Cutaneous Hydration Assessment in SCD

This study will validate the diagnostic accuracy of a cutaneous hydration sensor. This sensor will also be evaluated for its feasibility as a point-of-care device for the assessment of hydration status and its potential to guide hydration therapy in patients with sickle cell disease (SCD).

Vaso-occlusive episodes (VOE) are the leading cause of hospitalization for patients with SCD. Intravenous fluid replacement is one of the cornerstones of management of VOE in the emergency department and throughout hospitalization. However, there are no evidence-based guidelines specifying the optimal administration of maintenance fluids. Overly aggressive hydration therapy imparts the risk of hypervolemia and pulmonary edema, which may lead to acute chest syndrome and death. Thus, a reliable biomarker is needed to gauge hydration status and guide fluid replacement strategies with the goal of achieving euvolemia. The investigators propose a point-of-care test that may inform management (e.g., bolus vs. continuous infusion of maintenance intravenous fluid), and prevent over- or under-hydration. For this purpose, investigators seek to validate the diagnostic accuracy of a cutaneous hydration sensor, Delfin MoistureMeterEpiD (a non-significant risk device) and evaluate its feasibility as a point-of-care device for the assessment of hydration status and potentially guide hydration therapy in patients with SCD. Investigators will measure skin hydration in the clinic when participants are at baseline state of health. Skin hydration before and after fluid resuscitation therapy in patients with vaso-occlusive crisis (VOC) or VOE will also be assessed. Blood and urine will be collected to compare assessments of skin hydration with laboratory biomarkers of hypertonicity and red blood cell dehydration.

The device is a skin hydration sensor. The sensor is placed on the skin at the inner arm. It is non-invasive and measures in seconds the percent water content of the dermis by quantifying its dielectric constant.

Delfin MoistureMeterEpiD hydration sensor will be used to measure dermal water content in 20 participants at 1 time point.

Delfin MoistureMeterEpiD hydration sensor will be used to measure dermal water content in 10 participants.

Delfin MoistureMeterEpiD hydration sensor will be used to measure dermal water content in 10 participants.

Clinical assessment of dehydration will be ascertained by administering the 10-point Clinical Dehydration Scale (CDS). CDS uses clinical characteristics (general appearance, eyes, mucous membranes, and tears), each of which are scored 0, 1, or 2 for a total score of 0 to 8, with 0 representing no dehydration; 1 to 4, some dehydration; and 5 to 8, moderate/severe dehydration. It will be administered at 1 time point in 20 participants.

Serum osmolality values of >290 milliosmol/kg is considered abnormal. It will be measured at 1 timepoint in 20 participants.

As a marker of hyperadhesion capillary transit time will be measured using capillary mimicking microfluidic channels. It will be measured at 1 timepoint in 20 participants.

Cellular dehydration marker, the elongation index, will be measured by ektacytometry. It will be measured at 1 timepoint in 20 participants.

Point of sickling, a cellular dehydration biomarker, will be measured by ektacytometry. It will be measured at 1 timepoint in 20 participants.

Urine osmolality values <450 milliosmol/kg is considered abnormal in adults without fluid restrictions. It will be measured at 1 timepoint in 20 participants.

Measurement of serum osmolality as a laboratory biomarker of dehydration before resuscitation therapy in SCD participants with VOC or VOE

Serum osmolality values of >290 milliosmol/kg is considered abnormal. It will be measured at 1 timepoint in 10 participants.

Measurement of serum osmolality as a laboratory biomarker of dehydration after fluid resuscitation therapy in SCD participants with VOC or VOE

Serum osmolality values of >290 milliosmol/kg is considered abnormal. It will be measured at 1 timepoint in 10 participants.

Measurement of hyperadhesion as a laboratory biomarker of dehydration before fluid resuscitation therapy in SCD participants with VOC or VOE

As a marker of hyperadhesion, capillary transit time will be measured using capillary mimicking microfluidic channels. It will be measured at 1 timepoint in 10 participants.

Measurement of hyperadhesion as a laboratory biomarker of dehydration after fluid resuscitation therapy in SCD participants with VOC or VOE

As a marker of hyperadhesion, capillary transit time will be measured using capillary mimicking microfluidic channels. It will be measured at 1 timepoint in 10 participants.

Measurement of elongation index as a cellular biomarker of dehydration before resuscitation therapy in SCD participants with VOC or VOE

Cellular dehydration marker, the elongation index, will be measured by ektacytometry. It will be measured at 1 timepoint in 10 participants.

Measurement of elongation index as a cellular biomarker of dehydration after fluid resuscitation therapy in SCD participants with VOC or VOE

Cellular dehydration marker, the elongation index will be measured by ektacytometry. It will be measured at 1 timepoint in 10 participants.

Measurement of point of sickling as a cellular biomarker of dehydration before fluid resuscitation therapy in SCD participants with VOC or VOE

Cellular dehydration marker, the elongation index will be measured by ektacytometry. It will be measured at 1 timepoint in 10 participants.

Measurement of point of sickling as a cellular biomarker of dehydration after fluid resuscitation therapy in SCD participants with VOC or VOE

Cellular dehydration marker, the elongation index will be measured by ektacytometry. It will be measured at 1 timepoint in 10 participants.

Measurement of urine osmolality as laboratory biomarkers of dehydration before fluid resuscitation therapy in SCD participants with VOC or VOE

Urine osmolality values <450 milliosmol/kg is considered abnormal in adults without fluid restrictions. It will be measured at 1 timepoint in 10 participants.

Measurement of urine osmolality as laboratory biomarkers of dehydration after fluid resuscitation therapy in SCD participants with VOC or VOE

Urine osmolality values <450 milliosmol/kg is considered abnormal in adults without fluid restrictions. It will be measured at 1 timepoint in 10 participants.

Inclusion Criteria: Diagnosis of Sickle Cell Disease (genotypes SS, SC, Sß-thalassemia, SD, SOArab) Participants must be ≥12-years old Participants that provide legally effective consent to all study procedures Exclusion Criteria: Participants under 12-years old Participants being treated with experimental therapies in clinical trials