Transcript Pediatric Hematologic Disorders and Cancer

Presented by
Marlene Meador RN, MSN, CNE
Hematologic System
Adult
Pedi
 Life cycle of RBC- 120 days
 Life cycle of RBC- 100 days
 Cell production- marrow and
spleen
 RBC’s= 4.1 to 4.9 million/ml
 Hemoglobin=
 Hematocrit=




(neonate)
Cell production- red bone
marrow (infant)
#RBC’s= 5million/ml at birth
Hemoglobin= 17-18 g
Hematocrit= 45-50%
Iron Deficiency Anemia
 Cause
 Signs and symptoms
 Diagnostic tests
 Nursing interventions
 Oral supplements- What significant side
effects does the nurse need to remember?
 Dietary teaching- what specific foods?
What Parents Want to Know:
(p 1243)
 Specific foods: (based on age of child)
 Cream of wheat or iron fortified cereal
 Apricots, prunes, raisins and other dried fruits
 Egg yolks
 Dark green leafy vegetables
 Administration of Iron Supplements:
 Give with vitamin C –rich fluids
 Prevent staining from liquid iron supplements
 Changes in stool patterns
 Avoid mixing supplement with food/drink containing calcium
Sickle Cell disease
 Sickle cell trait- genetic disorders characterized
by production of elongated, crescent shaped
erythrocyte in the place of normal Hbg
 Precipitating factors (p 1248-Home Care of
the Child with Sickly Cell Disease)
 Signs and symptoms
Three Types of Sickle Cell Crisis
Vaso-occlusive
Acute sequestration
Aplastic
Types of Sickle Cell Crisis
 Vaso-occlusive- most common effects
 Pain
 Hand and foot syndrome (dactylitis)
 CVA- hemiplegia, aphasia, seizures, LOC changes, vision
changes, and headache
 Acute chest syndrome- chest pain, fever cough (leading
cause of death in SCD)
 Priapism
 Hepatomegaly
 Hematuria
Types of Sickle Cell Crisis
 Aplastic Crisis:
 Decreased RBC production- S&S malaise,
headache, pallor, lethargy, and fainting
(precipitated by infection)
 Splenic sequestration- life threatening S&S pallor,
irritability, tachycardia, hypovolemic shock
 Hyperhemolytic crisis- (not in text)- RBC’s destroyed
more rapidly than usual (immature cells)
Quick Review:
 What is most common reason for admission to
the ED for a child with SCD?
 What precipitates a sickle cell crisis?
 How does sickling effect the life span of an
RBC?
 what organs experience complications as a
result of chronic sickling crisis?
Diagnosis & Treatment
Cord blood testing if one parent is
known to carry trait
Blood transfusions
 Complications
 Nursing interventions before/during/after
Treatment
Patient/family teaching
Medications
Immunizations- why
important?
Clinical Judgment:
 Why are blood transfusions ordered for
the patient in sickle cell crisis?
 Can a neonate have a diagnosis of sickle
cell disease?
 What ethical issues relate to this
diagnosis?
Hemophilia (p 1291)
 X-linked trait
What factor is missing or defective?
 Who is the carrier, and who is effected by
this disorder?
Diagnosis & Treatment
When does diagnosis most
commonly occur?
What specific laboratory tests and
values?
What are signs & symptoms?
Nursing Care:
 Factor VIII- when should the patient
receive this medication?
 What does the family need to know
about factor VIII?
 Human plasma
 Vasopressin (DDAVP)
Nursing Care cont…
 What is the primary nursing goal for a patient
with hemophilia?
 Prevent or stop bleeding
 What are specific interventions to achieve this
goal?
 Administer Factor VIII
 Apply local pressure for 10-15 minutes
 Elevate the joint and immobilize
 Apply cold compresses
Complications of hemophilia
 Hemarthrosis- assess child for joint pain,
edema, or permanent deformity. Where
most common?
 At risk for hemorrhage
Death
Childhood Cancers
“…communication
promotes understanding
and clarity;
with understanding, fear
diminishes;
in the absence of fear,
hope emerges; and in the
presence of hope, anything
is possible” (Stovall, 1995)
Childhood Cancer
 C- continual unexplained weight loss, fatigue malaise
 H- headaches with vomiting (early morning)
 I- increased edema or pain in joints
 L- lump or mass, persistent lymphadenopathy
 D- development of whitish appearance in pupil of the eye
 R- recurrent or persistent fevers, night sweats
 E- excessive bruising or bleeding
 N- noticeable pallor
Childhood Cancer & Treatment
 Chemotherapy
 Surgery
 Radiation
 Stem Cell Transplantation
 Steroid Therapy
 Biologic Agents
 Complementary and Alternative Medical (CAM)
Chemotherapy: Antinoeplastic agents
 Titrated to specific formula- closely correlated with
cardiac output and blood flow to kidneys and liver
 Highly specialized nurses- The Association of Pediatric
Hematology/Oncology Nurses
 Administration routes:
 Oral
 Intravenously (large bore catheter)
 Intramuscularly
 Subcutaneously
 Intrathecally
Chemotherapy:
side effects/ nursing interventions
Bone Marrow
Suppression
Neutropenia
Anemia
Thrombocytopenia
GI and GU
Nausea/vomiting
Stomatitis
Anorexia
Renal damage
Integumentary
Changes
Hair loss
Hyperpigmentation
of skin
Hypersensitivity to
sunlight
Chemotherapy
Nursing Interventions:
 Protection of the patient:
 Isolation- what specific type?
 Exposure to sun, chemicals, skin irritants
 Nutritional needs:
 Prevent nausea & vomiting
 Types of foods to meet metabolic requirements
 Temperatures/textures/acid-base
 Fluid balance
 Intake and output
 Integumentary
 Self image
Clinical Judgment:
 Why does the nurse increase the amount of water/liquid
intake for a patient on chemotherapy?
 What specific interventions does the nurse need to follow
with relation to the patient’s output?
 Why are stool softeners very important?
 What are the best foods for a patient on chemotherapy?
Why?
 What do parents and family need to understand about
the patient’s psycho-social needs?
Radiation Therapy
 Purpose
 Palliative- prevent growth, reduce tumor size, pain relief
 Eradicate or kill a tumor
 Side effects similar to chemotherapy: Sub-acute & Late
 Somulence syndrome
 Fever
 Irritability
 Ataxia
 Anorexia
 Dysphasia
Surgical treatment for cancer
 Purpose
 Confirm diagnosis
 Debulking or resecting
 Removal of tumor as adjunct to chemotherapy/radiation
 Nursing Interventions
 How does the nurse prepare the patient for surgery?
 What interventions apply specifically to the family?
Hematopoietic Stem Cell and
Bone Marrow Transplantation :
 Conditioning- eradicate disease with high-dose
chemo/radiation therapy
 Infusion- implantation of stem cells or bone marrow
 Increase in patient’s WBC, RBC, and platelets signal success!
 Complications
 GI disturbances
 Graft-Versus-Host Disease (GVHD)

Maintain patient on anti-rejection medications
 Prednisone
 Cyclosporine
 Tacrolimus
Graft-Versus-Host Disease (GVHD)
Potentially lethal immunologic response of donor T cells
against the tissue of the recipient.
Prevention: Careful tissue typing, irradiation of blood
products to inactivate mature T lymphocytes.
 Signs & Symptoms
 rash, malaise, high fever, diarrhea, liver and spleen
enlargement
 Treatment
 Maintain patient on anti-rejection medications



Prednisone
Cyclosporine
Tacrolimus
Complementary /Alternative
Medical Therapies (CAM)
 Risks-vs- benefits
 Qualification of practitioner
 Delay or interfere with conventional
treatment
 Costs
 Contraindications
What signs and symptoms would lead to the
diagnosis of leukemia?
 Fever
 Pallor
 Overt signs of bleeding
 Lethargy or malaise
 Anrexia
 Large joint or bone pain
 Petechiae, frank bleeding
 Enlarged liver or spleen, changes in lymph nodes
 Neurologic changes
Lab values for a diagnosis of
leukemia: examination of CBC with at
least 25% blasts confirm the diagnosis
Normal
 Leukocytes < 10,000
Leukemia
 Leukocytes> 10,000
 Platelets 20-100,000
 Hemoglobin 7-11
Further diagnostic findings:
 Bone marrow aspiration- iliac crest (why this
site?)
 How does the nurse prepare the child/family
for this procedure?
 What are the nurse responsibilities for this
procedure?
Treatment and Plan of Care: (p 1274-1280)
Chemotherapy: three phases
Induction phase
Consolidation
 Delayed
intensification
Remission and maintenance
Nursing Care for a Child
Undergoing Chemotherapy: review
 Myelosupression- protect from injury
 Infection/sepsis (neutropenia)- protect
from infection
 Renal damage
 GI disturbances
 Metabolic emergencies
Intrathecal Medication
 Chemotherapy instilled
into spinal canal
Assess and monitor for
placement of
intrathecal catheter
and assess neuro
checks
Cranial Radiation
Head and neck tumors are more
sensitive to radiation than
chemotherapy.
When would chemotherapy become
an adjunct to radiation therapy?
Tumor Lysis Syndrome:
What causes tumor lysis syndrome?
What are signs and symptoms of this
complications
What nursing interventions apply to
treatment?
Clinical manifestations of
Neuroblastoma (p 1286)
 Smooth, hard, non-tender along sympathetic
nervous system
 Frequent location is abdomen
 Neck and facial edema from vena cava
syndrome
 Increased ICP
 Limp if metastasis to bone
 Pancytopenia
Nursing Management
 Assess by observation and inspection
palpation)
 Document bowel and bladder function
 Record height & weight, observe gait
 Chemotherapy, radiation, surgery
 Teach parents S&S of infection. Why?
(not
Osteosarcoma- most common
primary bone malignancy in
children
 Goal of treatment- remove tumor and prevent spread of
disease
 Biopsy
Chemo
Surgery
Chemo
(radiation=palliative pain control)
 Promote self esteem
 Side effects of chemotherapy
 Amputation of extremity
 Separation from friends and family
Ewing Sarcoma- second most common bone
tumor associated with children
 Pain, soft tissue swelling
 Anorexia, fever, malaise with metastasis
 Diagnosis same as osteosarcoma
 Management
 Chemo
 Surgery (decrease tumor bulk)
 Radiation
Rhabdomyosarcoma
Pathophysiology and Manifestations
 Most common soft tissue malignancy
 Divided by young (<10 yrs) and older
(adolescents) in location
 60% have positive prognosis
 Soft to hard, nontender mass (depends
on location)
 In pelvic tumors, may disrupt organ
function
Diagnosis and Treatment
 CT, BM aspiration and biopsy
 Renal function and liver function tests
 Treated with chemo, surgery and
radiation
Nephroblastoma- Wilm’s Tumor
 Soft renal tumor - one or both kidneys
 (p 1291) Metastasis or seeding spread by
palpation
 Nephrectomy treatment of Wilm’s tumor
Nursing treatment of Wilms’ tumor:
 Pain management
 Frequent reposition
 Noninvasive and pharmacologic pain interventions
 Prevent circulatory overload
 Weigh daily
 I&O, urine for specific gravity
 Prevent infection
 Hand washing
 Protective isolation
 Homecare needs
Retinoblastoma (p1292)
Retinoblastoma- rare malignant tumor
of the neural retina
 “cat’s eye” reflex seen as a white light in the
pupil is the most common “leukocoria”
 May have strabismus of involved eye
 Red painful eye is late symptom
 Staging based on extent of disease
Nursing care of the child/family
with a malignant disease: (p905-915)
 Initial focus on support of family members
 Nurses facilitate the educational process to allay fears
of unknown
 Encourage family members to verbalize fears and
questions
 Postoperative care if indicated
 Community resources (through the discharge planner,
case worker)
Death and Dying:
Understanding of death according to developmental age:
 < 3 years- no understanding/concept of death
 3-5 years- afraid of separation from parents
 5-9 years- understand death is permanent, irreversible
and sad. Concerns for fear of pain, being left alone and
leaving parents and friends.
 Age 10> have adult’s concept of death
Nursing Care and Grief
 Child- encourage child to express feelings,
allow choices, help maintain
independence
 Family- listen, answer questions, provide
information, encourage expression of
feelings and fears
Nursing Care for the Nurse
 Providing physical and psychosocial support for the
patient and family places additional stressors on
the staff and nurses
 Caring for dying children and their families is
emotionally demanding.
 Grief counseling plays an important role for both
family and staff
For questions or concerns please contact
Marlene Meador RN, MSN, CNE
[email protected]
References:
McKinney, James, Murray, & Ashwill.
Maternal- Child Nursing forth ed.(2015).
Saunders.