Care of the child with a hematologic disorder includes assessment, nursing diagnosis, planning, interventions, and evaluation. There are a number of general concepts related to the nursing process that may be applied to hematologic dysfunction in children.
Development of a plan of care will depend on which component of the blood is altered. A decrease in hemo- globin will necessitate evaluation of oxygen-carrying capacity and effects of hypoxia on the tissues. A reduc- tion in platelet production will lead the nurse to evaluate for prolonged bleeding, hemorrhage, and shock. An ele- vation in WBCs would require an evaluation for infection.
Remember Shaun, the 10-month-old with the laceration and prolonged bleeding?
what additional health history and physical examination assessment information should you obtain?
Assessment
Signs of changes in the hematologic system are often insidious and overlooked. Skin color changes such as pallor, bruising, and lushing are often the irst signs that a problem is developing. Changes in mental status such as lethargy can indicate a decrease in Hgb and a decreased amount of oxygen being delivered to the brain. Nursing assessment must include a thorough approach.
Health History
Elicit the birth and maternal history, noting low birthweight or gestational diabetes and ascertain- ing whether vitamin K was given after birth. The past medical history might be signiicant for recent illnesses that may contribute to a change in blood cell distribution. Determine the child’s sleep/wake
• Unusual bruising
• Excessive bleeding or dificulty stopping bleeding
• Pain: location, onset, duration, quality, and relieving factors
Physical Examination
A child’s general appearance gives great insight into his or her health and can indicate problems such as mal- nutrition or lead poisoning. Physical examination of the patterns and bowel elimination patterns, which may
be affected by alterations in circulating blood volume or changes in oxygenation. Explore the family history for inherited disorders such as hemophilia, sickle cell disease, and thalassemia. Evaluate the child’s typical diet for nutritional deicits. Determine risk for lead exposure based on the use of a standard questionnaire. When elic- iting the history of the present illness, inquire about:
• Fatigue or malaise
• Pallor of the skin
COMMON MEDICAL TREATMENTS 47.1
Treatment Explanation Indications Nursing Implications Blood product
transfusion
Intravenous administration of whole blood, packed red blood cells (PRBCs), platelets, or plasma
PRBCs: severe ane- mia, thalassemia, sickle cell disease Whole blood:
acute hemor- rhage or trauma Fresh-frozen
plasma: hemo- philia
Follow institution’s transfusion protocol.
Double-check blood type and product label with a second nurse. Use only leukode- pleted, CMV-negative blood products in the child with a hemoglobinopathy.
Monitor vital signs and assess child frequently to detect adverse reaction to blood transfusion.
If adverse reaction is suspected, imme- diately discontinue transfusion, run normal saline IV, reassess the child, and notify the physician.
Some children require premedication with diphenhydramine and/or acetamino- phen before receiving blood products.
Supplemental
oxygen Administration of oxygen via mask,
cannula, or blow-by Hypoxia associated with sickle cell crisis or severe anemia
Frequently monitor work of breathing, oxygen saturation via pulse oximetry, cardiopulmonary status, and level of consciousness.
Splenectomy Surgical removal of the spleen Life-threatening or recurrent splenic sequestration of sickle cell disease;
thalassemia
Provide immunization against the follow- ing organisms, because they place the child at risk for overwhelming infection:
Streptococcus pneumoniae, Neisseria meningitidis, and Haemophilus influenzae type B.
Monitor carefully for signs of infection.
Administer prophylactic antibiotics.
Instruct child or teen to wear medical alert bracelet.
Teach families to seek medical treatment at irst sign of infection or fever.
Hematopoietic stem cell trans- plantation
Bone marrow transplant: transfer of healthy bone marrow into a child with disease; the transplanted cells can then develop into functional cells.
Stem cell transplant: Peripheral stem cells are removed from the donor via apheresis, or stem cells are retrieved from the umbilical cord and placenta. The stem cells are then transplanted into the recipient.
Sickle cell disease, aplastic anemias, thalassemia
Maintain medical asepsis and protective isolation to prevent infection.
Monitor closely for graft-versus-host disease.
Provide meticulous oral care.
Avoid taking rectal temperatures and inserting suppositories.
Encourage appropriate nutrition.
Administer immunosuppressive medications as ordered.
CMV, cytomegalovirus.
DRUG GUIDE 47.1 COMMON DRUGS FOR HEMATOLOGIC DISORDERS
Medication Actions/Indications Nursing Implications Iron supplements
(ferrous sulfate, ferrous fumarate)
Supplemental iron in deicient child Iron-deiciency anemia
Dosage is based on milligrams of elemental iron.
Give with vitamin C–containing foods to increase absorption
Do not administer with milk or milk products.
May color stools and urine black.
Liquid can stain the teeth; mix with a small amount of juice; drinking with straw decreases tooth staining.
May cause constipation; increase iber and luid intake.
Deferasirox (Exjade) Binds with iron, which is removed in the feces
Iron toxicity (as in children chronically transfused)
Oral agent, should be taken at the same time daily, on an empty stomach.
Monitor hearing and vision.
Deferoxamine (Desferal)
Binds with iron, which is removed via the kidneys
Iron toxicity (as in children chronically transfused)
Rotate subcutaneous injection sites to decrease local reactions.
Apply corticosteroid cream to irritation.
Factor (VIII or IX) replacement
Replaces deicient clotting factors Hemophilia
Use ilter needle to draw up medication.
Administer IV when bleeding occurs.
Penicillin VK Kills susceptible bacteria
Prophylaxis of infection in asplenia
Determine whether penicillin allergy is present.
Monitor renal and hematologic function during prolonged use.
Folic acid Replaces the vitamin
Folic acid deiciency; questionable use with sickle cell anemia
Administer without regard to meals.
Monitor hematologic function.
Hydroxyurea Stimulates the development of hemo- globin F in sickle cell anemia
Monitor for mild GI discomfort, modest neutropenia, hyperpigmentation of the skin and nails.
Intravenous immune globulin (IVIG) (multiple manufacturers)
Provides exogenous IgG antibodies Idiopathic thrombocytopenic purpura
Do not mix with IV medications or with other IV luids.
Do not give IM or SQ.
Monitor vital signs and watch for adverse reactions fre- quently during infusion. Child may require antipyretic or antihistamine to prevent chills and fever during infusion.
Have epinephrine available during infusion.
Chelating agents:
dimercaprol (BAL), edentate calcium disodium (CaEDTA), suc- cimer (Chemet)
Remove lead from soft tissues and bone, allowing for its excretion via the renal system
Used for blood lead levels >45 mcg/dL
Monitor intake and output closely to ensure adequacy of renal system.
Encourage adequate oral hydration or provide IV hydration if required.
Follow lead levels as prescribed.
Ensure that lead is being removed from the child’s home.
GI, gastrointestinal; IM, intramuscularly; SQ, subcutaneously.
Adapted from Taketokmo, C. K., Hodding, J. H., & Kraus, D. M. (2011). Pediatric and neonatal dosage handbook (18th ed.).
Hudson, OH: Lexi-comp.
• Hematocrit (Hct): an indirect measure of red blood cells (number and volume)
• RBC indices
• Mean corpuscular volume (MCV): average size of the RBC
• Mean corpuscular hemoglobin (MCH): a calculated value of the oxygen-carrying capacity of the Hgb in the RBCs
• Mean corpuscular hemoglobin concentration (MCHC): a calculated value that relects the con- centration of Hgb inside the RBC
• Red cell distribution width (RDW): a calculated value that is a measure of the width of RBCs
• WBC count: actual count of the number of WBCs in a volume of blood
• Platelet count: number of platelets per blood volume
• Mean platelet volume (MPV): a measurement of the size of the platelets
Tables 47.1 and 47.2 provide age-related values for the CBC and leukocyte count. When evaluating the CBC, the nurse must take into account the presenting clinical picture of the child. For instance, the RBC count may be truly elevated (erythrocytosis or polycythe- mia) in certain diseases or in the case of dehydration from diarrhea or burns. When anemia is present, the RBC count is low. When the MCV is elevated, the RBCs are larger than normal (macrocytic). When the MCV is decreased, the RBCs are smaller than normal (micro- cytic). A decrease in the MCHC means that the Hgb is diluted in the cell and less of the red color is pres- ent (hypochromic). When the Hgb concentration is increased in the RBC, then the pigmentation (red color) is increased (hyperchromic). Alterations in these red blood cell indices assist the physician or nurse practi- tioner with diagnosis.
WBCs are the body’s defense against infection or injury. The speciic types of WBCs are discussed in Chapter 37. Platelets are necessary for clot formation, and if changes occur, problems may develop. Elevations in platelet levels can indicate an increase in clotting, while decreases can put the child at risk for increased bleeding. Decreases can result if the platelets are being used up when bleeding is present, if an inherited disor- der is present, or if the spleen holds them, as in hyper- splenism. Platelets are larger when they are new; thus, an elevation in the mean platelet volume indicates that an increased number of platelets are being produced in the bone marrow.
Common Laboratory and Diagnostic Tests 47.1 explains the most commonly used laboratory and diag- nostic tests for children with hematologic disorders. The tests can assist the physician or nurse practitioner in diagnosing the disorder and/or be used as guidelines in determining ongoing treatment. Laboratory or nonnurs- ing personnel obtain some of the tests, while the nurse child with a hematologic disorder includes inspection
and observation, palpation, and auscultation.
INSPECTION AND OBSERVATION
Note the child’s color and general appearance, includ- ing thin or emaciated appearance or obesity. Measure weight and height (or length) and plot on standardized growth charts. Observe the nail beds, palms, and soles for pallor. Evaluate the ingertips for clubbing, which occurs with chronic hypoxemia. Document the loca- tion and extent of bruises, petechiae, or purpura. Count the respiratory rate and observe for work of breathing.
Obtain a pulse oximeter reading to determine oxygen saturation of tissues. Note conjunctival color as well as color and moisture of oral mucosa. Determine uri- nary output, which may be altered with decreases in circulatory blood volume or inadequate oxygenation.
Note the child’s responsiveness to stimuli, movement of extremities, and quality of gait.
AUSCULTATION
Auscultate breath sounds, noting adequacy of air movement and depth of respiration. Note adventitious sounds or absence of breath sounds (which would occur in an area of the lung illed with blood). Auscul- tate heart sounds, listening closely for murmurs (which can develop with changes in blood viscosity and vol- ume). Note the rate and rhythm of the heart tones. Aus- cultate bowel sounds, noting presence and normalcy.
PALPATION
Measure blood pressure, which may change with alterations in blood volume. Palpate the peripheral pulses for strength and equality. Determine capillary reill time, which may be prolonged when the circulat- ing blood volume is decreased. Carefully palpate the abdomen for tenderness, hepatomegaly, or spleno- megaly (increased spleen size). Note temperature of the skin. Determine elasticity of skin, noting decreased turgor. Palpate the joints for tenderness. Assess range of motion.
Laboratory and Diagnostic Testing
The nurse must understand the main elements of the complete blood count (CBC; hemogram) to recognize critical values and intervene as appropriate. The com- ponents of the CBC are:
• RBC count: the actual number of counted RBCs in a certain volume of blood
• Hemoglobin (Hgb): measure of the protein made up of heme (iron surrounded by protoporphyrin) and globin, α- and β-polypeptide chains, primarily re- sponsible for the transport of nutrients and oxygen to the tissues
Chapter 47Nursing Care of the Child With a Hematologic Disorder 1711 Age
WBC (×103/mm3)
RBC (×106/mm3)
Hgb (g/dL)
Hct (%)
MCV (fL)
MCH (pg/cell)
MCHC (g/dL)
Platelets
(×103/mm3) RDW (%)
MPV (fL)
Birth–2 weeks 9.0–30.0 4.1–6.1 14.5–24.5 44–54 98–112 34–40 33–37 150–450 — —
2–8 weeks 5.0–21.0 4.0–6.0 12.5–20.5 39–59 98–112 30–36 32–36 — — —
2–6 months 5.0–19.0 3.8–5.6 10.7–17.3 35–49 83–97 27–33 31–35 — — —
6 months–1 year 5.0–19.0 3.8–5.2 9.9–14.5 29–43 73–87 24–30 32–36 — — —
1–6 years 5.0–19.0 3.9–5.3 9.5–14.1 30–40 70–84 23–29 31–35 — — —
6–16 years 4.8–10.8 4.0–5.2 10.3–14.9 32–42 73–87 24–30 32–36 — — —
16–18 years 4.8–10.8 4.2–5.4 11.1–15.7 34–44 75–89 25–31 32–36 — — —
>18 years (males) 5.0–10.0 4.5–5.5 14.0–17.4 42–52 84–96 28–34 32–36 140–400 11.5–14.5 7.4–10.4
>18 years (females)
5.0–10.0 4.0–5.0 12.0–16.0 36–48 84–96 28–34 32–36 140–400 11.5–14.5 7.4–10.4
Adapted from Fischbach, F. T. (2008). A manual of laboratory and diagnostic tests (8th ed.). Philadelphia, PA: Lippincott Williams & Wilkins.
TABLE 47.1 NORMAL HEMOGRAM VALUES
the nursing diagnoses. Nursing Care Plan 47.1 may be used as a guide in planning nursing care for the child with a hematologic disorder. Refer to Chapter 36 for additional information related to pain management.
Children’s responses to hematologic disorders and their treatments will vary, and nursing care should be indi- vidualized based on the child’s and family’s responses to illness. Other conditions may contribute to these nursing diagnoses and must also be considered when prioritizing care. Additional information about nursing management will be included later in the chapter as it relates to speciic disorders.
might obtain others. In either instance the nurse should be familiar with how the tests are obtained, what they are used for, and normal versus abnormal results. This knowledge will also be necessary when providing child and family education related to the testing.
Nursing Diagnoses, Goals, Interventions, and Evaluation
Upon completion of a thorough assessment, the nurse might identify several nursing diagnoses, including:
• Fatigue
• Pain
• Impaired physical mobility
• Ineffective health maintenance
• Anxiety
• Ineffective family coping
TABLE 47.2 NORMAL DIFFERENTIAL FOR LEUKOCYTES (WHITE BLOOD CELL DIFFERENTIAL)
Age
Bands/
Stab (%)
Segs/Polys (%)
Eos (%)
Basos (%)
Lymphs (%)
Monos (%)
Birth–1 week 10–18 32–62 0–2 0–1 26–36 0–6
1–2 weeks 8–16 19–49 0–4 0 38–46 0–9
2–4 weeks 7–15 14–34 0–3 0 43–53 0–9
4–8 weeks 7–13 15–35 0–3 0–1 41–71 0–7
2–6 months 5–11 15–35 0–3 0–1 42–72 0–6
6 months–1 year 6–12 13–33 0–3 0 46–76 0–5
1–6 years 5–11 13–33 0–3 0 46–76 0–5
6–16 years 5–11 32–54 0–3 0–1 27–57 0–5
16–18 years 5–11 34–64 0–3 0–1 25–45 0–5
>18 years 3–6 50–62 0–3 0–1 25–40 3–7
Adapted from Fischbach, F. T. (2008). A manual of laboratory and diagnostic tests (8th ed.). Philadelphia, PA: Lippincott Williams & Wilkins.
You have finished assessing Shaun and your findings include the following. The health history revealed he bled with all four of the teeth he has cut. Upon physical examination numerous bruises are noted. Based on the as- sessment findings, what would your top three nursing diagnoses be for Shaun?
Based on your top three nursing diagno- ses for Shaun, describe appropriate nursing interventions.