Thalassemia and sickle cell anemia – Maternal medicine Module part2

Thalassemia and sickle cell anemia questions- Maternal medicine Module part2

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A 34 year old woman with sickle cell disease attends for pre-conception counseling. To assess
her for chronic complications

A) Liver function tests should be done if not undertaken in the last 3 months

B) Renal function tests should be done if not undertaken in the last 12 months

C) Screening for pulmonary hypertension should be done using ECG

D) Screening for iron overload is done using serum ferritin and transferrin levels

E) Iron chelation is not recommended before conception

A 24-year-old woman with sickle cell disease is admitted forinduction of labour at 38 weeks gestation in her first pregnancy
that is otherwise uncomplicated. Three hours after commencement
of intravenous oxytocin, her oxygen saturation drops to
93%. What is the most appropriate immediate management?

A) Administer oxygen therapy and check arterial blood gases

B) Administer oxygen therapy and intravenous antibiotics

C) Category 1 caesarean section with general anaesthesia (GA)

D) Category 2 caesarean section with regional analgesia

E) Stop the oxytocin infusion

A woman with known sickle cell trait attends an antenatal booking clinic, What antenatal
complication is significantly more common than uncomplicated pregnancies?

A) Chest infection

B) Intrauterine growth restriction

C) Major postpartum hemorrhage

D) Placental abruption

E) Urinary tract infection

5) All are true about hemoglobinopathies except

A) If a couple both carry sickle cell trait, there is a 1:4 chance that their child will be affected by sickle cell disease

B) It is safe to use oral iron supplementation in alpha-thalassaemia trait

C) There is an increased risk of thromboembolic disease in a woman with sickle cell disease

D) There is an increased risk of fetal growth restriction with sickle cell trait

E) Women with HbSC are at risk from a sickle cell crisis in pregnancy

A patient with beta thalassemia , known diabetic seen in preconception clinic. How we monitor
her diabetes in pregnancy?

A) FBS

B) HbA1C

C) peptide levels

D) Serum fructosamine

E) Serum glucose

7) Regarding anemia in pregnancy

A) Haemoglobin should be maintained atleast above 80g/dl in thalassemia major

B) Haemoglobin of 70g/dl is maintained without transfusion in thalassemia intermedia

C) Haemoglobin upto 60g/dl is well tolerated in sickle cell disease

D) Levels below 15 μg/l are diagnostic of established iron deficiency and level below 30 μg/l in pregnancy should prompt treatment in non anaemic but at risk of developing anaemia

E) Women with known haemoglobinopathy should have serum ferritin checked and offered oral supplements if their ferritin level is <30 μg/l

Pregnant patient in second trimester with thalassemia major, did not come for preconceptional
counselling. On liver ferriscan was found to have high iron overload.

A) Cardiac MRI

B) Desferrioxamine chelation therapy

C) LMWH

D) LMWH + Aspirin

E) LDA

F) Deferasirox chelation

A 22-year-old Afro-Caribbean woman who is 30 weeks pregnant with known sickle cell disease
presents via A&E with a two-day history of progressively worsening cough, fever, excruciating
bilateral chest pain and shortness of breath. This is her first pregnancy and it has been uneventful so
far. She has had serial scans from 24 weeks of gestation, which have been normal.
Her observations are a blood pressure of 100/60 mmHg and pulse rate of 118 bpm. Her respiratory
rate is regular at 32 per minute and temperature is 38.2°C. Oxygen saturation is 84% on air, chest
auscultation reveals mild bilateral basal crepitations but no wheeze. There is no calf tenderness.

Investigations:
• white blood cells = 19.8
• haemoglobin = 93 g/l
• platelets = 325

What would be the most appropriate treatment for her?
• reticulocytes = 16.2%
• ESR = 35
• CRP = 80
• chest X-ray: PA view - pulmonary infiltrates were seen bilaterally
• VQ scan normal
• ECG sinus rhythm, 118 bpm

A) Oxygen, analgesia, broad spectrum antibiotics, prophylactic low molecular weight heparin

B) Oxygen, analgesia, broad spectrum antibiotics, prophylactic low molecular weight heparin, exchange transfusion

C) Oxygen, analgesia, salbutamol nebulisers, oral steroids, prophylactic low molecular weight heparin

D) Oxygen, analgesia, therapeutic low molecular weight heparin

E) Oxygen, analgesia, therapeutic unfractionated heparin

10) Which infection is known to produce an aplastic crisis in people with sickle cell disease?

A) Cytomegalovirus

B) Herpes virus

C) Erythrovirus

D) Parvovirus

E) Rubella virus

11) All contraception comes under Category 1 for sickle cell disease except;

A) Progesterone onluy pills

B) Depo-provera

C) Emergency contraception

D) Cu IUD

E) LNG-IUS

12)

Pregnant patient with thalassemia major who had history of splenectomy. Her platelet count is
660x10*9
Regarding her thromboprophylaxis all are true except;

A) She has prothrombotic tendancy due to presence of red cell fragments as she has undergone spleenectomy

B) She should be prescribed only aspirin as thromboprophylaxis

C) She should be prescribed LMWH and aspirin for thromboprophylaxis

D) The risk of thromboembolism is highest in thalassemia intermedia who don't receive blood transfusions

E) Good transfusion regime is protective against thromboembolism as it supresses endogenous erythropoesis

13) All are true about hemoglobinopathies except

A) There are two pairs of globin chains in each haemoglobin molecule

B) There is no prenatal diagnosis available for sickle cell disease

C) Iron deficiency anaemia and thalassaemia trait cannot co-exist

D) The most accurate screening blood indices for thalassaemia trait is the mean corpuscular haemoglobin (MCH

E) If two defective alpha-globin chains are present this is compatible with a diagnosis of alphathalassaemia trait

14)

. Pregnant patient with thalassemia major who had history of splenectomy. Her platelet count
is > 600x 10*

A) Cardiac MRI

B) Desferrioxamine chelation therapy

C) LMWH

D) LMWH + Aspirin

E) LDA

F) Deferasirox chelation

15)

Pregnant women booking blood shows Hb 98gm/l decreased MCH,decreased MCV , serum
ferritin 50 (normal)

A) B Thalassemia minor

B) Iron deficiency anaemia

C) sickle cell anemia

D) B thalassemia major

The midwife on the postnatal ward asks you to make a thromboprophylaxis plan for a 21 yr old
woman who has underwent ceserean section for intrauterine growth restrictions. She is having
sickle cell disease and no other risk factors.

16) Which of the following is a correct plan for her?

A) LMWH while in hospital

B) LMWH while in hospital and 7 days after discharg

C) LMWH while in hospital and upto 6 weeks

D) UFH while in hospital

E) UFH while in hospital and 7 days post discharge

19)

23 yr old woman with sickle cell disease is admitted to emergency dept with painful crisis.
Which analgesia should be avoided?

A) Morphine

B) NSAIDs

C) Dihydrocodeine

D) Co-codamol

E) Pethidine

A couple are anxious about the risk of their baby being born with an inherited autosomal
recessive condition. Their anxiety stems from the fact that their relative recently had a baby with an
autosomal recessive condition. They want to know what the most common autosomal recessive
condition worldwide is.

20) What do you tell them?

A) α-thalassaemia

B) β-thalassaemia

C) Cystic fibrosis

D) Haemophilia E. Sickle cell disease

E) Sickle cell disease

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