Sickle Cell Disease: Cardiopulmonary and Kidney Disease
Publication Date: December 3, 2019
Last Updated: March 14, 2022
Recommendations
Screening echocardiography
In asymptomatic children and adults with SCD, the ASH guideline panel suggests against performing a routine screening echocardiogram (ECHO) to identify PH. (2⊕ooo)
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Management of abnormal echocardiography
For asymptomatic children and adults with SCD and an isolated peak TRJV of ≥2.5 to 2.9 m/s, the ASH guideline panel suggests against right-heart catheterization. (2⊕ooo)
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For children and adults with SCD and a peak TRJV of ≥2.5 m/s who also have a reduced 6MWD and/or elevated NT-BNP, the ASH guideline panel suggests right-heart catheterization. (2⊕ooo)
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Treatment of PAH
For children and adults with SCD who do not have PAH confirmed by right-heart catheterization, the ASH guideline panel recommends against the use of PAH-specific therapies. (1⊕⊕oo)
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For children and adults with SCD and a diagnosis of PAH confirmed by right-heart catheterization, the ASH guideline panel suggests the use of PAH-specific therapies under the care of a PH specialist given the lack of alternative treatment options and associated high morbidity and mortality. (2⊕⊕oo)
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Screening pulmonary function testing
For asymptomatic children and adults with SCD, the ASH guideline panel suggests against performing routine screening pulmonary function testing (PFT). (2⊕ooo)
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Screening for sleep-disordered breathing
For asymptomatic children and adults with SCD, the ASH guideline panel suggests against screening with formal polysomnography (sleep study) for sleep-disordered breathing. (2⊕ooo)
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Management of albuminuria
For children and adults with SCD and albuminuria, the ASH guideline panel suggests the use of angiotensin-converting enzyme inhibitors (ACEi’s) or angiotensin II receptor blockers (ARBs). (2⊕ooo)
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Renal transplant for end-stage renal disease
For children and adults with SCD and advanced chronic kidney disease or end-stage renal disease, the ASH guideline panel suggests referral for renal transplant. (2⊕ooo)
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Use of hydroxyurea and erythropoiesis-stimulating agents for chronic kidney disease
In children and adults with SCD and worsening anemia associated with chronic kidney disease, the ASH guideline panel suggests combination therapy with hydroxyurea and erythropoiesis-stimulating agents. (2⊕ooo)
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Management of blood pressure
For adults with SCD, the ASH guideline panel recommends a blood pressure goal of ≤130/80 mm Hg over a goal of ≤140/90 mm Hg. (1⊕⊕⊕o)
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Management of VTE
For adults with SCD and first unprovoked VTE, the ASH guideline panel suggests indefinite anticoagulation over shorter, defined periods of anticoagulation. (2⊕⊕oo)
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For adults with SCD and first, surgically, or nonsurgically provoked VTE, the ASH guideline panel suggests defined periods of anticoagulation (3-6 months) over indefinite anticoagulation. (2⊕⊕oo)
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In adults with SCD and recurrent provoked VTE, the ASH guideline panel suggests indefinite anticoagulation over shorter, defined periods of anticoagulation. (2⊕⊕oo)
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Recommendation Grading
Disclaimer
Overview
Title
Sickle Cell Disease: Cardiopulmonary and Kidney Disease
Authoring Organization
American Society of Hematology
Publication Month/Year
December 3, 2019
Last Updated Month/Year
July 28, 2023
Supplemental Implementation Tools
Document Type
Guideline
External Publication Status
Published
Country of Publication
US
Document Objectives
These evidence-based guidelines of the American Society of Hematology (ASH) are intended to support patients, clinicians, and other health care professionals in their decisions about screening, diagnosis, and management of cardiopulmonary and renal complications of SCD.
Target Patient Population
Adults and children with sickle cell disease
Inclusion Criteria
Female, Male, Adolescent, Adult, Child, Older adult
Health Care Settings
Ambulatory, Hospice, Hospital, Outpatient
Intended Users
Nurse, nurse practitioner, physician, physician assistant
Scope
Diagnosis, Management, Treatment
Diseases/Conditions (MeSH)
D000755 - Anemia, Sickle Cell, D000925 - Anticoagulants, D013923 - Thromboembolism
Keywords
thromboembolism, anticoagulation, sickle cell disease, Anticoagulation
Source Citation
Blood Adv (2019) 3 (23): 3867–3897.