Chronic Fatigue Syndrome

Chronic fatigue syndrome (CFS) differs from fibromyalgia because the patients lack the typical trigger points associated with fibromyalgia. Like fibromyalgia, chronic fatigue syndrome is not associated with laboratory abnormalities, a known cause, or a known cure. Once the diagnosis of CFS has been established and other significant medical problems have been excluded, children with chronic fatigue syndrome should be rehabilitated just as I describe for fibromyalgia in the next section.

Read The Label Carefully And Dont Believe Everything You Hear

It is with dismay that I see parents bring in all sorts of things found in health food stores, noted on the Internet, advertised on television, or seen in the newspaper. Sometimes they even bring in a vitamin or supplement that another doctor was selling in his or her office (though this was declared unethical by several medical societies and may soon be illegal). The parents want me to say that it's all right to give it to their child. Most would not be fooled for a moment if the item promised...

Back Pain

In this age of increasingly large book bags and backpacks, backache is becoming a more common complaint among adolescents. Fortunately, serious back problems remain uncommon. As is true for other regions of the body, the key to assisting the physician in properly diagnosing the cause of your child's back pain is the information you provide. When you take your child to be evaluated, you should be prepared to provide answers to the following questions Did the pain come on suddenly following an...

Complications Of Lyme Disease

Fortunately, serious complications of Lyme disease are rare. The vast majority of children with Lyme-related arthritis recover promptly (within a few weeks) and completely with treatment. In children with Lyme who have large numbers of bands on their Western blot (e.g., nine or more), it is likely the infection has been present for a prolonged period. Some of these children will have continuing arthritis after the first thirty days of antibiotics. It is important to recognize that, like other...

Complications Of Osteoporosis And Osteopenia

The major complications of osteoporosis are fractures of various kinds. Stress fractures in the limbs of athletes become evident as pain at the site of the fracture. Vertebral fractures in children with chronic disease cause severe back pain. Occasionally, children are recognized to have osteoporosis only when they fracture an arm or leg after a minor fall. For children with an underlying rheumatic disease, the key to the prevention of osteoporosis is control of the inflammatory process....

Lower Leg Pain

Shin splints are a common complaint of children who do a lot of running. They typically occur in children who are trying to do too much too fast. Often the problem disappears with additional conditioning. Children with continuing pain in the shin area (tibia) should be evaluated with X-rays to be sure there is not a more serious problem (stress fractures, infections, etc.). Arch supports and orthotics may be helpful in chronic cases. Chronic compartment syndrome may be a cause of calf pain in...

Specific Conditions

Scoliosis is an abnormal curvature of the spine with rotation of the vertebrae. This curvature often results in one shoulder appearing higher than the other or the hips appearing uneven. Since the curvature occurs with growth, it is rare for scoliosis to become evident before the age of ten years. Once detected, it should be carefully evaluated and followed. Many children have only mild curvatures and require no treatment, but others have progressive disease requiring bracing and, less...

Methotrexate

Methotrexate has significantly improved the outlook for many children with arthritis. As noted above, much of what you may read about negative effects of methotrexate is related to the doses used in the treatment of children with cancer, which are frequently tens or in some cases hundreds of times higher than the doses used to treat arthritis. In the doses used in cancer therapy, methotrexate works by interfering with DNA synthesis and blocking the rapid reproduction of cells. However, in the...

Prognosis For Children With Osteoporosis Or Osteopenia

The prognosis for a child with osteopenia is very good. Identification of any underlying problem, correction of the diet, and appropriate medical therapy should lead to improvement. The prognosis for children with osteoporosis is more guarded. Parents must consider whether such children should be treated with bisphosphonates. Children with severe osteoporosis should be referred to large centers with experienced staff, where they can receive the best possible therapy. LIVING WITH A CHILD WHO HAS...

Treatment Of Osteoporosis And Osteopenia

The most important element of treatment for childhood osteoporosis is prevention. Every child should have an adequate calcium intake. If extra calcium is given in the form of supplements, it must be accompanied by vitamin D. However, while calcium and vitamin D are essential nutrients, excessive vitamin D consumption is dangerous and can even be fatal, and excessive intake of calcium should be avoided because it often causes constipation and stomach upset. Attention to diet and appropriate...

Other Vaccines

The consensus opinion is that the benefits outweigh the risks. I let parents make their own decision. Pneumococcal vaccine. It is now recommended by the American Academy of Pediatrics that young children receive the pneumococcal vaccine (Pneumovax) vaccination early in life. It prevents many types of serious pneumonia and other infections caused by the same family of bacteria. Most older children have not gotten it. It should be given to children if you think you are going to put...

Neck Pain

Most often it is due to muscle irritation involving the muscles that hold the head and shoulders in the proper position. Frequently this is because of a heavy backpack improperly carried over just one shoulder, which places unnecessary strain on the muscles. Before you go to the physician's office with your child's complaints of neck pain, think about the answers to the questions at the beginning of the section on back pain. Since the neck is simply the top...

Juvenile Ankylosing Spondylitis And Ankylosing Spondylitis

Virtually all adult men with ankylosing spondylitis (AS), which is chronic inflammation of the spine and sacroiliac joints, are positive for the genetic marker HLA B27 positive, but we do not know how many boys who are positive for HLA B27 will never develop AS. Most individuals with AS do not fulfill criteria for the diagnosis until after they reach thirty years of age, and it is difficult to keep track of HLA B27 positive teenagers for the fifteen years or more of follow-up needed to answer...

Gastrointestinal System Involvement in SLE

Irritation of the GI system occurs frequently in children with SLE. It may play a large role in the initial malaise that many children with SLE experience. The resulting pain and loss of appetite often lead to depression and weight loss. These are common problems in the period before diagnosis (note that this can happen in many other conditions as well). The most common GI complaint of children with SLE is nonspecific stomachache (abdominal pain). This can be located almost anywhere in the...

Organizations Dedicated to Other Diseases

Genzyme Corporation 500 Kendall Street Cambridge, MA 02142 (617)252-7500 Although this is a corporate Web site, it provides a wealth of information for patients with rare genetic syndromes that may be mistaken for rheumatic diseases fibromyalgia, chronic fatigue syndrome, etc. International Association for CFS ME (Chronic Fatigue Syndrome and Myalgic Chronic Fatigue and Immune Dysfunction Syndrome Association of America P.O. Box 220398 Charlotte, NC 28222-0398 (704) 365-2343 American...

Adalimumab

This is another of the new of the biologics. Adalimumab (trade name Humira) is an antibody to TNF-a that can be given as a subcutaneous injection either every week or every other week. Not only does it attack TNF-a in the circulation, but it can also attack TNF-a molecules on the surface of cells, destroying both the TNF-a and the cells. It is a very effective treatment for severe arthritis. It works well for most children with polyarticular-onset arthritis, spondyloar-thropathies, and...

Fibromyalgia

Children with fibromyalgia complain of widespread pain and fatigue that interfere with normal activities and often cause them to miss many days of school. These children have often been to a variety of physicians without a definitive diagnosis. The generally accepted criteria for a diagnosis of fibromyalgia require widespread pain (above and below the waist and on both the right and left sides) and the presence of at least eleven of eighteen defined trigger points (areas that when stimulated...

Cerebrospinal Fluid Testing

Cerebrospinal fluid (CSF) is analyzed by performing a lumbar puncture, also known as a spinal tap. It is a relatively easy test in most circumstances. The only time it is unsafe to do a lumbar puncture is if there is increased pressure in the brain, which can be detected by examining the patient's eyes for evidence of increased pressure or by a CAT scan or MRI of the brain. The fluid that is withdrawn from the lumbar puncture can be analyzed for evidence of infection, irritation of the brain,...

Other Books for Parents

Aldape, Virginia Tortorica, and Lillian S. Kossacoff. Nicole's Story A Book About a Girl with Juvenile Rheumatoid Arthritis. Minneapolis, MN Lerner Publications, 1996. Horstman, Judith, William J. Arnold, Brian Berman, J. Roger Hollister, and Matthew H. Liang, eds. The Arthritis Foundation's Guide to Alternative Therapies. Atlanta Arthritis Foundation and Longstreet Press, 1999. Lane, Nancy E., ed. The Osteoporosis Book. New York Oxford University Press, 1998. Lockshin, Michael. Guarded...

Osteoporosis and Osteopenia

Osteoporosis and osteopenia are conditions involving decreased bone mass and hence a decrease in the strength of the bones with an increased risk of fractures. Osteopenia has been defined as having bone mass that is more than one standard deviation below the normal level, and osteoporosis as having bone mass that is more than two and a half standard deviations below normal. These definitions have been difficult to apply to children because the normal levels have not been well defined for...

Reactive Arthritis Infectionassociated Arthritis And Reiters Syndrome

Reactive or infection-associated arthritis is a type of spondyloarthropathy brought on by infection. Reiter's syndrome is a special case of infection-associated arthritis with a particular set of systems (discussed later). Affected children are often very ill, with fever, rash, and arthritis. Sometimes the arthritis is in only one large joint, but at other times it may be widespread, affecting many joints both large and small. It was originally termed reactive arthritis because the arthritis...

Treatment Of Fibromyalgia And Chronic Fatigue Syndrome

My approach to treating children with fibromyalgia and CFS emphasizes rehabilitation and return to activities of daily living. If one views recovery from either of these conditions as one would view recovery from a major physical injury, the steps make good sense. The child, family, and physicians must recognize that there will not be a sudden, miraculous recovery. Progress is made through a rigorous program of slow and steadily increasing level of activities. It is important for every member...

Getting Your Child to Take the Medication

Getting your child to take his or her medication depends a lot on the age of the child. For babies, all you can do with pills is mash them up and hide them in applesauce or another favorite food and make sure it goes down. Liquid medications can often be put in with formula. A compounding pharmacist can make many medications that do not come in liquid form into liquids, and add a flavor that the child likes. You may have to ask around to find one, and your insurance company may not always pay...

Older Immunosuppressive Agents Cyclosporine and Tacrolimus

Cyclosporine (marketed as Neoral or Sandimmune) and tacrolimus (marketed as Prograf) are closely related immunosuppressive drugs that are primarily used to prevent organ transplant rejection. Cyclosporine was discovered first and is used more widely, but tacrolimus may be more potent and have fewer side effects. However, there is only limited experience with the use of tacrolimus for arthritis at present, and so I restrict the rest of this discussion to cyclosporine. Cyclosporine works...

Family History

This is one of the most important parts of evaluating children with chronic disease. Many diseases have a tendency to run in families. Often I request extra tests for a disease that I would not initially have suspected because there is a strong family history of the disease. I have discovered children with inflammatory bowel disease long before they were having abdominal symptoms because I requested the appropriate tests when I realized that they had joint pains and a family history of bowel...

Giving Your Childs History

As a parent evaluating your own child, ask yourself these questions and think about the answers. Your physician will want to know the answers to help arrive at an appropriate diagnosis. In addition, knowing the answers to these questions and what diseases they suggest will help you decide whether the physician's diagnosis makes sense to you. If it does not, discuss what you have noticed with your physician. If the physician is not interested in your child's history, something's wrong. The first...

When to Withhold Your Childs Medication

This is a subject that is frequently overlooked. If your child is sick with the flu or a virus and not keeping anything down, you do not want to give him or her NSAIDs or immunosuppressive medications. The only arthritis medication that is vital to take even when one feels sick is corticosteroids (see below). All the other medications should be avoided, and you should call the doctor for instructions. Children who are sick with a virus are also at risk of Reye's syndrome if they take certain...

Recommendations for Vaccinations in Children with Rheumatic Disease

Child with arthritis who are off all medications and have been well for six months or more All vaccines recommended may be given, but avoid rubella if the child has a positive titer, indicating immunity to rubella. Child with arthritis who is on NSAIDs but has been well for six months Most routine vaccinations are okay, but avoid rubella if the child has a positive titer. Do not give any live-virus vaccines (e.g., chicken pox herpes zoster and smallpox), as there is risk of disease and Reye's...

Getting the Best Results for Your Child

This is the most important chapter of this book. Everyone wants the best results for his or her child, but not everyone understands that you have to go out and get them. Reading this book means you are off to a good start. There are three major determinants of the outcome of any project your level of effort and knowledge, the effort and knowledge of the people who work with you, and luck. No one can control luck, so it is very important to maximize your efforts and choose the best possible...

Pauciarticularonset Juvenile Arthritis

The only divisions currently recognized among JA patients involve how the disease first appears. Children with pauciarticular onset have four or fewer joints involved during the first six months, while those with polyarticular onset have five or more joints involved during the first six months. The third category is systemic onset, which involves daily spiking fevers and rash the number of joints does not matter. To base the distinction between pauciarticular-onset and polyarticular-onset JA...

The Ultimate Responsibility For Your Childs Outcome Rests With

To provide the best possible care for your child, you must do some hard work. You must pick the right team, carefully choosing the doctors who take care of your child. Once you have chosen them, you must follow their advice. If you do not understand or do not like the advice, ask questions. Get answers. If you are not satisfied, get a second opinion. Then ask more questions. Even after you have found a doctor with whom you are comfortable, you have not transferred the responsibility for your...

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....

Alternative Medicine

Vitamins, Supplements, and Other Treatments You cannot treat illness without the risk of side effects. Whether you take the prescribed medications, buy over-the-counter medications, ignore the problem and hope it will go away, buy a supplement in a health food store, or try a cure from some other source, there are risks associated with every choice. The only difference is whether or not those risks are clearly explained to you. The physician is required to tell you about potential side effects...

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...

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...

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...

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...

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 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...

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...

Duration of Antibiotic Therapy for Lyme Disease

Children with a positive test for Lyme disease in the absence of arthritis or neurologic symptoms should be treated with 21 days of an appropriate antibiotic. If a child has arthritis, 30 days of antibiotic therapy are recommended. For a possible bacterial infection of the joint, the antibiotic may be started intravenously and switched to oral only after bacterial infection has been excluded. To control the inflammatory response in the joint, the child should also be treated with NSAIDs for as...

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...

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...

Infliximab

Infliximab (Remicade) is another antibody that is directed against TNF-a. In the published information on infliximab, it is suggested for use in conjunction with methotrexate. This is because, as with adalimumab, the antibody molecule that is the active part of infliximab sometimes stimulates an immune response in which the body produces human antichimeric antibodies, which attack the in-fliximab molecule and block its effect. There is reason to believe that taking methotrexate slows the...

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...

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...

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...

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...