SICKLE CELL DISEASE

Question 1

Types of crises in SCD

Answer 1

Vaso-occlusive bone pain crisis
Acute chest syndrome
Hyper-haemolytic crisis
Aplastic crisis
Sequestration crises

Question 2

Vaso-occlusive crisis

Answer 2

occlusion of small vessels by sickled cells
causing infarction and pain

Question 3

Acute chest syndrome

Answer 3

patient presents with sudden onset of cough, difficulty in breathing and fever

Question 4

Aplastic crisis

Answer 4

infection with parvovirus causes sudden severe anaemia with a low reticulocyte count

Question 5

Precipitating factors in sickle cell crisis

Answer 5

cold weather
dehydration
infection
physical exertion
mental stress

Question 5

Aplastic crisis

Answer 5

The spleen and liver enlarge rapidly due to trapping of red blood cell

Question 6

Symptoms of SCD

Answer 6

Joint and bone pain, especially during cold wet seasons
Easy fatiguability
Pallor
Jaundice
Difficulty in breathing with or without chest pain
Chronic leg ulcer
Abdominal pain, especially in the splenic area
Spontaneous sustained erection without sexual arousal in male patients

Question 7

Signs in SCD

Answer 7

Jaundice
Pallor
Hepatomegaly
Splenomegaly (may be absent in older patients)
Old or recent scarification marks by traditional healers, particularly
over the abdominal wall and joints
Venous ulcers
Skull Bossing
Dactylitis (hand and foot syndrome)
Gnathopathy
Growth delay or tall, lanky stature (‘marfanoid’ habitus)

Question 8

Skull bossing

Answer 8

An enlarged forehead
The result of extended hematopoietic bone marrow, causing widening of the medullary space, thinning of the trabeculae and cortices, and osteoporosis

Question 8

Gnathopathy

Answer 8

Sickle cell gnathopathy is an unusual maxillary protrusion considered as the result of a bone marrow hyperplasia induced by chronic haemolytic anemia

Question 9

Investigations in SCD

Answer 9

FBC
Blood film comment
Reticulocyte count
Sickling test
Haemoglobin electrophoresis
Urine examination
Chest X-ray in case of ACS
Blood and urine C/S when infection suspected
G6PD assay

Question 10

Treatment objectives in SCD

Answer 10

To prevent the development of sickle cell crises
To relieve pain
To identify and manage the precipitating cause of crises
To maintain a good steady state haemoglobin
To prevent long term complications and organ damage
To manage sickle cell crises and complications once developed

Question 11

Non-pharmacological interventions in SCD

Answer 11

Good hydration at all times by drinking adequate water/fluids
Avoidance of common precipitating causes of crises such as malaria (bed nets etc.), dehydration, stress, excessive exercise, and exposure to extremes of weather
Maintenance of good nutrition
Client education
Parental/guardian education
Genetic counselling with voluntary family size restriction
General public knowledge

Question 12

IV Fluids in vasoocclusive crisis

Answer 12

Adults
5% Dextrose in 0.9% Sodium Chloride 2-4 L daily
or
Normal Saline alternating with 5% Dextrose 2-4L daily

Children
5% Dextrose in 0.9% Sodium Chloride 150 ml/kg daily
or
Normal Saline alternating with 5% Dextrose 150 ml/kg daily

Question 13

When is blood transfusion necessary in vasoocclusive crisis

Answer 13

Transfusion will be necessary if haemoglobin level is < 5 g/dL

Question 13

First line Treatment for vasoocclusive crisis

Answer 13

Paracetamol PO/Recta or Ibuprofen PO or Diclofenac PO/Rectal
and
Morphine sulphate PO/IV
and
IV Fluids
and
Blood transfusion (packed cells) when needed, but not routine.

Question 13

Second line treatment for severe pain in vasoocclusive crisis

Answer 13

Diclofenac sodium, IM,
Adults
75-150 mg daily in divided doses

Then

Codeine phosphate, oral,
Adults and Children over 12 years
30-60 mg 4-6 hourly
Or
Tramadol, oral, 50-100 mg 8 hourly

Question 14

Paracetamol dose for pain in vasoocclusive crisis

Answer 14

Paracetamol, oral,
Adults
500 mg-1 g 6 - 8 hourly
Children
6-12 years; 250-500 mg 6-8 hourly
1-5 years; 120-250 mg 6-8 hourly
3 months-1 year; 60-120 mg 6-8 hourly
Or
Paracetamol, rectal,
Adults and Children
Doses as above

Question 14

Dose of diclofenac for pain in vasoocclusive crisis

Answer 14

Diclofenac, oral,
Adults
50 mg 8 hourly or 100 mg 12 hourly
Children
> 12 years; 50 mg 12 hourly
< 12 years; not recommended
Or
Diclofenac, rectal,
Adults
100 mg daily up to a maximum of 200 mg daily in divided doses
Children
75-100 mg daily

Question 15

Dose for codein phosphate for pain in vasoocclusive crisis

Answer 15

Adults and Children over 12 years
30-60 mg 4-6 hourly

Question 15

Dose for ibuprofen for pain in vasoocclusive crisis

Answer 15

Ibuprofen, oral,
Adults
400 mg 6-8 hourly
Children
6-12 years; 200-400 mg 6-8 hourly
1-5 years; 100-200 mg 6-8 hourly
3 months-1 year; not recommended

Question 15

Dose of IM Diclofenac for pain in vasoocclusive crisis

Answer 15

Adults
75-150 mg daily in divided doses

Question 16

Treatment of severe disease in SCD

Answer 16

Amoxicillin + Clavulanic Acid, IV, 1.2 g and urgently transferred to a tertiary centre
Hydroxycarbamide
Aspirin
Opiates
Chronic transfusion therapy

Question 16

Role of folic acid in SCD and dose

Answer 16

To maintain steady state Hb
Adults
5 mg daily
Children
> 1 year; 5 mg daily
< 1 year; 2.5 mg daily

Question 16

Dose for tramadol for pain in vasoocclusive crisis

Answer 16

Tramadol, oral, 50-100 mg 8 hourly

Question 16

Fluid requirements according to age in SCD

Answer 16

<1 year 50 ml/kg
1 year 140 ml/kg
2 years 130 ml/kg
4 years 110 ml/kg
6 years 100 ml/kg
8 years 90 ml/kg
10 years 85 ml/kg
12 years 70 ml/kg

Question 17

Prevention of pneumococcal infections in SCD

Answer 17

Pneumococcal conjugate vaccine 13 (PCV 13) vaccination in infancy, booster at 6 years