by Dr. Vinaya Kunjir
The rheumatic diseases of childhood represent a diverse group, majority of them result from a combination of genetic
predisposition,autoimmunity and environmental factors. Diseases like infectious arthritis and acute rheumatic fever
result from exposure to infectious agents.The examining physician must take a careful history & do a proper physical examination for a correct diagnosis of childhood arthritis. He must determine whether inflammation is present or not. If present, whether it is acute or chronic, whether it is articular or periarticular. Based on these findings, the
common forms of childhood arthritis are as follows.
No inflammation present :
Growing pains :
These are the most commonly occurring pains & often misdiagnosed as childhood rheumatism. The
syndrome of growing pains is seen in young children (age group 4-5 yrs.). It occurs in popliteal fossa (region posterior to knee joint) & calves and occurs only at night. It is relived by gentle massage & does not require any specific therapy.
Psychogenic rheumatism :
some children complain of joint pains & fatigue inspite of normal physical & laboratory evaluation. A child who is unable to attend school or participate in normal activities is worrisome. In such cases, the complaints are generally due to trouble in families. The physician should recommend psychological counseling for such children & their families.
Inflammation present :
The inflammation can be periarticular or articular.
Periarticular inflammation : It is the inflammation of soft tissue, tendons,ligaments, bursae, etc. Acute periarticular pain may result from orthopedic disorders such as fracture, osteomyelitis, neoplastic disorders like leukemia, lymphoma and rheumatic disorders such as enthesitis & heel pains in juvenile seronegative spondyloarthropathies.
Articular Inflammation
1) Septic arthritis – Certain infectious agents like staphylococci, streptococci, haemophilus influenza cause pain, swelling & tenderness in the joints accompanied by fever & an elevated ESR. It typically presents with a single, inflamed joint.
2) Reactive Arthritis – It may accompany or follow any bacterial, viral or fungal infection. Typically the child presents with an upper respiratory tract infection in the prior evening. The following morning the child is unable to walk due to hip joint inflammation. There is low-grade fever & ESR is usually not raised. The joint symptoms improve within few hours to few days following the treatment of the pre-onset infection.
3) Post- streptococcal reactive arthritis – Children with arthritis & elevated ESRs following a documented streprococcal infection should receive rheumatic fever prophylaxis.
Diseases with unique manifestations in childhood :
Juvenile Idiopathic Arthritis (JIA) – It is a heterogeneous group of chronic inflammatory arthritis that begins in childhood & is quite distinct from adult rheumatoid arthritis. The age of onset is defined as before 16 years and duration of arthritis is a minimum of 6 weeks in at least one joint. With a view to identify homogenous group of children & facilitate treatment,immunogenetics, epidemi-ology & other basic sciences for research, the children with JIA were classified into seven categories.
1) Systemic arthritis (SA) – The children typically present with arthritis and daily fever of at least 2 weeks duration accompanied by one of the following features –
- Evanescent, non fixed, erythematous rash
- Generalized Lymph node enlargement
- Hepatomegaly or splenomegaly
- Serositis
2) Oligo arthritis (OA) – involves arthritis affecting I – 4 joints during first 6 months of disease. It has 2 subsets.
- Persistent OA – affects not more than 4 joints throughout the disease course.
- Extended OA – affects a cumulative total of 5 joints or more after the first 6 months of disease. Young girls of oligarticular JIA, who are ANA (positive) are at a greater risk of developing complicating eye diseases (iridocyclitis).
3) Polyarthritis (PA) : – Involves arthritis affecting 5 or more joints during the first 6 months of disease.
It has 2 subsets –
- Rheumatoid Factor (RF) Positive.
- Rheumatoid Factor (RF) Negative.
RF positive adolescent girls have typical adult type R.A.
4) Psoriatic Arthritis (PsA) – Children with arthritis and psoriasis are said to have psoriatic arthritis. Some children have arthritis & dactylitis (sausage digits) – they probably suffer from psoriatic associated arthritis. Children with a close family history of psoriasis, nail abnormalities may be differentiated as having psoriasis associated arthritis.
5) Enthesitis related (ERA) arthritis – Enthesitis is the inflammation of tendonous insertion of muscle into bone. Children are said to have ERA if they have arthritis and enthesitis and enthesitis or arthritis plus two of the following :
- Sacro-iliac joint tenderness or inflammatory spinal pain.
- Of HLAB27 presence.
- Onset of arthritis in a boy > 6 years of age.
- Presence of anterior uveitis.
- Family history of either anterior uveitis,spondyloarthropathy or inflammatory bowel disease.
- Other Arthritis (OthA) – Children who have arthritis of at last 6 weeks duration but do not fulfill any of the other categories or fulfill criteria for > 1 category are said to belong to other arthritis category.
Arthritis associated primarily with vasculitic conditions :
Vasculitis is the inflammation of blood vessel primarily causing damage to vessel walls & subsequent damage to organs due
to vascular occlusion. Kawasaki disease & juvenile onset DM are vasculitic diseases with unique manifestations in childhood.
A) Kawasaki Disease – Typically affects children in the first 5 years of life. It presents with fever accompanied by rash, conjunctivitis & cervical adenopathy. As the disease progresses, there is dryness & cracking of lips, indurative edema of hands & feet followed by pealing of skin from tips of fingers & toes. Acute arthritis may accompany the disease. Untreated
Kawasaki disease is associated with 1% – 3% mortality caused by aneurysmal dilatation of coronary arteries with subsequent
thrombosis & myocardial infarction.
B) Childhood dermatomyositis (DM) appears commonly between ages 7-10 years. It occurs slightly more often in girls
than in boys. Children present with gradual onset of proximal muscle weakness. Characteristic rash of DM is present in 90%
causes. Some patients may present with an acute muscle pain with fever, malaise & abdominal pain. Distinctive features of
myopathy are atrophy, contractures and tissue calcifications. Visceral involvements like abnormal breathing, esophageal
dysmotility (difficulty in swallowing) & gastrointestinal malabsorption (diarrhea) have been reported.
Arthritis Associated with metabolic & inherited conditions in childhood
1) Marfan syndrome – These children are characteristically tall & exhibit ligamentous laxity. They are usually athletes & gymnasts. As a result of their athletic activities, their joints are subjected to repeated episodes of
‘micro-trauma’ & subsequent inflammation. These children are vulnerable to heart disease.
2) Ehlers – Danlos Syndrome – These children suffer from extreme form of joint hypermobility and abnormal connective
tissue. Recurrent joint injury secondary to chronic subluxation is common.
Management of childhood arthritis – The goal of treatment of chronic arthritis in children is to control the disease process while ensuring normal nutrition, growth & development. The choice of anti-rheumatic drugs is similar in children & adults, but the doses, treatment schedules and susceptibility to toxicity may differ considerably. Hence the rheumatologist who treats children should be aware of the nutritional, developmental issue, as well as the toxicity of various treatments.
The non-pharmacological manag-ement of childhood arthritis includes :
1) Splints : Rest splints are needed to support the joint and prevent deformities. Work splints are necessary to support the joints while in use.
2) Physiotherapy : exercise helps to maintain and improve the joint movements and also increases the muscle strength. Cycling and swimming are good forms of exercise especially for children with spinal, knee and hip arthritis.
3) Surgery : Surgery in childhood arthritis is seldom required. For flexion deformity in hip joint total replacement is possible after full growth of the child is attained.
4) Social and psychological support: Some children have substantial behavioral and psychological problems due to the
disease severity, mental anxiety and depression in patient, chronic family stresses and economical difficulties and
lower educational attainment.
It is very important that each member of the multidisciplinary team of rheumatologist,family physician, the parents and
paramedical workers should see that the child with arthritis should lead as normal a life as possible. The mother plays an
important role in the rehabilitation of the child.
The parents should encourage the child to be self sufficient in the activities of daily living and to attend school as regularly as possible. Severely disabled child may require improvised devices to help him to be selfreliant.
References :
1) Lippincott, Williams & Wilkins, Manual of rheumatology and outpatient orthopedic disorders; forth Edition, Paget et al: 2000, 172-180.
2) Rheumatic Disease Clinics of North America : vol 28 (3), Eds. – Michael Miller & Ronald Laxer : August 2002, 503-530.
Caring For A Child
by Dr. Vinaya Kunjir
This article is dedicated to parents who have a child with arthritis. It is always a difficult time for parents when they are told by the doctor that their child is suffering from arthritis. The word ‘arthritis’ itself strikes a terror in their hearts. It is very hard for them to believe that someone in their family especially their child is suffering from arthritis. If the parents and other members of the family have better understanding of the condition then it is easier for them to take care of the child with arthritis.
The word ‘arthritis’ means inflammation of the joint and this inflammation presents as pain and swelling of the joints. Childhood arthritis has an unpredictable course. There may be periods of severe pain and swelling of the joints and at times there may be periods of complete relief. The child may not be able to move the joint in periods of pain and swelling. If the period of active arthritis continues for a long time then it may cause muscle weakness, joint contractures and functional disability. Hence children with arthritis require both medical treatment as well as physical therapy. Fortunately with modern drugs, most children recover from arthritis without any significant joint damage and deformity. Studies showed that 30% children with arthritis who were not treated with modern medicines entered their adult life with acute disease and disability which led to decreased physical functions and pain. Hence proper care of children with arthritis should be undertaken. When a child is affected by arthritis life is significantly changed for all the family members including the parents, brothers and sisters, and even the grandparents. There are multiple issues which affect the children as well as the family members.
Sibling Issues
Brothers and sisters of the affected child may not like that their parents give extra time and attention to the affected child. Feeling of guilt and embarrassment and fear of catching the illness are some of the problems of the siblings. Parents should have a proper approach towards the siblings. They should talk with their unaffected children, make them take an important role in the medical treatment of the affected child such as reminding about medicines, take them on clinic visits. With proper approach the siblings become a great source of help and support in caring for children with arthritis.
School Issues
Childhood arthritis does not affect the ability of the child to learn and think except in some diseases like SLE “systemic lupus erythematosus” which may affect the control nervous system. The affected children should attend school and some special health care needs should be provided at school. The parents must talk with the teachers and the school authorities about the schooling problems of the affected children and plan some health care program for them. For example, swollen joint of the children can become stiff and more painful after sitting for long hours. So such children should be allowed to change position after every 20-30 minutes. Climbing stairs is difficult so the parents must be allowed to carry the children to the classroom and back. Swollen fingers can result in slow and messy hand writing. Teachers must be educated regarding this school concerns of the affected children.
Parent Education
Parents of the affected children should be informed about arthritis, the long duration of the disease and its nature, the medicines and the adverse effects of the medicines. They should be told about the need of physiotherapy to prevent joint deformities and contractures. The doubts and the wrong beliefs about arthritis must be discussed with the pediatrician and the rheumatologist. The most frequently asked question by the parent is ‘Why did my child get arthritis ? Does change in diet cause arthritis ?’ It is nobody’s fault when a child develops arthritis. It is not known why arthritis occurs but it is believed that something (probably an infection) triggers the immune system of our body, changes the immune system and causes the body to irritate its own joint and produce pain and swelling in the joints. Diet has no role in causing arthritis. It is very important that a child should have a balanced diet.
Counseling
A trusting relationship should be developed between the child and parents and the treating pediatrician and rheumatologist. One must listen to what the child and his parents have to say. One must find out the strengths and weaknesses of the family and build on the strengths like a joint family or caring school teacher or a loving grandparent. Some children might look different because of a rash or steroid therapy and this makes them self-conscious and isolated. Involvement of classmates, siblings in taking care of affected children will help them in their morale and taking part in social and family activities. Parents and pediatrician should prepare the growing children for adult life, for college, career plans.
Financial Issues
In present time a large amount of money is necessary for treatment and care of children suffering from chronic illness like arthritis and the disabilities. In the western countries there are insurance companies and health maintenance organizations (HMOs) which cover the financial aspect of childhood arthritis and the parents are educated about the child welfare systems and their benefits. In developing countries like ours, the treating doctors and the parents should work together to get access to the various organizations which provide services to the children with chronic diseases and disabilities.
Immunization
Parents should be encouraged to follow the regular schedule of immunization in children with arthritis except in a few special cases like (a) children with severe active arthritis should not receive any immunization (b) children who are on light dose of steroid (prednisone>20mg/day) should not receive vaccines. (c) children undergoing, immunosupressive therapy or glucocortieosteroid should not receive any live vaccine.
Occupational & Physical Therapy
Physical therapy is required to maximize the functions of joints, to prevent development of deformities and to help the affected child to achieve the developmental milestones – physical, emotional, educational and vocational.
For milder problems and prevention of contractions exercises to maintain the range of motion can be taught to parents by the rheumatologists.
If more aggressive treatment is required, they can be referred to physiotherapists. The therapists improve the activation of daily living by use of traction or splints if necessary, stretch the joints, train co-ordination of opposing muscle, and improve the posture and gait.
Thus the case of children with arthritis is a team effort. This team consists of parents, grandparents, brothers, sisters, teachers and all the people who step in to help and also a series of health professionals.
Camp for children with arthritis – An experience
by Dr. Vinaya Kunjir
On 27 November 2008, Center for Rheumatic Diseases (CRD), Pune appeared very busy with laughing, chatting children. It looked as if a school was getting ready for a picnic. True, they were school children but they were special children- children suffering from arthritis and CRD had arranged for a camp for them. It was sponsored by Bone and Joint
Decade India (BJD India): NAN and World BJD (International organization for musculo-skeletal diseases in the world).As an Associate Pediatric Rheumatologist in CRD, I was looking after these children when they visited CRD for management of
arthritis. So I took the responsibility of conducting the camp.
The venue was decided- it was a beautiful resort with water games and various fun- filled activities for children. The objective of this Camp for Children with arthritis was to create awareness about arthritis in children and their parents in a funloving way.Forty children along with their parents attended the camp. Some members of Mission Arthritis India (MAI) had kindly volunteered to help with the activities of the camp. The highlight of this event was the attendance of three renowned
pediatric rheumatologists- Dr Prudence Manners from Australia, Dr Ross Petty from Canada and Dr Chris Scott from South Africa. Mr Ben Horgan , the organizing secretary of BJD-NAN Australia also attended the camp. He is a patient of Juvenile Arthritis (JIA) and has been conducting.
Camps for children with arthritis since last 10 years.He was very keen to see the camp for these Indian children.
We had a combination of health education program and entertainment for the children. Senior pediatric rheumatologist, Dr Prudence Manners spoke about caring for children with arthritis. When a child suffers from arthritis, the whole family- the
parents, siblings, grandparents are affected. She spoke at length about the problems faced at school, about parent counselling regarding care of these children. Dr Ross Petty, the renowned pediatric rheumatologist from Canada spoke about the issues related to growing up of the children to adulthood. He stressed that growing up is nothing but becoming
more responsible. The kids should know about the illness, the medication and self-care.
Mr Ben Horgan who himself is a patient of juvenile arthritis/JIA, spoke about his personal experience about arthritis. Ben, who is now 36 years old, has been suffering from JIA since the age of two years and he talked about how he had coped with
arthritis as a child and in adulthood . He had deformities in almost all joints and has undergone many operations for repair of his damaged joints. Inspite of this, now he is a happily married man with kids and works in an organization for patients with arthritis and also holds similar camps for children with arthritis in Australia. The story of his struggle with
arthritis brought tears to the eyes of many parents present in the hall.
We also had a session where the children and parents got an opportunity to ask questions and difficulties to the pediatric rheumatologists . The parents asked many queries about Juvenile arthritis, diet, exercises, medication, about the longterm
effects of arthritis on the children . The affected children got a chance to mingle with the other children with similar complaints. They talked with each other, forgot their pain and played with each other and became aware about how the other
children with same illness coping with arthritis.
We also had entertainment programs for the children. The kids enjoyed the water games, the magic show and also music and dance. The program ended in the evening with ice- cream party and the children along with their parents said bye to each other with a promise to meet again next year in the Camp. The inspiration to hold a camp for these children came when I
had attended a JIA Camp in Perth, Australia organized by Ben Horgan last year.
This was our first attempt to organize a Camp for children with arthritis. There is a great need to hold such Camps in our country so as to create awareness about juvenile arthritis. It gives an opportunity to the children to learn from each other
about coping with arthritis and also making friends with arthritis.
