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Pediatric Hematologic Disorders and Cancer

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Title: Pediatric Hematologic Disorders and Cancer


1
Pediatric Hematologic Disorders and Cancer
  • Presented by
  • Marlene Meador RN, MSN, CNE

2
Hematologic System
  • Adult
  • Pedi
  • Life cycle of RBC- 120 days
  • Cell production- marrow and spleen
  • RBCs 4.1 to 4.9 million/ml
  • Hemoglobin
  • Hematocrit
  • Life cycle of RBC- 100 days (neonate)
  • Cell production- red bone marrow (infant)
  • RBCs 5million/ml at birth
  • Hemoglobin 17-18 g
  • Hematocrit 45-50

3
Iron Deficiency Anemia
4
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?

5
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

6
Sickle Cell Disease
7
Sickle Cell disease
  • Sickle cell trait- genetic disorders
    characterized by production of elongated,
    crescent shaped erythrocyte in the place of
    normal Hbg p 1282-1283
  • Precipitating factors (p 1284)
  • Signs and symptoms

8
Three Forms of Sickle Cell Crisis
  • Vaso-occlusive
  • Acute sequestration
  • Aplastic

9
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

10
Types of Sickle Cell Crisis
  • Aplastic Crisis
  • Decreased RBC production- SS malaise, headache,
    pallor, lethargy, and fainting (precipitated by
    infection)
  • Splenic sequestration- life threatening SS
    pallor, irritability, tachycardia, hypovolemic
    shock
  • Hyperhemolytic crisis- (not in text)- RBCs
    destroyed more rapidly than usual (immature
    cells)

11
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?

12
Diagnosis Treatment
  • Cord blood testing if one parent is known to
    carry trait
  • Blood transfusions
  • Complications
  • Nursing interventions before/during/after

13
Treatment
  • Patient/family teaching
  • Medications
  • Immunizations- why important?

14
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?

15
Hemophilia
16
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?

17
Diagnosis Treatment
  • When does diagnosis most commonly occur?
  • What specific laboratory tests and values?
  • What are signs symptoms?

18
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)

19
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

20
Complications of hemophilia
  • Hemarthrosis- assess child for joint pain, edema,
    or permanent deformity. Where most common?
  • At risk for hemorrhage
  • Death

21
The Child with Cancer
22
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)
23
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

24
Acute Lymphatic Leukemia- ALL
25
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

26
Lab values for a diagnosis of leukemia
examination of CBC with at least 25 blasts
confirm the diagnosis
  • Normal
  • Leukemia
  • Leukocytes lt 10,000
  • Leukocytesgt 10,000
  • Platelets 20-100,000
  • Hemoglobin 7-11

27
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?

28
Treatment and Plan of Care (p 1313/1321)
  • Chemotherapy three phases
  • Induction phase
  • Consolidation
  • Delayed intensification
  • Remission and maintenance

29
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

30
Intrathecal Medication
  • Chemotherapy instilled into spinal canal
  • Assess and monitor for placement of intrathecal
    catheter and assess neuro checks

31
Cranial Radiation
  • Head and neck tumors are more sensitive to
    radiation than chemotherapy.
  • When would chemotherapy become an adjunct to
    radiation therapy?

32
Tumor Lysis Syndrome
  • What causes tumor lysis syndrome?
  • What are signs and symptoms of this complications
  • What nursing interventions apply to treatment?

33
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 childs own bone marrow, or a compatible
    donor

34
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

35
Other Cancers of Childhood
36
Nephroblastoma- Wilms Tumor
  • Soft renal tumor - one or both kidneys
  • (p 1332) Metastasis or seeding spread by
    palpation
  • Nephrectomy treatment of Wilms tumor

37
Nursing treatment of Wilms tumor
  • Pain management
  • Frequent reposition
  • Noninvasive and pharmacologic pain interventions
  • Prevent circulatory overload
  • Weigh daily
  • IO, urine for specific gravity
  • Prevent infection
  • Hand washing
  • Protective isolation
  • Homecare needs

38
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

39
Nursing Management
  • Assess by observation and inspection (not
    palpation)
  • Document bowel and bladder function
  • Record height weight, observe gait
  • Chemotherapy, radiation, surgery
  • Teach parents SS of infection. Why?

40
Osteosarcoma
41
Osteosarcoma- most common primary bone malignancy
in children
  • Goal of treatment- remove tumor and prevent
    spread of disease
  • Biopsy Chemo Surgery Chemo
    (radiationpalliative pain control)
  • Promote self esteem
  • Side effects of chemotherapy
  • Amputation of extremity
  • Separation from friends and family

42
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

43
Rhabdomyosarcoma
44
Pathophysiology and Manifestations
  • Most common soft tissue malignancy
  • Divided by young (lt10 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

45
Diagnosis and Treatment
  • CT, BM aspiration and biopsy
  • Renal function and liver function tests
  • Treated with chemo, surgery and radiation

46
Retinoblastoma (p1333)
47
Retinoblastoma- rare malignant tumor of the
neural retina
  • cats 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

48
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)

49
Death and Dying
  • Understanding of death according to developmental
    age
  • lt 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 10gt have adults concept of death

50
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

51
  • For questions or concerns please contact
  • Marlene Meador RN, MSN, CNE
  • Email mmeador_at_austincc.edu
  • References
  • McKinney, James, Murray, Ashwill. Maternal-
    Child Nursing Third ed (2009). Saunders.
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