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Sickle cell patient policies


saralm88

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Hi everyone!

 

We are looking to put together a sickle cell policy in our institution since we seem to be having more visits from this patient population.  Can I pick everyone's brain about what they do at their facilities?  I use to work at an institution that provided E, C and K negative red cells (unless the phenotype proved otherwise).

 

Thanks!  I am looking forward to reading the responses :)

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We recommend matching the Rh system (CcEe) and K, hemoglobin S negative units; matching Duffy, Kidd, MNS system only if they make an antibody.  (If it becomes too difficult to match exactly we do the best we can, we also attempt to molecular type them and confirm if the patient has the GATA mutation) .  We're a reference lab so many of our hospitals have different policies.  Some just give hemoglobin S negative, a few give C-E-K-HgbS- unless the Rh system is different and a few follow the same policy we have.  The information we've looked at indicates that there is no uniform policy among institutions regarding transfusion of these patients but it is nice to hear what others are doing.

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We recommend ABO compatible, Rh-matched, K- and HbS-.

 

Obviously, if the patient has made a clinically significant alloantibody, we also honour that.

 

We do not match for S-, s-, U- unless or until the patient produces an anti-U, and the same applies for Fy(a-b-), unless or until the patient produces an anti-Fy3.

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We see very few sickle cell patients but do have a policy. They will receive ABO compatible, Rh and K antigen matched and Hgb S negative red cells. And of course we honor clinically significant antibodies. If the reference lab suggests molecular testing for a particular patient, we would follow their recommendation.

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We recommend matching the Rh system (CcEe) and K, hemoglobin S negative units; matching Duffy, Kidd, MNS system only if they make an antibody.  (If it becomes too difficult to match exactly we do the best we can, we also attempt to molecular type them and confirm if the patient has the GATA mutation) .  We're a reference lab so many of our hospitals have different policies.  Some just give hemoglobin S negative, a few give C-E-K-HgbS- unless the Rh system is different and a few follow the same policy we have.  The information we've looked at indicates that there is no uniform policy among institutions regarding transfusion of these patients but it is nice to hear what others are doing.

 

We do the same (free-standing pediatric hospital) as jmm8427, with the exception of the genotyping.  We don't currenlty perform molecular typing in-house, so only select patients are sent out - who knows how this may change in the future!  This usually includes possible Rh variants (allo vs auto), warm auto producers, and some to look for GATA mutation.  We only give Fya neg for patients who type Fy(a-b-) as Malcolm mentioned.  We currently have a database of about 400 sickle cell patients.  Only a percentage are chronically transfused or exchanged, but it is becoming increasingly difficult to obtain fresh, matched units for all of them from our supplier. :wacko:

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We give units negative for HgbS, and we match for Rh and K. If they start making antibodies, we'll also try to match for other antigens if possible (usually Duffy and Kidd).

Does anyone ever give irradiated red cells to sickle patients? All of my hematologists order it, and we have just made it a policy not to give irradiated as it is considered contraindicated (or at least not at all indicated) for sickle patients, due to shortened red cell survival.

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  • 2 weeks later...

My main problem is identifying who is a Sickle cell patient.  If the Hematology department next door (Our hospital is around 200 beds.) doesn't clue me in, I have no way of knowing that I am working with a new Sickle cell patient.  How does everyone else deal with this?  We are getting more Sickle patients every year.  We typically do not see a diagnosis with an order, and have to go out of our way to see an admitting diagnosis, which may only be "anemia."  Right now we are checking with our Hematology department and/or the patient's nurse to let us know.   I would like to set up a protocol to handle this and wonder if anyone else has some guidelines.

 

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chill: We have a required question built into the HIS whenever blood is ordered: Does your patient have sickle cell disease? If the answer is yes, we give C, E, K, and Hgb S negative units (and honor any other antibodies the patient has ever made). We are a community hospital ~200 beds. This information also crosses to our blood bank system automatically to the patient history. We have a similar question for transplant patients. 

 

If it's an error (they answer Yes but it's really No), we can fix it in our blood bank system (we use SoftBank).

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We have the same thing that cthherbal has but for us it's built into the Type and Screen order.

 

The query crosses into the lab module as a comment that appears when you receive the specimen, result the specimen, enter crossmatches and issue blood.

 

We provide Rh-matched, K-negative, Hgb S-neg RBCs as a general rule. For patients who have been known to sensitize we may match other phenotypes as well.

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Similar to cthherbal's response, we also have designed the EPIC electronic blood ordering to ask the question 'Does patient have Sickle Cell Anemia?" when blood is ordered.  the quesion and answer appear on the blood orders in blood bank.

 

Rh, Kell matching for unsensitzed patients

 

Add Jka, Jkb, Fya, S, s matching for a patient with any clin. significant Ab.

 

Add Fyb only for known Anti Fyb or Anti Fy3

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  • 3 weeks later...

We have just started with a difficult sickle cell patient too.  We have very few here and I had tons of questions.  Just found this thread and it answered a lot of them - thanks everyone.

 

But still - a few more questions -

 

1.  His home hospital says he has an Anti-Goa that is still detectable in his serum.  We are coombs crossmatching E neg, C neg, K neg units for him and hope to avoid the Goa as long as his serum keeps a high enough titer.  But, is there anything else we can do to avoid this antigen?  What little I can find on Goa indicates that it is associated with a partial D - is that the case or did I misunderstand everything?  If we use Rh negative units to avoid the C and the E, will we avoid the Goa too???

 

2.  I did not notice that anyone mentioned washing the cells routinely.  His home hospital washes all of their units for their sickle cell transfusions.  Does any one know the justification for washing?  We can't wash here and can't get it from our supplier without getting into unlicensed products (across statelines) so would really like to know if we are going to hurt him.

 

3.  At his home hospital, he actually gets RBC pheresis along with his transfusions.  Does anyone know anything about that?  What it is for, how it helps?  Unfortunately, we can't do that either - no hospital in the region does pheresis for RBCs, though we have one that does plasma pheresis procedures.

 

4.  Am I understanding your comments correctly that if the pt is Fya neg and Fyb neg and forms an Anti Fy3, that you can give Fya neg units and that is suffficient???  Our pt can only form an Anti-Fya since he is positive for Fyb, but I wasn't sure I was getting that  Anti-Fy3 problem correct and I know that a Fya neg, Fyb neg phenotyping is much more common in some populations.

 

Any help with these questions would be deeply appreciated.

Edited by carolyn swickard
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We follow the following transfusion policy for our Sickle patients:

-without alloantibody: give ABO compatible, Rh-and K- matched rbc;

-with alloantibody (ies): give ABO compatible, Rh-, K-, Kidd- and Duffy*- and S-matched rbc.

*A guide from our reference lab states that that it's not necessary to give Fyb antigen neg. rbc based a theory that sickle patients won't develop anti-Fyb due to gene mutation. However, I did see at least two sickle patients developing anti-Fyb so far. Could anybody clarify this?

Is that true more anti-Fy5 than anti-Fy3 is seen in black individuals? Does that mean more anti-Fy5 seen in Sickle patients than anti-Fy3? Thank you.

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Hi cswickard and Clarest,

 

1.  The Goa antigen is quite rare, being expressed on only about 2% of individuals within the Black population, and only then if they have the rare Partial D Category DIVa.  Therefore, you are correct in assuming that you will avoid Goa if you give your patient rr red cells.  However, if he is Ro himself, you can transfuse him with Ro blood, avoiding the C and E antigens, and, in 98% of transfusions, also avoid the Goa antigen.

 

2.  Unless the patient has anti-IgA, I would not have thought that he would require washed red cells (unless they are worried about the small amount of free Hb in the plasma adversely affecting his kidneys).  However, there may be another reason why they are giving washed red cells.  We would need more information.

 

3.  From what you write, it sounds as if he is receiving either partial or full exchange transfusions at his home hospital, and erthropheresis is just an automated method to achieve this.  It allows for a more controlled exchange, as there is little human intervention.

 

4.  The Fy(a-b-) phenotype is seen in about 68% of the Black population, but is also present in Arabs, Jews, Brazilians and Romanies.  It has only ever been found in about 5 individuals in the White population.  Most of the 68% of the Black population have the FYB gene present, but also are homozygous for a GATA-1 mutation upstream of the erythroid promoter region, and, therefore, the Fyb antigen cannot be expressed on their red cells.  However, the Fyb antigen is expressed on tissue other than red cells, and so the immune system does not recognise the Fyb antigen on transfused red cells as "foreign".  They will not, therefore, produce an anti-Fyb, or, come to that, an anti-Fy3 (rare individuals of this type have produced what appears to be an anti-Fy3, but there is a theory that this may be another Duffy specificity that mimics anti-Fy3).

Individuals who do produce an anti-Fy3 usually have the amorphic Duffy genotype of FY/FY, and once they have produced an anti-Fy3, should only be transfused with Fy(a-b-) blood, but this can be either of the GATA-1 type of Fy(a-b-), or the FY/FY type of Fy(a-b-).

 

Although Fy:-5 is more common in the Black population than in the White (by a long way!), most of these individuals are also Fy:-3.  It is till far more common for these individuals to produce an anti-Fy3 than it is for them to produce an anti-Fy5.  Anti-Fy5 is pretty rare.

 

I hope that helps.

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