Sickle Trait info for ED physicians?

[GeneralVeers]

I'm an ED physician in Nevada. At my place of practice we have a ton of "sickle crisis" patients who seem to get admitted almost weekly for tons of IV narcotics and a vacation from life. While I'd like to help out the true homozygous sickle disease patients, I want to weed out the sickle trait people who are likely abusing the system.

I've started sending Protein S electrophoresis on these patients, in the hope that there will be evidence as to whether or not they are truly sickle disease to help treat them appropriately on their inevitable next visit. The last two patients had Hemoglobin S fractions of 32 and 41 respectively. My understanding is that sickle trait usually has Hemoglobin S levels of < 45 and full Sickle Disease is around 98.

I just wanted some additional help with this issue, so any guidance is appreciated.

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[whoknows2012]

I'm an ED physician in Nevada. At my place of practice we have a ton of "sickle crisis" patients who seem to get admitted almost weekly for tons of IV narcotics and a vacation from life. While I'd like to help out the true homozygous sickle disease patients, I want to weed out the sickle trait people who are likely abusing the system.

I've started sending Protein S electrophoresis on these patients, in the hope that there will be evidence as to whether or not they are truly sickle disease to help treat them appropriately on their inevitable next visit. The last two patients had Hemoglobin S fractions of 32 and 41 respectively. My understanding is that sickle trait usually has Hemoglobin S levels of < 45 and full Sickle Disease is around 98.

I just wanted some additional help with this issue, so any guidance is appreciated.

The reality is a lot more complicated than you’ve laid out. First of all, sickle trait patients can get sickle pain crises. There are hosts of other hbS/x hemoglobinopathies as well which can lead to lower threshold for sickle/pain crises (sc, s beta thal etc.) trying to get a “number” on the percent of hbS to know whether or not the patient is truly having a crises is not in my assessment a good indicator. Indicators that are specific would be tachycardia, (from severe pain), elevated wbc count, other evidence of end organ damage suggestive of previous sickling (avn, pulm htn etc) direct hematologic evidence of sickling/hemolysis and marrow recover(smear, ldh hapto bilis retic count haptoglobin). Unfortunately you may have to accept that it’s nearly impossible to tell who truly has pain and who doesn’t besides using these indirect measures above

 

[thisampgoestoeleven]

It's not your job to adjudicate sickle cell diagnosis. Hb SC and other variants (sickle thal, sickle LA) - can go undiagnosed with your method. Hemolysis is not always present either in vaso occlusive crisis. Ask them if they have a hematologist and you can call them if you want to know. Check your privilege. Sickle is no vacation, and many of the patients die young with pulmonary hypertension. Slim chance of cure with transplant.

 

[whoknows2012]

AND the risk of improperly treating their pain could lead to splinting more hypoxia acs and eventual poor outcomes. I also agree it’s not your job to be making the call on who is having a “legitimate” sickle crises.

 

[GeneralVeers]

AND the risk of improperly treating their pain could lead to splinting more hypoxia acs and eventual poor outcomes. I also agree it’s not your job to be making the call on who is having a “legitimate” sickle crises.

Thanks for the input everyone! I'm always just dismayed, because the inpatient teams never seem to send the electrophoresis, and the rare hematology consults I see don't make mention of reviewing these results when they exist. Often it's not the patient's hematologist seeing them in the hospital (we have terrible outpatient specialties in Las Vegas).

Generally I use subjective measures. If they have no vital signs, are happy, smiling and chatting on the cell-phone they don't need IV dilaudid or admission. Obviously severe anemia, new white count or evidence of infection gets admitted. I try to use PO narcotics when possible especially if no outward signs of extreme pain.

Also what is the incidence of continual pain crises that require admission every week? Is this legitimately a common presentation?

 

[johnnyb255]

Thanks for the input everyone! I'm always just dismayed, because the inpatient teams never seem to send the electrophoresis, and the rare hematology consults I see don't make mention of reviewing these results when they exist.

There is no role for repeated haemoglobin studies other than potentially monitoring HbF levels for hydroxyurea (hydroxycarbamide) therapy compliance.

Some resources-

Glassberg JA. Improving Emergency Department-Based Care of Sickle Cell Pain. Hematology Am Soc Hematol Educ Program. 2017;2017(1):412–417. doi:10.1182/asheducation-2017.1.412

Only skimmed it but it's from the ASH Education Book so always a good read. Otherwise this blog entry seems relevant as well (and referenced)-

ED Management of Sickle Cell Vaso-occlusive Crises: Myths, Facts, and A Novel Approach to Acute Pain Management

EDIT: Just realised the Dr Glassberg assisted with the blog entry so is reputable. I also realised I've missed the point of the OP- trait patients having pain crisis. As mentioned, there are compound heterozygous sickling disorders.