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The Comprehensive Examination

This comprehensive examination by Dr. S. Johnson covers preventive health, psychosocial assessment, sickle cell education, surveillance for complications, and more. It delves into manifestations of sickle cell disease, acute and chronic complications, and management strategies for various health issues.

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The Comprehensive Examination

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  1. The Comprehensive Examination Cage S. Johnson, M.D. Professor Emeritus of Medicine Director, Sickle Cell Center Keck School of Medicine University of Southern California

  2. Adult Health Care • Preventive Health Maintenance: • Psychosocial Assessment: school achievement, occupational Hx, depression, anxiety, financial resources • Education: tobacco, alcohol, recreational drugs, safe sex techniques, sickle cell related education – fever, dyspnea, pain management • Surveillance for complications of SCDz, as well as for disorders of adulthood • Referral network, early treatment of complications

  3. General Internal Medicine • Thyroid • Cholesterol • Diabetes Mellitus • Hypertension

  4. General Internal Medicine • Thyroid • Cholesterol • Diabetes Mellitus • Hypertension

  5. Immunization • Pneumovax • Hib • Hepatitis B • HPV • Hepatitis A (confounder)

  6. Age appropriate cancer screening • PAP smear • Mammography • PSA • Occult blood

  7. Malignancy in SCD, No HU • Incidence of malignancy at Howard Univ. is 1.74 per 1000 pt-yrs Vs. 3.82 per 1000 pt-yrs in the Afro-American population. Am J Hematol 55:188, 1997 • 2 malignancies in 144 autopsies in Hb SS Br J Haematol 123:359, 2003 • “CSSCD reported 16 malgnancies in over 4000 patients during a 15 y follow-up” Cited in Am J Hematol 74:249, 2003

  8. IASCNAPA: ~n= 16,613 Am J Hematol 74:249, 2003 • All: 6 cases, 1 on HU • AML: 2 cases • CML: 1 case • Brain, Breast, Bladder, Bone, Gall bladder, GI tract, Lymphoma – HD & NHL, Myeloma, Pancreatic, Testis

  9. Manifestations of Sickle Cell Disease • Chronic Hemolysis • Intermittent “crises” • Frequent Infections • End organ dysfunction • Disorders unrelated to the Hbopathy

  10. Acute & Chronic Manifestations of Sickle Cell Disease Pain : ischemic, neuropathic, collapsed bone Brain: TIA, ischemic Stroke, hemorrhage Eye: retinopathy Bone: remodeling by marrow hyperplasia, osteonecrosis, collapse, osteomyelitis Lung: ACS, chronic restrictive lung disease (hypoxia) Heart : pulmonary hypertension Liver : gallstones, hepatitis, iron overload, sequestration Spleen : auto-infarction, sequestration, bacterial sepsis GU: infection, proteinuria, renal insufficiency, obstetrical, priapism Skin: leg ulcers

  11. Surveillance:(repeated annually) • H/O sickling related events: Pain-acute & chronic, CVA, PNA/ACS (ICU), Hepatitis, Cholelithiasis, Priapism, Retinopathy, Pregnancy, Leg Ulcer, Hematuria/Infection, Bone infections/AVN, Transfusion • Lab: CBC, Bun/Creatinine, Urine protein, Iron/Ferritin, Hepatitis markers, B12, Folate, RBC phenotype, pulse oximetry, allantibodies, EKG, additional assays based upon Hx/Px • X-ray: Chest, Hips, Shoulders, T/L Spines, etc.

  12. Br Med J 295:234, 1987

  13. Hepatic Diseases • Cholelithiasis • Chronic active hepatitis B/C RUQ ultrasound (biopsy) Viral load & genotype Rx: Interferon & Ribivarin • Iron overload

  14. Pulmonary Disease (CSSCD) • N = 318, > 18 y of age (mean 31  10), 41 % male • 90 % abnormal Restrictive - 74 % Obstructive - 1 % Mixed O/R - 2 %  DLCO - 13 % • Association between lung & kidney disease Am J Resp Crit Care Med epublished 23 Oct 2005

  15. J Clin Epidemiol 45:893, 1992

  16. Prevalence rates of proteinuria and renal insufficiency per decade of life Int J Artif Organs 13:231, 1990

  17. Proteinuria microalbuminuria:> 20 mg/g creatininemacroalbuminuria:> 300 mg/g creatinine Histology: Progressive focal segmental glomerular sclerosis • effacement of foot processes • thickening of basement membrane • Fe+++ deposition Differential diagnosis: • post streptococcal glomerulonephiritis • acute diffuse proliferative glomerulonephritis • acute diffuse mesangial glomerulonephritis • membranoproliferative glomerulonephritis • renal vein thrombosis Treatment: • ACE inhibitor

  18. Hematuria • Papillary necrosis • Glomerulonephritis • Medullary Carcinoma

  19. Renal Insufficiency • Anemia: EPO + HU • Acidosis -  ineffective erythropoiesis - Shohl’s Solution • Blood Pressure

  20. Management of Hypertension • Rx if BP > 130/80 • Assess for renal disease • Rx if BP rises from baseline by 20 mm Hg systolic or 10 mm Hg diastolic • With proteinuria: Rx if BP > 120/75 or with > 300 mg/24 h

  21. Management of Hypertension • ACE inhibitor – hyperkalemia (spurious) • Calcium channel blocker • dose adjustment in hepatic or renal disease • Beta-blocker • asthma, CHF, thrombocytopenia, bradycardia • Diuretic • dose adjustment for hyposthenuria/naturesis

  22. Air Travel • Identify a source of care at the destination • Oxygen if SpO2 < 88% • Non-caffeinated fluids • Walk every hr or more often • VTE prophylaxis with h/o prior VTE

  23. CHALLENGES in MANAGEMENTof the ADULT PATIENT: • Shorten the duration of acute complications • Reduce the frequency of acute complications • Reduce the frequency/severity of chronic complications • Improve survival • Enhance the Quality of Life

  24. Options • Stem Cell Transplantation • Hydroxyurea, L-Glutamine • Crizanlizumab • Transfusion/Chelation • Gene Therapy

  25. Mortality: Hydroxyurea • 40 % reduction (JAMA290:756, 2003) • Unclear (Blood 105:545, 2005)

  26. Leukemia in SCD on HU • ALL, Ph+:10 y F SS, 7 weeks of Rx Arch Dis Child 81:437, 1999; Blood 98:2878, 2001 • AML: 42 y F SS, 6 yrs of RxAnn Intern Med 133:925, 2000 • AML: 2 yrs of Rx Saudi Med J 23:1411, 2002 • APL(m3v): 21 y F SS, 8+ yrs of Rx Br J Haematol 123:368, 2003 • ANLL: 27 y F SO-Arab, 8 yrs of Rx on a background of MDSBlood 94:415a, 1999

  27. Leukemia in SCD, No HU • ALL: 4 cases Clin Pediatr 11:183, 1972Cent Afr J Med 34:158, 1988 Cancer Genet Cytogenet. 21:267, 1986 Arch Pathol Lab Med 123:745, 1999 • AML: 5 cases Ann Intern Med 39:920, 1953 Am J Med 73:268, 1982 N Engl J Med 311:780, 1984 Am J Hematol 21:223, 1986 Indian Pediatr 16:453, 1979 • CML, Ph+: 6 cases (plus 1 from USC) Hum Genetik 30:13, 1975 N Engl J Med 298:72, 1978 Am J Med 85:567, 1988 J Assoc Physicians India 34:821,1986 Am J Hematol 31:302, 1989 Arch Pathol Lab Med 129:423, 2005

  28. Long Term Follow-up of SCD on HU • MSH: no malignancy at 9 yrs (n=156 on HU>1 y) JAMA 289:1645, 2003 • MCG: no malignancy in 236 adults at 15 yrs Blood 195:545, 2005 • HUG-KIDS: no malignancy in 84 children at 2+ yrs Blood 94:1550, 1999 • France: 1 ALL in 101 children at 2+ yrs Arch Dis Child 81:437, 1999 • Belgium: 1 AML (M3v) in 141 children with 598 pt-yrs of F/U Br J Haematol 123:368, 2003

  29. Summary: Goal of reducing morbidity & mortality • More aggressive use of Hydroxyurea: www.ahrq.gov/clinic/tp/hydscdtp.htm • Additional therapies • Regular Surveillance • Prompt referral • Individualize therapy to the patient

  30. Gene Therapy • Clinical Trial in 5 Hb SS & 5  Thal (Paris) • Self-inactivating lentiviral vector • A-T87Q Ann NY Acad Sci 1054:308, 2005 ,

  31. Gene Therapy • BlueBird Bio

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