Sickle Cell Anemia Patients in the ER

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Dr2007

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I just had a clinical correlations class on sickle cell anemia, and I have a few questions with which you might be able to help me. Do people complaining of severe sickle cell crisis get any kind of priority in the ER? If the sickle cell patient has developed some resistant to the normally prescribed doses of narcotic painkillers is he given the dosage that he knows through experience will alleviate his pain, even if it is 4-5 times the normal amount? Do ER docs sometimes have a tendency to classify sickle cell patients simply as drug seekers? Is there any type of official documentation that a sickle cell patient can carry that will sufficiently prove to ER docs that he really has sickle cell or will diagnostic tests have to be run before any medication is administered? Just wondering if any of you guys have any experience in this area, because these are some of the issues that the patient brought up in class. Thanks.

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Hopefully, If you have been trained somewhere that sees a significant sickle cell population you will learn that these patients need to be treated aggresively and that there really is no way to distinguish the drug seekers from the real crisis patients so everyone gets treated like a sickle crisis until proven otherwise. In answer to your questions:

1. Triage is based on a number of criteria but severe pain can certainly get you bumped up. However, if we are dealling with multiple dieing patients you might still not make the top of the list

2. Most of the sickle patients in my city work with a small group of hematologists at a handful of hospitals. They have files in the the ED with each patient's individualized protocal. So if their protocal says start with 6mg of diluadid than that is what they will get. The protocals are usually fairly detailed including adjuvant meds (antiemetics, benedryl, etc...) escalating doses, baseline Hct, and criteria for admission.

3. If someone says they have SC I tend to treat first and prove it later. I do remember one guy that I actually sent a Hgb electrophoresis on and when it came back negative--like I thought it might-- I cut off his narcs and notified other ED's about his game. He wasn't with any of the hematologists in town and didn't appear to be of the typical racial background. I was sort of pissed 'cause I thought his behaivior would reflect badly on legitimate SC patients

4. Some SC patients do become drug seekers and some ED docs do become overly suspicious but I think it is worth remembering that even if the patients eventually become drug seekers they have a horrible disease which will likely shorten their lives. One of the SC patients in my residency program was notorious for abusing the system, even her hematologist told her she was abusing the system and could not possibly be having crises >320 days out of the year. She was enrolled in inpatient detox programs several times but alway ended up back on the narcs. However, we continued to treat her, follow her protocals, and frequently admit her. One day she died of a combination of pneumonia and sickle crisis in her mid 30's leaving behind a small child. So, I don't think us being all confrontational and abusive about her drug dependence would really have helped very much

5. The patient you talked to is right though if an SC patient goes to a strange ER that doesn't know him and doesn't see many SC patients he isn't likely to get the immediate treatment with adequate doses that he would like to get and should get.
 
Originally posted by ERMudPhud


3. If someone says they have SC I tend to treat first and prove it later. I do remember one guy that I actually sent a Hgb electrophoresis on and when it came back negative--like I thought it might-- I cut off his narcs and notified other ED's about his game. He wasn't with any of the hematologists in town and didn't appear to be of the typical racial background. I was sort of pissed 'cause I thought his behaivior would reflect badly on legitimate SC patients

4. Some SC patients do become drug seekers and some ED docs do become overly suspicious but I think it is worth remembering that even if the patients eventually become drug seekers they have a horrible disease which will likely shorten their lives. One of the SC patients in my residency program was notorious for abusing the system, even her hematologist told her she was abusing the system and could not possibly be having crises >320 days out of the year. She was enrolled in inpatient detox programs several times but alway ended up back on the narcs. However, we continued to treat her, follow her protocals, and frequently admit her. One day she died of a combination of pneumonia and sickle crisis in her mid 30's leaving behind a small child. So, I don't think us being all confrontational and abusive about her drug dependence would really have helped very much


For no. 3, I would have given that pt a hefty dosage of narcan for his trouble. For no. 4, I have to disagree with your prevalence and state that 100% of sickle cell patients become drug seekers. Add together a combination of patients mostly being poverty level patients with nothing to look forward to in their lives due to their disabilty, with a psychological dependence and a fear of being undertreated by physicians, you come out with a full blown addiction problem. I hate the patients who come in with "sickle crisis" after shooting or snorting cocaine. Yes, it's true that cocaine will probably put most sicklers into sickle crisis, but if the pain was truly intractable and unbearable, you'd think that they wouldn't come back to the ER with the exact same problem (cocaine-->SC) in a month. Of course, they usually don't tell you about the cocaine, but I would advise ordering tox screens and peripheral smears on all of your sicklers. I don't know, I've never walked in their shoes so I'm not judging them, I'm sure that if I were in their situation, I'd probably be the same way; but it does get annoying to think that you are feeding an addicts drug problem, and even if they do die of something other then drug abuse, it doesn't mean that drug abuse isn't a problem in their life. Even if you ignore the devastating effects that drug abuse has on individual's lives (the infections, drug seeking behavior and consumption of all of their productive and free time, eventual depression, dangerous lifestyle), you also have to consider the effects that drug addicts have on society (their family/kids, crime, stealing to get the drugs, paying money to support the illegal drug industry that murders). In my opinion, I'd say that even if the patient dies of a SC non drug abuse problem, you can't say that drug abuse wasn't a real problem for them and that treating their drug abuse wouldn't have improved their and people around them's lives.
 
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I had to make an interesting adjustment with sicklers when I moved from med school to residency. I went to med school at a big inner city place in Philly. We had sicklers up to our eyeballs so the system was when they showed up they got three boxes next to their names on the board and started on O2 and IVF. Every time they got a fix of a double D (demerol or dilaudid 'cause they're all "allergic" to anything but) they got a check mark. After three checks they had to leave or be admitted. When I showed up for residency in Sacramento I kept doing it the same way, three hits, in or out. Everyone freaked. "You can't d/c a sickler in crisis! We admit all these guys to Heme/Onc!" I couldn't believe it but that's how they did it. They got away with it because their sickler population is small. You could never do it that way back east.
 
Originally posted by docB
I had to make an interesting adjustment with sicklers when I moved from med school to residency. I went to med school at a big inner city place in Philly. We had sicklers up to our eyeballs so the system was when they showed up they got three boxes next to their names on the board and started on O2 and IVF. Every time they got a fix of a double D (demerol or dilaudid 'cause they're all "allergic" to anything but) they got a check mark. After three checks they had to leave or be admitted. When I showed up for residency in Sacramento I kept doing it the same way, three hits, in or out. Everyone freaked. "You can't d/c a sickler in crisis! We admit all these guys to Heme/Onc!" I couldn't believe it but that's how they did it. They got away with it because their sickler population is small. You could never do it that way back east.

So very true... most of the sicklers in Philly are well known... and if you admitted them all, you'd have a full hospital. I love the "allergies". I start cringing when I hear, "The only thing I can tolerate is dilaudid, and that's 8mg of dilaudid q2h... but don't forget my shot of benadryl!" You gotta love the little extra kick that they get from the benadryl. When did toradol become a common allergy????
 
We had the same 3 shots and you're in rule. (I had my own modification to safe ED time... after the 2nd dose I'd ask the patient how they felt, if they were better they got a 3rd dose and went home, if not they got a 3rd dose and came in...

On another note... sickle cell (or sick as hell) anemia is one disease I am very glad I don't have. In general the patients I have seen (about 1 a shift during my residency) were miserable, though there were some notable exceptions who lead very productive/full lives.

I would caution you about dismissing them as drug "seekers"-- these are patients with a crippling, life shortening, debilitating, painful disease. In the long run which choice do you want to answer for-- giving narcotics to someone who "doesn't need" them, or withholding them from a suffering patient...
 
The national epidemic of anaphylactoid Toradol reactions began when the seekers realized thet there was an IV pain med out there that doesn't get you high.
The people that demand a big D outright are annoying but I really can't stand the ones who act like they can't remember the name. "It's the only thing that works for me... It starts with a 'D.'" "Diltiazem?" I always ask. "No, but it's like that..." At this point I stop them and I have a little speech that goes something like "OK, you and I both know that you want Demerol. Don't act like you don't recall the name. Now I am going to gime you some because there's a chance you are really in pain but this manipulation you've engaged in proves that you have a problem with these drugs. You need to see your doctor, who I'll be calling, to get this straightened out."
This usually shuts up the real seekers and they don't come back when I'm around. The seekers who don't know they're seeking, the regular folks who got addicted because that's the "only thing" that helps their migraines or low back pain, get a real wake up call. HEY, YOU! You're addicted!
 
The whole demerol issue was nicely solved at a few hospitals I worked at in NYC when they just quit using it in the ED. They continued to use other narcs (dilaudid, fentanyl, morphine) but not demerol. A lot of their worst drug seekers went elsewhere but people who seemed to have legitimate pain stayed. There seems to be an extra high associated with demerol that the other narcs don't give-hence everyone's preference for demerol. I've dealt with this by telling patients requesting demerol that I don't give demerol because of the increased risk of seizures. I wish the FDA would give us a black box warning for demerol instead of the one they gave us for inapsine
 
I completely agree with ERMudPhud. I'd be very happy if my hospitals dropped Demerol right off the formulary. I also agree that an FDA "black box" would have been much more appropriate for Demerol than for droperidol.
 
We stopped giving demerol in both the EDs I rotated through. In fact one of the hospitals dropped it hospital wide.
 
I agree... Demerol is a terrible drug (supposedly it was created by the Nazis, though I have been unable to find documentation). It causes a tremendous euphoria, and "Mikey likes it".

Seizures- euphoria, and it apparently doesn't help the pain very much (why else would they need so much??)-- what a great drug.

Another choice you can try in Levo-Dromeran/levorphanol... lasts several hours, typically doesn't make people high, and nobody has heard of it so they're not allergic (of course it is also difficult to find).
 
Interesting thread. A few more thoughts:

I completely agree that Sickle Cell Anemia is a devastating illness I wouldn't wish on my worst enemy. Being a Med-Peds resident I've noticed a different perspective and approach to the disease between my IM and Peds colleagues. Perhaps those of us who have watched acute chest and splenic sequestration evolve in the PICU would triage a Hb-SS patient higher. (One of my favorite peds Heme-Onc staff teaches parents to say "My child has sickle cell and we're going inside now!"). In Sickle Cell there is a degree of survival of the fittest so those with worse disease may never reach adulthood and bother the internists with their addiction issues. I'm not saying addiction doesn't happen, I agree it does; not in 100% of patients but in too many.

As far as Demerol, I admit I'm still a resident but I haven't used it (even in pancreatitis the literature just doesn't support that anymore) as I agree that the risks outweigh the benefits. It's not on formulary at 3 of the hospitals we rotate through. It is at the other two but so are other things that work better. With Hb-SS patients demerol is a particularly poor choice due to the seizure risks especially since these patients already have increased seizure and CVA risk due to their underlying disease.

Compassion is key, I think we always have to meet patients (even addicted patients) where they are. One thing I've learned is that how I deal with children with sickle cell (and other chronic illnesses) can influence whether they grow into adults with drug-seeking and addiction issues. I accept the challenge and my experiences on the medicine side remind me of it's importance.
 
Originally posted by EMRaiden
I agree... Demerol is a terrible drug (supposedly it was created by the Nazis, though I have been unable to find documentation). It causes a tremendous euphoria, and "Mikey likes it".

Seizures- euphoria, and it apparently doesn't help the pain very much (why else would they need so much??)-- what a great drug.

Demerol created by the Nazis? Sounds like urban legend, though I guess anything's possible.

Re: seizures. Has anybody here actually ever seen a seizure from Demerol or even know someone who has? Thanks to some sicklers with allergies to almost everything but Demerol, I've given some pretty hefty doses of the drug sometimes over rather short periods of time. Never seen a seizure, nor has anybody I've ever talked to including the senior partner in our group who grandfathered into board certification ages ago.

Greg Nee, M.D.
 
Like I said, when I was up at the Univ of Maryland rotating through thier ED, the asisstant program director never uses Demerol. I asked their residents, and they said one day he gave them a moderate dose of Demerol and they died within a few minutes. Status Epilepticus? I don't know what htey died of, but anyways, most of the residents there dont' use Demerol (atleast when that attending is on).

Q, DO
 
Originally posted by Sessamoid
Demerol created by the Nazis? Sounds like urban legend, though I guess anything's possible.

Re: seizures. Has anybody here actually ever seen a seizure from Demerol or even know someone who has? Thanks to some sicklers with allergies to almost everything but Demerol, I've given some pretty hefty doses of the drug sometimes over rather short periods of time. Never seen a seizure, nor has anybody I've ever talked to including the senior partner in our group who grandfathered into board certification ages ago.

Greg Nee, M.D.

The seizure risk of demerol actually has to do with build up of a toxic metabolite during CHRONIC not acute use and the risk probably is quite low. When I get a chance I'll dig up a reference for you. That doesn't stop me from using seizures when I explain to patients that I won't be giving them demerol
 
I've heard Demerol amusingly referred to as "a pro-emetic with some analgesic properties." :)
 
Originally posted by ERMudPhud
When I get a chance I'll dig up a reference for you.

1: Hubbard GP, Wolfe KR.
Meperidine misuse in a patient with sphincter of Oddi dysfunction.
Ann Pharmacother. 2003 Apr;37(4):534-7.
PMID: 12659611 [PubMed - indexed for MEDLINE]

2: Hassan H, Bastani B, Gellens M.
Successful treatment of normeperidine neurotoxicity by hemodialysis.
Am J Kidney Dis. 2000 Jan;35(1):146-9.
PMID: 10620557 [PubMed - indexed for MEDLINE]

3: Pryle BJ, Grech H, Stoddart PA, Carson R, O'Mahoney T, Reynolds F.
Toxicity of norpethidine in sickle cell crisis.
BMJ. 1992 Jun 6;304(6840):1478-9. No abstract available.
PMID: 1611370 [PubMed - indexed for MEDLINE]

4: Kyff JV, Rice TL.
Meperidine-associated seizures in a child.
Clin Pharm. 1990 May;9(5):337-8. No abstract available.
PMID: 2350937 [PubMed - indexed for MEDLINE]

5: Goetting MG, Thirman MJ.
Neurotoxicity of meperidine.
Ann Emerg Med. 1985 Oct;14(10):1007-9.
PMID: 4037466 [PubMed - indexed for MEDLINE]

6: Hershey LA.
Meperidine and central neurotoxicity.
Ann Intern Med. 1983 Apr;98(4):548-9. No abstract available.
PMID: 6838077 [PubMed - indexed for MEDLINE]

7: Szeto HH, Inturrisi CE, Houde R, Saal S, Cheigh J, Reidenberg MM.
Accumulation of normeperidine, an active metabolite of meperidine, in patients
with renal failure of cancer.
Ann Intern Med. 1977 Jun;86(6):738-41.
PMID: 869353 [PubMed - indexed for MEDLINE]
 
Thanks for the references. I don't question whether it's happened, just whether it's actually common enough that anybody here has actually seen them. I was looking more for anecdotal rather than statistical experience. :)
 
This is a very interesting thread. The "controversy" around treating sickle cell patients has never really made sense to me. When any patient comes in complaining of sickle cell crisis the first question I always ask is "what usually works to treat your pain?" With the caveat that I never give Demerol, I always give them exactly what they want. For the most part the patients either leave the ED quicker or I determine they need admitting quicker this way than sc***wing around with low doses of meds. For the drug addiction argument I can only reply that a crack dependent sickle cell life must be a sorry life. And if I can ease some of the pain of having to live that sorry life then great. What's the difference between that and easing the chest pain of a fat a** addicted to Krispy Kream 50 y/o who is noncompliant with his BP meds?
 
Originally posted by Sessamoid
Thanks for the references. I don't question whether it's happened, just whether it's actually common enough that anybody here has actually seen them. I was looking more for anecdotal rather than statistical experience. :)

Yup, sounds like you belong in EM alright. ;)
 
I don't know why you all are waiting for demerol to be taken off the formulary. I just tell patients, "I don't prescribe demerol. Would you like to try something else?" Granted, I'm still an intern, but I haven't used Demerol yet.
 
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