Pain Management Protocol for Pediatric Sickle Cell Disease
Condition: Sickle Cell Disease
MeSH terms: Anemia, Sickle Cell
Study type: Observational

Summary: 
      Sickle cell disease (SCD) is a devastating chronic health condition that primarily affects
      African-Americans. Painful episodes are the most frequent form of morbidity in SCD and the
      most frequent reason for hospitalization. Cognitive-behavioral interventions for pain
      management have shown efficacy for improving coping abilities, reducing the amount of
      medication needed to manage pain, and improving daily functioning during painful episodes.
      However, difficulties with disseminating and implementing cognitive-behavioral treatments
      have resulted in almost no use of these techniques in pediatric settings. In South Carolina
      these difficulties are compounded by social and geographical factors that pose particular
      challenges. A major issue with implementing quality pain management protocols is the
      difficulty with providing adequate practice and monitoring of the use of the techniques,
      particularly given the rural population in South Carolina and transportation difficulties for
      economically disadvantaged families. Due to a history of under-treating pain in SCD it is
      also critical that psychological and medical treatments are presented in an integrated manner
      so that these approaches are viewed as complimentary, not mutually exclusive, approaches to
      pain management. Finally, we believe the same implementation issues for improving the use of
      behavioral coping skills are also important for improving adherence to medication protocols
      for appropriate home-based pain management.

      The purpose of this proposal is to develop, implement, and evaluate a pain management
      protocol that uses portable electronic devices and other technologies to increase the
      practice of psychosocial pain management techniques, improve adherence to the overall
      biopsychosocial pain management protocol, and improve the clinician's ability to track
      progress with fewer office visits. In addition to addressing important dissemination issues,
      by embedding methods to assess for adherence into the technology it will be possible to
      continuously evaluate and modify protocol efficacy, resulting in a product that is effective,
      empirically sound, and flexible. Participants will be randomly assigned to the intervention
      or waitlist control condition. Those on the waitlist condition will receive the same study
      procedures after a 2 month wait periods. We anticipate that the intervention will result in
      better pain management and less impairment in the participants.
    

Detailed description: 
      "Home-Based Pain Management Protocol for Children with Sickle Cell Disease"

      BACKGROUND: Sickle cell disease (SCD) is a devastating chronic health condition that
      primarily affects African-Americans. Painful episodes are the most frequent form of morbidity
      in SCD and the most frequent reason for hospitalization. Cognitive-behavioral interventions
      for pain management have shown efficacy for improving coping abilities, reducing the amount
      of medication needed to manage pain, and improving daily functioning during painful episodes.
      However, difficulties with disseminating and implementing cognitive-behavioral treatments
      have resulted in almost no use of these techniques in pediatric settings. In South Carolina
      these difficulties are compounded by social and geographical factors that pose particular
      challenges. A major issue with implementing quality pain management protocols is the
      difficulty with providing adequate practice and monitoring of the use of the techniques,
      particularly given the rural population in South Carolina and transportation difficulties for
      economically disadvantaged families. Due to a history of under-treating pain in SCD it is
      also critical that psychological and medical treatments are presented in an integrated manner
      so that these approaches are viewed as complimentary, not mutually exclusive, approaches to
      pain management. Finally, we believe the same implementation issues for improving the use of
      behavioral coping skills are also important for improving adherence to medication protocols
      for appropriate home-based pain management.

      PURPOSE: The purpose of this research is to implement and evaluate a pain management protocol
      that uses portable electronic devices (PDA) and other technologies to increase the practice
      of psychosocial pain management techniques, improve adherence to the overall biopsychosocial
      pain management protocol, and improve the clinician's ability to track progress with fewer
      office visits. In addition to addressing important dissemination issues, by embedding methods
      to assess for adherence into the technology it will be possible to continuously evaluate and
      modify protocol efficacy, resulting in a product that is effective, empirically sound, and
      flexible.

      STUDY PROCEDURES:

      Recruitment and Assignment to Study Conditions. Families will be approached as part of their
      routine psychosocial screenings currently conducted at the Children's Cancer and Blood
      Disorder Center. Parental informed consent and child assent will be obtained from families
      who meet inclusion criteria and agree to participate. Next, participants will be randomly
      assigned to waitlist control or intervention groups through the use of a random assignment
      procedure. The researcher will explain details of the participation incentive system to the
      families at this time, including the number of coupons assigned for each phase of study
      completion and how to redeem coupons for toys and gift cards. All participants (waitlist
      control group and intervention group) will complete the initial measures: Background
      Information Questionnaire ; Structured Pain History Interview - Parent and Child Forms;
      Coping Strategies Questionnaire (CSQ); Pediatric Quality of Life Inventory (PEDS-QL);
      Pediatric Pain Questionnaire (PPQ); Child Health Locus of Control during the initial meeting
      and the second set of measures (CSQ, PEDS-QL and PPQ) 4 weeks after the initial meeting.

      Cognitive-Behavioral Training. Following completion of the intake measures, families in the
      intervention condition will be provided education on pain medication use and explanation of
      active versus passive methods of coping with pain and the cognitive-behavior techniques will
      be introduced (Progressive Muscle Relaxation, Deep Breathing, Guided imagery and
      Distraction). These techniques will be tailored to the child's preferences and recorded onto
      the PDA.

      Next, the researcher will review with the family the pain flow chart, how to complete the
      Daily Pain and Activity Diary (DPAD), and use the PDA and Pain Management software.
      Participants and their parents will be provided with detailed instructions regarding care of
      PDA, who to contact if device is broken or lost, policy on continued participation if device
      is lost, and a padded addressed stamped envelope to return the PDA should it require
      attention. Three days after the initial visit families will be contacted to answer questions
      and ensure implementation of skills.

      Families will also receive a weekly telephone contact at a pre-arranged time to address any
      difficulties with protocol implementation. Pain diaries and frequency of skills practice will
      be electronically downloaded approximately every three days using wireless synchronization
      built into the PDA devices. One month following the initial session, all participants
      (waitlist and intervention groups) will complete the PPQ, PEDS-QL and CSQ outcome measures.
      Two months after the initial session, intervention participants will complete outcome
      measures, including PPQ, PEDS-QL, CSQ and Protocol Evaluation Form (PEF). Six months after
      the initial session, intervention participants will complete the follow-up measures,
      including child report on the CSQ, and parent and child report on the PPQ and PEDS-QL. At
      this time the participant's school will be contacted to obtain school attendance records so
      as to document absences due to illness over the previous year and participants medical
      records will be reviewed to examine patient's disease severity over the course of their study
      participation time and their lives (Medical Record Review Form). Participants in the waitlist
      control group will start participation in the intervention at the two month point, and follow
      the procedures outlined above for the intervention condition.

      Concurrent Validity Study Procedures. Assessment of pain experience will be obtained for
      study participants undergoing routine venipuncture as part of their clinic visit co-occurring
      with study entry and study completion. Observational, physiological response, parent-report,
      and self-report of pain experience will be obtained to provide convergent validity for
      measures of pain report. A brief measure of medical coping style, the Child
      Approach-Avoidance Rating Scale (CAARS; Appendix J) will be included to assess for shifts in
      coping strategies employed by the participant during painful procedures. Participants and
      parents will report on child pain experience before, during, and after the routine
      venipuncture that is part of their clinic visit on the day of initial contact. Participant
      response to routine venipuncture will also be captured via video recording to allow for an
      in-depth analysis of observable pain behaviors. Pre-procedural heart rate will be taken at
      three intervals: when the patient is first seen for the clinic visit, immediately prior to
      the venipuncture, and immediately following the venipuncture. Additionally, small extra vial
      of blood will be obtained (5mL) to measure the participant's level of endothelin-1, a
      biological marker of pain. This extra vial of blood will not require an additional needle
      stick. Finally, the medical records of all participants will be reviewed to obtain
      information about disease course.

      SUBJECT POPULATION:

      Participants will include up to 50 children and adolescents with SCD ranging between the ages
      of 8 to 18 years of age and their primary caregivers. Participants will be recruited from the
      Children's Cancer and Blood Disorder Center at Palmetto Health and four DHEC Children's
      Rehabilitative Services sites (Orangeburg, Columbia, Florence and Sumter). Reviews of
      behavioral interventions for rheumatoid arthritis or chronic low back pain have indicated
      typical treatment effect sizes for pain experience and coping of d = 0.46 or greater (Astin
      et al., 2002; van Tulder et al., 2001). Using this value as a basis for computing study power
      (with an alpha level of .05) the study has an expected power of .80 with a sample size of 50
      participants.

      Inclusion/Exclusion criteria:

      Inclusion criteria mandate that the families selected demonstrate adherence with standard SCD
      treatment as indicated by reliable attendance to clinic visits, and chart notes demonstrating
      adherence to prescribed medical care (e.g., follow-through with required specialist visits
      and appointments for diagnostic procedures). Participants must have had at least 1 major pain
      episode or 3 minor pain episodes in the previous 6 months that resulted in functional
      impairment, such as reduced school attendance. Finally, individuals with developmental
      disabilities such as mental retardation, autism, and Down syndrome, severe cognitive
      limitations following stroke, or diagnoses of severe psychopathology will be excluded from
      participation as these disorders have the potential to confound the findings.

      CONFIDENTIALITY: To minimize threats to confidentiality, only investigators will have access
      to participant data. For data management purposes and confidentiality, subjects will be given
      a unique identifier to use when recording data and electronically transmissions of data. This
      arrangement minimizes risk to loss of patient confidentiality. All study staff, including
      research assistants who may enter data, will be required to complete and pass the University
      of South Carolina Human Subjects on-line tutorial prior to commencing their work on this
      research project. All data will be stored in a locked file cabinet in the investigators
      office at Palmetto Health.

      EXPECTED RESULTS AND IMPLICATIONS: We anticipate that the intervention will result in better
      pain management and less impairment in the participants. These data will be used to seek
      funding for a larger-scale study and in the development of interventions to decrease the
      impact of pain on the lives of individuals with SCD.
    

Intervention name(s): Brief Cognitive-Behavioral Pain Management Training
Intervention type(s): Behavioral
Intervention description(s): Participants complete a manualized, single session of training which includes: (A) education about the causes of sickle cell pain, (B) education about how the nervous system processes pain signals, and (C) explanations of how one can use cognitive and behavioral treatment (CBT) strategies to decrease the extent of pain experienced (all based on how the nervous system processes pain). Four specific CBT skills are taught and practiced by the participant (Progressive muscle relaxation, Controlled Deep Breathing, Imagery, and Distraction). The protocol explicitly emphasizes the use of CBT as a complimentary pain management tool to use in addition to the standard of care methods.
Observational model: Case-Control
Time perspective: Prospective
Sampling method: Non-Probability Sample
Study population: 
        The study population are youths with sickle cell disease who are experiencing intermittent
        pain episodes that interfere with quality of life.
      
Keywords: Cognitive Behavioral Therapy; Pediatric Sickle Cell Disease; Pain Management