Near Infrared Spectroscopy in Sickle Cell Pediatric Patients

Near-infrared Spectroscopy in Sickle Cell Pediatric Patients During Pain Crisis and After Recovery

Endothelial dysfunction contributes to vaso-occlusion and acute pain in sickle cell disease. Near infrared spectroscopy (NIRS) technology can measure tissue oxygenation and endothelial function. The main objective of this study is to study the natural history of tissue muscle oxygenation using NIRS in pediatric sickle cell subjects experiencing acute pain and pediatric sickle cell patients in steady-state.

Study Overview

• Status: Withdrawn
• Conditions: Sickle Cell Disease
• Intervention / Treatment: Diagnostic test: Near infrared spectroscopy

• Study Type: Observational

Contacts and Locations

• Study Contact: Name: Suvankar Majumdar, MD; Phone Number: 2024763800; Email: smajumdar@childrensnational.org

Study Locations

• United States
  • District of Columbia
    • Washington, District of Columbia, United States, 20010
      • Children's National Medical Center

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: 6 years to 21 years (Child, Adult)
• Accepts Healthy Volunteers: No

• Sampling Method: Non-Probability Sample

Study Population

Subjects 6 to 21 years of age of all sickle cell genotypes

Description

Inclusion Criteria for Subjects with Sickle Cell Disease in Pain Crisis:

1. Age 6 to 21 years old.
2. Diagnosis of sickle cell anemia: a. Diagnosis of sickle cell disease (electrophoresis or HPLC documentation of hemoglobin SS, SC, S-beta-thalassemia or other hemoglobinopathies causing sickle cell disease is required).
3. Acute onset pain crisis in a distribution typical for that subject, onset within the last 7 days and for which hospitalization and parenteral narcotic pain treatment are required.
4. Ability to provide informed written consent.

Exclusion Criteria for Subjects with Sickle Cell Disease in Pain Crisis:

1. Pregnancy.
2. History of non-trivial injury, burns, surgery or skin ulcers on the arms.
3. Fever or suspected sepsis at time of pain crisis

4. Administration of any of the following drugs within the last 14 days:

   • Phosphodiesterase-5 inhibitors (sildenafil, vardenafil, tadalafil)
   • Endothelin-1 receptor blockers (bosentan, sitaxentan, ambrisentan, tezosentan)
   • Nitric oxide donors (nitroglycerin, nitroprusside, nitrates)
5. Ingestion of caffeine within the 12 hours before the start of the study appointment, or tobacco use within the 30 days before the study appointment.

6. Diagnosis with any of the following chronic diseases or conditions:

   • History of high blood pressure
   • History of high cholesterol
   • History of diabetes
   • History of chronic kidney disease (serum creatinine must not be greater than 2 mg/dL)
   • History of coronary artery disease or peripheral vascular disease
7. Received a blood transfusion within 7 days of the study procedure.

Inclusion Criteria for Subjects with Sickle Cell Disease in Steady State:

1. Age 6-21 years
2. Diagnosis of sickle cell disease (electrophoresis or HPLC documentation of hemoglobin SS, SC, S-beta-thalassemia or other hemoglobinopathies causing sickle cell disease is required).
3. Ability to provide informed written consent.

Exclusion Criteria for Subjects with Sickle Cell Disease in Steady State:

1. Pregnancy.
2. History of non-trivial trauma, burns, surgery or skin ulcers on the arms.
3. Fever or suspected sepsis at time of pain crisis
4. Experience of an acute pain crisis requiring intravenous (IV) narcotics and hospital admission within the last 14 days.
5. Ingestion of caffeine within the 12 hours before the start of the study appointment, or tobacco use within the 30 days before the study appointment.

6. Administration of any of the following drugs within the last 14 days:

   • Phosphodiesterase-5 inhibitors (sildenafil, vardenafil, tadalafil)
   • Endothelin-1 receptor blockers (bosentan, sitaxentan, ambrisentan, tezosentan)
   • Nitric oxide donors (nitroglycerin, nitroprusside, nitrates)

7. Diagnosis of any of the following chronic diseases or conditions:

   • History of high blood pressure
   • History of high cholesterol
   • History of diabetes
   • History of chronic kidney disease (serum creatinine must not be greater than 2 mg/dL)
   • History of coronary artery disease or peripheral vascular disease
8. Received a blood transfusion within 7 days of the study procedure.

Study Plan

How is the study designed?

Design Details

• Observational Models: Case-Control
• Time Perspectives: Prospective

Number of groups / cohorts

2

Cohorts and Interventions

Group / Cohort	Intervention / Treatment
Sickle cell pain crisis	Diagnostic test: Near infrared spectroscopy; NIRS is an emerging technology for measuring tissue oxygenation and endothelial function. NIRS light can penetrate through biological tissues, including skin, bone, and muscle. Similar to other optical methods, light is applied to the region of interest and undergoes scattering and absorption before being detected by a photosensor. This technique is commonly used to assess oxygen availability and consumption in living tissues. Using different wavelengths, NIRS can differentiate between oxygenated and deoxygenated hemoglobin in blood and can measure changes in total hemoglobin concentration, using the sum of oxygenated and deoxygenated hemoglobin.
Sickle cell steady-state	Diagnostic test: Near infrared spectroscopy; NIRS is an emerging technology for measuring tissue oxygenation and endothelial function. NIRS light can penetrate through biological tissues, including skin, bone, and muscle. Similar to other optical methods, light is applied to the region of interest and undergoes scattering and absorption before being detected by a photosensor. This technique is commonly used to assess oxygen availability and consumption in living tissues. Using different wavelengths, NIRS can differentiate between oxygenated and deoxygenated hemoglobin in blood and can measure changes in total hemoglobin concentration, using the sum of oxygenated and deoxygenated hemoglobin.

What is the study measuring?

Primary Outcome Measures

Outcome Measure	Time Frame
Tissue oxygenation	6 months

Secondary Outcome Measures

Outcome Measure	Time Frame
Nitric oxide	6 months

Collaborators and Investigators

• Sponsor: Children's National Research Institute
• Collaborators: National Institute of Allergy and Infectious Diseases (NIAID)

Study record dates

Study Major Dates

• Study Start (Actual): June 26, 2019
• Primary Completion (Actual): September 3, 2023
• Study Completion (Actual): September 3, 2023

Study Registration Dates

• First Submitted: July 22, 2019
• First Submitted That Met QC Criteria: July 22, 2019
• First Posted (Actual): July 24, 2019

Study Record Updates

• Last Update Posted (Actual): September 14, 2023
• Last Update Submitted That Met QC Criteria: September 12, 2023
• Last Verified: September 1, 2023

More Information

Terms related to this study

• Additional Relevant MeSH Terms: Hematologic Diseases; Genetic Diseases, Inborn; Anemia; Anemia, Hemolytic, Congenital; Anemia, Hemolytic; Hemoglobinopathies; Anemia, Sickle Cell
• Other Study ID Numbers: Pro00012424

Plan for Individual participant data (IPD)

• Plan to Share Individual Participant Data (IPD)?: NO

Drug and device information, study documents

• Studies a U.S. FDA-regulated drug product: No
• Studies a U.S. FDA-regulated device product: Yes
• product manufactured in and exported from the U.S.: No