This is the current release of the guideline.

The American College of Obstetricians and Gynecologists (ACOG) reaffirmed the currency of this guideline in 2011.

Hemoglobinopathies in pregnancy, including:

• Sickle cell disease
• Thalassemia (alpha and beta)

Screening/Risk Assessment

1. Risk assessment (e.g., ethnicity)
2. Laboratory testing (complete blood count, hemoglobin levels, hemoglobin electrophoresis, solubility testing for hemoglobin S, isoelectric focusing, high-performance liquid chromatography, mean corpuscular volume, serum ferritin levels)
3. Genetic testing (parents, fetus via chorionic villus sampling or amniocentesis, preimplantation)

Management

1. Genetic counseling
2. Folic acid supplementation
3. Cesarean delivery (not recommended routinely)
4. Epidural analgesia
5. Avoidance of triggers of painful crisis
6. Analgesia for painful crisis (e.g., opiates)
7. Detection and treatment of acute chest syndrome, infection, dehydration, severe anemia, cholecystitis, and hypersplenism
8. Blood transfusion, including prophylactic exchange transfusion
9. Multidisciplinary management
10. Antenatal fetal surveillance (serial ultrasound, nonstress test, contraction stress test)

2007 Guideline

The MEDLINE database, the Cochrane Library, and the American College of Obstetricians and Gynecologists' own internal resources and documents were used to conduct a literature search to locate relevant articles published between January 1985 and March 2005. The search was restricted to articles published in the English language. Priority was given to articles reporting results of original research, although review articles and commentaries also were consulted. Abstracts of research presented at symposia and scientific conferences were not considered adequate for inclusion in this document. Guidelines published by organizations or institutions such as the National Institutes of Health and the American College of Obstetricians and Gynecologists were reviewed, and additional studies were located by reviewing bibliographies of identified articles.

2011 Reaffirmation

Medline/Pubmed/Cochrane databases were searched for literature published from 2007-2011.

Not stated

Studies were reviewed and evaluated for quality according to the method outlined by the U.S. Preventive Services Task Force:

I Evidence obtained from at least one properly designed randomized controlled trial.

II-1 Evidence obtained from well-designed controlled trials without randomization.

II-2 Evidence obtained from well-designed cohort or case–control analytic studies, preferably from more than one center or research group.

II-3 Evidence obtained from multiple time series with or without the intervention. Dramatic results in uncontrolled experiments also could be regarded as this type of evidence.

III Opinions of respected authorities, based on clinical experience, descriptive studies, or reports of expert committees.

Not stated

2007 Guideline

Analysis of available evidence was given priority in formulating recommendations. When reliable research was not available, expert opinions from obstetrician–gynecologists were used. See also the "Rating Scheme for the Strength of Recommendations" field regarding Grade C recommendations.

2011 Reaffirmation

A committee member reviewed the document and new literature search on the topic. The document was then reviewed by the committee and the committee agreed that it is current and accurate.

Based on the highest level of evidence found in the data, recommendations are provided and graded according to the following categories:

Level A — Recommendations are based on good and consistent scientific evidence.

Level B — Recommendations are based on limited or inconsistent scientific evidence.

Level C — Recommendations are based primarily on consensus and expert opinion.

A formal cost analysis was not performed and published cost analyses were not reviewed.

Practice Bulletins are validated by two internal clinical review panels composed of practicing obstetrician-gynecologists generalists and sub-specialists. The final guidelines are also reviewed and approved by the American College of Obstetricians and Gynecologists (ACOG) Executive Board.

The grades of evidence (I-III) and levels of recommendation (A-C) are defined at the end of the "Major Recommendations" field.

The following recommendations are based on good and consistent scientific evidence (Level A):

• Individuals of African, Southeast Asian, and Mediterranean descent are at increased risk for being carriers of hemoglobinopathies and should be offered carrier screening and, if both parents are determined to be carriers, genetic counseling.
• A complete blood count and hemoglobin electrophoresis are appropriate laboratory tests for screening for hemoglobinopathies. Solubility tests alone are inadequate for screening because they fail to identify important transmissible hemoglobin gene abnormalities affecting fetal outcome.
• Couples at risk for having a child with sickle cell disease or thalassemia should be offered genetic counseling to review prenatal testing and reproduction options. Prenatal diagnosis of hemoglobinopathies is best accomplished by DNA analysis of cultured amniocytes or chorionic villi.

Definitions:

Grades of Evidence

I Evidence obtained from at least one properly designed randomized controlled trial.

II-1 Evidence obtained from well-designed controlled trials without randomization.

II-2 Evidence obtained from well-designed cohort or case–control analytic studies, preferably from more than one center or research group.

II-3 Evidence obtained from multiple time series with or without the intervention. Dramatic results in uncontrolled experiments also could be regarded as this type of evidence.

III Opinions of respected authorities, based on clinical experience, descriptive studies, or reports of expert committees.

Levels of Recommendation

Level A — Recommendations are based on good and consistent scientific evidence.

Level B — Recommendations are based on limited or inconsistent scientific evidence.

Level C — Recommendations are based primarily on consensus and expert opinion.

The original guideline document contains a clinical algorithm for "Specialized antepartum evaluation for hematologic assessment of patients of African, Southeast Asian, or Mediterranean descent."

The type of supporting evidence is identified and graded for each recommendation (see "Major Recommendations").

Appropriate screening and management of hemoglobinopathies during pregnancy

Epidural analgesia usually is well tolerated as long as care is taken to avoid hypotension and hypoxemia.

Hydroxyurea has been shown to reduce the frequency of painful crises in nonpregnant patients with severe sickle cell disease. However, the use of hydroxyurea is not recommended during pregnancy because it is teratogenic.

These guidelines should not be construed as dictating an exclusive course of treatment or procedure. Variations in practice may be warranted based on the needs of the individual patient, resources, and limitations unique to the institution or type of practice.

An implementation strategy was not provided.

Not applicable: The guideline was not adapted from another source.

American College of Obstetricians and Gynecologists (ACOG)

American College of Obstetricians and Gynecologists (ACOG) Committee on Practice Bulletins-Obstetrics

Not stated

Not stated

This is the current release of the guideline.

The American College of Obstetricians and Gynecologists (ACOG) reaffirmed the currency of this guideline in 2011.

Electronic copies: None available

Print copies: Available for purchase from the American College of Obstetricians and Gynecologists (ACOG) Distribution Center, PO Box 933104, Atlanta, GA 31193-3104; telephone, 800-762-2264, ext. 192; e-mail: sales@acog.org. The ACOG Bookstore is available online at the ACOG Web site .

None available

None available

This NGC summary was completed by ECRI Institute on October 8, 2007. The information was verified by the guideline developer on December 3, 2007. The currency of the guideline was reaffirmed by the developer in 2011 and this summary was updated by ECRI Institute on November 15, 2012.

This NGC summary is based on the original guideline, which is subject to the guideline developer's copyright restrictions.