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
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
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
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.
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
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.
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.
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
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