Chronic childhood arthritis, sometimes referred to as juvenile rheumatoid arthritis or juvenile chronic arthritis, is quite different from adult rheumatoid arthritis. In fact, many people have dropped the word 'rheumatoid' and now call chronic childhood arthritis juvenile arthritis, or JA.

JA is defined as continuous inflammation of one or more joints lasting at least six weeks for which no other cause can be found.

Arthritis is one of the most common chronic illnesses affecting children. It affects 1 in 1,000 Canadian children under the age of 16. Both boys and girls are affected by juvenile arthritis.

There are several unique features in JA:

Growth
One of the unique issues in JA is the effect of inflammation on the growing child. Occasionally, if the arthritis is severe, a slowing of growth may occur. The growth will usually return to normal once the arthritis has improved. In addition, there may be changes in the growth of the individual joints that have been affected by arthritis. For example, knee inflammation may cause the leg to grow slightly faster than the non-inflamed side. Jaw inflammation may result in a small chin. The healthcare team will pay careful attention to all aspects of your child's growth.  

Eye Problems
Sometimes a child with JA may suffer from inflammation inside the eye called 'chronic uveitis' that might reduce vision. Also known as iridocyclitis or iritis, this inflammation often occurs without symptoms or visible signs so that you and your child may not be aware of its presence. Therefore, it is important for the child's eyes to be checked regularly, even if they are not red or painful. In the early stages, this inflammation can only be detected by a special examination, which must be done by an ophthalmologist (a physician specially trained in eye care and treatment). Your doctor will make sure that regular eye examinations are arranged to follow your child.  

The uveitis is not related to the degree of inflammation in the joints. Eye disease is, in fact, most common in children with only one or a few joints involved; it may even start before arthritis is diagnosed. It can start in one or both eyes. If after six to 12 months the uveitis affects only one eye, it is unusual for the uveitis to then affect the other eye.  

Unpredictability
Arthritis in children sometimes may last for as little as several months to a year and then disappear forever. Most children, however, have an up-and-down course for many years, depending on what type of arthritis they have. Flares (also known as flare-ups or exacerbations) are those times when the arthritis seems to be getting worse. Remissions are times when the arthritis appears to have gone away. Sometimes, a mild infection, such as the 'flu,' may trigger a flare. Usually, the cause of the flare is not identified. It may be upsetting for parents to see such changes when it seemed the disease had disappeared or had improved. Nevertheless, parents should not give up hope and should remain optimistic. Fortunately for most children, these flares tend to become less severe and occur less often with time.

No one knows for sure what causes JA. We do know that it usually does not run in families, and arthritis is almost never passed from a parent to a child. Therefore, the chances of your child passing arthritis on to his/her children would be extremely rare. JA is not caused by any disease or infection that either parent may have had at any time, nor is it connected to any event during pregnancy. JA is not caused by eating the wrong foods, nor is there any proof that it can be improved by specific diets. Similarly, although many people feel that their arthritis is better in warm, dry climates, there is no proof that JA is caused or improved by any particular climate. And, although fever and rash may occur in some children with arthritis, JA is definitely not contagious.

The onset of JA may coincidentally follow a routine infection or injury, but such common events do not cause chronic arthritis. It is currently believed that the body's immune system may be responsible for the inflammation. The body's immune system is a complex and finely tuned defence mechanism, which fights infection by causing inflammation, as it does in tonsillitis. Tonsillitis is caused by a known bacterial infection, and the sore, red, inflamed tonsils are a sign that the body's immune system is acting normally to eliminate this infection. In JA there is also inflammation of one or more joints, but in contrast to tonsillitis, we do not know what causes this inflammation. In children with arthritis, the immune system seems to be overactive. It may respond inappropriately to infection or mistakenly identify something in the body for an infection, causing continuous inflammation. This inflammation results in warm, stiff, swollen and often painful joints.

The medication prescribed by your child's doctor acts to reduce joint swelling, pain and stiffness and makes the exercise program easier for your child to do. The nature and strength of this medication will depend on the severity and type of arthritis your child has.

Diagnosis
JA is defined as continuous inflammation of one or more joints lasting at least six weeks for which no other cause can be found. Since arthritis may be a part of many different illnesses and there is no test that can definitely diagnose JA, tests to exclude other causes of joint pain and swelling must be done. Therefore your child will likely require X-rays and blood tests. In addition, once the diagnosis of JA is confirmed, tests will need to be repeated from time to time to monitor the illness and the effects of medication.

There is great variation in both the symptoms and nature of childhood arthritis. Therefore, once a doctor has diagnosed or suspects arthritis in your child, referral to a specialist, such as a pediatrician or a rheumatologist (a physician specially trained in the diagnosis, evaluation and treatment of disorders of the joints, muscles and bones) will usually be required. A specific diagnosis is important and necessary so that a specific treatment program can be designed for each individual child.

The specific patterns and types of JA are:

Pauciarticular Onset Juvenile Arthritis
This is the name given to the most common and generally mildest form of JA. Four or fewer joints are involved, and girls are affected much more commonly than boys. It usually starts in children four years of age or younger. The most commonly affected joints are the knee, ankle, wrist and elbow. There is little or no disturbance of general health or growth. The course of pauciarticular juvenile arthritis may involve flares and remissions, but with appropriate treatment, there is rarely permanent damage to the joints. In fact, many affected children will be in permanent remission within a few years of the onset of the disease.

Up to 20% of children with pauciarticular juvenile arthritis will eventually develop uveitis. Therefore, all children with pauciarticular juvenile arthritis should have a regular, routine slit-lamp examination of their eyes at least three or four times a year for the first few years after diagnosis.

Systemic-Onset Juvenile Arthritis
Systemic-onset juvenile arthritis is less common but frequently a more severe form of JA. It occurs in about 20% of children with JA, affecting boys and girls equally. It usually involves many different joints.

Children with systemic-onset juvenile arthritis have spiking (rapidly rising and falling) fever, which usually occurs once (or sometimes twice) a day, and a rash that frequently comes and goes with the fever. In addition, they often have swollen lymph glands, and enlargement of the liver and spleen. They appear listless and unwell during the fever (most often in the late afternoon or evening), only to brighten up by the next day as their temperature returns to normal. When fever in systemic JA persists for several weeks, the child will be weak, lose weight and may become pale from anemia (a fall in the level of hemoglobin in the blood). Flares that last a long time may also interfere with growth, although the growth usually improves as the child's condition improves. Inflammation of internal organs may cause stomach pain or affect the heart or lungs but will not cause permanent damage.

In the early stages of systemic JA, there is sometimes no sign of joint inflammation. This may make it a very difficult disease to diagnose because there are many other illnesses that also cause a fever and rash in children. Therefore, many tests are done. The arthritis usually appears within the first six months after the start of the fever, and usually persists, even when the fever settles. The arthritis may be mild or severe, and may involve just a few, or many joints. However, even severe forms of arthritis sometimes go into remission within a few years. If the arthritis does not go into remission, it will follow a pattern of 'ups and downs' that is different for each child. In most cases, the pattern will gradually become less severe. Occasionally, as with all forms of JA, flares may occur after the disease has been inactive for a long time.

Polyarticular Onset Juvenile Arthritis
Many joints (five or more) are involved in polyarticular juvenile arthritis, but without rash or high fever. This type of JA can begin at any age, and usually, the arthritis starts in several joints at the same time. It is more common in girls than in boys. Occasionally it will begin in only one or two joints, then spread to other joints. The duration of polyarticular JA varies from six months to many years. In general, the arthritis runs a course that lasts several years. Interference with growth is less of a problem in polyarticular JA than it is in systemic JA, and inflammation of internal organs is rare.

Psoriatic Arthritis
Psoriasis is a skin disease that is accompanied by arthritis in many cases. When arthritis and psoriasis occur together, the disease is called psoriatic arthritis. This type of arthritis can occur at any age. It often affects only one or a few joints but may also involve the hips or back like in spondyloarthropathy, or the fingers and toes. In about half of children with psoriatic arthritis, the arthritis starts before any sign of skin disease. For this reason, a family history of psoriasis will help your doctor to diagnose this type of arthritis.

Spondyloarthropathy
This type of arthritis generally affects children over the age of 10, and is the only type of JA that is more common in boys. It usually involves just a few joints in the lower limbs and commonly affects the hips. Joint inflammation in spondyloarthropathy often persists into adulthood. This form of arthritis may sometimes progress to pain and stiffness of the back. Heel pain can also occur. Spondyloarthropathy is one of the few types of arthritis that may be hereditary or run in families. It may also occur with inflammation of the eyes, skin or bowel, either in the patient or in other family members. Many children with spondyloarthropathy carry a protein called HLA B-27 on their cells. Testing for this protein in the laboratory may help in the diagnosis in some cases.

Adult-Type Rheumatoid Arthritis
Occasionally, adult-type rheumatoid arthritis will begin in childhood. This type of arthritis may genuinely be labelled juvenile rheumatoid arthritis (JRA). It occurs most often in older girls and is identified with a blood test for a protein called rheumatoid factor. This form of JRA has a greater tendency to cause severe joint damage than most other types of arthritis. Thus, stronger medications may be recommended at an early stage for JRA.

Once your child has been diagnosed as having arthritis, the overall treatment plan will be co-ordinated by your doctor, and will include medication and rehabilitation therapy. An exercise program, specially designed by your doctor and therapists, is one of the cornerstones of treatment. Even if arthritis does cause joint damage, the bones and cartilage of growing children have amazing abilities to heal. Exercise helps prevent loss of joint movement and encourages normal growth.

While research has not yet provided us with a cure for childhood arthritis, there is effective therapy. Most children with JA have a good long-term outcome. With proper therapy, most children will enter adulthood without any major physical disability.

The uncertainty and doubt of the first several weeks following discovery of the arthritis can result in an emotionally difficult time for you, your child and your family. It is important for you to realize that it may take time for your doctor to be absolutely certain of the diagnosis of JA. You must also understand that it may take up to several weeks or sometimes even months for the medicines and exercises the doctor prescribes to have a noticeable effect.

Treatment
To achieve the best possible outcome, you and your child will need to work very closely with the arthritis treatment team. This may include your doctor, a nurse, a physiotherapist, an occupational therapist, a social worker, a psychologist, an orthopedic surgeon, an ophthalmologist, a dentist and your child's teachers. The specific members of the team involved in the care of your child will be determined by the nature of the arthritis and his or her special needs. The most important members of the team are you and your child. With your help and the guidance of the team, your child can deal with even severe arthritis.

The attitude and emotional response of a child and his or her family are extremely important in determining the future. Some joints may be damaged because of inflammation, but an exercise program and the use of splints can help to prevent deformities. It may take time to notice the benefits of therapy. Persistence with the suggested treatment and a positive attitude from you and your child are the keys to success.

It is extremely important that your child's social development be as normal as possible. Children who cannot participate in all the activities their friends enjoy may become depressed and feel isolated. Children with arthritis should be encouraged to participate in appropriate activities with friends to promote independence and build self-esteem.Each child with arthritis is an individual with his or her own unique needs and reactions. The treatment program must be designed by the arthritis team – you and your child, the doctor, nurse, ophthalmologist, orthopedic surgeon, therapists, social worker and teachers – to suit each child. The major aim of treatment is for the child to grow up to become a functioning and contributing member of society. In general, the treatment aims for your child will be to: achieve normal physical, social and intellectual development:

• achieve normal physical, social and intellectual development
• achieve and maintain an acceptable functional range of joint movement
• achieve emotional acceptance of any physical limitations
• attend a regular school
• relate to other children
• assume responsibility for his/her own health.

Most treatment approaches will start with anti-inflammatory medications. NSAIDs include a variety of medications that affect the processes involved in inflammation. By reducing pain, swelling and stiffness, these drugs alone often allow a child with arthritis to participate in normal day-to-day activities. NSAIDs do not cause addiction, and their effect on inflammation does not wear out over time.

Some of the more commonly prescribed medications in this group include Naprosyn¨ (naproxen), Tolectin¨ (tolmetin sodium), Indocid¨ (indomethacin), and Advil® or Motrin® (ibuprofen). The choice of medication is based on disease type, how easy it is to take and physician preference. Sometimes it may take up to eight to 12 weeks to see improvement. Often, one NSAID works while another does not, and it may occasionally be necessary to try several NSAIDs to find which one works best for your child.

The most common side effect that may occur with all drugs in this class is stomach upset, which can frequently be avoided by taking the medication with food. Some side effects are seen only with specific medicines, and will be reviewed with you and your child by the healthcare team. In order to ensure that there are no side effects from NSAIDs, your doctor may monitor their effect with blood and urine tests at clinic visits.

Aspirin® (generically referred to as acetylsalicylic acid or ASA) used to be the most commonly prescribed drug for JA. Although it remains a safe and effective drug for many children with JA, and continues to be used by many pediatric rheumatologists, some of the other NSAIDs are more convenient to take and are often better tolerated than Aspirin®. In children taking Aspirin® there is a very small risk of Reye's syndrome (sudden onset of altered state of consciousness together with liver disease) following infection with chickenpox or influenza. If you are concerned you should ask your physician for advice.

For children with prolonged arthritis in several joints that may lead to permanent damage, second line drugs are often prescribed. Drugs in this group include Rheumatrexª (methotrexate), Salazopryn¨ (sulfasalazine), gold injections, Plaquenil¨ (hydroxychloroquine), and Cuprimine¨ or Depen¨ (penicillamine). These agents, also called disease-modifying anti-rheumatic drugs, or DMARDs, are 'slow acting' drugs that can take up to six months to work. They are used to obtain better control of the arthritis than that achieved by NSAIDs alone. With all of these drugs, treatment must be continued for a long time (often months to years) even after the disease is controlled, in order to avoid a recurrence of arthritis. When they are started, your doctor will review the specific medication with you and your child in- depth.

Each drug has its own side effects that require careful monitoring by regular examination and laboratory tests. These will be carefully reviewed with you and your child by the healthcare team.

Joint Protection

Physiotherapy and occupational therapy are essential components of the treatment program for all children with JA to minimize long-term damage to joints and muscles and to preserve function.

Inflammation causes pressure and sometimes pain in and around the joint, causing muscles around the joint to go into spasm and tighten. Thus, the child with arthritis will tend to keep the inflamed joint(s) in the most comfortable position, which is usually bent. If a joint stays bent for too long, both the muscles and tendons (strong, rope-like structures that attach muscles to bones) will shorten and not grow properly. This results in the joint staying in a bent position, called a joint contracture.

It is very important to remember that in children, unlike adults, cartilage and bone can still grow, which allows for healing and repair of joint damage. However, joint tightness must be reduced by a routine of exercises and splinting; otherwise a deformity may develop, and the child will not be able to completely straighten the joint. If a joint remains in a bent position for too long, it may not grow properly and permanent damage and deformity could result.

The healthcare team may recommend that your child use splints. Splinting has several purposes in the management of arthritis in children. A splint at night (resting splint) and during naps will hold inflamed joints, such as knees and wrists, in a good position. Resting splints help to prevent, and can gradually correct, deformity.

Sleeping with splints can be at first difficult for some children, who may wake up and complain of pain or discomfort. However, resting splints are generally well tolerated as long as they fit well. They should not cause pain or red marks, and if they do, they must be checked. It is essential that you and your child persevere with the splints. Otherwise your child will tend to sleep in a curled-up position, and since joints gradually stiffen during the night, this could eventually lead to deformity.

Daytime splints (working splints) may also have to be worn to support and assist during normal daytime activities such as writing. Use of these types of splints can provide pain relief by protecting inflamed joints. Your child may resist wearing splints in front of friends, and it may help to have the teacher discuss the reason for wearing splints with the rest of the children in the class. When your doctor and therapists have decided splinting is no longer necessary, they will advise you on how to stop wearing them.

Splinting may also be very beneficial in helping to improve joint contractures. If a deformity has developed, serial splints can be made over a period of weeks or months. Each new splint will move the joint into a better position and gradually correct the deformity.

Exercise

It is important for children and families to take responsibility for the exercise program and make it a part of daily life. It is often helpful to have family members join in an exercise program. As the general condition improves, more active exercises will gradually strengthen muscles. Strong muscles stabilize and protect the joints. Encourage your child to participate as much as possible, and let your child set his or her own limits.

Some modification of activity may be needed during flares of arthritis. Your therapists can advise you as to which activities are the least stressful on the joints. Ordinary activities or play are not a complete substitute for a regular exercise program. However, some routine activities are especially good for joint mobility. Even lying flat on the stomach (lying prone) for some time each day will help keep the hips and knees straight. The child may read or watch television in this position.

Pre-schoolers are extremely curious, and mobility is extremely important for their development. For these youngsters, riding a tricycle is excellent exercise and can strengthen hips, knees and ankles when walking is too painful. Swimming is also an excellent exercise for most children. With the force of gravity greatly reduced, swimming can improve muscle tone and movement of large joints better than any other activity.

Active exercises are important even when there is swelling. The amount of joint swelling in JA is variable and not related to eventual joint damage. In fact, some children with only a small amount of joint swelling have particularly severe problems with stiff joints.

The benefits of exercises will take time. At the start, the exercises may be somewhat painful for your child, and it will be hard for you to constantly remind your child to do them. It will be harder still for you to be consistently positive about the future when your child has had a recent flare. Share your doubts, fears and questions with your caregivers. In that way, you and your child will be better able to maintain the positive attitude that is essential for the best possible outcome.

Rest

Children need extra rest when their arthritis is very active, but a child with active inflammation should not be allowed to rest in bed all day as this promotes stiffness and loss of mobility. The mattress should be firm and the top bed sheets should not be tucked in too tight to allow free movement in bed.

Even very ill children should have all their joints moved through as full a range of movement as possible each day. Exercise in a warm pool or in a deep tub bath at least once daily helps the muscles to relax, and allows the joints to be moved in as full a range as possible.

Good posture, even in bed, helps to prevent deformities. There is a right and a wrong way to lie in bed; a therapist can explain the difference. Inflamed joints may be placed in splints to maintain them in a good position. If the neck is involved, only one flat pillow should be used. Larger pillows will encourage the neck to bend forward. Pillows should never be placed under the knees, as this encourages permanently bent knees.

Surgery

Surgery is not commonly required but may ultimately be necessary after many years of severe arthritis. Your doctor may seek the advice of an orthopedic surgeon to help plan future treatments and surgical procedures. Sometimes the tendons and ligaments around joints, such as the hips, may need lengthening. When arthritis affects one knee more than the other, the side with the worse arthritis may grow faster. This may, temporarily, increase the length of the leg of the more involved side, but given time, the growth of the other leg will often catch up. Only very rarely is a surgical procedure required to correct the difference in leg lengths. The orthopedic surgeon may also be asked to make recommendations on splinting and rehabilitation.

If arthritis has severely affected the jaw, there may be poor chin growth. An orthodontist may recommend a splint to be worn in the mouth. Surgical procedures can correct this problem once the disease is in remission. Your doctor may seek the advice of an orthodontist to make recommendations regarding these procedures.

Arthritis of the jaw can make complicated dental work a problem. Be sure to tell your dentist that your child has arthritis so that he or she can give your child's teeth special attention. You should also strongly encourage your child to practice good dental care. As JA frequently affects the neck and jaw, it may make general anesthesia difficult, and it is advisable that an anesthetist see children with JA before any type of operation is planned.

Parenting

Parents are an important part of the overall health care team, and as such, should be as well informed as possible. When you have questions, write them down before the clinic appointment so that they are not forgotten. Feel free to question your doctor about medications and other parts of the treatment plan, and make sure that you understand the reasons behind all the recommendations.

Your Family
Fear of the future can affect how even the strongest family works together. It is very helpful if all family members can become involved in the child's program so that they can provide each other with the support needed to deal with, and overcome, a chronic illness.

Sometimes the parents of a child with a chronic health problem become overly involved with that child to the exclusion of other aspects of their lives. It is important for parents to socialize, maintain their friends and pursue other interests if they are to keep a balanced perspective and provide the optimism and strength their child needs.

The emotional stress of an ongoing illness like arthritis is felt keenly by other children in the family. Brothers and sisters are often confused as to how to feel about the child with arthritis. They want to help, but sometimes - understandably - they are jealous of all the extra attention the child with arthritis seems to receive. Brothers and sisters may have difficulty understanding why there is so little time left for them. Make sure their interests are not overlooked, and encourage them to get involved. It may be appropriate, for example, to have the other children participate in the exercise program with the child with arthritis. Allow them to openly discuss their own efforts and struggles to adjust to their siblings' arthritis. On a positive note, children who grow up in a home with a chronically ill brother or sister often develop rare insight into the problems of others and benefit from this experience.

Independence and Learning to Cope with Life
Parents should set limits and discipline the child with arthritis as normally as possible. There is a tendency for parents to excuse the child with arthritis for inappropriate behaviour. However, this may, at times, encourage the child to use the disability for her own advantage both in and outside the home, which can lead to behaviour problems later on in life. Often, parents need help from a professional in knowing how to best deal with some of these issues. A social worker or psychologist skilled in helping families deal with such problems may be of great benefit during these times.

It is important for parents to think in terms of what their child can do and encourage the child to discover his or her own limits from the start. It is important to let your child dress and undress herself, even if this seems to take too long. Involvement of the joints of the hands may make buttons hard to grasp. Often, restricted hip movement can make shoes and socks difficult to put on and take off. An occupational therapist can design aids that will help children manage despite such difficulties.

Your Child and School
It is crucial that your child's life remain as normal as possible. Attending a regular school should be a prime goal of the treatment program. If the arthritis is active, this will require the encouragement and help of understanding school staff. At the start of the year, set up a meeting with the principal, teachers, nurse and any others in the school who may be in contact with your child. Share with them your child's condition, limitations, strong points and special needs, and tell them about JA and your child's treatment program. There are special pamphlets for teachers of children with JA that are available from various organizations, including The Arthritis Society. If the school staff has further questions, it may be advisable for members of your child's arthritis team to meet with them, as well.

Early morning stiffness can be the greatest problem in getting to school. During the night, fluid accumulates in the joints making them stiff. As your child gets moving in the morning the stiffness decreases. Your child may have to wake up early so that the stiffness improves enough to move comfortably. A warm bath with a stretching exercise program is very helpful. And if at all possible, your child should get dressed without help, because dressing is itself an excellent joint exercise.

Since early morning joint stiffness may vary in duration from day-to-day, teachers should be told that your child might sometimes be late. Most children with arthritis attend school full-time, but if your doctor and therapist decide that half-days at school should be tried this may be better done in the afternoons.

Classmates will be curious about hand splints. If the teacher treats your child's splints in a matter-of-fact way, so will the class. A simple explanation to the class about the use of splints in the treatment of arthritis is best. Splints can be likened to teeth braces, which are used temporarily to make sure that part of the body grows straight. Painful wrists may make carrying books a problem; the school may provide another set of books for home. A backpack may also be helpful.

Children with arthritis often get stiff if they sit for too long a period of time. Teachers can help by having your child move around in the classroom. If the arthritis is very active, the child may be unable to participate in regular gym classes and arrangements can be made for the child to do physiotherapy during gym. If the adolescent's knees are swollen, it is best to avoid sports that require a lot of jumping or jarring movements, e.g. basketball or downhill skiing. However, your child should not be restricted. Unless the arthritis is severe he or she should be encouraged to participate in activities and set his or her own limits. It helps to have the classmates of a child with severe arthritis well informed, too. Getting them involved - assisting with boots and clothing, for example, or heavy doors, taking notes and so on - can promote understanding and cooperation, and make your child's school days much easier. Your child may also be able to suggest ways that classmates can help.

In high school, children are often expected to rotate from class to class. This may pose special problems for children with arthritis. It may be beneficial to have your child excused from class a few minutes early to 'beat the rush' to the next class. If possible, try to have classes scheduled so that there is as little distance as possible between different classrooms.

Adolescence
Adolescence is a particularly challenging time, as one of the main developmental tasks of adolescence is separating from the family and learning to become independent. You should encourage your child to pursue and enjoy active friendships. If there is an overly involved relationship between teen and parents, independence will be harder to attain.

Adolescents, like everyone else, need someone to talk to about their problems. Doctors, nurses, therapists, social workers and teachers can help during these difficult years.