Man With Severe Joint Pain and Normal Hemogram

A 32-year-old man presents to the emergency department(ED) with generalized joint pain of several days'duration, which he believes is a sickle cell crisis similarto others he has had. He says that 4 to 6 mg of oralhydromorphone usually relieves the pain of these crises,but he recently ran out of his medication and is unableto contact his primary care physician because she ison vacation. He frequently interrupts to ask for a 6-mghydromorphone injection.HISTORY
The patient gives a history of sickle cell anemia. Hesays he typically has a painful crisis every 1 to 2 monthsand names several local hospitals where he was recentlytreated. His records indicate that he has been treatedfor painful crises in this ED approximately 20 times in thepast 3 years; however, he was never admitted. He has nohistory of stroke, visual changes, priapism, acute chestsyndrome, leg ulcers, cholelithiasis, or cholecystectomy.He smokes half a pack of cigarettes daily; he denies useof alcohol or illicit drugs.PHYSICAL EXAMINATION
Initially, the patient is lying in the fetal position onthe stretcher, gripping and rubbing his knees and elbowsand moaning. Temperature is 37.7C (99.9F); heart rate,100 beats per minute; respiration rate, 20 breaths perminute; blood pressure, 106/78 mm Hg; and oxygen saturation,97% on room air. There is no icterus or conjunctivalpallor. Lungs are clear. Abdomen is nontender with normalbowel sounds, and there is no hepatosplenomegaly.Joints are grossly tender on palpation, but no effusions,warmth, or skeletal abnormalities are noted. LABORATORY RESULTS
No previous laboratory data are readily available.The patient is given intravenous narcotics, which providemoderate relief of his pain. Meanwhile, a complete bloodcell (CBC) count and hemoglobin electrophoresis are ordered.However, the patient leaves before results becomeavailable; he is given a prescription for hydromorphoneat discharge.The hemoglobin level, hematocrit, red blood cellcount, mean corpuscular volume, and mean corpuscularhemoglobin content are within the normal range. A peripheralblood smear reveals normochromic and normocyticred blood cells without sickle cells; white blood cellsand platelets are normal in number and morphology. Hemoglobinelectrophoresis (results of which are availableseveral days later) shows 2 spikes: 1 in the hemoglobinA region and 1 in the hemoglobin S region-in a 60:40ratio, respectively.Which of the following is most likely in this patient?A. He is malingering to obtain drugs.B. He has homozygous sickle cell disease in crisis.C. He has septic arthritis.D. He is experiencing narcotic withdrawal.CORRECT ANSWER: A
This patient's story at first seems consistent with a vasoocclusivecrisis of sickle cell anemia (choice B). However,the history and physical examination provide clues that hemay not have sickle cell anemia. The frequency of his painepisodes suggests severe disease, yet he has no record ofadmission and has had no serious complications (eg,acute chest syndrome, cerebrovascular accident, priapism).Most patients with sickle cell anemia must undergocholecystectomy by young adulthood because of the effectsof frequent hemolysis, but this patient has never hadcholelithiasis. He has no icteric changes, which often resultfrom hemolysis, or conjunctival pallor, which might beexpected with severe anemia. Finally, the patient frequentlyinterrupts the physician to ask for narcotics.The laboratoryresults arealso inconsistentwith sickle cellanemia. TheCBC count is normal;the peripheralblood smear isalso normal, withoutsickle cells.Hemoglobin levelis 13.5 g/dL,whereas thisvalue in patientswith sickle cellanemia is typicallybetween 6 and9 g/dL. The hemoglobin electrophoresis results confirm thepresence of sickle cell trait (Hb S/A:40/60), which is notassociated with painful crises. However, sickle cell trait willresult in a positive finding on a sickle prep test, which thepatient may have used to justify his "sickle cell" diagnosis.This man is most likely malingering to obtaindrugs (choice A). Malingering is a common problem inboth inpatient and outpatient settings. According to theDiagnostic and Statistical Manual of Mental Disorders(fourth edition), the essential feature of malingering is the"intentional production of false or grossly exaggeratedphysical or psychological symptoms, motivated by externalincentives such as avoiding military duty, avoidingwork, obtaining financial compensation, evading criminalprosecution, or obtaining drugs."¹ The exact incidence isunknown. Strongly suspect malingering when:

• There is a lack of objective findings consistent with thediagnosis.
• The patient is not cooperative during evaluation and/ortreatment.
• The patient suffers from antisocial personality disorder.Such patients account for more than half of "habituallywandering patients"- those who drift from hospital tohospital.²

Treatment of a malingering patient can be difficult.The best approach is to be objective, order appropriatetests if necessary, and avoid confronting the patient ordemonstrating anger toward him or her (which can resultin termination of the relationship or further deception).Through such an approach, you may gain insight into thepatient's motives and thus may be able to provide appropriateintervention.Septic arthritis (choice C) is not typically polyarticular.Moreover, it is usually characterized by effusions,physical findings of intense inflammation in affected joints,and such systemic signs as fever and leukocytosis. Noneof these findings are present here.Similarly, none of the typical physiologic findings ofnarcotic withdrawal (choice D)- watery eyes, rhinorrhea,mydriasis, tremor- are present. The patient may indeedbe addicted and have drug cravings, but he is not manifestingphysiologic withdrawal.

References:

REFERENCES:

1.

Task Force on DSM-IV,

American Psychiatric Association. Diagnostic and StatisticalManual of Mental Disorders.

4th ed. Washington, DC: American Psychiatric Press; 1994.

2.

Pankartz L, Jackson J. The habitually wandering patient.

N Engl J Med.

1994;331:1752-1755.

FOR MORE INFORMATION:

Kaplan HI, Sadock BJ.

Kaplan and Sadock’s Synopsis of Psychiatry: BehavioralSciences/Clinical Psychiatry.

8th ed. Philadelphia: Lippincott Williams & Wilkins;1998.