Pediatric hematologic disorders and cancer
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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

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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)

  • Cell production- red bone marrow (infant)

  • #RBC’s= 5million/ml at birth

  • Hemoglobin= 17-18 g

  • Hematocrit= 45-50%


Iron Deficiency Anemia


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)

  • 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 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

  • 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


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 & 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


The Child with Cancer


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


Acute Lymphatic Leukemia- ALL


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

Leukemia

  • Leukocytes < 10,000

  • 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 1313/1321)

  • Chemotherapy: three phases

    • Induction phase

    • Consolidation

      • Delayed intensification

    • Remission and maintenance


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

  • 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?


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:

  • 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


Other Cancers of Childhood


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:

  • 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)

  • 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 (not palpation)

  • Document bowel and bladder function

  • Record height & weight, observe gait

  • Chemotherapy, radiation, surgery

  • Teach parents S&S of infection. Why?


Osteosarcoma


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


Retinoblastoma (p1333)


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:

  • 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


For questions or concerns please contact

Marlene Meador RN, MSN, CNE

Email mmeador@austincc.edu

References:

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


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