Juvenile Spondyloarthopathy

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Juvenile Spondyloarthopathy

Treatment options

Early diagnosis and appropriate treatment provides children with the best possible opportunity for a favorable outcome. Your child's treatment will be based on the kind of arthritis he/she has and on their specific symptoms.

The goals of any treatment program for juvenile arthritis are: control inflammation, relieve pain, prevent or control joint damage and maximize joint and body function.

Your child's treatment program will usually include medication, exercise, eye care, dental care and healthy eating practices. Treatments such as surgery may be necessary for special long-term problems. Some physicians have also found that pain can be lessened by combining medical treatment with techniques such as progressive muscle relaxation, meditative breathing and guided imagery.

Because so many techniques are used to treat children with JSp, the ideal type of care is sometimes called team care or coordinated care. Your child's health-care team will include many different specialists who work together to offer your child a complete treatment program. These may include a pediatric rheumatologist, nurse clinician, physical or occupational therapist, dietitian, ophthalmologist, psychologist, nephrologist, neurologist, gastroenterologist, cardiologist, pulmonologist, dentist, social worker or orthopaedic surgeon.

Pediatric rheumatology centers in many major medical centers offer this care in one location. If you do not live near a pediatric rheumatology center, your child's physician will refer you to the specialists he/she needs.

Medications
The immediate goal of drug therapy is to reduce inflammation, relieve pain and swelling, and maximize function. Long-range goals are to alter the progress of the disease and the prevent damage to bone, cartilage and soft tissues such as muscles, tendons and joint capsules. It must be remembered that medication dosages in children must be carefully calculated based on their weight and body size. Medication doses should be adjusted regularly as the child grows to make sure the proper dosages are used. In addition, it is well known that children metabolize some medications differently than do adults. Therefore, drug dosages may be much higher than one would expect based just on the child's smaller body size. Side effects may be different in children, or occur rarely compared to adults taking the same medication.

The following medications may be used to treat children with arthritis and related conditions.

NSAIDs

Nonsteroidal anti-inflammatory drugs (NSAIDs) are the first line of medication used in juvenile arthritis and are the mainstay of the initial therapy. NSAIDs must be taken for at least three to four weeks to tell whether they are helping control pain and inflammation. Laboratory tests may be done a few times a year to monitor medication side effects. These medications come in liquid or pill form and are taken from one to four times per day, depending on the drug prescribed. Some common NSAIDs on the market approved for children include: ibuprofen, naproxen, tolmentin, aspirin, choline magnesium trisalicylate and indomethacin. Indomethacin is often one of the first NSAIDs tried for children with JSp since it seems to work very well for these types of arthritis. Children with JEA may be harder to treat with these drugs due to the problems with colitis that the child already has.

Possible side effects of NSAIDs include: occasional stomach pain, nausea and vomiting; anemia; headache; and uncommonly, blood in the urine; fluid retention; thinning and scarring of the skin (especially with naproxen); difficulty concentrating; and rarely, stomach ulcer.

Aspirin

Aspirin is a rarely prescribed NSAID these days. If chosen by your doctor, it may be used to control joint pain and swelling and to reduce fever just like the other NSAIDs. It is prescribed in large amounts and is given three or four times a day. Young children should not suck or chew on the aspirin because this may damage the chewing surfaces of the teeth and irritate the gums. Instead, try crushing the dose and having the child swallow it in a small amount of a favorite food such as applesauce or yogurt.

Possible side effects of aspirin include stomach pains or stomach bleeding; toxic reactions can occur from too a high dose built up in your child's system (rapid or deep breathing, ringing in the ears, decrease in hearing, drowsiness, nausea, vomiting, irritability, unusual behavior and black stools). A rare complication called Reye's syndrome can occur in children who have the chicken pox or the flu and are taking aspirin. Symptoms include frequent vomiting, very painful headaches, unusual behavior, extreme tiredness and confusion. The different NSAIDs should never be combined together without your doctor's instructions.

Slow-Acting Anti-Inflammatory Drugs

These drugs do not relieve pain or inflammation right away; instead, they are given to change the progress of joint disease (such as joint erosions or cartilage and bone destruction) weeks to months after therapy is begun. Therefore, they are referred to as slow-acting anti-rheumatic drugs (SAARDs) or disease modifying anti-rheumatic drugs (DMARDs). These drugs are often used in combination with NSAIDs. Because they are more powerful medications, children will need to have more frequent laboratory tests for monitoring of possible side effects. Some of these medications are described below

Sulfasalazine
Sulfasalazine (Azulfadine) is one of the most commonly used SAARDs for the JSp. It is given in pill form. This medication helps the joint pain, stiffness and swelling. In cases with JEA, it can also help the symptoms of colitis. It has a sulfa antibiotic component in it, so cannot be used by people with sulfa allergies. It takes 6-12 weeks to work.

Side effects may include stomach upset, achiness, diarrhea, dizziness, headache, light sensitivity, itching, appetite loss, liver abnormalities, lowered blood count, nausea, vomiting or rash. Blood work is checked within a few weeks of starting this medication then every few months to check for these changes.

Hydroxychloroquine
Hydroxychloroquine (Plaquenil) is only rarely used for the JSp. It is a pill used to control joint pain and swelling. It may take 3 to 6 months to work. While not helpful in all cases, it may be useful when methotrexate or gold have not been completely effective and can be used alone or in combination with other drugs.

Side effects of hydroychloroquine include upset stomach, skin rash and a rare complication, eye damage. A child who takes this drug should have his/her eyes examined at least every six months by an ophthalmologist familiar with this medication.

Gold Compounds
Gold compounds (Auranofin, Ridaura, Myochrysine and Solganol) are also rarely used to treat the JSp. They can be used to ease morning stiffness and control joint swelling and pain. The oral form is taken daily. Injections are usually given every week for five or six months, then one or twice a month for as long as necessary. They take 3-6 months to work.

Side effects may include skin rash, mouth sores, kidney problems, a low blood count or anemia. Blood and urine tests are checked every 1 to 4 weeks while taking gold compounds.

Doxycyline and Antibiotic Therapy
With the strong association of the JSp and infections, especially JReA, antibiotics play an important role in the treatment of some of these children. Tetracycline antibiotics, most often doxycycline, have been shown to help some of these children. These antibiotics must not be given to children under 8 years old due to bone and teeth side effects. The medication is taken twice a day and may cause nausea and sun sensitivity of the skin. It may take 2-3 months for these medications to be effective. Doxycycline may be taken as the sole SAARD or in conjunction with other agents.

Immune System Medications
Methotrexate
Methotrexate (Rheumatrex) is given weekly either orally as a liquid or in pill form, or by injection. It is one of the most commonly prescribed SAARDs for children with the JSp. It works best for JPsA, JEA and less so for JAS. It can help control eye inflammation (uveitis, scleritis) in more severe cases. It takes 4-8 weeks to work.

Few side effects are typically reported at the low doses at which methotrexate is usually prescribed (typically 7.5 to 25 mg a week), but regular laboratory monitoring is still important. Blood tests are usually checked every month at first then every 6-8 weeks later on. This is also a cancer chemotherapy drug but the dosages used in children with JRA are much lower. Therefore, the side effects are less frequent.

Side effects may include nausea, mouth sores, moodiness, diarrhea, low white blood cell count, lung irritation, infections and liver irritation. Avoid all alcohol intake and smoking while on this medication.

Azathioprine
Azathioprine (Imuran) is rarely used for the JSp. It is given in pill form. This is an immune system suppressing medication. It is saved for children that have failed or cannot take methotrexate. It takes about 3 months to work.

Side effects can include cough, fever and chills, loss of appetite, nausea or vomiting, skin rash, unusual bleeding or bruising, unusual tiredness or weakness, or possibly, sterility.

Cyclophosphamide
Cyclophosphamide (Cytoxan) is also rarely used for the JSp. It is given in pill form daily or intravenously in a single dose, usually monthly. This drug is often given in children with systemic lupus erythematosus, an arthritis-related condition. It may take several months to work.

Side effects include blood in the urine or burning on urination, confusion or agitation, cough, dizziness, fever and chills, infertility, loss of appetite, nausea or vomiting, unusual bleeding or bruising, unusual tiredness or weakness, and an increased risk for cancer.

Cyclosporine
Cyclosporine (Sandimmune) is given in liquid or pill form. This drug helps the joint inflammation as well as the skin rash in children with JPsA. It may take a 1-2 months to work. Blood levels are often checked to determine the best dosage for your child.

Side effects include bleeding, tender or enlarged gums; fluid retention; high blood pressure; increased hair growth; kidney problems; loss of appetite; or trembling or shaking of hands.

Etanercept and Other Biologic Agents
Biological agents are a new class of medications made of synthetic proteins. These drugs may be made of antibodies that block high levels of inflammatory proteins in patients with arthritis. The drugs available include etanercept (Enbrel) which blocks the protein TNF, and was approved in 1998 by the FDA for RA treatment in adults, and in 1999 for the treatment of JRA. Infliximab (Remicade) is another anti-TNF medication that is approved to treat RA and has begun testing in JRA. Both these agents have been studied in Europe and the United States for the spondyloarthropathies. They show great promise and will likely be approved to treat JPsA in the near future.

Intravenous immunoglobulin (IVIG) is used to treat several childhood rheumatic diseases. It is usually given intravenously once a month. It is sometimes used as part of the treatment of systemic JRA. Side effects include the risk of allergic reactions, headaches, stomachache and flu-like symptoms.

Researchers are developing other experimental biologic therapies that are aimed at specific proteins to control a variety of different diseases.

Glucocorticoid Drugs
Glucocorticoid medications (Dexamethasone, Methylprednisolone, Cortef, Prednisolone and Prednisone) are the most potent anti-inflammatory agents. These drugs are used to treat a variety of rheumatic diseases when the disease is severe or has not responded to other drugs. Sometimes glucocorticoids are used for a few weeks until other slower medications can become effective in controlling the arthritis.

Possible side effects of these medications include high blood pressure, osteoporosis (brittle bones), Cushing's syndrome (weight gain, moon face, thin skin, muscle weakness) cataracts, slowed growth rate, reduced resistance to infection, sudden mood swings, increased appetite and weight gain, or increased risk for ulcers. Many of these side effects occur only when the dose is more than 7.5 to 10 mg a day (dose for an average size adult who is taking prednisone).

Glucocorticoids should never be stopped suddenly if they have been taken for more than a month. Your child's own natural gluco-corticoids may not be produced enough after using these drugs for a month or more. A schedule to slowly discontinue or taper these medications will be given to you by your doctor and will prevent this problem. A child taking glucocorticoids should wear a medical alert bracelet or necklace. This will alert emergency medical personnel to give extra medication if the child has surgery or is seriously injured.

Because of their many side effects, glucocorticoids must be used with caution. If these drugs are prescribed, the lowest possible dose should be used for the shortest length of time. Usually the drug is given by mouth as a pill or liquid. It can also be given as an injection into the joint itself, into a muscle, or vein (IV).

Analgesics
Analgesics (acetaminophen [Tylenol, Panadol], tramadol [Ultram]) do not relieve inflammation, but provide pain relief. They should be taken only under a physician's advice in conjunction with other medications. Acetaminophen has few side effects when taken in small doses; very high doses (overdoses) can cause liver damage. Side effects of tramadol may include dizziness, nausea, constipation, headache and sleepiness.

Exercise
Exercise is a very important part of treatment for juvenile arthritis. For children with arthritis, exercise helps keep joints mobile; keep muscles strong; regain lost motion or strength in a joint or muscle; make everyday activities like walking or dressing easier; and improve general fitness and endurance.

Therapeutic Exercise

While medications reduce pain and inflammation, only therapeutic exercise can restore lost motion in a joint. These exercises can make it easier for children to walk and perform other activities of daily living such as walking, eating and writing.

Range-of-motion exercises keep joints flexible and are especially important for children who have lost motion in a joint, or whose joints have become fixed in a bent position. Strengthening exercises build muscles.

A physical or occupational therapist will teach your child how to perform these exercises at home. Most of these must be done daily. The therapist will show your child how to use hot baths, hot packs and/or cold treatments before exercise to make the therapy easier.

Sports and Recreational Activities

Recreational activities help your child exercise joints and muscles, develop important social skills and have fun. But remember that recreational activities cannot take the place of therapeutic exercise.

Participating in sports and recreational activities helps children with arthritis develop confidence in their physical abilities. Encourage activities that exercise the joints and muscles without putting too much stress on them, like swimming.

Strong muscles and joint protection are the keys to participating in sports. Although contact sports are never recommended, even aggressive sports like soccer and basketball may not be off-limits for your child. Special exercises and protective equipment can further reduce the risk of injury, and help your child play sports she likes.

Splints
Splints help keep joints in the correct position and relieve pain. If a joint is developing a contraction (bent in the wrong position), a splint may help stretch that joint gradually back to its normal position. Commonly used splints include knee extension splints, wrist extension splints and ring splints for the fingers.

An occupational or physical therapist usually makes the splint. Arm and hand splints are made from plastic; leg splints are sometimes made of cast material. The therapist custom-makes the splint for your child and can adjust the splint as your child grows, or as the joint position changes.

Morning Stiffness Relief
Many children experience a period of stiffness when they get up each day. This morning stiffness can be one of the best measures of disease activity; the longer the stiffness lasts, the more active the disease. Taking a hot bath or shower, sleeping in a sleeping bag or warm bed, doing range-of-motion exercises, or using a hot or cold pack can help relieve it. Although most children do better with warmth, there are a few who respond to cold treatments (a plastic bag filled with ice or frozen vegetables works well).

Surgery
Surgery is rarely used to treat juvenile arthritis in the early course of the disease. However, surgery can be used to relieve pain, release joint contractures and replace a damaged joint.

In joint replacement surgery, the entire joint is replaced with an artificial joint. This procedure is used mainly in older children whose growth is complete or almost complete and whose joints are badly damaged by arthritis. This operation is usually used to replace the hip, knee or jaw joints. It can reduce pain and improve function.

Soft tissue release may sometimes help to improve the position of a joint which has pulled out of line by a contracture. In this operation, the surgeon cuts and repairs the tight tissues that caused the contracture, allowing the joint to return to a normal position.

Eye Care
Several forms of eye inflammation are associated with various forms of juvenile arthritis. However, frequent eye exams can identify inflammatory problems early and reduce the potential for serious eye complications.

Acute or sudden onset of eye inflammation is called iritis. This may occur particularly in the spondyloarthropathies. Any child with one of the JSp should be examined promptly if eye redness and pain develop. Routine examination of the eyes by an ophthalmologist familiar with this complication should be performed yearly.

Chronic uveitis or iridocyclitis occurs sometimes in children with JSp. This type of eye inflammation is most common in young girls with JRA involving a few joints (pauciarticular JRA) whose blood contains an antibody protein called the ANA. ANA tests are usually not found in children with JSp. However, this type of inflammation of the eyes may occur without obvious signs or symptoms. Therefore, it is important for all children with JRA to have their eyes checked by an ophthalmologist as soon as the diagnosis is suspected. This allows the doctor to detect any eye problems early and to begin proper treatment to avoid serious problems.

The ophthalmologist will give your child a complete medical eye evaluation, including a slit lamp test. This simple and painless procedure can spot problems before symptoms are present. Children should continue to get periodic eye exams, since eye inflammation may occur when the joint disease is inactive. The frequency of eye exams will depend on your child's risk for developing eye problems and any history of previous eye inflammation.

Dental Care
Children with arthritis may have limited jaw movement, which can make brushing and flossing their teeth difficult. Your child's dentist may suggest various toothbrush handles, electric toothbrushes, floss holders, toothpicks and rinses that will help your child maintain healthy teeth and gums.

Medications may also affect your child's oral health and development. Always inform your dentist about the status of your child's disease and the medications she is taking. The dentist will consider these when planning any treatment, general anesthesia, sedation or oral surgery. Older children who have had joint replacements may require an antibiotic before dental treatment.

The joint in front of the ears, where the lower jaw connects to the base of the skull, is called the temporomandibular joint (TMJ). Arthritis may affect this joint in the same way it does others, by causing pain, stiffness and altered growth. Jaw exercises and therapy may be recommended for the pain and stiffness. If the lower jaw does not develop properly, it may create an overbite. Your child's dentist may recommend an early consultation with an orthodontist if this occurs. Surgery is also sometimes necessary for this condition.

Diet
Children with arthritis sometimes have poor appetites, leading to weight loss and poor growth. This problem may be even more severe in children with JEA, due to the colitis. Alternatively, medication side effects may cause excessive weight gain.

Chronic disease places increased demands on a child's body and creates a need for additional calorie intake. Children may have little appetite when they feel ill, or may have a difficult time eating when they have painful joints or limited mobility. Some children with arthritis might feel too sick or too tired to eat. Encourage him/her to eat a well-balanced diet at regular meal times and include planned snacks even when he/she may not feel like eating. Try to reduce the amount of food he/she needs to eat by increasing the nutrient content of each bite of food or drink. For example, add melted cheese, gravies, margarine and dips to foods, and offer whole milk. This can help prevent weight loss and poor growth.

At the opposite end of the spectrum, children with arthritis may gain too much weight due to medication side effects or limited activity. Excess weight puts more stress on joints such as knees, hips and ankles. Appropriate exercise combined with eating a well-balanced diet based on the basic four food groups can help your child keep a normal body weight. A registered dietitian can teach you ways to improve your child's diet.

Many children with arthritis need additional calcium and vitamin D to help strengthen their bones. Giving your child a multivitamin with breakfast is usually worth the effort.

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