Sickle Cell Disease: Cardiopulmonary and Kidney Disease

Publication Date: December 3, 2019

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

Publication Month/Year

December 3, 2019

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.