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Pediatric Hematologic Disorders and Cancer. Presented by Marlene Meador RN, MSN, CNE. Hematologic System. Adult . Pedi. Life cycle of RBC- 120 days Cell production- marrow and spleen RBC’s= 4.1 to 4.9 million/ml Hemoglobin= Hematocrit= . Life cycle of RBC- 100 days (neonate)

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pediatric hematologic disorders and cancer

Pediatric Hematologic Disorders and Cancer

Presented by

Marlene Meador RN, MSN, CNE

hematologic system
Hematologic System

Adult

Pedi

  • Life cycle of RBC- 120 days
  • Cell production- marrow and spleen
  • RBC’s= 4.1 to 4.9 million/ml
  • Hemoglobin=
  • Hematocrit=
  • Life cycle of RBC- 100 days (neonate)
  • Cell production- red bone marrow (infant)
  • #RBC’s= 5million/ml at birth
  • Hemoglobin= 17-18 g
  • Hematocrit= 45-50%
iron deficiency anemia1
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 1281
What Parents Want to Know: (p 1281)
  • 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 disease1
Sickle Cell disease
  • Sickle cell trait- genetic disorders characterized by production of elongated, crescent shaped erythrocyte in the place of normal Hbgp 1282-1283
    • Precipitating factors (p 1284)
    • Signs and symptoms
three forms of sickle cell crisis
Three Forms of Sickle Cell Crisis
  • Vaso-occlusive
  • Acute sequestration
  • Aplastic
types of sickle cell crisis
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 crisis1
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
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
Diagnosis & Treatment
  • Cord blood testing if one parent is known to carry trait
  • Blood transfusions
    • Complications
    • Nursing interventions before/during/after
treatment
Treatment
  • Patient/family teaching
  • Medications
  • Immunizations- why important?
clinical judgment
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
Hemophilia (p 1291)
  • X-linked trait
  • What factor is missing or defective? Factor VIII
  • Who is the carrier, and who is effected by this disorder?
diagnosis treatment1
Diagnosis & Treatment
  • When does diagnosis most commonly occur?
    • What specific laboratory tests and values?
  • What are signs & symptoms?
nursing care
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
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
Complications of hemophilia
  • Hemarthrosis- assess child for joint pain, edema, or permanent deformity. Where most common?
  • At risk for hemorrhage

Death

childhood cancers
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
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
what signs and symptoms would lead to the diagnosis of leukemia
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
slide26
Lab values for a diagnosis of leukemia: examination of CBC with at least 25% blasts confirm the diagnosis

Normal

Leukemia

  • Leukocytes < 10,000
  • Leukocytes> 10,000
  • Platelets 20-100,000
  • Hemoglobin 7-11
further diagnostic findings
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 1313 1321
Treatment and Plan of Care: (p 1313/1321)
  • Chemotherapy: three phases
    • Induction phase
    • Consolidation
      • Delayed intensification
    • Remission and maintenance
nursing plan of care for a child undergoing chemotherapy
Nursing Plan of Care for a Child Undergoing Chemotherapy:
  • Myelosupression- protect from injury
  • Infection/sepsis (neutropenia)- protect from infection
  • Renal damage
  • GI disturbances
  • Metabolic emergencies
intrathecal medication
Intrathecal Medication
  • Chemotherapy instilled into spinal canal

Assess and monitor for placement of intrathecal catheter and assess neuro checks

cranial radiation
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
Tumor Lysis Syndrome:
  • What causes tumor lysis syndrome?
  • What are signs and symptoms of this complications
  • What nursing interventions apply to treatment?
bone marrow and stem cell transplantation
Bone marrow and Stem Cell Transplantation
  • Used to treat leukemia, neuroblastomas and some noncancerous conditions-aplastic anemia
  • Goal to administer a lethal dose to kill the cancer, and resupply the body with stem cells from the child’s own bone marrow, or a compatible donor
develop a plan of care for the child undergoing treatment for leukemia
Develop a plan of care for the child undergoing treatment for leukemia:
  • Risk for injury:
    • Soft tissue/mucous membranes
    • Generalized trauma
  • Risk for infection:
  • Risk for alteration in bowel elimination
  • Risk for GI distress
  • Fluid volume delicate
nephroblastoma wilm s tumor
Nephroblastoma- Wilm’s Tumor
  • Soft renal tumor - one or both kidneys
  • (p 1332) Metastasis or seeding spread by palpation
  • Nephrectomy treatment of Wilm’s tumor
nursing treatment of wilms 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
clinical manifestations of neuroblastoma p 1327
Clinical manifestations of Neuroblastoma(p 1327)
  • 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
Nursing Management
  • Assess by observation and inspection (not palpation)
  • Document bowel and bladder function
  • Record height & weight, observe gait
  • Chemotherapy, radiation, surgery
  • Teach parents S&S of infection. Why?
osteosarcoma most common primary bone malignancy in children
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
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
pathophysiology and manifestations
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
Diagnosis and Treatment
  • CT, BM aspiration and biopsy
  • Renal function and liver function tests
  • Treated with chemo, surgery and radiation
retinoblastoma rare malignant tumor of the neural retina
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
Nursing care of the child/family with a malignant disease:
  • 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
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
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
slide51
For questions or concerns please contact

Marlene Meador RN, MSN, CNE

Email [email protected]

References:

McKinney, James, Murray, & Ashwill. Maternal- Child Nursing Third ed (2009). Saunders.

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