Chapter 21 ATI 2019 - Hematology PDF

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CHAPTER 21 UNIT 2

SYSTEM DISORDERS

CLIENT EDUCATION

SECTION: CARDIOVASCULAR AND HEMATOLOGIC DISORDERS ●

CHAPTER 21

Hematologic Disorders

Blood disorders that can affect children include epistaxis, iron deficiency anemia, sickle cell anemia, and hemophilia.

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Iron deficiency anemia ●

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Epistaxis ●

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For recurrences, sit up and slightly forward so blood OZP^YZ_ʮZbOZbY_SP_S]ZL_LYONL`^PNZ`RSTYR Bleeding usually stops within 10 min.

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Short, isolated occurrences of epistaxis (nosebleeds) are common in childhood. Although epistaxis is rarely an emergency, it causes anxiety for the child and the child’s caregivers.

4]ZYOPʭNTPYNdLYPXTLT^_SPXZ^_[]PaLWPY_ anemia worldwide. Adolescents are at risk due to poor diet, rapid growth, menses, strenuous activities, and obesity. The production of hemoglobin (Hgb) requires iron. Iron OPʭNTPYNdbTWW]P^`W_TYOPN]PL^PO3RMWPaPW^ 4]ZYOPʭNTPYNdLYPXTL`^`LWWd]P^`W_^Q]ZXLY inadequate dietary supply of iron, and is the most preventable mineral disturbance.

ASSESSMENT RISK FACTORS

ASSESSMENT

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RISK FACTORS ●

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Trauma (picking or rubbing the nose) can cause mucous membranes in the nose, which are vascular and fragile, to tear and bleed. Low humidity, allergic rhinitis, upper respiratory infection, blunt injury, or a foreign body in the nose can precipitate a nosebleed. 8POTNL_TZY^_SL_LʬPN_NWZ__TYRQLN_Z]^NLY increase bleeding. Epistaxis can be the result of underlying diseases (von Willebrand disease, hemophilia, idiopathic thrombocytopenia purpura, leukemia).

PHYSICAL ASSESSMENT FINDINGS ● ●

Active bleeding from nose Restlessness and agitation

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Infants: GERD, pyloric stenosis Older Child: GI polyps, colitis, Adolescents: Menorrhagia

EXPECTED FINDINGS ● ● ● ● ● ●

PATIENT-CENTERED CARE NURSING CARE ● ●

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Maintain a calm demeanor with the child and family. Have the child sit up with the head tilted slightly forward to prevent aspiration of blood. Apply pressure to the lower nose with the thumb and QZ]PʭYRP]QZ]L_WPL^_XTY If needed, pack cotton or tissue into the side of the nose that is bleeding. Encourage the child to breathe through their mouth while pressure is being applied to their nose to control the bleeding. Apply ice across the bridge of the nose if bleeding continues.

Premature birth resulting in decreased iron stores Excessive intake of cows’ milk in toddlers ◯ Milk is not a good source of iron. ◯ Milk takes the place of iron-rich solid foods. Malabsorption disorders Poor dietary intake of iron Increased iron requirements (blood loss)

Tachycardia Pallor -]T__WP^[ZZY^SL[POʭYRP]YLTW^ Fatigue, irritability, and muscle weakness Systolic heart murmur Cravings for non-nutritive substances (ice, dirt, paper)

LABORATORY TESTS CBC: Decreased RBC count, Hgb, and Hct Hgb levels: Vary with age RBC indices: Decreased, indicating microcytic/ hypochromic RBCs ● Mean corpuscular volume: Average size of RBC ● Mean corpuscular Hgb: Average weight of RBC ● Mean corpuscular hemoglobin concentration: Amount of Hgb relative to size of cell Reticulocyte count: Can be decreased (indicates bone marrow production of RBCs) Total iron binding capacity: Elevated Transferrin: 10% indicative of anemia Stool analysis: Guaiac test

RN NURSING CARE OF CHILDREN

CHAPTER 21 HEMATOLOGIC DISORDERS

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PATIENT-CENTERED CARE NURSING CARE ●

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Provide iron supplements for preterm and low-birth-weight infants by the age of 2 months. Provide iron supplements to full term infants by the age of 4 to 6 months. =PNZXXPYOT]ZYQZ]_TʭPOQZ]X`WLQZ]TYQLY_^bSPY solids are introduced Modify the infant’s diet to include high iron, and vitamin C. Monitor formula intake in infants. ◯ Limit formula intake to 32 oz (960 mL) per day. ◯ Encourage intake of iron-rich foods. ◯ ;]ZaTOPT]ZYQZ]_TʭPONP]PLWbSPY^ZWTOQZZO^ are introduced ◯ Allow frequent rest periods. If packed RBCs are required, follow protocols for administration.

MEDICATIONS Iron supplements NURSING ACTIONS ●

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Give 1 hr before or 2 hr after milk, tea, or antacid to prevent decreased absorption. Gastrointestinal (GI) upset (diarrhea, constipation, nausea) is common at the start of therapy. These will decrease over time. If tolerated, administer iron supplements on an empty stomach. Give with meals and start with reduced dose and gradually increase if GI distress occurs. Give with vitamin C to increase absorption. Use a straw with liquid preparation to prevent staining of teeth. Use a Z-track into deep muscle for parenteral injections. Do not massage after injection.

CLIENT EDUCATION ●

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Expect stools to turn a tarry green color if dose is adequate. Brush teeth after oral dose to minimize or prevent staining.

SEVERE ANEMIA THERAPY IV Administration Ferrous Sulfate ● ●

Very painful infusion Requires close monitoring of the child during administration.

Blood Transfusion PRBC preferred

>`[[WPXPY_LWZcdRPY

CLIENT EDUCATION ●

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Diarrhea, constipation, or nausea can occur at the ^_L]_ZQ_SP]L[dM`__SP^PLOaP]^PPʬPN_^L]P`^`LWWd self-limiting. Understand appropriate iron administration. 4YN]PL^PʭMP]LYOʮ`TO^TQNZY^_T[L_TZYOPaPWZ[^ To prevent toxicity, store no more than 1 month’s supply in a child-proof bottle out of reach of children. Allow adequate time for the child to rest. The length of treatment will be determined by the child’s response to the treatment. If Hgb levels are not increased after 1 month of therapy, further evaluation is warranted. Return for follow-up laboratory tests to determine the PʬPN_TaPYP^^ZQ_]PL_XPY_ Schedule universal anemia screens at 12 months of age.

Dietary sources of iron ● INFANTS:4]ZYQZ]_TʭPONP]PLW^LYOQZ]X`WLZ]PcNW`^TaP breastfeeding ● OLDER CHILDREN: Dried beans and lentils; peanut M`__P]&R]PPYWPLQdaPRP_LMWP^&T]ZYQZ]_TʭPOM]PLO^LYO ʮZ`]&[Z`W_]d&LYO]POXPL_

COMPLICATIONS Developmental delay NURSING ACTIONS ● ● ●

Assess level of functioning. Improve nutritional intake. Refer to appropriate developmental services.

CLIENT EDUCATION: Provide support to the family.

Sickle cell anemia Sickle cell disease (SCD) is a group of diseases in which abnormal sickle hemoglobin S (HbS) replaces normal adult hemoglobin (Hgb A). Sickle cell anemia (SCA) is the homozygous and most common form of SCD. ● Manifestations and complications of SCA are the result of RBC sickling, which leads to increased blood aT^NZ^T_dZM^_]`N_TZYZQMWZZOʮZbLYO_T^^`PSd[ZcTL Manifestations of SCA are not usually apparent until later in infancy due to the presence of fetal Hgb. ● Tissue hypoxia causes tissue ischemia, which results in pain. ● Increased destruction of RBCs occurs. Sickle cell trait: Child has the genes but is asymptomatic. Sickle cell crisis is the acute exacerbation of SCA.

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CHAPTER 21 HEMATOLOGIC DISORDERS

CONTENT MASTERY SERIES

ASSESSMENT RISK FACTORS ● ●

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SCD is an autosomal recessive genetic disorder. >.,[]TXL]TWdLʬPN_^,Q]TNLY,XP]TNLY^:_SP]QZ]X^ ZQ>./NLYLʬPN_TYOTaTO`LW^ZQ8POT_P]]LYPLY4YOTLY or Middle Eastern descent. Children who have the sickle cell trait do not manifest _SPOT^PL^PM`_NLY[L^^_SP_]LT__Z_SPT]Zʬ^[]TYR

EXPECTED FINDINGS ● ● ● ● ● ● ● ●

Family history of sickle cell anemia or sickle cell trait Reports of pain Shortness of breath, fatigue Pallor, pale mucous membranes Jaundice Hands and feet cool to touch Dizziness Headache

Vaso-occlusive crisis (painful episode) Acute (related to dehydration and decreased oxygen) ● Severe pain, usually in bones, joints, and abdomen ● Swollen joints, hands, and feet ● Abdominal pain ● Hematuria ● Obstructive jaundice ● Visual disturbances Chronic ● Increased risk of respiratory infections and osteomyelitis ● Retinal detachment and blindness ● Systolic murmurs ● Renal failure and enuresis ● Liver cirrhosis; hepatomegaly ● Seizures ● Skeletal deformities; shoulder or hip avascular necrosis

LABORATORY TESTS Screening for SCA in newborns is mandatory in all 50 US states and territories. CBC to detect anemia. Sickle-turbidity screening tool detects the presence of 3M>M`_bTWWYZ_OTʬP]PY_TL_P_SP_]LT_Q]ZX_SPOT^PL^P Hemoglobin electrophoresis separates the various forms ZQ3RMLYOT^_SPOPʭYT_TaPOTLRYZ^T^ZQ^TNVWPNPWWLYPXTL

Sickle-cell crisis Hgb: decreased WBC count: elevated Bilirubin and reticulocyte levels: elevated Peripheral blood smear reveals sickled cells

DIAGNOSTIC PROCEDURES Transcranial Doppler (TCD) test ● @^PO_ZL^^P^^TY_]LN]LYTLWaL^N`WL]ʮZbLYOOP_PN__SP risk for cerebrovascular accident (CVA). ● A TCD is performed annually on children ages 2 to 16 years who have SCD.

PATIENT-CENTERED CARE NURSING CARE ● ● ●

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Sequestration ●

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Excessive pooling of blood primarily in the spleen (splenomegaly), and sometimes in the liver (hepatomegaly) Reduced circulating blood volume results in hypovolemia and can progress to shock Hypovolemic shock: irritability, tachycardia, pallor, decreased urinary output, tachypnea, cool extremities, thready pulse, hypotension

Aplastic crisis ●

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Extreme anemia as a result of a temporary decreased RBC production Typically triggered by an infection with a virus

Hyperhemolytic crisis Increased rate of RBC destruction leading to anemia, jaundice, and/or reticulocytosis

RN NURSING CARE OF CHILDREN

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Promote rest to decrease oxygen consumption. Administer oxygen as prescribed if hypoxia is present. Provide intense hydration therapy while maintaining ʮ`TOLYOPWPN_]ZWd_PMLWLYNP ◯ Monitor I&O. ◯ 2TaPZ]LWʮ`TO^ ◯ ,OXTYT^_P]4Aʮ`TO^bT_SPWPN_]ZWd_P]P[WLNPXPY_ ◯ Caution with potassium replacement. Administer blood products, usually packed RBCs, and exchange transfusions per facility protocol. Observe for manifestations of hypervolemia and transfusion reaction. Treat and prevent infection. ◯ Administer antibiotics. ◯ Perform frequent hand hygiene. ◯ Give oral prophylactic penicillin. ◯ Administer pneumococcal conjugate vaccine, meningococcal vaccine, and 3LPXZ[STW`^TYʮ`PYeLP type B vaccine. Monitor and report laboratory results

Pain management ● ●

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Use an interprofessional approach. Treat mild to moderate pain with acetaminophen or ibuprofen. Manage severe pain with opioid analgesics. Apply comfort measures (warm packs to painful joints). Schedule administration of analgesics to prevent pain.

CHAPTER 21 HEMATOLOGIC DISORDERS

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MEDICATIONS

COMPLICATIONS

Opioids

Stroke

Codeine, morphine sulfate, oxycodone, hydromorphone, and methadone provide analgesia for pain management.

Sickled cells can occlude blood vessels.

NURSING ACTIONS

Assess and report manifestations. ● Seizures ● Abnormal behavior ● Weakness of, or inability to move an extremity ● Slurred speech ● Visual changes ● Vomiting ● Severe headache

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Administer orally (immediate- or sustained-release) or IV. Administer on a regular schedule to maintain control, or prevent pain if possible. Use patient-controlled analgesia if appropriate.

CLIENT EDUCATION ● ●

Avoid activities that require mental alertness. Analgesics can be necessary in high doses, and addiction is rare.

ANTINEOPLASTICS Hydroxyurea not FDA approved for SCA but used widely to increase oxygenation to the cells.

NURSING ACTIONS

CLIENT EDUCATION: Blood transfusions are usually performed monthly to prevent a repeat stroke.

Acute chest syndrome ● ● ●

NURSING ACTIONS ● ●

Administer PO Monitor nutrition intake

CLIENT EDUCATION: Antineoplastics can cause anorexia

THERAPEUTIC PROCEDURES Exchange transfusions: Replaces the sickled blood cells with normal blood cells. Hematopoietic stem cell transplant: Permanent solution for SCD. High risk of neurologic complications.

NURSING ACTIONS Assess and report manifestations. ● Chest, back, or abdominal pain ● Fever of 38.5° C (101.3° F) or higher ● Cough ● Tachypnea ● Dyspnea, wheezing ● Retractions ● Decreased oxygen saturations

CLIENT EDUCATION ●

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Provide emotional support, and refer to social services if appropriate. Observe for manifestations of crisis and infection and notify the provider promptly. Promote rest and provide adequate nutrition for the child. Practice good hand hygiene and avoid individuals who have colds/infections/viruses. ,OSP]P_Zʮ`TOTY_LVP]P\`T]PXPY_^TPSZbXLYd MZ__WP^Z]RWL^^P^ZQʮ`TO^SZ`WOMPNZY^`XPOOLTWd_Z prevent dehydration. Consider genetic counseling. Maintain up-to-date immunizations. BPL]LXPOTNLWTOPY_TʭNL_TZYb]T^_MLYOZ]XPOTNLW TOPY_TʭNL_TZY_LR^ Attend school regularly and avoid contact sports.

CHAPTER 21 HEMATOLOGIC DISORDERS

Can be life-threatening Common in adolescents Increased risk for pneumonia due to decreased oxygen to lung tissue

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May need blood transfusion Take antibiotics as prescribed

Infections ● Risk for infections (streptococcus pneumoniae, 3 TYʮ`PYeLP ● Spleen decrease in size ● Increased risk for septicemia

Other complications ● ● ●

Kidney scarring Visual acuity decreased Priapism (males)

CONTENT MASTERY SERIES

Hemophilia Hemophilia is a group of bleeding disorders characterized MdOTʯN`W_dNZY_]ZWWTYRMWPPOTYR/PʭNTPYNTP^TY_SP clotting factors. ● Bleeding time is extended due to lack of a factor required for blood to clot. Bleeding is internal or external. ● Bleeding tendencies are sometimes recognized during infancy following circumcision, but might not become apparent until the infant becomes more active and prone to injuries during the toddler years. ● Hemophilia varies in severity based on the percentage of clotting factor a child’s body contains. For example, a child who has mild hemophilia can have up to 40% of the normal factor VIII in his body, while a child who has severe hemophilia has very little factor VIII. ● Both hemophilia A and B are X-linked recessive disorders.

Hemophilia A ● ● ●

/PʭNTPYNdZQQLN_Z]A444 Referred to as classic hemophilia Accounts for 80% of cases

Hemophilia B ● ●

/PʭNTPYNdZQQLN_Z]4C Referred to as Christmas disease

DIAGNOSTIC PROCEDURES DNA testing: Detects classic hemophilia trait in females

PATIENT-CENTERED CARE NURSING CARE Management of bleeding in the hospital ● Administer injections via the subcutaneous route instead of the intramuscular route whenever possible. ● Avoid unnecessary skin punctures and use surgical aseptic technique. ● APYT[`YN_`]P^L]P[]PQP]]POZaP]ʭYRP]Z]SPPW^_TNV^QZ] blood sampling. ● 8ZYT_Z]`]TYP^_ZZWLYOYL^ZRL^_]TNʮ`TOQZ] occult blood. ● Do not administer aspirin or any products that contain aspirin. ● Acetaminophen is an acceptable substitute for aspirin. ● Control localized bleeding. ◯ Administer factor replacement. ◯ :M^P]aPQZ]LOaP]^PPʬPN_^bSTNSTYNW`OPSPLOLNSP ʮ`^STYRWZb^ZOT`XLYOLW_P]L_TZY^TYSPL]_]L_P and blood pressure. ◯ Encourage the child to rest and immobilize the LʬPN_POUZTY_^ ◯ 0WPaL_PLYOL[[WdTNP_Z_SPLʬPN_POUZTY_^

Von Willebrand

MEDICATIONS

Inherited lack of the von willebrand factor protein characterized by the inability of the platelets to aggregate

1-deamino-8-d-arginine vasopressin (DDAVP)

ASSESSMENT EXPECTED FINDINGS Episodes of bleeding, excessive bleeding, reports of joint [LTYLYO^_TʬYP^^TX[LT]POXZMTWT_dPL^dM]`T^TYRLYO activity intolerance

PHYSICAL ASSESSMENT FINDINGS ●

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Active bleeding, which includes bleeding gums, epistaxis, hematuria, and/or tarry stools Hematomas and/or bruising, even with minor injuries 3PXL]_S]Z^T^L^PaTOPYNPOMdUZTY_[LTY^_TʬYP^^ warmth, swelling, redness, loss of range of motion, and deformities Headache, slurred speech, and a decreased level of consciousness

LABORATORY TESTS ● ● ●

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Prolonged partial thromboplastin time (aPTT) 1LN_Z]^[PNTʭNL^^Ld^_ZOP_P]XTYPOPʭNTPYNd Platelets and prothrombin time are within expected reference ranges Whole blood clotting time is within expected range or prolonged

RN NURSING CARE OF CHILDREN

DDAVP is a synthetic form of vasopressin that increases plasma factor VIII (antihemophilic factor). ● 0ʬPN_TaPQZ]XTWOM`_YZ_^PaP]PSPXZ[STWTL ● 9Z_PʬPN_TaPQZ]SPXZ[STWTL-bSTNSTYaZWaP^LQLN_Z] 4COPʭNTPYNd

NURSING ACTIONS: Can be given prior to dental or surgical procedures

Factor VIII, products that contain factor VIII, pooled plasma, and recombinant products Used to prevent and treat hemorrhage

NURSING ACTIONS: Administer by IV infusion. CLIENT EDUCATION ● ●

Treatment can require numerous doses. ;P]TZOTNLOXTYT^_]L_TZYSL^[]ZaPYPʬPN_TaPQZ] preventing bleeding complications.

Corticosteroids Used to treat hematuria, acute episodes of hemarthrosis, and chronic synovitis

NURSING ACTIONS: Monitor for infection and bleeding. CLIENT EDUCATION: Maintain good hand hygiene and avoid individuals with colds/infection/viruses.

CHAPTER 21 HEMATOLOGIC DISORDERS

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E-aminocaproic acid (EACA, amicar)

COMPLICATIONS

Inhibits clot destruction

Uncontrolled bleeding

9ZY^_P]ZTOLWLY_TTYʮLXXL_Z]dLRPY_^

Intracranial hemorrhage, airway obstruction from bleeding in mouth, neck, or chest

Used with caution to treat chronic synovitis.

NURSING ACTIONS: Monitor for infection. CLIENT EDUCATION ●

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Administer cautiously due to potential inhibition of platelet function. Take the medication with food.

NURSING ACTIONS ● ● ●

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INTERPROFESSIONAL CARE

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An interprofessional approach includes the pediatrician, hematologist, orthopedist, nurse, nurse practitioner, physical therapist, school nurse, and social worker.

CLIENT EDUCATION ●

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Prevent bleeding at home. ◯ Environment should be made as safe as possible to prevent injury. ◯ Provide a safe home and a play environment. ◯ Set activity restrictions to avoid injury. Acceptable activities include low-contact sports (bowling, ʭ^STYR^bTXXTYRRZWQBSTWP[L]_TNT[L_TYRTY_SP^P activities, children should wear protective equipment. ◯ Use soft-bristled toothbrushes. Perform regular exercise and physical therapy after active bleeding is controlled. Maintain up-to-date immunizations. BPL]XPOTNLWTOPY_TʭNL_TZY Observe for manifestations of internal bleeding and hemarthrosis...