Sickle Cell Disease in Pregnancy GTG Flashcards
What is the inheritance of sickle cell?
AR
Number ore pregnancies per year in women with SCD?
How many babies born with the condition?
100-200/year
300 born/year
What is sickle cell Disease/Trait?
Disease: Abnormality of the haemoglobin HbSS or HBS + other clinically abnormal haemoglobin HbSC, HbSB
Trait: 1 Abnormal gene HbAS carrier, - mostly asymptomatic, risk UTI, microscopic haematuria
Common clinical presentation of SCD
Painful crisis
* Avascular necrosis
* Stroke
* Pulmonary hypertension
* Retinal disease
* Leg ulcers
* Acute chest syndrome (ACS)
* Renal dysfunction
* Cholelithiasis
What should be offered in pre-conception care for women with sickle cell disease?
Review meds - stop ACEi/ARB/hydroxycarbamine, iron chelators
Vaccines: Influenza/pneumococaal
Start meds - 5mg Folic acid, ensure daily penicillin (hyposplenic), aspirin 12 weeks, consider LMWH in 1st trimester depending VTE, vitmain D
Assess chronic disease
-ECHO (PAH)
- BP/urine/O2 sats
- U+E/LFT
- Retinal screen
- Screen red cell antibodies
- Test partner
Which partner haemoglinopathies which conditions should be referred for PGD/counselling
HbS
B thalassaemia
O-Arab
HbC
D-punjab
Consider
DB thalassaemia, Lepore, HbE, hereditary persistance fetal haeomgloin
Vaccines for patients with SCD or thalassaemia + splenectomy
Vaccine:
* Pneumococcal vaccine
* Hepatitis B
* H. influenzae type b
* Influenza and swine flu
* Conjugated meningococcal C
* Meningitis B and ACWY
+ daily penicillin
What gestation can NSAIDS be given?
12-28 weeks
When should USS be offered?
Early 7-9 weeks (increased risk misc)
11-14 weeks
Detailed 20 weeks
Every 4 weeks from 24 weeks
When should be seen antenatally?
Review MDT Obs and Haemophilia @ 16 + 20 weeks
From 24 weeks MDT every 4 weeks, and MW every 4 weeks - seen every 2 weeks
What should be carefully checked at each appointment
BP and urine - high risk PET, carefully monitor for rise in BP
When should FBC be checked?
20, 28 and 32 weeks
How common is alloimmunisation in SCD?
18-36% - risk delayed transfusion reaction, haemolytic disease of newborn
When should blood transfusion be offered fort SCD?
Do not offer routinely, MDT discussion
Previous serious medical/obstetric/fetal complication - Exchange/top-up
Transfusion regime pre-pregnancy for stroke prevention - continue in pregnancy
Twin preg - prophylactic transfusion
Acute anaemia - top-up
Acute chest syndrome/actute stroke - exchange
How common is acute pain crisis in pregnancy?
57% of pregnancies
17% required hospital admission
How to investigate actue pain crisis
A-E assessment
Observations, <95% give oxygen
Hydration status
Consider - ACS, acute stroke, aplastic crisis, infection, osteomyelitis, splenic sequestration
Bloods - looks at FBC, reticulocytes, renal function
What treatment to offer in acute pain crisis?
MDT (Obs, MW, haem,anaesthetics)
Offer analgesia within 30 mins
Consider Laxatives, antipruritic, antiseptic
Montior pain/sedation/vitals - O2 sats every 20-30 mins until pain controlled
VTE prophylaxis
Consider IVI
> 28 weeks CTG, consider USS
What analgesia to give in pain crisis
Do not give pethidine
Start PO - opioids
NSAIDS if 12-28
Consider opioid toxicity if <RR, consider naloxone
Should have plan for pain crisis
How common is acute chest syndrome in pregnancy? How does it present?
10% SCD in pregnancy
Fever +/- resp symptoms/ new pulmonary infiltrated on CXR.
DD PE/pneumonia
How to manage acute chest syndrome
Analgesia
Oxygen
Incentive spirometry
Tx bacterial/viral infection - give Abx that cover atypical Abx
Blood transfusion - if falling or <65, severe exchange
Consider critical care for non-invasive/invasive ventilation if deteriorating
If requires transfusion should be offer blood transufiouns for the rest of pregnancy.
If SCD women present with neurological Sx, what to consider and mange?
Acute stroke - infarctive or haemorrhage.
Urgent CT/MRI
Discuss with stroke physician and haem.
DD PET/eclamasia
Required exchange transfusion, discuss thromboylsis
What is an important cause of acute anaemia to consider? What management?
Consider Parvovirus B19
Reticulocyte - will be low, red cell maturation arrest
Test serology
Blood transfusion & isolate
Refer to MDU - asses fetal anaemia
Consider malaria, splenic sequestration
VTE assessment in SCD
- Calculate score in early pregnancy
- If SCD alone for LMWH from 28 weeks and 6 weeks PP, if additional RF, from beginning of pregnancy
- LMWH during admission
- Higher risk if complications such as vast-occlusive disease
When should pregnancy women with SCD and a normally grown baby be delivered?
38-40 weeks
Can offer SVD/IOL or ELCS
Can offer VBAC
Optical intrapartum care for SCD
Deliver in hospital able to manage complication of SCD/high risk pregnancy
MDT (haem, Obs, anaesthetic, senior MW)
Good analgesia
Avoid dehydration - fluid balance
Regular O2 Sats <94% ABG & oxygen
Avoid protracted labour
Avoid pethidine
Hourly obs including temp
Cont CTG
X match if atypical antibodies
Good positioning - especially if hip replacement (avascular nerosis of hip)
Keep patient warm
If EMCS - regional avoid GA
What is the risk of sickle cell crisis in the postpartum period?
21-25%, more common post GA
How to avoid sickle cell crisis post partum?
Same as intrapartum, good analgesia, fluid balance, mobilise.
Can women with SCD breastfeed? Any precautions?
Yes, encourage BF
Avoid prescribing codeine to mother, can have dihydrocodiene and tramadol at lowest dose
How long PN LMWH?
6 weeks
What are the preferred options of contraception with women with SCD? What contraceptives have cautions?
Lowest rate of failure - LNG-IUS and IM DMPA - can reduced painful crisis
CuIUD - Category 2 - increased risk blood loss
CHCs Category 2 - risk of thrombosis and SC crisis maybe increased
When should fetal testing of SCD be performed?
If partner is a carrier or partner affect or unknown status
