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How I treat the older adult with sickle cell disease

Swee Lay Thein and Jo Howard

Article Figures & Data

Figures

  • Figure 1.

    Complications commonly encountered in the older adult with SCD. One study showed that by the fifth decade, almost 50% of surviving SCD patients had documented damage in the organs above.11 In this study, of the 8 organ systems, sickle chronic lung disease was most common, followed by renal failure, retinopathy, osteonecrosis, priapism, gallbladder disease, leg ulcers, and cerebrovascular disease. Frequency of these complications increase with age, but it is also clear that some complications (such as bilirubin levels and gallstones, and sickle nephropathy) are influenced by predisposing or protective genetic variants. The 8 organ system and some of the complications are represented here. (A-D) Sickle retinopathy. Fundus photographs of characteristic retinal lesions observed in sickle cell retinopathy. (A) Arteriolar occlusion with retinal infarcts. (B) Black “sunburst.” (C) Autoinfarcted “sea fan.” (D) Neovascularization in peripheral retina. (E) Cerebrovascular system. Cerebral hemorrhage (left) and atrophy of right cerebral hemisphere (right). (F) Sickle hepatopathy. Light microscopy (silver stain) showing cirrhotic nodules in liver (left) and gallbladder with gallstones (right). (G) Pelvis, avascular necrosis of hip joints. Right hip replacement (left) and early avascular necrosis of left hip (right). (H) Long bones. Infarction of femoral-tibia joint (left) and imaging of osteomyelitis in lower femur (right). (I) Cardiovascular disease. Histopathology showing left ventricular hypertrophy (LVH) of the heart (left) and light microscopy (hematoxylin-and-eosin stain) showing fibrous obliteration of pulmonary vessel with recanalization (right). (J) Sickle nephropathy. Light microscopy (hematoxylin-and-eosin stain) demonstrating large glomerulus and with mesangial hypercellularity (left) and large glomerulus showing sickled red blood cells within the capillaries of the glomerulus (right). (K) Leg ulceration. Chronic intractable ulceration of lower leg above ankle joint.

  • Figure 2.

    Management of acute complications in older adults with SCD. In general, the management of older adults presenting with acute complications of SCD will not differ significantly from that in younger patients. Note, however, that older adults are more likely to have chronic renal, cardiorespiratory, and/or liver impairment, and may require appropriate dose adjustments of certain medications. Areas where due consideration should be given in older adults include those shown.110-113

  • Figure 3.

    Management strategies for the older adult with SCD. The overarching principle of care for adults with SCD is regular comprehensive review, sometimes known as the “annual review,” using the approach as outlined. During the review, aggregate supportive therapies hydroxycarbamide plus or minus EPO, blood transfusion, and iron chelation therapy, should be discussed and offered if appropriate. The comprehensive review is also an opportunity to provide patient information on new therapies and research studies such as stem cell gene therapy and transplantation.

Tables

  • Table 1.

    Commonly recognized sickle-related complications in adults

    ComplicationDefinitionMajor risk factors and prevalence
    PainAcute pain, most commonly in the long bones, chest, backEpisodes of acute pain commence from around 6 mo of age and continues throughout life
    Adults with SCD experience pain on >54% of days but only access health care on 3.5% of days.36
    Chronic pain is pain lasting for >3 moEstimated to occur in >50% adults with SCD; 40% of adults with SCD take daily opioids.99
    AnemiaAcute anemia: a decline in hemoglobin of 2 g/dL or more from steady-state valuesVariety of causes including infection (transient red cell aplasia most commonly caused by acute parvo virus B19); acute hemolysis accompanying severe VOC, delayed transfusion reaction
    Chronic hemolytic anemia: severity increases with age and major contributor to insidious organ dysfunctionChronic hemolysis predisposes to gallstones and gallbladder disease
    ACSAcute onset of respiratory symptoms with features similar to pneumonia24.5/100 PYO in young children; 8.8/100 PYO in older adults101; outcome more severe in adults
    Pulmonary hypertensionMean pulmonary artery pressure of >25 mm Hg at rest, measured by right heart catheterization6.0%-10.4% prevalence during adulthood.41-43,46,47
    Cardiac dysfunctionLeft ventricular failure is most common abnormalityUniversal to some degree in adults over age 30.44 Diffuse myocardial fibrosis is a common pathology, and associated with diastolic dysfunction, anemia, and high NT-proBNP. Fibrosis-mediated diasystolic dysfunction may contribute to elevated TRV in older adults via pulmonary venous hypertension.45
    Chronic sickle lung diseaseProgressive restrictive lung function deficit with fibrotic changes on high resolution CT scanRestrictive lung defects seen in >70% adults.38
    AsthmaAsthma is seen more commonly in children than in adults
    Sleep-disordered breathingSleep disordered breathing is seen in 40%-60% of adults.19
    StrokeAcute cerebrovascular accidentEffective screening and prevention have dramatically reduced infarctive stroke in children. Hemorrhagic stroke affects both children and adults, threefold more in adults.102 Risk factors for ischemic stroke include hypertension, diabetes mellitus, hyperlipidemia, atrial fibrillation, and renal disease in adults.103
    Silent cerebral infarctsClinically silent lesions of 3 mm or more on magnetic resonance imaging scanningImportant contributing factor to neurocognitive deficits; 53% in adults.20
    Acute renal injuryAcute deterioration in renal function2% during pain crisis and up to 14% during ACS
    Renal failureDeteriorating renal function, reduced concentrating ability, proteinuria, and progressive renal failureAdvanced disease (stage III-IV) in 4%-18% of adults.21
    PriapismUnwanted painful and sustained erection of the penis for >4 h, often recurrent or persistent20%-89% lifetime prevalence in boys and men.104
    Avascular necrosis of bonesAvascular necrosis of any bone, most commonly the femoral head and shoulder joint>20% lifetime prevalence of symptomatic disease. Increased prevalence of asymptomatic disease.105
    Leg ulcerationMost commonly around the malleolar regions>14% lifetime prevalence.106,107
    CholelithiasisGallstones and gallbladder diseaseImportant genetic modifier is polymorphic (AT) repeats in promoter of UGT1A1 gene.100 Gallbladder disease in 28% at median age of 28 y.11
    RetinopathyGrade 2-4 retinopathy>30% of patients.108 Increased in HbSC.109
    • ACS, acute chest syndrome; AT, adenine-thymidine; proBNP, N-terminal pro b-type natriuretic peptide; PYO, person-years of observation, VOC, vaso-occlusive crisis.