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2 | Nursing Care Management 109 1

UNIT 2: A CHILD WITH MUSCULOSKELETAL DISORDER

1.0 Intended Learning Outcomes


a. Identify risk factors predisposing to musculoskeletal disorder.
b. List signs, symptoms of a child with musculoskeletal disorder.
c. Describe the importance of obtaining a history in patient with musculoskeletal
disorder.
d. Describe the most suitable management option(s)/ nursing care for patient
with musculoskeletal disorder.

1.1. Introduction

The skeletal system, composed of more than 200 bones connected by the joints and tendons,
provides the structural casting or protective armor for the internal organs of the body and supplies
the body with red and white blood cells grown in the central marrow. Skeletal muscles, which are
attached to the bones by connective tissue, tendons and ligaments, allow for voluntary movement,
including gross motor activities, such as running and fine motor activities such as writing.
Together, the skeletal and muscular systems both support the body and make coordinated
movement possible. Because their bones and muscles are still growing, children suffer from
disorders of the musculoskeletal system more frequently than do adults. The difference between
adults and children is growth. Childhood fractures (break in the continuity or structure of bone)
heal much more quickly than fractures do in adults and because a child’s bone grows from the
epiphysis or growth plate, a fracture within this area may cause serious complication, such as a
deformity. Nurses play a key role in teaching parents’ ways to expose children who have muscular
or skeletal disorders to the same sorts of stimuli that might be experienced if they were able to move
around independently (Silbert-Flagg, J. & Pillitteri, A. (2018).

1.2 Topics/Discussion (with Assessment/Activities)

OSTEOMYELITIS

Osteomyelitis is an infection of the bone (Tafin-Kampe and Kamsu-Foguem, 2013). Acute


osteomyelitis is often the result of a hematogenous spread of bacteria such as Staphylococcus
aureus in older children and by Streptococcus pyogenes in younger children. The infection may
occur after extensive impetigo, burns or something as simple as furuncle (skin abscess) when the
infectious organism is then carried through the blood supply to the bone. It also may occur directly
by outside invasion from a penetrating wound, open fracture or contamination during surgery
(Silbert-Flagg, J. & Pillitteri, A.,2018).

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The age of the child often determines where the infection will take place. In younger than 1 year of
age, the capillaries near the epiphysis are often the transport for bacteria. In older children, the rich
sluggish blood flow makes the metaphysic the most common site based on the location of the
vascular sinusoids (Schreiber, Illingworthe and Ward, 2016).

Pathophysiology

Invasion by one or more pathogenic microorganisms stimulated the inflammatory response in bone
tissue, a condition known as osteomyelitis. The inflammation produces an increased vascularity
and edema often involving the surrounding soft tissues. Once inflammation is established, the
vessels in the area become thrombosed and release exudates into bony tissue. Ischemia of bone
tissue follows and necrotic bone results. This area of necrotic bone separates from surrounding
bone tissue and sequestrum is formed. The presence of sequestrum delays bone healing and causes
superimposed infection, often in the form of bone abscess. As shown below (Figure 54-3), the cycle
repeats itself as the superimposed infection leads to further inflammation, vessel thromboses and
necrosis (Ignativicius and Workman, 2006).

Osteomyelitis is categorized as exogenous, in which infectious organisms enter from outside the
body as in an open fracture, or endogenous, in which organisms are carried by the bloodstream

from other areas of infection in the body. Endogenous osteomyelitis may also be referred to as
hematogenous osteomyelitis. A third category is contiguous, in which bone infection results from
skin infection of adjacent tissues. Osteomyelitis can be further divided into two major types; acute
osteomyelitis and chronic osteomyelitis (Ignativicius and Workman, 2006).

Source: Ignatavicius, D. and Workman, M. L. (2006). Medical Surgical Nursing Critical Thinking for Collaborative
Care (5th Edition, Volume 1 and Volume 2). Elsevier (Saunders).

Causes

Most cases of osteomyelitis are caused by staphylococcus bacteria, types of germs commonly found
on the skin or in the nose of even healthy individuals.

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Germs can enter a bone in a variety of ways, including:

• The bloodstream. Germs in other parts of your body — for example, in the lungs from
pneumonia or in the bladder from a urinary tract infection — can travel through your
bloodstream to a weakened spot in a bone.
• Injuries. Severe puncture wounds can carry germs deep inside your body. If such an injury
becomes infected, the germs can spread into a nearby bone. Germs can also enter the body if
you have broken a bone so severely that part of it is sticking out through your skin.
• Surgery. Direct contamination with germs can occur during surgeries to replace joints or
repair fractures (https://www.mayoclinic.org/diseases-
conditions/osteomyelitis/symptoms-causes/syc-20375913).

Risk factors

Your bones are normally resistant to infection, but this protection lessens as you get older. Other
factors that can make your bones more vulnerable to osteomyelitis may include:

Recent injury or orthopedic surgery

A severe bone fracture or a deep puncture wound gives bacteria a route to enter your bone or
nearby tissue. A deep puncture wound, such as an animal bite or a nail piercing through a shoe, can
also provide a pathway for infection.

Surgery to repair broken bones or replace worn joints also can accidentally open a path for germs to
enter a bone. Implanted orthopedic hardware is a risk factor for infection
(https://www.mayoclinic.org/diseases-conditions/osteomyelitis/symptoms-causes/syc-
20375913).

Circulation disorders

When blood vessels are damaged or blocked, your body has trouble distributing the infection-
fighting cells needed to keep a small infection from growing larger. What begins as a small cut can
progress to a deep ulcer that may expose deep tissue and bone to infection.

Diseases that impair blood circulation include:

• Poorly controlled diabetes


• Peripheral artery disease, often related to smoking
• Sickle cell disease (https://www.mayoclinic.org/diseases-
conditions/osteomyelitis/symptoms-causes/syc-20375913).

Problems requiring intravenous lines or catheters

There are a number of conditions that require the use of medical tubing to connect the outside
world with your internal organs. However, this tubing can also serve as a way for germs to get into
your body, increasing your risk of an infection in general, which can lead to osteomyelitis.

Examples of when this type of tubing might be used include:

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• Dialysis machine tubing


• Urinary catheters
• Long-term intravenous tubing, sometimes called central lines
(https://www.mayoclinic.org/diseases-conditions/osteomyelitis/symptoms-causes/syc-
20375913).

Conditions that impair the immune system

If your immune system is affected by a medical condition or medication, you have a greater risk of
osteomyelitis. Factors that may suppress your immune system include:

• Cancer treatment
• Poorly controlled diabetes
• Needing to take corticosteroids or drugs called tumor necrosis factor inhibitors
(https://www.mayoclinic.org/diseases-conditions/osteomyelitis/symptoms-causes/syc-
20375913).

Illicit drugs

People who inject illegal drugs are more likely to develop osteomyelitis because they may use
nonsterile needles and are less likely to sterilize their skin before injections
(https://www.mayoclinic.org/diseases-conditions/osteomyelitis/symptoms-causes/syc-
20375913).

Assessment/Clinical Manifestations/Signs And Symptoms

• Localized bone pain


• Tenderness, heat and edema in the affected area
• Guarding of the affected area
• Restricted movement in affected area
• Purulent drainage from a skin abscess (https://www.rnpedia.com/nursing-notes/medical-
surgical-nursing-notes/osteomyelitis-nursing-management/).

Systemic symptoms

• High fever and chills in acute osteomyelitis


• Low-grade fever and generalized weakness in chronic osteomyelitis
(https://www.rnpedia.com/nursing-notes/medical-surgical-nursing-notes/osteomyelitis-
nursing-management/).

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Source: Ignatavicius, D. and Workman, M. L. (2006). Medical Surgical Nursing Critical Thinking for Collaborative
Care (5th Edition, Volume 1 and Volume 2). Elsevier (Saunders).

Laboratory and diagnostic study findings

• White blood cell count reveals leukocytosis


• Erythrocyte sedimentation rate is elevated
• Blood culture identifies the causative organisms.
• Radiographs and bone scan demonstrate bone involvement in advanced disease
(https://www.rnpedia.com/nursing-notes/medical-surgical-nursing-notes/osteomyelitis-
nursing-management/).

Medical Management

• Initial goal is to control and arrest the infective process.


• Affected area is immobilized; warm saline soaks are provided for 20 minutes several times a
day
• Blood and wound cultures are performed to identify organisms and select the antibiotic
• Intravenous antibiotic therapy is given around-the-clock.
• Antibiotic medication is administered orally (on empty stomach) when infection appears to
be controlled; the medication regimen is continued for up to 3 months
• Surgical debridement of bone is performed with irrigation; adjunctive antibiotic therapy is
maintained (https://www.rnpedia.com/nursing-notes/medical-surgical-nursing-
notes/osteomyelitis-nursing-management/).

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Nursing Diagnosis

• Pain related to inflammation and swelling


• Impaired physical mobility associated with pain, immobilization devices, and weight-
bearing limitations
• Risk for extension of infection: bone abscess formation
• Deficient knowledge about treatment regimen (https://www.rnpedia.com/nursing-
notes/medical-surgical-nursing-notes/osteomyelitis-nursing-management/).

Nursing Management

1. Protect the affected extremity from further injury and pain by supporting the limb above
and below the affected area.
2. Prepare the client for surgical treatment, such as debridement, bone grafting or amputation,
as appropriate.
3. Administer prescribed medications, which may include opioid and non-opioid analgesics
and antibiotics.
4. Promote healing and tissue growth.
o Provide local treatments as prescribed (e.g. warm saline soaks, wet to dry dressings)
o Provide a diet high in protein and vitamins C and D
(https://www.rnpedia.com/nursing-notes/medical-surgical-nursing-
notes/osteomyelitis-nursing-management/).

Nonsurgical/ Surgical Management

Nonsurgical Management. To reverse osteomyelitis, the health care provider initiates antibiotic
therapy as soon as possible.

➢ Drug therapy – teach the client and family that antibiotic therapy must be continued over a
long period of time to be effective. Wound may be irrigated, either continuously or
intermittently, with one or more antibiotic solutions. Medications are also needed to control
pain.
➢ Infection control – strict aseptic techniques is used when dressings are changed to prevent
further contamination.
➢ Hyperbaric oxygen therapy (HBO) – with HBO therapy, the affected area is exposed to a
high concentration of oxygen that diffuses into the tissues to promote healing (Ignativicius
and Workman, 2006).

Surgical Management. Antibiotic therapy alone may not be sufficient to meet the goals of treatment.
Surgical techniques are used to minimize the disfigurement that heretofore has been a devastating
result of severe osteomyelitis. Most often, surgery is reserved for clients with chronic osteomyelitis.

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➢ Sequestrectomy – because bone cannot heal in the presence of necrotic tissue, a


sequestrectomy is performed to debride the infected bone and allow revascularization of
tissue.
➢ Bone graft – One of the most popular surgical techniques is the Papineau procedure, or open
cancellous bone graft, which is used primarily with large bone and soft-tissue defects. The
surgeon excises necrotic bone, grafts the bone and covers the skin, if necessary. The donor
bone is most often taken from the client’s posterior ileum, but can be taken from other sites.
If needed, the surgeon performs a skin graft, usually a simple split-thickness graft, often
between 8 and 16 weeks after the bone graft.
➢ Bone segment transfers – when infected bone is extensively resected, reconstruction with
microvascular bone transfer may be useful. In general, a bone transfer is reserved for larger
skeletal defects. The most common donor sites are the client’s fibula and iliac crest. The
steps of the procedure are similar to those of cancellous grafting in that debridement
precedes bone transfer.
➢ Muscle flaps – if the bony defect is relatively small, a muscle flap may be the only surgery
required (Ignativicius and Workman, 2006).

Postoperative Care. Neurovascular assessments must be done frequently because the client
experiences swelling after surgical procedure. Elevate the affected extremity to increase venous
return, and thus control swelling. Assess the client’s neurovascular status, including the following:

➢ Pain
➢ Movement
➢ Sensation
➢ Warmth
➢ Temperature
➢ Distal pulses
➢ Capillary refill (not as reliable as the above indicators) (Ignativicius and Workman, 2006).

Complications

Osteomyelitis complications may include:

• Bone death (osteonecrosis). An infection in your bone can impede blood circulation within
the bone, leading to bone death. Areas where bone has died need to be surgically removed
for antibiotics to be effective.
• Septic arthritis. Sometimes, infection within bones can spread into a nearby joint.
• Impaired growth. Normal growth in bones or joints in children may be affected if
osteomyelitis occurs in the softer areas, called growth plates, at either end of the long bones
of the arms and legs.
• Skin cancer. If your osteomyelitis has resulted in an open sore that is draining pus, the
surrounding skin is at higher risk of developing squamous cell cancer
(https://www.mayoclinic.org/diseases-conditions/osteomyelitis/symptoms-causes/syc-
20375913).

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Prevention

If you've been told that you have an increased risk of infection, talk to your doctor about ways to
prevent infections from occurring. Reducing your risk of infection will also help your risk of
developing osteomyelitis.

In general, take precautions to avoid cuts, scrapes and animal scratches or bites, which give germs
easy access to your body. If you or your child has a minor injury, clean the area immediately and
apply a clean bandage. Check wounds frequently for signs of infection
(https://www.mayoclinic.org/diseases-conditions/osteomyelitis/symptoms-causes/syc-
20375913).

SCOLIOSIS

➢ A lateral curvature of the spine, may be found in thoracic, lumbar, or thoracolumbar spinal
segment.
➢ The curve may be convex to the right (more common in lumbar curves) or to the left (more
common in lumbar curves).
➢ Rotation of the vertebral column around its axis occurs and may cause rib cage deformity.
➢ It is often associated with kyphosis (humpback) and lordosis (swayback) (Antipuesto, 2010).

Etiology And Pathophysiology

1. Idiopathic scoliosis – exact etiology is unknown. Accounts for 65% of cases. Possible causes
include genetic factors, vertebral growth abnormality. Classified into three groups based on
age at time of diagnosis.
o Infantile – birth to age 3.
o Juvenile – presentation between age 11 and 17.
2. Congenital scoliosis – exact etiology unknown; represented as malformation of one or more
vertebral bodies that results in asymmetric growth.
o Type I – failure of vertebral body formation e.g. isolated hemivertebra, wedged
vertebra, multiple wedged vertebrae, and multiple hemivertebrae.
o Type II – failure of segmentation e.g. unilateral unsegmented bar, bilateral block
vertebra.
o Commonly associated with other congenital anomalies.
3. Paralytic or musculoskeletal scoliosis – develops several months after symmetrical paralysis
of the trunk muscles from polio, cerebral palsy, or muscular dystrophy.
4. Neuromuscular scoliosis – child has a definite neuromuscular condition that directly
contributes to the deformity.
5. Additional but less common causes of scoliosis are osteopathic conditions, such as fractures,
bone disease, arthritic conditions, and infections.
6. Miscellaneous factors that can cause scoliosis include spinal irradiation, endocrine disorders,
post thoracotomy, and nerve root irritation.
7. As the deformity progresses, changes in the thoracic cage increase. Respiratory and
cardiovascular compromise can occur in cases of severe progression (Antipuesto, 2010).

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Assessment

1. Poor posture, uneven shoulder height.


2. One hip more prominent than the other.
3. Scapular prominence.
4. Uneven waist line or hemline
5. Spinal curve observable or palpable on both upright and bent forward.
6. Back pain may be present but is not a routine finding in idiopathic scoliosis.
7. Leg length discrepancy (Antipuesto, 2010).

Nursing Diagnosis

• Disturbed body image related to negative feelings about spinal deformity and appearance in
brace.
• Risk for impaired skin integrity related to mechanical irritation to brace.
• Risk for injury related to postoperative complications (Antipuesto, 2010).

Diagnostic Evaluation

1. X-ray of the spine in the upright position, preferably on one long 36-inch cassette, show
characteristic curvature.
2. MRI, myelograms, or CT scan with three-dimensional reconstruction may be indicated for
children with severe curvatures who have a known or suspected spinal column anomaly,
before management decisions are made.
3. Pulmonary function tests for compromised respiratory status.
4. Evaluate for renal abnormalities in children with congenital scoliosis (Antipuesto, 2010).

Therapeutic Management

Usually, if the spinal curve is less than 20 degrees, no therapy is required except for close
observation and X-rays about every 6 months until the child reaches growth maturity. If the curve
is greater than 20 degrees, treatment consist of a conservative, nonsurgical approach using a body
brace; it could include surgery or a combination of both surgical and nonsurgical measures. Curves
greater than 40 degrees may require surgery with spinal fusion. The goal of both surgery and
mechanical bracing is to maintain spinal stability and to prevent further progression of the
deformity until bone growth is complete. Because of this, regardless of the type of treatment
chosen, the child and family must be prepared for long-term treatment. During prepuberty and
adolescence, children tend to be very concerned about body image and so can become very
impatient with scoliosis correction. Offering them a great deal of support at healthcare visits to help
them cope can be an important nursing contribution (Silbert-Flagg, J. & Pillitteri, A., 2018).

Bracing

For slight spinal curves in a child who is still skeletally immature, bracing – one of the oldest forms
of correction – is still a prime intervention (AAOS, 2015c). The Miwaukee brace was the first type
used for this purpose, so although today’s braces look much different, they may still be referred to
by this name. Originally, these braces extended to the neck and were almost impossible to conceal
under clothing; today, they are underarm thoracolumbar supports (e.g., a Boston brace) that fit
under clothing. Braces are now designed by computer-aided techniques to ensure that the brace

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will be both comfortable and will accomplish maximum correction (Cobetto, Aubin, Parent, et al.,
2016). The child wears the prescribed brace for 23 hours per day, removed only for showering. At
night, a child may be prescribed a Charleston bending brace that confines the spine to an
overcorrected position. Assess whether children and parents know how to safely apply these
braces. Frequent follow up healthcare visits are necessary to check that the device still fits snughly
without rubbing on bony prominences, such as the iliac crests. Caution children and parents not to
loosen straps if rubbing occurs because this causes the brace to fit loosely, thus not allowing for it to
exert adequate compression and traction on areas that are curved. Alert parents and children to
notify their healthcare provider instead (Silbert-Flagg, J. & Pillitteri, A., 2018).

Halo Traction

Halo traction is the use of opposing forces to straighten and reduce spinal curves that are severe
when first diagnosed (over 80 degrees) or that are progressing despite bracing. Halo traction is
achieved using a ring metal (a halo) held in place with about four stainless steel pins inserted into
the skull bones (Koller, Zenner, Gajic, et al., 2012). Counter-traction is applied by pins inserted into
the distal femurs or the iliac crests. A halo traction apparatus is bulky and looks frightening.
Children cannot help but worry that when the pins are inserted into their skull (under general
anesthesia), they will slip and penetrate their brain. Halo traction is typically used for those
children who have severe scoliosis that they experience respiratory involvement or cervical
instability or have a high thoracic deformity or decreased vital capacity from severe spinal
curvature and rotation. Halo traction is prescribed until optimal spinal correction is achieved. For
the first 24 hours after application, most children experience a nagging level of pain at the pin
insertion sites and perhaps in their back; offer analgesia as prescribed. After optimal spinal
correction has been achieved with the device, the equipment is easily removed. The pin sites in the
skull heal within 1 week without obvious scarring (Silbert-Flagg, J. & Pillitteri, A., 2018).

Surgical intervention: Spinal instrumentation

Surgical correction usually is necessary if the spinal curvature is greater than 40 degree.
Instruments such as rods and screws are placed next to the spinal column to provide firm reduction
of the curvature; the spine is then fused in the corrected position. A disadvantage of fusion is that it
prevents further spinal growth and limits motion of the lumbar section if involved – a concern that
can be solved by the use of fusionless interventions such a intervertebral stapling or expandable
rods (Dede, Demirkiran and Yazici, 2014). Although this is back surgey, the surgical approach may
be either from the back (posterior) of the child or from the front (anterior) through the abdomen
(Silbert-Flagg, J. & Pillitteri, A., 2018).

Nursing Interventions

1. Prepare the child for casting or immobilization procedure by showing materials to be used
and describing procedure in age-appropriate terms.
2. Promote comfort with proper fit of brace or cast.
3. Provide opportunity for the child to express fears and ask questions about deformity and
brace wear.
4. Assess skin integrity under and around the brace or cast frequently.
5. Provide good skin care to prevent breakdown around any pressure areas.
6. Instruct the patient to examine brace daily for signs of loosening or breakage.
7. Instruct patient to wear cotton shirt under brace to avoid rubbing.

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8. Instruct about which previous activities can be continued in the brace.


9. Provide a peer support person when possible so the child can associate positive outcomes
and experiences from others (Antipuesto, 2010).

JUVENILE IDIOPATHIC ARTHRITIS (JIA)

JIA, also called simply juvenile arthritis (JA) or juvenile rheumatoid arthritis (JRA), primarily
involves the joints of the body, although is also affects blood vessels and other connective tissue
(Silbert-Flagg, J. & Pillitteri, A., 2018).

• It represents a group of disorders that share the clinical manifestation of chronic joint
inflammation.
• Connective tissues are those that provide a supportive framework and protective covering
for the body, such as the musculoskeletal system and skin and mucous membranes (Belleza,
2021).

Pathophysiology

Juvenile rheumatoid arthritis is a genetically complex disorder in which multiple genes are
important for disease onset and manifestations.

• Humoral and cell-mediated immunity are involved in the pathogenesis of JRA.


• T lymphocytes have a central role, releasing proinflammatory cytokines (eg, tumor necrosis
factor–alpha [TNF-α], interleukin [IL]-6, IL-1) and favoring a type-1 helper T-lymphocyte
response.
• Studies of T-cell receptor expression confirm recruitment of T-lymphocytes specific for
synovial noncell antigens.
• Evidence for abnormalities in the humoral immune system include the increased presence of
autoantibodies (especially antinuclear antibodies), increased serum immunoglobulins, the
presence of circulating immune complexes, and complement activation.
• Chronic inflammation of synovium is characterized by B-lymphocyte infiltration and
expansion.
• Macrophages and T-cell invasion are associated with the release of cytokines, which evoke
synoviocyte proliferation.
• Some pediatric rheumatologists view systemic-onset JRA as an autoinflammatory disorder,
such as familial Mediterranean fever (FMF) or cryopyrin-associated periodic fever
syndromes, rather than a subtype of JRA.
• This theory is supported by work demonstrating similar expression patterns of a phagocytic
protein (S100A12) in systemic-onset JRA and FMF, as well as the same marked
responsiveness to IL-1 receptor antagonists (Belleza, 2021).

Statistics and Incidences

The occurrence of Juvenile rheumatoid arthritis appears to peak at two age levels: 1 to 3 years and 8
to 12 years.

• Approximately 300,000 children in the United States are estimated to have some type of
arthritis.

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• For JRA, the prevalence has ranged from 1.6 to 86.1 cases per 100,000.
• Worldwide, JRA appears to occur more frequently in certain populations (eg, indigenous
peoples) from such disparate areas as British Columbia and Norway.
• Disease-associated mortality for JRA is difficult to quantify, but it is estimated to be less than
1% in Europe and less than 0.5% in North America.
• Most deaths associated with JRA in Europe are related to amyloidosis, and most in the
United States are related to infections.
• Girls with an oligoarticular onset outnumber boys by a ratio of 3:1. In children with uveitis,
the ratio of girls to boys is 5-6.6:1, and in children with polyarticular onset, girls outnumber
boys by 2.8:1 (Belleza, 2021).

Causes

The etiology of Juvenile rheumatoid arthritis is unknown, and the genetic component is complex,
making clear distinctions between the various subtypes difficult (Belleza, 2021).

Clinical Manifestations

Physical findings are important to provide criteria for diagnosis and to detect abnormalities
suggestive of alternative etiologies.

• Arthritis. Arthritis is defined as either intra-articular swelling on examination or as


limitation of joint motion in association with pain, warmth, or erythema of the joint.
• Loss of motion. The hips, temporomandibular joint, and small joints in the spine do not
demonstrate swelling when affected by synovitis but demonstrate the combination of loss of
motion and pain.
• Synovitis. In synovitis, the fingers may appear swollen, and the range of motion becomes
painful.
• Swelling. A soft, boggy swelling is appreciated in the popliteal fossa.
• Joint inflammation. Joint inflammation occurs first; if untreated, inflammation leads to
irreversible changes in joint cartilage, ligaments, and menisci (Belleza, 2021).

Assessment and Diagnostic Findings

The diagnosis of juvenile rheumatoid arthritis (JRA) is based on the history and physical
examination findings.

• Inflammatory markers. The erythrocyte sedimentation rate (ESR) or C-reactive protein


(CRP) level is usually elevated in children with systemic-onset JRA (with a disproportionate
increase in the CRP) and may be elevated in those with polyarticular disease; however, it is
often within the reference range in those with oligoarticular disease; other markers of
inflammation include thrombocytosis, leukocytosis, complement, and, in a reverse fashion,
albumin and hemoglobin.
• Complete blood count and metabolic panel. A complete blood count, liver function tests (to
exclude the possibility of viral or autoimmune hepatitis), and assessment of renal function
with serum creatinine levels should be done before starting treatment with nonsteroidal
anti-inflammatory drugs (NSAIDs), methotrexate (MTX), or tumor necrosis factor–alpha
inhibitors.

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• Antinuclear antibody test. A positive ANA is a marker for increased risk of anterior uveitis;
children younger than 6 years at arthritis onset with a positive ANA finding are in the
highest risk category for development of uveitis and need slit lamp screening every 3-4
months.
• Radiography. When only a single joint is affected, radiography is important to exclude other
diseases, such as osteomyelitis.
• Computed tomography and magnetic resonance imaging. CT scanning is the best method
for analyzing bony abnormalities, but it has been largely superseded by MRI in the overall
assessment of JRA; and MRI provides the most sensitive radiologic indicator of disease
activity.
• Ultrasonography. On ultrasonograms, inflamed synovium can appear as an area of mixed
echogenicity lining the articular cartilage; the vascularity of the synovium can be assessed
with Doppler flow studies (Belleza, 2021).

Medical Management

The ultimate goals in managing rheumatoid arthritis are to prevent or control joint damage, to
prevent loss of function, and to decrease pain.

• Exercise. Exercise preserves joint range of motion and muscular strength, and it protects
joint integrity by providing better shock absorption; types of exercises that may be advised
include a muscle-strengthening program, range-of-motion activity, stretching of deformities,
and endurance and recreational exercises.
• Synovectomy. Synovectomy is rarely needed, and long-term outcome is poor; however, it
may be used in children in whom a single joint or just a few joints are involved and who
have very active, proliferative synovitis.
• Osteotomy and arthrodesis. Osteotomy and arthrodesis are salvage procedures for patients
whose JIA is associated with severe joint destruction or deformity.
• Total hip and knee replacements. Total hip and knee replacements provide excellent relief
of pain and restore function in a functionally disabled child with debilitating disease
(Belleza, 2021).

Pharmacologic Management

Treatment is aimed at inducing remission with the least toxicity from medications with hopes of
inducing a permanent remission.

• Nonsteroidal anti-inflammatory drugs (NSAIDs). Nonsteroidal anti-inflammatory drugs


(NSAIDs) interfere with prostaglandin synthesis through inhibition of the enzyme
cyclooxygenase (COX), thus reducing swelling and pain; NSAIDs are used to treat all
subtypes of JIA; they may help with pain and decrease swelling.
• Disease-modifying antirheumatic drugs (DMARDs). Disease-modifying antirheumatic
drugs (DMARDs) can retard or prevent disease progression and, thus, joint destruction and
subsequent loss of function.
• Corticosteroids. Corticosteroids are potent anti-inflammatory drugs used in patients with
JIA to bridge the time until DMARDs are effective.
• Immunomodulators. The recognition of tumor necrosis factor-alpha (TNF-alpha) and
interleukin (IL)–1 as central proinflammatory cytokines has led to the development of agents
that block these cytokines or their effects (Belleza, 2021).

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Nursing Management

The treatment and nursing management goal for juvenile rheumatoid arthritis is to maintain
mobility and preserve joint function (Belleza, 2021).

Nursing Assessment

The most important steps in diagnosing JRA are the medical history and physical exam.

• Medical history. Assess for the duration of symptoms, onset, affected joints, pain
description, changes in physical activity, general health, history of arthritis, previous illness,
and other symptoms associated with JRA.
• Physical exam. Assess the vital signs, auscultate the heart and lungs, palpate the abdomen,
and examine the skin (Belleza, 2021).

Nursing Diagnosis

Based on the assessment data, the major nursing diagnoses are:

• Acute pain related to tissue distension by fluid accumulation / inflammation, joint


destruction.
• Impaired physical mobility related to skeletal deformities, pain, discomfort, activity
intolerance, decreased muscle strength.
• Disturbed body image related to changes in ability to perform usual tasks.
• Self-care deficit related to musculoskeletal impairment.
• Deficient knowledge related to lack of exposure/recall (Belleza, 2021).

Nursing Care Planning and Goals

The nurse should focus on the following nursing care planning goals for the child with juvenile
rheumatoid arthritis:

• Report pain is relieved/controlled.


• Appear relaxed, able to sleep/rest and participate in activities appropriately.
• Follow prescribed pharmacological regimen.
• Incorporate relaxation skills and diversional activities into pain control program.
• Maintain position of function with absence/limitation of contractures.
• Maintain or increase strength and function of affected and/or compensatory body part.
• Demonstrate techniques/behaviors that enable resumption/continuation of activities.
• Verbalize increased confidence in ability to deal with illness, changes in lifestyle, and
possible limitations.
• Verbalize understanding of condition/prognosis, and potential complications. Formulate
realistic goals/plans for future (Belleza, 2021).

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2 | Nursing Care Management 109 15

Nursing Interventions

The nursing intervention appropriate for a child with JRA are:

• Physical therapy. Physical therapy includes exercise, application of splints, and heat.
• Activity. Stress to the caregivers the importance of encouraging the child to perform
activities of daily living to maintain function and independence.
• Pain relief. Recommend or provide firm mattress or bed board, small pillow; elevate linens
with bed cradle as needed; and suggest patient assume position of comfort while in bed or
sitting in chair.
• ROM exercises. Assist with active and passive ROM and resistive exercises and isometrics
when able.
• Emotional support. Encourage verbalization about concerns of disease process, future
expectations; give positive reinforcement for accomplishments; and acknowledge and accept
feelings of grief, hostility, dependency.
• Health education. Review disease process, prognosis, and future expectations; discuss
patient’s role in management of disease process through nutrition, medication, and balanced
program of exercise and rest; and assist in planning a realistic and integrated schedule of
activity, rest, personal care, drug administration, physical therapy, and stress management
(Belleza, 2021).

Evaluation

Goals are met as evidenced by:

• Reported pain is relieved/controlled.


• Appeared relaxed, able to sleep/rest and participate in activities appropriately.
• Followed prescribed pharmacological regimen.
• Incorporated relaxation skills and diversional activities into pain control program.
• Maintained position of function with absence/limitation of contractures.
• Maintained strength and function of affected and/or compensatory body part.
• Demonstrated techniques/behaviors that enable resumption/continuation of activities.
• Verbalized increased confidence in ability to deal with illness, changes in lifestyle, and
possible limitations.
• Verbalized understanding of condition/prognosis, and potential complications. Formulate
realistic goals/plans for future (Belleza, 2021).

MYASTHENIS GRAVIS

For nerve conduction to cause muscles to contract effectively, a neurotransmitter, acetylcholine


(ACh), must be released at synaptic junctions. The release of ACh is governed by the enzyme
cholinesterase. With myasthenia gravis, there is interference in ACh processing, which leads to
symptoms of progressive muscle weakness or inability to contract. The fault may be impaired
synthesis or storage of ACh, insufficient ACh release, inadequate ACh receptors present at motor
end plates, opposition of ACh by an anti-ACh factor, or excessive cholinesterase. In adults, the
defect is probably most often a motor end plate insufficiency (a decreased number of ACh receptors
are present). In children, myasthenia gravis probably occurs most often from an autoimmune
process (autoantibodies may block receptor sites for ACh or other receptors such as muscle-specific

C. M. D. Hamo-ay
2 | Nursing Care Management 109 16

kinase (MuSK) receptors (VanderPluym, Vasjar, Jacob, et al., 2013). The disease occurs in three
forms in childhood: neonatal transient myasthenia, congenital myasthenia and juvenile myasthenia
(Silbert-Flagg, J. & Pillitteri, A., 2018).

Description

1. Myasthenia Gravis is a neuromuscular disease characterized by considerable weakness and


abnormal fatigue of the voluntary muscles.
2. A defect in the transmission of nerve impulses at the myoneural junction occurs.
3. Causes include insufficient secretion of acetylcholine, excessive secretion of cholinesterase,
and unresponsiveness of the muscle fibers to acetylcholine
(https://www.rnpedia.com/nursing-notes/medical-surgical-nursing-notes/myasthenia-
gravis/).

Causes

MG, thought to be an autoimmune disorder, is caused by a loss of acetylcholine (ACh) receptors in


the postsynaptic neurons at the neuromuscular junction. About 80% of all MG patients have
elevated titers for ACh receptor antibodies, which can prevent the ACh molecule from binding to
these receptor sites or can cause damage to them. MG is often associated with thymic tumors
(https://www.rnpedia.com/nursing-notes/medical-surgical-nursing-notes/myasthenia-gravis/).

Assessment
➢ Weakness and fatigue
➢ Difficulty chewing
➢ Dysphagia
➢ Ptosis
➢ Diplopia
➢ Weak, hoarse voice
➢ Difficulty breathing
➢ Diminished breath sounds
➢ Respiratory paralysis and failure (https://www.rnpedia.com/nursing-notes/medical-
surgical-nursing-notes/myasthenia-gravis/).

Source: https://www.rnpedia.com/nursing-notes/medical-surgical-nursing-notes/myasthenia-gravis/

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2 | Nursing Care Management 109 17

Complications

Myasthenic Crisis

• This sudden onset of muscle weakness is usually the result of undermedication or no


cholinergic medication at all. Myasthenic crisis may result from progression of the disease,
emotional upset, systemic infections, medications, surgery, or trauma. The crisis is
manifested by sudden onset of acute respiratory distress and inability to swallow or speak
(https://www.rnpedia.com/nursing-notes/medical-surgical-nursing-notes/myasthenia-
gravis/).

Cholinergic Crisis

• Caused by overmedication with cholinergic or anticholinesterase drugs, cholinergic crisis


produces muscle weakness and the respiratory depression of myasthenic crisis as well as
gastrointestinal symptoms (nausea, vomiting, diarrhea), sweating, increased salivation, and
bradycardia (https://www.rnpedia.com/nursing-notes/medical-surgical-nursing-
notes/myasthenia-gravis/).

Primary Nursing Diagnosis

• Ineffective airway clearance related to difficulty in swallowing and aspiration


(https://www.rnpedia.com/nursing-notes/medical-surgical-nursing-notes/myasthenia-
gravis/).

Diagnostic Evaluation

• Injection of edrophonium (Tensilon) is used to confirm the diagnosis (have atropine


available for side effects). Improvement in muscle strength represents a positive test and
usually confirms the diagnosis.
• MRI may demonstrate an enlarged thymus gland.
• Test includes serum analysis for acetylcholine receptor and electromyography (EMG) to
measure electrical potential of muscle cells (https://www.rnpedia.com/nursing-
notes/medical-surgical-nursing-notes/myasthenia-gravis/).

C. M. D. Hamo-ay
2 | Nursing Care Management 109 18

Source: https://www.rnpedia.com/nursing-notes/medical-surgical-nursing-notes/myasthenia-gravis/

Holding your arms above your shoulders until they drop is one exercise that may be performed
during the Tensilon test. In this test, the drug Tensilon is administered, and the response in the
muscles are evaluated to help diagnose myasthenia gravis or to help differentiate between
myasthenia gravis and other conditions (https://www.rnpedia.com/nursing-notes/medical-
surgical-nursing-notes/myasthenia-gravis/).

Medical Management

Management is directed at improving function through the administration of anticholinesterase


medications and by reducing and removing circulating antibodies. Patients with MG are usually
managed on an outpatient basis unless hospitalization is required for managing symptoms or
complications (https://www.rnpedia.com/nursing-notes/medical-surgical-nursing-
notes/myasthenia-gravis/).

Pharmacologic Highlights

• Anticholinesterase drugs such as Pyridostigmine bromide (Mestinon), neostigmine bromide


(Prostigmine); neostigmine methylsulfate can be given as a continuous infusion if the patient
cannot take oral medication. Anticholinesterase drugs blocks the action of the enzyme
anticholinesterase, thereby producing symptomatic improvement; atropine must be readily
available to treat cholinergic side effects and medications must be administered on time, or
the patient may be too weak or unable to swallow the drug.
• Prednisone is used to suppresses the autoimmune activity of MG
• Nonsteroidal immunosuppressants such as Azathioprine (Imuran), cyclophosphamide
(Cytoxin) is used to suppress autoimmune activity when patients do not respond to
prednisone; can produce extreme immunosuppression and toxic side effects
(https://www.rnpedia.com/nursing-notes/medical-surgical-nursing-notes/myasthenia-
gravis/).

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2 | Nursing Care Management 109 19

Nursing Interventions

1. Monitor respiratory status and ability to cough and deep breathe adequately.
2. Monitor for respiratory failure.
3. Maintain suctioning and emergency equipment at the bedside.
4. Monitor vital signs.
5. Monitor speech and swallowing abilities to prevent aspiration.
6. Encourage the client to sit up when eating.
7. Assess muscle status.
8. Instruct the client to conserve strength.
9. Plan short activities that coincide with times of maximal muscle strength.
10. Monitor for myasthenic and cholinergic crises.
11. Administer anticholinesterase medications as prescribed.
12. Instruct the client to avoid stress, infection, fatigue, and over-the counter medications.
13. Instruct the client to wear a Medic-Alert bracelet.
14. Inform the client about services from the Myasthenia Gravis Foundation
(https://www.rnpedia.com/nursing-notes/medical-surgical-nursing-notes/myasthenia-
gravis/).

1. What is the most common cause of osteomyelitis?


2. What are the 3 types of scoliosis?
3. What is the best treatment for myasthenia gravis?

1.3 References

Silbert-Flagg, J. & Pillitteri, A. (2018). Maternal and Child Health Nursing (8th Edition, Volume 2, pp.
1199-1201).Wolters Kluwer.

Ignatavicius, D. and Workman, M. L. (2006). Medical Surgical Nursing Critical Thinking


for Collaborative Care (5th Edition, Volume 1 and Volume 2). Elsevier (Saunders).

https://www.mayoclinic.org/diseases-conditions/osteomyelitis/symptoms-causes/syc-20375913.

https://www.rnpedia.com/nursing-notes/medical-surgical-nursing-notes/osteomyelitis-nursing-
management/.

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2 | Nursing Care Management 109 20

Antipuesto, D. J. (2010, October 5). Scoliosis. https://nursingcrib.com/nursing-notes-


reviewer/medical-surgical-nursing/scoliosis/.

Belleza, M. (2021, February 11). Juvenile Rheumatoid Athritis. https://nurseslabs.com/juvenile-


rheumatoid-arthritis/.

https://www.rnpedia.com/nursing-notes/medical-surgical-nursing-notes/myasthenia-gravis/.

1.4 Acknowledgment

The images, tables, figures and information contained in this module were
taken from the references cited above.

C. M. D. Hamo-ay

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