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Fibromyalgia |
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Background:
Fibromyalgia is a syndrome
characterized by multiple soft tissue tender
points (TPs), musculoskeletal pain, stiffness in
multiple areas, absence of other systematic
manifestations, and normal routine laboratory
tests.
Although children and adults
with fibromyalgia syndrome (FMS) experience
similar symptoms, children seem to experience more
sleep disturbances and fewer TPs than adults.
Yunis and Masi first described similarities and
differences of juvenile FMS as compared to
presentation in adults. Diagnosis is made by
history, physical examination, lab studies, and
exclusion of other causes of findings. The 1990
American College of Rheumatology (ACR) diagnostic
criteria for FMS include diffuse pain and 11 or
more TPs. The goal of treatment is to control the
symptoms using multiple therapies of medication,
physical therapy, exercise regime, support groups,
and psychological therapy. Incidence of FMS in
children may be as high as 6.2% of the general
pediatric population. The prognosis of children
with FMS is more favorable than in adults.
The ACR defined 2 major
diagnostic criteria for classifying FMS in adults.
The first is a history of widespread pain for at
least 3 months involving both sides of the body
above and below the waist. Specific areas include
the cervical skeleton (eg, spine, anterior chest),
the shoulders or buttocks (considered for each
involved side), and the lower back (considered
below the waist). The second criterion requires
pain on palpation of 11 of 18 defined TPs when
digitally palpated with approximately 4 kg per
unit area of force. The patient must indicate that
palpation is painful for a positive result.
The 1990 criteria for adult FMS
was found to be less sensitive to the events that
occur in childhood FMS. The term juvenile primary
fibromyalgia syndrome results from a better
understanding of the child's experience with FMS.
Two investigators, Yunis and Masi, proposed FMS
criteria that are slightly different for children
and adolescents. Their criteria take into account
a more variable presentation along with a
dependence on adult input to make the diagnosis.
Pediatric FMS criteria include the presence of 2
major criteria and some minor diagnostic
symptomatology.
The Yunis and Masi diagnostic
criteria are similar to the latter ACR criteria
and include 3 months of widespread pain in the
absence of other underlying causes for the
symptoms and severe pain in 5-11 TPs with
palpation of less than 4 kg per unit area of
force.
In FMS, routine laboratory tests
are by definition in normal range and 3-10 of the
following minor criteria are present: chronic
anxiety or tension; fatigue; poor sleep; chronic
headaches; irritable bowel syndrome; subjective
soft tissue swelling; numbness; pain modulation by
physical activities, weather conditions, or
anxiety and stress.
Bennett describes FMS as
involving a core feature of pain (eg, widespread
musculoskeletal pain, multiple TPs), typical
features (eg, fatigue, stiffness, skin tenderness,
postexertional pain, sleep disturbance), and
associated features (eg, irritable bowel symptoms,
poor memory, tension headaches, dizziness, fluid
retention, paraesthesias, restless legs, bruising,
Raynaud's phenomenon). The chronic musculoskeletal
pain affects quality of life, while fatigability
influences motor response and ability to complete
activities of daily living in an expedient time
frame.
Children usually differ from
adults in that they may have increased frequency
of pain aggravated by overactivity, with pain
being relieved by moderate activity, increased
subjective swelling, and decreased pain modulation
by anxiety and weather. Children have less lower
back pain, hand pain, and paraspinal TPs; however,
children experience ankle pain, and increased pain
associated with overactivity.
Pathophysiology:
FMS is a physiological
entity and not a psychiatric disorder, though the
physiological cause of FMS in children is unknown.
Studies have implicated possibilities such as
abnormalities in muscle structure or repair,
endocrine abnormalities, psychological components,
or biochemical changes in lower spine or upper
back. FMS may be either primary or secondary to
hypothyroidism, malignancy, osteoarthritis,
rheumatic diseases, sports related over-activity,
or trauma. Some authors also describe a reactive
FMS, which arises after a discrete illness or
after a specific episode of trauma. There is also
a greater than 30% psychological co-morbidity. The
ACR recommends against the use of primary and
secondary designations, but these continue to
prove useful in clinical and research settings. A
number of abnormalities have been suggested as a
possible pathogenesis, including abnormalities in
CNS neurotransmitter levels, delta sleep
disturbance, muscle metabolic aberrations, and
various psychopathologies.
Frequency:
- In the US:
FMS accounts for 7.5% of
new diagnoses made among children and
adolescents by pediatric rheumatologists.
- Internationally:
FMS occurs in
6.2% of Israeli school children and 1.3% of
Mexican school children.
Mortality/Morbidity:
- In a review of 59 children
with pediatric FMS, Gedalia and colleagues found
the following symptoms:
- Generalized aches 97%
- Headaches 76%
- Sleep disturbances 70%
- Stiffness 30%
- Subjective joint swelling
24%
- Fatigue 20%
- Abdominal pain 17%
- Joint hypermobility 14%
- Depression 7%
- Siegel and colleagues found
the following symptoms at the initial
presentation of 45 children with FMS:
- Sleep disturbance 96%
- Diffuse pain 93%
- Headaches 71%
- General fatigue 62%
- Morning stiffness 53%
- Morning fatigue 49%
- Depression 43%
- Feeling worse with exercise
42%
- Subjective swelling 40%
- Irritable bowel 38%
- Dysmenorrhea 36%
- Illness changes with
weather 36%
- Paresthesias 24%
- Global anxiety 22%
- Lack of energy 18%
- Raynaud's phenomenon 13%
- Studies of children with FMS
have documented a high association of sleep
disturbances. Tayag-Kier et al reported that
children with FMS presented with long sleep
latency, shortened total sleep time, decreased
sleep efficiency, and increased wakefulness
during sleep. Additionally, Tayag-Kier et al
found a subset of children with FMS exhibited
periodic limb movement in sleep (PLMS) in which
subjects experienced significantly higher
wakefulness after sleep onset.
- In addition, other associated
symptoms of FMS in children include irritable
bowel syndrome, migraines, premenstrual
syndrome, Raynaud's phenomenon, female urethral
syndrome, and restless leg syndrome.
Race:
In the United States, FMS is less
common among African American children.
Sex:
Female children are diagnosed more
commonly with FMS than are male children. Studies
show that girls are at least 3-7 times more likely
than boys to be diagnosed with FMS.
Age:
Pediatric FMS most frequently
presents in adolescents aged 13-15 years. The
earliest reported case in pediatrics is of a
5-year-old child.
History:
FMS is characterized by musculoskeletal
pain, stiffness, and aching. The severity of pain
at the TPs rates 8 on a scale of 10. Complaints of
fatigue, anxiety, and depression are reported.
Adolescents with FMS often describe abnormal sleep
patterns that interfere with school and family
activities. Descriptions of difficulty falling
asleep, frequent awakenings due to discomfort, and
feeling unrested in the morning are common.
- Questions for patient and
family should explore presence of the following:
- Widespread pain or aching
- Headaches
- Morning stiffness and
fatigue
- Subjective joint swelling
- Abdominal pain
- Symptoms of depression
- Quality and amount of sleep
- Assess quality of pain (eg,
when, what, where, how long)
- When did the pain start?
- What makes it better?
- What makes it worse?
- How long does it last?
- Does it vary throughout the
day?
- Does it wake you up at
night?
- Where is the pain?
- What is it like (eg, sharp,
dull, aching, deep)?
- What is the appearance of
the affected area (eg, swelling, edema)?
- Other associated symptom
questions
- Do you have fever?
- Do you have any change in
appetite?
- Do you have any weight
loss?
- Describe your sleep
pattern.
- Are you disturbed easily
during sleep?
- Do you have frequent
awakenings?
- Do you feel rested in the
morning?
- Do you have any bowel or GI
symptoms?
- Do you feel anxious, down,
depressed?
- Are your muscles weak?
- Psychosocial aspect questions
- Are you experiencing any
stressors or problems at school?
- Are you experiencing any
stressors or problems in your family?
- Are you tired in school?
- Are you able to keep up
with the other children at school and outside
activities?
- What has been the impact of
the pain on routine activities?
- How has your family
responded to the pain?
- Is there any one else at
home with similar problems?
- Common aggravating factors of
FMS
- Anxiety/stress
- Cold weather
- Humid weather
- Inactivity
- Physical overactivity
- Poor sleep
- Common alleviating factors of
FMS
- Hot shower/bath
- Moderate activity
- Stretching/exercising
- Warm weather
Physical:
A
standard physical examination to diagnose FMS is
essential.
- Perform thumb palpitation of
18 specific TP sites with a force of 4 kg per
unit area. This force is approximately the
pressure necessary to blanch the examiner's
nail. It is important to note that this criteria
is suggested but not agreed upon among
practitioners. Neumann, Smythe, and Buskilia
suggest using a 3-kg criterion rather than 4 kg
in children because their threshold is different
from adults. In the child, palpation elicits
tenderness in 5 of 11 TPs at the following
locations:
- Occiput - Bilateral, at the
suboccipital muscle insertions
- Low cervical - Bilateral,
at the anterior aspects of the intertransverse
spaces at C5-C7
- Trapezius - Bilateral, at
the mid point of the upper border
- Supraspinatus - Bilateral,
at origins, above the scapula spine near the
medial border
- Second rib - Bilateral, at
the second costochondral junctions just
lateral to the junctions on upper surfaces
- Lateral epicondyle of
humerus - Bilateral, 2 cm distal to the
epicondyles
- Gluteal - Bilateral, in
upper outer quadrants of buttocks in anterior
fold of muscle
- Greater trochanter -
Bilateral, posterior to the trochanteric
prominence
- Knee - Bilateral, at the
medial fat pad proximal to the joint line
- In general, Calabro describes
examination of joints in juvenile FMS to reveal
normal findings despite tenderness and spasms in
soft tissue on palpation. Physical findings that
should be explored are the presence of
hypermobility in the joints using the criteria
developed by Carter and Wilkerson and modified
by Bird, presence of swelling or joint edema,
abdominal tenderness, and range of motion to
determine joint stiffness.
Causes:
Various etiologies proposed for FMS
exist, though the actual cause of the syndrome is
unknown. Pellegrino, Waylonis, and Sommer studied
evidence of inherited primary FMS and found the
mode of inheritance as autosomal dominant;
therefore, FMS may be an inherited condition.
Other proposed etiologies include neurotransmitter
abnormalities, immune disorders, endocrine
abnormalities, allergic factors, viral infections,
and structural muscle changes.
Other Problems to be
Considered:
Anterior chest wall syndrome
Benign rheumatoid nodules
Bursitis
Depression
Dysautonomia
Early spondyloarthropathy
Growing pains
Hypermobility syndrome
Hypochondriasis
Inflammatory bowel disease
Malingering
Multiple sclerosis
Reflex sympathetic dystrophy
Restless leg Syndrome
Tendinitis
Thyroid Disease
Syndrome of multiple chemical sensitivities
Lab Studies:
- Erythrocyte sedimentation
rate (ESR) - Mean ESR is 15 mm/h
- Rheumatoid factor (RF) -
Negative
- C-reactive protein and
antinuclear antibody (ANA) titer - May be
positive. However, given the high incidence of
ANA in the general population, ANA testing
should be avoided unless the history and
physical exam point to features and
abnormalities not found in fibromyalgia.
- Prolactin serum levels -
Negative
- Liver function tests -
Normal
- Purified protein derivative
(PPD) - Negative
- Blood and urine cultures -
Negative
- Thyroid Function Tests -
Normal
Imaging Studies:
- Plain radiographs including
chest, ribs, back - Normal
- Ultrasounds of abdomen,
pelvis, paravertebrae - Findings normal
- CT scan/MRI studies - Normal
- Polysomnography, including
PLMS assessment, to evaluate possible sleep
disorders - Normal
Medical Care:
The recommendation for
effective treatment of FMS is a multidisciplinary
approach due to the multifaceted problems that
occur in FMS. Treatment is directed at physical
conditioning, analgesia, helping sleep
disturbances, and assisting children in coping
with the pain while maintaining physical
activities and function. The goal of treatment is
to reduce pain and depression, decrease sleep
disturbances, and promote physical activity.
Activity is a mainstay of treatment for FMS (see
Activity).
- Support: Although a better
understanding of what causes FMS would be
helpful in determining treatment options, a
holistic approach to the child and family living
with this problem is the current recommendation.
Supporting the child and family to maintain as
normal a lifestyle as possible is important
because they live with a potentially chronic
disorder. Emphasis on both the child and family
understanding of the disorder is helpful in
learning to live with the problems and overcome
them. Attendance at school and other usual
activities is imperative. Modifying
participation or attendance may be necessary in
light of the child's ability to keep up with the
expected activities.
- Sleep: Bennett and Tayag-Kier
et al suggest that a sleep analysis in children
is helpful in determining treatable causes of
sleep disturbance and PLMS. Studies in children
are few at this point; however, low-dose
tricyclic antidepressants or cyclobenzaprine
have been used to help promote deeper sleep.
Gedalia et al first tried cyclobenzaprine at
bedtime to help promote sleep and then switched
to low-dose antidepressants when 25% of the
patients failed to respond to the muscle
relaxant.
- Psychological treatment: The
use of cognitive-behavioral therapy has proven
helpful in some cases, though conclusive studies
do not yet exist to substantiate this therapy.
Studies are few that look at the use of
psychological interventions completed on
children with FMS; however, those studies that
were completed support improvement with the use
of cognitive therapy. Waco and Ilowite found
that the use of a cognitive-behavioral program
showed improvement in symptoms over a 4- to
24-month period. Likewise, Vereker studied the
use of counseling, behavioral techniques, and
physical activity in 5 children with shown
improvement in symptoms.
Surgical Care:
No surgical treatment is
indicated.
Consultations:
Due to the multifaceted
symptoms that present, refer the patient to other
disciplines for evaluation and treatment.
- Physical medicine and
rehabilitation
- Pulmonary medicine for
evaluation of sleep disorders that may cause
fatigue and presence of PLMS
Activity:
- The goal of an exercise
regime is to improve cardiovascular health and
musculoskeletal fitness through nonimpact
aerobic activity.
- Returning to normal activity
is imperative for the child who has stopped
sport and social activities due to pain because
this helps to modulate the pain. A physical
therapist may be extremely helpful in
establishing a reasonable exercise and activity
regime.
- Other modalities found to be
helpful in modulating pain include hypnotherapy,
cognitive-behavioral intervention, physical
therapy, and transcutaneous electrical nerve
stimulation (TENS). Using palliative measures to
treat symptoms and minimizing physical
disability is an important treatment mainstay.
- Maintaining the child's
physical conditioning is imperative in the
long-term outcome of FMS.
Typical medication regimens for
pediatric FMS primarily include skeletal muscle
relaxants and low-dose tricyclic antidepressants.
Some evidence reports that pain and symptom
management with nonsteroidal anti-inflammatory
drugs (NSAIDs) in combination with antidepressants
and nonaddictive analgesics is effective. The most
well-described medications used in the treatment
of pediatric FMS are low-dose antidepressants,
skeletal muscle relaxants, and NSAIDs. Low-dose
antidepressants, such as amitriptyline (Elavil),
and skeletal muscle relaxants, such as
cyclobenzaprine (Flexeril), help decrease the
hyperarousal mechanisms in FMS and, in turn, help
the child and adolescent sleep better. Both
medications are administered at bedtime or 1-2
hours before bedtime. Some debate exists in the
literature as to which medication is used
initially. Some authorities, such as Gedalia et
al, suggest use of cyclobenzaprine first in
treatment, while others begin medication therapy
with low-dose tricyclic antidepressants.
Depending on which medication is
started first, either skeletal muscle relaxants or
low-dose tricyclic antidepressants have been used
when the child or adolescent fails to respond to
the initial medication. An NSAID or acetaminophen
is used in conjunction with the muscle relaxants
or antidepressants in some cases that are
unresponsive to the mainstay therapies alone.
Active investigation is underway looking at the
potential role for S-adenosylmethionine (SAMe) and
the selective serotonin reuptake inhibitors (SSRIs)
in the adult population.
Drug Category: Tricyclic
antidepressants -- Help decrease pain
intensity and improve sleep quality. They
counteract the hyperarousal mechanism in FMS and
promote deeper sleep in children and adolescents.
Both medications are administered at bedtime or
1-2 hours before bedtime. SSRIs have been found
useful for treating chronic pain states.
Drug Name
|
Amitriptyline (Elavil) -- Used
for analgesia for certain chronic and
neuropathic pain. |
| Adult Dose |
30-100 mg PO hs |
| Pediatric
Dose |
<2 years: Not recommended
Children: 0.1 mg/kg PO qhs, may increase as
tolerated over 2-3 wk to 0.5-2 mg/kg hs
Adolescents: 5-40 mg qhs or 2 h before bedtime
|
|
Contraindications |
Documented hypersensitivity;
patient has taken MAOIs in past 14 d; has
history of seizures, cardiac arrhythmias,
glaucoma, and urinary retention |
|
Interactions |
Phenobarbital may decrease
effects; coadministration with CYP2D6 enzyme
system inhibitors (eg, cimetidine, quinidine)
may increase levels; inhibits hypotensive
effects of guanethidine; may interact with
thyroid medications, alcohol, CNS depressants,
barbiturates, and disulfiram |
| Pregnancy |
D - Unsafe in pregnancy
|
|
Precautions |
Caution in cardiac conduction
disturbances and history of hyperthyroidism,
renal or hepatic impairment; avoid using in
elderly patients |
Drug Category: Skeletal
muscle relaxants -- May act centrally by a
selective action on the CNS and are principally
used for relieving painful muscle spasms or
spasticity occurring in musculoskeletal and
neuromuscular disorders. Their mechanism of action
may be due, in part, to their CNS-depressant
activity.
Drug Name
|
Cyclobenzaprine (Flexeril) --
Helps decrease the hyperarousal mechanisms in
FMS and, in turn, helps the child sleep
better. Is structurally related to tricyclic
antidepressants and exhibits similar
pharmacological effects. Acts primarily on the
CNS at the brain-stem level. |
| Adult Dose |
20-40 mg/d PO divided bid/qid;
not to exceed 60 mg/d |
| Pediatric
Dose |
<15 years: Not established
>15 years: 5-30 mg PO qhs
|
|
Contraindications |
Documented hypersensitivity;
concomitant use of MAOIs or within 14 d after
their discontinuation; depression;
hyperthyroidism; urinary retention; cerebral
palsy; QT prolongation |
|
Interactions |
Coadministration with MAOIs and
tricyclic antidepressants may increase
toxicity; cyclobenzaprine may have additive
effect when used concurrently with
anticholinergics; effects of alcohol, CNS
depressants, and barbiturates may be enhanced
with cyclobenzaprine |
| Pregnancy |
B - Usually safe but benefits
must outweigh the risks. |
|
Precautions |
Urinary retention,
angle-closure glaucoma, increased intraocular
pressure |
Drug Category: Nonsteroidal
anti-inflammatory drugs -- Used for their
anti-inflammatory effects, analgesic, and
antipyretic effects. They are useful for the
relief of mild to moderate pain.
Drug Name
|
Ibuprofen (Motrin, Ibuprin) --
May help achieve analgesia when used in
combination with skeletal muscle relaxants or
tricyclic antidepressants. Inhibits
inflammatory reactions and pain by decreasing
prostaglandin synthesis. |
| Adult Dose |
200-400 mg PO q4-6h while
symptoms persist; not to exceed 3.2 g/d
|
| Pediatric
Dose |
4-10 mg/kg/dose PO q6-8h
|
|
Contraindications |
Documented hypersensitivity;
peptic ulcer disease, recent GI bleeding or
perforation, renal insufficiency, or high risk
of bleeding |
|
Interactions |
Coadministration with aspirin
increases risk of inducing serious NSAID-related
side effects; probenecid may increase
concentrations and, possibly, toxicity of
NSAIDs; may decrease effect of hydralazine,
captopril, and beta-blockers; may decrease
diuretic effects of furosemide and thiazides;
may increase PT when taking anticoagulants
(instruct patients to watch for signs of
bleeding); may increase risk of methotrexate
toxicity; phenytoin levels may be increased
when administered concurrently |
| Pregnancy |
B - Usually safe but benefits
must outweigh the risks. |
|
Precautions |
Category D in third trimester
of pregnancy; caution in congestive heart
failure, hypertension, and decreased renal and
hepatic function; caution in anticoagulation
abnormalities or during anticoagulant therapy |
Drug Name
|
Acetaminophen (Tylenol,
Feverall, Tempra) -- DOC for pain in patients
with documented hypersensitivity to aspirin or
NSAIDs, with upper GI disease, or who are
taking oral anticoagulants. |
| Adult Dose |
325-650 mg PO q4-6h or 1000 mg
tid/qid; not to exceed 4 g/d |
| Pediatric
Dose |
<12 years: 10-15 mg/kg/dose PO
q4-6h prn; not to exceed 2.6 g/d
>12 years: 325-650 mg PO q4h; not to exceed 5
doses in 24 h
|
|
Contraindications |
Documented hypersensitivity;
known G-6-P deficiency |
|
Interactions |
Rifampin can reduce analgesic
effects of acetaminophen; coadministration
with barbiturates, carbamazepine, hydantoins,
and isoniazid may increase hepatotoxicity
|
| Pregnancy |
B - Usually safe but benefits
must outweigh the risks. |
|
Precautions |
Hepatotoxicity possible with
overdose or chronic high doses; severe or
recurrent pain or high or continued fever may
indicate a serious illness; contained in many
OTC products and combined use with these
products may result in cumulative doses
exceeding recommended maximum dose |
Prognosis:
- Improvement in signs and
symptoms of FMS is likely in children and
adolescents. Gedalia and colleagues observed
children in a rheumatology clinic collecting
data on 50 children with an average follow-up
period of 18 months. They found that 60% of the
children had improved, 36% stayed the same, and
4% worsened compared to their initial
presentation. Nearly all of the children needed
to continue medications for up to 4 years after
initial presentation.
- Buskila and colleagues
studied FMS among children aged 9-15 years. Data
on 15 of the children showed that 73% (ie, 11 of
the 15) no longer met criteria for FMS at
30-months follow-up. The mean number of TPs and
the amount of force necessary to elicit pain at
each point showed significant improvement.
Complaints among the 4 children who still met
criteria for FMS included abdominal pain,
headache, paresthesias, morning stiffness, and
sleep disturbance. Additionally, 7 children were
observed who did not progress to the point of
meeting the full criteria over the 30 months,
and all 7 children had improved.
- Siegel and colleagues
observed 33 patients with a mean follow-up of
2.6 years. Improvement was observed in the
majority of patients during that follow-up time,
with all patients showing some positive response
to treatment. Given prognostic findings,
children with FMS as a whole are more likely to
have a favorable outcome than adults diagnosed
with FMS.
Patient Education:
- Health care providers are
responsible to educate children and families
about every aspect of FMS in an effort to
improve basic knowledge and coping mechanisms to
deal with the long-term aspects of the disease.
It is imperative for all involved to have full
understanding of the goals of treatment,
including exercise regimes, expectations of
medication therapy, and overriding aspects of
living with chronic pain. Successful treatment
and improved outcomes are enhanced when the
patient has a multifaceted approach to
treatment, including medical care, psychological
interventions, and physical therapy. Education
concerning every aspect of care and intervention
is a key to successful treatment of FMS.
- Summary: The understanding of
FMS in children still is in its infancy stage;
however, strides in both diagnosis and treatment
modalities have progressed in the past 10 years.
Because prevalence of FMS in children is
increasing, diagnosing the disorder early in its
course and then recommending a multidisciplinary
approach to the child's disorder is important.
An approach that involves support for the family
and specific recommendations for treatment may
help decrease the symptomatology and increase
the child's functioning.
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