SCD With Severe Phenotype (HbSS, HbSβ0 Thalassemia, HbSOARab)
Conditions
Brief summary
There has been little progress for effective treatment of pain in sickle cell disease (SCD) patients. Many organizations have recognized that understanding the causes and reducing the burden of pain in SCD is critical in order to improve the quality of life in SCD patients. As patients with SCD face the challenge of living with both acute and chronic pain which is often improperly treated, our translational and interdisciplinary project aims to identify objective measures of pain sensitivity and its biochemical and genetic correlates. We hypothesize that SCD patients will have decreased tolerance to thermal and electrical stimuli.
Interventions
Sponsors
Study design
Eligibility
Inclusion criteria
* SCD with severe phenotype (HbSS, HbSbeta0 thalassemia, HbSOArab) * Relatives of SCD patients who do not have sickle cell trait or SCD; healthy controls
Exclusion criteria
* Completed overt clinical stroke or transient ischemic attack; * Known severe vasculopathy or Moyamoya disease on brain MRA (Magnetic Resonance Angiography). * history of having consumed alcohol within the last 12 hours prior to testing.
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Measuring thermal responsiveness (perception and tolerance) in the outpatient groups. | change between baseline and at 90day follow-up | Using a TSA (thermal sensory analyzer), the patients hot and cold perception and tolerance will be measured in the outpatient groups (high-pain and low-pain frequency and controls). |
| Measuring thermal responsiveness (perception and tolerance) in the inpatient groups. | change over 8 consecutive days | Using a TSA thermal sensory analyzer, the patients hot and cold perception and tolerance will be measured in the inpatient groups (pain crisis and pain service). |
| Measure mechanical responsiveness in outpatient groups. | change between baseline and 90 day follow-up | Using the Wagner PPIX 50 Pressure device, patient's tolerance to pressure is assessed in the outpatient groups (high-pain and low-pain frequency and controls). |
| Measure mechanical responsiveness in inpatient groups. | change over 8 consecutive days | Using the Wagner PPIX 50 Pressure device, patient's tolerance to pressure is assessed in the inpatient groups (pain crisis and pain service). |
| Measuring the pupil responsiveness in outpatient groups. | change between baseline and 90 day follow-up | Using the Pupillometer device, pupil responses are assessed in the outpatient groups (high-pain and low-pain frequency and controls). |
| Measuring the pupil responsiveness in inpatient groups. | change over 8 consecutive days | Using the Pupillometer device, pupil responses are assessed in the inpatient groups (pain service and pain crisis). |
| Measuring electrical sensitivity in outpatient groups. | change between baseline and at 90day follow-up | Using the Neurometer device, to assess electrical sensory perception and tolerance in the outpatient groups (high-pain and low-pain frequency and control). |
| Measuring electrical sensitivity in inpatient groups. | change over 8 consecutive days | Using the Neurometer device, to assess electrical sensory perception and tolerance in the outpatient groups (pain service and pain crisis). |
Countries
United States