Acceptance and Commitment Training for Adolescents and Young Adults With Neurofibromatosis Type 1, Plexiform Neurofibromas, and Chronic Pain
Condition: Neurofibromatosis Type 1; Plexiform Neurofibromas
MeSH terms: Neurofibromatoses; Neurofibromatosis 1; Neurofibroma; Neurofibroma, Plexiform; Chronic Pain
Intervention: Acceptance and Commitment Therapy (ACT)
Intervention description: Acceptance and Commitment Therapy (ACT), a newer generation of cognitive-behavioral therapy, focuses on encouraging individuals to engage in more adaptive ways of coping with pain.
Phase: Phase 3
Study type: Interventional
Primary purpose: Supportive Care
Allocation: Randomized
Intervention model: Parallel Assignment
Masking: None (Open Label)
Enrollment: 66.0
Sex: All
Minimum age: 16 Years
Maximum age: 59 Years
Healthy volunteers: No
Keywords: Psychological Intervention; Coping; Mindfulness; Randomized

Summary:

      Background:

      - People with neurofibromatosis type I (NF1) and plexiform neurofibroma (PN) tumors often
      have chronic pain that his hard to control. People usually take medicines for the pain, but
      they may not work well and might cause side effects. A new strategy called Acceptance and
      Commitment Training (ACT) may help these people cope with chronic pain. ACT focuses on things
      like values and living in the moment.

      Objective:

      - To see if Acceptance and Commitment Training improves pain coping in people with NF1 pain.

      Eligibility:

      - People age 16-34 who have NF1, 1 or more PN tumors, and pain that interferes with their
      daily functioning.

      Design:

        -  Participants will be screened with a physical exam, medical history, and questions about
           their pain.

        -  Participants will fill out questionnaires about their pain and feelings. Their heart
           rate will be measured via electrocardiogram (ECG).

        -  Participants will be divided into 2 groups randomly. One will wait 8 weeks.

        -  The other will start training right away.

        -  Participants will have 2 two-hour sessions with an ACT trainer. They will learn
           techniques for setting goals based on personal values and other ways to cope with pain.
           They will get a workbook and a compact disc (CD) to take home for practice.

        -  Participants will do practice exercises at home between sessions. They will get weekly
           emails with a practice exercise. They will join video chat sessions via home computer
           with their trainer.

        -  All participants will return to National Institutes of Health (NIH) after 8 weeks for
           questionnaires and an ECG. The wait group will then start training. They will return 8
           weeks later for questionnaires and an ECG.

        -  Six months later, they will complete questionnaires from home by computer.
    

Eligibility criteria:

        -  INCLUSION CRITERIA FOR PARTICIPANT

               1. Patients must be between 16 and 59 years of age at the time of the baseline
                  assessment. Because the research on the effectiveness of Acceptance and
                  Commitment Therapy (ACT) with younger children is still emerging, children 15 and
                  younger will be excluded from the present study.

               2. Diagnosis of Neurofibromatosis Type 1 (NF1) through germline mutation OR clinical
                  diagnosis; for the clinical diagnosis of NF1 all study subjects must have two or
                  more diagnostic criteria for NF1 listed below (National Institutes of Health
                  (NIH) Consensus Conference):

                    -  Six or more cafe-au-lait spots (greater than or equal to 0.5 cm in
                       prepubertal subjects or greater than or equal to 1.5 cm in postpubertal
                       subjects)

                    -  Greater than or equal to 2 neurofibromas or 1 plexiform neurofibroma

                    -  Freckling in the axilla or groin

                    -  Optic glioma

                    -  Two or more Lisch nodules

                    -  A distinctive bony lesion (dysplasia of the sphenoid bone or dysplasia or
                       thinning of long bone cortex)

                    -  A first-degree relative with NF1

               3. Participants must have documentation of a plexiform neurofibromas (PN), based on
                  either clinical exam or imaging.

               4. Patient must self-report having chronic pain for at least the past 3 months that
                  has interfered with their daily functioning, as assessed by the Pain Interference
                  Index (must get a mean score of 2.0 or higher, or score a 3 on three or more
                  individual items).

               5. Patients must have regular access to a computer or tablet with internet access.

               6. Ability of subject or Legally Authorized Representative (LAR) to understand and
                  the willingness to sign a written informed consent document.

               7. No anticipated major changes in their pain treatment regimen (i.e., new class of
                  pain medication starting or change in the class of pain medication) or enrollment
                  on a new treatment study presumed to impact pain in the near future.

               8. Subjects must be able to read and comprehend the English language, since the
                  highly trained ACT therapists are not fluent enough to conduct the trainings in
                  Spanish or other languages.

        EXCLUSION CRITERIA FOR PARTICIPANT

          1. In the opinion of the Principal Investigator (PI) or an Associate Investigator (AI),
             the subject has significant cognitive or emotional difficulties that would prevent
             them from being able to understand and/or participate fully in the intervention or the
             measures.

          2. Subjects who are participating in any other treatment studies, either medical or
             behavioral, specifically for pain management.

          3. Subjects who began a medical intervention for treatment of their disease that has a
             possible impact on pain (including Mitogen-activated Protein/Extracellular
             Signal-regulated Kinase Kinase (MEK) trials) will not be eligible until after one year
             on the medical treatment; at that time, eligibility will be discussed with the PI of
             the medical study to assess the stability of the patient s pain and whether further
             pain-related changes due to the medical treatment are likely.

          4. Inability to travel to the NIH, for example, due to physical limitations, for the
             in-person evaluation(s).

        No groups in regards to gender, race, or ethnicity are being excluded from participation in
        the trial.