Sickle Cell Disease in Pregnancy Flashcards
Definition of SCD
a group of genetic single-gene autosomal recessive disorders caused by the “sickle” gene, which affects haemoglobin structure.
What are the hemoglobin types of Sickle cell trait
Hb A & Hb S
Ratio of HbA:HbS in Sickle cell trait
60:40
Type of Hb in Sickle Cell Anemia
HbSS
incidence of pregnancies with SCD in UK
100-200 Pregnancies per year
How many children born with SCD worldwide each year
300 K infants
How many children born with SCD in UK each year
300 infants
Average life expectancy of SCD
mid 50s
Is vomiting a red flag in SCD pregnant woman
Vomiting leads to Dehydration which leads Sickle cell Crisis
In SCD it’s importnat to check for End-organ Damage like:
- Echo for PHTN
- BP & Urinalysis for HTN &/or Proteinuria
- Retinal Scan for Proliferative retinopathy
- Cardiac MRI for Iron Overload (if woman has high ferritin or multiple transfusion)
- RFTs & LFTs anually
importance of retinal screening in SCD
for Proliferative Retinopathy (most common in pts with SCD esp. in HbSD)
Medications to be stopped in pregnant women with SCD
- Hydroxycarbamide (hydroxyurea): stop before 3 months of conception - teratogenic
If peregnancy happened before stopping-> stop it, US for malformations (termination isn’t indicated) - ACEI
- ARB
SCD patient is susciptable for what infections
SCD patients are in risk of infection with encapsulated bacteria
1. Strept Pneumococci
2. Hemophilus Influenza
3. Niesseria Gonnorreah
4. Salmonella
Vaccinations that SCD patient shoud have before pregnancy
- H. Influenza vaccine (single dose)
- Conjugated Meningeococcal. C vaccine (single dose)
- Pneumococcal vaccine (every 5 years)
- Hepatitis B vaccine
- Ifluenza and Swine flu vaccine* (annualy)*
US schedule for Pregnant SCD patient
7-9 W: viability scan (in view of inc. risk of miscarriage)
11-14 W: Routine 1st trimester scan
20 W: Anomaly Scan
From 24 W: Serial Growth Scan (every 4W)
Medications to be given in SCD pregnant patient
- Folic Acid 5mg/d
- Penicillin Prophylaxis
- Iron supplements if iron def. anemia
- Low dose Aspirin 75mg/d from 12W -> dec. risk of PE
- Prophylactic LMWH & TED socking in antenatal admissions
- NSAIDs allowed only bet. 12-28 W
What to do if pregnancy happedned while in SCD patient is on Hydoxycarbamide?
Stop the medication -> US for malformations
Termination isn’t indicated
Blood Transfusion to pregnant SCD patient
- Routine prophylactic transfusion isn’t recommended (except in twins pregnancy)
- Alloimunization is common in SCD (18-36%)
- Rhesus Matching
- Blood should be tested negative for CMV
Indication for blood transfusion in SCD patient during pregnancy
- Clinical findings
- hemoglobin under 6 g/dl
- Fall of over to 2g/dl from baseline
When is exchange transfusion is indicated in pregnant SCD patient
- Acute Coronary syn. ACS
- Acute Stroke
What is Acute Painful Crisis
Also named vaso-occlusive crisis, it is the main clincal feature of SCD, should be excluded in SCD pt. who become unwell
Management of Acute painful crisis in pregnancy
- Rapid Assessment and appropriate analgesia given
- Pethidine shouldn’t be used (risk of seizures)
- Fluids & O2 when needed
- Thromboprophylaxis should be given
Most frequent complication of SCD in pregnancy
Acute painful crisis (most common cause of admission)
Indication for referral to hospital in women with painful crisis
- Pain doesn’t relieved by simple analgesics
- Fever
- Atypical pain or chest pain or shortness of breath
Investigations of Painful Crisis
- FBC
- Reticulocytes count
- RFT
- Others depending on clincal scenario
Management of painful crisis
- Mild pain: Paracetamol
- mild to moderate: NSAIDs (if 12-28w)
- Moderate: Weak opioids (co-dydramol- dihydrocodiene)
- Severe pain: Strong Opiates (morphine)
Monitor pain severity, RR, O2 saturation every 20 minutes
If RR<10/min omit maintainanace analgesia (naloxone)
When treating painful crisis with strong opioids, how to follow up
if a mother recieved opiates in late preg. for ong time: neonate should be observed for signs of opioid withdrawal
Assesing infection in painful crisis
- offer antibiotics if febrile
- WBCs are high in SCD, doesn’t mean infection
- Thromboprophylaxis to women w/ SCD and Painful crisis
Fluid intake in management of a case of painful crisis
at least 60 ml/kg/24 hours
Management of Acute stroke d.t. SCD in pregnant patient
Rapid exchange blood transfusion, can dec. long term neurological damage
Thrombolysis isn’t indicated
Woman with SCD presented with acute hypoxia, what to suspect?
suspect Pulmonary embolism which has a raised risk with SCD.
There should be a low threshold for considering pulmonary embolism
Causes of Acute anemia in patient with SCD
Due to Erythovirus infection:
- Causes aplastic anemia
- risk of vertical transmission to fetus causin hydrops fetalis
Other causes:
malaria & splenic sequestration
Delivery of SCD patient
- It isn’t a CI of Vaginal delivery or VBAC
- Eletive birth by IOL or elective CS if indicated, after 38w with no FGR
- Blood Crossmatch if there are atypical anitbodies
- if there are no antibodies just “group and save”
Delivery of SCD patient
- It isn’t a CI of Vaginal delivery or VBAC
- Eletive birth by IOL or elective CS if indicated, after 38w with no FGR
- Blood Crossmatch if there are atypical anitbodies
- if there are no antibodies just “group and save”
Optimal mode of anaesthesia
- regional is recommended in CS
- General should be avoided
Postpartum care of aptient with SCD
- Early testing of baby for SCD
- maintain maternal O2 saturation >94%
- Adeguate hydration
- LMWH while in hospital for 7 days after vaginal delivery and 6 weeks after CS
5.
Postpartum contraceptive safe with SCD
- POP, Depo-provera & Mirena are safe and effective.
- Estrogen containing pills - Cu IUD(2nd line)
- DMPA: less painful episodes
What is Acute Chest Syndrome
Acute chest syndrome affects the lungs and is diagnosed by a new pulmonary infiltrate, dyspnea, hypoxia after ruling out pulmonary embolism or pneumonia.
What is Acute Chest Syndrome
Acute chest syndrome affects the lungs and is diagnosed by a new pulmonary infiltrate, dyspnea, hypoxia after ruling out pulmonary embolism or pneumonia.
Management of acute chest syndrome
Acute chest syndrome, a complication of sickle cell disease, when severe is best treated with partial exchange transfusion.
