Sickle cell disease in pregnancy anaemia by mikesanye

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									Sickle cell disease in
  •A disease of tremendous
      clinical variability.
•Clinical presentation seldom
    similar between any 2
      Common clinical features
   Chronic haemolytic anaemia
   The occurrence of episodic
    sometimes catastrophic
   Typically unpredictable timing

   People with sickle cell disease do
    not feel ill
   Participate in regular activities
   A few achieve high education and
    professional life
   Survival into adulthood: still little
    assurance of continued wellbeing
   Chronic haemolytic anaemia
   Micro and macrovascular occlusion
    leading to chronic organ damage
   Joint, Renal,cardiac,chest,
   Retinopathy
    Will I have problem conceiving?
   Fertility of women with sickle cell
    disease is generally unaffected
   Except for the usual reason
     • Deferred age
     • Poor semen
     • Ovulatory disorders
     • Tubal disease
    Which contraception should I
   No contraception is contra-indicated
   Barrier methods: Easily
    available,safe,low PI
   Progestogen only pill
   Low dose combined oral
    contraceptives:Not contra-indicated
   Depot progestogen
   IUCD: Relative contra-indication thus
    Mirena instead
    Pre-pregnancy counselling

   Combined clinic ideal
   Partner testing/PND discussion
   Pattern and frequency of crises
   Previous CVA,infarction,VTE
   Analgesic dependency
   Transfusion history
   Echocardiography
      Prepregnancy counselling
   Immunization status
   Antibiotics prophylaxis
   Renal and hepatic status
   Cardiopulmonary status
   Eye status
   High dose folic acid
   Past Obstetric history
   Individualized care plan
    Pregnancy and hydroxyurea
   Safety remains unclear
   Very large dose in animals shown to
    be teratogenic
   To date no teratogenic effects
    reported in humans
   Avoid pregnancy,
   If become pregnant on it, stop
    medication and no need to terminate
Antenatal and neonatal screening:
   To reduce infant morbidity and
    mortality from undiagnosed SCD
   Alerting women to the possibility of
    having affected child
   Counseling about the condition
   To offer the option of prenatal
   Occasionally picks up new and milder
Neonatal screening: Where are we
   Newborns-Universal screening by
    March 2005
   Guthrie test at 5-8
   Fully implemented in London since
    Sept 2003
   London prevalence of SCD 1:750

                         Alison Streetly(Programme
Antenatal screening: Where are we
   Two phase roll out
   Full coverage by March
   Universal thalasaemia screening
   Sickle cell high or low prevalence
   Family origin question(Dyson et al,2006)
   Midwives role pivotal in the process

                           Aliosn Streetly (program
          Suitability for PGD
    Fertility problems, objection to
    abortions, or history of multiply
    affected babies
   Family or personal history of
    Mendelian disorder or chromosomal
   PGD License and technique for
   Payment - either personal or local
          Limitations of PGD

   Not suitable for majority of couples
   Stressful, time-consuming,
    requiring high level of commitment
   Only 15-20% of PGD cycles result
    in babies
   Reduces risk rather than eliminates
    - roughly 5% failure rate, due to
    limitation of single-cell analysis
    Non-invasive Fetal Diagnosis
   Isolation of fetal DNA from maternal blood
    • small amounts of free fetal DNA present in
      maternal plasma
    • technically easy to concentrate and analyse by
    • limited application - dominant diseases,
      screening for paternal contributions to
      compound heterozygous states
    • currently being developed at KCH for HbSC
         Maternal and fetal risk
   Maternal                 Fetal
   Anaemia                  Miscarraige
   Infection(UTI,Chest      Intra-uterine
    ,puerperal sepsis)        growth restriction
   Crises                   Increased risk of
   PET                       prematurity
   Thromboembolism          Increased risk of
                              fetal distress
          Antenatal care

  To give appropriate care to ensure
       healthy mother and baby
Avoidance and early treatment of crises
 Low threshold for admission if unwell
Screening of partner and offer prenatal
      diagnosis where indicated

Early booking between 6 and 12 weeks
           is recommended
      Early dating scan essential
 Ensure taking folic acid and penicillin
  Booking investigations +PET bloods
   Antenatal sickle clinic(ASC)

    Once a month Joint clinic with
Haematologist,Specialist nurse and high
             risk midwives
  Once a month review by high risk
 Access to antenatal and haematology
        day unit 7 days a week
             ASC clinic

At 20 week scan uterine artery dopplers
        4 weekly growth scans
 Delivery between 38-40 weeks unless
     obstetric indication otherwise
Reasons for admission

Sickle cell crises and pain
    Blood transfusion
   Shortness of breath
   Induction of labour
Sickle crises:Precipitating factors

         Prolonged labour
         operative delivery
       Keep warm and hydrated
  Maintain strict fluid balance chart
If initial saturation below 94%, give
         humidified Oxygen 4l
             Do blood gases
         Appropriate pain relief
            Infection screen
 Physiotherapy if evidence of chest
     Blood transfusion

   Only if clinically indicated
On going high transfusion regime
       Multiple pregnancy
    Complicated pregnancy
        Recurrent crises
Umbilical cord blood banking
●   UK national cord blood bank
    currently stores over 7000 units
    of cord blood samples
●   Donated altruistically for non-
    directed use
●   Under the auspices of National
    Blood service
●   Currently seven other private
    banks in the UK
     Umbilical cord banking
    Dual public-private approach
●   1 in 10 000 to 1 in 20 000
    likelihood for personal use
●   Used in preference to Bone
    ●   Fewer complications,easier
    ●   higher matching success esp BME.
        Limitations:cells from 1 cord
    ●   Defect already present in the
        child own cord cells
     Pitfalls in management

Haemoglobin SC disease may not run a
       benign course in pregnancy
 Prompt diagnosis of crises saves lives
  Acute chest syndrome may develop
  from an initial uncomplicated crises
    Signs of ACS can be non-specific
      Acute chest syndrome

Pulmonary crises-infarction+/-infection
       Adults are often afebrile
 Lung examination can be normal but
         progressive hypoxia
    CXR: Lower lobe involvement
  Management of Acute chest
Involve haematologist and anaesthetist
Blood should be leucocyte depleted and
           phenotype specific
         Exchange transfusion
          Therapeutic clexane
      Oxygen and physiotherapy
Sudden death in sickle cell
   Recognised in SS, SC and AS
       Postmorten evidence
Intravascular plugs of sickle RBCS
Extensive fibromascular dysplasia
  Abnormal fibrosis through out
        conducting system
Lethal cardiac electrical instability

    Sickle cell disease is an inherited
disorder of varying clinical severity with
    potentially serious complications
Pregnant woman with sickle cell disease
is at considerable risk of morbidity and
 mortality and perinatal mortality rates
                  are high

  Expert knowledge of the condition,
    proper crises intervention and
appropriate management can alleviate
     some of these complications

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