Nursing Care of Clients with Immune Disorders Flashcards by Kelsey Klipfel

Normal immune system protects/defends
Disorders: lack of the ability of the body to protect itself against organism or actually attack itself

Immune disorders: concepts

How well did you know this?

Not at all

Perfectly

Excesses or deficiencies of competent cells
Alterations in function of cells
Exaggerated responses to specific antigens
Immunologic attack on self

Disorders: lack of the ability of the body to protect itself against organism or actually attack itself

How well did you know this?

Not at all

Perfectly

Gammopathies or Primary Immunodeficiency

Excesses or deficiencies of competent cells

How well did you know this?

Not at all

Perfectly

Secondary Immune deficiencies

Alterations in function of cells

How well did you know this?

Not at all

Perfectly

Hypersensitivity reactions

Exaggerated responses to specific antigens

How well did you know this?

Not at all

Perfectly

Autoimmune disorders

Immunologic attack on self

How well did you know this?

Not at all

Perfectly

Born with
Rare; genetic cause
(more common male to female 5-1)
More often recessive - often on X chromosome
Usually dx. in infants/toddlers
Some seen in adolescence/young adult
Often accompany autoimmune disorders
Common types
Clinical Manifestations:
Medical Treatment:

Immunodeficiency disorders: primary

How well did you know this?

Not at all

Perfectly

Antibody deficiency
Phagocytic dysfunction
B cells or T cells, (or both) defects = not do job
Complement system deficiency

Common types - Immunodeficiency disorders: primary

How well did you know this?

Not at all

Perfectly

Severe/recurrent infections, failure to thrive or positive family history

Clinical Manifestations: - Immunodeficiency disorders: primary

How well did you know this?

Not at all

Perfectly

Antibiotics: infection/prophylactic - something that likely result in an infection (dental)
IVIG (IV immunoglobulin) or SQ IG
Hematopoietic stem cell transplant
No live vaccines - may get disease attempting to vaccinate against
Genetic counseling - highly recommended

Medical Treatment: - Immunodeficiency disorders: primary

How well did you know this?

Not at all

Perfectly

Curative - give this - going to make new cells cont to make WBCs can cure

Hematopoietic stem cell transplant

How well did you know this?

Not at all

Perfectly

Acquire
Etiology
Causes:
Diagnostics
Treatment
Assessment/Data Collection
N. Diagnosis
N. health promotion/pt teaching

Immunodeficiency disorders: secondary acquired immune deficiency

How well did you know this?

Not at all

Perfectly

Due to malnutrition or HIV
Result of underlying disease processes or treatment
See Neutropenia = once become at high risk for sepsis
WBC <1,000/mm3 (5000-10000 mm3) - on neutropenic precautions

Etiology - Immunodeficiency disorders: secondary acquired immune deficiency

How well did you know this?

Not at all

Perfectly

Autoimmune disorders
Immunotoxic medications
Alcoholism, drug abuse
Spleen removal
Malnutrition/stress
HIV

Causes: - Immunodeficiency disorders: secondary acquired immune deficiency

How well did you know this?

Not at all

Perfectly

Corticosteroids = long-term; prolonged NSAID, Chemo - particular bllod cells; radiation
NADER - predive point where going to have low WBC count in response to med - highest risk for infection
Absolute neutrophil count - check before give chemo - status of WBCs before give chemo - not want bottom out numbers

Immunotoxic medications

How well did you know this?

Not at all

Perfectly

WBC/diff: severe neutropenia - lab levels carefully - number segs and bands with pts
Segs - fully matured WBCs; should be high
Bands - immature WBCs; should have low; higher number - bandemia (band count greater than 10%) - consider what going on with pt - turning out immature WBCs increasing number immature ones
Bone marrow biopsy

Diagnostics - Immunodeficiency disorders: secondary acquired immune deficiency

How well did you know this?

Not at all

Perfectly

Infections
Intravenous immunoglobulin (IVIG)
Hematopoietic stem cell transplant
Monoclonal antibody therapy
Growth factors (neupogen)

Treatment - Immunodeficiency disorders: secondary acquired immune deficiency

How well did you know this?

Not at all

Perfectly

Provide to those lacking immunoglobulin

Intravenous immunoglobulin (IVIG)

How well did you know this?

Not at all

Perfectly

Potential
Not curative
Can help reduce some comps

Hematopoietic stem cell transplant

How well did you know this?

Not at all

Perfectly

Target antibodies to specific antigens
-mab

Monoclonal antibody therapy

How well did you know this?

Not at all

Perfectly

Increase production of WBCs for pts

Growth factors (neupogen)

How well did you know this?

Not at all

Perfectly

History-past infections, treatment response to various infections
Nutritional status, hygiene, use of alcohol/drugs/tobacco
Physical: monitor for Manifestations of infection
Pts do not have typical manifestations of infection - do more detailed assessment
Monitor: Manifestations infection

Assessment/Data Collection - Immunodeficiency disorders: secondary acquired immune deficiency

How well did you know this?

Not at all

Perfectly

VS, lab values, C&S reports from wounds, lesions, sputum, urine, blood - determine infection
Pay attention to WBC - segs and bands
Trend imp

Monitor: Manifestations infection

How well did you know this?

Not at all

Perfectly

Ineffective protection
Risk for Infection
Risk for impaired skin integrity
Ineffective health maintenance
Imbalanced nutrition: less
Social isolation
Fear r/t threat to well-being

N. Diagnosis - Immunodeficiency disorders: secondary acquired immune deficiency

How well did you know this?

Not at all

Perfectly

Prevention of infection Lifestyle mod. to reduce risk Nutrition and diet Neutropenic precautions Manifestations of infection Medication teaching Prophylactic med regimen: Follow-up care with physicians/specialists

N. health promotion/pt teaching - Immunodeficiency disorders: secondary acquired immune deficiency

Frequent and quality Handwashing Avoid crowds/infections Good Hygiene and cleaning home Daily bath, foot care, good dental hygiene Scrub raw fruits and veg throroughly, food storage and preparation - heat to correct temp Cleaning kitchen, bathroom surfaces

Prevention of infection

Acquired Pathophysiology Stage I: Category A Stage 2: Category B Stage 3: Category C Risk factors HIV Lab tests for diagnosing and tracking Nurse role: HIV screening process N. counseling Gerontology considerations Medical care PrEP med Medical treatment: probs with compliance: Collaborative care Opportunistic diseases N. process: assessment/diagnosis Diagnosis AIDS N. process: planning/goals AIDS N. interventions N. health maintainence

Immunodeficiency disorders: secondary HIV/AIDS

Retrovirus: intracellular parasite - act as a parasite HIV targets cells with CD4 receptors (special T cells) Complex life cycle that turns the host cells into “factories” for HIV Retrovirus hijac RNA - turn CD4 into factories that produce HIV to go throughout body

Pathophysiology

Contract infection Primary infection: Rapid viral replication (1-3 weeks) Feel cold, flu like symptoms - broad symptoms then go Asymptomatic (can go for 10 yrs) - spread illness during time CD4 - 500-1200 normally

Stage I: Category A

HIV symptomatic: CD4 <200 - now classified as AIDS AIDS begins

Stage 2: Category B

Severe AIDS-CD4 <100 At risk for Opportunistic diseases - not typ see in gen pop

Stage 3: Category C

Sharing infected injection drug use equipment - share needles Having sex with infected individuals (male and female) Infants born to mothers with HIV infection and/or breast-fed by HIV-infected mothers People who received organ transplants, HIV-infected blood, or blood products (esp between 1978-1985) Reduce risk Postexposure prophylaxis (PEP)

Risk factors HIV

Abstinence Being faithful - limit partners Always use condoms Transmitted in body fluid = more blood and sexual transmission Occupational exposures - needle stick

Reduce risk

Stuck self needle already injected in pt that HIV + Reduce chance of seroconversion - reduce change starting make HIV virus and reduce chance become +; has start within 72 hrs of exposure

Postexposure prophylaxis (PEP)

EIA Western blot Viral load CD4/CD8 OraQuick

Lab tests for diagnosing and tracking

Enzyme immunoassay Antibodies are detected, resulting in + results and marking end of window period Do serial testing with them Not enough to confirm HIV - do Western blot

EIA

Definitive confirmation test Able detect antibodies Also detects HIV antibodies; confirms EIA

Western blot

Measures HIV RNA in the plasma Low or high viral load - more likely transmit the virus with a higher viral load

Viral load

monitor CD4 and CD8 cells These are markers found on lymphocytes HIV kills CD4 cells which results in significantly impaired immune sys

CD4/CD8

In-home HIV test Get results of viral load and CD4 count

OraQuick

Pre and post test counseling - nervous Sequence due to “Window Period” - virus not fully ramped up Retesting essential at 3 wks, 6 wks, and 3 mo 3 weeks - 3 months between infection with HIV and seroconversion (production of antibodies against the virus) - body time to seroconvert; to take PEP need HIV test to start - not previously HIV + = come back again for further testing later Serial testing imp HIV screening may be negative if done early - early tests not always indicative

Nurse role: HIV screening process

Focus on client's own unique circumstances/risks - pt situation Acknowledge/support for positive steps already made - explain what happened and make no judgments Enhance self-perception of risk - engage risky behaviors - educate them Use explicit language - plain, straightforward language Help client set goal to reduce chance of acquiring/ transmitting HIV - PEP if qualify; HIV+ steps take to avoid transmitting and not + avoid virus Avoid providing unnecessary information Use condoms, use protection during oral sex, don’t share sex toys - anything exposed to body fluid - not share

N. counseling

One quarter of people living with HIV: age > 50 Reasons Unprotected intercourse - esp postmenopausal women Do not consider themselves at risk Social bias toward homosexuality - NOT TRUE Current or past use IV drugs (share needles) Received HIV-infected blood before 1985 - if received blood before 85 should get a HIV test Reduction in immune system function

Gerontology considerations

Antiretroviral therapy (ART) to suppress virus Prevent or decrease complications Monitor disease progression & immune function - CD4 counts and viral load Manage symptoms Prevent dev. of opportunistic disease Detect and treat early Prevent transmission of HIV to partners/others

Goals: - Medical care

Suppress HIV replication/prevents drug resistance Reduce morbidity and prolong duration of life/quality of survival Restore and preserve immunologic function Suppress plasma HIV viral load - less risk spread or immunosuppression Prevent HIV transmission In U.S., ART is now recommended for all HIV-infected patients regardless of their viral load or CD4+ count

Goals of ART: - Medical care

Truvada (combo: emtricitabine and tenofovir) Pre-Exposure Prophylaxis High risk indivs Reduces risk of transmission From known HIV+ people to HIV- Need to use condoms Need baseline HIV testing and other labs every 3 months - hard kidneys, liver Does not protect from STD Daily dose; Do not miss any doses - decrease effectiveness of med

PrEP med

Risk of damage to kidney/liver Lactic acidosis Osteopenia (bone softening) - higher risk for fractures Headache Abd. Pain - diarrhea Weight loss

Adverse effects - PrEP med

Do not miss any doses of TRUVADA. Missing doses may increase your risk of getting HIV-1infection. To further help reduce your risk of getting HIV-1: Know your HIV status and the HIV status of your partners. If your partner is living with HIV, your risk of getting HIV is lower if your partner consistently takes HIV treatment every day. Get tested for other sexually transmitted infections. Other infections make it easier for HIV to infect you. Practice safer sex by using latex or polyurethane condoms to lower the chance of sexual contact with body fluids. Talk to your healthcare provider about all the ways to help reduce HIV risk.

Patient ed - PrEP med

CNS: depression, neuropathies, fatigue GI/Bili: N/V/D - subside after used to it but chronic use can wax and wane, Pancreatitis, Hepatotoxicity - monitor liver labs Renal: stones, nephrotoxicity, renal failure - monitor labs CV: HTN, MI, stroke, dysrhythmias Pulmonary: bronchitis, dyspnea MS/skin: Osteopenia; Fat redistribution syndrome (lipodystrophy) Hematologic: anemia, neutropenia, thrombocytopenia - serial labs Endocrine: Insulin resistance - longer on it so can turn into diabetes

AE - long-term antiretroviral therapy - PrEP med

Compliance is huge issue with HIV patients CDC estimates 36% of HIV pts use ART; only 76% have suppressed viral loads - follow-up how compliant with ART Positive relationship between patient and health care provider is associated with better adherence - nonjudgemental; consistency with HCPs

Medical treatment: probs with compliance:

Side effects of meds - fatigue, diarrhea, nausea Psychological/mental illness/neurocognitive impairment Low health literacy - not realize importance Low social support - psych issue; afford meds High alcohol consumptions/active substance abuse Homelessness/poverty Nondisclosure of HIV serostatus, denial stigma Inconsistent access to medication

Barriers to adherence to treatment plan

Opportunistic infections Respiratory failure Cachexia and wasting - before ART initiated Medication side effects Education and support PT, OT, dietary, counseling (indiv and group) Educate about self-care Community support systems Emotional and ethical concerns

Collaborative care

Psoriasis Kaposi's sarcoma Respiratory Neurologic Manifestations Gynecologic Manifestations Integumentary Manifestations GI Manifestations Candidiasis-common Patho where body can typ fight it off HIV wasting syndrome -10% weight loss Cancers: Eyes: Cytomegalovirus Retinitis Oral lesions from genital warts

Opportunistic diseases

Worse in people with HIV+

Psoriasis

most common opportunistic eye infection seen in patients who are immunosuppressed is cytomegalovirus (CMV) Type cancer Gen can fight off if not immunocompromised

Kaposi's sarcoma

Pneumocystis jiroveci pneumonia Cytomegalovirus (CMV) Legionella species Frequently resides in venting and ducting Mycobacterium avium complex Tuberculosis

Respiratory

HIV encephalopathy Cryptococcus neoformans Fungal meningitis

Neurologic Manifestations

Candidiasis (thrush - seen often), genital warts, PID, cancer

Gynecologic Manifestations

Herpes zoster and herpes simplex Eczema or psoriasis

Integumentary Manifestations

(oral to rectum)

GI Manifestations

Diarrhea/weakness/fever >30 days

HIV wasting syndrome -10% weight loss

Kaposi's sarcoma (human herpesvirus-8) Lymphoma

Cancers:

Leading cause of blindness in AIDS

Eyes: Cytomegalovirus Retinitis

More likely to have wart outbreaks

Oral lesions from genital warts

Assess physical and psychosocial status Identify risk factors: IV drug use, sexual practices Immune system function Nutritional status Skin integrity - imp to prevent infections Respiratory status and neurologic status Fluid and electrolyte balance Knowledge level of disease process - health literacy imp

N. process: assessment/diagnosis

Impaired skin integrity Diarrhea Risk for infection Activity intolerance Disturbed thought processes Ineffective airway clearance Pain Imbalanced nutrition Social isolation Anticipatory grieving Deficient knowledge

Diagnosis

Absence of infection Expression of grief - expected and norm; need them to cope and not feel hopeless Improved nutritional status Increased socialization Increased knowledge regarding disease prevention and self-care Increased comfort Improved airway clearance Improved activity tolerance Maintenance of skin integrity Resumption of usual bowel patterns Improved thought processes

AIDS N. process: planning/goals

Interventions: Neuro Status Interventions: Activity Interventions: Skin Interventions: Bowels Interventions: Nutrition Interventions: Isolation

AIDS N. interventions

Assess mental/neurologic status Use clear, simple language (mental status) Establish and maintain a daily routine Orientation techniques Ensure patient safety and protect from injury Strategies to maintain and improve functional ability Instruct and involve family in communication and care Ensure patient safety -protect

Interventions: Neuro Status

Balance between activity and rest Maintain balance between activity and rest Fatigued easily Instruction regarding energy conservation techniques Relaxation measures Collaboration with other members of the health care team

Interventions: Activity

Assess / reposition every 2 hr Pressure reduction devices Skin care

Interventions: Skin

Eliminate food irritants Meds as prescribed Frequent routine assessment of skin and mucosa Encourage patient to maintain balance between rest and activity Reposition at least every 2 hours and as needed Pressure reduction devices Instruct patient to avoid scratching Use gentle, nondrying soaps or cleansers Avoid adhesive tape Perianal skin care Assess bowel pattern; factors that exacerbate diarrhea Avoid foods that act as bowel irritants, such as raw fruits and vegetables, carbonated beverages, spicy foods, and foods of extreme temperatures Small, frequent meals Administer medications as prescribed Assess and promote self-care strategies to control diarrhea

Interventions: Bowels

Control N/V/oral discomfort Weigh daily Relieve thrush as best as possible High calorie/protein food Fluid 2-3L day Monitor weight, I&O, dietary intake Dietary consult:high calorie, high protein, low fat Control of nausea with anti-emetics Oral hygiene Treatment of oral discomfort Dietary supplements Enteral feedings or parenteral nutrition Fluid intake 2-3 L day Advise no alcohol Fluid intake 2-3 L/day Promotion of nutrition Monitor weight Provide high calorie, high protein, low fat May become lactose intolerance Destruction of intestinal villa Can not absorb diet high in fat Persons develop high metabolic rate as disease progresses and have an ↑ loss of body protein

Interventions: Nutrition

Acceptance and understanding Promote an atmosphere of acceptance and understanding Assess social interactions and monitor behaviors Allow patient to express feelings Address psychosocial issues Provide information R/T spread of infection Educate ancillary personnel, family, and partners

Interventions: Isolation

Encourage exercise Don’t stop medications - prolong quality and length of life Stress reduction - avoid excessive Maintain vaccinations Monitor labs Assess for infections Know When to call HCP for s/s of infection Discuss risky behaviors and how to adjust these

N. health maintainence

Rejection Graft vs host disease Hyperacute Acute Chronic rejection N. implications N. diagnosis

Transplant

Graft versus Host Graft - organ transplanted into the patient Host - pt receiving transplant Hyperacute Acute Chronic Medications to prevent rejection Prednisone Tacrolimus (Prograf) Cyclosporine (Neoral) Mycophenolate Mofetil (CellCept) Imuran (Azathioprine) Rapamune (Rapamycin, Sirolimus) Azathioprine (Imuran) Sirolimus (Rapamune) Everolimus (Zortress) Specific types of transplants located in diff body systems - rejection process can be very complication - organ not self and try to destroy anything not self - suppress immune sys

Rejection

Graft = Transplant Host = Receptacle Natural killer and cytotoxic cells Attack transplant Immune system reacts to non-self and destroys transplant Must suppress the immune system

Graft vs host disease

Occurs immediately Antibodies react to antigens Activates Complement cascade Activates Blood clots throughout new organ - lead to necrosis - and various enzymes Huge Inflammation Massive cellular destruction Kidneys most at risk Once starts, cannot be stopped s/s: Physiologic distress Low BP High HR Elevated Cr Protein in urine Mass rejection Inflammatory response

Hyperacute

1 week to 3 months 2 diff mechanisms 1.Antibody mediated vasculitis leads to vessel necrosis - destroys vessels 2.Cytotoxic and NK cells start inflammatory process and cellular lysis - kills cells - lyse cells so organ cannot func Organ death may occur or may be medically managed. Recognized quick enough may be able to save the organ with med changes

Acute

Result of Inflammation and scarring - scar tissue develops from inflammation Vessel occlusion Chronic ischemia because of blood vessel injury Specific to type of transplanted organ From of graft versus hosts - foreign organ In all pts - rate which occurs or controlled varies Cure: retransplantation - delay as much as possible, preserving the integrity of the transplanted organ

Chronic rejection

Be aware of rejection in conjunction with specific transplanted material (ie renal failure in kidney transplant Give scheduled anti-rejection medication as scheduled - imp of regimen Know anti-rejection medication and side effects Know the anti-rejection medication lab values for therapeutic readings Watch for manifestations of infection (Elevated temperature and heart rate)

N. implications

Some of the most common side-effects of anti-rejection medicines can include higher blood pressure, higher blood sugar, weight gain, an increased chance of having infections, and increased risk of some forms of cancer. is an increased risk of infection. Other, less serious side effects can include loss of appetite, nausea, vomiting, increased hair growth, and hand trembling. These effects typically subside as the body adjusts to the immunosuppressant drugs stomach upset

Know anti-rejection medication and side effects

Prednisone is available in liquid as Prelone (15 mg/5 ml) or in 1 mg, 2.5 mg, 5 mg, 10 mg, 20 mg, or 50 mg tablets. The tablets can be cut in half if necessary. Prograf is available as liquid or in 0.5 mg, 1 mg, and 5 mg capsules. Neoral is available in liquid form or in 25 mg and 100 mg capsules. CellCept is available in liquid or in 250 mg capsules or 500 mg tablets. Imuran comes as a 50 mg tablet that can be broken up. Some pharmacies, including the outpatient pharmacy at Cincinnati Children's, can also prepare a liquid version of Imuran for patients who have trouble taking the tablet. Rapamune comes in 1 mg and 2 mg tablets or as solution containing 1 mg/1 ml 5-20 ng/mL

Know the anti-rejection medication lab values for therapeutic readings

Ineffective Protection r/t immunotherapy suppression

N. diagnosis

Types Type I -IgE mediated (allergy, angioedema, anaphylactic) - most severe and most rapid intervention Type II -Cell specific (hemolytic reactions with blood transfusion) Type III-Immune complex mediated (autoimmune disorders - RA) Type IV-Cell-mediated (TB test and is +; Poison Ivy, Transplant rejection) Types May occur simultaneously and/or sequentially (one can turn into another type)

Altered immune response: hypersensitivity disorders

IgE Hypersensitive reaction to an allergen Involves blood vessels, all layers of skin, mucous membranes, and subcutaneous tissues Depth of swelling can result in airway issue Most common area: Lips, face, tongue, larynx, and neck Cause: ingested meds Most common ACEIs and NSAID; food allergies Greatest risk within the first 24 hours after taking the first dose, reaction can occur after days, months, and even years of therapy Collaborative care and interventions

Angioedema (Type 1)

Potential for airway obstruction due to mucosal swelling Anxiety due to cerebral hypoxia and threat of death Interventions focus Stop reaction and ensuring an adequate airway Reverse angioedema before laryngeal edema forms and intubation is needed - edema not reversed may do emergent tracheostomy if the pt does have airway that blocks off Maintain gas exchange Apply O2 Decrease swelling Epinephrine Corticosteroids Diphenhydramine - Benadryl Laryngeal edema forms If intubation is not possible emergency tracheostomy if cannot stop swelling before airway blocked off Antigen drug still in system – continue dose of meds to fight off drug Go to ICU

Collaborative care and interventions - Angioedema (Type 1)

Most severe Unanticipated severe allergic reaction with rapid onset Response = amount of allergen, amount of mediator released, sensitivity of target organ and person, route of entry Clinical Manifestation: Causative agents Mild to severe; local or systemic N. - prevention Collaborative management pt/fam edu: care and use of automatic epinephrine injections

Anaphylaxis (type 1)

Overall edema, laryngeal edema, hypotension, bronchospasm, cardiovascular collapse, shock (anaphylactic shock because decrease in fluid from intravascular space)

Clinical Manifestation: - Anaphylaxis (type 1)

Medications -Antibiotics and radiocontrast agents (most serious) Foods, antitoxins, insect stings, latex

Causative agents - Anaphylaxis (type 1)

Avoidance of potential Allergens ALWAYS check for allergies/assess risk Stay with patient when receiving new drugs especially IV antibiotics Teach patient use of EpiPen, wear Medical ID bracelet or necklace Desensitization therapy Body exposure to small injection of allergen, gradually increasing doses to build up immunity (under medical supervision)

N. - prevention - Anaphylaxis (type 1)

Stop drug/Change IV tubing/fluid bolus - fluid support - stablize BP - support kidneys to help clean it out Call Rapid Response/Code cart Patent Airway/Oxygen Apply O2 100% NRB (non rebreather), place pt. in High Fowler’s position Suction prn/ABGs prn Monitor VS/pulse oximetry - tele Medication Subcutaneous epinephrine 1:1000 (0.3 to 0.5 ml) followed by continuous IV Diphenhydramine (Benadryl)–block histamine; can give H2 blockers - pepsid type medications - reduce inflammation Corticosteroids: second line drug ICU- monitor for rebound (delayed reaction - still in their body) 4-8 hours after initial reaction

Collaborative management - Anaphylaxis (type 1)

Practice assembly of injection device training device Keep the device with you at all times. Use device when any symptom of anaphylaxis is present and call 911 When needed, inject drug into top of high on outside of thigh high, with needle entering straight down. Inject drug right through pants; avoid seams/pockets (fabric thicker) After use go to nearest hospital for monitoring (next 4 to 6 hours) Keep two drug-filled devices in case more than one dose is needed Protect device from light and avoid temperature extremes

pt/fam edu: care and use of automatic epinephrine injections - Anaphylaxis (type 1)

Autoantibodies directed against self cells that have foreign protein or other antigen Autoantibodies bind with self cell creating immune complex Self cell destroyed with attached protein Examples Hemolytic transfusion reactions (patient receives wrong blood type during a transfusion) Clinical Manifestations Collaborative management

Cytotoxic reactions (Type 2): EX: Hemolytic

Pallor Fatigue Lightheadedness Weakness Dark urine Jaundice Heart murmur tachycardia Enlarged liver/spleen

Clinical Manifestations - Cytotoxic reactions (Type 2): EX: Hemolytic

Stop offending drug or blood product Plasmapheresis Filtration of blood to remove antibodies) Treat symptoms Complication Hemolytic crisis and kidney failure Hemolytic crisis Large numbers of red blood cells are destroyed over a short time Body cannot make enough RBCs to replace destroyed RBC-severe anemia leads to kidney damage Precipitated by nonspecific factors infection, surgery and transfusion Kidney damage occurs due to the large deposits of debris and clotting

Collaborative management - Cytotoxic reactions (Type 2): EX: Hemolytic

Antigen-antibody complexes formed in circulation and deposited later in vessel walls or other tissue (kidney, vessels, skin, joints) IgG and IgM antibody-antigen immune complexes in circulation Harmful effects caused by complement cascade activation Attracts neutrophils Unsuccessful phagocytosis Release of lysosomal enzymes into inflammatory site Many immune disorders are type 3 Ex: S. lupus, Rheumatoid arthritis Autoimmune diseases

Immune complex rxns (type III)

Production of antibodies against the body’s own cells; destructive process Both antibody- and cell-mediated responses and products are directed against normal body cells Incidence in general population around 5% Over 200 diseases Autoimmune disorders overlap more than one disorder tend to occur Women most commonly affected Can affect all ages and gender Autoimmune CT disorders Primary target two major structural protein molecules Elastin: major component of ligaments and skin Collagen: component of tissues and blood vessels Classic Diseases Rheumatoid Arthritis Systemic Lupus Erythematosus Scleroderma Many others

Autoimmune diseases - Immune complex rxns (type III)

Damage to joints bilaterally & symmetrically Women child bearing years Exacerbations/remission Triggers (physical and emotional stress) Clinical Manifestations Diagnosis criteria: American Rheumatism Association

Difficult to rise AM (pain, stiffness) Joint pain/deformities Rheumatoid nodules Systemic

Clinical Manifestations - RA

Four of Seven Criteria for diagnosis Morning stiffness > 1 hour 6 wk duration Soft tissue swelling of wrist, metacarpophalangeal or proximal interphalangeal joints for 6 wk duration Symmetric soft tissue swelling Rheumatoid nodules Positive serum rheumatoid factor test Radiographic changes in hands or wrist joints

Diagnosis criteria: American Rheumatism Association - RA

Red = erythematosis Lupus = wolf Red wolf Immune complexes invade organs Characterized by variability Mild to severe Women in childbearing years Exacerbation/remissions Triggers: Clinical manifestations Criteria for diagnosis (American college of rheumatology)

Systemic Lupus Erythematosus

Hormones Menses/pregnancy exacerbate disease Sun exposure Infections Drugs

Triggers: - Systemic Lupus Erythematosus

Fever/fatigue/weak Polyarthritis (non-deforming) Myalgias Raynaud’s/rashes Peripheral neuropathies Nephritis leading cause of death May affect many organs

Clinical manifestations - Systemic Lupus Erythematosus

Four or more serially or simultaneously Malar rash Discoid rash Photosensitivity Oral ulcers Arthritis (usually non-deforming) ANA titer abnormal (>20) Serositis (Pleuritis/Pericarditis) Other organ involvement

Criteria for diagnosis (American college of rheumatology) - Systemic Lupus Erythematosus

“Disease turns pt to stone” Inflammation and progressive tissue fibrosis and occlusion of microvasculature by collagen Two types CM Diagnostics tests and med treatment (autoimmune CT diseases)

Scleroderma (systemic sclerosis)

African-American Women middle age Exacerbation/remissions

Inflammation and progressive tissue fibrosis and occlusion of microvasculature by collagen - Scleroderma (systemic sclerosis)

Limited cutaneous disease-most common Symmetric skin thickening limited to distal extremities and face. Diffuse cutaneous disease Organ involvement

Two types - Scleroderma (systemic sclerosis)

Raynaud’s (early sign 90%) Skin hardening Sclerodactyly (Stiff fingers) Telangiectasias Dilated superficial blood vessels Heartburn/dysphagia Joint pain Pulmonary fibrosis Anti-centromere antibody SCL-70

CM - Scleroderma (systemic sclerosis)

Specialized blood tests (RA factor, ANA, LE prep, complement levels) CBC/Chemistry Liver/renal function X-ray joints Chest X-ray ECG NO Cure Pain control Meds: Immunosuppressive Physical therapy Diet high in calories /vitamins Joint support Surgery Correct deformity, restore function

Diagnostics tests and med treatment (autoimmune CT diseases) - Scleroderma (systemic sclerosis)

Anxiety Fatigue Pain Impaired mobility Ineffective coping Potential for Infection Body Image Knowledge deficit Readiness for enhanced self-care Assess for organ involvement Prevent infection Joint protection Pain management Skin Protection Manage fatigue/Energy conservation Enhance body image Compliance with therapeutic regimen Manage medications and side effects Maintain optimal role function and self image

Autoimmune CT diseases: n. Diagnosis and intervetnions

Assist with coping Hereditary concerns Pregnancy and sexual counseling Ability to work Physical changes may cause problems for body image and social isolation

Psychological Issues - Autoimmune CT disease: n. Counseling and edu

Coping with chronic illness Avoidance of stress Protection from infections Establish exercise program Diet and nutrition counseling Sun restriction/physical limits Pregnancy

Patient / Family Education - Autoimmune CT disease: n. Counseling and edu

Mediated by T cell lymphocytes – do not involve antibody Delayed: 12-72 hours after exposure to antigen Example: TB test, poison ivy, Graft vs Host Graft-versus-host disease (GVHD) Immune cells present in donor tissue (the graft) attack host's own tissues Clinical manifestations Skin rash, jaundice, GI Immunosuppressive medications (prednisone; cellcept)are primary therapy

Delayed hypersensitivity (Type IV)