HEMATOLOGIC SYSTEM PEDIATRICS

HEMATOLOGIC SYSTEM

LEUKEMIA – the most frequent type of childhood cancer

Brain tumors – 2nd

Etiology: 

       1.    Environmental

2. Viruses
3. Familial/genetic
4. Host factors

  STAGES OF TREATMENT

1. INDUCTION

•       Goal: to remove bulk of tumor

•       Methods:  surgery, radiotherapy, chemotherapy, BM transplant

•       Effects: often the most intensive phase

•       Side effects are potentially life threatening

2. CONSOLIDATION

•       Goal: to eliminated any remaining malignant cells

•       Methods:  chemo/radio

•       Side effects will still be evident

3. MAINTENANCE

•       Goal:  to keep the child disease free

•       Chemotherapy

•       This phase may last several years

4. OBSERVATION

•       Goal:  to monitor the child at intervals for evidence of recurrent

            disease and complications of treatment

•       Method:  treatment is complete

•       Child may continue in this stage indefinitely

5. LATE EFFECTS OF TREATMENT

•       Impaired growth and development

•       CNS damage

•       Impaired pubertal development

•       Development of secondary malignancy

•       Psychologic problems related to living with a life threatening disease and complex treatment regimen

•        

NURSING INTERVENTION

1. Help child cope with intrusive procedures

•       Provide information geared to developmental level and emotional

            readiness

•       Explain what is going to happen, why it is necessary, how it will feel

•       Allow child to handle to handle and manipulate equipments

•       Allow child some control in situations

            -  positioning, selecting injection site

2. Support child and parents

•       Maintain frequent clinical conferences to keep all informed

•       Always tell the truth

•       Acknowledge feelings and encourage child/family to express them

•       Provide contact with another parent or support group

3. Minimize side effects of treatment

1. Skin breakdown

•       Keep clean and dry; wash with warm water; no soaps or creams

•       Do not wash off radiation marks

•       Avoid all topical agents with alcohol

•       Do not use heating pads or hot water bottle

2. Bone marrow suppression

•       Provide frequent rest periods

•       Avoid crowds

•       Evaluate any potential site of infection

•       Monitor temperature

•       Avoid use of aspirin

•       Select activities that are physically safe

3. Nausea and vomiting

•       Administer antiemetic at least half an hour before chemotherapy

•       Eat light meal prior to administration of therapy

•       Administer IVF if needed

4. Alopecia

•       Reduce trauma of hair loss

•       Buy wig before hair falls out

•       Discuss various head coverings

•       Avoid exposing head to sunlight

4. Nutrition deficits

•       Establish baseline

•       Provide high calorie, high protein

5.      Developmental delay

•       Facilitate return to school as soon as possible

•       Discuss limit setting, discipline

LEUKEMIA       

•         most common form of childhood cancer

•         peak 3-5 years

•         proliferation of abnormal wbc that do not mature beyond the blast phase

•         blast cells – infiltrate other organs – liver, spleen, lymph tissue

Types:

1. Acute Lymphocytic leukemia (ALL)

•       80-85%

•       Acute

•       75% chance of surviving

2. Acute nonlymphocytic leukemia

•       Includes granulocytic and monocytic types

•       60-80% will obtain remission

•       30-40% cure rate

Assessment:

1. anemia, weakness, pallor, dyspnea
2. Bleeding, petecchiae, spontaneous bleeding, ecchymoses
3. Infection, fever, malaise
4. Enlarged lymph node
5. Enlarged spleen and liver
6. Bone pain

Management:

1. diagnosis:  blood studies, BMA
2. Treatment stages

1. Induction
2. CNS prophylaxis
3. Maintenance

Nursing Intervention:

1. Provide care for the child receiving chemo and radiotherapy
2. Provide support for the family/child
3. Support child during painful procedures

1. Use distraction, guided imagery
2. Allow the child to retain as much control as possible
3. Administer sedation prior to procedure as ordered

Sickle Cell Disease

•        hemoglobin A is partly or completely replaced by  abnormal sickle hemoglobin (HgbS)

•        HgbS sensitive to changes in the oxygen content of the red blood cell

•        Risk factors: African American

•        Insufficient oxygen causes the cells to assume a sickle shape and the cells become rigid and clumped together, obstructing capillary blood flow

•        The sickling response – reversible under adequate oxygenation

•        Sickle cell crises – vaso-occlusive crisis, splenic sequestration, aplastic crisis

Assessment:

1. Vaso-occlusive crisis

•      Most common type of crisis

•      Caused by stasis of blood with clumping of the

            cells in microcirculation, ischemia and infraction

•      Fever, pain and tissue engorgement

2. Splenic sequestration

•      Pooling of blood in the spleen

•      Profound anemia, hypovolemia, shock

3. Aplastic crisis

•      Caused by the diminished production and increased destruction of RBCs, triggered by viral infection or the depletion of folic acid

•      Profound anemia and pallor

Implementation:

•        Administer oxygen and blood transfusions

•        administer analgesics

•        maintain adequate hydration and blood flow with IV normal saline as prescribed and with oral

            fluids

•        Assist the child to assume a comfortable position so that the child keeps the extremities extended

            to bed no more than 30 degrees

•        avoid putting strain on painful joints

•        encourage consumption of a high calorie, high protein diet with folic acid supplementation

•        administer antibiotics as prescribed

•        Monitor for signs of increasing anemia and shock (pallor, vital sign changes)

IRON DEFICIENCY ANEMIA

•        Iron stores are depleted, resulting in a decreased supply of iron for the manufacture for the

            manufacture of hemoglobin in RBCs

•        Results from blood loss, increased metabolic demands, syndromes of GI malabsorption, dietary

            inadequacy

Assessment:

•        pallor

•        weakness and fatigue

•        irritability

Implementation:

•        Increase the oral intake of iron

•        Instruct the child and parents in food choices that are high in iron

•        Administer iron supplements as prescribed

•        Liquid iron prep stains – stains teeth; with straw

•        side effects of iron therapy

APLASTIC ANEMIA

•        a deficiency of circulating erythrocytes resulting from the arrested development of RBCs within the bone marrow

•        causes:  exposure to toxic agents, viruses, infection, autoimmune disorders, allergic states

•        definitive diagnosis:  BMA

•        Therapeutic management:  blood transfusions, splenectomy, corticosteroids, immunosuppressive therapy, bone marrow transplantation

Assessment

•        Pancytopenia

•        Petecchiae, purpura, bleeding, pallor, weakness,  tachycardia and fatigue

Implementation:

•        Blood transfusion

•        corticosteroids and immunosuppresives

•        splenectomy

•        bone marrow transplant

•        Medic Alert bracelet

HEMOPHILIA

•        x linked recessive trait

•        Hemophilia A – deficiency of Factor VIII

•        Hemophilia B – deficiency of factor IX

•        Males inherit hemophilia from their mothers and females inherit the carrier status from their

            fathers

Assessment:

•        prolonged bleeding after minor injury

1. At birth after cutting cord
2. Following circumcision
3. Following IM immunization
4. Increase bruising as child learns to crawl and walk

•        abnormal bleeding in response to trauma

•        joint bleeding – pain, tenderness, swelling limited range of motion

•        tendency to bruise easily

•        prolonged PTT

•        normal BT, PT,  platelet count

Implementation:

•        prepare to administer Factor VIII concentrate /cryoprecipitate

1. Thaw slowly
2. Gently rotate bottle
3. Infuse immediately; deteriorates at room temperature

•        monitor for bleeding

•        monitor for joint pain; immobilize the affected extremity if joint pain occurs

•        assess neurological status ( child at risk for IC bleed)

•        monitor urine for hematuria

•        Control bleeding by immobilization, elevation,  application of ice; apply pressure (15 mins)

            for superficial bleeding

•        instruct parents regarding activities of the child

•        avoidance of contact sports

BETA THALASSEMIA MAJOR

•        Cooley’s anemia

•        autosomal recessive disorder

•        characterized by reduced production of one of the globin chains in the synthesis of hemoglobin

•        incidence highest in Mediterranean descent

Assessment:

•        severe anemia

•        pallor

•        failure to thrive

•        hepatosplenomegaly

•        microcytic, hypochromic RBCs

Implementation

•        Instruct the administration of folic acid (Vit B9)

•        administer blood transfusion as prescribed

•        Monitor for iron overload

•        iron overload – chelation therapy with deferoxamine

•        genetic counseling

IDIOPATHIC THROMBOCYTOPENIC PURPURA

•        increased destruction of platelets with resultant platelet count less than 100,000/mm3

•        characterized by petechiae and ecchymoses of skin

•        exact mechanism unknown

•        often preceded by a viral illness

•        spleen not enlarge

Assessment:

•        petechiae: spider web appearance of bleeding under the skin due to small size of platelets

•       ecchymosis

•        blood in any body secretions, bleeding from mucus membranes, nosebleeds

•        diagnostic test:  platelet count decreases, anemia

Management:

•        steroids and immunosuppressives

•        platelet transfusion

•        surgery; splenectomy

Nursing Intervention

•        control bleeding

1. Administer platelet transfusion as ordered
2. Apply pressure to bleeding sites as needed
3. Position bleeding part above heart level if possible

•      Prevent bruising

•      Protect from infection

•      Measure normal circumference of extremities for baseline

•      Administer meds orally, rectally or IV rather than IM; hold pressure on site for 5 mins

•      Avoid aspirin

•      Provide client teaching and discharge planning

1. Pad crib and playpen
2. Provide soft toys
3. Provide protective headgear during toddlerhood
4. Use soft toothbrush
5. Avoid contact sports