Transcript Catastrophic Diseases

Oncology
Elisa A. Mancuso RNC-NIC, MS, FNS
Professor of Nursing
White Blood Cells
(Leukocytes)
White Blood Cells (WBC)
• Formed in bone marrow and lymphatic
tissue
• Destroy foreign cells via
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phagocytosis and antibody production
Granulocytes
• Phagocytic cells
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produced in the bone marrow
Granulocytes
Neutrophils
• fight bacteria
Eosinophils
• fight parasites
• responds to allergens
• influences the inflammatory process
Basophils
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contain histamine
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activate the inflammatory response
Agranulocytes
Participate in inflammatory and immune reactions
Monocytes (macrophages)
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First line of defense in inflammatory process
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Phagocytize large cells & necrotic tissue
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Important for chronic infections
Lymphocytes
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Blast cells in bone marrow, spleen, thymus and
other lymph glands and tissue
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Responsible for immune protection
T Lymphocytes
• T-cells
– Made in thymus
– Cell mediated immunity (RT an antigen)
• B cells
– Humoral immunity
• “memory” cells that produce antibodies to specific antigens
• Natural killer cells
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kill certain type of tumor cells and viruses
Acute Lymphocytic Leukemia (ALL)
Cancer is the 2nd cause of death <15 years
• Leukemia
– malignant disease of bone marrow and lymph system
• ALL
– most common form of childhood cancer
– Peak onset 3-5 years of age
– 80% of cases of acute leukemia in childhood
– Etiology;
• Genetic abnormalities
– Philadelphia chromosome (↓ prognosis)
– Trisomy 21 = 20 x ↑ Risk
• Chernoble - Nuclear Radiation exposure
• Alkylating agents or certain chemical agents
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Virus trigger of oncogene
ALL Pathophysiology
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Abnormal, poorly differentiated blast cells
DNA mutation of an immature white blood cell
Causes the cell to multiply uncontrollably
Infiltrate bone marrow & ↑ vascular RE organs
• Hepatomegaly
• Splenomegaly
• Lymphadenopathy
Malignant blast cells replace the functioning WBC’s in
bone marrow causing:
Anemia (↓↓ RBCs)
Neutropenia (↓↓ WBCs)
Thrombocytopenia (↓↓ Plts)
Signs and symptoms
1st sign: Infections that linger > 2 weeks (↓WBC)
• Fever
• Chills
• Anorexia
• Weight loss (↑ metabolic demands of CA cells)
• Bone & joint pain (Marrow expansion)
• Abdominal pain (Hepatosplenomegaly)
• Pallor, fatigue, lethargy (↓ RBCs)
• Ecchymosis, petechiae, GI bleeding (↓ Plts)
• CNS = ↑ICP ( HA, Vomiting & Irritability)
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Late stage RT
Brain protected by blood barrier.
ALL Diagnosis
• Bone Marrow Aspiration @ iliac spine
• >25% blast cells = + diagnosis
• Lumbar puncture (LP)
– √ any CNS involvement
• PET, CT & MRI Scans
Good PrognosisPoor Prognosis
• WBC <10,000/mm3
WBC >50,000/mm3
• Age 1-10
Age <1 or >10
• Female
Male
• Early + response
Poor treatment response
• No CNS involvement
CNS involvement
Chemotherapy Meds
Corticosteroids
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Anti-inflammatory
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↓ and kill lymphoblastic cells (↓ WBC)
Prednisone - 40 mg/m2 PO QD
Dexamethasone – 2.5 -10mg/m2/day IM/IV
÷ q6-8H
Side Effects:
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Hyperglycemia
Na & Fluid retention = wt gain, puffy moon face
Peptic ulcers, mood changes
Delayed growth pattern
Chemotherapy Meds
Enzymes
• ↓ levels of amino acid (asparagine) →
• ↓↓ tumor growth
L-Asparaginase (Elspar) 10,000 u/m2/day IM
2x/week
• Side Effects:
– Allergic rxn = chills, fever & rash
– Jaundice √ LFTs
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Respiratory distress & ↓ BP
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N & V, DM
Chemotherapy Meds
Plant Alkaloids
• Anti-neoplastic = Inhibits cell division
Vincristine (Oncovin) 1.5 mg/m2 IV
• Side Effects
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Peripheral neuropathy
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severe constipation
↓ bowel innervation
Stomatitis, N & V,
Anemia
Thrombocytopenia
Chemotherapy Meds
Alkylating Agents
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Interferes with cell growth
Cyclophosphamide (cytoxan, CTX) 60-250 mg/m2/day
Ifosfamide (Ifos) 1.2gm/m2/day
Cisplatin (Platinol) 30-70 mg/m2/day
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Side Effects
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Alopecia
Pulmonary fibrosis
Hemorrhagic cystitis
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(caused by chemical irritation of drugs)
Leukopenia
Anorexia, N & V
Chemotherapy Meds
Antibiotics
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Documented bacterial infections
Actinomycin D (dactinomysin, ACT-D) 2.5 mg/m2/wk
Bleomycin (Blenoxane) 10-20 U/m2/wk
Doxorubicin (Adriamycin) 20mg/m2/wk
Side Effects
– Cardiotoxic!
– Red urine (Not hematuria)
– Alopecia
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N & V and stomatitis
CNS Prophylactic
Antimetabolites
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Inhibits folic acid reductase = inhibits DNA
synthesis and cellular replication. Inhibits
replication of neoplastic cells
Methotrexate (MTX, Amethopterin) 20mg/m2/week PO
IV or Intrathecal
Mercaptopurine (6-MP) 75mg/m2/day IV
Cytarabine (Ara-C, Cytosar-U) 100-200mg/m2/day IV
5-Fluorouracil (5-FU) 7-12mg/kg IV
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Side Effects
Leukopenia, chills/fever, vomiting
Red rash, Alopecia
↓ Folic Acid metabolism
Hyperurecemia
Other Agents
Allopurinol (zyloprim)
• Inhibits production of uric acid.
• CA cell destruction = ↑ uric acid levels
– accumulates in tubules → renal calculi
• Side Effects
– ↑ SGOT & SGPT = hepatotoxicity
– Blocks metabolism of 6-MP = 6-MP toxicity
• Need 1/3 -1/4 normal dose of 6-MP
Other Agents
Mesna (mesnex)
• Ifosamide detoxifying agent.
• Binds to toxic metabolites.
• Prevents hemorrhagic cystitis
• Use with alkylating agents
– Cytoxan, Ifos, Platinol
Radiation
• Prophylactic in high risk patients
• Minimize CNS involvement
• Side Effects after 7-10 days
– GI
• dysphagia, stomatitis, N & V, diarrhea
– Skin
• Erythema, desquamination, alopecia
– Myleosuppression ↓ RBCs ↓ WBCs↓ Plts
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• Fatigue, Infection, Bruising/Bleeding
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Pneumonitis
↑ RR ↑HR Dyspnea & dry cough
Transfusions
Used to correct specific deficiencies
• PRBC
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Epoetin (Epogen)/Procrit
↑ RBC in 2-6 weeks
Platelets
Granulocyte Colony Stimulating Factors-GCSF
Filgrastin (Neupogen)
↑Neutrophils (ANC)
Stimulate dev of new white blood cells 10-14 days
SE: Bone pain, fever, malaise & HA
Whole blood transfusions
Rarely used since ↑ risk of fluid overload
Bone Marrow Transplant
• Replaces pt own bone marrow.
– Need 500 cc -1 Liter
– Takes 1-3 weeks for marrow to self
produce
• Autologous
– uses own bone marrow if in remission
• Allogenic (Donor)
– √ Compatible = match 6 HLA antigens
– Prevent Graft vs. Host Disease (GVHD)
Bone Marrow Transplant
• 1st give ↑↑ dose chemo and radiation (total body)
– Rids body of CA cells
– Suppresses immune system to prevent rejection
• Strict reverse isolation
• Neutropenic Precautions
– No fresh flowers, fruit, veggies
– Monitor visitors √ immunization status
• Monitor s/s of infection
– √ Temp, CBC, Activity
– √ Absolute Neutrophil Count (ANC) <500
– ↑ risk for overwhelming infection
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ANC = WBC times the % of neutrophils
Nursing Interventions
Prevent Infections
• Live vaccines are contraindicated.
– No MMR or Varicella
• Inactivated vaccines
– Wait @ least 6 months after chemo
for appropriate immune response
• ↑↑ predisposition to resistant organisms
• Broad spectrum prophylactic antibiotics
Nursing Interventions
Nutrition
• ↑↑ Hydration ↑ Protein ↑Caloric Intake
• Bland , easily digestible diet
• Encourage nutritious foods
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Allow pt to choose
↑ Pt participation with meal planning
No acidic juices or spicy foods
Nursing Interventions
Mouth Care
• Frequent cleansing
– Magic Mouthwash
(Malox/Benadryl/HO)
• Cotton swabs not toothbrush for ↓ Plts
• Stomatitis
– Chloroseptic spray
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Viscous Lidocaine
Nursing Interventions
Skin Care
• High risk for rectal ulcers from diarrhea
• Keep area clean and dry & OTA
• Turn & Position
• Sheepskin or Air mattress
• √ SE from meds & radiation
– ↑risk for skin breakdown & irritation
Nursing Interventions
Nausea and Vomiting
• Small frequent feeding
• ↑ PO intake via ices, jello, favorite fluids
• √ weight √ I and O’s
Antiemetics
• Ondanesetron (Zofran) [Aloxy]
– Blocks 5-HT3 site in brain
• Dronabinol (Marinol)
– THC synthetic active component of marijuana
Nursing Interventions
Peripheral Neuropathy
• ↓ bowel innervation → constipation
• Foot drop, tremors, jaw pain
• Weakness & numbness of extremities
Maintain safe environment
• Assist with ambulation
• Sneakers, hand rails & walkers
Nursing Interventions
Alopecia
• Prepare child & family ( temp condition)
• Allow kids to cut their own hair!
• Obtain wig before hair is lost
• Scarfs or hats
• Re-growth 3-6months
– Darker, thicker & curlier
Nursing Interventions
Hemorrhagic Cystitis
• Chemical irritation to the bladder
• ↑ Fluid intake (1.5 x daily amount)
• ↑ Voiding frequency
Medication
• Mesna
– ↓ Urotoxicity of Ifos & Cisplatin
Nursing Interventions
Pain relief
• Evaluate non-verbal and verbal cues
• Note cultural differences & accommodate needs
• Position
– H2O beds, bean bag chairs, stuffed animals
• Change environment
– ↓ Sensory stimulation (lights, noise, activity)
• Relaxation techniques
– Massages, rocking, guided imagery, distraction,
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Humor!
Pain Meds
• Give ATC to maintain steady state
– Give meds before pain is severe
– Adhere to scheduled med time
– Kids have ↑ BMR
• Need more frequent dosing not ↑ dose
• Tylenol [10-15 mg/kg/dose q 4-6 H]
– Maximum 90 mg/kg/dose (hepatotoxic)
• Tylenol with codeine [Codeine 0.5 -1 mg/kg/dose]
– Tylenol No. 1 (Codeine 7.5 mg & Acetaminophen 300 mg)
– Tylenol No. 2 (Codeine 15 mg & Acetaminophen 300mg)
• Percocet [oxycodone 0.1 mg/kg/dose]
– [Oxycodone 5 mg & Acetaminophen 325 mg]
• Tylox
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– [Oxycodone 5 mg & Acetaminophen 500 mg]
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Vicodin
[Hydrocodon 5mg & Acetaminophen 500 mg]
Pain Meds
NSAIDS
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Ibuprophen (Motrin) 40 mg/kg/day
• SE: Skin rash, abdominal cramps, N, dizziness
Opioids
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Hydromorphone (Dilaudid) 0.4 -1mg/kg q 4-6 H
• Quick onset of action 15 minutes
• Shorter duration than MSO4
• ↑ potency 1 mg Dilaudid = 4 mg MSO4
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Morphine SO4 (Roxanol) 0.025 -2.6 mg/kg/H
• SE: Sedation, ↓ RR ↓BP Constipation Flushed
face
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Methadone (Dolophine) 0.2 mg/kg q 6-8 H
• Long ½ life 24 -36 H
• SE: Confusion, Sedation, ↓BP Constipation
Nursing Interventions
Emotional support
• Guidance with honest answers
• Education
– Serious signs & symptoms, adverse drug
effects
– When to seek medical attention
• Establish good plan for FU care
• Encourage verbalizations or fears/ concerns
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Reassure pt will be comfortable
Neuroblastoma
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Most common solid malignant tumor in kids
↑ risk < 2 years old.
75% before child is 5 years old.
Tumors begin as embryonic cells
– Develop into the adrenal medulla and
sympathetic nervous system (ganglia).
• Majority a non-familial, sporadic pattern
• Silent Tumor
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70% Dx after metastasis
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Poor Prognosis
Clinical Manifestations
• Primary sites:
– Abdomen & Pelvis, Chest, Head & Neck
• Retroperitoneal region (65%)
– Adrenal medulla - ↑↑ E/NE release
• ↑ HR ↑ BP ↑ Bounding Pulses +3,
diaphoresis
– Abdominal mass-bloating/constipation
• Anorexia
– Kidney compression
• Polyuria → Polydipsia
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Spinal chord compression
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Pain & Paresthesia
Clinical Manifestations
• Mediastinum (15%)
– Compresses trachea & bronchi
• Tracheal deviation
• Persistent cough, Dyspnea & SOB
• Stridor & Chest pain
– Lymphadenopathy
• Cervical, supraclavicular & groin
– Neck/facial edema
– ↑ ↑ HA in AM & ↑ ↑ HC
– Supraorbital ecchymosis (Raccoon eyes)
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Infection
Clinical Manifestations
• Systemic
– Weight loss
• RT Anorexia RT ↓↓ Bowel function
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Irritability
Fatigue
Myoclonus ataxia syndrome
Anemia
Febrile, ↑ HR ↑ BP
Changes in urination, bowel elimination
Diagnosis
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CT: Chest, Abdomen & Pelvis
Bone Scan IVP Abdominal Sonogram
Bone Marrow aspiration and biopsy
CBC: √Anemia √Thrombocytopenia
24 H urine collection of VMA
Vanillylmandelic Acid = ↑ DA & NE
Treatment
• Surgery if tumor is localized
• Radiation
– ↓ size of tumor a & p surgery
• Chemotherapy
– Diffuse & advanced disease
– Cytoxin, Vincristine & Cisplatin
– 3F8 immunotherapy
Wilm’s Tumor
(Nephroblastoma)
• Common type of abdominal tumor
– ↑ Incidence with Hypospadias & Cryptorchidism
• 80% diagnosed at <5years
– ↑ risk @ 3 years
• 90% survival rate
– ↑ Cure rate with early diagnosis
• Encapsulated Tumor
– Arises from renal parenchyma
– Rapidly growing tumor
• Favors left kidney and usually unilateral
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10% of cases have both kidneys involved
Clinical signs
• Non-tender mid-line abdominal mass
• Flank pain
• ↑↑ BP
– RT kidney & adrenal compression & Renin
• Anemia RT Hematuria
• Rare Mets → Lung & Bone
Diagnosis ASAP!
• Abdomen & Chest
– CT scan, X-Ray & Ultrasound
• IVP
• Renal function tests
• CBC with differential
• Bone scan
Therapy
• 1st Place sign on wall:
– DO NOT PALPATE ABDOMEN!
• Radiation and chemo a & p surgery
• Surgery
– Radical Nephrectomy
– whole kidney and adrenal
– Large Y autopsy-like incision:
• Examine entire abdominal cavity
Nursing Interventions
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Prepare family for scar
Prepare for chemo and radiation
Abdominal surgical care
I&O’s
Monitor bleeding
No contact sports
Watch for any kidney infections or
⇊ function
Osteogenic Sarcoma
Osteosarcoma
• Arises from bone forming osteoblasts
and bone digesting osteoclasts
• Most common bone tumor in children
– 10 – 15 years, can go up to 25 years
• Femur, tibia or shoulder near growth
plate
– ↑ Frequency during growth spurt
Signs and Symptoms
• Gradual onset
Insidious, intermittent local joint pain
• Palpable mass – (Bone Biopsy)
• Pain more intense with activity
• Limp & change in gait, ↓ ROM
• High serum alkaline PO4, and LDH
• Pathological fractures
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Starburst formation on x-ray
Therapy
• R/O Metastasis
– Bone Scan, CT, MRI & Lung Scan
• Surgery
– Amputation 3” proximal to tumor or joint
– Limb salvage
• Chemotherapy
– ↑ Methotrexate, Adriamycin,
– Cisplatin, Ifos
Pre-op
• Exercise to strengthen upper arms
• Prepare patient for extensive PT
• Emotional support
– allow pt to grieve for limb loss
– Focus on what the pt can do
• Support Group:
– ACS-Osteo Support Group; Camping &
youth directed activities
– www.candlelighters.org
Post-op
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√ signs of hemorrhage q1H x 24 then q4H
Tourniquet at bedside (arterial bleed)
Venous oozing reinforce dressing
Pressure dressing
– Mold and shape for prosthesis
• Phantom limb pain
– Stimulation of nerve endings
– Burning, aching, tingling & cramping.
– It is real!
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Pain meds & Elavil
Post-op
• Position
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1st 24 H - Elevate stump with pillow
>24 H No pillow below knee
Position prone to prevent hip flexion
No external rotation or abduction
• Place prosthesis immediately after
surgery.
– Fosters early function and adjustment
Ewing’s Sarcoma
• 2nd most common malignant bone tumor
• Highly invasive into bone marrow.
• Infiltrates soft tissue around the bone
– Pain with soft tissue mass
• Sites:
– Femur, tibia, fibula, ulnar, ribs and vertebrae
• 5 – 25 years of age (peaks @ age 10-20)
• Prognosis depends on degree of infiltration
Therapy
• Chemotherapy
– Shrinks tumor & control mets
– VAC
– Vincristine, Actinomycin & Cytoxan
• Intensive Total Body Radiation
– (6-8 weeks)
• No Surgery – tumor is too invasive
Nursing Interventions
• Anticipatory guidance RT Therapy SE
• Radiation burns
– Erythema, blisters, pain
– Hyperpigmentation
• Loose clothing, protective cream,
• Protect against sunlight
• Avoid sudden changes in temp
– No ice/heat packs
Non-Hodgkin’s Lymphoma
• Malignancy of lymphatic system
– Proliferation of T or B lymphocytes
– Lymphoblastic Lymphoma 30%
• 75% Medialstinal mass, Pleural effusion
Lymphadenopathy
– Large B Cell Lymphoma 20%
• Lymphadenopathy & Invades other tissues
• Associated with Epstein Barr virus
– Small,non-cleaved type 50%
• Burkitts Lymphoma-90% (intrabdominal mass)
• Generalized and very aggressive
• ↑ Incidence with age
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Males 2x > females
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↑ Incidence with AIDS
Sign and Symptoms
• Acute onset & progression
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– Pain & swelling in chest or abdomen
– Lymphadenopathy in neck, underarm or groin
Fever, malaise & Night Sweats
Mediastinal mass = SOB ↑ RR ↑ Cough
CNS = HA & vomiting (no nausea)
Superior Vena Cava Syndrome (SVCS)
– Obstruction of SVC
• Edema of face, neck & trunk
• Bone Marrow Infiltration
– Petechia, Bruising, Bleeding & Bone Pain
Diagnosis
• Biopsy from tumor site
• Staging (I – IV)
– Bone marrow & Lumbar puncture
– CT: Chest, Abdomen & Pelvis
– PET Scans (total body) ↑ activity & uptake
– Gallium Scans- Cardiac
• Tumor Lysis Syndrome (WBC > 50,000)
– Release of purines from destroyed lymphoblasts
– ↑ Uric acid levels →Renal Failure
– Therapy
• IV NaHCO3 keep urine pH > 7-8
• Allopurinol (Zyloprim) ↑ uric acid secretion
Treatment
• Chemotherapy
– Multi Agent aggressive R-CHOP protocol
– R= Retuxin (monoclonal AB therapy)
– CHOP
• Cytoxin, Adriamycin, Oncovin
(Vincristin) & Prednisone
• Radiation
– 20 - 40 treatments @ tumor site
Nursing Interventions
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Chemotherapy & Radiation SE
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Aranesp, Procrit, PRBC Transfusions
Neupogen & Neutropenic Precautions
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No fresh fruit or Vegetables
↓ Exposure to infections
Immunizations
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Flu, PPCV, Gamma Globulins,
Acyclovir
Leuprolide (Lupron) suppress ovaries