Nursing Care of Children with Immunologic Alterations

1. Nursing Care of Children with Immunologic Alterations By Nataliya Haliyash, MD, BSN Insitute of Nursing, TSMU

2. Lecture objectives Upon completion of this chapter you will be able to: • Describe the normal functions of the immune system. • Describe the etiology, clinical manifestations, and medical treatment for the common immune system alterations, juvenile idiopathic arthritis (JIA), systemic lupus erythematosus (SLE), human immunodeficiency virus (HIV), and allergic reaction to drugs. • Identify nursing management of children with immune system alterations, including developmental and psychosocial needs. • Identify the education, resource, and support needs of families who have children with immune system alterations.

3. Functionsof the immune system • to prevent or ameliorate infections, • to recognize self from nonself, • to maintain homeostasis.

4. Twobasic divisions • The innate immune systemacts as the first line of defense against infections, andincludes biochemicaland physical barriers. • The adaptive immune systemproduces a specificreaction to each infectious agent, remembers that agent, and canprevent a later infection by the same agent.

5. The immune system includes: • the spleen, lymph nodes, and lymphoidtissue, • cellular elements such as the white blood cells orleukocytes, phagocytes, and natural killer cells.

6. Each lymphoid organ plays a role in the production and activation of lymphocytes. Lymphoid organs include: • adenoids (two glands located at the back of the nasal passage) • blood vessels (the arteries, veins, and capillaries through which blood flows) • bone marrow (the soft, spongy tissue found in bone cavities) • lymph nodes (small organs shaped like beans, which are located throughout the body and connect via the lymphatic vessels) • lymphatic vessels (a network of channels throughout the body that carries lymphocytes to the lymphoid organs and bloodstream) • Peyer's patches (lymphoid tissue in the small intestine) • spleen (a fist-sized organ located in the abdominal cavity) • thymus (two lobes that join in front of the trachea behind the breast bone) • tonsils (two oval masses in the back of the throat)

8. A Lymphocytes Cell at Work • T cell (left) recognizes antigens on the surface of a cell infected with a virus (right), enabling the T cell to bind to and kill the infected cell.

9. The immune system of neonates and young children isimmature. • Because of this immaturity, infants and youngchildren are susceptible toinfectious organisms that cancause illness and its associated morbidity. • A child's immune system matures by three to six years of age.

10. Immunity • The term refers to all the processes used bythe body to protect against foreign material from environmentalsources, including microorganisms or their toxins,foods, chemicals, pollen, dander, or drugs. • Innate or natural immunity • Acquired immunity

11. Innate or natural immunity • nonspecific, • function against mostthreats to the body in a broad sense. • Is represented by physicalbarriers such as: • the skin, mucous membranes, • coughreflex; • chemical barriers such as pH of the stomach, fattyacids and proteolytic enzymes of the small intestine, • fever. • Nonspecificimmune cells such as phagocytes (macrophages, neutrophils,natural killer cells), and lymphocytes whose granules releaselysing chemicals.

12. Acquired immunity • is specific immunity, triggeredwhen a person has had prior contact with a foreign agent. • the humoral system, consisting of primarily B lymphocytes • and/or the cell mediated system of primarily the Tlymphocytes

13. Although immune system alterations occur less commonly in childrenthan other types of alterations, the effects are often disabling or terminal. • In addition, the immune system interacts with other body systems sosymptoms may not appear to be immune related but rather primarilymusculoskeletal, • juvenile arthritis, • or integumentary • systemic lupus erythematosus. • HIV, another immune system disease,can affect all organ systems.

14. AUTOIMMUNITY: The inability of the body to distinguish"self" from other, leads to an immune responseaimed at parts of one's own body. • INFLAMMATION: Increased blood flow and permeabilityof blood vessels; results in increased fluid productionand attraction of lymphocytes and leukocytesto the area, caused by the release of inflammatorysubstances called cytokines.

15. Juvenile Idiopathic Arthritis (Juvenile rheumatoid arthritis (JIA, JRA)) • A term used for a group of idiopathic chronic autoimmune inflammatory diseases affecting joints and connective tissues in children with onset before 16 years of age • JIA is the most common pediatric connective tissue disease with arthritis being the principal manifestation. • The incidence is 1:1,000. African-American and Asian children are less likely to suffer from JRA.

16. Pathophysiology of JRA • Current research suggests T cell activation triggers developmentof antigen-antibody complexes, which cause release ofinflammatory substances called cytokines in targeted organssuch as joints and skin. • This causes inflammation of the synovialmembranes and other tissues leading to joint effusionand swelling. • Chronic inflammation eventually evolves intoerosion of articular cartilage and other symptoms of inflammatorydiseases

17. Juvenile Idiopathic Arthritis Patho (arthritis) • Bone overgrowth • Inflamed synovial membrane • Excess synovial fluid • Thinning cartilage

18. Clinical manifestations Systemic onset Fever (usually high) Rash (Salmon-pink, migratory, macular/papular, most common late afternoon or early evening) Arthralgia/myalgia Arthritis Fatigue/malaise Lymphadenopathy Hepatosplenomegaly Possible signs of carditis Juvenile Idiopathic Arthritis arthritis is defined as joint swelling or effusion, or two of the following: warmth, pain on motion, or limited range of motion (continues)

20. Juvenile Idiopathic Arthritis • Polyarticular onset • Arthritis in many joints (five or more) • most particularly the joints of the knees, wrists, ankles, and proximal interphalangeal joints of the fingers. • often neck and temporomandibular (TMJ) joints are affected. • Low-grade fever • Pauciarticular onset • Arthritis in a few joints (less than 4) • most particularly joints of the knees and ankles. • Inflammation of the eyes • common in anti-nuclear antibody positive preschool girls.

22. Diagnosis of Juvenile Idiopathic Arthritis • American College of Rheumatology diagnostic criteria • Onset before 16 years of age • Arthritis of at least 6 weeks’ duration (objectively observed) • A defined subtype (by onset characteristics) • Exclusion of other conditions such as other rheumatic diseases (continues)

23. Diagnosis of Juvenile Idiopathic Arthritis • There are no specific laboratory tests for JRA. • Laboratory data: • elevated erythrocyte sedimentation rate (ESR), • elevated C-reactive protein (CRP), • elevated white blood count, • decreased hemoglobin, • and increased platelet count. • Antinuclear antibody (ANA) and rheumatoid factor (RF) are positive in a proportion of children with arthritis • X rays can demonstrate characteristic changes such as: • soft tissue swelling and joint effusion. • bony erosions and narrowing of the joint spaces • Subluxations and malalignment

24. Treatment of Juvenile Idiopathic Arthritis • Multidisciplinary approach • Medications • Physical and occupational therapy • Nutritional considerations • Family teaching

25. Systemic Lupus Erythematosus • Incidence and etiology: • Although systemic lupus erythematosus (lupus or SLE) can develop at any age, onset in childhood usually occurs after the age of 5 years or during adolescence • Peak age of childhood onset is 11 to 15 years • Involving females 8 to 10 times as often as males • Pathophysiology: • is an autoimmune process requiring a genetic susceptibility and probably a viral or bacterial trigger

26. SLE: Uncontrolled Dialog Between T and B Cells

27. Diagnosis of Lupus Erythematosus • Clinical manifestations (American College of Rheumatology Ad Hoc Committee of Systemic Lupus Erythematosus diagnostic criteria) • Malar rash: Erythematous, flat or raised over the cheeks. • Discoid rash: Erythematous raised patches with scaling. • Photosensitivity: Skin rash from exposure to sun. • Oral or nasal ulcers (continues)

28. The round or disk shaped (discoid) rash of lupus produces red, raised patches with scales. The pores (hair follicles) may be plugged. Scarring often occurs in older lesions. The majority (approximately 90%) of individuals with discoid lupus have only skin involvement as compared to more generalized involvement in systemic lupus erythematosis (SLE).

29. SLE: Malar rash • This young woman has a malar rash (the so-called "butterfly" rash because of the shape across the cheeks// Sparing nasolabial fold). Such a rash suggests lupus.

30. Diagnosis of Lupus Erythematosus • Nonerosive arthritis: • Two or more peripheral joints with tenderness, swelling, or effusion. • Pleuritis or pericarditis • Renal disorder: • Persistent proteinuria OR cellular casts; • can progress to hypertension, nephrotic syndrome, renal insufficiency, and end stage renal disease requiring transplantation. • Neurological disorder: • Seizures OR psychosis without other cause. • Hematological disorder • Immunologic markers • ANA (antinuclear antibody) positive • Alopecia • 4 of the 11 criteria must be present

31. Retinal involvement in SLE

32. Lupus Erythematosus • Treatment • Preventing exacerbations • Treating exacerbations when they occur • Minimizing organ damage and complications • Medications • Nursing management

33. Human Immunodeficiency Virus (HIV) - Incidence: The figures below show the number of children (defined by UNAIDS as under-15s) directly affected by HIV and AIDS: • At the end of 2008, there were 2.1 million children living with HIV around the world. • An estimated 430,000 children became newly infected with HIV in 2008. • Of the 2 million people who died of AIDS during 2008, more than one in seven were children. Every hour, around 31 children die as a result of AIDS. • Most children living with HIV ­– around 9 out of 10 – live in Sub-Saharan Africa, the region of the world where AIDS has taken its greatest toll. Large numbers of children with HIV also live in the Caribbean, Latin America and South/South East Asia. • Around 90% of all children living with HIV acquired the infection from their mothers during pregnancy, birth or breastfeeding.

34. Effects of HIV on children The direct effects of HIV on children • Many children are themselves infected with HIV The effects of HIV on a child’s family • Children live with family members who are infected with HIV. • Children act as carers for sick parents who have AIDS. • Many children have lost one or both parents to AIDS, and are orphaned. • An increasing number of households are headed by children, as AIDS erodes traditional community support systems. • Children end up being their family’s principal wage earners, as AIDS prevents adults from working, and creates expensive medical bills. The effects of HIV on a child’s community • As AIDS ravages a community, schools lose teachers and children are unable to access education. • Doctors and nurses die, and children find it difficult to gain care for childhood diseases. • Children may lose their friends to AIDS. • Children who have HIV in their family may be stigmatized and affected by discrimination.

35. Human Immunodeficiency Virus (HIV) • Etiology • HIV infection: Human Immunodeficiency Virus that attacks the immune system • HIV disease (4 stages) • Acquired immunodeficiency syndrome (AIDS): HIV causes AIDS by damaging the immune system cells until the immune system can no longer fight off other infections that it would usually be able to prevent.It takes around ten years on average for someone with HIV to develop AIDS. • Age-related differences         • Revised pediatric classification system: clinical categories • Pathophysiology

36. How is HIV passed on? HIV is found in the blood and the sexual fluids of an infected person, and in the breast milk of an infected woman. HIV transmission occurs when a sufficient quantity of these fluids get into someone else's bloodstream. There are various ways a person can become infected with HIV: • Unprotected sexual intercourse with an infected person: Sexual intercourse without a condom carries the risk of HIV infection. • Contact with an infected person's blood: If sufficient blood from somebody who has HIV enters someone else's body, then HIV can be passed on in the blood.

37. How is HIV passed on? • Use of infected blood products: Many people in the past have been infected with HIV by the use of blood transfusions and blood products which were contaminated with the virus. In much of the world this is no longer a significant risk, as blood donations are routinely tested for HIV. • Injecting drugs: HIV can be passed on when injecting equipment that has been used by an infected person is then used by someone else. In many parts of the world, often because it is illegal to possess them, injecting equipment or works are shared. • From mother to child: HIV can be transmitted from an infected woman to her baby during pregnancy, delivery and breastfeeding.Without treatment, around 15-30% of babies born to HIV positive women will become infected with HIV during pregnancy and delivery. A further 5-20% will become infected through breastfeeding.

38. HIV and breastfeeding • For most babies, breastfeeding is without question the best way to be fed, but unfortunately breastfeeding can also transmit HIV. • Advice for HIV-positive mothers in developed countries • avoid breastfeeding altogether because the risk of HIV transmission far outweighs the risks associated with replacement feeding. Replacement feeding means giving a baby commercial infant formula (prepared from powder and boiling water) • Advice for HIV-positive mothers in developing countries • In countries with fewer resources, where replacement feeding can be much more hazardous, the recommendations for infant feeding usually depend on a mother's individual situation. When replacement feeding is acceptable, feasible, affordable, sustainable and safe, avoidance of all breastfeeding by HIV-infected mothers is recommended. Otherwise, exclusive breastfeeding is recommended during the first months of life. • For an overview of detailed information, please see 2009 WHO guidelines page.

39. An HIV positive mother and her HIV positive baby in India and an African HIV positive woman breastfeeding her baby

40. An HIV-positive Ukrainian mother practices cup feeding her infant.

41. Human Immunodeficiency Virus (HIV) Clinical manifestations • CD4 counts • To judge whether an HIV-positive person requires treatment, a CD4 test is usually carried out. This measures the number of T-helper cells – white blood cells that are attacked by HIV – in an individual’s blood. It can either measure the absolute number of CD4 cells, or the percentage of white blood cells that are CD4 cells, in a sample of blood. • Normal count: asymptomatic • A falling CD4 count is a sign that HIV is progressing • Associated symptoms of opportunistic infections • The younger the child at time of acquisition, the more severe the symptoms, faster progression, poorer prognosis • Variations by age

42. Diagnosis of HIV • Careful history focusing on risks • Timing of transmission from mother to child • Antibody tests are inexpensive and very accurate. • ELISA (antibody test (enzyme-linked immunoabsorbent) also known as EIA (enzyme immunoassay) • Western blot assay – One of the oldest but most accurate confirmatory antibody tests. It is complex to administer and may produce indeterminate results if a person has a transitory infection with another virus. • An indirect immunofluorescence assay – Like the Western blot, but it uses a microscope to detect HIV antibodies. • A line immunoassay - Commonly used in Europe. Reduces the chance of sample contamination and is as accurate as the Western Blot. Using a rapid oral HIV test

43. Rapid HIV tests • An OraQuick HIV-1/2 rapid test kit • These tests are based on the same technology as ELISA tests, but instead of sending the sample to a laboratory to be analysed, the rapid test can produce results within 20 minutes. • Rapid tests can use either a blood sample or oral fluids. They are easy to use and do not require laboratory facilities or highly trained staff. • All positive results from a rapid test must be followed up with a confirmatory test, the results of which can take from a few days to a few weeks. OraQuick HIV-1/2 test kit

44. Diagnosis of HIV PCR test • A PCR test (Polymerase Chain Reaction test) can detect the genetic material of HIV rather than the antibodies to the virus, and so can identify HIV in the blood within two or three weeks of infection. The test is also known as a viral load test and HIV NAAT (nucleic acid amplification testing). • Babies born to HIV positive mothers are usually tested using a PCR test because they retain their mother's antibodies for several months, making an antibody test inaccurate. • Blood supplies in most developed countries are screened for HIV using PCR tests. However, they are not often used to test for HIV in individuals, as they are very expensive and more complicated to administer and interpret than a standard antibody test.

45. Collection of dried blood spots from an infant for HIV testing at the Lapolagang Clinic in Botswana.

46. HIV develops very rapidly among infants and children, and, without treatment, a third of infected children will die of AIDS before their first birthday, with half dying before they are two. In 2008, there were 280,000 deaths attributed to HIV in under-15s, most of which could have been prevented through early diagnosis and effective treatment. Approaches: Multidisciplinary approach HAART (highly active antiretroviral therapy) Prevention of opportunistic infections Nursing management and family teaching Home School Community Treatment of HIV

47. Treatment of HIV • The most effective treatment for HIV-positive children is antiretroviral therapy. This requires several antiretroviral drugs (ARVs) be taken every day. • Starting antiretroviral treatment in children • There is ongoing debate about when it is best to start antiretroviral treatment in HIV-positive children. There is a complex balance between the immediate benefits of providing treatment to children who are not showing any symptoms of AIDS-related illness, and concerns about long-term resistance and antiretroviral drug side effects if treatment is started too early. • CD4 counts in children • In healthy, uninfected adults, absolute CD4 count is usually between 500 and 1500 cells per cubic millimetre of blood • Absolute CD4 counts vary with age, and younger children usually have a much higher CD4 count than adults. This makes it difficult to judge the health of a child's immune system based on CD4 count. Percentage CD4 count does not vary in the same way as absolute CD4 count, and is therefore recommended for children under five.

48. Which antiretroviral drugs should be used? • As with adults, antiretroviral therapy with at least three drugs is recommended for children as this prevents HIV from becoming resistant to any single drug. • It is usually recommended that this therapy should consist of • two nucleoside reverse transcriptase inhibitors (NRTIs: Epivir (lamivudine), Retrovir (zidovudine), Viread (tenofovir)) combined with • either one non-nucleoside reverse transcriptase inhibitor (NNRTI): Intelence (etravirine) • or a protease inhibitor (PI): Aptivus (tipranavir), Reyataz (atazanavir).

49. Dosing and drug formulations in children “Since there are still no available, easy-to-use triple drug combinations for children, I do what most doctors are doing: I try to show caregivers such as grandparents how to break adult tablets, hoping that the children will get the doses they need.” - Dr Fasineh Samura, Malawi • The dose of antiretroviral drugs given to children is generally based on either weight or body surface area. • As children’s bodies are constantly changing, drug doses need to be altered to make sure that a child is not given too much, or too little, of a drug. • Healthcare workers also need to take into account that children under the age of six metabolise drugs faster than adults, so even after adjusting for body weight, they may need to be given higher quantities of ARVs to achieve the same effect that the drugs would have in adults.

50. Side effects of paediatric HIV treatment • Because children’s bodies are still developing, and they are likely to be exposed to treatment for prolonged periods of time, they may be particularly vulnerable to some complications. • Side effects can occur at various stages of a child’s course of treatment. • They may be • acute (occurring directly after drug administration), • sub-acute (within one or two days after administration) • late (after prolonged drug administration).