Polyarticularonset Juvenile Arthritis

Polyarticular-onset disease is defined as arthritis involving five or more joints during the first six months of disease, without other explanation. In my opinion, any involvement of small joints indicates polyarticular-type disease even if initially there are fewer than five joints involved. Polyarticular-onset disease may occur in any age group but is found more often in girls than in boys. There are two major peaks in the age at onset of disease in young children between eighteen months and...

Prognosis for Children with Pauciarticular Onset JA

The prognosis for the typical child with true pauciarticular arthritis is very good. Most children respond as expected to therapy and are well within a few months. My normal standard is to treat a child until there has been no evidence of active disease for six months. This often means nine months to a year of treatment. At that point, I will discontinue the NSAIDs and watch carefully. Most children (about 80 percent) will remain well without medication. In a few children, the disease will...

Nonsteroidal Antiinflammatory Drugs

Nonsteroidal anti-inflammatory drugs (NSAIDs) are the mainstay of therapy for most children with rheumatic disease. All of them interfere to varying degrees with the cyclooxygenase pathway, which is responsible for the production of prostaglandins, important inflammatory mediators (chemicals that cause fever, pain, and irritation). By blocking the production of these inflammatory mediators, NSAIDs serve to reduce the amount of pain, fever, and irritation that the child experiences. Most of the...

Note About Sports And Pain In Children

Sports injuries are the most common causes of muscle, bone, and joint pains in childhood. Some of these, particularly overuse injuries, are described in the preceding sections. Often these are minor injuries that resolve over a few hours or days at most. However, pain that is severe or persists requires medical attention. Also, any child who is repeatedly injured or in pain every time he or she participates needs to be evaluated to find out why. You may have heard the saying No pain, no gain....

Ankle Pain

The ankle is particularly vulnerable in growing children. Most ankle sprains are easily recognized and treated. More severe injuries should be evaluated by a physician to determine whether X-rays are necessary to exclude the possibility of a fracture. This is especially true if there is evidence of bruising. Chronic or recurrent ankle injuries require careful medical evaluation. Some of these are due to partial tears of the ligaments that support the ankle. Others may be due to damage to the...

Appendix

There are thousands of organizations, Internet sites, books, and other resources for families of children with rheumatic diseases and related problems. There is no way I could list all of them here. I have listed the major organizations and others with which I have worked. I'm sure this list is incomplete. Many of these organizations have a Web page that may direct you to many others. My own Web page is http www.goldscout.com. I regularly update the site with the latest information. I have also...

Arthroscopic Knee Surgery And Knee Replacement

Total knee replacement (TKR) surgery has been very successful in maintaining the ability of children to carry out their activities of daily living. With improved medications and the ability to suppress arthritis in the knees using intra-articular corticosteroids, TKR due to arthritis has become rare in children. Continuing active synovitis in a knee that has not responded to routine medications or intra-articular corticosteroid injection may be treated with an arthroscopic synovec-tomy...

Bone Scans Gallium Scans Pet Scans

Bone scans are based on the uptake of the radioactive isotope technetium-99 (Tc-99) by active bone cells. Normal bone cells are not very active, but if there is an injury, arthritis, infection, or tumor, the bone cells become active in trying to repair the damage, and so they take up more calcium and related substances. When Tc-99 is injected, active bone cells take it up. Because it is radioactive, a picture of where it went in the body can be made using a gamma counter. Bone scans can find...

Celecoxib

Celecoxib (marketed as Celebrex) is a useful NSAID that provides a good level of relief for some children. Celecoxib is available as a liquid and has been specifically approved for children. It has a reduced frequency of side effects, but this medication does contain sulfa, and children who are allergic to sulfa drugs may be allergic to celecoxib. There has been a lot of concern about the incidence of heart attacks in people taking COX-2 inhibitors, some of which were pulled off the market...

Children Who Fight Taking Their Medication

There are a number of elements to consider in dealing with children who fight taking their medication. The easiest answer is to avoid getting into this situation. Finding a suitable compromise by making the medication into a liquid or offering ice cream, candy, and so on is usually preferable to an all-out battle. For children in the younger age groups, a reward system (or bribery) is often the easiest and most effective answer. As parents, we all believe that we should not have to bribe our...

Complications Of Mctd

The complications of MCTD are highly dependent on the evolution of the disease. The arthritis, rash, malaise, and Raynaud's phenomenon are usually easily treated with NSAIDs, low-dose prednisone, a calcium-channel blocker for the Raynaud's, and hydroxychloroquine. Inadequately treated children may have recurrent problems with Raynaud's and may have distal fingertip ulcers and problems with blood supply to parts of the digits in the cold. Physicians must emphasize to families the importance of...

Complications of Nonspecific Spondyloarthropathies

Complications that do not involve the joints (extra-articular complications) are most common in children who have one of the associated conditions that are discussed below. However, a few specific complications are well recognized to occur in children with nonspecific spondyloarthropathies. Acute anterior uveitis is the most common. This is a painful eye disease involving the front of the eye. Often the eye appears very red, and vision may be affected. This is quite different from the silent...

Complications Of Raynauds

If a child is known to have an underlying rheumatic disease, the presence of Raynaud's phenomenon does not necessarily indicate more serious disease. However, there are several complications related to Raynaud's that must be considered. In more severe cases, children with Raynaud's may have problems not only in their fingers but also in their toes, earlobes, and tip of the nose. All of these areas should be protected if the child is to be subjected to significant cold exposure. It is also...

Complications Of Sjogrens Syndrome

The most common complications of Sjogren's syndrome are a direct result of the dry eyes. Dry eyes are easily scratched, and the scratches (corneal abrasions) are both painful and ultimately damaging to the lens of the eye. These children need frequent monitoring by an ophthalmologist. The dry mouth results in a very high frequency of cavities, as saliva normally helps wash away the bacteria responsible for tooth decay. These complications can become quite serious, and it is important to make...

Complications Of

Complications of SLE in childhood may be complications of the disease, of the therapy, or of both. It is very important to understand that half of the children diagnosed with SLE in the 1950s (before the routine use of corticosteroids) died within two years. While diagnosis and treatment have improved and the picture is much brighter today, it remains true that untreated SLE may be a rapidly fatal disease. While treatment certainly has possible side effects, those must be balanced with the risk...

Corticosteroids

The discovery of corticosteroids was a major advance in the care of children and adults with rheumatic diseases. The beneficial effects of steroids result from their ability to block the effects of most inflammatory messengers (cytokines) and decrease the activity of the cells that promote inflammation. For children with severe diseases, the corticosteroids have vastly improved their quality of life. However, the excessive use of corticosteroids has many negative effects, and they should be...

Cyclophosphamide

Cyclophosphamide (Cytoxan) is the most potent of the commonly used immunosuppressive agents. It is rarely used in the treatment of children with JA. Its use in the treatment of SLE is discussed in detail in Chapter 9. Cyclophosphamide can be given as daily pills or as intravenous injections. The intravenous injections are given on a variety of schedules, but the most common is monthly initially, then every three months until the course of therapy is completed. Except in special situations,...

Dealing With Friends And Neighbors

To someone who does not have a child with a chronic problem, this section might sound unnecessary. However, all of us involved with children with serious diseases know how hard it is to deal with the questions and the stares when you are out in public with a child who looks or acts different. Our number one goal as physicians is to make it so that no one will be able to tell your child has a problem, but we don't always succeed completely. The hardest part of this is that many people who stare...

Dealing With Schools

Many children with relatively minor disease escape having significant problems at school. But if your child has a chronic condition that is obvious to everyone, or even a mild condition that prevents full participation in physical education, the school will need to know. There are a number of important things to know when dealing with your child's school. The most important is that the Americans with Disabilities Act put all the power on your side. If a few notes and an occasional phone call...

Depression Patient Fatigue

I have been discussing how hard it is to be the parent of a child with arthritis. What I have not talked about yet is how hard it is to be the child with arthritis or another chronic condition. When children are young, their problems are the responsibility of their parents. The children expect their parents to make them better. For most children with arthritis and related conditions, this is possible. The vast majority of the children I care for go on to live productive lives. However, the...

Diagnosis Of Kawasaki Disease

The most important aspect of the diagnosis and treatment of KD is prompt recognition of the disease. However, there are a variety of illnesses that may produce a similar appearance. It is very important to be sure that the child is not suffering from a significant infection. Measles, streptococcal infections, drug reactions, and many forms of vasculitis may result in a clinical picture that satisfies the criteria for a diagnosis of KD. Hemolytic uremic syndrome has also been confused with KD....

Diagnosis Of

The first step in treating a child with RSD is to make sure the diagnosis is correct. While I have often seen physicians fail to make this diagnosis, I also have had children with undiagnosed arthritis referred as having RSD. In RSD, the blood work is usually normal. In long-standing cases, X-rays may show some mild osteoporosis due to disuse. An MRI may show some patchy marrow edema, and the bone scan may show increased uptake, decreased uptake, or patchy increased and decreased uptake. On...

Diagnosis When To Suspect

SLE most often affects girls in the teenage years, but it may affect boys and girls at any age. Although a butterfly-shaped rash on the face is considered a characteristic feature of the disease, it is found in only one-third of children when they first come to the doctor's office. Since many doctors do not think of SLE unless they see the butterfly rash, many children have symptoms of SLE for months before the proper diagnosis is made. The key to a prompt diagnosis of SLE is for primary-care...

Diagnostic Ultrasound Sonography

Ultrasound is a relatively new technology that may allow us to study muscles, bones, and joints at far less cost and without the risks associated with radiation. At the present time, the use of ultrasound to evaluate joint swelling and tendon inflammation is rapidly advancing. These tests may be available to your doctor and may be quite helpful in evaluating problems in the muscles and joints. Ultrasound can also be used to guide the doctors when they need to inject joints they cannot see, such...

Differential Diagnosis Of Mctd And Laboratory Findings

As noted, the clinical manifestations of MCTD overlap with dermatomyositis, SLE, JA, and early scleroderma. Consequently, physicians must rely more heavily on laboratory findings to confirm this diagnosis. The source of much of the confusion in diagnosing MCTD results from the unclear nature of the disease itself. Indeed, it is quite possible that we will ultimately realize that at least two different diseases have been grouped together as MCTD. A high-titer speckled-pattern ANA is...

Penicillamine

D-penicillamine clearly affects the course of JA, morphea, and scleroderma, but it has a very slow onset of action and a high frequency of toxic reactions (routine blood test abnormalities, skin rashes, kidney irritation, and neurologic abnormalities). Because of these drawbacks, D-penicillamine was rapidly replaced by methotrexate for the treatment of children with arthritis. D-penicillamine may still have a role for children who have not responded to other medications, but it is rare to see...

Druginduced

Drug-induced SLE refers to the development of a positive ANA and SLE-like symptoms in association with certain drugs. Among the drugs that can do this are commonly used medications such as tetracyclines (including doxycycline, used in the treatment of teenagers with acne), certain of the drugs used to treat children with seizures, and some antibiotics (including isoniazid, used to treat tuberculosis). For some drugs, the association with drug-induced SLE is well known, but for many others it is...

Ehlersdanlos Syndrome

The classical presentation of Ehlers-Danlos syndrome is in a child who is tall (usually thin) with long arms, long legs, and long thin fingers (children with Marfan's syndrome may also have some of these characteristics). These children have a severe defect in their collagen and are easily recognized because it is easy to stretch their skin. If these children have a cut, it will heal poorly and the scars often become unusually large and thin. By the time they are ten or eleven years old, these...

Erythrocyte Sedimentation Rate ESR

There are several variations of the ESR, but all of them reflect how the red cells (erythrocytes) interact with each other in the blood, as measured by how fast the red blood cells in a vial of blood settle to the bottom. During physiological stress (including but not limited to illness), the body produces proteins called acute phase reactants, which cause the red blood cells to move closer together and thus to settle to the bottom faster. Children with severe illness often have a high ESR, and...

Family Issues

Most of the parents reading this book will not have a child with a serious chronic condition. They can skip this section. However, if your child does have a significant disability, or you think your child's medical care is starting to take over your life, please read on. If you have lived with a child with a chronic condition for any period of time, you know that this places a strain on everyone in the family. Any chronic childhood illness is not the child's problem it is the family's problem....

Figuring Out Whats Wrong

When a child has muscle, bone, or joint pain, it is important to understand that each of the conditions that may be responsible has a typical set of problems it causes (symptoms), a typical age group in which it occurs, and other typical findings that usually make it easy for an experienced physician to diagnose. At the same time, if a child does not have the typical problems or is not the typical age, it's much less likely that the suspected condition is the proper explanation. This is why...

Following Advice And Keeping Appointments

It may seem obvious, but following your doctor's advice and keeping your appointments are the most important things you can do for your child. All of us want to believe the problem will just go away if we ignore it, but when it comes to rheumatic disease and other chronic illnesses, ignoring the problem makes it worse. All too often I see children who were supposed to be back in two weeks who come back in six months and still have the problem. Often the parents will say that the child took a...

Foot Surgery Tarsal Fusion

One of the more difficult problems for children with arthritis is involvement of the joints between the tarsal bones of the feet. These joints are important when you walk over an irregular surface or bend your feet. If they become significantly involved by arthritis, it may result in a lot of pain when walking. A surgical procedure called a triple arthrodesis will fuse these bones together. This results in a stiff foot and will cause difficulty when the child walks over an uneven surface, but...

Getting Back on Schedule

Anyone who has traveled overseas and suffered jet lag can immediately relate to the impact of disordered sleep patterns that is typical of fibromyalgia and CFS. Just as the key to overcoming jet lag is to promptly begin to set your body's wake sleep cycle on the appropriate schedule for the time zone you are in, families must start children back on a regular schedule of getting up at an appropriate time in the morning (the same time as if they were going off to school normally). This should be...

Growing Pains

Whenever a child limps or complains that an arm or leg hurts, our first thought is that he or she must have injured it, even if the child does not remember having done so. The family is likely to seek medical advice only if the pain is very severe or persists for more than a day or two. Even when the pain or limp continues beyond a few days, many parents and physicians dismiss the problem as growing pains. Children do have growing pains in fact, they are fairly common. Unfortunately, many...

Growing Up Is Hard To Do

Another difficult situation for families of an older child with chronic disease is allowing the young adult to accept increasing responsibility and ultimately total responsibility for his or her care. Some hospitals have special adolescent clinics, while others sponsor specialized transition clinics for older children with chronic disease. But in most situations, if the physicians and family have done a good job of preparing them, as they get older the children will naturally make the...

How to Use This Book

If you are the parent of a child who has been given a diagnosis, you should begin by reading about that diagnosis to see whether it accurately describes your child's symptoms. For parents who know their child's diagnosis, this book has useful chapters on the latest medications, the meaning of laboratory test results, family issues, getting the best care for your child, and reconstructive surgery. You will find a lot of information that will allow you to make informed choices. If you are the...

Hypogammaglobulinemia Associated Problems

IgA deficiency is a low level of immunoglobulin A (see Chapter 22). Children with IgA deficiency develop rheumatic diseases much more frequently than the normal population. While this often takes the form of a spondyloarthropathy, studies have shown an increased frequency of children with IgA deficiency in virtually every rheumatic disease. There are two important problems to be aware of regarding a child who is recognized to have IgA deficiency. The first is to recognize that...

Intraarticular Corticosteroids

Triamcinolone (Aristospan), betamethasone (Celestone), and methylprednisone (Depo-Medral) are corticosteroid preparations often used for intra-articular injections, where the drug is injected directly into the inflamed joint. MRIs have shown that these injections have a very positive effect on reducing the inflammation and promoting healing of the cartilage. However, it is important that children not resume activities that may be harmful to their joints simply because the injections have made...

Intravenous Gamma Globulin

Intravenous gamma globulin (IV IgG) received a lot of attention in the early 1990s. It is still used for many diseases by some physicians, but the cost is extremely high and its use has largely been supplanted by the biologies. Some physicians still believe this is an excellent medication for dermatomyositis. It is very effective for children with Kawasaki disease (see Chapter 15). The difference is that Kawasaki disease is an acute illness. Once you have treated a child for Kawasaki disease...

Introduction

I am writing this book in the hope that it will speed the proper diagnosis and treatment of children with rheumatic disease. The pages that follow are based on my thirty years of experience and will provide the information you need to understand what is happening to your child and your family, and what you need to do to get the best outcome. One of the questions parents most frequently ask me as they come to grips with their child's diagnosis of rheumatic disease is Why did this happen Medical...

Laboratory Findings

As previously noted, normal test results do not necessarily mean the absence of disease. Many children with chronic diseases begin to lose their energy and feel unwell long before their routine laboratory results become abnormal. It is important to make sure that all of the appropriate tests have been done. Also, remember that things change over time. Tests that were normal on a first visit may not be normal four or six months later. In the search for an explanation for a chronically ill child,...

Laboratory Testing And Typical Patterns Of Disease In Children With

SLE is an enormously variable illness. Different patterns of abnormalities may help to distinguish children with severe disease from others with milder cases, helping to ensure they get appropriate treatment before significant damage occurs. Virtually all of the children with SLE are ANA-positive. However, many children test positive for ANA but do not have SLE. Physicians soon recognized that there were different patterns of ANA as seen under the microscope (see Chapter 22 for more on this)....

Laboratory Testing Of A Child With Raynauds

Laboratory evaluation of a child with Raynaud's should include a complete blood count, erythrocyte sedimentation rate, antinuclear antibody, rheumatoid factor, anticardiolipin antibodies, clotting studies, and thyroid function tests. If there are no suggestive findings on history or physical examination and all of these tests are normal, there is little likelihood of an underlying rheumatic disease. However, the presence of a positive ANA, positive RF, anticardiolipin antibodies, or an elevated...

Medical Treatment

While medications are a key component of therapy for adults with fibromyalgia or CFS, they are less important in children. However, NSAIDs, including tramadol, may be helpful in controlling the aches and pains associated with activity. Pregabalin is a medication that has been approved for the treatment of fibromyalgia. It reduces the transmission of nervous impulses carrying the sensation of pain. It certainly helps, but it is not magic. Amitriptyline is an antide-pressant that has been found...

Medications

It is important to tell the physician about all the medications your child is taking, including vitamins, supplements, and any medications you obtained without a prescription. (I gave Johnny one of Grandma's pills that she had left over from when she had bronchitis can turn out to be the explanation for the entire problem if it's an allergic reaction to Grandma's medicine.) The physician needs to know what has been done to treat the problem in the past. He or she also needs to know what other...

Miscellaneous Resources School Related Resources

A Guide to the Individualized Education Program by the Office of Special Education and Rehabilitation Services U.S. Department of Education, July 2000 Parent Advocacy Coalition for Educational Rights Band-aides and Blackboards Joan Fleitas, Ed.D., R.N, Associate Professor of Nursing Lehman College Bronx, NY 10468 This site is dedicated to helping children with chronic disease and their parents deal with school-related issues.

Mixed Connective Tissue Disease

Children with mixed connective tissue disease (MCTD) are ANA-positive like children with SLE, but typically have high titers of antibodies to RNP as well. The precise relationship of MCTD to SLE and the other rheumatic diseases remains unclear. Symptoms of MCTD may also be characteristic of dermatomy-ositis and scleroderma. Obviously, these diseases are somehow interrelated, but there is no clear explanation yet. Although MCTD was originally described as a subtype of SLE, it is probably more...

Morphea

Morphea is the most common form of scleroderma in childhood. Morphea consists of areas of thickened skin commonly on the body (trunk), but sometimes on arms or legs. Its cause is unknown. Families usually notice a patch of pink and irritated skin that looks like many common skin conditions. If the patch is due to morphea, it typically does not itch or hurt. Often the area of reddened skin does not attract any particular attention until it has persisted for several weeks. It will not improve...

Naproxen and Ibuprofen

Naproxen (marketed as Aleve and Naprosyn, among other brand names) and ibuprofen (Motrin, Advil, Nuprin) are available in liquid form and are used extensively in the treatment of children with arthritis. Naproxen has an advantage in being given twice a day, while ibuprofen may need to be given as many as four times a day. Ibuprofen liquid and pills, and some forms of naproxen pills, are available without a prescription. The reason ibuprofen liquid is available without prescription while...

Newer Biologic Agents

There are several agents under development that may be available soon, but the uncertain nature of the final stages of clinical testing and governmental approval make this uncertain. One is a different agent to block IL-1 that has a different site of action and only needs to be given every two weeks. There are several drugs undergoing testing that block IL-6, a potent inflammatory mediator that is known to go up and down at the same time as the fever and rash come and go in children with severe...

Other Modalities Plasmapharesis

Plasmapharesis (also called apharesis) is a technique by which the patient's blood is removed from the body so the plasma (the clear fluid left after the blood's cells are removed) can be removed and replaced, while the blood cells are returned to the patient. If there is a toxic element in the plasma, plasmapharesis will remove it. At one point, plasmapharesis was considered a possible therapy for a wide variety of diseases. However, it was found to lack lasting beneficial effects. Today,...

Other Overlapping Conditions

Somehow all of these diseases that look so different in their typical forms are interrelated, but we clearly do not understand the connections. I have seen several children who simultaneously have skin lesions of morphea on their backs and abdomens, indentations typical of linear scleroderma en coup de sabre on their foreheads, ocular inflammation such as that seen in children with juvenile arthritis, and areas of linear scleroderma on their legs. Other overlap conditions may also occur. The...

Past Medical History

This is another long set of questions that doctors ask and families frequently wonder about. The physician needs to know whether the child has other illnesses or conditions that may be related to the symptoms or relevant to the treatment. You should try to give the doctor as much information as possible. A child might be ten or eleven years old, but the strange problem he or she is having may be the result of something that happened in the neonatal intensive care unit shortly after birth. The...

Physical and Occupational Therapy

Physical and occupational therapy play a vital role in the care of children with severe fibromyalgia. Just as a child severely injured in an auto accident would never be expected to recover without physical therapy, a child with severe fibro-myalgia or CFS should not be expected to do it on his or her own, either. The key is finding a therapist who understands that the injury in fibromyalgia is every bit as real as the injury to the child who was in an accident. The program of slowly increasing...

Prognosis For Children With Dermatomyositis

The prognosis for most children with dermatomyositis is very good. Most children have unicyclic disease and do well. With early diagnosis and prompt therapy, most children with polycyclic disease also have a good outcome. An experienced medical team can usually handle recurrent infections of subcutaneous calcifications without major problems, provided the patient seeks care promptly. Most parents should regard dermatomyositis as a very treatable condition with an excellent prognosis. Children...

Prognosis For Children With Sjogrens Syndrome

The long-term prognosis for children with primary Sjogren's syndrome is unclear. Some children develop other rheumatic diseases over time. In that case, the underlying rheumatic disease determines the prognosis. Failure to attend properly to recurrent ocular or dental problems may have significant consequences. Serious complications related to kidney disease and vasculitis are infrequent. Because Sjogren's syndrome is rare in childhood, there are no good reports describing the extended...

Proper Monitoring

In this section I discuss the monitoring procedures that I believe should be done to get the best possible results. Keep in mind that this may not reflect every doctor's standard of care. Medicines tend to have two types of side effects. The first is an idiosyncratic reaction, in which the patient responds to the drug in an unusual way. This is similar to an allergic reaction but not usually a true allergy. Some people are just unusually sensitive to certain medicines. The other type of side...

Psychological and Emotional Support

During the initial weeks of establishing a program of consistent wake sleep hours and exercise, it is normal for the child to increase his or her complaints at certain points and the parents to become discouraged. Psychological and emotional support from the family and the psychologist treating the child is critical at this point. Antidepressant medications may be necessary for some children during this stage. At the beginning of the program, children will experience difficulty adjusting their...

Quantitative Immunoglobulins IgG IgA IgM

Immunoglobulins, the antibodies made to fight infections, are measured in the blood by the total protein but can be more precisely measured by specific tests. In a normal immune response, the body recognizes a foreign antigen and begins making antibodies against it by making IgM. As the immune response matures, the body begins to make IgG. IgA is also made in the secondary stage. There are additional antibody classes IgE and IgD that play a role in specific diseases. IgG is the main class of...

Review of Systems

The review of systems is your doctor's last try to find out anything you forgot to mention. Is the child allergic to any drugs Does the child have any bleeding problems Are there any problems with the hair, eyes, or ears I ask about everything from the top of the head to the bottom of the feet. No one not the physician, the child, or the family knows for sure whether the answer to the problem is going to become obvious from these questions. Often it does not, but we never know until we ask.

Second Opinions

One of the most difficult issues for parents is when their child does not seem to be getting better. First, be sure you are doing what the doctor recommended. If you are, let the doctor know your concerns and discuss them with him or her. A good doctor knows that the child's health is the most important thing. If a family wants a second opinion, a good doctor will not act insulted. Instead, he or she will encourage the family. However, make sure you go to a well-qualified physician for the...

Sucralfate

This drug is not an anti-inflammatory, but I include sucralfate (sold as Carafate) under this category because it was developed to minimize stomach irritation secondary to other medications. It is frequently added by rheumatologists if a child complains of stomach irritation with NSAIDs. It essentially coats the stomach but lets the medicine get absorbed into the body. The key thing to remember about sucralfate is that it takes time for the medication to dissolve in the stomach and spread over...

Sulfasalazine

Sulfasalazine (Azulfidine), which combines salicylic acid (a precursor of aspirin) with sulfapyridine (an antibiotic), is an older drug that is very effective for spon-dyloarthropathies, especially in children who have not responded to other NSAIDs, but less effective for other forms of arthritis in childhood. Its mechanism of action is unclear. Sulfasalazine can be used in addition to other NSAIDs. The major disadvantage of sulfasalazine is that anyone who is allergic to sulfa drugs will be to...

Tests for the Evaluation of Children Who Are Just Not Doing Well

Erythrocyte sedimentation rate (ESR) T3, T4, TSH, antithyroid antibodies, antithyroid peroxidase (thyroid function studies) IgA tissue transglutaminase (antiendomysial antibodies, associated with celiac disease) Creatine kinase (CK) and aldolase (muscle enzymes) Complete chemistry panel (a broad panel of general tests, including liver and kidney function) Glucose, calcium, albumin, and total protein Electrolytes including sodium, potassium, carbon dioxide, and chloride Kidney tests including...

The Child Who Hurts All Over

Children who complain of constant pain are a source of great concern for both parents and physicians. While there are many possible explanations, these symptoms may be the first indication of rheumatic or other serious diseases. Parents who are concerned should schedule a full physical examination, making sure the staff knows this is not a routine physical. Your child is far more likely to get the care he or she needs if your physician knows why you are coming and is thinking about the problem...

The Complete Physical Examination

If you have a child with an obvious injury to an arm or leg, then you want the physician to examine the limb carefully. However, if your child is having recurrent problems with injuries or has complaints without an obvious explanation, you want to be sure the child is examined completely. Physicians who are used to dealing with injuries often forget this. There's no space in this book to go into the many specific findings that an experienced physician will look for during a physical...

Treatment of Benign Hypermobile Joint Syndrome

The joint pains and irritation associated with benign hypermobile joint syndrome can be relieved with mild nonsteroidal anti-inflammatory drugs (NSAIDs), but the key to proper treatment is minimizing the activity until the body matures further. Physical therapy is often useful to strengthen the muscles around the joint. Surgery is necessary only if severe joint damage has occurred, which is rarely the case. Surgery intended to prevent the bone from repeatedly popping out of the joint is usually...

Treatment Of Mctd

The standard treatment for MCTD is a low dose of corticosteroids combined with hydroxychloroquine and an NSAID for relief of the arthritis. The corticos-teroid dosage should be adjusted as necessary to correct the elevated IgG level, erythrocyte sedimentation rate, anemia, and clinical symptoms. Significant Raynaud's syndrome should be treated with an appropriate calcium-channel blocker. Although stronger agents are available if these do not provide relief, most children with MCTD will do well...

When Nothing Is Found

Families and physicians often become frustrated when, despite an entirely normal diagnostic evaluation, a child continues to complain of feeling unwell and is unable to continue his or her normal daily activities. Often the relationship between the family and the physician becomes adversarial the family knows there is something wrong with the child, but the physician knows he or she cannot find anything wrong. It is vital for everyone involved to recognize that they are on the same side. Often...

Why Take Medicines if They Are Potentially Dangerous

It can be difficult for a parent to give a child medication that carries the risk of serious side effects. However, if the benefits of these drugs did not far outweigh the risks, no physician would be recommending them. And most medicines are not normally dangerous. In cases where a child's medical condition is life-threatening, the choice of whether to give a medication that potentially has serious side effects is usually not difficult. But parents may be more reluctant if the problem seems to...

Wrist Surgery

The wrist is often significantly involved in children with psoriatic arthritis and some related spondyloarthropathies. It may also be involved in polyarticular-onset juvenile arthritis. There are two major problems. One is a wrist that hurts whenever it is bent. Some children get relief of their symptoms by wearing a splint on the wrist. If there is active arthritis, the splint may even result in the wrist fusing itself. If the splint does not provide adequate relief, it may be necessary to...

Side Effects of Corticosteroids

The side effects of corticosteroids are numerous and very common. They include Cushing's syndrome, fluid retention, increased appetite and weight gain, truncal obesity (skinny arms and legs but increased fat on back and stomach), moon face (fat cheeks), stretch marks, acne, growth retardation, bone-weakening calcium loss, avascular necrosis, muscle weakness, poor blood sugar control (diabetes), cataracts, increased intraocular pressure, increased infections, oral and vaginal thrush,...

Treatment of Nonspecific Spondyloarthropathies

Treatment for children with spondyloarthropathies must be appropriate to their level of discomfort and their risk of developing severe disease. For example, girls who are at low risk of significant long-term complications infrequently require second-line agents unless they have obvious swollen joints or an elevated erythrocyte sedimentation rate. Of the NSAIDs, diclofenac, nabumetone, piroxicam, etodolac, oxaprozin, and indomethacin are generally more effective for enthesi-tis than ibuprofen or...

Eosinophilic Fasciitis A Disease That Can Evolve Into Scleroderma

Eosinophilic fasciitis is an unusual disorder characterized by the acute onset of pain and swelling in an extremity. It is named for the fact that in affected individuals, the connective tissue, or fascia, is inflamed (fasciitis) and there is an abundance of eosinophils (a white blood cell type associated with allergies and not commonly present in the fascia). Typically, the skin in the affected area is very red, tender, and swollen. This inflammation is followed by hardening of the skin and...

Systemiconset Juvenile Arthritis

Systemic-onset juvenile arthritis refers to the onset of arthritis with fever and a characteristic rash. Systemic-onset juvenile arthritis has no relationship to adult-onset rheumatoid arthritis and most likely no relationship to the other forms of juvenile arthritis, either. It is best considered an entirely separate disease. Although all children with systemic-onset arthritis share key characteristics, the outcome of systemic-onset disease is so varied that it is difficult to believe it is in...

Stateoftheart Care For Children With Juvenile Arthritis

State-of-the-art care for children with pauciarticular-onset, polyarticular-onset, and systemic-onset juvenile arthritis requires that physicians and families make sure the inflammation is promptly brought under control and not allowed to cause continuing joint damage. In the past physicians believed that children who had evidence of low-grade active disease but seemed to be doing okay should not be treated aggressively. We now know this is wrong, as the earliest damage is to the rapidly...

Eye Complications of Juvenile Arthritis and Related Conditions

In juvenile arthritis and related conditions, the eyes may be involved even when there is no evidence of active joint disease. Ocular complications may take several forms. Children with pauciarticular-onset, polyarticular-onset, and psoriatic arthritis are all at risk of developing eye inflammation (chronic anterior uveitis). In this condition, inflamed cells accumulate in the eye and the resultant irritation may cause damage to the colored part of the eye (iris), the lens, and other structures...

Complications of Reactive Arthritis

Since, by definition, infection-associated arthritis resolves within a year of onset, long-term complications are infrequent. There may be complications related to the initial infection, but most children recover fully and do well. Reiter's syndrome, as noted previously, is a special case of reactive arthritis. It is distinguished from other cases of reactive arthritis by the occurrence of arthritis, urethritis (irritation of the urinary tract), and conjunctivitis. Children with Reiter's...

Treatment of Reactive Arthritis

The most important step in the treatment for children with any kind of infection-associated arthritis involves making sure the infection is properly treated. Once it is clear that the infection associated with the arthritis is no longer active, these children should be treated just like other children with spondyloarthropathies. Most respond well to NSAIDs. In most cases, the arthritis resolves completely over a period of a few months. TNF inhibitors are sometimes required, while other children...

Complications of Psoriatic Arthritis

The primary complications of psoriatic arthritis are related to recurrent arthritis. Some children develop significant joint damage over the course of their disease. Serious eye involvement can occur in young children, and children with ANA-positive psoriatic arthritis must be monitored just as carefully as children with ANA-positive pauciarticular-onset JA. Fortunately, eye involvement is less common in teenagers but still possible. Persistent wrist and finger involvement is often prominent....

Treatment of Arthritis Associated with Gastrointestinal Complaints

Adalimumab has recently been approved as therapy for inflammatory bowel disease. This agent is extremely effective for the arthritis of IBD as well as for the disease itself. Use of adalimumab is increasing, as there is growing hesitation on the part of gastroenterologists to use NSAIDs in children with inflammatory bowel disease because of the medications' propensity to cause gastrointestinal side effects. Physical and occupational therapy for these children is the same as for other children...

Post Streptococcal Reactive Arthritis and Rheumatic Fever

Post-streptococcal reactive arthritis is another form of infection-associated arthritis. It differs in that we know this form of infection-associated arthritis is initiated by a group A streptococcal infection. Poststreptococcal reactive arthritis behaves just like the other forms of infection-associated arthritis. Sometimes the arthritis is in a single large joint such as the hip, and at other times it can affect multiple joints. There is a lot of confusion regarding the relationship of...

Organizations Dedicated Primarily to Arthritic Conditions Juvenile Rheumatoid Arthritis Spondyloarthropathy Ankylosing

Box 7669 Atlanta, GA 30357-0669 (800) 283-7800 http www.arthritis.org The American Juvenile Arthritis Organization (AJAO, is now part of the Arthritis Foundation and may be accessed via its contact numbers. Arthritis Society of Canada 393 University Avenue, Suite 1700 Toronto, Ontario M5G 1E6, Canada (416) 979-7228 http www.arthritis.ca Arthritis Insight http www.arthritisinsight.com This is a Web-based, question-and-answer site with information for children and...

Juvenile Arthritis

Arthritis is defined as pain, swelling, or limitation of motion in a joint. According to the criteria of the American College of Rheumatology, juvenile rheumatoid arthritis (JRA) is the proper diagnosis for any child with the onset of arthritis before sixteen years of age if the arthritis lasts at least six weeks in more than one joint or three months in a single joint, without other explanation. Based on this definition, every child with chronic arthritis has JRA. However, it is very clear...

Specific Hip Conditions

Toxic synovitis is an inflammation of the hip that typically occurs in children from four to six years of age. Although it often occurs in children with evidence of a viral respiratory infection, its cause is unknown. Children with toxic synovitis have a very characteristic story. Most often the child went to bed well or with a slight sniffle the night before. In the morning the child has severe hip pain and is unable to walk. Because the symptoms are so dramatic, the children are immediately...

Complications of Polyarticular Onset JA

The complications of polyarticular-onset juvenile arthritis are primarily those of the arthritis itself. Pain, swelling, and limitation of motion may result in weakness, bone loss (osteoporosis), and difficulty in activities of daily living. This type of arthritis may spread to involve the hip in some children, and hip replacement surgery is sometimes necessary to maintain function (see Treatment of Polyarticular-Onset JA, below). Involvement of the wrists and fingers may also limit function....

Treatment of Polyarticular Onset JA

Medical therapy for severe arthritis has progressed dramatically in the last twenty years. The potential side effects and appropriate monitoring for each of the medications are discussed in detail in Chapter 20. A few children with mild polyar-ticular-onset arthritis will respond well to NSAIDs most of these need the stronger NSAIDs (diclofenac is often my first choice), but some find nabume-tone, naproxen, or ibuprofen adequate. For children with more severe disease, sulfasalazine is often an...

Complications of Systemic Onset JA

The complications of systemic-onset arthritis may take many forms. In addition to the problems of fever, rash, and arthritis, many children will have a small pericardial effusion (fluid around the heart) on an echocardiogram. These are often insignificant, but if they become large they may cause difficulty. An enlarged liver or spleen may be found. An excess of protein in the urine is a major cause for concern, as there is a strong association between systemic-onset arthritis and amyloidosis...

Treatment of Systemic Onset JA

The medications used in the treatment of systemic-onset arthritis are essentially the same as those used to treat polyarticular-onset disease. However, there are a number of key differences. Virtually all of the medicines used to treat JA may irritate the liver, but in children with systemic-onset arthritis, the disease itself often causes irritation of the liver. As a result, children with systemic-onset arthritis must be monitored carefully for signs of liver irritation, especially after any...

Prognosis for Children with Systemic Onset JA

The course of systemic-onset arthritis is highly varied. Some children make a complete recovery in a short period of time and never have further problems. Other children have chronic debilitating disease that leaves them with permanent limitations. There are three general groups of children with systemic-onset arthritis, but not all children fit one of these descriptions. The first group consists of children who have an acute onset of fever, rash, and arthritis that responds quickly to...

Nonspecific Spondyloarthropathies

Nonspecific spondyloarthropathies, also called enthesitis-associated arthritis or seronegative enthesitis-arthritis syndrome (SEA syndrome), are very common. Children with this diagnosis have the typical findings of a spondyloarthropathy without a recognized associated condition (for example, reactive arthritis). The majority of these children have little if any joint swelling and rarely experience significant problems. But the discomfort and stiffness associated with the enthes-itis may result...

Systemic Lupus Erythematosus

Systemic lupus erythematosus (SLE) is a complex autoimmune disease that most often affects teenage girls and young adult women. Less frequently, SLE occurs in both older and younger individuals and in boys. SLE often begins with fevers, rash, joint pains, and fatigue. Although these symptoms are worrisome, it is the ability of SLE to affect many different internal organs (brain, heart, lungs, muscles, kidneys, skin, joints, liver, or intestine) that makes this a serious illness. It is important...

Systemic Forms Of Scleroderma Progressive Systemic Sclerosis

The form of scleroderma called progressive systemic sclerosis (PSS), also referred to as diffuse scleroderma, is the most severe form of the disease, with the subtle onset of skin tightening and in many cases shortness of breath. Children and families may be unaware of the disease until it has become well advanced. Because the onset of disease is slow and gradual, families often are unaware of the problem until a dramatic event occurs a cold and numb finger, the inability to play favorite...

Immunosuppressive Drugs

If a child with arthritis has failed to respond to NSAIDs, hydroxychloroquine, and sulfasalazine, the family will have to consider immunosuppressive medications or one of the newer biologics discussed below (although the biologics are classified separately, they are in fact immunosuppressive). The majority of children with arthritis do not require immunosuppressive drugs, but children with more severe arthritis will do much better if they are appropriately treated and their disease is brought...

Raynauds Phenomenon

Maurice Raynaud (1834-1881) was a French medical student who noted color changes in the hands of some women while standing outside in the cold waiting for the streetcar during the winter in Paris. Raynaud's phenomenon refers to the typical hand changes he described. Raynaud's disease (primary Raynaud's) refers to the typical hand changes occurring in the absence of any other rheumatic disease. Raynaud's syndrome (secondary Raynaud's) refers to the typical changes occurring in the setting of an...

TNF Inhibitors

There are a variety of biologic agents currently available which interfere with the activity of tumor necrosis factor alpha (TNF-a), and several more are in early clinical testing. When large amounts of TNF-a are in the circulation, people tend to feel very ill. The effects of blocking TNF-a are often very impressive. Parents frequently report striking improvement within hours of the first dose. For most children, the improvement continues for as long as the child remains on a TNF inhibitor....

Rituximab

Rituximab (marketed as Rituxan) was initially developed for the treatment of lymphomas. It is a monoclonal antibody directed against CD20, which is a surface marker present on activated B cells and perhaps some other lymphocytes, but not on mature plasma cells. When it was noted that some lymphoma patients who also had rheumatoid arthritis improved, studies of rituximab therapy for rheumatoid arthritis were begun. It is now recognized the rituximab is effective in the treatment of rheumatoid...

Choosing the Correct NSAID

As a physician, I want to give children with arthritis the NSAID that makes them better with the least likelihood of side effects and the greatest convenience. Like most doctors, I start with the easy ones and carefully move on to the stronger ones if the response to the easy ones is inadequate. The list of side effects is essentially the same for all the NSAIDs it is the probability of the side effects that changes. One of the most common problems is for children to be treated with only one...

Side Effects of NSAIDs

Allergic reactions can occur with any medications. Few children are allergic to NSAIDs, but if there is a history of allergy to aspirin or similar medications, caution is warranted. Some children who are allergic to aspirin are allergic to all NSAIDs, but others are not. You will need to work carefully with your doctor to evaluate this problem if it occurs. All NSAIDs may irritate the lining of the stomach. This may cause indigestion or loss of appetite. It is important to make sure the child...