Pediatric Immunologic Disorders

IMMUNOLOGIC DISORDERS

Pediatric Human Immunodeficiency Virus/Acquired Immunodeficiency Syndrome

Human immunodeficiency virus (HIV) infection for infants, children, and adolescents is represented by a continuum of immunologic and clinical classifications ranging from no to severe immunologic suppression and asymptomatic to severely symptomatic. Acquired immunodeficiency syndrome (AIDS) is the end-stage of the continuum. Also see Chapter 29, HIV disease and AIDS, page 1043.

Epidemiology

HIV is a pandemic, affecting people all over the world; the World Health Organization estimated 34 million people living with HIV in 2011.
Children under age 15 account for 3.3 million infected with HIV worldwide; in North America, there are 4,500 cases.
In North America, UNAIDS reported there were more than 1.4 million people living with the virus in 2011.
In the United States it is estimated that youth aged 13-24 represent the second highest rate of newly acquired HIV.
HIV is transmitted through sexual contact with an infected person; through exposure to infected blood and blood components (sharing needles via IV drug use, tattooing, piercing, and unsterilized medical equipment); or from an HIVinfected mother to her baby before or during birth or through breastfeeding.
Numerous studies have indicated that a majority of HIVinfected males and females continue to be sexually active and the incidence of sexually acquired HIV infection among adolescents, in particular, has increased.
The availability of highly active antiretroviral therapy (HAART) and effective perinatal transmission prevention approaches has resulted in an increased number of HIVinfected females who choose to become pregnant.
In the United States, perinatally acquired HIV infection has dramatically decreased from about 25% to less than 2% (although the number of new cases worldwide continues to rise).
- This decrease reflects concerted efforts to provide voluntary HIV testing for pregnant women, along with the use of combination antiretroviral regimens during pregnancy, additional IV zidovudine during labor (ideally more than 4 hours before delivery), and zidovudine to the HIV-exposed
  infant for the first 6 weeks of life, along with the avoidance of breastfeeding.
- The use of cesarean birth in the mother whose viral load is greater than 1,000 copies/mL may also be indicated to reduce vertical transmission.
An HIV-infected mother can have a child who is infected and subsequent children who are not infected. Likewise, one infant in a multiple birth can be infected, whereas the other infants are not.
Children born to mothers with high HIV RNA viral load, low CD4+ lymphocyte counts, and severely symptomatic HIV disease are at greater risk for infection.
- Infants born to such mothers and who become infected appear to be at increased risk for more rapid disease progression.
- Infants born to mothers who seroconvert during pregnancy are at an increased risk for becoming infected.
With the advent of HAART, the morbidity and mortality in HIV-infected children has decreased significantly. Thus, in those who have access to medication and who are adherent to therapy, HIV has become a chronic, manageable illness.

Pathophysiology and Etiology

Table 53-1 Pediatric HIV Classification^*

IMMUNOLOGIC CATEGORIES	CLINICAL CATEGORIES
	N: No Signs/Symptoms	A: Mild Signs/Symptoms	B: + Moderate Signs/Symptoms	C: + Severe Signs/Symptoms
1: No evidence of suppression	N1	A1	B1	C1
2: Evidence of moderate suppression	N2	A2	B2	C2
3: Severe suppression	N3	A3	B3	C3
^*Children whose HIV infection status is not confirmed are classified by using the above grid with a letter E (for perinatally exposed) placed before the appropriate classification code (eg, EN2)
+Category C and lymphoid interstitial pneumonitis in Category B are reportable to state and local health departments as acquired immunodeficiency syndrome.
Centers for Disease Control and Prevention. (1994). 1994 revised classification system for human immunodeficiency virus infection in children less than 13 years of age. Morbidity and Mortality Weekly Report, 43(RR-12), 1-10. Available: www.cdc.gov/mmwr/preview/mmwrhtml/00032890.htm.

The causative agent is a retrovirus that damages the immune system by infecting and depleting the CD4+ lymphocytes (T4-helper cells). These CD4+ lymphocytes play a central role in the regulation of the immune system.
There are also abnormalities in the function of cellular and humoral immunity (B cells, CD8+ cells, natural killer cells, monocytes, macrophages, and specific antibodies).
Progressive destruction of the immune system leads to:
- Increased incidence of serious bacterial infections, such as bacteremias, bacterial pneumonias, and osteomyelitis.
- Opportunistic infections, which commonly include Pneumocystis jirovecii pneumonia (PCP), esophageal candidiasis, and Mycobacterium avium infection.
- More aggressive forms of viral infections, such as severe varicella, disseminated zoster, and cytomegalovirus pneumonitis.
- Cancers, especially lymphomas.
- Wasting syndromes and encephalopathy.
Multisystem organ involvement results in cardiomyopathies, nephropathies, neurologic impairment, GI dysfunction, endocrine disorders, dermatologic manifestations, musculoskeletal abnormalities, ocular impairments, ear, nose, and throat problems, and hematologic disorders.
An AIDS diagnosis occurs when a child presents as severely symptomatic (Category C) or with lymphoid interstitial pneumonitis (LIP from Category B) along with severe immune suppression (see Tables 53-1 and 53-2).
Once an AIDS diagnosis is made, it is not reversed even with reconstitution of the immune system and recovery from the opportunistic infection.

Clinical Manifestations

Generalized lymphadenopathy, especially in less common sites, such as epitrochlear and axillary areas.
Persistent, recurrent oral candidiasis.
Failure to thrive.
Developmental delays or loss of previously acquired milestones.
Hepatomegaly.
Splenomegaly.
Persistent diarrhea.
Hypergammaglobulinemia or hypogammaglobulinemia (elevated or diminished levels of immunoglobulin [Ig] G, IgM, IgA).
Parotitis.
Unexplained anemias, thrombocytopenia.
Unexplained cardiac or kidney disease.
Recurrent mild or serious bacterial infections.

Table 53-2 Immunologic Categories Based on Age-specific CD4+ T-lymphocyte Counts and Percentage of Total Lymphocytes

IMMUNOLOGIC CATEGORY	AGE OF CHILD
	Younger than Age 12 Months		Ages 1-5		Ages 6-12
	mm³	(%)	mm³	(%)	mm³	(%)
1: No evidence of suppression	≥1,500	(≥25)	≥1,000	(≥25)	≥500	(≥25)
2: Evidence of moderate suppression	750-1,499	(15-24)	500-999	(15-24)	200-499	(15-24)
3: Severe suppression	<750	(<15)	<500	(<15)	<200	(<15)
Centers for Disease Control and Prevention. 1994 revised classification system for human immunodeficiency virus infection in children less than 13 years of age. Morbidity and Mortality Weekly Report, 43(RR-12), 1-10. Available: www.cdc.gov/mmwr/preview/mmwrhtml/00032890.htm.

Diagnostic Evaluation

All pregnant women in the United States should be tested for HIV before delivery. Neonatal testing with enzyme-linked immunoassay (ELISA) is an indication of the presence of circulating maternal antibodies to HIV and is not an indication of the child’s infection status.
Polymerase chain reaction (PCR) assay is the most sensitive, specific, and preferred method for detecting HIV infection in a child younger than 18 months. The 2011 Centers for Disease Prevention and Control (CDC) guidelines recommend testing for infants using PCR at ages 14 to 21 days, 1 to 2 months, and 4 to 6 months.
After age 18 months, HIV antibody assays can be used for testing because maternal antibodies should not be present by this age. As with adults, a reactive ELISA must be followed by a confirmatory Western blot test.
Additional laboratory studies important to follow on the HIV-exposed infant include complete blood count (CBC) with differential to monitor for anemia and neutropenia and lymphocyte subsets (CD4+ counts).
For a known infected child, CBC with differential, platelet counts, serum chemistries, serial CD4+ counts, and HIV PCR (viral load) should be done every 3 to 6 months (depending on previous results and the stage of disease). In addition, a baseline echocardiogram and chest x-ray should be done and repeated yearly.
In known infected children or at-risk infants (those born to an HIV-infected mother but whose own status is still indeterminate), computed tomography scanning or magnetic resonance imaging of the head may be indicated if there is evidence of neurologic impairment, falling rate of head growth, or developmental delays or regression.

Table 53-3 Recommendations for PCP Prophylaxis and CD4+ Monitoring for HIV-Exposed Infants and HIV-Infected Children, by Age and HIV-Infection Status

AGE/HIV-INFECTION STATUS	PCP PROPHYLAXIS	CD4+ MONITORING
Birth to ages 4-6 weeks, HIV exposed	No prophylaxis	1 month
Ages 4-6 weeks to 4 months, HIV exposed	Prophylaxis	3 months
Ages 4-12 months, HIV infected or indeterminate	Prophylaxis	6, 9, and 12 months
Under ages 4-12 months, HIV infection reasonably excluded	No prophylaxis	None
Ages 1-5, HIV infected	Prophylaxis if: CD4+ count is <500 cells/L or CD4+ percentage is <15%	Every 3-4 months
Ages 6-12, HIV infected	Prophylaxis if: CD4+ count is <200 cells/L or CD4+ percentage is <15%	Every 3-4 months
Adapted from Centers for Disease Control and Prevention. (1995). 1995 revised guidelines for prophylaxis against Pneumocystis carinii pneumonia for children infected with or perinatally exposed to human immunodeficiency virus. Morbidity and Mortality Weekly Report, 44(RR-4), 1-11. Available: www.cdc.gov/mmwr/preview/mmwrhtml/00037275.htm.

Management

Initiation of PCP prophylaxis is indicated when low CD4+ counts occur, with the exception that all infants born to HIVinfected women should be started on PCP prophylaxis at age 4 to 6 weeks, regardless of their CD4+ count (see Table 53-3).
- The first drug of choice is cotrimoxazole.
- Pentamidine, atovaquone, and dapsone are alternative choices.
- PCP prophylaxis should be discontinued in infants whose infection has been reasonably excluded on the basis of two or more negative viral diagnostic tests performed at >4 weeks and >8 weeks of age. Some experts may choose not to prophylaxis infants in whom antenatal and peripartum management has been appropriate and in whom the risk of infection is very low.
The choice to start therapy early while an individual is still asymptomatic versus delaying therapy until clinical or immunologic symptoms appear continues to generate considerable controversy among pediatric and adult HIV experts. Antiretroviral therapy is recommended for all HIV-infected children <1 year of age and in older children who have clinical symptoms of HIV or evidence of immune suppression regardless of the age of the child or HIV PCR level (viral load).
- Clinical trial data from both adults and children have demonstrated that antiretroviral therapy in symptomatic patients slows clinical and immunologic disease progression and reduces mortality.
- Seven classes of antiretrovirals (ARVs) have been approved for use in children: nucleoside reverse transcriptase inhibitors (NRTIs), non-nucleoside reverse transcriptase inhibitors (NNRTIs), protease inhibitors (PIs), integrase inhibitor, CCR5 antagonist, and fusion inhibitor.
- Combination therapy is now standard care; using at least three ARVs from at least two drug classes. Special emphasis on adherence is essential to prevent viral resistance; particularly, NNRTI resistance develops rapidly with even slight fluctuations in drug levels.
Adherence is the basis for successful viral suppression; strategies should be developed to encourage adherence.
Historically, use of IV immune globulin has been and continues to be common. It is still recommended for HIV-infected children who have had two or more serious bacterial infections within 1 year and for the treatment of HIV-related thrombocytopenia or immunoglobulin deficiency.
Use of antifungal drugs, such as nystatin, ketoconazole, fluconazole, itraconazole, and clotrimazole, for persistent or recurrent oral candidiasis.
Use of antivirals, such as acyclovir, valacyclovir, ganciclovir, and cidofovir, for suppression or treatment of recurrent viral infections.
Aggressive, prompt assessment and treatment of febrile illnesses.
Nutritional support.
Evaluation and treatment of developmental delays and regression.
Adequate pain management in advanced or end-stage disease.
Support and interventions for the child and family (including all caregivers) with issues of bereavement, disclosure, parental guilt, and long-term care.

Complications

Untreated or inadequately treated HIV:
- Repeated, overwhelming infections and certain cancers, particularly lymphomas.
- Hearing loss, tooth and gum disease, acute and chronic ear, nose, and throat infections.
- Opportunistic infections.
- Reactive airway disease.
- Cardiomyopathy.
- Failure to thrive, malabsorption, wasting.
- Chronic atopic dermatitis and other skin reactions.
- Nephropathy.
- Neuropathy, myopathy.
- Anemia, thrombocytopenia, neutropenia.
- Loss of treatment options related to the development of drug resistance.
- Developmental delay.
- Learning disabilities.
- Psychological challenges for children and caregivers, including multiple losses, issues of disclosure, fear, discrimination, stigmatization, and isolation.
- Death.
HIV treatment:
- NRTIs—anemia, neutropenia, lactic acidosis, pancreatitis, abacavir hypersensitivity reaction, peripheral neuropathy, lipodystrophy/lipoatrophy, renal dysfunction, osteopenia, headache, nausea, potential for vascular dysfunction.
- NNRTIs—psychological changes, sleep disturbances/nightmares, neural tube defects (fetal exposure), hepatitis, rash.
- PIs—dyslipidemia, GI upset, diarrhea.
- Fusion inhibitors—injection site reactions.

Nursing Assessment

Review maternal records to identify infants who may be at risk for HIV disease. Infected infants are not easily identifiable by outward appearance.
Review records of at-risk or known infected children to determine nutritional status, growth and development, frequency of serious bacterial infections, presence or risk of opportunistic infections, laboratory values, and immunization status.
Assess growth, development, lymph nodes, hepatomegaly, splenomegaly, and oropharynx for presence of oral candidiasis and dental caries.
Assess the family’s understanding of the child’s condition, care needs, prognosis, and medical care plan.
Assess the family’s coping mechanisms, comfort with disclosure issues, and long-term plans for care including transition plans for the child to an adult care program.
Assess the health of primary caregiver and discuss long-term care plans for respite and permanent alternative caregivers (including guardianship issues), as appropriate.
Assess the child’s understanding of health condition and medications.
In children with advanced or end-stage disease, assess the level of pain and discomfort.
Assess the child’s coping and response to the frequent painful and invasive procedures experienced as part of the ongoing diagnosis and management of the disease.
Assess for animal contact.
Take a thorough travel history to evaluate risk of coinfections, such as tuberculosis (TB), malaria, or other parasitic infections. Also evaluate potential travel plans, particularly to developing countries.
In the adolescent, assess increased at-risk behaviors, such as substance use, piercing, or sexual activity. Also determine methods of birth control, as appropriate.

Nursing Diagnoses

Risk for Infection related to immunodeficiency, neutropenia.
Ineffective Therapeutic Regimen Management related to insufficient knowledge of HIV, noncompliance, and limited resources.
Imbalanced Nutrition: Less than Body Requirements related to malabsorption, anorexia, or pain.
Impaired Oral Mucous Membranes related to candidiasis and herpes stomatitis.
Diarrhea related to enteric pathogens, disease process, and medications.
Hyperthermia related to HIV infection and secondary infection.
Delayed Growth and Development related to HIV Central Nervous System (CNS) infection.
Ineffective Family Coping related to parental guilt and nature of disease.
Ineffective Coping by child related to unresolved issues around disclosure, hospitalization, and losses.
Pain related to advanced or end-stage HIV disease.
Fear related to frequent invasive procedures, diagnosis, stagmatization.

Nursing Interventions

Preventing Infection

Have a high index of suspicion for secondary infection even when clinical manifestations are subtle or absent.
Administer or teach caregiver to administer prescribed pharmacologic agents that may help prevent serious bacterial infections, prevent opportunistic infections, and treat infections that occur.
- Educate caregiver or patient about the importance of more than 95% compliance with prescribed HAART regimen to maximize viral reduction, which, in turn, will maximize immune reconstitution.
Monitor viral load and total white blood cell (WBC) count. The absolute neutrophil counts may drop significantly as an adverse effect of antiretroviral drugs.
Maintain cleanliness of the environment and teach family members the essentials of environmental sanitation.
Use aseptic techniques when performing invasive procedures.
Administer and teach the family the importance of proper oral hygiene to prevent dental caries, which is a source of secondary infection.
Administer and teach the family good skin care—a break in the skin is a source of secondary infection.
Teach the family the importance of safe food preparation, including use of safe water, to avoid introducing pathogens through contaminated or undercooked food.
Monitor immunization status and advise families of the need to complete all recommended childhood immunizations (see Table 53-4, page 1720). In general, live virus vaccines should be used with caution. Also see page 1411 for routine pediatric immunization information based on the CDC. In general, live vaccines should be used with caution in children with HIV infections.
- The measles, mumps, rubella (MMR) and varicella vaccines are recommended for HIV-infected children who are not severely immunocompromised.
- Oral polio vaccine should be avoided because there is an alternative injectable inactivated polio vaccine available.
- Yellow fever vaccine should be avoided unless the risk of exposure is extremely high; consultation with an expert is recommended.
- In the United States, the bacille Calmette-Guérin vaccine is also not recommended.
- Yearly influenza vaccine and a 3- to 5-year pneumococcal vaccine are also recommended in addition to the standard childhood immunization schedule.
- An annual TB skin test should also be administered.
Provide appropriate chemoprophylaxis following exposure to communicable diseases.
Educate families on the potential risks associated with pet ownership. In particular, cats may transmit Toxoplasmosis or Bartonella infections; birds may transmit Cryptococcus neoformans or M. avium; reptiles may cause salmonellosis. Good handwashing with soap and water is recommended after handling animals; contact with animal feces should be avoided.
Encourage families to meet with an expert in travel medicine before traveling—particularly to developing countries—to minimize the risk of acquiring opportunistic pathogens.
Educate teens about the use and importance of using condoms to prevent spread of HIV and to protect them from getting secondary sexually transmitted diseases such as syphilis or chlamydia.

Table 53-4 Vaccination of Individuals with HIV Based on the Canadian Immunization Guide

INACTIVATED/COMPONENT VACCINES
VACCINE	HIV/AIDS
Dtap, Tdap, Td	Routine use^*
IPV	Routine use
Hib	Routine use
Influenza	Recommended
Pneumococcal	Recommended
Meningococcal	Routine use
Hep A	Recommended (MSM, IDU)
Hep B	Recommended/Routine use^†
Human papilloma virus	Recommended
^*Routine vaccination schedules should be followed with age-appropriate booster doses. ^†Hep B vaccine (HB) is now recommended for all infants.

LIVE VACCINES
VACCINE	HIV/AIDS
MMR	Routine use^‡ (if no significant compromise)
Varicella	Consider in asymptomatic and mild symptomatic disease
OPV	Contraindicated (alternate available)
Oral typhoid	Contraindicated (alternate available)
BCG	Contraindicated
Yellow fever	Contraindicated^**
Oral cholera	Contraindicated
^‡Most HIV-positive children can receive the first MMR vaccine without significant risk. Administration of the second MMR dose (particularly in adults) must be evaluated on a case-by-case basis. ^**Unless the risk of exposure is extremely high, consultation with an expert is recommended.
Modified from Public Health Agency of Canada and National Advisory Committee on Immunization. (2006). Canadian immunization guide. Immunization of immunocompromised persons. Available: www.phacaspc.gc.ca/publicat/cig-gci/p03-07-eng.php.

Maximizing Adherence to HAART

Develop a trusting relationship with patient by maintaining a nonjudgemental attitude to maintain open communication and assist in assessing barriers to adherence.
Assess drug readiness and strategies to maximize adherence before initiating therapy.
Provide the simplest regimen possible; for example, least number of pills (combination therapies), least often (once daily), based on viral resistance.
Stress the need for adherence at every visit.
Use several strategies for evaluating adherence (eg, viral load, pill count, self reporting, pharmacy refill checks).
Collaborate with patient to determine what reminder tools may work the best (pill box, calendar, stickers, alarm, associating pill-taking with daily routine).

Maintaining Adequate Nutrition

Carefully monitor growth parameters (height, weight, head circumference).
Consult with a dietitian to develop strategies for nutritional care, including additional calories, nutritional supplements while reducing cholesterol.
Teach the family to prepare high-calorie, nutritious meals that are pleasing and acceptable to children.
As age-appropriate, involve the child in meal planning.
Encourage small, frequent meals if the child is experiencing absorption problems.

Maintaining Oral Mucosa and Dental Integrity

Include regular examinations of the oral mucosa and teeth in the physical assessment of the child.
Administer prescribed antifungal and antiviral therapy.
Offer fluids and pureed foods to minimize chewing and facilitate swallowing; avoid highly seasoned or acidic foods.
Encourage regular dental hygiene and dental visits.

Minimizing Effects of Diarrhea

Monitor for the presence or development of diarrhea.
Monitor daily weight when diarrhea is present.
Monitor intake and output and assess skin and mucous membranes for turgor and dryness.
Use enteric precautions.
Avoid foods that increase intestinal motility.
Administer IV hydration, as indicated.
Plan a regimen of skin care, including cleansing/blot drying of the anal area and application of ointment or skin barrier cream.
Teach the family safe food preparation techniques to minimize contamination of food.
Administer medications, as directed, for relief of severe diarrhea and enteric infections.

BOX 53-1 Transition to Adult Care

Transition is the purposeful, planned movement of adolescents and young adults with chronic illness/disabilities from childcentered to adult-oriented systems. With the advent of highly active antiretroviral therapy, children with human immunodeficiency virus (HIV) are surviving and living well into adulthood. As they become adults, they are graduating from pediatric health care programs—with parents the child’s advocate—to adult programs where individual care becomes the focus, with the young adult becoming his or her own advocate.

It is imperative for the pediatric health care team to prepare children and families for the changes and challenges of adolescence, with a focus on increased autonomy and self-reliance and independence.

The transition into adolescence is a normal part of growth and development; however, special considerations need to be addressed when caring for a teenager with a chronic illness.

KEY ELEMENTS OF TRANSITION:

Focus the orientation on the future and keep it proactive and flexible.
Start as early as possible.
Build on knowledge about HIV and issues of confidentiality and disclosure.
Make sure your approach fosters personal and medical interdependence and creative problem solving.
Provide opportunities for own consultations during clinic visit.
Have open, honest, age-appropriate sexual health discussions.
Develop a written transition policy—agreed upon by all members of the multidisciplinary team and target adult services— posted for families and young adults to see.
Discuss different care models and possible settings in which the young adult can access care as an adult.
Provide liaison personnel in both pediatric and adult health care centers.
Coordinate an initial meeting for teen to meet adult care provider and staff.
Ensure a flexible policy on the timing of events, with anticipation of change.
Provide an education program for young people and their families that addresses medical, psychosocial, and educational/vocational aspects of care.
Develop an individualized health-care transition plan by age 14, in collaboration with the young adult and his or her family, and continuously update, as needed.
Identify a network of relevant community agencies and adult primary and subspecialty care providers.
Implement a training program for pediatric and adult providers on transition and adolescent issues.
Provide for appropriate primary preventive care.
Ensure affordable continuous health and medication insurance coverage.

Detecting and Controlling Fever

Monitor for fever and report any core temperature over 101° F (38.3° C).
Institute comfort measures, such as sponge baths, dry clothing and linens, and antipyretics, as ordered.
Teach the family how to accurately monitor the child’s rectal temperature.
Teach the family to promptly report any febrile episode over 101° F rectally.
Administer antipyretics.

Promoting Developmental Goals

Assess the child’s developmental status on a regular basis.
Report regression or delay in achieving developmental milestones.
Make appropriate referrals for more in-depth developmental evaluation when delays and regression are noted.
Teach the family appropriate developmental stimulation activities for their child.
Implement recommendations for developmental stimulation (including school-based programs) and assist the family in doing likewise.
Facilitate successful transition to adult care (see Box 53-1).

Promoting Effective Family Coping

Practice within a framework of family-centered care.
Assess the family’s coping mechanisms, strengths, and weaknesses.
Provide emotional support to the family.
Refer the family to appropriate community resources for grief counseling and legal assistance (eg, wills, custody issues).
Help the family set realistic goals and expectations for the child.
Maintain a nonjudgmental attitude and nonprejudicial approach.
Allow the family to use denial as a protective mechanism because this gives them some sense of control.
Explain all care and treatment plans to the family.
Involve the family in planning for the care and management of their child.
Give strategies and guidance to families regarding partial truth telling so they can answer child’s questions as honestly as possible.
Provide support to new mothers who grieve the loss of not being able to breastfeed. Provide strategies on how to deflect questions about the choice not to breastfeed.
Encourage parent to allow adolescent to start taking increased responsibility for own health care, including individual time alone with health care provider and self-advocacy.
Provide family and disclosed patient with hopeful information about the future, such as continuing education or, possibly, getting married and having a family.
Refer the family to community resources for home care, daycare of other siblings, transportation services, school-based services, respite care, and hospice.

Strengthening Coping Skills of the Child

Introduce the subject of disclosure of diagnosis to the child with the family. Offer the family guidelines as to age-appropriate approaches.
Accept that some families may not be able to disclose the nature of the disease to their child, even after much support and guidance.
- Explore with families how they want you to address questions the child may present to you.
- The illness and its impact can be effectively handled without actually telling the child about the HIV disease or AIDS status, if the family refuses to disclose it.
Answer the child’s questions as honestly as possible within parental constraints that may be present.
Actively involve the child in as much of the care as possible, such as by having the child decide where to place the IV line, cleaning off skin for needle insertion, and so forth.
Use therapeutic play techniques (age-appropriate), such as drawing, playing with dolls, playing with medical equipment, and storytelling, to allow the child to express him- or herself in less verbal ways.
Refer the child and family for counseling if the child’s coping skills do not progress or there appears to be regression in handling the issue of illness and chronic health care needs. Signs indicating possible need for additional in-depth interventions include more acting out by the child, withdrawn behavior, and difficulty in school (either behaviorally or academically).
Recommend and facilitate peer support groups.

Minimizing Discomfort

Assess for signs of pain in the child. In the infant or nonverbal child, such signs include restlessness, crying, withdrawn behavior, and changes in vital signs.
Discuss concerns over pain issues with the primary health care provider and team providing care for the child. Initiate or request referral to a pain management team if the child does not experience relief.
Stress to the parent or guardian the need for adequate pain management in children; many people do not acknowledge that infants or children suffer from pain and that this needs to be alleviated.
Use appropriate techniques to assess the level of pain or discomfort a child is experiencing.

Minimizing Fear

Make sure that all painful and invasive procedures are done in a location other than the child’s bed in the hospital.
Monitor central line if used for venous access. Central line access may be less traumatic over time than repeated peripheral access attempts. Some children with HIV will need longterm, probably lifelong, IV therapies and blood monitoring, and a central line greatly eliminates the pain and anxiety associated with these procedures.
Make sure that the parent/caregiver realizes that most clinic visits will involve blood tests so that they do not falsely reassure the child that “no needlesticks” will occur.
Use diversionary techniques, such as bubble-blowing, for distraction during painful procedures. Practice first with the child and tell him or her this is an activity that you will help with during the procedure. Children can also be taught other relaxation techniques.
Offer small rewards (such as stickers, small toy items) after painful procedures to reduce anxiety. Such rewards should be given regardless of how the child reacted. The reward is not for “not crying” but acknowledges difficulty in going through painful and invasive procedures on a regular basis.
Involve the child in the procedure as much as possible (ageappropriate). Such involvement can include selecting a possible site for accessing or receiving IV medications, cleaning
the site, selecting a special bandage for after the procedure. Use topical anesthetic.

Community and Home Care Considerations

Assess home environment for resources, such as nutritious foods and safe food preparation; adequate water, heat, electricity, and space; developmentally appropriate toys; and supplies needed for care and hygiene.
Assess caregiver’s correct administration of medications.
Draw blood samples using universal precautions and careful transport of blood and used equipment.
Perform developmental assessment periodically.
Act as liaison among family, specialists, school officials, and other team members.

Family Education and Health Maintenance

Teach families and other caregivers of the child universal precautions. In caring for HIV-infected infants and children, gloves are recommended when handling potentially contaminated body fluids (such as blood) but are not considered necessary for routine diaper-changing unless bloody diarrhea or hematuria exists.
Facilitate supply of adequate numbers of latex (or nonlatex, if necessary) gloves to families to use, as indicated.
Facilitate education as well as a supply of latex condoms and other forms of birth control for teens who are sexually active or about to become sexually active.
Offer guidance as to how to initiate discussion of their child’s HIV status with schools and daycare settings. The “need to know” principle serves as a guideline when deciding to whom disclosure should be made (generally the principal, school nurse, and classroom teacher). It is not currently required that schools and daycare centers be advised, but is generally encouraged because this alerts the school to notify the parent promptly in the event of an outbreak of infectious disease that could pose a threat to the HIV-infected child (such as varicella).
Offer guidance to teens about how to initiate disclosure of HIV status to sexual partners or peers. Provide support during the process and offer to help facilitate the disclosure in the clinical setting.
Give families concrete guidelines as to when to notify health care provider about their child’s condition. Signs and symptoms of illness require prompt notification, all fevers over 101° F (38.3° C) should be reported, and any adverse experiences or suspected adverse experiences with medications need prompt reporting.
Assess whether the parents are receiving care for themselves. Typically, parents will neglect their own care to provide for their child. As a result, the parents’ immune status may become more quickly compromised.
Refer families to a social worker or social services agency. Children with HIV may qualify for certain entitlement programs that help with their health care and the family’s financial situation. Many states have initiated case management programs for people with HIV.
Provide guidance using a childhood chronic illness model that addresses such psychosocial issues as disclosure and parental guilt as well as such medical issues as multiorgan involvement and likelihood of exacerbations of a variety of related conditions (eg, infections and nutritional deficiencies).
Assist parents in obtaining the latest information regarding treatment protocols of pediatric HIV. Such information can be obtained through (800) TRIALS-A or www.aidsinfor.nih.gov.

Evaluation: Expected Outcomes

Absolute neutrophil count within normal limits; aseptic technique maintained.
Maintains medication regimen, keeps follow-up appointments; achieves an undetectable viral load (less than 40 copies/mL).
Nutritional intake adequate to meet body requirements; growth curve maintained.
Oral mucous membranes without ulceration, good dentition.
Two to three loose stools per day; perianal skin without irritation.
Afebrile; parent or caregiver demonstrates accurate monitoring of temperature.
Achieves normal growth patterns as evidenced by height, weight, and developmental tasks.
Parents discuss their feelings about the diagnosis of HIV; family seeks help through community resources.
Child participates in care, asks questions; adolescent successfully transitions to an adult care program without disruption in care.
Reports increase in comfort level.
Practices distraction techniques during procedures; reports reduced fear.

Primary Immunodeficiencies

Primary immunodeficiencies (PID) encompass more than 150 types of disorders that affect distinct components of the innate and adaptive immune systems. More than 120 genes have been identified to account for the known forms of PID. The overall incidence of PID is estimated to be 1:10,000. However, the exact incidence and prevalence of PID is unknown. The most common presentation of an immunodeficiency is increased frequency of infections or infections that are unusual or difficult to treat. PID diseases are often undiagnosed or have a significant delay in diagnosis, which can lead to significant end-organ damage from recurrent infections.

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Tags: Lippincott Manual of Nursing Practice

Jul 20, 2016 | Posted by drzezo in NURSING | Comments Off

Nurse Key

Fastest Nurse Insight Engine

Pediatric Immunologic Disorders

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Nurse Key

Fastest Nurse Insight Engine

Pediatric Immunologic Disorders

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