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

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):

• 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)
• 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

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.