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Sickle cell disease and sickle cell pain mimic other disorders
Abstracts
The Journal of Pain
S11
(140) Dose-response study of topical menthol and capsaicin for evaluation of small peripheral nerve fiber function
(142) Bilateral gustatory paroxysmal jaw pain as a suspected sign of diabetic neuropathy
B Rawson, J Harden, and N Sehgal; University of Wisconsin, Madison, WI
K Wajima, W Muraoka, H Saisu, and H Sato; Keio University, Tokyo, Japan
Neuropathic pain is distinguished from other types of pain in that the thermonociceptive sensory system is affected. Characterization of neuropathic pain is limited by the lack of objective testing. Burning and cooling sensations have distinct anatomic pathways and cellular receptors (TRPV1 and TRPM8 receptors). These receptors can be selectively activated by capsaicin and menthol, respectively. We present an investigator-initiated, double-blinded study examining the effect of applying two distinct concentrations of commercially available capsaicin and menthol in normal subjects. The goal of this study is to exploit the selectivity of the thermo-nociceptive receptors and to establish a concentration-response curve in normal subjects with dermal application of pre-determined concentrations of capsaicin and menthol. Approximately 60 subjects meeting the inclusion criteria will participate in this study. The primary outcome is perceived pain measured quantitatively based on ratings using a mechanical visual analog scale, and qualitatively based on descriptions of evoked sensations. Dermal assessments are also performed throughout the study. Each area of application is examined for perception of sensory modalities including light brush, vibration, pinprick, warm, cool, heat and cold. The intensity of the stimuli is compared to the adjacent skin in order to ascertain any possible hypo- or hyperalgesic effects. We will present preliminary data and results from this ongoing study. Final results from this study will establish whether a single or two concentrations of menthol and capsaicin would need to be applied in patients to assess the functional status of TRPM8 and TRPV1 receptors and associated small peripheral nerve fibers. These results may prove beneficial as an additional diagnostic tool that could be administered in the clinic setting for treating neuropathic pain.
Gustatory sweating is a relatively common sequela to parotidectomy and is almost always unilateral in nature. This article presents cases of bilateral gustatory jaw pain in patients with no history of facial trauma or surgery. An immediate, electric-like, short, bilateral pain was evoked by sweet and tart gustatory stimulation. A thorough examination determined the cause of paroxysmal jaw pain to be autonomic neuropathy as a result of elevated blood-sugar levels due to diabetes mellitus or steroid therapy. The immediate pain that was experimentally induced with sucrose placed on the tongue could not be prevented with carbamazepine. Few cases of this rare complication of diabetes have been described, but there are no reports about cases which are caused by elevated blood-sugar levels due to steroid therapy. . Case 1 A 55-year-old Japanese man presented with a sharp paroxysmal jaw pain in the bilateral lower jaw that developed immediately after tasting sweet or tart substances. He had complicated diabetes with evidence of dysautonomia. Pain quality was electric like and lasted for 5 to 10 minutes. Although various gustatory stimuli, such as drinking orange juice or eating onions, reliably evoked episodes within seconds that lasted for up to 10 minutes. The patient did not report an association of the pain with emotional distress. Many treatments had been tried over the years. The patient reported benefit from tricyclic antidepressants but Pregabalin, Carbamazepine and Selective noradrenaline serotonin reuptake blockers had not been helpful. Case 2 A 62-year-old Japanese women present with same symptoms as above case. She had complex autonomic neuropathy as a result of elevated blood-sugar levels due to steroid therapy for myasthenia gravis. Case 3, 4 They are 20’s Japanese women present with same symptoms as above cases, who are taking steroid for treating SLE, systemic connective tissue disease.
(141) Sickle cell disease and sickle cell pain mimic other disorders
A07 Meta-analysis and Systematic Reviews
S Ballas; Thomas Jefferson University, Philadelphia, PA Sickle Cell Disease (SCD) is an inherited disorder of hemoglobin (Hb) structure where the insoluble deoxy form of sickle Hb causes sickling of red blood cells and vasoocclsion. The phonotypic expression of SCD is so complex that its diagnosis is often missed thus delaying the appropriate therapeutic intervention. This abstract describes three patients where this occurred. The first patient was a 47 year-old African American man who presented with severe low back pain. He never had a similar episode before. X-ray and MRI of the lumbar spine showed osseous changes suggestive of multiple myeloma. Extensive workup showed no abnormalities except for the bone biopsy that showed erythroid hyperplasia. Hematologic work up showed sickle cell beta plus thalassemia with mild anemia and microcytic hypochomic indices. In retrospect he reported having episodes of mild pain in his back and legs. The second patient was a 35 year-old African American woman who presented with fever, severe leukocytosis and generalized lymphadenopathy suggestive of lymphoma. Extensive workup including bilateral lymphangiography and lymph node biopsy was negative for lymphoma. Hematologic work up showed Hb SC disease. Surprisingly she never had painful crises. The third patient was a 26 year-old African American woman who presented with sore throat, fever, left foot weakness, hemolytic anemia and acute renal failure that required hemodialysis. The combination of fever, weakness, renal failure, hemolysis and abnormal peripheral smear made the diagnosis of thrombotic thrombocytopenic purpura and she was started on plasmapheresis. She developed stroke after that due to increased blood viscosity. Hematologic workup revealed a diagnosis of sickle cell anemia. She was started on total blood exchange with significant improvement. In summary patients with mild sickle cell disease in the absence of a history of painful crises are often misdiagnosed causing dangerous delays in initiating appropriate therapy.
(143) Yoga as an intervention for chronic low back pain: a metaanalytic review R Beggs and S Holtzman; University of British Columbia, Okanagan Kelowna, British Columbia The primary objective of the present study was to evaluate the efficacy of yoga as an intervention for chronic low back pain (CLBP) using a meta-analytic approach. Over the past several years, there has been a surge of scientific interest into the possible benefits of yoga as an adjunctive treatment for individuals suffering from CLBP. However, to our knowledge, there has been no prior attempt to quantify the effect sizes of randomized controlled trials (RCTs) of yoga in this population. For the purposes of the current study, a thorough search was conducted in the MEDLINE, PSYCinfo, CINAHL and Global Health databases, as well as the Cochrane Central Register of Controlled Trials, from the time of their inception until April 2011. A total of six RCTs fit our criteria for inclusion, and two outcome measures (functional disability and pain) were selected for analysis. All six RCTs measured functional disability as an outcome variable, and five RCTs measured pain intensity. The methodological quality of each of the included studies was rated independently by the authors. Results of the analysis indicated that yoga had a large effect on functional disability (Cohen’s d = .764, 95% CI = .539 - .988) and a medium-to-large effect on pain intensity (Cohen’s d = .684, 95% CI = .358 - 1.009). Despite a number of methodological differences between the six studies (e.g., outcome measure selection, style of yoga, and treatment duration), only a low to moderate degree of heterogeneity in effect sizes was found between studies. The results of this meta-analysis suggest that yoga may be an efficacious intervention for improving functional disability and pain intensity among CLBP sufferers. Clearly, further research on this topic is warranted. There are a number of methodological concerns that need to be addressed in subsequent trials, and suggestions for future directions will be discussed.
The Journal of Pain
S11
(140) Dose-response study of topical menthol and capsaicin for evaluation of small peripheral nerve fiber function
(142) Bilateral gustatory paroxysmal jaw pain as a suspected sign of diabetic neuropathy
B Rawson, J Harden, and N Sehgal; University of Wisconsin, Madison, WI
K Wajima, W Muraoka, H Saisu, and H Sato; Keio University, Tokyo, Japan
Neuropathic pain is distinguished from other types of pain in that the thermonociceptive sensory system is affected. Characterization of neuropathic pain is limited by the lack of objective testing. Burning and cooling sensations have distinct anatomic pathways and cellular receptors (TRPV1 and TRPM8 receptors). These receptors can be selectively activated by capsaicin and menthol, respectively. We present an investigator-initiated, double-blinded study examining the effect of applying two distinct concentrations of commercially available capsaicin and menthol in normal subjects. The goal of this study is to exploit the selectivity of the thermo-nociceptive receptors and to establish a concentration-response curve in normal subjects with dermal application of pre-determined concentrations of capsaicin and menthol. Approximately 60 subjects meeting the inclusion criteria will participate in this study. The primary outcome is perceived pain measured quantitatively based on ratings using a mechanical visual analog scale, and qualitatively based on descriptions of evoked sensations. Dermal assessments are also performed throughout the study. Each area of application is examined for perception of sensory modalities including light brush, vibration, pinprick, warm, cool, heat and cold. The intensity of the stimuli is compared to the adjacent skin in order to ascertain any possible hypo- or hyperalgesic effects. We will present preliminary data and results from this ongoing study. Final results from this study will establish whether a single or two concentrations of menthol and capsaicin would need to be applied in patients to assess the functional status of TRPM8 and TRPV1 receptors and associated small peripheral nerve fibers. These results may prove beneficial as an additional diagnostic tool that could be administered in the clinic setting for treating neuropathic pain.
Gustatory sweating is a relatively common sequela to parotidectomy and is almost always unilateral in nature. This article presents cases of bilateral gustatory jaw pain in patients with no history of facial trauma or surgery. An immediate, electric-like, short, bilateral pain was evoked by sweet and tart gustatory stimulation. A thorough examination determined the cause of paroxysmal jaw pain to be autonomic neuropathy as a result of elevated blood-sugar levels due to diabetes mellitus or steroid therapy. The immediate pain that was experimentally induced with sucrose placed on the tongue could not be prevented with carbamazepine. Few cases of this rare complication of diabetes have been described, but there are no reports about cases which are caused by elevated blood-sugar levels due to steroid therapy. . Case 1 A 55-year-old Japanese man presented with a sharp paroxysmal jaw pain in the bilateral lower jaw that developed immediately after tasting sweet or tart substances. He had complicated diabetes with evidence of dysautonomia. Pain quality was electric like and lasted for 5 to 10 minutes. Although various gustatory stimuli, such as drinking orange juice or eating onions, reliably evoked episodes within seconds that lasted for up to 10 minutes. The patient did not report an association of the pain with emotional distress. Many treatments had been tried over the years. The patient reported benefit from tricyclic antidepressants but Pregabalin, Carbamazepine and Selective noradrenaline serotonin reuptake blockers had not been helpful. Case 2 A 62-year-old Japanese women present with same symptoms as above case. She had complex autonomic neuropathy as a result of elevated blood-sugar levels due to steroid therapy for myasthenia gravis. Case 3, 4 They are 20’s Japanese women present with same symptoms as above cases, who are taking steroid for treating SLE, systemic connective tissue disease.
(141) Sickle cell disease and sickle cell pain mimic other disorders
A07 Meta-analysis and Systematic Reviews
S Ballas; Thomas Jefferson University, Philadelphia, PA Sickle Cell Disease (SCD) is an inherited disorder of hemoglobin (Hb) structure where the insoluble deoxy form of sickle Hb causes sickling of red blood cells and vasoocclsion. The phonotypic expression of SCD is so complex that its diagnosis is often missed thus delaying the appropriate therapeutic intervention. This abstract describes three patients where this occurred. The first patient was a 47 year-old African American man who presented with severe low back pain. He never had a similar episode before. X-ray and MRI of the lumbar spine showed osseous changes suggestive of multiple myeloma. Extensive workup showed no abnormalities except for the bone biopsy that showed erythroid hyperplasia. Hematologic work up showed sickle cell beta plus thalassemia with mild anemia and microcytic hypochomic indices. In retrospect he reported having episodes of mild pain in his back and legs. The second patient was a 35 year-old African American woman who presented with fever, severe leukocytosis and generalized lymphadenopathy suggestive of lymphoma. Extensive workup including bilateral lymphangiography and lymph node biopsy was negative for lymphoma. Hematologic work up showed Hb SC disease. Surprisingly she never had painful crises. The third patient was a 26 year-old African American woman who presented with sore throat, fever, left foot weakness, hemolytic anemia and acute renal failure that required hemodialysis. The combination of fever, weakness, renal failure, hemolysis and abnormal peripheral smear made the diagnosis of thrombotic thrombocytopenic purpura and she was started on plasmapheresis. She developed stroke after that due to increased blood viscosity. Hematologic workup revealed a diagnosis of sickle cell anemia. She was started on total blood exchange with significant improvement. In summary patients with mild sickle cell disease in the absence of a history of painful crises are often misdiagnosed causing dangerous delays in initiating appropriate therapy.
(143) Yoga as an intervention for chronic low back pain: a metaanalytic review R Beggs and S Holtzman; University of British Columbia, Okanagan Kelowna, British Columbia The primary objective of the present study was to evaluate the efficacy of yoga as an intervention for chronic low back pain (CLBP) using a meta-analytic approach. Over the past several years, there has been a surge of scientific interest into the possible benefits of yoga as an adjunctive treatment for individuals suffering from CLBP. However, to our knowledge, there has been no prior attempt to quantify the effect sizes of randomized controlled trials (RCTs) of yoga in this population. For the purposes of the current study, a thorough search was conducted in the MEDLINE, PSYCinfo, CINAHL and Global Health databases, as well as the Cochrane Central Register of Controlled Trials, from the time of their inception until April 2011. A total of six RCTs fit our criteria for inclusion, and two outcome measures (functional disability and pain) were selected for analysis. All six RCTs measured functional disability as an outcome variable, and five RCTs measured pain intensity. The methodological quality of each of the included studies was rated independently by the authors. Results of the analysis indicated that yoga had a large effect on functional disability (Cohen’s d = .764, 95% CI = .539 - .988) and a medium-to-large effect on pain intensity (Cohen’s d = .684, 95% CI = .358 - 1.009). Despite a number of methodological differences between the six studies (e.g., outcome measure selection, style of yoga, and treatment duration), only a low to moderate degree of heterogeneity in effect sizes was found between studies. The results of this meta-analysis suggest that yoga may be an efficacious intervention for improving functional disability and pain intensity among CLBP sufferers. Clearly, further research on this topic is warranted. There are a number of methodological concerns that need to be addressed in subsequent trials, and suggestions for future directions will be discussed.