Index of van den Tweel et al for Severity of Sickle Cell Disease (SCD) in a Pediatric Patient

Patient selection: pediatric patient with sickle cell disease

The authors listed a number of key clinical and laboratory findings, then compared 3 scores utilizing different weightings for the findings (scores A, B and C). Score C was found to distinguish between patients with mild, moderate and severe disease.

Parameters (total 12 items):

(1) lifetime cumulative incidence (6 items)

(2) number of recurrences in past 2 years (2 items)

(3) laboratory values (4 items)

where:

• Painful crises involve pain in the arms, legs, back, abdomen, chest and/or skull lasting >= 2 hours, leading to hospital admission and due to sickle cell disease.

total score =

= SUM(points for all 12 items)

Interpretation:

• minimum score: 0

• maximum score: 270 (285 according to authors)

• The higher the score the more severe the disease.

• In score C clinical mild disease had scores from 0 to 6, moderate 10 to 23, and severe 8 to 70. In an implementation mild might be 0-6, moderate 7-20 and severe > 20.

Limitations:

• The maximum score listed in the paper differ from summations for Scores A and C.

• Score B adds up to 47 and is reported to have a maximum score of 47.

• Score A adds up to 12 but is reported to have a maximum of 20.

• Score C adds up to 270 but is reported to have a maximum of 285.