Sickle cell disease: a natural model of acute and chronic pain

Brandow, Amanda M.; Zappia, Katherine J.; Stucky, Cheryl L.

doi:10.1097/j.pain.0000000000000824

Cited by 56 publications

(36 citation statements)

References 71 publications

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“…This study uniquely focuses on HU's association with evoked and clinical pain to address the knowledge gap about HU and non-VOC pain. 9 Hydroxyurea is indicated for individuals with hemoglobin (Hb) SS and Hb Beta 0 thalassemia, so this study only includes data from participants with these genotypes (n = 58). The Institutional Review Board for Johns Hopkins University approved all study-related procedures, and all participants provided written consent.…”

Section: Methodsmentioning

confidence: 99%

“…2 Positive outcomes associated with HU include reductions in early mortality, 29 acute VOCs, 15 and daily symptom interference. 31 Because few studies have examined HU's impact on non-VOC pain, 9 this study tested the hypotheses that (1) non-VOC pain would be less severe in SCD adults taking HU (HU+) compared with those not taking HU (HU−), and (2) CS, captured by quantitative sensory testing (QST), 5 would be reduced among HU+ individuals.…”

Section: Introductionmentioning

confidence: 99%

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Preliminary evidence that hydroxyurea is associated with attenuated peripheral sensitization in adults with sickle cell disease

Letzen

Lanzkron

Bond

et al. 2019

PR9

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Introduction: Hydroxyurea (HU) is a drug that targets the underlying pathophysiology of sickle cell disease (SCD); however, it continues to be an underutilized treatment for adults. Previous research suggests that HU treatment can result in fewer hospital contacts for acute vaso-occlusive pain crises (VOC). Hydroxyurea's impact on non-VOC pain, however, is not well established. Objectives: This study examined whether HU moderated patterns of static and dynamic pain processing and clinical pain in SCD individuals. Methods: Fifty-eight patients with SCD (N taking HU = 17) underwent quantitative sensory testing (QST) and completed twice daily symptom diaries for 12 weeks. Quantitative sensory testing established thermal threshold and tolerance, mechanical thresholds, and thermal and mechanical temporal summation of pain. Results: Groups did not differ in age, sex, or opioid use. After controlling for morphine use, QST results showed that participants taking HU had higher heat and mechanical pain thresholds (static QST measures) but not thermal and mechanical temporal summation (dynamic QST measures). Participants taking HU also reported lower VOC pain compared with SCD participants not taking HU; however, HU did not moderate non-VOC clinical pain ratings. Conclusion: Findings cautiously suggest that HU acts on pain hypersensitivity and VOC pain, rather than inhibiting pain facilitation and non-VOC pain. These differences may reflect HU's influence on peripheral rather than central sensitization. Future research is warranted to replicate these findings in a larger sample and determine whether early HU administration can prevent peripheral sensitization in SCD individuals.

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Section: Methodsmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Preliminary evidence that hydroxyurea is associated with attenuated peripheral sensitization in adults with sickle cell disease

Letzen

Lanzkron

Bond

et al. 2019

PR9

View full text Add to dashboard Cite

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“…This matter is further complicated because of the subjective nature of pain, and the advent of the opioid epidemic, wherein a perceived over exaggeration of symptoms by patients is often encountered in the EDs in the United States [14]. However, in this specific case, it is believed that the patient developed a hypersensitivity to pain due to changes in afferent neurons in the central nervous system due to repeated pain episodes, which was previously supported by findings in animal studies [15]. Eczematous dermatitis of the patient's extremities with scarring mimicking that of second-degree burns may have physiologically changed mechanoreceptors, nociceptors, and thermoreceptors in the dermis and epidermis of her extremities.…”

Section: Discussionmentioning

confidence: 93%

Mixed Bullous-Eczematous Contact Dermatitis From a Black Henna Tattoo in an African American Female With Sickle Cell Disease With Post-Dermatitis Pain

Persaud-Sharma

Govea

Hernandez

2020

Cureus

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Traditionally practiced in East Asian and Southeast Asian countries, Henna tattooing has gained western popularity in creating temporary decorative patterns on the skin. Derived from the Lawsonia inermis shrub prevalent in Asia/Southeast Asia, the leaves of this plant are ground to create a paste with a brown pigment commonly called Mehndi or Henna which have deep-rooted cultural values/practices. The pure organic form of these compounds has few reported side effects. However, with gaining western popularity, synthetic additives to the natural paste to create color variation, shorten application times, and increase shelf-life have led to an increase in the incidence of adverse reactions. Namely attributed to synthetic compounds like para-phenylenediamine (PPD) or para-toluylenediamine, this synthetic type of mixture is called black henna. Although multiple types of adverse reactions with black henna have been documented as an eczematous type of reaction, few if any cases of adverse reactions of black henna affecting patients with sickle cell disease (SCD) have been documented. In this case, we aim to present an atypical mixed bullous-eczematous contact dermatitis reaction secondary to a PPD containing black henna dye applied to the skin of a patient with homozygous SCD. We intend to raise awareness of the deleterious cosmetic sequelae and chronic post-dermatitis pain manifestations which may arise in patients with SCD, as the popularity of black henna tattooing grows in the United States where SCD is one of the most prevalent hemoglobinopathies amongst black Americans.

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“…A concerning trend demonstrated lower academic mean scores by older age cohorts. Correlational analysis suggests that at least a portion of this academic difficulty is associated with missed school, suggesting that the increase in SCD symptomology over time 42 , combined with the cumulative impact of missed school, leads to a negative cycle of academic underachievement in children with SCD. Results of testing suggested a subset of patients with attention (25%) and executive functioning concerns (18%) and a similar range of patients (20-31%) with parent-reported concerns related to executive functioning.…”

Section: Discussionmentioning

confidence: 99%

Clinical Outcomes of a Neurocognitive Screening Program for Pediatric Sickle Cell Disease

Karst

Miller

Heffelfinger

et al. 2020

Preprint

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Objective: Sickle cell disease (SCD) is associated with neurocognitive and academic impairment. Routine screening is recommended to evaluate for possible concerns. This paper describes results from a neurocognitive screening battery and evaluates relationships with psychosocial and medical variables. Methods: Participants included 61 patients stratified among three age groups, including ages 6-7, ages 11-12, and ages 15-16. Patients completed a screening assessing cognitive performance, academic functioning, and attention (older subjects completed a task of executive function) and a measure assessing HRQL. Caregivers completed a clinical interview and forms evaluating HRQL, executive functioning, and attention. The impact of relevant medical variables and socioeconomic status (SES) was also considered. Results: Between 20% and 33% of participants scored [?] 1 SD below age-based means on intellectual subtests, and between 31 and 48% scored [?] 1 SD below grade-based means on academic subtests. Attention concerns were noted in 25% of patients, while 20-31% of parents reported executive function concerns. Neither disease subtype nor hemoglobin were associated with cognitive or academic functioning. There was an association between mean corpuscular volume (MCV) and both cognitive abilities and psychosocial health. Cognitive scores were correlated with SES, suggesting an impact of socioeconomic disparities on performance. Conclusions: Sickle Cell Disease negatively impacts cognitive and academic functioning both directly and indirectly due to pain, missed school, and through socioeconomic and racial disparities. These concerns may increase over time as disease processes and medication compliance often worsen. Longitudinal research is needed to further explore these trends.

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Sickle cell disease: a natural model of acute and chronic pain

Cited by 56 publications

References 71 publications

Preliminary evidence that hydroxyurea is associated with attenuated peripheral sensitization in adults with sickle cell disease

Preliminary evidence that hydroxyurea is associated with attenuated peripheral sensitization in adults with sickle cell disease

Mixed Bullous-Eczematous Contact Dermatitis From a Black Henna Tattoo in an African American Female With Sickle Cell Disease With Post-Dermatitis Pain

Clinical Outcomes of a Neurocognitive Screening Program for Pediatric Sickle Cell Disease

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