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Sickle Cell Disease: Outpatient Management and Complications. Hilary Suzawa Baylor Med/Peds June 16, 2008. Sickle Cell Disease. First described 1910 Ethnic backgrounds: Black (African) Mediterranean Arabian peninsula Indian subcontinent Caribbean South and Central America.

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sickle cell disease outpatient management and complications

Sickle Cell Disease: Outpatient Management and Complications

Hilary Suzawa

Baylor Med/Peds

June 16, 2008

sickle cell disease
Sickle Cell Disease
  • First described 1910
  • Ethnic backgrounds:
    • Black (African)
    • Mediterranean
    • Arabian peninsula
    • Indian subcontinent
    • Caribbean
    • South and Central America
hemoglobin s
Hemoglobin S
  • Substitution of a valine for glutamic acid at the sixth amino acid of the beta globin chain
  • Produces a hemoglobin tetramer (alpha2/beta S2) that is poorly soluble when deoxygenated
variants
Variants
  • Hemoglobin SS (SCD-SS)
  • Hemoglobin SC (SCD-SC)
  • Sickle Beta Thalassemia
    • SCD-SB+thal or SCD-SB0 thal
  • In US ~2/3 of SCD are SCD-SS
  • SCD-SC or Sickle Beta Thalassemia may NOT have significant anemia
average lifespan
Average Lifespan
  • Sickle Cell Disease (SCD-SS)—
    • 45 years
    • One generation ago average survival was as low as 14 years
  • SCD-SC or Sickle Beta Thalassemia—
    • 50-60 years
prenatal or preconception
Prenatal or Preconception
  • Amniocentesis if needed
  • Genetics evaluation
  • High-risk ob if needed
  • Maternal mortality <2% and neonatal mortality <5%
  • Increased risk of pre-eclampsia, pre-term labor, low-birth weight
birth 6 months
Birth-6 months
  • Visit Q2 months (2 wk, 2 mth, 4 mth, 6 mth)
  • CBC every visit
  • Heptavalent conjugated pneumococcal vaccine (Prevnar) at 2 mth, 4 mth, 6 mth
  • PCN VK 125 mg po BID starting at 2-3 mths
6 months 2 years
6 months-2 years
  • Visit Q3 months (6 mth, 9 mth, 12 mth, 15 mth, 18 mth, 21 mth, 2 yrs)
  • CBC every 3-6 mths
  • UA annually
  • Ferritin or serum iron and TIBC once at 1-2 yrs
  • BUN, Cr, LFTS once at age 1-2 yrs
  • Prevnar at 15 mths
  • Flu vaccine
  • Start folic acid daily at 1 year
  • Consultation with pedi heme
2 years
2 years
  • Screening with specialized Doppler US transcranial blood-flow velocity (TBV)
  • Recommended for asymptomatic children with sickle cell dz beginning at age 2 yrs
  • Recommended annually from 2-16 yrs age
  • TBV 200 cm/sec or more are at increased risk of stroke
2 years 5 years
2 years-5 years
  • Visit Q6 months
  • CBC at least yearly
  • UA at least yearly
  • BUN, Cr and LFTs every 1-2 yrs
  • 23-valent Pneumococcal vaccine (Pneumovax) at age 2 yrs; booster at age 5 yrs
  • Meningococcal vaccine after age 2 yrs
  • Flu vaccine annually
  • Increase PCN VK to 250 mg po BID at 3 yrs old
5 years
>5 years
  • Visits Q6-12 months
  • CBC at least yearly
  • UA at least yearly
  • BUN, Cr and LFTs every 2-3 years
  • Flu vaccine yearly
  • Consider stop PCN VK
    • NOT if pt has had splenectomy OR h/o invasive pneumococcal infxn
  • Continue folic acid
  • Annual eye exam starting at 10 yrs (focus on retina)
growth
Growth
  • Decrease in height and weight compared to peers
    • During adolescent growth spurt begin to catch up with peers
    • Do NOT experience growth failure, only growth delay
  • Delayed puberty
    • Menarche delayed 2-2.5 years
    • Dysmenorrhea may precipitate pain episodes
    • Puberty, although delayed, proceeds normally once started
adolescence
Adolescence
  • Visits annually
  • CBC yearly
  • UA yearly
  • BUN, Cr and LFTs every 2-3 years
  • Ferritin or serum iron and TIBC at least once
  • Flu vaccine yearly
  • Transition from pediatric to adult care
adult
Adult
  • Biannual visit for pt >30 years
  • Annual eye exam
  • Vaccinations
    • Hepatitis vaccine
    • Pneumococcal vaccine (Pneumovax)
  • Evaluate for elevated BP and pulmonary HTN
  • Foot care and protective shoes
  • Consider PCN VK prophylaxis
  • Folic acid daily to ensure adequate amounts for high erythrocyte turnover
  • Consider hydroxyurea
pneumovax
Pneumovax
  • First dose at 2 years
  • If pt <10 years, then give booster at age 3-5 years
  • If pt >10 years, then give booster 5 years after first dose
    • Support for a one time booster 5 years after first dose; some institutions give Q5 years
blood pressure
Blood Pressure
  • Pt with SCD-SS have lower systolic and diastolic blood pressure compared to others
  • Modest increases in BP within the normal range for general population may indicate underlying renal dz
pulmonary hypertension
Pulmonary Hypertension
  • Monitor for pulmonary hypertension
    • Unexplained dyspnea or hypoxemia
    • Loud P2
    • Enlarged heart (RV) on CXR
    • Check Echo
pcn vk prophylaxis
PCN VK prophylaxis
  • By age 2 months, start PCN VK 125 mg po BID
  • Age 3 years increase dose to PCN VK 250 mg po BID
  • Functional asplenia occurs <1 year age in pt with SCD-SS and SCD SB0thal
  • SCD-SS patients have usually infarcted their spleens before 4 years
  • Can be discontinued after age 5 years except in child who has had splenectomy or invasive infxn
hydroxyurea
Hydroxyurea
  • Increases Hb F levels
  • Improves red cell deformability and hydration
  • Reduces red cell adhesion to vascular endothelium
blood transfusion
Blood Transfusion
  • Post-transfusion, Hb level should not exceed 10-11 g/dL
  • Check for infection: HIV, Hepatitis panel
  • Alloimmunization—in up to 30% of pt who receive frequent transfusions
  • Volume overload
  • Iron overload
    • Use of chelator
    • Exchange transfusion
clinical case 1
Clinical Case #1
  • 2 yo AAM with h/o SCD-SS presents for sick visit.
  • On physical exam pt has palpable spleen not previously observed.
  • Pt’s baseline Hb ~8 but current Hb ~3
  • Reticulocyte count increased from pt’s baseline
clinical case 122
Clinical Case #1
  • What medical condition do you suspect?
    • Acute Splenic Sequestration Crisis: sudden enlargement of spleen, precipitous fall in Hct (at least 2 g/DL), rise in reticulocyte count.
  • What is most common age for this to develop?
    • Often occurs between 6 months and 3 years
    • Hb SS at age <3 years. Variants at any time during childhood.
clinical case 123
Clinical Case #1
  • What treatment do you recommend?
    • Hospitalization
    • IVF +- blood transfusion
    • After first or second crisis, consider elective splenectomy when child is stable
    • Preventive education: Caretaker should know how to palpate for splenic size and know about risk of sequestration crisis
clinical case 2
Clinical Case #2
  • 17 yo AAF with h/o RAD and SCD-SS was recently hospitalized for a vaso-occlusive pain crisis. Presents 1 week after discharge for hospital follow-up.
  • Now c/o minimal bilateral LE pain, chest discomfort, fever (T=100)
  • Pulse ox in the office Sat 88% on RA
  • Chest X-ray: Normal
clinical case 225
Clinical Case #2
  • What medical condition do you suspect?
    • Acute Chest Syndrome
    • Ages birth-9 years
    • May be accompanied by or preceded by pain the chest or extremities, fever, respiratory distress and low O2 Sat
    • Hypoxemia is not necessary for dx
    • CXR may be normal initially with later development of infiltrate
    • 30% of all Hb SS pt will have one episode of Acute Chest Syndrome; ½ of these pt will have recurrent episodes
    • Asthma increases risk of ACS
clinical case 3
Clinical Case #3
  • 5 yo AAM with SCD-SS disease presents with a new limp
  • What medical conditions would you consider?
  • What other information would you like to know?
ddx limp in hb ss disease
DDX Limp in Hb SS Disease
  • DDX
    • Osteomyelitis
    • Avascular Necrosis of the hip
    • Stroke
  • Other information:
    • H/o HA
    • Fever
    • Labs: WBC, ESR
    • Extremity exam, hip exam
    • Neuro exam
slide28
AVN
  • Most common site of AVN
    • Femoral head
    • Humeral head
    • Knee
    • Small joints of hands and feet
  • Most common orthopedic complication in SCD and occurs in up to 50% of all pt
  • SCD is the most common cause of AVN in children
stroke
Stroke
  • Stroke occurs in 7-11% of children with SCD
  • SCD-SS overall risk of stroke 3.75% throughout lifetime
  • <2 yrs have lowest incidence of stroke
  • 2-5 years have highest incidence infarctive stroke
  • 20-29 years have highest incidence hemorrhagic stroke
questions
Questions
  • What is the most common cause of osteomyelitis in pt with SCD-SS?
    • Salmonella #1, S. aureus #2
  • How much does Hb usually drop with aplastic crisis?
    • >= 30% reduction from baseline Hb
  • What is the most common cause of aplastic crisis?
    • Parvovirus B19 causes ~70%
bacterial infection
Bacterial infection
  • What are most common bacteria causing infxn in SCD-SS?
    • Streptococcus pneumoniae
    • Staphylococcus aureus
    • Salmonella species
    • Haemophilus influenza type b
    • Escherichia coli
    • Klebsiella species
complications
Complications
  • Airway/Breathing
    • Acute chest syndrome
    • Chronic Pulmonary Hypertension
  • CNS
    • Stroke
    • Pain
  • Cardiovascular
    • Hypertension
gi complications
GI Complications
  • GI
    • Cholelithiasis, Choledocholithiasis, Acute Cholecystitis
    • Abdominal Pain Crisis
    • Sickle Hepatopathy
    • Pancreatitis
    • Peptic Ulcer Disease
  • Hematologic
    • Acute splenic sequestration crisis
    • Aplastic crisis
    • Blood transfusion complications
  • GU/Gyn
    • Priapism
    • Pregnancy Complications, need for Genetic Counseling
complications34
Complications
  • Infection
    • Osteomyelitis
  • Renal Disease
  • Orthopedic
    • Aseptic Necrosis of the hip (AVN)
    • Chronic Leg Ulcers
  • Multiple Organ Failure Syndrome:
    • fever, decrease Hb, decrease plt, rhabdomyolysis, mental status changes
bibliography
Bibliography
  • Koshy, M and Dorn, L. “Continuing Care for Adult Patients with Sickle Cell Disease.” Hematology/Oncology Clinics of North America 1996; 10 (6): 1265-1273.
  • Lottenberg, R and Hassell, K. “An Evidence-Based Approach to the Treatment of Adults with Sickle Cell Disease.” Hematology 2005; 58-65.
  • Mehta, S et al. “Opportunities to Improve Outcomes in Sickle Cell Disease.” American Family Physician 2006; 74 (2): 303-310.
bibliography36
Bibliography
  • Redding-Lallinger, R and Knoll, C. “Sickle Cell Disease—Pathophysiology and Treatment.” Curr Probl Pediatr Adolesc Health Care 2006; 36: 346-376.
  • Wethers, D. “Sickle Cell Disease in Childhood: Part I. Laboratory Diagnosis, Pathophysiology and Health Maintenance.” American Family Physician 2000; 62 (5): 1013-1020.
bibliography37
Bibliography
  • Wethers, D. “Sickle Cell Disease in Childhood: Part II. Diagnosis and Treatment of Major Complications and Recent Advances in Treatment.” American Family Physician 2000; 62 (6): 1309-1314.