SICKLE CELL DISEASE Flashcards
Types of crises in SCD
Vaso-occlusive bone pain crisis
Acute chest syndrome
Hyper-haemolytic crisis
Aplastic crisis
Sequestration crises
Vaso-occlusive crisis
occlusion of small vessels by sickled cells
causing infarction and pain
Acute chest syndrome
patient presents with sudden onset of cough, difficulty in breathing and fever
Aplastic crisis
infection with parvovirus causes sudden severe anaemia with a low reticulocyte count
Precipitating factors in sickle cell crisis
cold weather
dehydration
infection
physical exertion
mental stress
Aplastic crisis
The spleen and liver enlarge rapidly due to trapping of red blood cell
Symptoms of SCD
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
Signs in SCD
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)
Skull bossing
An enlarged forehead
The result of extended hematopoietic bone marrow, causing widening of the medullary space, thinning of the trabeculae and cortices, and osteoporosis
Gnathopathy
Sickle cell gnathopathy is an unusual maxillary protrusion considered as the result of a bone marrow hyperplasia induced by chronic haemolytic anemia
Investigations in SCD
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
Treatment objectives in SCD
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
Non-pharmacological interventions in SCD
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
IV Fluids in vasoocclusive crisis
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
When is blood transfusion necessary in vasoocclusive crisis
Transfusion will be necessary if haemoglobin level is < 5 g/dL
First line Treatment for vasoocclusive crisis
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.
Second line treatment for severe pain in vasoocclusive crisis
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
Paracetamol dose for pain in vasoocclusive crisis
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
Dose of diclofenac for pain in vasoocclusive crisis
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
Dose for codein phosphate for pain in vasoocclusive crisis
Adults and Children over 12 years
30-60 mg 4-6 hourly
Dose for ibuprofen for pain in vasoocclusive crisis
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
Dose of IM Diclofenac for pain in vasoocclusive crisis
Adults
75-150 mg daily in divided doses
Treatment of severe disease in SCD
Amoxicillin + Clavulanic Acid, IV, 1.2 g and urgently transferred to a tertiary centre
Hydroxycarbamide
Aspirin
Opiates
Chronic transfusion therapy
Role of folic acid in SCD and dose
To maintain steady state Hb
Adults
5 mg daily
Children
> 1 year; 5 mg daily
< 1 year; 2.5 mg daily
Dose for tramadol for pain in vasoocclusive crisis
Tramadol, oral, 50-100 mg 8 hourly
Fluid requirements according to age in SCD
<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
Prevention of pneumococcal infections in SCD
Pneumococcal conjugate vaccine 13 (PCV 13) vaccination in infancy, booster at 6 years
