Delivering Integrated Care and Cost Management
The IHTC works collaboratively with payors to optimize care. We ensure that the patients and families we serve have access to care and therapies, thereby helping to contain costs and reduce both bleeding events and utilization of resources.
The IHTC Pharmacy’s ability to purchase clotting factor through the Public Health Service 340B discount program and our overall pricing structure benefit payors and patients by dispensing clotting factor at significantly reduced prices.
Pregnancy carries some increased risk for a woman with sickle cell disease as well as her fetus, but should not be a contraindication for desired childbearing. Prenatal care by a practitioner with expertise in high-risk pregnancy is desirable. Ideally, the monitoring should be every 2 weeks until the 36th week of pregnancy, then weekly thereafter.
Pregnant patients should receive folic acid 1mg daily. This may be given in addition to other prenatal vitamins, minerals, and iron supplements (unless the patient has increased iron stores from multiple prior transfusions).
All patients should have blood screening for presence of alloantibodies (regardless of transfusion history). Management of the alloimmunized mother should include Rh/antibody titers, administering Rh immunoglobulin if indicated, and performing amniocentesis to assess fetal development and bilirubin concentration.
Fetal growth should be monitored by ultrasound. If intrauterine growth failure is detected, bed rest is recommended and early delivery considered if growth does not accelerate or if urinary estriol decreases. Stress tests should be used if there are questions of uteroplacental adequacy.
Prophylactic red blood cell transfusions may be used to keep the Hb S% ≤ 50 in an attempt to decrease sickling events during pregnancy, however, this should be an individualized decision.
If painful vaso-occlusive episodes occur during pregnancy, they should be treated as usual. Opioids can be used in conventional doses. Use of ibuprofen as adjunctive analgesia is generally discouraged (especially in the third trimester).
Other complications such as acute chest syndrome, stroke, or septicemia should be managed as they are for nonpregnant patients. Preexisting renal disease and congestive heart failure are likely to worsen during pregnancy. Toxemia, thrombophlebitis, pyelonephritis, and spontaneous abortions are more common in women with sickle cell disease. Treatments for these conditions remain the same as for any other pregnant patient.
If delivery is uncomplicated, local or regional anesthesia cause no problems. If general anesthesia is necessary for cesarean section, pre-op simple transfusion and adequate IV hydration are recommended if time and condition permits. It is desirable to have the % hemoglobin S <50 for any general anesthetic.
Standard postpartum care should be implemented with extra attention to maintaining adequate hydration and preventing pulmonary atelectasis. Fevers should be fully evaluated and treated aggressively.
In the state of Indiana, all newborns are screened for hemoglobinopathies as well as other genetic disorders. The results are sent to the primary care provider and the Indiana State Department of Health.
Contraception and Pregnancy Termination:
Sickle cell disease is not a contraindication for use of oral contraceptives. Nor is it an indication for sterilization. Low-estrogen oral contraceptives are safe and effective for most women with sickle cell disease. IUD’s are not generally recommended due to the increased risk for bleeding and infection. Condoms, of course, are recommended as barrier protection against sexually transmittable diseases.
If termination of pregnancy is considered at fewer than 13 weeks, local analgesia and curettage suction is recommended. Termination of the pregnancy at fewer that 7 weeks can be associated with increased risk of incomplete removal fetal tissue leading to bleeding and infection. Beyond 13 weeks, hypertonic urea solutions are injected into the uterus and contractions are stimulated with prostaglandin F2. Hypertonic sodium chloride should not be injected, as it can induce sickling. Rh-negative women should receive Rh immunoglobulin after spontaneous or therapeutic abortion.