Acceptance and Commitment Therapy for Adolescents and Young Adults With Neurofibromatosis and Chronic Pain
Acceptance and Commitment Therapy for Adolescents and Young Adults With Neurofibromatosis and Chronic Pain
Background:

 -  Neurofibromatosis type 1 (NF1) is a genetic disorder that can cause frequent and
 significant pain. This pain can have a severe impact on a person s quality of life.

 -  Acceptance and Commitment Therapy (ACT) is a new type of therapy that may help people
 manage pain. It teaches behavioral techniques that can be used to change how people
 react to pain. ACT may help people with NF1 cope better with the pain associated with
 the disorder. Researchers want to test ACT techniques to see if they can improve coping
 in people with NF1 pain.

 Objectives:

 - To see if ACT can help treat chronic pain in adolescents and young adults who have NF1.

 Eligibility:

 - Adolescents and young adults between 12 and 21 years of age who have chronic pain
 associated with NF1.

 Design:

 -  Participants will be screened with a physical exam and medical history. They will also
 complete a set of questionnaires about their pain levels, quality of life, and
 medications that they are taking.

 -  Participants will take part in a training workshop for ACT. It will consist of three
 2-hour sessions over 2 consecutive days. The workshop will teach techniques for setting
 goals that reflect personal values, and changing the focus from pain avoidance to pain
 management. There will be practice exercises between each session.

 -  At the end of the workshop, participants will receive a workbook with the exercises to
 continue to practice at home. Participants will be encouraged to spend at least 20
 minutes, three times per week, on these practice exercises.

 -  There will be a followup phone call to check on pain management after the workshop.

 -  Three months after the first study visit, participants will complete the study
 questionnaires again from home. They will provide more information about how they are
 managing their pain. The questionnaires will be returned by mail in an envelope provided
 by the study team.
Background:

 -  Neurofibromatosis type 1 (NF1) is a genetic disorder that affects approximately 1 in
 3,500 individuals.

 -  A number of common clinical manifestations, including plexiform neurofibromas,
 scoliosis, and chronic headaches, can cause frequent and significant pain and impact
 quality of life.

 -  Often, NF1-related pain is not well-controlled with medication and many of the
 medications cause significant side effects.

 -  To our knowledge, no studies have examined the effectiveness of behavioral interventions
 for chronic pain in individuals with NF1.

 -  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 or distress.

 -  In helping individuals with pain, the goal of ACT is not to eliminate the person s
 pain, but to optimize the person s quality of life despite their pain.

 Objectives:

 -  To evaluate whether the Acceptance and Commitment Therapy (ACT) group demonstrates
 improvement on the Modified Brief Pain Inventory scale from baseline to 3 months in
 adolescents and young adults with NF1 and chronic pain.

 -  To examine changes in patient s functional ability, quality of life, pain-related
 coping, pain-related anxiety, pain severity, and depression from baseline to 3 months

 -  To examine the effects of ACT on parents psychological well-being, overall stress, and
 ability to cope with their child s pain

 -  To explore the relationship between changes in patients pain interference, functional
 ability, pain-related coping, pain-related anxiety, pain severity, depression and
 treatment adherence with demographic variables, parent stress, and parent coping

 -  To collect preliminary data on the value of ACT to guide the potential development of a
 future phase II trial

 Eligibility:

 Patients ages 12 to 21 with a confirmed diagnosis of NF1.-Parents of participating
 patients.-The patient must indicate a score of 3 or higher on a pain item (1 5 scale) from a
 quality of life measure assessing the extent to which pain has interfered with the patient s
 daily functioning over the past month.

 Design:

 -  This is a behavioral study to determine the feasibility and potential benefit of an ACT
 intervention on pain interference from baseline to 3 months post-intervention.

 -  A sample size of 12 patients is needed to provide 90% power for detecting a change of
 .77 standard deviations on the total pain interference score of the M-BPI using a
 1-sided .1 level test.

 -  ACT workshops will be scheduled multiple times over approximately five months and will
 be comprised of three 2-hour sessions held over two consecutive days

 -  Measures assessing pain interference, pain coping, pain-related anxiety, quality of
 life, functional disability, depression, and pain severity will be administered to
 patients prior to the ACT intervention and 3 months later.

 -  At the same time points, a parent or legal guardian of each patient will complete
 measures assessing their child s quality of life, functional disability, depression, and
 pain severity, as well as the parent s own psychological functioning and ability to cope
 with their child s pain
-  INCLUSION CRITERIA FOR PARTICIPANT:

 Subjects must be between 12 and 21 years of age at the time of the intervention. Because
 the research on the effectiveness of ACT with children younger than 12 is still emerging,
 children 11 and younger will be excluded from the present study.

 For the clinical diagnosis of NF1 all study subjects must have two or more diagnostic
 criteria for NF1 listed below (NIH Consensus Conference):

 1. 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 postpuberta subjects)

 2. Greater than or equal to 2 neurofibromas or 1 plexiform neurofibroma

 3. Freckling in the axilla or groin

 4. Optic glioma

 5. Two or more Lisch nodules

 6. A distinctive bony lesion (dysplasia of the sphenoid bone or dysplasia or thinning of
 long bone cortex)

 7. A first-degree relative with NF1

 Response of 3 or higher on a pain item (1 5 scale) from a self-report quality of life
 measure (Impact of Pediatric Illness Scale) assessing the extent to which pain has
 interfered with the patient s daily functioning over the past month.

 Ability of subject or Legally Authorized Representative (LAR) to understand and the
 willingness to sign a written informed consent document.

 Subjects may be participating in other medical interventions for the treatment of
 their disease, or standard care techniques for pain management.

 No anticipated major changes in their pain regimen or enrollment on a new treatment
 study in the near future.

 Subjects must be able to read and comprehend the English language.

 EXCLUSION CRITERIA FOR PARTICIPANT:

 In the opinion of the PI or an 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.

 Subjects who are participating in any other treatment studies, either medical or
 behavioral, for pain management.

 Subjects who are scheduled to begin a new drug protocol for the treatment of their NF1
 during the time of their enrollment on the current study.

 Inability to travel to the NIH, for example, due to physical limitations, for the
 scheduled workshop and evaluations.

 No groups in regards to gender, race, or ethnicity are being excluded from
 participation in the trial.

 INCLUSION CRITERIA FOR PARENTS:

 Parents must have a child with NF1 who is participating in this protocol as a patient.

 Ability to read and comprehend the English language.

 Ability to understand and the willingness to sign a written informed consent document.

 If a minor patient meets all eligibility criteria but his or her parent does not
 (i.e., in the case where a parent does not speak English), the patient will not be
 allowed to participate.