What IVF (In Vitro Fertilization) protocol is recommended for patients with Sickle Cell Disease (SCD)?

IVF Protocol Recommendations for Patients with Sickle Cell Disease

For patients with sickle cell disease (SCD), elective single embryo transfer (eSET) is strongly recommended to minimize maternal and fetal risks associated with multiple pregnancies. 1

Fertility Considerations in SCD

Patients with SCD face unique reproductive challenges that must be addressed when planning IVF treatment:

• Fertility risk factors specific to SCD patients:
  • Chronic inflammation
  • Oxidative stress
  • Transfusion-related hemochromatosis
  • Ovarian sickling causing ischemia and reperfusion injury 2
  • Potential impact of disease-modifying therapies (hydroxyurea)

IVF Protocol Components for SCD Patients

1. Pre-IVF Assessment and Preparation

• Extended red cell antigen profiling before starting IVF treatment to facilitate any necessary transfusions during the process 1
• Comprehensive SCD management optimization prior to starting IVF to minimize disease-related complications
• Hydroxyurea considerations: Discontinuation is recommended when planning pregnancy due to potential teratogenicity 2

2. Stimulation Protocol

• Antagonist controlled ovarian hyperstimulation protocol with individualized gonadotropin dosing is recommended 3
• Trigger medication options:
  • Leuprolide acetate
  • Human chorionic gonadotropin 3

3. Fluid Management During IVF

• Careful intravenous fluid administration is critical as inappropriate fluid management can lead to:
  • Volume overload
  • New oxygen requirements
  • Acute chest syndrome
  • Acute kidney injury 4
• Avoid normal saline when possible as it may be associated with adverse outcomes 4

4. Embryo Transfer Strategy

• Elective single embryo transfer (eSET) is strongly recommended regardless of:
  • Previous unsuccessful ART treatments
  • Duration of infertility
  • Previous pregnancies or live births
  • Embryo quality 1

5. Genetic Testing Considerations

• Preimplantation genetic testing (PGT) should be offered to:
  • Identify embryos unaffected by SCD
  • Potentially identify embryos that could be future matched sibling donors for stem cell transplant 5, 6

Special Considerations and Risks

Potential Complications During IVF

Patients with SCD undergoing IVF are at risk for serious SCD-related complications:

• Respiratory failure
• Painful vaso-occlusive crises
• Post-harvest painful crises 3

Risk Mitigation Strategies

• Disease-modifying therapy prior to IVF (except hydroxyurea if planning immediate transfer)
• Prophylactic red cell antigen matching (Rh and K antigens) for any necessary transfusions 1
• Close monitoring throughout the stimulation and retrieval process
• Immediate access to SCD expertise during the IVF cycle

Multidisciplinary Approach

IVF for SCD patients should involve:

• Reproductive endocrinologist with SCD experience
• Hematologist with SCD expertise
• High-risk obstetrician
• Transfusion medicine specialist if transfusions are needed 1

Follow-up and Pregnancy Management

If pregnancy is achieved:

• Co-management by a hematologist with SCD expertise and a high-risk obstetrician is essential
• Pregnancy may increase frequency of SCD complications, but most pregnancies have successful outcomes 1

By following these specific protocols and precautions, patients with SCD can undergo IVF with appropriate risk management to maximize chances of a successful outcome while minimizing SCD-related complications.