Sickle Cell Disease in Pregnancy

BY: Dr. Olagoke A. Ewedairo

Outline

• Introduction• Brief overview of Sickle cell disease• Sickle Cell Disease and Pregnancy• Management• Complication• Prognosis• Role of the Family Physician

Introduction

• Sickle cell disease (SCDx) has been associated with a reduced quality of life and life expectancy amongst certain groups of people

• Pregnancy on it’s own also carries some risk of mortality from the pregnancy itself, delivery or conditions aggravated by the pregnancy

Introduction cont’d

• Together there is geometric increase in the risk of death with each pregnancy

• The outcome of a pregnancy as well as the overall well being of a patient with SCDx is dependent on several factors, top of the list are socio-economic status of the patient and the quality of their health care delivery system.

Introduction Cont’d

• In any part of the world, sickle cell disease in pregnancy is termed “high risk pregnancy”

• In Nigeria were majority of the citizenry live below the poverty line and whose health care system is flawed at practically all levels, what shall we then term SCDx in pregnancy?

•SUPER HIGH RISK?

Brief Overview if Sickle cell disease

• SCDx is an autosomal recessive haemoglobinopathy, it results from abnormality of the 6th amino acid of the β-globin chain of the haemoglobin

• Valine replaces Glutamine normally seen in the A genotype this is responsible for the S gene. While for the C gene, Lysine is the substitute for Glutamine

Overview Cont’d• Epidemiology: seen in Africans African Americans,

Mediterranean's, Caribbean's, East Indians, and South and Central Americas.

• In Nigeria, an estimated 91,000 babies are born with the disorder each year. By 2050, those numbers are expected to rise to nearly about 140,000.

• No Sex variation• 25% of Africans have the S gene, most of whom are

asymptomatic carriers (AS), however 2-3% of Africans are affected by the disease (SS, SC or rarely Sβ thallasaemia)

• Homozygote SS are said to have Sickle Cell Anaemia

Pathology of sickle cell disease

• The abnormality in the haemoglobin results inability of the affected red cell to be pliable, thus in the presence of certain conditions the red cell sickle, and are unable to pass through micro vasculature resulting in thrombus formation which leads to Ischaemia and Infarction.

• This accounts for the characteristic bone pain experienced by the patients (i.e Vaso- occlussive crisis)

• Other forms of crisis experienced by patients with SCDx include;

• Haemolytic crisis (RES)• Aplastic crisis• Sequestration crisis• Acute Chest syndrome

Sickle cell disease and pregnancy

• It was initially rare to find women with SCDx who were pregnant, this is because most of them did not live long enough to get to puberty let alone get pregnant.

• With improved methods of care, more and more women with SCDx are living to have children, though they are advised to limit deliveries to 1 or 2

• All the above mentioned forms of crisis are also seen in pregnancy.

• In addition to all the above mentioned problems with sickle cell disease, there are some others which are seen in pregnancy

• These are classified in three broad groups

Effect of Sickle cell disease on pregnancy

• Increased risk of Miscarriage• Increased risk Operative delivery• Increased risk of Preterm delivery• Pseudotoxaemia (Systollic Hypertension, Proteinuria,

severe bone pain crisis)• Reduced tolerance for haemorrhage• Increased risk of heart failure• Increased risk of maternal mortality

Effect of pregnancy on sickle cell disease

• Increased risk of thrombosis (pregnancy is a hypercoagulable state)

• Increased number of painful crisis (even in SC)• Increased severity of crisis• Increased level of haemolysis (Late pregnancy,

intrapartum, Immediate post partum)• Increased risk of severe anaemia (malaria, dilutional)• Increased need for blood transfusion and its

attending complications

Effect of sickle cell disease on the foetus

• Increased risk of pregnancy loss• Increased risk of Intrapartum hypoxia (placental infarct)• Increased risk of Birth asphyxia• Increased risk of IUGR (1-25%)• Increased risk of LBW babies• Increased risk of preterm delivery (1-30%)• Increased risk of Prematurity and their attendant

problems• Increased risk of still birth (1-6%)• Increased risk of peri-natal mortality (5-10 fold )

Management of Sickle cell disease in pregnancy

• The management of a pregnant woman with sickle cell disease starts before she even gets pregnant

• The care of these patients is there fore divided into • Preconception care• Antenatal care• Intrapartum care• Post partum care

Preconception care

• The aim of preconception care is to optimize a woman's health and knowledge before she conceives to ensure the well being of the mother and the child after delivery

• Stop all teratogenic drugs (e.g Hydroxyurea)• Treat any infection vigorously

Preconception care cont’d

• Optimize Hb and PCV (treat malaria, IPT, F/acid increased to tds, Antihelmintic)

• Ensure adequate and nutritional diet• Multivitamin supplements• Prophylactic transfusion (to increase Hb to at least

10g/dl)• Counsel on need for early booking for ANC

Antenatal Care• Ensure early booking (8weeks GA)• Hb and PCV at booking, monthly and at onset of

labour• Urinalysis to detect UTI at booking and monthly

(proteinuria, nitrites, leucocytes) Treat even if asymptomatic.

• VDRL, HBsAg, HIV sceening• F/acid 5mg tds, no Iron sipplementation• Proguanil 100-200mg daily• USScan at booking, 20weeks and 32weeks

Antenatal care cont’d

• Tetanus toxoid• 2 weekly antenatal visits,

increase to weekly in later part of pregnancy

• Fundal height monitored closely to detect IUGR

• Fetal kick monitoring by mother especially close to term

• Ensure availability of 2 units of blood available before delivery

• Ensure adequate analgesia when required

• Counsel on prompt arrival at hospital when labour begins or if any danger sign is noted

• Discuss post delivery contraception

Intrapartum care

• Labour and delivery must be conducted at a tertiary institution regardless of parity.

• PCV, Urinalysis at onset of labour• Unduly long labour not allowed (early augmentation

& assisted delivery if required) • Operative delivery is not routine, if performed, EBL is

replaced ml for ml also applicable for vaginal delivery• Monitor FHR every 15mins, CTG monitoring were

available

Post Partum Care• Immediate post delivery PCV check and repeated

4hourly x 24hours • Pseudotoxaemia (Adequate analgesia, O₂

supplementation, Blood transfusion to increase O₂ carrying capacity, Delivery by C/S)

• Heparinization should be commenced 2hours post delivery 10,000U stat then 5000-15,000U 6hrly (maintain clotting time ≥17minutes)

• Breast feeding should commence within 30minutes of delivery (further decrease PPH)

• Transfuse if necessary (recommended)• Monitor PR, BP q 30mins for the 1st 6 hours • Reiterate the need for contraception

Complications of Sickle cell disease• CVA (usually thrombotic)• Ophthalmic complications (Retinal infarcts,

proliferative retinopathy, retinal detachment)• Pulmonary hypertension• Congestive cardiac failure (chronic anaemia)• Autosplenectomy• Gall stones• AVN• Chronic ankle ulcer

Prognosis• Prognosis is generally poor as most centers can not

provide the kind care these patients require• The successful birth of one child by a patient with

SCDx does not guarantee the success of subsequent pregnancies.

• Improved nutrition and socio economic status have been associated with better outcomes

• Prenatal diagnosis in 1st trimester using CVS gives women the opportunity to decide whether to have a child with SCDx or not.

Role of the Family Physician

• Education on primary prevention of Sickle cell disease (“premarital counseling” Preconception counseling and prenatal diagnosis)

• We offer continued, comprehensive and coordinated care. From childhood till when she is pregnant

• Ensure optimal steady state prior to getting pregnant• Provide coordinated care when in labour

Conclusion• Sickle cell disease is a major health problem and an

important risk factor for perinatal morbidity and mortality.

• Management should be multidisciplinary• Despite all the effects of sickle cell disease on a

patient and the attendant problems of a co-existing pregnancy, with adequate and skilled care of these patients, a happy ending can realistically still be expected.

• Thank you!