Juvenile Primary Fibromyalgia Syndrome (JPFS)
What is Juvenile Primary Fibromyalgia Syndrome (JPFS)?
Juvenile Primary Fibromyalgia Syndrome (JPFS) is a syndrome in which a person experiences musculoskeletal pain and fatigue as the primary symptoms. As far as we know, JPFS is not an autoimmune condition. Traditionally, rheumatologists care for patients with fibromyalgia, primarily because the most prominent symptom in many patients is musculoskeletal pain.
Who gets JPFS?
JPFS occurs in children, typically in the teenage years. Females are more likely than males to be diagnosed with JPFS. Many patients (up to 75%) will have a family history of fibromyalgia.
What other symptoms can persons with JPFS experience?
Common symptoms in addition to musculoskeletal pain and fatigue include disturbed sleep, morning stiffness, headaches, abdominal pain, anxiety or tension, tight muscles and brief periods of swelling. Less common symptoms include dizziness, light-headedness and difficulty concentrating.
What are tender points?
Patients with JPFS are likely to feel pain when pressure is applied to particular sites throughout the body called, "tender points." These places may remain tender when pressure is applied even after many of the symptoms of JPFS have improved significantly.
What causes JPFS?
The exact cause of JPFS is unknown. The familial occurrence of fibromyalgia is quite striking, but a genetic cause has not been found to date. Abnormalities of neurotransmitters and some hormones have been found, but a causal role for these chemicals has not been proven.
What we see in patients is that almost all are stuck in a cycle of pain, fatigue, poor sleep and decreased exercise. Difficulties could begin in any aspect of this cycle, but the cycle is perpetuated by the interactions of the aspects of this cycle.
For example, pain causes poor sleep and decreased exercise. In turn, poor sleep increases a patient's experience of pain. As a result, it becomes more difficult to sleep. As the person continues to have poor quality of sleep, fatigue becomes a major factor. With fatigue, the person is even less likely to exercise. Lack of exercise leads to poor quality of sleep and increased lethargy. Lack of exercise also increases the likelihood that a person will experience pain as a result of poor quality of sleep. One can easily see that this cycle is difficult to break.
What are some triggers for JPFS?
Triggers can start the cycle at any place along the cycle. Injury, illness and stress are common triggers. Anything that causes pain or interrupts the normal pattern of exercise for an individual can trigger JPFS.
What are the sleep abnormalities in JPFS?
The common thread of sleep abnormalities is that persons with JPFS experience non-restorative sleep. Some patients sleep many hours and awaken with no relief of their fatigue. Others have difficulty falling asleep or have frequent awakenings throughout the night. Some patients experience excessive movement of their limbs during sleep. Many patients nap during the day because they are so tired. All of these patients experience non-restorative sleep.
What are the treatments for JPFS?
Proven treatments for JPFS are education about the disease and cognitive behavioral therapy (CBT). Other treatments proven to work in adults with fibromyalgia are frequently used with success in children, but have not been definitively studied in a clinical trial in children at this point.
CBT is a system whereby children can decrease their experience of pain by understanding their triggers for pain and response to pain and learning to modify these factors. Techniques include relaxation, imagery, positive thoughts, and pacing. CBT is best learned from a trained psychologist.
Exercise is a mainstay of treatment in JPFS and has proven quite successful in clinical trials in adults. The most effective exercise is aerobic exercise, meaning any exercise in which the heart rate is increased. Examples include walking, biking, swimming, running, elliptical trainer, rowing or the stair stepper. Some sports such as basketball, soccer and track provide adequate aerobic exercise. Other sports such as baseball, softball and volleyball do not provide adequate sustained aerobic exercise.
Medications which have been proven to work in adult fibromyalgia include amitriptyline (Elavil), cyclobenzaprine (Flexeril), fluoxetine (Prozac), venlafaxine (Effexor) and duloxetine. These medications act through altering signaling by nerves. Some of these drugs are also utilized to treat depression. Amitriptyline (Elavil) is the most common medication used to treat JPFS. Non-steroidal anti-inflammatory medications (ibuprofen, Advil, Motrin, Alleve, many others) have not been proven effective in long-term treatment of fibromyalgia but may provide temporary pain relief.
I hurt when I exercise. How is this making me better?
It is very common early in treatment that exercise will result in increased pain. Some of this pain results from muscles being out of shape. Other pain is as a result of fibromyalgia. It is important to start slow with exercise and increase gradually to at least twenty minutes at least three times weekly. The most important aspect of exercise is regularity. This means exercising regularly, even if this is for a short time. Forming a habit of exercise which is part of your lifestyle is very important.
What is the relationship between depression and JPFS?
Some symptoms, such as fatigue and disturbed sleep, are very similar to symptoms experienced by patients with depression. Most patients with JPFS do not meet criteria for major depression, but some patients do. Your doctor should include questions about mood in determining how you are doing. In some cases, the doctor may recommend further evaluation for depression. It is important to explore all possible avenues to try to improve your overall well-being.
What is the long-term prognosis for persons with JPFS?
Many patients improve. Some patients evaluated several years later no longer experience enough symptoms to meet diagnostic criteria for fibromyalgia. We feel that persons with JPFS will need to pay careful attention to sleep, exercise and stress levels throughout their life to minimize symptoms of fibromyalgia. With careful attention to a healthy lifestyle, people with JPFS can live very happy and healthy lives. They can experience excellent personal and professional quality of life.
What is the relationship between JPFS and chronic fatigue syndrome (CFS)?
Many patients with JPFS may also be diagnosed with CFS. Symptoms are very similar, but patients with JPFS generally experience more pain. Treatment for JPFS should improve fatigue, even if patients also are diagnosed with CFS.
Written 6/05; Revised 4/07
