Immune Disorders

Immune Disorders

Immune System

The main function of the immune system is to protect the body by recognizing “self” from “nonself.” The system launches a protective process to eliminate any foreign antigens through the function of specific cells. The process is complex and uses overlapping defense reactions. A child’s immune system won’t reach maturity until about 6 years of age, which makes infants and children susceptible to infectious organisms. Lymphoid tissue reaches adult size by 6 weeks of age, enlarges during the prepubertal age, and returns to normal size by puberty. The thymus reaches full size before puberty. The spleen reaches full size by adulthood. When the immune system is fully functioning, the risk of being infected by invading organisms is lower.

Antibodies are needed to fight infections. IgG is the only antibody that crosses the placenta. Full-term infants receive adult levels of IgG, which provides protection from bacterial infections. There is a physiologic drop in IgG at 6 to 8 months, and it does not return to adult levels until 7 to 8 years of age. IgM, IgE, IgA, and IgD do not cross the placenta and do not reach adult levels until later. IgM reaches adult level by 1 year of age; IgA and IgE reach adult level by 6 to 7 years of age.

The body’s protective responses are either nonspecific or specific. The nonspecific response is a generalized reaction not specific to a particular antigen. The injury site produces inflammation to which phagocytes respond and ingest the antigens. If the response is effective, the inflammation clears. Should the generalized response not be successful, a more specific response is initiated. Two additional immune functions respond, the humoral and cell-mediated functions. The B lymphocytes that promote humoral response produce antibodies (IgG, IgM, IgA, IgD, and IgE) and memory cells. Each antibody has specific antigen targets; for example, IgM activates with bacterial and viral infections, and IgE is activated with allergic response. The antibodies bind with the antigen and tag it so that other components of the immune system can destroy the antigen. Phagocytes are the immune cells that destroy antigens by engulfing and digesting. The function of memory cells is to detect previously identified antigens and facilitate a quicker response to the specific antigen.

The cell-mediated response involves T lymphocyte cells. T cells are produced in the thymus. There are 3 specialized types of T cells: helper cell, killer (cytotoxic) cell, and suppressor cell. The function of T cells is to attack and destroy bacteria, viruses, and other pathogens. The killer T cells directly bind to the foreign antigen and disrupt the cell membrane. This results in the antigen’s destruction. The helper T cell has several functions. It stimulates the B cell to mature and aid in the destruction of the antigen. The helper T cell retains information about specific antigens and is also able to provide that information, resulting in a quicker response. In addition, the helper T cell assists the killer cell in recognizing antigen. The suppressor T cells assist in slowing the immune response. Both the humoral and cell-mediated system work together to provide protection from infections.

Disorders of the immune system are a result of several different causes. Deficiencies of the immune cells resulting in the inability of the body to resist an infection are considered immunodeficiency. This group of disorders includes HIV/AIDS. They are discussed in Chapter 15. Other disorders are caused by an abnormal and excessive immune response to a foreign antigen. These disorders are hypersensitivity disorders such as allergies and atopic dermatitis (see Chapter 16 for further discussion on these disorders).

Disorders resulting from an abnormal and excessive response to self and these disorders are referred to as autoimmune disorders.

Kawasaki Disease

Kawasaki disease (KD) is an acute severe vasculitis of all blood vessels, especially medium-sized vessels such as the coronary artery. The cause of the illness is unknown but is thought to be infectious. It is seen in children younger than 5 years of age, in boys more often than in girls, and has a higher incidence in those of Asian background. It is the leading cause of acquired heart disease in children (AAP, 2009). Coronary involvement can lead to aneurysms, ischemia, and infarcts.

Signs and Symptoms

KD progresses through three stages: acute, subacute, and convalescent. The acute stage presents with a prolonged high fever that is unresponsive to antimicrobials or acetaminophen or ibuprofen. Additional classic symptoms are conjunctival redness without drainage, strawberry tongue with oral and pharyngeal redness, red swollen hands and feet, rash on the trunk, enlarged cervical lymph nodes, and extreme irritability. The subacute stage begins after 1 to 2 weeks when the fever and acute signs resolve. Irritability, conjunctival redness, and anorexia continue with the appearance of desquamation of hands and feet, arthritis, thrombocytosis, and coronary aneurysms (Figure 19-1). This stage can last up to 4 weeks. The last stage of convalescence begins when all signs have disappeared and ends when erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) have returned to a normal value. This stage may last from 6 to 8 weeks.

There is no single diagnostic test for KD. Diagnosis is made based on the clinical signs and laboratory tests (ESR, CRP, WBC, RBC, and platelet counts). A two-dimensional echocardiogram is done for cardiac evaluation.

Treatment and Nursing Care

Treatment involves reducing inflammation with intravenous immunoglobulin (IVIG) and high-dose aspirin. It has been shown that IVIG and aspirin given within the first 10 days of onset has reduced the incidence of coronary disease (AAP, 2009). The aspirin dose is reduced after fever has been controlled for 48 hours, which usually occurs at about 14 days. Aspirin administration will continue for 6 to 8 weeks and is discontinued if there are no coronary abnormalities. Most children recover without long-term complications. Low-dose aspirin is continued indefinitely for children with coronary abnormalities, and they are followed for several months or years depending on severity.

Nursing care for the acute stage focuses on relief of symptoms. Irritation of the skin may be helped with loose-fitting clothes and cool cloths. Mouth care is important. Children may not want to eat but should be encouraged to take cool liquids and soft foods. Intake and output is monitored closely. Aspirin should be in liquid form. IVIG is administered per hospital protocol.

The most challenging nursing issue is the extreme irritability. Providing a quiet environment may be beneficial. Parents will need a lot of emotional support as they try to find ways to comfort their child. Parents need to understand that irritability is a classic symptom for KD and they should not feel embarrassed or guilty with their child’s behavior. Discharge planning should include instructing parents that they will have to administer aspirin at home for a length of time. Parents should be instructed to watch for side effects. While on antiplatelet therapy, contact activities should be avoided to prevent potential bleeding. Fevers should be reported to the physician.

Stevens-Johnson Syndrome

Stevens-Johnson syndrome is a severe form of erythema multiforme that involves lesions of the skin and mucous membranes. It is thought to be associated with a hypersensitivity reaction to certain drugs or to follow a respiratory infection. Drugs including sulfonamides, nonsteroidal antiinflammatory drugs (NSAIDs), antimicrobials, and anticonvulsants are usually the cause of the reactions.

Signs and Symptoms

This disorder presents with flulike symptoms of fever, malaise, fatigue, and sore throat. Mucosal lesions erupt in the eyes, mouth, and entire gastrointestinal tract (Figure 19-2). Pain can be severe. Lesions may appear in crops and may take weeks to heal. There is no definitive test for diagnosis. Laboratory testing may be done to assist with diagnosis.

Treatment and Nursing Care

Treatment is supportive. An ophthalmologist will monitor for any corneal scarring. Oral lesions benefit from mouthwashes and glycerin swabs. Topical anesthetics such as viscous lidocaine can provide pain relief. Lesions can be washed with saline or Burow’s solution. Hydrogel dressings can also be soothing. If the child is not able to ingest oral intake, IV fluids may be necessary. Nutritional support may require soft foods and liquids, and severe oral lesions may require parenteral feedings. Skin should be kept clean and dry and inspected for any secondary infections. Specialty mattresses may be necessary to help with skin integrity. Corticosteroids may be used to help with inflammation. Antimicrobials are not necessary unless there is a documented infection. The disease is self-limiting and will gradually disappear without scarring. Parents should be aware that their child is at risk for recurrence, and the medical record should reflect that the child is allergic to the specific medication.

Juvenile Idiopathic Arthritis

Juvenile idiopathic arthritis (JIA) is the most common arthritic condition of childhood. Formerly considered to be one disease with several subtypes, it is now classified as several different syndromes. These inflammatory diseases involve the joints, connective tissues, and viscera. JIA is seen frequently in children. The exact cause is unknown, but infections and an autoimmune response have been implicated. The symptoms mimic many of those of nonrheumatic conditions, such as Lyme disease, septic arthritis, and osteomyelitis; these conditions need to be ruled out before a diagnosis of JIA is made.

Signs and Symptoms

The most common types of JIA are listed in Table 19-1. Symptoms vary from one child to the next, and each type has a distinct method of onset: systemic (acute febrile), oligoarticular (involving five joints or fewer), and polyarticular (involving more than five joints). The course is chronic, with remissions and exacerbations. Some children who present with oligoarticular progress to polyarticular. Children rarely have permanent joint deformity, although many have some functional limitations. In systemic JIA, joint symptoms may be absent at onset but do develop in most cases.

Table 19-1

Major Types of Juvenile Idiopathic Arthritis

Systemic 10%-20% Both equally Any Few to multiple High fever (especially in the evening), chills, rash on trunk and extremities, enlarged liver and lymph nodes, pericarditis/pleuritis, leukocytosis, abdominal pain, anemia, arthralgias before arthritis begins Approximately 50% develop chronic joint disease; prognosis depends on number of joints involved
Polyarticular, RF-positive 5%; may be familial 90% girls >8 yr Any or multiple large and small joints, upper and lower extremities, symmetrical pattern Rapid, severe course; rheumatoid nodules (palpable near elbows); low fever; slight anemia Early joint erosion, with many having permanent disability
Polyarticular, RF-negative 20%-30% 70%-75% girls Early childhood, school age Multiple large and small joints Arthritis persists, loss of bone mass, small percentage with iridocyclitis, growth disturbances, low fever, malaise, anorexia, anemia Small percentage have joint damage
Oligoarticular 40%-55%; may be familial Girls: 20%-35% develop a polyarticular course after approximately 3 yr Early childhood Knees, ankles, elbows (<4 joints); asymmetric pattern Chronic iridocyclitis with occasional loss of vision, malaise, low fever, slight anemia; slightly enlarged liver, spleen, and lymph nodes during active disease More favorable prognosis regarding long-term joint function
Boys: Many develop spondyloarthropathies Late childhood Large joints of lower extremities, hips, spine Small percentage have acute iridocyclitis  


Affected joints are swollen, warm, and stiff (Figure 19-3). Joint stiffness occurs mainly in the morning or after a period of inactivity (the “gel” phenomenon). Joint effusion and thickening synovial membrane eventually erode cartilage and can cause joint destruction.

Children with oligoarticular disease are at risk for iridocyclitis, an inflammation of the iris and ciliary body of the eye. Symptoms include redness, pain, photophobia, decreased visual acuity, and nonreactive pupils. This condition occurs most frequently in young girls. Its course is unpredictable. All children with oligoarticular arthritis need slit-lamp eye examinations several times a year for at least the first 5 years after the diagnosis of JIA. Distortion of the pupil and cataracts may occur. The long-term visual prognosis is uncertain.

Bone mass increases most during adolescence, and the process is related to weight, amount of exercise, and diet. Because children with JIA are less mobile than other children their age, they are likely to experience anorexia and reduced nutritional intake. Medications they are taking for their disease makes them more susceptible to osteopenia (low bone mass). If adequate bone mass is not attained during the teen years, osteoporosis in adulthood is more likely. The skeletal bone mass should be monitored closely.

There are no specific tests for JIA. The diagnosis is determined from clinical manifestations, radiographs, laboratory test results (CBC, ESR, rheumatoid factor [RF] assay, antinuclear antibody [ANA] assay), and the exclusion of other disorders. Aspirated joint fluid is yellow to green and cloudy and has a low viscosity. The goals of therapy are to reduce pain and swelling, to promote mobility, to preserve joint function, to educate the child and family, and to help the child and family adjust to living with a chronic disease.

Treatment and Nursing Care

Treatment is supportive. Drug therapy and exercise are the mainstays of therapy. First-line medications used to treat JIA are the NSAIDs, which include ibuprofen (Advil, Motrin), naproxen (Naprosyn), and tolmetin. Ibuprofen now comes in liquid form, making it easier to give to small children. Aspirin is given less frequently than previously because of its association with Reye’s syndrome. These agents do not change the course of the disease but reduce pain and stiffness. Along with the NSAIDs, intraarticular long-acting corticosteroid injections may be administered to preserve joint function.

If initial treatment with NSAIDs fails after adequate trial (at least 2 to 3 months of each medication chosen), other approaches are used. Systemic corticosteroids are usually avoided unless extreme disease presents. Disease-modifying antirheumatic drugs (DMARDs) have demonstrated effectiveness; these include hydroxychloroquin (Plaquenil) and sulfasalazine (Azulfidine). Biologics (anti-TNF agents) can be effective and include etanercept (Enbrel), infliximab (Remicade), and adalimumab (Humira) (Abramson, 2008).

Regular monitoring of all medications is imperative. NSAIDs are given with meals to avoid gastric irritation. Parents should be informed of the side effects, which include tinnitus, lethargy, hyperventilation, dizziness, headaches, nausea, and vomiting.

The nurse functions as a member of a team that includes the pediatrician, rheumatologist, social worker, physical therapist, occupational therapist, psychologist, ophthalmologist, and school and community nurses. The child may be hospitalized during an acute episode or for an unrelated illness. Treatment consists of medications, warm tub baths, joint exercises, and rest. Physical and occupational therapy are included in the treatment. Their purpose is to maintain function and reduce pain. The physical therapist oversees the type and amount of exercise performed. Daily range-of-motion exercises and play activities that incorporate specific routines help preserve function, maintain muscle strength, and prevent deformities. Morning tub baths and the application of moist hot packs help lessen stiffness. Resting splints may be ordered to prevent flexion contractures and preserve functional alignment. Proper body alignment with a regular change to the prone position (unless contraindicated) facilitates comfort. Either no pillow or a small flat pillow is advocated. Measures to alleviate boredom should be undertaken.

Facilitating School Attendance. 

Encourage school attendance. Excess absence from school, particularly for nonspecific symptoms, might suggest that the child is depressed or excessively preoccupied with the illness. In such cases, the meaning of the illness to the child and family and its effect on daily life need to be explored. Careful communication with the school nurse is essential for the child to have a successful school experience without interruptions in learning. The child should allow adequate preparation time in the morning to work out stiffness before arriving at school. There should be planned rest periods during the day, especially during disease flare-ups. Unobtrusive access to the school health office is important for these children so that they do not feel different from their peers.

Meeting Emotional Needs. 

Parents need assistance with establishing limits. Consistent negative behavior in social situations can present more problems than the actual disability. Overindulgence and preferential treatment often compromise the child’s potential for happiness and independence. Siblings of chronically ill children may resent the special attention received by the child. They may also be torn between loyalty to the brother or sister and their own need to be with others. Parents need ongoing counseling and the services of various community resources. One resource is the Arthritis Foundation. The child may benefit from association with other children who have arthritis.

This disease is characterized by periods of remission and exacerbations. There is no known cure for JIA. Parents need assistance in understanding the chronic nature of the disease and the potential for recurrence of signs and symptoms. Nurses can serve as advocates for the child by recognizing the impact of the disease and by openly communicating with the child, the family, and other members of the health care team.

Infectious Mononucleosis

Infectious mononucleosis is a global disease caused by a herpes-type Epstein-Barr virus (EBV). It occurs chiefly in older children and adults; its peak incidence is in persons between 17 and 25 years of age or earlier in low socioeconomic groups. Studies suggest that the organism is transmitted by contact with saliva, either directly or on contaminated eating utensils; however, its communicability is considered low. The incubation period is from 1 to 2 months.

Signs and Symptoms

Symptoms vary from mild to moderately severe and may last for several weeks. They include low-grade fever, sore throat, headache, fatigue, skin rash, and general malaise. The lymph glands enlarge. Splenomegaly develops in approximately half the children. Liver involvement with mild jaundice occurs in a small number of persons and requires bed rest until liver function returns to normal.

The diagnosis is confirmed with the examination of peripheral blood. Lymphocytosis and the presence of atypical lymphocytes are seen. A rising titer of antibody to EBV is also indicative; the MonoSpot test is rapid, can detect the infection earlier than the heterophile antibody test, and is now widely used. Complications, although uncommon, include rupture of the spleen, secondary pneumonia, neurologic manifestations, and heart involvement.

Treatment and Nursing Care

Treatment is supportive because the disease is self-limiting. Acetaminophen, aspirin, or an NSAID such as ibuprofen is given as needed. An antipyretic is given to reduce fever and discomfort. An initial period of rest or restricted activities is usually needed, and returning to usual activities is based on the child’s energy level. Gargling with warm saline solution and sucking on throat lozenges can be helpful for pharyngitis. Adequate fluid intake is necessary, in particular, bland, cool liquids that are not irritating to the throat. Smoking should be discouraged. There is no special diet. Isolation is not necessary. The child is alerted to signs of secondary infection. Activities are increased as the fever and fatigue diminish. The child with an enlarged spleen is cautioned to avoid heavy lifting, trauma to the abdomen, and vigorous athletics until the splenomegaly subsides. For the athlete with an enlarged spleen, contact or collision activities should be restricted for at least 3 to 4 weeks. Severe abdominal pain is unusual, except in the presence of splenic rupture, which requires immediate attention.

The teenager with mononucleosis may be discouraged and depressed. The teenager worries about job, schoolwork, and the ability to continue extracurricular activities of importance. Open communication with school officials and classmates helps alleviate some of the anxieties. The prognosis in mononucleosis is good. It is no longer considered a prolonged, debilitating disease. Many cases go unrecognized.

Diabetes Mellitus

Diabetes mellitus (DM) is a chronic metabolic condition in which the body is unable to metabolize and use carbohydrates, fats, and proteins properly because of a deficiency of insulin, an internal secretion of the pancreas. Insulin deficiency leads to impaired glucose transport (sugar cannot pass into the cells) and accelerated metabolism of stored fats for energy.

Diabetes is not a single entity but several different disorders. These disorders differ in cause, pathophysiology, and genetic predisposition. All result in disturbed glucose metabolism. Previously, DM was classified by treatment. The old classification was insulin-dependent DM (IDDM, or type 1) and non–insulin-dependent DM (NIDDM, or type 2). The American Diabetes Association now uses a classification system that includes type 1 and type 2 (Table 19-2).

Table 19-2

Classification of Types of Diabetes Mellitus

Type 1 DM Primarily in childhood but can occur at any age Polydipsia, polyuria, polyphagia, weight loss occurring for usually less than 1 mo Child requires insulin to maintain life
Type 1 can be diagnosed in children before symptoms appear and in family members at risk
Type 1 appears to have both genetic and environmental components
Hyperglycemia: random plasma glucose above 200 mg/dL with symptoms or fasting blood glucose above 126 mg/dL and 2-hr postprandial blood glucose above 200 mg/dL with no symptoms
Glycosuria, ketonuria
Vaginal monilial infections in adolescent girls
Elevation of antiislet or antiinsulin antibodies and presence of other indicators of immune connection
Type 2 DM Usually after age 40 yr but can occur at any age Hyperglycemia: fasting blood glucose above 126 mg/dL and 2-hr glucose tolerance test result above 200 mg/dL on more than one occasion without precipitating factors Condition has some genetic basis
Some affected children need insulin on occasion (e.g., during severe stress or illness)
Child is usually obese
Serum insulin level can be normal or less than normal

Dec 22, 2016 | Posted by in NURSING | Comments Off on Immune Disorders
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