crises

Debilitating acute pain crises are a hallmark of the disease and may continue into adulthood1
1. Kanter J, Kruse-Jarres R. Blood Rev. 2013;27(6):279–287.

Cardiac Disease

Signs of cardiac disease (including LVH and cardiomegaly) may begin to emerge in childhood/adolescence1,2
LVH, left ventricular hypertrophy.
1. Kanter J, Kruse-Jarres R. Blood Rev. 2013;27(6):279–287; 2. Loar RW, et al. Echocardiography. 2021;38(2):189–196.

Chronic Kidney Disease

Approximately 15% of adolescents with SCD have stage 1 CKD, with 12% having stage 2 CKD1
CKD, chronic kidney disease; SCD, sickle cell disease.
1. McPherson Yee M, et al. Clin J Am Soc Nephrol. 2011;6:2628–2633.

Priapism

Priapism is a common and distressing complication of SCD in males that may result in permanent vascular damage and impotence1
SCD, sickle cell disease.
1. Serjeant GR. Cold Spring Harb Perspect Med. 2013;3:a011783.

Avascular Necrosis

Avascular necrosis may limit mobility, potentially requiring prosthetic surgery1
1. Serjeant GR. Cold Spring Harb Perspect Med. 2013;3:a011783.

Leg Ulceration

Leg ulcers – a major problem in adolescence – are characterised by a chronic healing/relapsing course1
1. Serjeant GR. Cold Spring Harb Perspect Med. 2013;3:a011783.

Delayed Menarche

Menarche is typically delayed by an average of 2.5 years in females1
1. Serjeant GR. Cold Spring Harb Perspect Med. 2013;3:a011783.

Psychosocial manifestations of SCD in adolescence

The teenage years are a defining moment in any individual’s life, but for young people with SCD there are several additional challenges.5

Uncertainty and loss of control

  • Adolescents may fear disease complications such as stroke and downstream consequences, affecting sight and cognition1
  • They face the possibility of death and must confront this fear2
  • Adolescents may therefore feel a sense of loss of control2
1. Kulandaivelu Y, et al. JMIR Pediatr Parent. 2018;1:e11058; 2. Pinckney RB, Stuart GW. JCAPN. 2004;17:5–12.

Peer relationships

  • Adolescents may miss out on activities with friends because of disease symptoms and medical appointments, affecting their ability to create and maintain friendships1
  • They may feel that they are different from peers, and experience bullying because they seem different1
  • Some find it difficult to talk to friends or teachers about their disease, instead internalising their feelings1
1. Kulandaivelu Y, et al. JMIR Pediatr Parent. 2018;1:e11058.

Discrimination

  • Discrimination stems from a lack of understanding about SCD1
  • Children/adolescents with SCD may experience discrimination from peers and teachers, which contributes to anxiety and distress1,2
1. Jenerette CM, Brewer C. J Natl Med Assoc. 2010;102:1050–1055; 2. Dyson SM, et al. Soc Sci Med. 2010;70(12):2036–2044.

Delayed growth

  • Patients may experience issues relating to self-image as a result of delayed growth1,2
Adapted from Rhodes M, et al. 2009.1
1. Rhodes M, et al. Pediatr Blood Cancer. 2009;53:635–641; 2. Jenerette CM, Brewer C. J Natl Med Assoc. 2010;102:1050–1055.

Bone mineral density

  • Patients may experience issues relating to self-image as a result of bone age retardation1,2
Adapted from Rhodes M, et al. 2009.1
1. Rhodes M, et al. Pediatr Blood Cancer. 2009;53:635–641; 2. Jenerette CM, Brewer C. J Natl Med Assoc. 2010;102:1050–1055.

Sexual maturation

  • Patients may experience issues relating to self-image as a result of delayed sexual maturation1,2
Adapted from Rhodes M, et al. 2009.1
1. Rhodes M, et al. Pediatr Blood Cancer. 2009;53:635–641; 2. Jenerette CM, Brewer C. J Natl Med Assoc. 2010;102:1050–1055.

Transitioning to adult healthcare

  • Without adequate transfer preparation, patients with SCD may experience challenges with their care as complications continue to emerge1,2
  • Poor transition from paediatric to adult care can lead to recurrent hospitalisation, poor trust in the healthcare system and worse overall outcomes1
  • Transition to adult healthcare should begin early, involve both paediatric and adult healthcare teams, and take place over a period of several years3
1. Jenerette CM, Brewer C. J Natl Med Assoc. 2010;102:1050–1055; 2. Howard J, Thein SL. Hematology Am Soc Hematol Educ Program. 2019;2019:505–512; 3. de Montelembert M, Guitton C. Br J Haematol. 2014;164:630–635.
Abbreviations: SCD, sickle cell disease.
References: 1. Kanter J, Kruse-Jarres R. Blood Rev. 2013;27(6):279–287; 2. Cançado RD. Rev Bras Hematol Hemoter. 2012;34:175–187; 3. Jenerette CM, Brewer C. J Natl Med Assoc. 2010;102(11):1050–1055; 4. Asnani MR, et al. Glob Pediatr Health. 2017;4:2333794X17739194; 5. Pinckney RB, Stuart GW. JCAPN. 2004;17:5–12.

People live with the consequences of SCD throughout their lives. Explore the physical and psychosocial impact at each stage below.

The physical effects of SCD in adults

Years of progressive organ damage compromise both the quality and quantity of life.3–10 Mortality remains stubbornly high in people with SCD and life expectancy is reduced by approximately 22 years vs the general population (54 years vs 76 years).*9

STROKE

Stroke occurs in 24% of patients by age 45 years1
1. Verduzco LA, Nathan DG. Blood. 2009;114:5117–5125.

NEUROCOGNITIVE IMPAIRMENT

Neurocognitive impairment may manifest as impaired processing speed and executive functioning1
1. Martin S, et al. Neuropsychol Rehabil. 2020;30:1666–1681.

RETINOPATHY AND BLINDNESS

Retinopathy, affecting up to 40% of patients, can result in permanent vision loss1
1. Menaa F, et al. J Multidiscip Healthc. 2017;30;10:335–346.

CARDIAC FAILURE

Cardiac failure, possibly secondary to pulmonary hypertension and/or reduced compliance of the left ventricular wall, becomes more common with age1
1. Serjeant GR. Cold Spring Harb Perspect Med. 2013;3:a011783.

PULMONARY HYPERTENSION

Cumulative organ damage results in lung injury, such as pulmonary hypertension1

Pulmonary hypertension affects ~10% of adults with SCD2
1. Serjeant GR. Cold Spring Harb Perspect Med. 2013;3:a011783; 2. Gordeuk VR, et al. Blood. 2016;127(7):820–828.

KIDNEY FAILURE

Early kidney failure may occur and is associated with a poor prognosis (4.2% of patients with SCD in one study, median age 23.1 years, survival time – despite dialysis – being 4 years)1
SCD, sickle cell disease.
1. Powars DR, et al. Ann Intern Med. 1991;115(8):614–620.

Psychosocial manifestations of SCD in adults

SCD has a huge impact on everyday life.1 The chronic, debilitating fatigue resulting from SCD is correlated with poorer health-related quality of life (HRQoL);2 these features can be further exacerbated by depression and anxiety.3,4

Cognitive impairment may be mistaken for maladaptive behaviour or non-compliance, which can trigger negative responses from others – such as care providers who are unaware of SCD.3,5 Patients seeking care may be mislabelled as malingerers, manipulators or even drug-seekers.3

Not all features of SCD are visible. The impact of SCD on everyday life may be hidden from healthcare professionals.2–6

Fatigue

  • Fatigue correlates with reduced HRQoL for patients with SCD1
Adapted from Ameringer S, et al. 2014.1
BFI, Brief Fatigue Inventory; HRQoL, health-related quality of life; SF-36, 36-item short form.
1. Ameringer S, et al. J Pediatr Oncol Nurs. 2014;31:6–17.

HRQoL

Adults with SCD have reduced HRQoL vs adults with other chronic health conditions*

  • HRQoL was comparable or reduced in SCD vs other chronic states, and substantially reduced compared with healthy adults1
*Data taken from Kato GJ, et al. 2018.1
HRQoL, health-related quality of life; SCD, sickle cell disease
1. Kato GJ, et al. Nature Rev. 2018;4:18010.

Depression

  • Depression is associated with reduced health-related quality of life1
Adapted from Levenson JL, et al. 2008.1
SF-36, 36-item short form.
1. Levenson JL, et al. Psychosomat Med. 2008;70:192–196.

Loss of income

  • Frequent pain episodes, hospitalisations and loss of income/employment may lead to low self-esteem and feelings of hopelessness1
Adapted from Lubeck D, et al. 2019.1
SCD, sickle cell disease.
1. Lubeck D, et al. JAMA Network Open. 2019;2:e1915374.

Impact on everyday life

  • SCD has profound impacts on many elements of everyday life1
Adapted from Osunkwo I, et al. 2021.1
1. Osunkwo I, et al. Am J Hematol. 2021;96:404–417.
*US data.9 Life expectancy in the general population and in people with SCD will differ between countries.
Abbreviations: SCD, sickle cell disease.
References: 1. Kanter J, Kruse-Jarres R. Blood Rev. 2013;27(6):279–287; 2. Cançado RD. Rev Bras Hematol Hemoter. 2012;34:175–187; 3. Verduzco LA, Nathan DG. Blood. 2009;114:5117–5125; 4. Martin S, et al. Neuropsychol Rehabil. 2020;30:1666–1681; 5. Powars DR, et al. Ann Intern Med. 1991;115(8):614–620; 6. Serjeant GR. Cold Spring Harb Perspect Med. 2013;3:a011783; 7. Porter J, Garbowski M. Hematology Am Soc Hematol Educ Program. 2013;2013(1):447–456; 8. Coates TD, Wood JC. Br J Haematol. 2017;177(5):703–716; 9. Lubeck D, et al. JAMA Netw Open. 2019;2(11):e1915374; 10. Menaa F, et al. J Multidiscip Healthc. 2017;30;10:335–346; 11. Osunkwo I, et al. Am J Hematol. 2021;96:404–417; 12. Ameringer S, et al. J Pediatr Oncol Nurs. 2014;31:6–17; 13. Jenerette CM, Brewer C. J Natl Med Assoc. 2010;102:1050–1055; 14. Levenson JL, et al. Psychosomat Med. 2008;70:192–196; 15. Crawford R, Jonassaint CR. J Adv Nurs. 2016;72:1409–1416; 16. Haywood C, et al. J Gen Intern Med. 2014;29:1657–1662.