Immunology and Cellular changes



The Immune System...




Leukemia







Leukemia’s:
Malignant diseases of the bone marrow and lymphatic system

Pathophysiology:
Over production of immature WBCs in the blood producing tissues of the body
WBCs are low - leuk’emia’
Compete with normal red blood cell production
So…
Anemia - decreased RBCs
Infection - neutropenia
Bleeding - decreased platelet production
Weakened bone…fractures
Bone pain from invasion and pressure
Spleen, liver and lymph infiltrated

More common in Caucasian males between 2 and 5
Acute lymphoid leukemia (ALL) cure rate ~ 80%
Acute Nonlymphoid leukemia (acute myelogenous leukemia) AML cure rate ~50%






Survival rates are influenced by:
Initial WBC count
Age at diagnosis
Type of cell involved
Sex
Karotype analysis

Management:
Induction therapy
Elimination of leukemic cells in bone marrow
CNS prophylactic therapy - prevents CNS invasion
Intensification therapy – eliminates residual cells
Maintenance therapy – maintain remission
Hematopoietic stem cell transplant

Possible second malignancy

Nursing Care:
*Education due to all procedures – lumbar punctures, bone marrow aspiration and biopsy, finger sticks and blood draws
Pain relief
Prevent infection, hand washing, and restrict sick visitors, private rooms
Bleeding, administer platelets, aseptic technique with sticks, activities restriction
Observe for 20 minutes after chemo administration
Safeguard nurses
Venous access device

Complications from drug toxicity:
Anaphylaxis
Nausea and vomiting – zofran, dexamethasone, before the chemo
Anorexia
Mucosal ulceration – bland, soft diet, saline washes, soft tooth sponges, local anesthetics
Rectal ulceration sitz baths, stool softners, no rectal temps
Neuropathy
Hemorrhagic cystitis (chemical irritation) increase fluid, frequent voiding, meds
Alopecia maybe, hair regrows in 3-6 months, thicker, curlier, darker
Moon face from steroids (increase appetite, mood)
Mood changes (steroids)


Lymphomas
Hodgkin and Non-Hodgkins


Hodgkins Lymphoma



Hodgkins lymphoma ‘hogs’ the lymph nodes
Age 15-19
Originates in the lymph nodes
Metastasizes to spleen, liver, bone marrow, lungs
A – asymptomatic
B – symptomatic – fever, night sweats, weight loss, abdominal pain, nausea, cough, pruritis

Tests:
CBC
Erythrocyte sedimentation rate
Serum copper
Ferritin
Fibrinogen
Immunoglobulins
T-cell function
Uric acid
Liver function tests
Urinalysis
Lymph node biopsy – Reed Sternberg cells
Bone marrow aspiration

Primary treatment:
Radiation and chemotherapy
Early diagnosis 90% survival, advanced 65-75% survival

Nursing:
Preparation, explanation, support
Fatigue
Infertility – sperm bank

Non-Hodgkins lymphoma ‘not a hog, shares and spreads’
Younger than 14
Diffuse and disseminated
Lymphoblastic
Burkitt or Non-Burkitt
Large cell

Clinical manifestations depend on site and extent
Organ symptoms from enlarged lymph nodes
Leukemia like symptoms
Hodgkins like symptoms

Tests:
Lymph node biopsy
Bone marrow examination
Radiographic studies esp. of GI and lungs
Lumbar puncture

Management:
Aggressive irradiation and chemotherapy
Prognosis excellent with localized disease
Relapse after 2 years is rare, survival after 24 months is considered a cure
Nursing care is directed at chemo administration, managing side effects and support

Wiki Notes Immunology/Cellular changes

HIV
Retrovirus transmitted by lymphocytes and monocytes
Found in blood, semen, vaginal secretions and breast milk
Incubation of months to years
Horizontal/Vertical transmission

HIV virus infects T lymphocytes…the virus takes over the cell machinery=dysfunctional
Lymphocytes decrease leading to immunodeficiency, opportunistic illnesses, death

1980s - At first in pediatrics acquired from mother, rarely blood or abuse
Teens drug use, sexual activity
Decline since due to testing
Perinatal - Highly Active Antiretroviral Therapy (HAART)
HAART combination of 6 drug classes including 25 medicines
HAART must be taken everyday, as prescribed, not a cure but controls the virus
Medications prevent viral replication so there is less virus in the system
This leaves your immune system a chance to recover and to fight off disease
Left untreated HIV attacks the immune system and becomes AIDS






Diagnosis:
>18 months enzyme-linked immunosorbent assay (ELISA), western blot immunoassay
Will be positive till 18 months due to maternal antibodies

Mild symptoms
Lymphadenopathy
Parotitis
Hepatosplenomegaly
Recurrent sinusitis or otitis media
Oral candidiasis
Failure to thrive
Developmental delay
Recurrent diarrhea

Moderate
Lymphoid interstitial pneumonitis (LIP), organ specific dysfunctions or infections

Severe
AIDs defining illnesses
Wasting syndrome
Cytomegalovirus disease
*Pneumocystis carinii pneumonia (PCP), most common opportunistic
Candidal esophagitis
Pulmonary candidiasis
Herpes Simplex

Therapeutic Management:
Goal to slow the growth of the virus
Antiretroviral drugs suppress replication
Therapy lifelong




X linked recessive disorders:

X linked agammaglobulinemia:

Diagnosis: delayed due to immune development and maternal transfer of IgG
Eventual bone marrow transplant, hematopoietic stem cell transplant (HSCT)

Nursing Care:
Prevent infection
Support
Genetic counseling

Wiskott-Aldrich Syndrome (WAS)

Triad: eczema, thrombocytopenia, immunodeficiency

Management:
Platelets for bleeding
IV gamma globulin
Prophylactic antibiotics
Bone marrow transplant (HSCT)


Blood Transfusion Therapy:
Order
Consent
Baseline vital signs, then q15 for 1 hour
Check ID with another nurse
Administer first 50 ml and stay with child
Use appropriate tubing and filter (not microdrops, regular 10-15 drops/ml)
Gently shake
Use within 30 minutes or send back
Infuse within 4 hours
Rx: STOP transfusion, leave line open with normal saline and new tubing
Contact physician

Complications:
Hemolytic rx
Fever (acetaminophen)
Allergic rx (antihistamines)
Circulatory overload (transfuse slowly)
Air emboli
Hypothermia (allow blood to warm)
Alloimmunization (sensitization)





Hematopoietic Stem Cell Transplant (HSCT)



Hematopoietic Stem Cell Transplant (HSCT)

Steps:
Immune system is suppressed/destoyed by chemo and radiation
Stem cells are transfused and begin to repopulate the bone marrow
*Once this begins no turning back, no rescue
*Problem: GVHD (graft versus host disease)
Ex. Kidney transplant/dialysis = rescue

Allogeneic - matching bone marrow
Umbilical stem cell, cord blood
Autologous - patient’s own marrow

Management:
3-6 week hospitalization
Fear complications




Apheresis:
Removal of blood
Separation into blood components
Reinfusion

Self versus Nonself:

Juvenile Idiopathic Arthritis, JIA, (Juvenile Rheumatoid Arthritis, JRA)





Juvenile idiopathic arthritis:
Chronic autoimmune disease causing chronic inflammation of joints and tissues
Before age 16 with peak between ages 1 and 3
Twice as many girls afflicted
Joint inflammation > effusion > erosion > fibrosis
Can be systemic with other organs affected such as skin, heart and eyes
Frequent eye exams to watch for chronic or acute uveitis (infammation of the iris and cillary body)

Goal is to decrease pain, inflammation and disfigurement. Promote growth and development.

No definitive tests

Treatment/Nursing interventions:
Medication administration
  • Nonsteroidal antiinflammatory drugs (NSAIDs)
  • Methotrexate
  • Corticosteroids
  • Entanercept (Enbrel) * a newer successful medication
  • Slow acting antirheumatic drugs (SAARDs) sulfasalazine, hydroxychloroquine, gold, and D-penicillamine

Physical and occupational therapy
Ophthalmologic exams
Splints - usually at night
Comfort measures - warm baths, hand soaks, swimming
Dietary support - calibrated to activity level so child doesn't struggle with obesity which can put more work on joints
School and psychosocial support - may need to come in later after joints warm up in am
Low impact sports



Systemic Lupus Erythematosus (SLE)
Chronic multisystem auto immune disease of connective tissue and blood
Can affect any system
Symptoms can be mild or life threatening
Cause is unknown but has a hereditary component and triggers (ultraviolet light, pregnancy, hormones, infections)
*The Antinuclear Antibody Test (ANA) is often used for diagnosing SLE



Manifestations:
Fever, fatigue, weight loss, anorexia
Butterfly rash over nose, alopecia
Arthritis, myositis
Headache, behavioral changes
Pericarditis, myocarditis, endocarditis
Abdominal pain, nausea, vomiting, colitis
Hepatomegaly, splenomegaly, lymphadenopathy
Anemia, cytopenia
Retinopathy
Raynaud syndrome

Nursing interventions/treatments
Medication administration - corticosteroids, NSAIDs, antimalarial drugs, antihypertensives, aspirin, antibiotics or immunosuppressants
Supportive care








References:

Axton, S. E., & Fugate, T. (2003). Chapter 8: Care of Children with musculoskeletal dysfunction. In M. Connor & Y. Kopperman (Eds.), Pediatric nursing care plans (2nd ed., pp. 187-216). Upper Saddle River, New Jersey: Prentice Hall Pearson Education, Inc..

Axton, S. E., & Fugate, T. (2003). Chapter 9: Care of children with neurological/neuromuscular dysfunction. In M. Connor & Y. Kopperman (Eds.), Pediatric nursing care plans (2nd ed., pp. 217-252). Upper Saddle River, New Jersey: Prentice Hall Pearson Education, Inc..


Wilson, D. (2005). The child with musculoskeletal or articular dysfunction. In M Hockenberry, D. Wilson & M. Winkelstein (Eds.), Wong's essentials of pediatric nursing (7th ed., pp. 1147-1186). St. Louis, Missouri: Elsevier Mosby.

Videos:

JIA 1 located at
http://youtu.be/z5RvDF4SdNM

JIA 2 video located at
https://youtu.be/6Iq8EBAoDAw

SLE located at
http://youtu.be/H_QKeq70sbI

Lupus
http://youtu.be/KM9zxwuZGz4

Lupus on Good Morning America
http://youtu.be/urlwpvPXMW0
References:

Bryant, R. (2009). Chapter 26: The child with hematologic or immunologic dysfunction. In M. J. Hockenberry & D. Wilson (Eds.), Wong's essentials of pediatric nursing (8th ed., pp. 911-948). St. Louis, MO: Mosby Elsevier.

Videos:

The Immune Center
http://youtu.be/IWMJIMzsEMg

The Immune System
http://youtu.be/q5o-APJcCA8

Leukemia
http://youtu.be/CFx6hz-2ots

Leukemia
http://youtu.be/cFlk2bmsJv4

ALL and AML
http://youtu.be/NE5BtHcZZXo

Hodgkins Lymphoma
http://youtu.be/tvIw9BIX0cs

Lymphomas
http://youtu.be/dVFUIhqj1xE

Videos:

HIV and Pregnancy
http://youtu.be/y_0qya2oCNs


HIV
http://youtu.be/54T0KMmwoTs

Blood administration:
http://youtu.be/WJuqiTEc2tk

Blood types
http://youtu.be/oz4Ctau8mC8

HSCT
http://youtu.be/b5UnL9JocLg

Caiden's Story located at
http://youtu.be/o8mj_G3JfsI