Guideline:
Hemoglobinopathies in pregnancy
Bibliographic Source(s)
- American College of Obstetricians and Gynecologists (ACOG). Hemoglobinopathies in pregnancy. Washington (DC): American College of Obstetricians and Gynecologists (ACOG); 2007 Jan. 9 p. (ACOG practice bulletin; no. 78). [26 references]
Guideline Status
This is the current release of the guideline.
Guideline Category
Counseling
Management
Risk Assessment
Screening
Intended Users
Physicians
Guideline Objective(s)
- To aid practitioners in making decisions about appropriate obstetric and gynecologic care
- To provide recommendations for screening and clinical management of hemoglobinopathies during pregnancy
Target Population
- Pregnant women with hemoglobinopathy including sickle cell disease and thalassemia
- Couples at risk for having a child with hemoglobinopathies especially individuals of African Southeast Asian and Mediterranean descent
Interventions and Practices Considered
Screening/Risk Assessment
- Risk assessment (e.g. ethnicity)
- 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)
- Genetic testing (parents fetus via chorionic villus sampling or amniocentesis preimplantation)
Management
- Genetic counseling
- Folic acid supplementation
- Cesarean delivery (not recommended routinely)
- Epidural analgesia
- Avoidance of triggers of painful crisis
- Analgesia for painful crisis (e.g. opiates)
- Detection and treatment of acute chest syndrome infection dehydration severe anemia cholecystitis and hypersplenism
- Blood transfusion including prophylactic exchange transfusion
- Multidisciplinary management
- Antenatal fetal surveillance (serial ultrasound nonstress test contraction stress test)
Major Outcomes Considered
- Completion of pregnancy
- Maternal and fetal perinatal morbidity and mortality
Methods Used to Collect/Select Evidence
Hand-searches of Published Literature (Primary Sources)
Hand-searches of Published Literature (Secondary Sources)
Searches of Electronic Databases
Description of Methods used to Collect/Select the Evidence
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.
Number of Source Documents
Not stated
Methods Used to Assess the Quality and Strength of the Evidence
Weighting According to a Rating Scheme (Scheme Given)
Rating Scheme for the Strength of the Evidence
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.
Methods Used to Analyze the Evidence
Systematic Review
Description of the Methods Used to Analyze the Evidence
Not stated
Methods Used to Formulate the Recommendations
Expert Consensus
Description of Methods Used to Formulate the Recommendations
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.
Rating Scheme for the Strength of the Recommendations
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.
Cost Analysis
A formal cost analysis was not performed and published cost analyses were not reviewed.
Method of Guideline Validation
Internal Peer Review
Description of Method of Guideline Validation
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.
Major Recommendations
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.
Clinical Algorithm(s)
The original guideline document contains a clinical algorithm for "Specialized antepartum evaluation for hematologic assessment of patients of African Southeast Asian or Mediterranean descent."
Type of Evidence supporting the Recommendations
The type of supporting evidence is identified and graded for each recommendation (see "Major Recommendations").
Potential Benefits
Appropriate screening and management of hemoglobinopathies during pregnancy
Potential Harms
Epidural analgesia usually is well tolerated as long as care is taken to avoid hypotension and hypoxemia.
Contraindications
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.
Qualifying Statements
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.
Description of Implementation Strategy
An implementation strategy was not provided.
Implementation Tools
Clinical Algorithm
For information about availability see the "Availability of Companion Documents" and "Patient Resources" fields below.
IOM Care Need
Living with Illness
Staying Healthy
IOM Domain
Effectiveness
Patient-centeredness
Bibliographic Source(s)
- American College of Obstetricians and Gynecologists (ACOG). Hemoglobinopathies in pregnancy. Washington (DC): American College of Obstetricians and Gynecologists (ACOG); 2007 Jan. 9 p. (ACOG practice bulletin; no. 78). [26 references]
Adaptation
Not applicable: The guideline was not adapted from another source.
Source(s) of Funding
American College of Obstetricians and Gynecologists (ACOG)
Guideline Committee
American College of Obstetricians and Gynecologists (ACOG) Committee on Practice Bulletins-Obstetrics
Composition of Group that Authored the Guideline
Not stated
Financial Disclosures/Conflicts of Interest
Not stated
Guideline Status
This is the current release of the guideline.
Guideline Availability
Electronic copies: None available
Print copies: Available for purchase from the American College of Obstetricians and Gynecologists (ACOG) Distribution Center PO Box 4500 Kearneysville WV 25430-4500; telephone 800-762-2264 ext. 192; e-mail: sales@acog.org. The ACOG Bookstore is available online at the ACOG Web site.
Availability of Companion Documents
None available
Patient Resources
None available
NGC STATUS
This NGC summary was completed by ECRI Institute on October 8 2007. The information was verified by the guideline developer on December 3 2007.
COPYRIGHT STATEMENT
This NGC summary is based on the original guideline which is subject to the guideline developer's copyright restrictions.
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