SCD occurs as a result of mutation resulting in change in single amino acid: - substitution of thymine for adenine in the sixth codon of the beta-chain gene, GAG to GTG, hence, resulting in replacement of glutamic acid with valine in the beta-globin subunit of hemoglobin. In stressful environments or presence of low oxygen condition, red blood cells (RBCs) polymerize into a more elongated rigid and fragile sickled or S shape form prone to hemolysis, endothelial adhesion, and subsequent vaso-occlusion, this causes the vast majority of clinical features, including acute painful crisis, increased haemolysis and chronic anaemia due to reduced red cell life span. 

Diagnosis follows the typical pattern of history taking, physical examination and laboratory investigations which may include genetic testing. History taking to ascertain the symptomatology, prior history of frequent hospital visits and diagnosis especially in pregnancy. Physical examination to determine the facies which may include pallor due to chronic anaemia, craniofacial alterations in SCA patients occur as the result of hyperplasia and compensatory expansion of the bone marrow, resulting in exaggerated growth/protrusion of the midface, maxillary expansion, a predominance of vertical growth, mandibular retrusion, a convex profile, and maxillary protrusion, hepatomegaly, splenomegaly and so on. 

The laboratory techniques for detecting hemoglobinopathies are hemoglobin electrophoresis, high performance liquid chromatography, isoelectric focusing, smartphone-based application (to capture the images of sickled cells) and DNA molecular testing either during or outside pregnancy.

American College of Obstetricians and Gynecologists, recommended that all patients presenting for pre-pregnancy or prenatal care should be offered a complete blood count (CBC) with RBC indices and hemoglobin electrophoresis testing. Those diagnosed with SCT or disease should have their partner undergo hemoglobinopathy evaluation to assess the risk of SCD or other hemoglobinopathies to their offspring.

Best conducted by a feto-maternal specialist with interest in Sickle Cell Disease in pregnancy Assess the woman’s overall health and her potential risks during pregnancy. Pre-pregnancy counseling allows a patient with SCD the opportunity to make an informed decision regarding pregnancy after consideration of the maternal and fetal risks and the potential risks to the offspring. Reproductive planning options that reduce fetal risks include preimplantation genetics diagnosis and using donor egg or sperm (screened for hemoglobinopathies) and their associated risks. Surrogacy and adoption are other viable options if it is acceptable to them. She and her partner should be counseled accordingly, education on how SCD could affect pregnancy and vice versa and the principles of improving outcomes for mother and baby. Ensuring optimal management and screen for end organ damage. A pre-pregnancy history of very frequent hospitalizations and/or episodes of acute chest syndrome are associated with poorer overall health and increased risks during pregnancy. An assessment of systemic complications such as renal and ophthalmic complications, hypertension should be treated to lower SBP to ≤140 mmHg and DBP to ≤90 mmHg. Some experts recommend that all SCD patients be screened for pulmonary hypertension with Doppler echocardiography. Women with significant pulmonary hypertension and significant cardiomyopathies classified as modified World Health Organization Class IV diseases should be counseled that pregnancy is contraindicated and termination should be considered. Folic acid (5 mg) should be given once daily preconception and during conception to reduce the risk of neural tube defect and to make up for increased demand for folate during pregnancy. Vaccinations against encapsulated organism such as pneumococcal and meningococcal organism can be offered to them where they are available. Use of Hydroxyurea, ACE-Inhibitors and Angiotensin receptor blockers should be discontinued at least 3 months before conception. 

Best provided by a multidisciplinary team involving a feto-maternal specialist and hematologist and other specialist as regards affected end organ if any, in other parts of the world other specialist such in geneticist, pain specialist, and social workers or behavioral health staffs could as well be involved in their care. Early, detailed evaluation/screening for end organ damage. Numerous studies have shown that management by a multidisciplinary team is the single most important determinant of pregnancy outcome. Health education on the events of pregnancy, labour and puerperium are of crucial importance. Laboratory evaluation includes assessing for anaemia, evaluating iron stores, obtaining a type and screen to assess for alloimmunization, in addition to routine investigations done for general antenatal care for other women.

Blood pressure, oxygen saturation check and urinalysis at each visit are important in them, monthly midstream urine for MCS because they are at risk of asymptomatic bacteriuria. Adequate health education also important in them, Ultrasound scan to confirm /date pregnancy, anomaly scan at 20-22 weeks and fetal biometry 4 weekly from age of viability. Folic acid supplementation. Iron supplementation only with laboratory proof of Iron deficiency. Low dose aspirin for women at risk of early onset PIH. Low molecular weight heparin during hospital admissions or periods of restricted activity. Antimalarial prophylaxis with proguanil.

Role of Multidisciplinary team cannot be over emphasized at all levels of care and also ensure rapid clinical assessment. Adequate analgesia, avoid pethidine because of risk of seizures. If pain is severe and oral analgesia is not effective, give strong opioids (e.g. morphine). Give adjuvant non-opioid analgesia: paracetamol, NSAID (if 12–28 weeks of gestation). Monitor pain, sedation, vital signs, respiratory rate and oxygen saturation every 20–30 minutes until pain is controlled and vital signs are stable, then monitor every 2 hours (hourly if receiving parenteral opiates) Give a rescue dose of analgesia if required. If respiratory rate is less than 10/minute, omit maintenance analgesia; consider naloxone. Consider reducing analgesia after 2–3 days and replacing injections with equivalent dose of oral analgesia. Use of fluids and oxygen should be individualized. Thromboprophylaxis should be provided. A painful crisis is the most common complication of SCD in pregnancy with a quarter to half of women having at least an episode. Most frequent cause of hospital admission. Known precipitants like dehydration, infections and cold stressors should be avoided in them. Discharge the woman when pain is controlled and improving without analgesia or on acceptable doses of oral analgesia. 

Inform multidisciplinary team of obstetrician, haematologist, anaesthesiologist, paediatrician. Close maternal monitoring and also fetal monitoring with Continuous CTG. Adequate intravenous fluids using a fluid balance chart if oral intake is not tolerated or is inadequate. Maintain Adequate maternal oxygen saturation. Adequate Analgesia; opiates may be used except pethidine. PCV might need to be checked about 2 to 3 times during labour as the case may be. Second stage of labour should be assisted. Regional analgesia/ anaesthesia for caesarean section.

Early testing for SCD if the baby is at high risk of SCD, maternal oxygen saturation should be above 94% until discharge. Adequate hydration based on fluid balance. Thromboprophylaxis with low molecular weight heparin for 7 days postpartum and antithrombotic stockings are also important in them. PCV check every 4 to 12 hour as the case may be because the risk of vaso-occlusive and hemolytic crises is also high during this period so that prompt red cell replacement can be instituted

Small family sizes and good spacing should be advised. Progestogen only contraceptives, barrier contraception are viable options. Fear of increased risk of thromboembolism with use of combined contraceptive preparations due to estrogen component has remained a point of concern to obstetricians in prescribing this to them. Permanent form of contraception such as bilateral tubal ligation can be offered to those whom pregnancy is contraindicated. 

Sickle cell anemia in pregnancy poses significant risks for both mothers and infants in Sub-Saharan Africa, where the disease is prevalent and healthcare resources are often limited. Pregnant women with sickle cell disease are at increased risk of complications such as preeclampsia, preterm labor, and infections, contributing to higher rates of maternal and neonatal mortality. Although advancements in healthcare are improving outcomes, challenges remain due to inadequate access to specialized care, early diagnosis, and management. Strengthening healthcare systems, enhancing prenatal care, and increasing awareness are crucial to improving outcomes for pregnant women with sickle cell anemia in this region.