Stroke due to Sickle Cell Disease

Need some assistance in the principal diagnosis of a patient who is admitted with a stroke due to sickle cell disease.  The physician noted that the patient is not in sickle cell crisis.  Focus of documentation is on the stroke with hemiparesis.  The patient has had two exchange transfusions.  Is the principal diagnosis the stroke, the acute condition or the chronic sickle cell disease.  

Thanks!  Karen


  • edited January 31
    Do they have Moya Moya?  I do believe our coders code the type of stroke, the Moya Moya and then the hgb ss disease (rarely hgb sc).  also, any significant illness in hgb ss is treated with plasmapheresis to try to remove the sickle cells
  • I would code the stroke as PDX
  • Thanks Val and Jeff!   Appreciate the responses.  And, while I initially agree with Jeff, my coders selected the sickle cell anemia w/o crisis as the principal diagnosis.  As they felt that the stroke was a manifestation of the sickle cell.   And, after another careful review of Coding Clinics, I now agree that sickle cell is the principal diagnosis (not that I agree with coding clinic-but they over rule me). 

    Coding Clinic supports sickle cell anemia as the  principal with acute chest, splenic sequestration, admission due to hemochromatosis due to repeated blood transfusions.  So, it follows that the principal diagnosis should be the sickle cell anemia in patients who have had a stroke due to sickle cell anemia. 

    Thanks, Karen 

  • I agree with the advice from Coding Clinic on the sequencing of those conditions but I am still on the fence about the PDX for a patient with stroke due to sickle cell dz. I think it would be good to write them and get some official advice. Also I would be interested in hearing how other Pedi hospitals would sequence. I am just thinking that sickle cell not in crisis for PDX really isn't representative of the acute condition occasioning the admission.
  • There is a sharp clinical difference between an acute sickle cell event such as an acute chest or splenic sequestration or acute iron overload which would make sense to sequence sickle cell as the pdx.

    Sickle cell with acute chest or splenic sequestration have their own codes anyway. 

    The coding clinic you referred to for Hemochromatosis, was very different.  That patient was IN CRISIS during that admission as well.  Its 4th Q 2010 and the question actually says "the patient was admitted FOR sickle cell crisis' in the body of the question.  VERY VERY different situation there., 

    Is this a Medicare/Medicaid patient?  The problem as I see is is that CMS or its auditors may not accept Sickle Cell without crisis as sufficiently meeting the UHDDS definition of a PDX and while ICD 10 itself may accept Sickle Cell in this scenario (or even coding clinic) CMS and DRG's are a payment system sometimes in conflict with the OCG and Coding clinic.  The diagnosis for CMS has to be the condition which CHIEFLY occasioned  the admission of the patient to the hospital after careful study and I have seen CMS and the Recovery Auditors over rule things in guidelines in favor of the UHDDS.

    If SS without crisis is something we normally do not admit patients for (and we don't) and you sequence that before the acute stroke as something we would routinely admit a patient for (and we do) then you have a conflict on your hands which could potentially manufacture a denial. 

    ICD 10 heavily favors etiology over manifestation (more so than I9) but auditors often favor the acute condition which most closely meets the UHDDS defintion in any case where there is wiggle room and the same is even more true for commercial payers.   
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