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....

Abatacept

Abatacept (brand name Orencia) is a new agent that decreases the signaling between T cells and other cells in the immune system. Abatacept's mechanism of action is termed co-stimulatory blockade. In the presence of infection, cells in the immune system interact to alert the immune system to the need for an inflammatory response. To minimize inappropriate messages, cells communicating with each other need to interact at several different levels they need to be HLA-compatible, they need to be...

Aggressive Versus Conservative Approaches

There are aggressive and conservative physicians. What you want is a physician who is conservative when appropriate and aggressive when appropriate, not one who is always aggressive or always conservative. Some muscle, bone, and joint diseases tend to resolve over time. These are best treated conservatively. Some get steadily worse, and the longer you wait before you stop them, the more damage accumulates. You need a physician who can tell which is which and respond appropriately. The only way...

Alternative Medical Systems

We've all probably heard stories of people who got better after they prayed to a certain saint, went to Lourdes, had acupuncture done, or took up Zen. Thirty years of caring for children with rheumatic disease have made it clear to me that I cannot always explain why their symptoms get better or worse on a given day. The diseases do wax and wane over time. Sometimes people are going to get lucky and get better right after doing something unlikely to really help. Arguing with them that it was a...

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...

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...

Aspirin

Aspirin was the original NSAID and was the mainstay of therapy when I began my career. On a worldwide basis aspirin remains one of the most widely used treatments for children with arthritis. However, its use in the United States is limited by the ready availability of other medications that are more convenient, equally effective, and less likely to cause side effects. It has disadvantages it needs to be given three or four times a day, it is available only as pills, and it frequently upsets...

Azathioprine

Azathioprine (Imuran), which works by inhibiting DNA synthesis, is one of the oldest of the immunosuppressive drugs. There is extensive experience using aza-thioprine in the treatment of children with arthritis and the vasculitic diseases. It has been useful in children with severe polyarticular-onset or systemic-onset JA. In the past, it was the primary immunosuppressive therapy, but with the widespread use of methotrexate, the biologics, leflunomide, and mycophenolate mofetil, there has been...

Benign Hypermobile Joint Syndrome

The diagnosis of benign hypermobile joint syndrome requires that a child be able to do each of the following Bend the fingers back over the wrist so that they are parallel with the forearm (i.e., they point straight backward) Easily bend the thumb back to touch the forearm Fully extend the elbow all the way beyond straight (hyperextension) Fully extend the knee beyond straight Bend over and touch the palms to the floor with the knees straight The ability to bend joints to a greater than normal...

Benign Hypermobile Joint Syndrome and Ehlers Danlos Syndrome

The role of the ligaments is to maintain the joints in proper position with respect to each other. If they are loose, they don't perform this function properly. Children with either benign hypermobile joint syndrome or Ehlers-Danlos syndrome have loose ligaments, but that is where the similarity ends. Benign hypermobile joint syndrome is not really a disease at all. Instead, it is an inherited variation on normal, and children with benign hypermobile joint syndrome are often referred to as...

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...

Brain and Nervous System Involvement in SLE

Seizures, strokes, and other serious abnormalities are possible but relatively infrequent consequences of SLE. Brain damage due to strokes is usually very dramatic. It can result in sudden inability to use a hand, leg, or whole side of the body. Sometimes the stroke may affect a speech center, making it impossible for the child to talk, or the balance centers, making the child unable to walk normally. Whenever there is a sudden change in the ability of a child with SLE to function normally, the...

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...

Chlorambucil

Chlorambucil (brand name Leukeran) is a very potent immunosuppressive agent that does not have the bladder-irritating properties of cyclophosphamide. It has been used for systemic-onset arthritis, vasculitic diseases, uveitis, and a variety of other life-threatening conditions. Chlorambucil does significantly impair the ability to deal with infections. Chlorambucil is not widely used because it has been associated with the development of leukemia, sterility, and other complications far more...

Clinical Manifestations Of

The patterns of disease involvement with SLE range from children with fever, rash, aches, and pains but no serious organ damage to children with no complaints of fever or rash who are found to have serious kidney or blood (hemato-logic) involvement on a routine screening test. Serious internal organ involvement most often takes the form of kidney involvement, but the brain, heart, lungs, and other internal organs also may be seriously affected. Many children with SLE have a mild version that is...

Complications of Benign Hypermobile Joint Syndrome

Significant complications of benign hypermobile joint syndrome are very rare. However, the same material that makes up ligaments also makes up internal structures such as the aortic root, the ring of tissue that connects the aorta (the large main blood vessel that carries blood to the rest of the body) to the heart. If a child has very lax ligaments, he or she should have an echocardiogram done to look for any loosening of the aortic root. If this ring tears, it can cause a rip to form in the...

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...

CREST Syndrome

CREST syndrome is a variation of systemic scleroderma that has several peculiar aspects. The name CREST is an acronym that comes from the findings of calcinosis (pieces of calcium under the skin), Raynaud's phenomenon, esophageal problems, sclerodactyly (tight skin on the fingers), and telangiectasias (small red spots due to abnormal blood vessels in the skin). The key findings that distinguish CREST from PSS are the presence of telangiectasias and anticentromere antibodies. The general...

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...

Information for Patients on Various Medical Procedures

Total Hip Replacement A Guide for Patients hipreplace index.html Total Knee Replacement A Guide for Patients Virtual Hospital has a variety of other useful pages found at http www.vh.org. Textbooks for Physicians Anderson, Steven J., and J. Andy Sullivan, eds. Care of the Young Athlete. Rosemont, IL American Academy of Pediatrics and American Academy of Orthopedic Surgeons, 2000. Cassidy, J., and R. Petty. Textbook of Pediatric Rheumatology, 4th ed. Philadelphia W. B. Saunders, 2001. This is...

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...

Kawasaki Disease

Kawasaki disease usually begins in a young child as fever and irritability without apparent explanation. Within the first few days, some children develop a swollen lymph node below the jaw. Usually this is thought to be a bacterial infection and is treated with antibiotics. Young children with high fevers who do not develop a swollen lymph node are frequently begun on antibiotics for presumed ear infections. Some children already have a rash at this point, but often the child will develop a...

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 Tests And Other Evaluations

Proper diagnostic testing may include blood tests, X-rays (radiographs), bone scans, MRI, ultrasound (sonograms), and even biopsies of affected joints and tissues. Chapter 22 offers an extensive discussion of common laboratory and diagnostic tests. It is important to remember that diagnostic tests are not a substitute for knowledge and judgment. I often see children who previously have had thousands of dollars' worth of unnecessary tests, resulting in the wrong diagnosis. The key to proper...

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...

Medications and Immunizations

I want you to understand why your child should take medicine (if it has been prescribed) and how to be sure your child gets the best results. Understanding your child's medications and making sure he or she takes them appropriately are vitally important in getting the best outcome. I do not discuss every possible medication in this chapter, just the medications I commonly use in the treatment of rheumatic disease I do use others as the situation warrants. Different physicians have different...

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...

Piroxicam

Piroxicam (marketed as Feldene) is an NSAID that is very effective for many older children with spondyloarthropathies and the related types of arthritis. It has a very long half-life and slowly builds up to an effective level in the body. Often it takes two or three weeks to start having an effect. However, it is frequently very effective when other NSAIDs have not been sufficient. Gastrointestinal side effects and renal side effects may be more common with piroxicam than with other NSAIDs and...

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...

Prognosis For Children With

Despite the foregoing information about how the most difficult cases are treated, you should know that with early and aggressive treatment the vast majority of children do just fine. Some children do die from SLE, but the survival rate at five years is above 90 percent in every experienced center, and in some centers it is much higher. Of course, thinking just in terms of five-year survival is certainly not good enough for a fourteen-, fifteen-, or sixteen-year-old child. The main reason for...

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...

Reconstructive Surgery

The goal of every pediatric rheumatologist is to maintain and restore the fullest possible level of function. In an ideal world, every child would receive proper medical attention early in the disease course and respond dramatically to therapy. Unfortunately, in the real world children do not always receive proper medical attention early in the course of their disease. In addition, some of those who do receive proper medical attention nonetheless fail to respond well. For those patients, our...

Reflex Sympathetic Dystrophy

Reflex sympathetic dystrophy (RSD), reflex neurovascular dystrophy, and complex regional pain syndrome are all various names for the same condition. It is a cause of great frustration for parents and physicians, and it is far more common than you might think. RSD can involve the foot or hand. It rarely occurs in children under the age of eight, but may occur at any age thereafter. Because there is virtually always a well-documented history of injury preceding the chronic pain, families and...

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.

Sources Of Help

There are local and national organizations that can help parents of children with a wide variety of conditions. See the Resources for a listing. These organizations can be very helpful in directing you to other parents who have had similar experiences. They can provide useful information regarding the nature of your child's condition, experienced doctors, and other resources. They can also help you to find experts for dealing with the many problems of insurance, school, and so on. Many of the...

Specialized Laboratories For Rheumatic Disease Testing

Rheumatology Diagnostics Laboratory 10755 Venice Boulevard Los Angeles, CA 90034 (310) 253-5455 or (800) 338-1918 Prometheus Laboratories, Inc. 9410 Carroll Park Drive San Diego, CA 92121 (888) 423-5227 Mayo Medical Laboratories Multiple sites and phone numbers see the Web page for details for your area.

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...

Treatment Of Sjogrens Syndrome

Artificial tears and artificial saliva are very important parts of the treatment of children with Sjogren's syndrome. Some teenagers are reluctant to use them at school for fear of seeming different. However, the dry eyes and dry mouth can do so much damage that it is very important to encourage routine use of artificial tears and saliva even at school. Hydroxychloroquine is often helpful in slowing the progression of the disease. Corticosteroids are useful if the symptoms are more severe or...

Vitamins

Every child with chronic disease should be on a daily vitamin that contains the appropriate amounts of vitamins A, B (all types), C, D, E, and K, folic acid, iron, and calcium. Children with iron deficiency, whether from inadequate intake, from chronic blood loss, or because their bodies don't utilize iron well, may need additional iron supplementation. However, iron-containing medications may cause upset stomach, and overdosage of iron can be harmful and possibly even fatal, so keep the pills...

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...

Xrays

If a bone is painful, it is appropriate to begin the evaluation (after a complete history is taken and a thorough physical examination is done) with an X-ray to eliminate the possibility of fracture or structural abnormality. X-rays are also useful to determine whether bones are out of alignment or abnormally curved. An X-ray may be the only study necessary to establish the diagnosis of a broken bone, slipped capital femoral epiphysis, scoliosis, or any of a number of other orthopedic...

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....