Alterations in Immune Function

1. Alterations in Immune Function Ball and Bindler Donna Hills APN EdD ( c)

2. Signs and symptoms • In children, immune disorders are often non-specific. • Why? • How do children fight off infection? • How are their immune systems anatomically different from the adult?

3. Pediatric Differences • Natural Immunity • Intact skin, body pH, natural Ab from mother, inflammatory and phagocytic properties of WBC’s • T cells active early; Natural killer cells; complement.. • Acquired Immunity • Humoral Ab and cell mediated immunity • Not fully developed until about 6 yrs.

4. Immunodeficiency Disorders • Primary immunodeficiency: congenital. • Can be a failure in B cell, T cell or combination. • Secondary immunodeficiency: acquired. • Human immunodeficiency virus (HIV)

5. Acquired Immunodeficiency Syndrome • Caused by HIV-1 • Destroys body’s ability to fight infection • In advanced stages, cannot fight off even opportunistic organisms • AIDS is a major cause of death in children; most new cases in young children is perinatal. • HIV found in blood, semen, vaginal fluids, breast milk, saliva and tears. • Not known to transmit via saliva, tears or sweat.

6. AIDS Statistics in Children • In 2001, half of all new HIV cases occurred in young people 13-24yr. • Most cases acquired through sexual transmission • In 2003, 59 cases in children under 13yrs, 59 additional cases in children 13-14yrs, and 1991 cases in persons 15-24yrs. • Worldwide: 2.5 million children under 15yrs are living with HIV or AIDS. • 500,000 deaths in children less than 15 yrs.

7. Pathophysiology of HIV • HIV destroys CD4 T cells, crucial to the normal function of cellular immunity • Also effects humoral immunity • Normal values: T4 (CD4)=500-1500 cells/mm3 • Moderate suppression=200-499 • Severe suppression=<200

8. Perinatal Transmission Significantly Reduced with use of AZT • AZT (zidovudine) • Given during pregnancy and delivery to mother • Given to the neonate post delivery

9. Presentation in the Child • Neonate is asymptomatic • Child presents with non-specific findings including lymphadenopathy, failure to thrive/weight loss, delayed development, chronic diarrhea, chronic eczema, dermatitis and fever. • May also have frequent infections, and/or severe forms of bacterial infections. • As disease progresses my present with opportunistic infections (Pneumocystis carinii pneumonia) and/or malignancies such as lymphoma. • Encephalopathy may develop and result in developmental delays, deterioration of motor skills and intellectual functioning. • Adolescents with HIV are often infected with Hep B as well.

10. Diagnostic Tests • In infants, test can be performed within 48 hrs. of birth (40% of infected infants can be identified this way). • If initially negative, need to retest at 1-2 mos. • If negative again, retest at 3-6mo. • If negative again, retest between 15-18mo. • Need 2 positive tests with two separate specimens

11. Types of Tests for HIV • PCR (polymerase chain reaction) • Preferred test:$ 175.00 • Some false positives • HIV culture • Not universally available • OraQuick Advance HIV1/2 Antibody test • Tests oral fluids (not just saliva), and/or plasma • Both forms correctly identifies HIV positive individuals 99%, as well as HIV negative individuals 99%. • However, if positive, still need a second test. • If negative and have a known exposure, retest in 3 mo.

12. Treatment of HIV • Identify prenatally • Treatment of mother with AZT prenatally, child delivered by C-section, reduces chance of transmission down to 1%. • Perinatal transmission treated with AZT as well. • 12 antiretroviral agents approved in children >3mo. • HAART: highly active antiretroviral therapy. • Drug regime aimed at maximizing the effect of viral load suppression. • Dramatic impact on children with HIV • Combination therapy recommended for all children and adolescents except infant in first 6 wks who is prophylactically treated with AZT for perinatal transmission.

13. Treatment of HIV (cont) • All infants born to mothers with HIV are prophylactically treated for PCP • Drugs used: Bactrim (Septra) or aerosolized pentamidine • IVIG • The earlier the child converts to AIDS, the poorer the prognosis. • Children living longer with improved protocols. • Average age of survival after diagnosis of HIV is 8yrs. • Younger children more likely to die of pulmonary infections. • Those who survive past 10yrs more likely to die of cardiac disease, malnutrition, encephalopathy and infection with Mycobacterium avium complex.

14. Nursing Management • Prevent transmission initially • Once a child has HIV, nursing care focused on: Management of symptoms Promoting growth and development Promoting good nutrition followed by frequent measurement of height and weight. Screening for infection, opportunistic infections, anemia. Reducing child’s exposure to infectious organisms Preventing further transmission of HIV Provide psychological support for the child and family Provide modified immunization schedule: no Varicella vaccine but provide MMR unless severely affected with AIDS. Annual PPD should be given.

15. Case Study (A) Amanda is a 2 ½ yr old admitted with HIV admitted for pneumonia. When you bring in her antiretroviral medications, Amanda is quite difficult and refuses to take any from you. Her mother asks why you don’t just give it all together in one drink? You are responsible for developing her plan of care. What information would you need to know about Amanda and her mother? (History) What would you need to assess? (Physical) What is your plan of care? (Interventions)

16. Case Study (B) Upon discharge, Amanda’s mother is considering enrolling Amanda in a nursery school situation 3 mornings per week. Discuss some of the issues involved in sending a child with HIV to a preschool or day care. Consider the following: Disclosure Risk of Infection Development and Socialization Medication Regiment

17. Autoimmune Disorders • Group of diseases with primary feature of tissue injury caused by a probable immunologic reaction of the host with its own tissues. • Systemic Lupus Erythematosus (SLE) • (Covered elsewhere) • Juvenile Rheumatoid Arthritis (JRA)

18. JRA • Chronic, autoimmune inflammatory disease with remissions/exacerbations • Characterized by joint swelling, pain, limitation of motion. • Some forms affect organs: liver, heart, lungs and eyes. • Can interfere with normal growth and development. • 70% experience remission by adulthood.

19. Three types of JRA • Pauciarticular Arthritis • 50% of children with JRA have this type • <4 joints • Early onset <5yrs. • Systemic Arthritis • Fever, rash, organ involvement • 15% of those with JRA • Polyarticular Arthritis • Many joints: >5 joints including hips, knees, neck as well as smaller joints.

20. JRA: Goal of care • Multidisciplinary with drug therapy, OT/PT, and when necessary, surgery. • To relieve pain, suppress inflammation, prevent contractures, preserve joint function, promote normal growth and development. • Promote self care at the child’s highest level of functioning without producing harm.

21. Medication for JRA • NSAIDS; Ibuprofen, ASA, Naproxen • Cox-2 inhibitors (Celebrex ?off market?) • Disease Modifying Antirheumatic Drugs (DMAD) • Methotrexate • Sulfasalazine • Plaquenil • Corticosteroids: Prednisone • Biologic Response Modifiers: Remicade (TNF inhibitor)

22. Nutrition in JRA • Well balanced diet with adequate calories • But may have decr metabolic demands if less mobile • Increased weight can increase joint strain and increase pain. • Pain may decrease appetite • Decreased intake can increase constipation, a risk of decreased mobility. • Balance according to the child’s needs.

23. Allergic Reactions • Increased incidence of dx of children with allergies • Allergy is an abnormal or altered reaction to an allergen. • Antigen-antibody reaction manifested as anaphylaxis, atopic disease, serum sickness, contact dermatitis.

24. Types of Allergic Reactions • No reaction first time exposed but develop IgE Ab against Ag. • Type I: immediate histamine response: skin Rx or systemic (asthma, hay fever) • Type II: 15-30 min later. Sx vary but may include fever and dyspnea. (ABO, Tx reac) • Type III: peaks within 6 hrs. Sx similar to Type II but rx later. (serum sickness) • Type IV: 24-72hr. Fever, erythema, pruritis. (PPD)

25. Management • Diagnosis by complete history of meds, foods, contacts, behaviors. • Elimination of potential allergens • Benedryl to control immediate response and then Q 6 hrs. • Allergy testing: serum and skin, xray, PFT, RAST. • Desensitization therapy • Epipen for home, travel and school/camp.

26. Anaphylaxis • Potentially life threatening systemic allergic response • Minutes to 2 hrs • Those with asthma, eczema, and hay fever at greater risk • Peanut allergy leading cause of anaphyllaxis in children in US (50-100/yr.) • Other causes: latex, medications, insect stings, foods: milk, eggs, shellfish.

27. Clinical Manifestations of Anaphyllaxis • Mucous membrane and/or hand/foot swelling • Cough, dyspnea, pallor, diaphoresis, tachycardia • Itching, hives, erythema local or generalized • Large histamine release; massive vasodilation, hypotension, vascular collapse, respir distress, pulmonary edema and death within minutes if not treated.

28. Management of Anaphylaxis • Early recognition * • Epipen or Epinephrine (SC, IM or via ETT) • Maintain airway • Supine with legs elevated (not Trendelenburg) • Antihistamine and steroids adjunct therapy to Epinephrine. • *EMS: maintain unstable airway and IV fluids to treat vascular collapse and establish venous access.

29. Latex Allergy • Most common among health workers • 10% health workers • 50% children with neural tube defects • 34% children with >3 surgeries. • Sources • Gloves, catheters, drains, intravenous ports, rubber stoppers to some medication vials/IV connections (now latex free).

30. Manifestations and Care • Skin reaction; redness and inflammation, blisters, hives, wheals. • Itchy eyes, conjunctivitis, cough, asthma or anaphylaxis • Intraoperative deaths have occurred due to exposure during surgery. • Medical alert bracelet; needs to be listed as allergy in chart. • No latex balloons allowed in hospitals. • Avoidance and use alternative products.