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8-Thalassaemia In Pregnancy > Nice > Flashcards

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1
Q

Explain the reduction in fertility associated with thalassaemia ?
How to manage?

A

🔴In transfusion dependent women where chelation has been suboptimal 👉 iron overload
👉 damage to the anterior pituitary
🔴 they require ovulation induction using injectable gonadotrophins

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2
Q

What is the optimum preconceptual care for women with thalassaemia?

A

1-Screening for end organ damage
2- optimization of complications prior to pregnancy.
3- review transfusion requirements
4-compliance with chelation therapy
5- assessment of body iron burden

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3
Q

What are the contraceptions that may be used in women with thalassaemia?

A

Any contraindications ( OC-pop- implants- mirena)

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4
Q

Are there any interventions which are beneficial at preconceptual stage ?

A

Aggressive chelation: can reduce iron burden and reduce end organ damage.
(⬇️ endocrinopathy / ⬇️ cardiac problems).

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5
Q

Why it is necessary to optimize iron burden prepregnancy?

A

Because: all chelation therapy should be regarded as potential teratogenic in the first trimester.

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6
Q

What chelation agent has evidence for use in 2nd & 3rd trimester?

A

Desferrioxamine
* has a short half life
* safe for infusion during ovulation induction
* safe after 20 weeks at low doses

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7
Q

Since diabetes is common in women with thalassaemia.. how to consult a diabetic women with thalassaemia prepregnancy?

A

Should have serum Fructosamine concentrations < 300 for at least 3 months prior to conception
Equivalent to HbA1c of 43 mmol/mol

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8
Q

Why serum fructosamine is preferred for monitoring diabetic women with thalassaemia?

A

Because HbA1c may diluted by transfused blood and the results in underestimation

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9
Q

How to preform screening organ damage in women with thalassaemia preconception?

A

1- pancreas: 👉 diabetes
2- thyroid; thyroid functions
3- heart: echocardiogram & ECG
T2 cardiac MRI
4- liver : -assess liver iron concentration using FerriScan/ liver T2
- ultrasound for liver and gallbladder
5- Bone density scan
6- serum vit D
7- red cells antibodies: ABO and full blood group genotype and antibody titres

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10
Q

How to asses the cardiac status in women with thalassaemia preconception?

A

🛑All women should be assessed by cardiologist expertise in iron overload.,prior to pregnancy
❤ By : 1- echocardiogram ( cardiomyopathy)
2- ECG ( arrhythmias)
3- T2 cardiac MRI

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11
Q

How to asses liver status in women with thalassaemia preconception?

A

1- Women should be assessed for liver iron concentration using : FerriScan or liver T2
2 - liver and gallbladder ultrasound

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12
Q

Liver and gallbladder ultrasound in women with thalassaemia is used to detect what?

A

1- cholelithiasis
2-liver cirrhosis due to iron overload
3- transfusion related viral hepatitis

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13
Q

What is the target of liver iron in women with thalassaemia before pregnancy?

A

Should be less than 7 mg/ g

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14
Q

If liver iron exceeds 15 mg/g prior to conception.. what is the management in pregnancy?

A

Iron chelation with low dose of desferrioxamine between 20-28 weeks
( because the risk of increased myocardial iron loading)

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15
Q

What makes osteoporosis a common finding in women with thalassaemia?

A
  • underlying thalassaimic bone disease
  • chelation of Ca by chelation drugs
  • hypothyroidism
  • Vit D deficiency
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16
Q

What is the prevalence of allo immunity in individuals with thalassaemia?

A

16% 👉 indicate a risk of hemolytic disease of the fetus & newborn
👉 may be challenges in obtaining suitable blood for transfusion.

17
Q

What medications should be reviewed preconcepually in women with thalassaemia?

A

*Iron chelators: - deferasirox oral
- deferiprone oral
( women should be converted to
Desferrioxamine subcutaneous)
* bisphosphonate
🛑 should ideally be discontinued
3 months prior to conception

18
Q

What is the role of genetic screening in women with thalassaemia?

A

PGD should be considered in the presence of haemoglobinopathies in both partners so that :
A homozygous /
Compound heterozygous
Can be avoided

19
Q

What are the conditions in the partner that require counseling if the mother is affected by thalassaemia?

A

risk of serious haemoglobinopathy :
- B thalassaemia
- HbS
- HbE
- delta bata thalassaemia
- Hb lepore
- HbO arab
- Hb constant spring
Risk of mild to moderate haemoglobinopathy:
- HbC
- other variant haemoglobin

20
Q

What is the importance of immunization in women who are transfused ?

A
  • Hepatitis B vaccination
  • determine Hepatitis C status ( common & asymptomatic)
    • If positive 👉 RNA titres should
      be determined
21
Q

What is the importance of immunization/ Antibiotics prophylaxis in women who have had splenectomy?

A
  • daily penicillin prophylaxis: high risk of infection from encapsulated bacteria As: - Neisseria meningitis
    -Streptococcus Pneumonia
    - Haemophilus influenza type b
  • vaccination for :
    • Haemophilus influenza type b
    • conjugated meningococcal C
      Single dose
      -pneumococcal vaccine: every 5 y
22
Q

What vitamin supplements should be recommended preconceptually to women with thalassaemia?

A

Folic acid 5 mg / d ( 3 months)

23
Q

How is antenatal care provided to women with thalassaemia?

A

1- visits : monthly until 28 weeks and fortnightly thereafter.
2- thalassaemia major 👉 cardiac assessment at 28 w
3- women with diabetes 👉 monthly -assessment of serum Fructosamine
- review in diabetic pregnancy clinic
4- women with hypothyroidism 👉 monitor thyroid function

24
Q

What is the recommended schedule of US during pregnancy in women with thalassaemia?

A

Should be offered:
- early scan 7-9 w ( high risk of early pregnancy loss)
- routine 11-14 w
- routine anomaly scan 18-20 w
-From 24 w : every 4 w

25
Q

How should the transfusion regimen be managed during pregnancy in women with thalassaemia major?

A

❤ should be receiving blood transfusions on regular basis
Aim : pretransfusion Hb of 10

26
Q

How should the transfusion regimen be managed during pregnancy in women with thalassaemia intermedia ?

A

Consider regular transfusion if :
- worsening maternal anaemia
- evidence of FGR
❤ woman asymptomatic with normal fetal growth and low Hb :
**Should be assessed in late pregnancy:
* Hb > 8 at 36 w : avoid transfusion
* Hb < 8 : 2 units at 37-38 w

27
Q

What antenatal thromboprophylaxis is recommended in women with thalassaemia?

A

❤Aspirin 75 mg / d:
- who have undergone splenectomy
OR - have plt count > 600,000
❤ LMWH + Aspirin:
- who have undergone splenectomy
AND - have plt count > 600,000

28
Q

Among pregnant women with thalassaemia who are in the highest risk of venous thromboembolism?

A

Splenectomised women with thalassaemia intermedia who are not receiving transfusions
■{ good transfusion regimen suppresses endogenous erythropoiesis}■

29
Q

What is the optimum antenatal management of women with myocardial iron?

A
  • regular cardiology review
  • monitor cardiac EF
    If the woman describes symptoms:
    Palpitations- breathlessness- syncope- orthopnoea- peripheral oedema:
    1- echocardiography: ⬇️EF / increasing ventricular volume 👉 increasing risk of heart failure
    2- 24h ECG
    3- cardiac MRI
30
Q

What are the cardiac MRI findings in women with myocardial iron?

A

T2 > 20 ms👉don’t require chelation
T2 < 20 ms👉increasing risk of cardiac decompensation
T2< 10 ms👉at high risk of cardiac decompensation

31
Q

What is the optimum management of women at highest risk of cardiac decompensation?

A

Low dose subcutaneous desferrioxamine on a minimum of
4-5 days/ week from 20-24 w

32
Q

What is the optimum management of women with liver iron ?

A

Consider low dose of subcutaneous desferrioxamine from 20 w
🔴If liver iron > 15 mg/ g dw ( MRI)
To reduce the risk of myocardial iron overload.

33
Q

What is the best intrapartum management for women with thalassaemia major or intermedia ?

A

1-No complications ( as diabetes/ FGR/ cardiomyopathy..) 👉aim for vaginal birth. [ inform multidisciplinary team as soon as the woman is admitted]
2- continuous CTG
3- in the presence of ABO antibodies
+ Hb< 10 👉cross match 2 units
In the absence of ABO antibodies
+ Hb< 10 👉 group and save blood
4- in thalassaemia major 👉 IV desferrioxamine 2g/ 24 h in labour
5- Active management for the third stage.

34
Q

What is the major risk in women with thalassaemia post delivery?

A

Venous thromboembolism:
LMWH in hospital and after discharge;
* 7 days post discharge ( vaginal delivery)
* 6 weeks post discharge ( CS )

35
Q

What should be the optimum care regarding breastfeeding?

A

Breastfeeding 👉restart desferrioxamine as soon as the initial 24h IV finishes
Not breastfeed 👉IV or subc desferrioxamine until discharging

8-Thalassaemia In Pregnancy (1 decks)

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