Sickle Cell Disease Is a Multiorgan Disorder

Sickle cell disease (SCD) is a chronic vascular disease, with a range of acute and chronic complications driven by ongoing vaso-occlusion. The diverse clinical manifestations of SCD can affect organs throughout the body. The self-perpetuating cycle of vaso-occlusion and vaso-occlusive crises (VOCs) may account for the majority of the burden of SCD.1-3

 

Central nervous system complications with sickle cell disease

Among patients with SCD, chronic and acute complications can include:

  • Acute Pain is the number one cause of hospital admissions4
  • Cerebrovascular accident
    •  Transient ischemic attack occurs in ~4%5
    •  Overt stroke occurs in ~16%5
    •  Silent cerebral infarction occurs in ~20% of children6
    •  Screening for vascular disease and stroke starting at age 2 years and continuing until at least age 16 years7
  • Retinopathy occurs in ~23%5
  • Seizures occur in ~12%5

Musculoskeletal complications associated with sickle cell disease

Among patients with SCD, chronic and acute complications can include:

  • Bone pain crisis (~30%) is the most common complication of vaso-occlusive pain, and can lead to bone or bone marrow infarction and osteonecrosis8,9
  • Avascular necrosis: 21%10

Cardiopulminary complications associated with sickle cell disease

Among patients with SCD, chronic and acute complications can include:

  • Acute chest syndrome (ACS) occurs in ~75%5
  • Pulmonary hypertension occurs in ~20%6
  • Left-sided heart disease occurs in ~13%6

 

 

 

Reticuloendothelial complications associated with sickle cell disease

Among patients with SCD, chronic and acute complications can include:

  • Splenic sequestration, which occurs in ~50% of patients who survive their first episode and can worsen anemia9,11

Gastrointestinal and Urogenital complications associated with sickle cell disease

Among patients with SCD, chronic and acute complications can include:

Gastrointestinal

  • Cholelithiasis, acute cholecystitis, biliary sludge, and acute choledocholithiasis are common7
  • Gallstones occur in ~75%7

Urogenital

  • Renal dysfunction begins at an early age, and ~30% experience chronic renal failure6
  • Priapism occurs in ~35% of men12

Sickle Cell Disease Is Associated With Impaired Cognition and Psychological Disorders13

 

 

IQ SCORES

~20% lower IQ scores in patients with sickle cell disease

~20%
lower IQ scores have been reported in patients with SCD (eg, verbal and performance)14*

DEPRESSION

~20% lower IQ scores in patients with sickle cell disease

35%
of patients with SCD are diagnosed with depression15†

ANXIETY

13% lower IQ scores in patients with sickle cell disease

13%
of patients with SCD experience anxiety16‡

*Comparing 64 patients with sickle cell anemia and 64 subjects without SCD (control group).
†A total of 142 patients completed the study.
‡Based on 253 adult patients with SCD.

 

 

Other Cognitive and Psychological Consequences of SCD

Cognitive17,18

  • • Overt and/or silent stroke may affect cognitive function, eg,
  • - executive skills
  • - decreased attention span
  • - memory impairment
  • - communication skills

Psychological18,19

  • • SCD-associated stigma
  • • Feeling of worthlessness
  • • Post-traumatic stress disorder

Increased Hospitalization and Cost of Care

SCD ER Visits Are25:

4 times
higher than for those with congestive heart failure
 
Sickle cell disease ER visits are 13 times higher than for those with HIV
13 times
higher than for those with HIV

 

 

References:

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2. Steinberg MH. Sickle cell disease and associated hemoglobinopathies. In: Goldman L, Ausiello D, eds, Cecil Medicine, 23rd ed. Philadelphia, PA; Saunders Elsevier; 1991:Chap 167. 
3. Piel FB, Steinberg MH, Rees DC. Sickle cell disease. N Engl J Med. 2017;376(16):1561-1573. 
4. Ballas SK, Gupta K, Adams-Graves P. Sickle cell pain: a critical reappraisal. Blood. 2012;120(18):3647-3656. 
5. Elmariah H, Garrett ME, De Castro LM, et al. Factors associated with survival in a contemporary adult sickle cell disease cohort. Am J Hematol. 2014;89(5):530-535. 
6. Rees DC, Williams TN, Gladwin MT. Sickle-cell disease. Lancet. 2010;376(9757):2018-2031. 
7. Yawn BP, John-Sowah J. Management of sickle cell disease: recommendations from the 2014 expert panel report. Am Fam Physician. 2015;92(12):1069-1076. 
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9. National Institutes of Health. National Heart, Lung, and Blood Institute. The Management of Sickle Cell Disease, 4 ed. NIH Publication No. 02-2117. http://purl.access.gpo.gov/GPO/LPS22097. 10. Powars DR, Chan LS, Hiti A, Ramicone E, Johnson C. Outcome of sickle cell anemia: a 4-decade observational study of 1056 patients. Medicine (Baltimore). 2005;84(6):363-376. 
11. Ware RE, de Montalembert M, Tshilolo L, Abboud MR. Sickle cell disease. Lancet. 2017;390(10091):311-323. 
12. Adeyoju AB, Olujohungbe ABK, Morris J. Priapism in sickle-cell disease; incidence, risk factors and complications–an international multicentre study. BJU Int. 2002;90(9):898-902. 
13.Hasan S, Hashmi S, Alhassen M, Lawson W, Castro O. Depression in sickle cell disease. J Natl Med Assoc. 2003;95(5):533-538. 
14. Castro IPS, Viana MB. Cognitive profile of children with sickle cell anemia compared to healthy controls. J Pediatr (Rio J). 2017;1-7. doi.org/10.1016/j.jped.2018.04.012. 
15. Adam SS, Flahiff CM, Kamble S, et al. Depression, quality of life, and medical resource utilization in sickle cell disease. Blood Adv. 2017;1(23):1983-1992. 
16. Anie KA, Egunjobi FE, Akinyanju OO. Psychosocial impact of sickle cell disorder: perspectives from a Nigerian setting. Global Health. 2010;6:2. doi: 10.1186/1744-8603-6-2. 
17. Kassim AA, Galadanci NA, Pruthi S, DeBaun MR. How I treat and manage strokes in sickle cell disease. Blood 2015.125(22):3401-3410. 
18. Chakravorty S, Tallett A, Witwicki C, et al. Patient-reported experience measure in sickle cell disease. Arch Dis Child. 2018;103(12):1104-1109. 
19. Alao AO. Sickle cell disease and posttraumatic stress disorder. Int J Psychiatry Med. 2002;32(1):97-101. 
20. Swanson ME, Grosse SD, Kulkarni R. Disability among individuals with sickle cell disease. Am J Prev Med. 2011;41(6S4):S390-S397. 
21. Brandow AM, Brousseau DC, Panepinto JA. Post-discharge pain, functional limitations, and impact on caregivers of children with sickle cell disease treated for painful events. Br J Haematol. 2009;144(5):782-788. 
22. Côbo Vde A, Chapadeiro CA, Ribeiro JB, Moraes-Souza H, Martins PR. Sexuality and sickle cell anemia. Rev Bras Hematol Hemoter. 2013;35(2):89-93. 
23. Brandow AM, Brousseau DC, Panepinto JA. Post-discharge pain, functional limitations, and impact on caregivers of children with sickle cell disease treated for painful events. Br J Haematol. 2009;144(5):782-788. 
24. Lentz MB, Kautz DD. Acute vaso-occlusive crisis in patients with sickle cell disease. Nursing2018. 2017;41(1):67-68. 
25. Lanzkron S, Carroll CP, Haywood C. The burden of emergency department use for sickle cell disease: an analysis of the national emergency department sample database. Am J Hematol. 2010;85(10):797-799. 
26. American Society of Hematology. State of Sickle Cell Disease: 2016 Report. Washington, DC: 2016. http://www.scdcoalition.org/report.html. 27. Ballas SK, Lusardi M. Hospital readmission for adult acute sickle cell painful episodes: frequency, etiology, and prognostic significance. Am J Hematol. 2005;79(1):17-25. 
28. Smith WR, Pemberthy LT, Bovbjerg VE, et al. Daily assessment of pain in adults with sickle cell disease. Ann Intern Med. 2008;148(2):94-101. 
29. Brousseau DC, Owens PL, Mosso AL, Panepinto JA, Steiner CA. Acute care utilization and rehospitalization for sickle cell disease. JAMA. 2010;303(13):1288-1294. 
30. Robbins JM, Webb DA. Diagnosing diabetes and preventing rehospitalizations: the Urban Diabetes Study. Med Care. 2006;44(3):292-296. 
31. VanSuch M, Naessens JM, Stroebel RJ, Huddleston JM, Williams AR. Effect of discharge instructions on readmission of hospitalized patients with heart failure: do all of the Joint Commission on Accreditation of Healthcare Organizations heart failure core measures reflect better patient care? Qual Saf Health Care. 2006;15(6):414-417. 
32. Jasti H, Mortensen EM, Obrosky DS, Kapoor WN, Fine MJ. Causes and risk factors for rehospitalization of patients hospitalized for community-acquired pneumonia. Clin Infect Dis. 2008;46(4):550-556. 
33. Reznik M, Hailpern SM, Ozuah PO. Predictors of early hospital readmission for asthma among inner-city children. J Asthma. 2006;43(1):37-40. 

 

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