Chapter 20 n n The Child With Altered Musculoskeletal Status 997

The child may be at risk for self-esteem or body image with lupus in certain families. In addition, genes on chro-
disturbances because of the limitations imposed by illness mosome 6 called immune-response genes were also associ-
and any physical manifestations. Encourage the child and ated with the disease.
family members to speak openly about the child’s condition SLE is a multisystem autoimmune disease involving
and to feel comfortable asking others for assistance when both the humoral and cellular aspects of the innate and
needed. Support organizations can provide educational acquired immune systems. The autoimmune reactions are
materials, specialized services, and financial aid for qualified directed against the cell nucleus, especially the DNA. Au-
children and their families (see for organiza- toantibodies are produced against the nuclear antigens,
tions). Important measures that promote adaptation to the cytoplasmic antigens, and blood cell surface antigens.
disease and enhance development include encouraging the When the autoantibodies bind to their specific antigens,
child’s natural skills and characteristics to develop. Provide complement activation occurs, and immune complexes
opportunities for the child to reach their maximum poten- accumulate within the blood vessel walls, causing ischemia.
tial while ensuring personal safety and management of their Ischemia within the blood vessels leads to thickening of
condition. the internal lining, fibrinoid degeneration, and thrombus
formation. At this point, manifestations of SLE appear.
Community Care
The child with JA can be managed primarily in the home. Assessment
Clinic, home health, and school nurses are primarily
The manifestations of SLE vary, depending on the
responsible for collaborating with the healthcare team to
organs affected and degree of their involvement. The
monitor and manage the needs of the child with this con-
American College of Rheumatology has established crite-
dition. Issues of impaired physical mobility, altered nutri-
ria of 11 manifestations to distinguish SLE from other
tion, risk for impaired skin integrity, and chronic pain
connective tissue diseases (Chart 20–1). Evidence of four
must be managed on a daily basis. The child also faces
manifestations in the absence of other definable disease
challenges related to altered growth and development,
entities is sufficient for the diagnosis of SLE. Generally,
and the potential for body image disturbances caused by
nonspecific findings that may be assessed at the time of
the debilitating nature and sometimes disfiguring out-
disease onset include fever, malaise, weight loss, recur-
comes of the child’s arthritic condition (see TIP 20–3).
rent abdominal pain, anorexia, and fatigue. Headaches
are present in more than 10% of children at the time of
diagnosis. Conjunctivitis also is a common early
Systemic Lupus Erythematosus (SLE) manifestation.
Arthritis and arthralgia are the most common present-
SLE is a systemic inflammatory disease that affects many ing symptoms of SLE. The child may complain of morn-
organs in the body. Lupus, the Latin term for wolf, was ing stiffness and joint pain or swelling. The arthritis of
combined with erythema to name this disease—lupus SLE is usually symmetric and affects both small and
erythematosus—because one of the presenting symptoms large joints. Commonly affected joints are hands, wrists,
is a facial rash that looks like the face of a wolf. Lupus is and knees. Joint deformities or erosions are rare. Rheu-
an autoimmune disorder that may involve any organ sys- matoid nodules may appear during periods of disease
tem but most commonly involves skin, joints, and kid- exacerbations and may disappear as the disease activity
neys. The onset may be sudden, affecting one or more diminishes.
major organ systems, or it may be insidious in nature, Dermatologic findings are the second most common
with nonspecific symptoms such as fever, fatigue, or joint manifestations. These may include maculopapular and
and muscle pain. When the onset is insidious, diagnosis is vasculitic rashes, livedo reticularis (reddish blue mottling
often delayed for weeks or months. of skin, exacerbated by exposure to cold), and periungual
Childhood SLE accounts for 15% of all cases, affecting erythema or other nail bed changes. Many acutely
approximately 5,000 to 6,000 U.S. children annually. affected individuals may have a butterfly rash across the
The incidence is highest among African American, His- bridge of the nose and on the cheeks that may spread to
panic, and Asian girls. When males are affected they the scalp, neck, chest, and extremities. The rash may
manifest symptoms at an earlier age and have more severe become bullous, possibly leading to a secondary infec-
disease (Adams, MacDermott & Lehman, 2006; Croker & tion. Photosensitivity is a classic dermatologic sign of
Kimberly, 2005). The mortality rate has decreased with SLE, especially if it occurs in the presence of arthritis.
the evolution of newer treatment options. Infection is Other skin eruptions may include vasculitic lesions
currently the leading cause of death. with ulceration; purpuric lesions; and subcutaneous nod-
ules on the palms, fingertips, soles, extremities, or trunk.
Macular and painless ulcerative lesions in the mouth and
Pathophysiology nose may be present. Alopecia, resulting from inflamma-
The cause of SLE is unknown, but genetic, hormonal, tion around the hair follicles, may lead to patchy or gen-
environmental, and immunologic factors are believed to eralized loss of hair. Hair may be coarse, dry, and brittle.
interact and lead to disease expression. Studies indicate Polyserositis, inflammation of several mucous mem-
there is a genetic predisposition to lupus. Genome studies branes, is another clinical manifestation of SLE. Pericardi-
have indicated a gene on chromosome 1 that is associated tis, peritonitis, or pleuritis may be present. Cardiovascular
998 Unit III n n Managing Health Challenges

CHART 20–1 American College of Rheumatology Classification Criteria for Systemic

Lupus Erythematosus

The diagnosis of systemic lupus erythematosus requires the presence of four or more of the following 11criteria, serially or
simultaneously, during any period of observation.

1. Malar rash: fixed erythema, flat or raised, over the malar eminences, tending to spare the nasolabial folds
2. Discold rash: erythematous, raised patches with adherent keratotic scaling and follicular plugging: possibly astrophic
scarring in older lesions
3. Photosensitivity: skin rash as a result of unusual reaction to sunlight, as determined by patient history or physician
observation
4. Oral ulcers: oral or nasopharyngeal ulceration, usually painless, observed by physician
5. Arthritis: nonerosive arthritis involving two or more peripheral joints, characterized by swelling, tenderness, or effusion
6. Serositis: pleuritis, by convincing history of pleuritic pain, rub heard by physician, or evidence of pleural effusion; or
pericarditis documented by electrocardiography, rub heard by physician, or evidence of pericardial effusion
7. Renal disorder: persistent proteinuria, > 500 mg per 24 hour (0.5 g per day) or >3þ if quantitation is not performed;
or cellular casts (may be red blood cell, hemoglobin, granular, tubular, or mixed cellular casts)
8. Neurologic disorder: seizures or psychosis occurring in the absence of offending drugs or known metabolic derange-
ment (e.g., uremia, ketoacidosis, electrolyte imbalance)
9. Hematologic disorder: hemolytic anemia with reticulocytosis; or leukopenia, < 4,000 per mm3 (4.0 3 103 per L) on
two or more occasions; or lymphopenia, < 1,500 per mm3 (1.5 3 103 per L) on two or more occasions; or thrombocy-
topenia, < 100 3 103 per mm3 (100 3 103 per L) in the absence of offending drugs
10. Immunologic disorder: antibody to double stranded DNA antigen (anti-dsDNA) in abnormal titer; or presence of anti-
body to Sm nuclear antigen (anti-Sm); or positive finding of antiphospholipid antibody based on an abnormal serum
level of lgG or lgM anticardiolipin antibodies, a positive test result for lupus anticoagulant using a standard method, of
a false-positive serologic test for syphilis that is known to be positive for at least 6 months and is confirmed by negative
Tieponema pallidum immobilization or fluoresoent treponemal antibody absorption test
11. Antinuclear antibodies: an abnormal antinuclear antibody titer by immunofluoresoence or equivalent assay at any time
and in the absence of drugs known to be associated with drug induced lupus

Adapted with permission from Tan EM, Cohen AS, fries JF, Mast AT, McShane DI, Rothfield NF; et al. The 1982 revised criteria for the classification of
systemic lupus erythematosus, Arthritis Rheum 1982;25:1274, and Hochberg MC. Updating the American College of Rheumatology revised criteria for
the classification of systemic lupus erythematosus [Letter], Arthritis Rheum 1997;40:1725.

symptoms that may develop over time include pericarditis, headache, lethargy, dizziness, seizures, ataxia, and halluci-
substernal or precordial pain, murmurs, persistent tachy- nations. Ongoing use of corticosteroids lowers the
cardia, transient dysrhythmias, and pleural and pericardial threshold for seizure activity and may cause personality
effusions. Raynaud’s phenomenon, in which vasoconstric- changes such as depression or euphoria. Depression can
tion causes blanching, cyanosis, and erythema in the toes also occur as a result of learning that one has SLE or
and fingers in response to cold or stress, may be noted. from coping with the issues associated with acute disease
Renal involvement is common in children. Nephrotic activity that limit the child’s activities.
syndrome and acute glomerulonephritis may develop and With SLE, exacerbations and remissions that vary in
are considered life-threatening occurrences. Renal insuf- severity, depending on the particular organ system
ficiency is manifested by weight gain, hypertension, involved, occur. After the first 2 years, the disease only
edema, increased serum creatinine levels, and decreased rarely involves previously unaffected organ systems. Dis-
creatinine clearance. Additionally, urinalysis reveals he- tinguishing the symptoms of disease exacerbation from
maturia, proteinuria, and increased urinary sediment. those of infectious complications may be difficult. Sus-
Almost all children with SLE have one or more hema- pect infection if fever, coughing, shortness of breath,
tologic abnormalities, including anemia, leukopenia, and chest pain, and changes in behavior and visual acuity
thrombocytopenia. Lymphoid involvement may be noted occur until proved otherwise.
in the form of generalized lymphadenopathy and
hepatomegaly.
Central nervous system symptoms may arise as indica-
Diagnostic Tests
tions of the central nervous system vasculitis, as toxic Initial screening for SLE includes the following tests: a
symptoms resulting from the medication regimen, or as complete blood count with differential, ESR, C-reactive
behavioral outcomes of this chronic illness. Central nerv- protein measurement, antinuclear antibody count, and a
ous system vasculitis can lead to irritability, depression, test to detect RF. Additional laboratory values may be
 Chapter 20 n n The Child With Altered Musculoskeletal Status 999

determined to rule out other disease processes The anti- gloves, and layered clothes. Also instruct them to avoid
nuclear antibody test identifies the presence of antinuclear tight clothing.
antibodies in the blood. The presence of antinuclear anti- Photosensitivity can be well controlled by using
bodies is a marker of an autoimmune process and is most sunscreens, avoiding sun exposure, and using steroid
commonly seen in SLE. therapy. When the child must be exposed to the sun,
emphasize the importance of using sunscreens that block
Interdisciplinary Interventions both ultraviolet A and B rays (SPF 15 or higher); avoid-
ing long-term exposure; and wearing long-sleeved cloth-
No one treatment for SLE exists; management depends
ing, pants, large-brimmed hats, and sunglasses. Not only
on the manifestations and severity of the disease. Medical
can sun exposure exacerbate the skin rash, but it may also
management is tailored to meet the individual child’s
precipitate systemic exacerbations.
needs, based on organ system involvement and on the se-
verity of inflammation at the time of evaluation. The goal
of therapy is to control both the acute exacerbations of Interventions for Systemic Involvement
the illness and the ongoing chronic disease manifestations
Major organ system involvement in SLE usually necessi-
to enable optimal functioning, to prevent scarring in any
tates the use of corticosteroids. Symptoms such as fever,
organ system, and to prevent intolerable side effects of
skin manifestations, pleuropericarditis, and lymphadenopa-
the therapy. Collaboration among healthcare professio-
thy usually can be effectively treated with low-dose predni-
nals is necessary. Child life specialists in the hospital set-
sone or hydroxychloroquine. Alternate-day steroid therapy
ting and a variety of allied health professionals in
is currently being used to minimize the linear growth and
outpatient settings, including social workers and psychol-
sexual maturation problems associated with steroid use.
ogists, may help the family and child to cope with issues
(e.g., body image concerns related to disease and medica-
tions) in a positive manner.
----------------------------------------------------
KidKare A child with SLE should wear a
Interventions for Joint Involvement MedicAlert bracelet to alert emergency personnel to
his or her dependence on steroids.
Joint involvement, specifically, arthralgias, are usually ----------------------------------------------------
controlled with NSAIDs and antimalarial medications
such as hydroxychloroquine. Aspirin is not recommended, High-dose oral prednisone for a period of 4 to 6 weeks
because the large doses needed may cause liver toxicity. may be indicated for the child with central nervous sys-
tem and renal involvement. Long-term use of high-dose
prednisone is avoided whenever possible because of the
Alert! Ibuprofen has been associated with an aseptic meningitis syn- serious complications (e.g., cataracts, fractures, hyperten-
drome in SLE; therefore, it should not be administered to children with SLE sion, and metabolic disturbances) that may occur. The
(Nguyen, Gal, Ransom, & Carolos, 2004). aim of therapy is to control activity of the disease with
the lowest possible dose of prednisone. To aid in this
process, steroid-sparing agents such as azathioprine,
methotrexate, and hydroxychloroquine may be used.
Medications must be taken daily to maintain adequate
When the child’s condition has been stabilized, the
blood levels. NSAID therapy requires careful monitoring
high-dose steroids are tapered to prevent the negative
of renal function, because these agents decrease glomeru-
effects of sudden medication withdrawal.
lar blood flow and can precipitate acute renal failure in
Instruct parents regarding the need for an ophthalmic
children with SLE.
evaluation for retinal damage within the first 30 to 60
A physical therapy program can be implemented to
days after initiating drug therapy and every 6 months
help the child manage joint pain, enhance ROM, and
thereafter. These evaluations aid in detecting and pre-
prevent injury and contractures. Periods of rest should be
venting macular inflammatory problems.
incorporated into the child’s daily routine, especially dur-
Periodic blood work for evaluating drug toxicity and
ing periods of exacerbation.
disease activity must also be discussed with the patients
and parents.
Sexually active adolescent females need counseling
Interventions for Skin Involvement about birth control and pregnancy. Inform them that
The rash of SLE is generally treated with an antimalarial SLE can become more active with the use of certain oral
drug, preferably hydroxychloroquine. Topical steroids contraceptives and during pregnancy. It is usually recom-
may also be used, if cutaneous involvement is limited. mended that a diaphragm and spermicide be used for
Because hydroxychloroquine carries a risk of retinal dam- birth control because they do not have adverse side
age, an eye examination should be performed every effects that affect the child’s SLE. However, low-dose
6 months in patients receiving this drug. oral contraceptives may be used if the patient is carefully
Rashes and lesions require careful monitoring for signs monitored, especially if compliance with barrier contra-
of infection. In addition, assess the toes and fingers for ception is poor. The fetus is at risk for injury both by the
vascular compromise. Urge the child and parents to keep mother’s disease and by the side effects of the drugs
the extremities warm during cold weather by using socks, required to treat the mother’s disease.
1000 Unit III n n Managing Health Challenges

Nutritional Support esteem. The undesirable effects of medication therapy,

such as weight gain and ‘‘moon face,’’ the restrictions
The child receiving steroid therapy needs to be moni- related to photosensitivity, and the increased risk for
tored for weight gain and fluid retention. If renal involve- depression are among the factors that can affect the
ment is a concern, a low-sodium, low-protein diet may be child’s self-concept. The child may refuse to follow pre-
instituted. Adolescent females on steroid therapy should ventive measures, thereby precipitating an acute episode
ingest increased amounts of calcium. of the condition (Developmental Considerations 20–2).
The healthcare team and family members should strive
Interventions to Enhance Growth and to help the child achieve as normal a lifestyle as possible
within the constraints of the illness. Encourage independ-
Development ence in decision making while still monitoring for safety
The child common needs help to deal with any problems and wellness. Social workers or psychologists trained to deal
that he or she may have related to self-image and self- with chronic illness in children may be helpful in assisting

Developmental
Considerations 20—2
Management Issues for Children With Systemic Lupus Erythematosus

Actions Demonstrated Healthcare Provider and

Concerns of the Child by Child Family Interventions
‘‘I don’t want to be differ- Refuses to take medica- Arrange medication schedule to elimi-
ent than other tions while at school nate or minimize need to take medi-
children.’’ cations during school and social
activities.
‘‘I don’t like the effects of Avoids taking medication Have parent unobtrusively monitor
medications on how I child taking medication.
look.’’
‘‘I want to participate in Refuses to avoid the sun Help child select hats, clothing,
summer activities in the lotions, and other protective gear
sun.’’ that can be used to protect against
overexposure to the sun.
‘‘Applying sunscreen is Does not use sunscreen Review with the child the importance
messy and not cool.’’ consistently each day of using sunscreen. Have the child
when out in the sun select a favorite type of sunscreen to
use. Have the child apply sunscreen
at home before meeting friends.
‘‘Rashes make me look Does not want to socialize Encourage the child to use hypoaller-
funny.’’ with others or go out in genic cosmetics to cover rashes.
public
‘‘I can’t do anything. I Feels depressed or Assist parents to emphasize child’s
can’t be like my other different positive attributes and accomplish-
friends.’’ ments. Encourage participation in
counseling or support group.
‘‘I look fat.’’ Feels depressed; cries Help the child select clothes that cam-
ouflage weight gain.
‘‘I am too tired to play Wants to be excused from Assist the child to pace activities and
with other kids.’’ school activities and periods of rest to minimize fatigue.
extracurricular activities Assist the child to select activities
because of fatigue that are not as physically demand-
ing, such as piano playing, art work,
and computer activities.
 Chapter 20 n n The Child With Altered Musculoskeletal Status 1001

them to learn to cope with their frustrations. Support Fractures

groups that enable children to meet others with SLE and to
discuss common concerns are useful for some children. A fracture occurs when the bone receives more stress
Several organizations offer written information about SLE than it can absorb. The most common reasons for frac-
and can direct families to local support groups and activities tures in children are falls, motor vehicle accidents, bicycle
in their community (see for organizations). accidents, and accidents that occurred while at school.
The incidence of sports-related fractures is increasing
(Rewers et al., 2005).
Fractures in children vary in several important ways
Musculoskeletal Trauma from fractures in adults, and these variables affect care.
First, a child’s bone heals faster than an adult’s bone. The
younger the child, the faster the bone heals, because
Question: The radiograph in the emergency
younger children have a proportionately higher metabolic
department shows that Chase has an oblique fracture
rate. Children’s bones are also softer than an adult’s
of the fifth metacarpal. It is a closed fracture and the bone is
bones, and their bones may bend or buckle rather than
not out of alignment. He has a fiberglass cast applied to his
break. Another crucial difference between managing adult
right hand. What are the developmental principles of working
and pediatric fractures is that children’s bones have an
with adolescents that you will use to develop an individual
open growth plate (epiphysis). Any damage to the growth
teaching plan for Chase, who now has a cast?
plate can result in limb length discrepancy, joint incon-
gruity, and progressive angular deformity of the limb.
Childhood trauma has been the leading cause of death During the birthing process, fractures may occur in the
among children for nearly 50 years, and it has been the newborn. However, fractures generally are rare during
second leading cause of morbidity. The most common the first year of life, because the child has limited mobil-
childhood musculoskeletal trauma includes sprains, fol- ity. Multiple, severe fractures in an infant may be an indi-
lowed by lacerations, fractures (excluding the skull), in- cation of a metabolic bone disease, such as osteogenesis
tracranial injuries, and internal injuries. The most serious imperfecta. During the first 2 years of life, many fractures
injuries tend to be fractures and intracranial injuries. in children may be the result of physical abuse.

Alert! In a nonambulatory child, the accidental occurrence of a spiral

Sports Injuries fracture is rare. Thus, spiral fractures in young children are most often the
result of twisting of the extremity by an abusive adult.
During the past 20 years, participation in organized ath-
letics by children and adolescents between ages 6 and 21
years has grown considerably. An estimated 3 million
injuries are incurred annually during sports participation When the child starts to walk, the clavicle and radius
by children in the United States. Approximately 25% to are the most commonly fractured bones. Fractures can
30% of sports injuries occur during organized sports, and occur from trauma, such as sudden twisting of a limb or a
another 40% occur during unorganized sports (Bautista force applied to the limb, such as a kick. Pathologic frac-
& Flynn, 2006). More than 200,000 injuries per year are tures occur when preexisting diseases weaken a bone, as
related to use of playground equipment, with 88% of happens with bone tumors.
these injuries occurring on monkey bars, jungle gyms, In general, fractures in children heal without complica-
swings, and slides. The highest injury rate among high tions. Possible complications such as limping, decreased
school athletes is in baseball, followed by soccer, basketball, ROM, and nerve deficits rarely occur.
and football. Gymnastics, tennis, track and field, and cross-
country events have lower rates (Bautista & Flynn, 2006).
In recent years, injury rates have been dramatically
reduced because of changes in safety requirements for
Pathophysiology
children involved in sports. Preseason examinations, medi- The amount of force required to fracture a bone depends
cal coverage at sports events, proper coaching, adequate on the strength of the bone, the size of the bone, and
hydration, proper officiating, proper equipment, and other extrinsic factors, such as the direction of the force.
improved field and surface playing conditions have the After the fracture occurs, inflammation develops at the
potential to reduce sports injuries. Chapters 6 and 7 dis- site. Osteoblasts (bone-forming cells) activate within 24
cuss safety concerns and sports activities in more detail. hours to begin making new bone. During the ensuing few
Table 7–1 discusses the ergogenic aids, such as steroids, weeks, callus forms and knits together into compact bone.
that young athletes are using or considering in attempts to This process takes 4 to 12 weeks in children, depending
enhance their athletic performance. Use of these aids may on their skeletal age. Remodeling, a process that rounds
have severe health outcomes for the youth and may con- off angles and fills in hollows, continues for up to 1 year
tribute to sports injuries. after the fracture. Because the ability of bone to remodel
This section describes the care of children with fractures, is enhanced in children, the ends of the fracture do not
overuse injuries, sprains and strains, and dislocations. have to be aligned perfectly, as they do in adults.