Description

Wali et al described a severity index for evaluating children with sickle cell disease (SCD). This can be used to gauge the overall severity of the illness in a particular patient. The authors are from Sultan Qaboos University in Al-Khod, Muscat, Oman.


Parameters:

(1) number of sickle-related crises per year

(2) number of episodes of acute chest syndrome (ACS)

(3) number of episodes with splenic sequestration

(4) number of chronic complications (avascular necrosis of bone, stroke, cardiomyopathy, multiorgan dysfunction)

 

Parameter

Finding

Points

number of sickle crises per year

less than 1

0

 

1 per year

1

 

2 to 5 per year

2

 

6 or more

3

acute chest syndrome (ACS)

none

0

 

1

2

 

2 or more

3

splenic sequestration

none

0

 

1

2

 

2 or more

3

chronic complications

none

0

 

1

2

 

2 or more

3

 

severity index =

= SUM(points for all 4 parameters)

 

Interpretation:

• minimum index: 0

• maximum index: 12

• The higher the index the more severe the sickle cell disease.


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