Tuesday, October 5, 2010
Thursday, September 2, 2010
Cerebral Palsy Research with Low Level Laser Therapy
Bonnie L Brandes, MEd
Cerebral palsy (CP) is a postural and movement disorder caused by brain malformation or injury in the prenatal, perinatal (around birth), or
postnatal time period. The illness is non-progressive within the brain, but musculoskeletal and other tissues affected by the condition can
continue to weaken.
n the United States CP is the major disability affecting children’s functional development, with a prevalence of 1.5 to 2.0 cases per 1000. The
affected child is often unable to control motor functions normally. The illness is also associated with cognitive impairment and other
developmental disabilities. The child may have difficulties speaking, seeing, hearing, learning, and becoming independent depending on the
location and severity of the brain damage.
Many patients have normal intelligence, but communication skills are diminished because of speech and motor difficulties. It is therefore most
important not to underestimate the child’s intellect; he or she should be given every opportunity to learn. Lower life expectancy is related to
severe disabilities, such as mental retardation, necessity for tube feedings, and seizures, but otherwise, normal life expectancy may be expected.
Although a definitive cure does not yet exist, therapy, education, and technology can improve the child’s functioning and quality of life. The last
section of this paper reports on treatment successes using low level laser therapy.
Risk Factors
In approximately 25% to 40% of children with CP, birth was at less than 37 weeks gestation. Those with birth weight less than 1500 g (3.3
pounds) are at the highest risk. Damage to the periventricular white matter of the brain is the most common cause of CP in preterm infants. In
more than 30% of children with CP, etiology or risk factors are unknown.
Types of CP
CP can be classified according to the dominant motor characteristics—spastic, hypotonic, athetotic, dystonic, and ataxic, or by the pattern of limb
damage—monoplegia, diplegia, triplegia, hemiplegia, or quadriplegia.
Another way to classify the disease is by the two physiological types, pyramidal (spastic) and extra pyramidal (nonspastic). Pyramidal/spastic CP
is caused by damage in the corticospinal pathways of the brain. It accounts for 70% to 80% of all cases. In approximately 30% of cases, there is
cognitive impairment. The most conspicuous feature is increased muscle tone; other features include hyperreflexia, clonus (involuntary muscular
contractions), and persistent primitive reflexes, including Babinski.
Extrapyramidal (nonspastic) CP is caused by nerve damage outside of the pyramidal tracts in the basal ganglia or the cerebellum. There are two
subtypes, dyskinetic and ataxic. The disability is global, with inadequate regulation of muscle tone, poor control over posture, and abnormal
coordination.
Hypotonic CP, characterized by motor delays, is classified as CP only when myopathy and neuropathy have been ruled out as causes. Infants
with this CP have a marked decrease in muscle tone and a significant delay in motor milestones. Problems in feeding result from weak facial and
mouth muscles.
There are persistent primitive reflex patterns and hyperreflexia.
Different combinations of CP types are seen in some children.
Diagnosis of CP
Diagnosis is made mainly from clinical observation. Major signs include delayed motor milestones, abnormal neurological exam, persistence of
primitive reflexes, and abnormal postural reactions. No single abnormality is conclusive. Generally, the more severely ill child is diagnosed by the
age of six months, or later when symptoms are milder. The earlier the diagnosis, the sooner the child’s abnormal behavior can be understood by
the parents, allowing time to plan for long-term management.
Brain damage documented by cranial ultrasound, CAT scan, or MRI can facilitate diagnosis. When a child with identified lesions shows delayed
motor milestones, abnormal muscle tone, and retained primitive reflexes, an earlier diagnosis can be made. A series of examinations noting
progression is also useful in diagnosis.
Persistence of primitive reflexes is a hallmark of the condition. Most primitive reflexes are integrated within the first four to eight months of life, but
they may persist into adulthood in CP. Persistence of these reflexes adversely affects muscle tone and limb position, preventing the development
of voluntary motor movements. There may also be failure to develop protective reflexes such as parachute response or asymmetrical response.
In the neurological examination, the following are significant: abnormal muscle tone, weakness, deep tendon reflexes, clonus persisting beyond
12 months, and side to side asymmetries in muscle tone or functional abilities.
Prognosis
Predicting future abilities or disabilities is difficult at the time of diagnosis, leading to a “wait-and-see” approach.
Prediction of Ambulation:
How can the child’s ambulation potential be predicted? Sala and Grant present three categories of predictors: primitive reflexes and postural
reactions, gross motor skills, and type of CP. (1)
Persistence in these primitive reflexes by two years of age—asymmetrical tonic neck reflex (ATNR), symmetrical tonic neck reflex (STNR), Moro
reflex, tonic labyrinthine reflex, and to a lesser degree, positive supporting reflex—have been shown to be associated with inability to ambulate.
The same is true of abnormal postural reactions of foot placement and parachute reactions. (1)
In the category of gross motor skills, Jones and Morgan (2) report the following: In all types of CP, independent sitting by age 2 was found to be
reliably predictive for eventual walking. Achievement of head balance before nine months, ability to put weight on hands while prone and rolling
from supine to prone by 18 months, and motor control of crawling by 30 months were also found to be predictors of ambulation.
For type of CP as a predictor, Sala and Grant report the following: spastic hemiplegia, very good prognosis; spastic diplegia, good prognosis
(over 85%); spastic quadriplegia, much less favorable, with a wide range outcomes. (1)
Classifications of Severity:
In defining severity, a 5-level Gross Motor Function Measure has been developed by Rosenbaum, et al. (3) In Level 1 the patient walks without
restrictions (some limitations in advanced gross motor skills); Level II, able to walk without devices; Level III: able to walk with mobility devices;
Level IV, self mobility with limitations (power mobility); Level V, self mobility severely limited, even with technology. Parents often seek categories
of mild, moderate, and severe. Jones et al gives the following rule of thumb: mild – walking without assistance; moderate – requires medications,
bracing, and adaptive equipment to ambulate; severe – only mobile in a wheelchair. (2)
Success of LLLT (Low Level Laser Therapy) in Treating CP
Several clinical studies have been published concerning treatment of CP by soft laser, including application to acupuncture points on the scalp.
Scalp acupuncture was developed in China by Dr. Jiao Shunfa in 1971. Combining acupuncture in clinical practice and modern anatomy and
physiology, Dr. Shunfa developed a methodology based on 16 scalp areas. Studies have shown that scalp acupuncture increases cerebral
blood flow. (4) The World Health Organization reviewed and standardized scalp acupuncture in 1984.
In an early prospective study in 2001 - 2002, Fadaie and Khan performed a seven month treatment program of children with cerebral palsy and
other brain damage, applying laser therapy at scalp acupuncture points. (5) Seventeen boys and 12 girls, aged 5 months through 11 years,
were treated at the Fazal Hospital in Lahore, Pakistan.
Results showed significant improvement:
16 with brain damage and spasticity: six recovered fully, six had mild to moderate improvement, four had no improvement.
6 with brain damage and flaccidity or normal musculature: all six showed improved muscle function.
29 with brain damage and speech disorders: one showed great improvement; seven showed mild to moderate improvement; 21 showed little
improvement.
4 with brain damage and epileptic fits: Two showed improvement, but frequency of fits increased. It was concluded that treatment with laser
acupuncture should be continued, but with longer inter-session intervals and use of anti-epileptic medication. In a subsequent study in 2003 –
2004, no increase in epileptic fits was noted. (6)
10 children with cortical blindness: four recovered completely.
5 with impaired hearing or deafness: all five improved.
2 with autism: autism improved little, although other mental faculties improved.
Best results were noted in children less than two years old; the second best results were in ages less than 5. In ages 5 through 10, results were
poor. For the one subject 11 years old, results were very poor.
In a larger study conducted on children with cerebral palsy in 2003 through 2006 at Children’s Hospital in Lahore, Pakistan, 250 children were
treated with Aculaser therapy directed at acupuncture sites, including sites on the skull. (7) Aculaser therapy consists primarily of low level laser
therapy, acupuncture, and physical therapy. A minimum of 15 treatments for a minimum of six weeks was given to each of the children, who were
classified according to type of CP.
Results showed significant improvement:
171 children with spasticity and stiffness: 147 showed marked improvement (87% success).
126 with epileptic fits: 91 showed a significant reduction in intensity, frequency, and duration of fits (72% success).
48 children with cortical blindness: 30 showed improvement (63% success).
105 with hearing difficulty: 63 showed marked improvement (60% success).
190 with speech disorders, 122 showed improvement (64% success).
96 with hemiplegia, 71 showed improvement in movement, tone, and power (74% success).
76 with quadriplegia, 52 showed improvement in gross and fine motor functions (69% success).
58 with paraplegia of lower limbs, 44 showed improvement in weight bearing, standing, and movement (76% success).
Children given a break in treatment for one month to one year did not show any reversal of symptoms.
As in the earlier study, best results were noted in children less than two years old; the second best results were in ages less than 5.
Similar successful results were demonstrated by the same group in another study using Aculaser therapy. (6) For the scalp, a red diode laser
was used with a wavelength 650 nm, power of 5mW, and duration of 30 - 45 seconds. For body and auricular points, a HeNe laser was used with
a wavelength 632.8, power of 30 mW, and duration of 15 seconds.
It is concluded from these studies that the low level laser can be a valuable therapy in reducing the degree of disability in persons with CP.
Success Story:
Annie, age 6-1/2 years, received three Quantum Reflex Integration sessions during a three month period. The sessions included using the
Quantum Wave Laser with the "Unwinding" protocol (developed by Paul Weisbart), QRI laser acu-points, and QRI laser reflex integration
techniques for active primary reflexes. Primary reflexes that were addressed: Moro, Babinski, Leg Cross Flexion, Spinal Galant, Spinal Perez,
Hands Grasping, Automatic Gait, and Tonic Labyrinthine.
Annie's mother stimulated QRI acu-points manually as a home program times 3 - 4 times a week for approximately 30-40 minutes each session.
Mother does not have access to a laser between therapist sessions.
After the first session, Mom reported that Annie's ability to jump on a mini trampoline improved from 0 inches to 3 inches, with some assistance
for balance. Also after the first session, Annie's balance and endurance in walking without braces improved.
After three sessions, Mother reports that Annie has more confidence and better balance, and has met her first goal of jumping off the ground
independently. She has continued to improve in mobility and strength as demonstrated by her ability to walk around the yard without braces and
move a chair from one area to another. She now is able to pump her legs while swinging on a swing set, walk up steps of 4" without assistance
or holding on to a rail, and has increased the length of her stride.
Previous therapies include Physical Therapy and Occupational Therapy; current therapies and programs include QRI, Physical Therapy and
Occupational Therapy.
QRI Home Training Programs allow parents and therapists to successfully give their children possibilities, options, and successful
outcomes using soft lasers and reflex integration.
References:
1. Sala DA, Grant AD. Prognosis for Ambulation in Cerebral Palsy. Developmental Medicine and Child Neurology. 2008;37(11):1020-1026.
2. Jones MW, Morgan E, Shelton JE, Thorogood C. Cerebral Palsy: Introduction and Diagnosis (Part I). J Pediatr Health Care. 2007;21(3):146-152.
3. Rosenbaum PL, Walter SD, Hanna SE, Palisani RJ et al. Prognosis for gross motor function in cerebral palsy. JAMA. 2002;288:1357-1363.
4. Jing L, Jianhua X, Guirong D. Clinical study on effect of scalp acupuncture in treating acute cerebral hemorrhage. Chinese Journal of Integrative
Medicine. 1993;5(2):141.
5. Fadaie M; Khan M, Shah S. Application of Laser Acupuncture in Children with Cerebral Palsy. Majid Fadaie, Medical Acupuncturist, Lic AC (China) and
Malik Khan, pediatric neurosurgeon, Children’s Hospital, Lahore, Pakistan. 2002.
6. Anwar S, Khan M et al. Role of Aculaser Therapy in Cerebral Palsy Children. Anwar Shah’s First CP & Paralysis Clinic and Research Center. Lahore,
Pakistan. 2004.
7. Anwar S, Khan M et al. Treating cerebral palsy with Aculaser therapy. Anwar Shah’s First CP & Paralysis Clinic and Research Center, Lahore, Pakistan.
2006.
Cerebral palsy (CP) is a postural and movement disorder caused by brain malformation or injury in the prenatal, perinatal (around birth), or
postnatal time period. The illness is non-progressive within the brain, but musculoskeletal and other tissues affected by the condition can
continue to weaken.
n the United States CP is the major disability affecting children’s functional development, with a prevalence of 1.5 to 2.0 cases per 1000. The
affected child is often unable to control motor functions normally. The illness is also associated with cognitive impairment and other
developmental disabilities. The child may have difficulties speaking, seeing, hearing, learning, and becoming independent depending on the
location and severity of the brain damage.
Many patients have normal intelligence, but communication skills are diminished because of speech and motor difficulties. It is therefore most
important not to underestimate the child’s intellect; he or she should be given every opportunity to learn. Lower life expectancy is related to
severe disabilities, such as mental retardation, necessity for tube feedings, and seizures, but otherwise, normal life expectancy may be expected.
Although a definitive cure does not yet exist, therapy, education, and technology can improve the child’s functioning and quality of life. The last
section of this paper reports on treatment successes using low level laser therapy.
Risk Factors
In approximately 25% to 40% of children with CP, birth was at less than 37 weeks gestation. Those with birth weight less than 1500 g (3.3
pounds) are at the highest risk. Damage to the periventricular white matter of the brain is the most common cause of CP in preterm infants. In
more than 30% of children with CP, etiology or risk factors are unknown.
Types of CP
CP can be classified according to the dominant motor characteristics—spastic, hypotonic, athetotic, dystonic, and ataxic, or by the pattern of limb
damage—monoplegia, diplegia, triplegia, hemiplegia, or quadriplegia.
Another way to classify the disease is by the two physiological types, pyramidal (spastic) and extra pyramidal (nonspastic). Pyramidal/spastic CP
is caused by damage in the corticospinal pathways of the brain. It accounts for 70% to 80% of all cases. In approximately 30% of cases, there is
cognitive impairment. The most conspicuous feature is increased muscle tone; other features include hyperreflexia, clonus (involuntary muscular
contractions), and persistent primitive reflexes, including Babinski.
Extrapyramidal (nonspastic) CP is caused by nerve damage outside of the pyramidal tracts in the basal ganglia or the cerebellum. There are two
subtypes, dyskinetic and ataxic. The disability is global, with inadequate regulation of muscle tone, poor control over posture, and abnormal
coordination.
Hypotonic CP, characterized by motor delays, is classified as CP only when myopathy and neuropathy have been ruled out as causes. Infants
with this CP have a marked decrease in muscle tone and a significant delay in motor milestones. Problems in feeding result from weak facial and
mouth muscles.
There are persistent primitive reflex patterns and hyperreflexia.
Different combinations of CP types are seen in some children.
Diagnosis of CP
Diagnosis is made mainly from clinical observation. Major signs include delayed motor milestones, abnormal neurological exam, persistence of
primitive reflexes, and abnormal postural reactions. No single abnormality is conclusive. Generally, the more severely ill child is diagnosed by the
age of six months, or later when symptoms are milder. The earlier the diagnosis, the sooner the child’s abnormal behavior can be understood by
the parents, allowing time to plan for long-term management.
Brain damage documented by cranial ultrasound, CAT scan, or MRI can facilitate diagnosis. When a child with identified lesions shows delayed
motor milestones, abnormal muscle tone, and retained primitive reflexes, an earlier diagnosis can be made. A series of examinations noting
progression is also useful in diagnosis.
Persistence of primitive reflexes is a hallmark of the condition. Most primitive reflexes are integrated within the first four to eight months of life, but
they may persist into adulthood in CP. Persistence of these reflexes adversely affects muscle tone and limb position, preventing the development
of voluntary motor movements. There may also be failure to develop protective reflexes such as parachute response or asymmetrical response.
In the neurological examination, the following are significant: abnormal muscle tone, weakness, deep tendon reflexes, clonus persisting beyond
12 months, and side to side asymmetries in muscle tone or functional abilities.
Prognosis
Predicting future abilities or disabilities is difficult at the time of diagnosis, leading to a “wait-and-see” approach.
Prediction of Ambulation:
How can the child’s ambulation potential be predicted? Sala and Grant present three categories of predictors: primitive reflexes and postural
reactions, gross motor skills, and type of CP. (1)
Persistence in these primitive reflexes by two years of age—asymmetrical tonic neck reflex (ATNR), symmetrical tonic neck reflex (STNR), Moro
reflex, tonic labyrinthine reflex, and to a lesser degree, positive supporting reflex—have been shown to be associated with inability to ambulate.
The same is true of abnormal postural reactions of foot placement and parachute reactions. (1)
In the category of gross motor skills, Jones and Morgan (2) report the following: In all types of CP, independent sitting by age 2 was found to be
reliably predictive for eventual walking. Achievement of head balance before nine months, ability to put weight on hands while prone and rolling
from supine to prone by 18 months, and motor control of crawling by 30 months were also found to be predictors of ambulation.
For type of CP as a predictor, Sala and Grant report the following: spastic hemiplegia, very good prognosis; spastic diplegia, good prognosis
(over 85%); spastic quadriplegia, much less favorable, with a wide range outcomes. (1)
Classifications of Severity:
In defining severity, a 5-level Gross Motor Function Measure has been developed by Rosenbaum, et al. (3) In Level 1 the patient walks without
restrictions (some limitations in advanced gross motor skills); Level II, able to walk without devices; Level III: able to walk with mobility devices;
Level IV, self mobility with limitations (power mobility); Level V, self mobility severely limited, even with technology. Parents often seek categories
of mild, moderate, and severe. Jones et al gives the following rule of thumb: mild – walking without assistance; moderate – requires medications,
bracing, and adaptive equipment to ambulate; severe – only mobile in a wheelchair. (2)
Success of LLLT (Low Level Laser Therapy) in Treating CP
Several clinical studies have been published concerning treatment of CP by soft laser, including application to acupuncture points on the scalp.
Scalp acupuncture was developed in China by Dr. Jiao Shunfa in 1971. Combining acupuncture in clinical practice and modern anatomy and
physiology, Dr. Shunfa developed a methodology based on 16 scalp areas. Studies have shown that scalp acupuncture increases cerebral
blood flow. (4) The World Health Organization reviewed and standardized scalp acupuncture in 1984.
In an early prospective study in 2001 - 2002, Fadaie and Khan performed a seven month treatment program of children with cerebral palsy and
other brain damage, applying laser therapy at scalp acupuncture points. (5) Seventeen boys and 12 girls, aged 5 months through 11 years,
were treated at the Fazal Hospital in Lahore, Pakistan.
Results showed significant improvement:
16 with brain damage and spasticity: six recovered fully, six had mild to moderate improvement, four had no improvement.
6 with brain damage and flaccidity or normal musculature: all six showed improved muscle function.
29 with brain damage and speech disorders: one showed great improvement; seven showed mild to moderate improvement; 21 showed little
improvement.
4 with brain damage and epileptic fits: Two showed improvement, but frequency of fits increased. It was concluded that treatment with laser
acupuncture should be continued, but with longer inter-session intervals and use of anti-epileptic medication. In a subsequent study in 2003 –
2004, no increase in epileptic fits was noted. (6)
10 children with cortical blindness: four recovered completely.
5 with impaired hearing or deafness: all five improved.
2 with autism: autism improved little, although other mental faculties improved.
Best results were noted in children less than two years old; the second best results were in ages less than 5. In ages 5 through 10, results were
poor. For the one subject 11 years old, results were very poor.
In a larger study conducted on children with cerebral palsy in 2003 through 2006 at Children’s Hospital in Lahore, Pakistan, 250 children were
treated with Aculaser therapy directed at acupuncture sites, including sites on the skull. (7) Aculaser therapy consists primarily of low level laser
therapy, acupuncture, and physical therapy. A minimum of 15 treatments for a minimum of six weeks was given to each of the children, who were
classified according to type of CP.
Results showed significant improvement:
171 children with spasticity and stiffness: 147 showed marked improvement (87% success).
126 with epileptic fits: 91 showed a significant reduction in intensity, frequency, and duration of fits (72% success).
48 children with cortical blindness: 30 showed improvement (63% success).
105 with hearing difficulty: 63 showed marked improvement (60% success).
190 with speech disorders, 122 showed improvement (64% success).
96 with hemiplegia, 71 showed improvement in movement, tone, and power (74% success).
76 with quadriplegia, 52 showed improvement in gross and fine motor functions (69% success).
58 with paraplegia of lower limbs, 44 showed improvement in weight bearing, standing, and movement (76% success).
Children given a break in treatment for one month to one year did not show any reversal of symptoms.
As in the earlier study, best results were noted in children less than two years old; the second best results were in ages less than 5.
Similar successful results were demonstrated by the same group in another study using Aculaser therapy. (6) For the scalp, a red diode laser
was used with a wavelength 650 nm, power of 5mW, and duration of 30 - 45 seconds. For body and auricular points, a HeNe laser was used with
a wavelength 632.8, power of 30 mW, and duration of 15 seconds.
It is concluded from these studies that the low level laser can be a valuable therapy in reducing the degree of disability in persons with CP.
Success Story:
Annie, age 6-1/2 years, received three Quantum Reflex Integration sessions during a three month period. The sessions included using the
Quantum Wave Laser with the "Unwinding" protocol (developed by Paul Weisbart), QRI laser acu-points, and QRI laser reflex integration
techniques for active primary reflexes. Primary reflexes that were addressed: Moro, Babinski, Leg Cross Flexion, Spinal Galant, Spinal Perez,
Hands Grasping, Automatic Gait, and Tonic Labyrinthine.
Annie's mother stimulated QRI acu-points manually as a home program times 3 - 4 times a week for approximately 30-40 minutes each session.
Mother does not have access to a laser between therapist sessions.
After the first session, Mom reported that Annie's ability to jump on a mini trampoline improved from 0 inches to 3 inches, with some assistance
for balance. Also after the first session, Annie's balance and endurance in walking without braces improved.
After three sessions, Mother reports that Annie has more confidence and better balance, and has met her first goal of jumping off the ground
independently. She has continued to improve in mobility and strength as demonstrated by her ability to walk around the yard without braces and
move a chair from one area to another. She now is able to pump her legs while swinging on a swing set, walk up steps of 4" without assistance
or holding on to a rail, and has increased the length of her stride.
Previous therapies include Physical Therapy and Occupational Therapy; current therapies and programs include QRI, Physical Therapy and
Occupational Therapy.
QRI Home Training Programs allow parents and therapists to successfully give their children possibilities, options, and successful
outcomes using soft lasers and reflex integration.
References:
1. Sala DA, Grant AD. Prognosis for Ambulation in Cerebral Palsy. Developmental Medicine and Child Neurology. 2008;37(11):1020-1026.
2. Jones MW, Morgan E, Shelton JE, Thorogood C. Cerebral Palsy: Introduction and Diagnosis (Part I). J Pediatr Health Care. 2007;21(3):146-152.
3. Rosenbaum PL, Walter SD, Hanna SE, Palisani RJ et al. Prognosis for gross motor function in cerebral palsy. JAMA. 2002;288:1357-1363.
4. Jing L, Jianhua X, Guirong D. Clinical study on effect of scalp acupuncture in treating acute cerebral hemorrhage. Chinese Journal of Integrative
Medicine. 1993;5(2):141.
5. Fadaie M; Khan M, Shah S. Application of Laser Acupuncture in Children with Cerebral Palsy. Majid Fadaie, Medical Acupuncturist, Lic AC (China) and
Malik Khan, pediatric neurosurgeon, Children’s Hospital, Lahore, Pakistan. 2002.
6. Anwar S, Khan M et al. Role of Aculaser Therapy in Cerebral Palsy Children. Anwar Shah’s First CP & Paralysis Clinic and Research Center. Lahore,
Pakistan. 2004.
7. Anwar S, Khan M et al. Treating cerebral palsy with Aculaser therapy. Anwar Shah’s First CP & Paralysis Clinic and Research Center, Lahore, Pakistan.
2006.
Sunday, July 18, 2010
Sweet Vallen, juvenile rheumatoid arthritis
Flare and swaleen a week a go
A week after. No flare, reduce edema. Transcutaneus and scann mode low level laser.
She just 6 y o. Came to me with swollen at all fingers and both knee. Yesterday, the 2nd laser application. At the 1st , just transcutaneus for 1 hour. Yestesday I found the finger and knees not flare anymore. I gave ½ hour transcutaneus, 15 minutes scan for both knee, and the fingers. At the end of the treatment, she could moved her knee and fingers without tears.
I couldn’t ask how her pain number by pain scale. I’ll try next time.
Thursday, July 15, 2010
Juvenile Rheumatoid Arthritis: Follow-up
Juvenile Rheumatoid Arthritis: Follow-up
Author: C Egla Rabinovich, MD, MPH, Associate Professor and Co-Division Chief, Department of Pediatrics, Division of Pediatric Rheumatology, Duke University Medical Center
Contributor Information and Disclosures
Updated: Apr 7, 2010
Follow-up
Further Inpatient Care
Further inpatient care is required for persisting fevers of unknown origin or when children with known juvenile idiopathic arthritis (JIA) have severe exacerbation of disease. Admit for evaluation any child who loses the ability to walk for unknown reasons. Development of pericarditis in children with systemic juvenile idiopathic arthritis is usually an indication for admission.
Further Outpatient Care
Patients may have a gradually responsive disease (particularly those with pauciarticular juvenile idiopathic arthritis).
A small number of patients with pauciarticular juvenile idiopathic arthritis develop aggressive arthritis confined to a single joint; such patients may require more intensive medical treatment and physical therapy.
Some patients with polyarticular juvenile idiopathic arthritis demonstrate rapid response to treatment; however, most have prolonged courses, requiring frequent adjustments in medical and nonmedical therapy. Some have sufficient problems with activities of daily living, and they may benefit from courses of outpatient (and sometimes inpatient) rehabilitation.
Inpatient & Outpatient Medications
See Medication.
Transfer
Consider outpatient evaluation in a pediatric rheumatology center for all patients with known and suspected disease. Inpatient care for individuals with intercurrent illnesses may best be carried out at local hospitals; complications from juvenile idiopathic arthritis usually indicate transfer to a hospital with a pediatric rheumatology center.
Treatment of macrophage activation syndrome (MAS) is a medical emergency and should be done by physicians familiar with this complication.
Deterrence/Prevention
No prevention methods are known. The best means of deterrence is compliance with recommended treatment. As many as one half of patients may not comply with every detail of recommended treatment. Persisting noncompliance is a problem that increases risk of morbidity. Parents of such patients often admit noncompliance only to the child's primary care physician, rather than to a pediatric subspecialty team. The continued monitoring of compliance by the primary care physician, together with continuing communication between the pediatric subspecialist and primary physician, is an important part of the treatment of children with juvenile idiopathic arthritis and any chronic illness.
Complications
The following complications may occur:
• Systemic-onset juvenile idiopathic arthritis
o Pericarditis (patients often presenting with orthopnea and responsive to intravenous corticosteroid treatment)
o Hemolytic anemia
o Disseminated intravascular coagulopathy (DIC), often present at a low level of activity: The levels of D-dimer and fibrinogen may be elevated; their return to reference range levels is observed with successful treatment.
o MAS
This is a rare, but important, complication, in which numbers of all 3 bloodlines become rapidly decreased. Hypofibrinogenemia, thrombocytopenia, and elevated aspartate aminotransferase levels are hallmarks.
Hypotension, CNS disease, and marked hepatosplenomegaly may be noted as complications of a release of massive amounts of cytokines.
Bone marrow aspiration may reveal histiocytic consumption of bone marrow precursors, which confirms the diagnosis and excludes malignancy. One does not need to see the histiocytic consumption for diagnosis of MAS.
MAS often responds to cyclosporin A, and some case reports have detailed response to anakinra.
o Endarteritis resulting in circulatory compromise of the digits with threatened autoamputation
This complication is even more rare than MAS.
Central administration of prostaglandin E1 may be of potential benefit, similar to its use for patients with scleroderma and endarteritis.
• Pauciarticular juvenile idiopathic arthritis
o Knee flexion contractures: This complication requires splinting at night, in addition to medical treatment, to restore range of motion, allow recovery of muscle strength, and avoid subluxation of the joint. Intra-articular corticosteroid injection should be strongly considered.
o Uveitis
Often asymptomatic, patients are typically young girls who have positive levels of antinuclear antibody (ANA).
In such patients, evaluation using a slit-lamp examination by a pediatric ophthalmologist every 4 months can detect early disease.
Treatment with topical corticosteroid medication and with mydriatic agents (to prevent closed-angle glaucoma) often can prevent progression of disease to development of calcium deposition in the lens (band keratopathy) and adhesions of the iris to the lens (posterior synechiae), in which an irregular pupillary margin develops.
Such complications may herald a chronic active disease, in which vision is threatened; immunosuppressive agents, such as methotrexate or cyclosporin, may help to control chronic uveitis. Infliximab can be effective in some patients who are resistant to immunosuppressive agents.
o Leg length discrepancy (can result from neovascularization of growth plates of an affected knee)
The problem may not be detected in patients with a knee flexion contracture until the contracture is corrected.
Treatment consists of a shoe lift on the nonaffected side.
• Polyarticular juvenile idiopathic arthritis
o Skeletal abnormalities - Increased size of epiphyses, accelerated bone age, narrowed joint spaces, swan-neck and/or boutonniere deformities, and joint subluxation
o Cervical spine involvement
Difficulty flexing the spine may create a problem for intubation prior to surgery; inform anesthesiologists of the patient's diagnosis. Screening cervical spine radiography (in both flexion and extension) may help screen for potential difficulties during induction of anesthesia.
High-level subluxation is a potential complication.
Prognosis
Some studies suggest that many children with juvenile idiopathic arthritis can lead productive lives. However, other studies suggest many patients, particularly those with polyarticular disease, may have problems with active disease throughout adulthood, with sustained remission attained in a minority of patients. Early hip or wrist involvement, symmetric disease (even in pauciarticular patients), presence of rheumatoid factor, and prolonged active disease have been associated with poor long-term outcomes.
Children with systemic disease tend to have either complete responsiveness to medical therapy or development of a polyarticular course that tends to be refractive to medical treatment, with disease persisting into adulthood.
Most children with pauciarticular disease demonstrate eventual permanent remission; a small number progress to persisting polyarticular disease.
Patient Education
Educating the patient, family, and school personnel (eg, classroom teachers, physical education teachers, nurses) about juvenile idiopathic arthritis and its presentation, treatment, and potential effects is continually necessary. Members of the pediatric rheumatology team in pediatric rheumatology clinics are the best educators about juvenile idiopathic arthritis. Another important source of information is the American Juvenile Arthritis Organization, a council of the Arthritis Foundation.
For excellent patient education resources, visit eMedicine's Arthritis Center. Also, see eMedicine's patient education articles Juvenile Rheumatoid Arthritis and Understanding Rheumatoid Arthritis Medications.
Miscellaneous
Medicolegal Pitfalls
The major medicolegal pitfall lies in diagnosing juvenile idiopathic arthritis (JIA) when another problem exists (eg, infection, malignancy, orthopedic problem). Whenever possible, referral to a pediatric rheumatologist can help address this issue. Careful attention to presenting history and initial physical examination findings can lower the likelihood of such a pitfall. However, the chance of such a pitfall can never be eliminated completely.
At the time of diagnosis, inform parents and/or caregivers of the possible need to revise the diagnosis of juvenile idiopathic arthritis should new symptoms, physical findings, or unusual laboratory results develop.
Special Concerns
Chronic illness imposes burdens on families, who may vary in their abilities to cope. Social workers can help provide assessment and assist families in finding resources (including counseling). Remind parents and/or caregivers to bring all questions to the pediatric rheumatology team, who can often help. Any unusual symptom may signal a new complication of disease or adverse effect of medication. In the current health care environment, managed care can result in initial denial of services (eg, physical therapy), resulting in delays in treatment with subsequent morbidity. Advocacy by the primary care physician and pediatric rheumatologist can help point out the need for such services.
Acknowledgments
The authors and editors of eMedicine gratefully acknowledge Michael L Miller, MD, to the original writing and development of this article.
Author: C Egla Rabinovich, MD, MPH, Associate Professor and Co-Division Chief, Department of Pediatrics, Division of Pediatric Rheumatology, Duke University Medical Center
Contributor Information and Disclosures
Updated: Apr 7, 2010
Follow-up
Further Inpatient Care
Further inpatient care is required for persisting fevers of unknown origin or when children with known juvenile idiopathic arthritis (JIA) have severe exacerbation of disease. Admit for evaluation any child who loses the ability to walk for unknown reasons. Development of pericarditis in children with systemic juvenile idiopathic arthritis is usually an indication for admission.
Further Outpatient Care
Patients may have a gradually responsive disease (particularly those with pauciarticular juvenile idiopathic arthritis).
A small number of patients with pauciarticular juvenile idiopathic arthritis develop aggressive arthritis confined to a single joint; such patients may require more intensive medical treatment and physical therapy.
Some patients with polyarticular juvenile idiopathic arthritis demonstrate rapid response to treatment; however, most have prolonged courses, requiring frequent adjustments in medical and nonmedical therapy. Some have sufficient problems with activities of daily living, and they may benefit from courses of outpatient (and sometimes inpatient) rehabilitation.
Inpatient & Outpatient Medications
See Medication.
Transfer
Consider outpatient evaluation in a pediatric rheumatology center for all patients with known and suspected disease. Inpatient care for individuals with intercurrent illnesses may best be carried out at local hospitals; complications from juvenile idiopathic arthritis usually indicate transfer to a hospital with a pediatric rheumatology center.
Treatment of macrophage activation syndrome (MAS) is a medical emergency and should be done by physicians familiar with this complication.
Deterrence/Prevention
No prevention methods are known. The best means of deterrence is compliance with recommended treatment. As many as one half of patients may not comply with every detail of recommended treatment. Persisting noncompliance is a problem that increases risk of morbidity. Parents of such patients often admit noncompliance only to the child's primary care physician, rather than to a pediatric subspecialty team. The continued monitoring of compliance by the primary care physician, together with continuing communication between the pediatric subspecialist and primary physician, is an important part of the treatment of children with juvenile idiopathic arthritis and any chronic illness.
Complications
The following complications may occur:
• Systemic-onset juvenile idiopathic arthritis
o Pericarditis (patients often presenting with orthopnea and responsive to intravenous corticosteroid treatment)
o Hemolytic anemia
o Disseminated intravascular coagulopathy (DIC), often present at a low level of activity: The levels of D-dimer and fibrinogen may be elevated; their return to reference range levels is observed with successful treatment.
o MAS
This is a rare, but important, complication, in which numbers of all 3 bloodlines become rapidly decreased. Hypofibrinogenemia, thrombocytopenia, and elevated aspartate aminotransferase levels are hallmarks.
Hypotension, CNS disease, and marked hepatosplenomegaly may be noted as complications of a release of massive amounts of cytokines.
Bone marrow aspiration may reveal histiocytic consumption of bone marrow precursors, which confirms the diagnosis and excludes malignancy. One does not need to see the histiocytic consumption for diagnosis of MAS.
MAS often responds to cyclosporin A, and some case reports have detailed response to anakinra.
o Endarteritis resulting in circulatory compromise of the digits with threatened autoamputation
This complication is even more rare than MAS.
Central administration of prostaglandin E1 may be of potential benefit, similar to its use for patients with scleroderma and endarteritis.
• Pauciarticular juvenile idiopathic arthritis
o Knee flexion contractures: This complication requires splinting at night, in addition to medical treatment, to restore range of motion, allow recovery of muscle strength, and avoid subluxation of the joint. Intra-articular corticosteroid injection should be strongly considered.
o Uveitis
Often asymptomatic, patients are typically young girls who have positive levels of antinuclear antibody (ANA).
In such patients, evaluation using a slit-lamp examination by a pediatric ophthalmologist every 4 months can detect early disease.
Treatment with topical corticosteroid medication and with mydriatic agents (to prevent closed-angle glaucoma) often can prevent progression of disease to development of calcium deposition in the lens (band keratopathy) and adhesions of the iris to the lens (posterior synechiae), in which an irregular pupillary margin develops.
Such complications may herald a chronic active disease, in which vision is threatened; immunosuppressive agents, such as methotrexate or cyclosporin, may help to control chronic uveitis. Infliximab can be effective in some patients who are resistant to immunosuppressive agents.
o Leg length discrepancy (can result from neovascularization of growth plates of an affected knee)
The problem may not be detected in patients with a knee flexion contracture until the contracture is corrected.
Treatment consists of a shoe lift on the nonaffected side.
• Polyarticular juvenile idiopathic arthritis
o Skeletal abnormalities - Increased size of epiphyses, accelerated bone age, narrowed joint spaces, swan-neck and/or boutonniere deformities, and joint subluxation
o Cervical spine involvement
Difficulty flexing the spine may create a problem for intubation prior to surgery; inform anesthesiologists of the patient's diagnosis. Screening cervical spine radiography (in both flexion and extension) may help screen for potential difficulties during induction of anesthesia.
High-level subluxation is a potential complication.
Prognosis
Some studies suggest that many children with juvenile idiopathic arthritis can lead productive lives. However, other studies suggest many patients, particularly those with polyarticular disease, may have problems with active disease throughout adulthood, with sustained remission attained in a minority of patients. Early hip or wrist involvement, symmetric disease (even in pauciarticular patients), presence of rheumatoid factor, and prolonged active disease have been associated with poor long-term outcomes.
Children with systemic disease tend to have either complete responsiveness to medical therapy or development of a polyarticular course that tends to be refractive to medical treatment, with disease persisting into adulthood.
Most children with pauciarticular disease demonstrate eventual permanent remission; a small number progress to persisting polyarticular disease.
Patient Education
Educating the patient, family, and school personnel (eg, classroom teachers, physical education teachers, nurses) about juvenile idiopathic arthritis and its presentation, treatment, and potential effects is continually necessary. Members of the pediatric rheumatology team in pediatric rheumatology clinics are the best educators about juvenile idiopathic arthritis. Another important source of information is the American Juvenile Arthritis Organization, a council of the Arthritis Foundation.
For excellent patient education resources, visit eMedicine's Arthritis Center. Also, see eMedicine's patient education articles Juvenile Rheumatoid Arthritis and Understanding Rheumatoid Arthritis Medications.
Miscellaneous
Medicolegal Pitfalls
The major medicolegal pitfall lies in diagnosing juvenile idiopathic arthritis (JIA) when another problem exists (eg, infection, malignancy, orthopedic problem). Whenever possible, referral to a pediatric rheumatologist can help address this issue. Careful attention to presenting history and initial physical examination findings can lower the likelihood of such a pitfall. However, the chance of such a pitfall can never be eliminated completely.
At the time of diagnosis, inform parents and/or caregivers of the possible need to revise the diagnosis of juvenile idiopathic arthritis should new symptoms, physical findings, or unusual laboratory results develop.
Special Concerns
Chronic illness imposes burdens on families, who may vary in their abilities to cope. Social workers can help provide assessment and assist families in finding resources (including counseling). Remind parents and/or caregivers to bring all questions to the pediatric rheumatology team, who can often help. Any unusual symptom may signal a new complication of disease or adverse effect of medication. In the current health care environment, managed care can result in initial denial of services (eg, physical therapy), resulting in delays in treatment with subsequent morbidity. Advocacy by the primary care physician and pediatric rheumatologist can help point out the need for such services.
Acknowledgments
The authors and editors of eMedicine gratefully acknowledge Michael L Miller, MD, to the original writing and development of this article.
Juvenile Rheumatoid Arthritis: Treatment & Medication
Juvenile Rheumatoid Arthritis: Treatment & Medication
Updated: Apr 7, 2010
Treatment
Medical Care
Medical care of children with juvenile rheumatoid arthritis (JRA) must be provided in the context of a team-based approach, considering all aspects of their illness (eg, physical functioning in school, psychological adjustment to disease). Using medications in the absence of an appropriate physical therapy program and attention to problematic social issues of the family is not successful. Success of medications is monitored best with repeated physical examinations and history. Both the number of joints involved and the duration of morning stiffness should demonstrate continued decrease, with elimination reflecting success.
Surgical Care
Surgery is not usually needed; however, some children with persisting pauciarticular juvenile rheumatoid arthritis, despite medical treatment, may benefit from intra-articular steroid injection. Such injections may also be effective in treating temporomandibular arthritis in children with polyarticular juvenile rheumatoid arthritis. Usually, delay joint replacement (often of the hips, in patients with polyarticular juvenile rheumatoid arthritis) until bone growth has completed, which is reflected by epiphyseal closure. The consistent effective use of medical treatment has consigned synovectomy to a rarely used intervention. Large leg length discrepancies may need surgical treatment.
Consultations
The subspecialty team includes the following:
- Pediatric rheumatologist (when available)
- Nurses (who help with education)
- Physical and occupational therapists: Nonmedical approaches (eg, physical and occupational therapy) are an important part of treatment. At presentation, arthritis may be so active as to preclude the use of an aggressive program of muscle strengthening. Nevertheless, the use of pain modalities during this period may permit the gradual introduction of an active program of exercises and stretching.
- Social workers: Social work evaluation helps to determine how well each family is coping with their child's disease in terms of emotional and financial resources. Social workers can offer invaluable guidance for helping children to maintain healthy relationships both within their families and at school. Transition programs for adolescents with arthritis can help prepare them for higher education and future vocations.
Pediatric ophthalmologists help provide slit-lamp examinations to exclude uveitis. Pediatric orthopedic surgeons can offer consultation when orthopedic diagnoses are being considered. The development of profound anemia or a drop in 2 or more cell lines may require the help of a pediatric hematologist. A pediatric gastroenterologist may help with hepatic abnormalities or symptoms suggesting inflammatory bowel disease.
Diet
No specific diet helps in the treatment of juvenile rheumatoid arthritis. However, because active juvenile rheumatoid arthritis has been associated with decreased osteoblastic activity and a risk of osteopenia, encourage the inclusion of at least 3 servings of calcium-rich foods each day. Consider behavioral intervention when poor calcium intake persists.
Activity
Encourage patients to be as active as possible. Except in individuals with severe systemic disease, bed rest is not a part of the treatment. In fact, the more active the patient the better the long-term prognosis is. Children may experience increased pain during routine physical activities. As a result, these children must be allowed to self-limit their activities, particularly during physical education classes. A consistent physical therapy program, with attention to stretching exercises, pain modalities, joint protection, and home exercises, can help ensure that patients are as active as possible.
Medication
Classes of medications are suggested below, and specific drugs are covered in detail by category. See the therapeutic algorithm below
One set of suggested algorithms for the treatment of patients with juvenile arthritis. This should not be considered dogmatic because treatment is not standardized and remains empiric and, at times, controversial.
Nonsteroidal anti-inflammatory drugs (NSAIDs) are used to treat all subtypes of juvenile rheumatoid arthritis (JRA). They may help with pain and decrease swelling. They are often used in conjunction with second-line agents. These medications are effective because of inhibition of prostaglandin synthesis. Naproxen is listed below as an example of an NSAID used in treatment; other NSAIDs commonly used include ibuprofen, tolmetin, diclofenac, and indomethacin. In addition, sulfasalazine is sometimes used as a second anti-inflammatory drug in some children with persisting pauciarticular and polyarticular disease. Its use may be considered as an intermediate step prior to adding a second-line drug such as methotrexate (MTX).
Aspirin is no longer the drug of first choice because of the increased frequency of gastric toxicity and hepatotoxicity when compared to other NSAID medications, along with its association with Reye syndrome. The discovery that cyclooxygenase (COX) in gastric and intestinal endothelium (ie, COX-1) is different in structure from that in leukocytes (ie, COX-2) has led to the development of anti-inflammatory drugs specific for COX-2. COX-2 inhibitors have been found to be effective in treatment of adults with rheumatoid arthritis. Studies of COX-2 inhibitors in persons with juvenile idiopathic arthritis are underway. Besides the benefit of greatly reducing gastric toxicity (although hepatotoxicity remains a possible adverse event), COX-2 inhibitors do not inhibit platelet aggregation. Thus, these agents may find a role in the treatment of inflammatory conditions in which a bleeding diathesis is a potential problem, such as in the postoperative setting.
NSAIDs alone are usually adequate for treatment of pauciarticular disease. However, an aggressive arthritis sometimes develops in this subtype, requiring the need to add a second-line drug. Various second-line drugs have been used in addition to first-line NSAIDs. Gold salt injections were used until approximately 15 years ago, when studies by the Pediatric Rheumatology Collaborative Study Group demonstrated the efficacy of oral (PO) MTX; these injections are not currently used for most children. Subsequent studies have demonstrated that some children with polyarticular arthritis unresponsive to PO MTX benefit from subcutaneous (SC) or intramuscular (IM) administration. The use of high-dose intravenous (IV) steroids as a therapeutic bridge in selected patients has been beneficial in some patients, particularly during an early period before MTX may have a full therapeutic effect.
Etanercept, a biologic agent administered SC twice weekly and containing a receptor to tumor necrosis factor (TNF) ligated to an Fc portion of immunoglobulin, has been found to be effective in controlling polyarticular arthritis not controlled by conventional medical treatment. Adalimumab is another anti-TNF agent now approved for use in juvenile idiopathic arthritis. These medications are for those children treated by pediatric rheumatology centers who are unresponsive to treatment including conventional second-line drugs.
Abatacept is a biological immune modulator with a delayed onset of action (4 mo) that may be useful in polyarticular disease.
Finally, the treatment of systemic juvenile idiopathic arthritis may require, in addition to treatment with NSAIDs, the careful use of either PO or high-dose pulse IV corticosteroids. Such treatment is best reserved for patients in whom definite arthritis has developed to avoid premature treatment in a patient who may prove to have a disease other than juvenile rheumatoid arthritis. Corticosteroids may be avoided with the use of anakinra, which is relatively new, inhibits interleukin (IL)-1 activity, and appears to have unique efficacy on the systemic signs and symptoms of systemic juvenile idiopathic arthritis. Rilonacept (anti-IL-1) may be an alternative treatment that is being studied, as is tocilizumab, which is anti-IL-6. Thalidomide has also been reported to be useful in these children.
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