Hematologic & Immunologic Disorders in Children

A. Kaposi's sarcoma C. Wasting syndrome D. Pulmonary candidiasis

A nurse is admitting a child who has severely symptomatic HIV. Which of the following findings should the nurse expect? (Select all that apply.) A. Kaposi's sarcoma B. Hepatitis C. Wasting syndrome D. Pulmonary candidiasis E. Cardiomyopathy

B. Hemoglobin electrophoresis D. Standard C. Oral Candidiasis

A nurse is caring for an infant whose screening test reveals that he might have sickle cell disease. Which of the following tests should be performed to distinguish if the infant has the trait or the disease? A. Sickle solubility test B. Hemoglobin electrophoresis C. Complete blood count D. Transcranial Doppler A nurse is caring for a child who has AIDS. Which of the following isolation precautions should the nurse implement? A. Contact B. Airborne C. Droplet D. Standard A nurse is admitting a child who has HIV. The nurse should identify which of the following findings as an indication that the child is in the mildly symptomatic category of HIV?(SATA) A. Herpes zoster B. Anemia C. Oral Candidiasis D. Hepatomegaly E. Lymphadenopathy

A. Press the nares together for at least 10 min. C. Pack cotton or tissue into the naris that is bleeding.

A nurse is providing teaching about epistaxis to the parent of a school‑age child. Which of the following should the nurse include as an appropriate action to take when managing an episode of epistaxis? (Select all that apply.) A. Press the nares together for at least 10 min. B. Breathe through the nose until bleeding stops. C. Pack cotton or tissue into the naris that is bleeding. D. Apply a warm cloth across the bridge of the nose. E. Insert petroleum into the naris after the bleeding stops.

A. Sit up and lean forward. D. My child should take the supplement through a straw B. Use the Z‑track method when administering the dose.

A nurse is providing teaching about the management of epistaxis to an adolescent. Which of the following positions should the nurse instruct the adolescent to take when experiencing a nosebleed? A. Sit up and lean forward. B. Sit up and tilt the head up. C. Lie in a supine position. D. Lie in a prone position. A nurse is providing teaching to the parent of a child who has a new prescription for liquid oral iron supplements. Which of the following statements by the parent indicates an understanding of the teaching? A. I should take my child to the ED if his stool becomes dark B. My child should avoid eating citrus fruits while taking the supplements C. I should give the iron with milk to help prevent an upset stomach D. My child should take the supplement through a straw A nurse is preparing to administer iron dextran IM to a school‑age child who has iron deficiency anemia. Which of the following actions by the nurse is appropriate? A. Administer the dose in the deltoid muscle. B. Use the Z‑track method when administering the dose. C. Avoid injecting more than 2 mL with each dose. D. Massage the injection site for 1 min after administering the dose.

B. "HIV is transmitted through IV substance use." d. Decreased oxygen-carrying capacity of blood. c. topical application of local anesthetic can eliminate venipuncture pain.

A nurse is teaching a group of adolescents about HIV/AIDS. Which of the following statements should the nurse include in the teaching? A. "You can contract HIV through casual kissing." B. "HIV is transmitted through IV substance use." C. "HIV is now curable if caught in the early stages." D. "Medications inhibit transmission of the HIV virus." What is the primary result of anemia? a. Increased blood viscosity. b. Depressed hematopoietic system. c. Presence of abnormal hemoglobin. d. Decreased oxygen-carrying capacity of blood. Several blood tests are ordered for a preschool child with severe anemia. She is crying and upset because she remembers the venipuncture done at the clinic 2 days ago. The nurse should explain that: a. venipuncture discomfort is very brief. b. only one venipuncture will be needed. c. topical application of local anesthetic can eliminate venipuncture pain. d. most blood tests on children require only a finger puncture because a small amount of blood is needed.

A. Obtain yearly influenza vaccination. C. Avoid individuals who have colds. D. Provide nutritional supplements.

A nurse is teaching a parent of a child who has HIV. Which of the following information should the nurse include? (SATA) A. Obtain yearly influenza vaccination. B. Monitor a fever for 24 hr before seeking medical care. C. Avoid individuals who have colds. D. Provide nutritional supplements. E. Administer aspirin for pain.

b. Adequate hydration c. Pain management

A school-age child is admitted in vaso-occlusive sickle cell crisis. The child's care should include which intervention? (Select all that apply.) a. Correction of acidosis b. Adequate hydration c. Pain management d. Administration of heparin e. Replacement of factor VIII

b. Delay disease progression b. Varicella d. Circulatory overload

A young child with human immunodeficiency virus is receiving several antiretroviral drugs. What is the expected outcome of these drug therapies? a. Cure the disease b. Delay disease progression c. Prevent spread of disease d. Treat Pneumocystis jiroveci pneumonia Which immunization should be given with caution to children infected with human immunodeficiency virus? a. Influenza b. Varicella c. Pneumococcus d. Inactivated poliovirus An 8-year-old girl is receiving a blood transfusion when the nurse notes that she has developed precordial pain, dyspnea, distended neck veins, slight cyanosis, and a dry cough. These manifestations are most suggestive of what associated adverse reaction? a. Air embolism b. Allergic reaction c. Hemolytic reaction d. Circulatory overload

Bed rest

Anemia · Anemia: a condition where the RBCs or hemoglobin concentration is low. This diminished O2 carrying capacity of the blood, causing a reduction in the O2 available to tissues § Consequences of Anemia: not enough O2 in blood -> not enough O2 for cells. Hemodilution from anemia decreases peripheral vascular resistance, causing greater amounts of blood to return to the heart (can produce a murmur). Due to this increased cardiac workload (especially during exercise or stress), cardiac failure could occur. § Cyanosis is NOT typically evident in children. Growth restriction and anorexia is a common finding in anemic children, as well as delayed sexual maturation in older children § S/S: lack of energy, easy fatigability, pallor, decreased RBCs, decreased hgb/hct · Nursing Interventions for Anemia § Identify the underlying cause so you can treat the underlying cause · Look for statements like "my kid drinks a lot of milk"-> iron deficiency · Stool examinations for occult blood to identify lactase deficiency that causes GI irritation/bleeding · Usually frequent blood tests are needed. Use EMLA cream before needle sticks, explain every test and why they aren't done all at once, encourage family to stay with child during procedure, allow child to play with equipment or participate in procedure (holding a Band-Aid), let older children view blood cells under microscope/photograph (if desired) · Bone marrow aspiration is usually not needed except for aplastic anemia · Explain blood components: RBCs carry oxygen you breathe to your body, WBCs help keep germs from causing infection, Platelets help stop bleeding by making a scab, Plasma is the liquid portion of blood (which also has clotting factors to stop bleeding) § Blood Transfusion after hemorrhage if needed § Nutritional intervention for nutritional deficiency anemias § Supportive care § Intravenous (IV) fluids to replace intravascular volume § Oxygen therapy § ______________________ (high activity will increase O2 demands). Signs of exertion include tachycardia, palpitations, tachypnea, dyspnea, SOB, hypernea, dizziness, lightheadedness, diaphoresis, pallor, limp posture, slow movements, poor activity tolerance, babies have poor sucking § Measures to prevent infection: hand washing, don't allow visitors who are sick, maintain nutrition

Anti-thymocyte globulin (ATG) + cyclosporine

Aplastic Anemia · Aplastic Anemia: Rare life-threatening condition of bone marrow failure (pancytopenia = profound anemia, leukopenia, and thrombocytopenia) · Two Forms - Congenital (Fanconi Syndrome) or acquired as a result of a virus, infection, an immune disorder, chemo agents, certain cleaning agents, cancers, or other unknown causes · Manifestations/Diagnosis of Aplastic Anemia: § Anemia, leukopenia, thrombocytopenia § Bone marrow examination: shows conversion of red bone marrow to yellow, fatty bone marrow § Less than 500 absolute granulocyte count; less than 20,000 platelet count; less than 40,000 absolute reticulocyte count · Treatment and Nursing Interventions for Aplastic Anemia: § Immunosuppressive Therapy: _____________________________________ to suppress autoimmune response. · ATG is usually given orally for several weeks to months, given IV over 12-16hr for 4 days · Responses to treatment generally don't occur until 3-4 months · Because of hypersensitivity response associated with ATG, methylprednisone is given IV to prevent these side effects § Bone Marrow Transplant

c. X-linked recessive inherited disorder in which a blood-clotting factor is deficient d. Immune thrombocytopenia c. Acquired immunodeficiency syndrome (AIDS)

As related to inherited disorders, which statement is descriptive of most cases of hemophilia? a. Autosomal dominant disorder causing deficiency in a factor involved in the blood-clotting reaction b. X-linked recessive inherited disorder causing deficiency of platelets and prolonged bleeding c. X-linked recessive inherited disorder in which a blood-clotting factor is deficient d. Y-linked recessive inherited disorder in which the red blood cells become moon shaped What name is given to identify an acquired hemorrhagic disorder that is characterized by excessive destruction of platelets? a. Aplastic anemia b. Thalassemia major c. Disseminated intravascular coagulation d. Immune thrombocytopenia Which condition is caused by a virus that primarily infects a specific subset of T lymphocytes, the CD4+ T-cells? a. Wiskott-Aldrich syndrome b. Idiopathic thrombocytopenic purpura (ITP) c. Acquired immunodeficiency syndrome (AIDS) d. Severe combined immunodeficiency disease

Anemia

Hematologic and Immunologic History · Child's lack of energy · Food diary of poor iron sources · Frequent infections · Bleeding that is difficult to control · Pallor, petechiae, bruising · Epistaxis Treatment: lean the head forward, and apply pressure to the nose · _______________: The most common hematologic disorder of childhood

Platelets and FFP

Disseminated Intravascular Coagulation (DIC) · Disseminated Intravascular Coagulation (DIC) (consumption coagulopathy): diffuse fibrin deposition in the microvasculature, consumption of coagulation factors, and endogenous generation of thrombin and plasmin. It is a secondary disorder that occurs as a complication of many pathologic processes § Bleeding and clotting that occur simultaneously with hypoxia, acidosis, shock, and endothelial damage; and many other severe systemic diseases (CHD, NEC, sepsis) · Pathophysiology: Fibrinogen rapidly converts to fibrin and platelets are actively destroyed leading to uncontrolled hemorrhage · Manifestations of Disseminated Intravascular Coagulation (DIC): § Petechiae & Purpura § Bleeding from openings in skin (venipuncture, incisions) § Bleeding from umbilicus, trachea (newborn) § GI bleeding § Hypotension § Organ dysfunction from infarction and ischemia § Prolonged PT, PTT, thrombin time, and increased D-dimer antigen § Decreased platelets, fragmented RBCs, and depleted fibrinogen · Treatment is aimed at controlling the cause · ___________________________________ are infused to replace lost plasma components, especially in children whose disease remains uncontrolled · Use of IV heparin is most often RESTRICED to patients who have no response to treatment of underlying disease or replacement of platelets · Care in PICU typically needed

1. Have child sit up and lean forward (aspiration can occur if they lean back) 2. Apply continuous pressure to nose for 10 minutes (make sure child breaths through mouth) 3. Insert cotton or wadded tissue into each nostril and apply ice/cold cloth to nose bridge if bleeding persists 4. Keep child calm and quiet

Epistaxis · Epistaxis: nose bleeding; common in childhood § Recurrent episodes can indicate underlying diseases, like vascular abnormalities, leukemia, thrombocytopenia, or clotting factor deficiency diseases (hemophilia) · Emergency treatment of Epistaxis: 1 2 3 4

Idiopathic Thrombocytopenic Purpura (immune thrombocytopenia)

· Manifestations of __________________________: § Easy bruising: · Petechiae - pinpoint hemorrhages · Ecchymoses · Most often over bony prominences § Bleeding from mucous membranes: · Epistaxis · Bleeding gums · Internal hemorrhage: hematuria, hematemesis, melena, hemarthrosis, menorrhagia, hematomas over lower extremities § Purpura - larger purplish areas of hemorrhage in which blood collects under the tissues DIAGNOSTICS: Platelets < 20,000 *No definitive test; several tests are used to rule out other causes

cow's milk

Iron Deficiency Anemia · How to Replace Iron for Iron Deficiency Anemia § For formula-fed infants, use supplemental iron or iron-fortified cereals/formulas starting at 6 months. Infants younger than 12months should not be fed ______________ because it can increase the risk for GI blood loss from irritation. § Provider iron supplements for preterm and LBW babies by 2 months of age § Iron-rich Foods for Children: dried beans and lentils, peanut butter, leafy vegetables, iron-fortified breads and flour, red meat § Transfusion of PRBCs for severe anemia. § Ferrous Iron Supplement: Dietary intake of ORAL Fe+ given through a straw, syringe or medicine dropper in back of mouth; brush teeth after. Give in two divided doses with meals (vitamin C), but don't give with milk · Fe+ replacement therapy IM (Z-track method, do not massage) or IV if levels fail to rise after one month. (check for blood loss, malabsorption, noncompliance, improper administration, or other anemias) · IV Fe+ only if Fe+ malabsorption, chronic hemoglobinuria, or intolerance to PO. Monitor for anaphylaxis. It is a very painful infusion, so monitor closely · Stools should be green, tarry, dark/black (possible constipation) with iron supplements · Increase fluids and fiber when taking iron supplements (prevent constipation) · Take iron with vitamin C (orange juice) to promote absorption · DON'T take with milk, tea, or antacids · Iron toxicity can be fatal, so parents should be instructed to keep no more than 1 month's supply in the home and store it safely · Preterm babies, LBW babies, or infants with low iron stores should be given iron supplements at 2 months of age

dried beans and lentils peanut butter leafy vegetables iron-fortified breads and flour red meat

Iron Deficiency Anemia · How to Replace Iron for Iron Deficiency Anemia § For formula-fed infants, use supplemental iron or iron-fortified cereals/formulas starting at 6 months. Infants younger than 12months should not be fed cow's milk because it can increase the risk for GI blood loss from irritation. § Provider iron supplements for preterm and LBW babies by 2 months of age § Iron-rich Foods for Children: _____________________________________________ § Transfusion of PRBCs for severe anemia. § Ferrous Iron Supplement: Dietary intake of ORAL Fe+ given through a straw, syringe or medicine dropper in back of mouth; brush teeth after. Give in two divided doses with meals (vitamin C), but don't give with milk · Fe+ replacement therapy IM (Z-track method, do not massage) or IV if levels fail to rise after one month. (check for blood loss, malabsorption, noncompliance, improper administration, or other anemias) · IV Fe+ only if Fe+ malabsorption, chronic hemoglobinuria, or intolerance to PO. Monitor for anaphylaxis. It is a very painful infusion, so monitor closely · Stools should be green, tarry, dark/black (possible constipation) with iron supplements · Increase fluids and fiber when taking iron supplements (prevent constipation) · Take iron with vitamin C (orange juice) to promote absorption · DON'T take with milk, tea, or antacids · Iron toxicity can be fatal, so parents should be instructed to keep no more than 1 month's supply in the home and store it safely · Preterm babies, LBW babies, or infants with low iron stores should be given iron supplements at 2 months of age

fast growth rate combined with poor eating habits, menses, obesity, or strenuous activities

Iron Deficiency Anemia · Iron Deficiency Anemia: anemia caused by inadequate supply of dietary iron -> The body does not have enough iron to produce hemoglobin (Hgb) § Hgb decrease = decrease oxygen capacity in blood = weakness & fatigue § Can be caused by anything that decreases supply of iron, impairs iron absorption, increases body's need for iron, or affect synthesis of Hgb. · Premature infants at risk due to low iron (Fe+) stores (2-3months) · Children ages 12-36 month at risk due to cow's milk consumption and not eating enough iron-containing food. Milk is not a good source of iron and takes the place of iron-rich solid foods · Adolescents are at risk due to _______________________________________________________________ · Other Risk Factors: malabsorption disorders, GERD, pyloric stenosis, GI polyps, colitis, · Iron Deficiency Anemia can result in: 1. Delayed growth 2. Cognitive and motor delays 3. Behavioral changes

Iron-fortified cereals and formulas

Iron Deficiency Anemia · Iron Deficiency Anemia: anemia caused by inadequate supply of dietary iron -> The body does not have enough iron to produce hemoglobin (Hgb) · Iron Deficiency Anemia is Mostly Preventable § Inadequate supply of dietary iron § __________________________________ for infants (especially after 4-6 months of age) § Adolescents at risk due to rapid growth and poor eating habits

c. Injecting deeply into a large muscle d. Iron-fortified infant cereal can be introduced at approximately 6 months of age b. Sickle cell anemia

Iron dextran is ordered for a young child with severe iron deficiency anemia. What nursing consideration should be considered? a. Administering with meals b. Administering between meals c. Injecting deeply into a large muscle d. Massaging injection site for 5 minutes after administration of drug What intervention should the nurse share with parents on how to prevent iron deficiency anemia in a healthy, term, breastfed infant? a. Iron (ferrous sulfate) drops after age 1 month b. Iron-fortified commercial formula can be used by ages 4 to 6 months c. Iron-fortified solid foods are introduced at 3 months d. Iron-fortified infant cereal can be introduced at approximately 6 months of age What term is used to identify the condition in which the normal adult hemoglobin is partly or completely replaced by abnormal hemoglobin? a. Aplastic anemia b. Sickle cell anemia c. Thalassemia major d. Iron deficiency anemia

§ Stress § Infection § Dehydration § Physical exertion § Excessive Cold

· Signs and Symptoms of Sickle Cell Anemia: § Joint Pain § SOB, fatigue, jaundice, visual disturbances § Pallor, pale mucous membranes § Hands/Feet cool to touch § Dizziness, Headache § Hematuria § Splenomegaly § Hepatomegaly § Other: growth restriction, chronic anemia (Hgb of 6-9), delayed sexual development, susceptibility to sepsis · Triggers for Sickle Cell Anemia: - - - - -

b. Swimming d. Golf e. Bowling

Parents of a school-age child with hemophilia ask the nurse, "Which sports are recommended for children with hemophilia?" Which sports should the nurse recommend? (Select all that apply.) a. Soccer b. Swimming c. Basketball d. Golf e. Bowling

Vaso-Occlusive Crisis

Sickle cell crises: _____________________________________: characterized by ischemia causing mild to severe pain that may last from minutes to days or longer. Most common, severe, painful form requiring round the clock opioids; complications include CVA, Acute Chest Syndrome § Signs and Symptoms of ______________________________________________: pain in involved areas · Extremities: swelling with painful joints in hands/feet · Abdomen: severe pain that could resemble acute surgical condition · Chest: S/S of pneumonia or pulmonary disease · Liver: jaundice or hepatic coma · Kidney: hematuria · Genitalia: priapism · Cerebral: stroke, visual disturbances

1 Low = Anemia 2 High = Dehydration, burns, polycythemia 3 Hemoglobin: Protein/Iron, nutrients, O2

The Hematologic System · Red Blood Cells (Erythrocytes) - transport nutrients & oxygen · Platelets (Thrombocytes) - clotting · White Blood Cells (Leukocytes) - fighting infection · RBC: 1 2 3 · Platelets § Low = Increased bleeding § High = Increased clotting

b. Risk for Infection related to inadequate secondary defenses or immunosuppression c. Ineffective Protection related to thrombocytopenia d. Ineffective Tissue Perfusion related to anemia

The nurse is caring for a child with aplastic anemia. Which nursing diagnoses are appropriate? (Select all that apply.) a. Acute Pain related to vaso-occlusion b. Risk for Infection related to inadequate secondary defenses or immunosuppression c. Ineffective Protection related to thrombocytopenia d. Ineffective Tissue Perfusion related to anemia e. Ineffective Protection related to abnormal clotting

b. Avoidance of intramuscular (IM) injections c. Acetaminophen for mild pain control d. Soft toothbrush for dental hygiene

The nurse is planning care for a school-age child admitted to the hospital with hemophilia. Which interventions should the nurse plan to implement for this child? (Select all that apply.) a. Fingersticks for blood work instead of venipunctures b. Avoidance of intramuscular (IM) injections c. Acetaminophen for mild pain control d. Soft toothbrush for dental hygiene e. Administration of packed red blood cells

c. Finding a suitable donor involves matching antigens from the human leukocyte antigen (HLA) system. a. Immobilization and elevation of the affected joint a. Perinatal transmission c. Puppet play in the child's room

The parents of a child diagnosed with aplastic anemia tell the nurse that a bone marrow transplant (BMT) may be necessary. What should the nurse recognize as important when discussing this with the family? a. BMT should be done at time of diagnosis. b. Parents and siblings of child have a 25% chance of being a suitable donor. c. Finding a suitable donor involves matching antigens from the human leukocyte antigen (HLA) system. d. If BMT fails, chemotherapy or radiotherapy must be continued. What is the priority nursing intervention for a child hospitalized with hemarthrosis resulting from hemophilia? a. Immobilization and elevation of the affected joint b. Administration of acetaminophen for pain relief c. Assessment of the child's response to hospitalization d. Assessment of the impact of hospitalization on the family system What is the most common mode of transmission of human immunodeficiency virus (HIV) in the pediatric population? a. Perinatal transmission b. Sexual abuse c. Blood transfusions d. Poor hand washing The nurse is planning activity for a 4-year-old child with anemia. Which activity should the nurse plan for this child? a. Game of "hide and seek" in the children's outdoor play area b. Participation in dance activities in the playroom c. Puppet play in the child's room d. A walk down to the hospital lobby

chelation therapy (only with HIGH LEVELS)

Toxins & medication Exposures · Lead Poisoning § Lower Levels · Behavior changes · Learning difficulties § Higher Levels · Encephalopathy · Seizures · Brain Damage § Treatment: watch and wait or _________________________________________ · Aplastic Anemia § Failure of bone marrow to produce cells § Acquired from viral infections or exposure to certain drugs § Labs: CBC (anemia, leukopenia, low platelets) § Complications · Severe overwhelming infection, hemorrhage, death · Symptoms: epistaxis, bleeding, HA, fatigue, ecchymosis, tachycardia § Treatment: stem cell transplant and immunosuppressive therapy

MMR, varicella, rotavirus

Treatment for Children with HIV/AIDs: · Slow growth of the virus, prevent/treat infections, providing nutritional support and symptomatic treatment · Antiviral medicines (zidovudine, didanosine, stavudine) lifelong § Monitor CBC, WBC, and LFTs; take on regular schedule and don't skip doses · Pneumocystis carinii is most common infection in infants born to HIV-infected women at ages 3-6 months and should receive prophylaxis (trimethoprim/sulfamethoxazole) · Immunizations (flu vaccines, pneumococcal) § AVOID LIVE VACCINES: ________________________________ · Nutrition or possible TPN to prevent growth failure · Awareness of disease progression, disease spread, and safety · Prevent infections: keep skin clean/dry, wash hands, good oral care, deep breathing/coughing, avoid sick people, encourage immunizations, take antibiotics as prescribed for existing infections · Psychological concerns about disclosing the diagnosis · Family planning · Adolescents need education to prevent HIV spread: safe sex practices, avoiding IV drug use · Pain medications for children in pain (caused by infection, encephalopathy, medications, or unknown source) · Issues about confidentiality and ostracism

Anti-D antibody

Treatment of Idiopathic Thrombocytopenic Purpura (immune thrombocytopenia): § Activity restrictions at onset while platelets count is low and active bleeding or progression of lesions is occurring § Meds: prednisone, IV immunoglobulin (IVIG), and Anti-D antibody § ___________________________________________ can be given in one dose over 5-10 minutes and is less expensive the IVIG. Patients must be premedicated with acetaminophen 5-10min before administration · Anti-D antibody Therapy Criteria: 1. Age between 1-19yr; Rh (D) positive blood type 2. Normal WBC and hemoglobin; platelet of 20,000 3. No active mucosal bleeding 4. No history of reaction to plasma products 5. No known IGA deficiency 6. No infection 7. Absence of Evans syndrome 8. No suspicions of SLE or other collagen vascular disorder 9. No splenectomy · After administration of Anti-D antibody, observe child for 1 hour and maintain patent IV. Check baseline vitals before infusion and again 5, 20, and 60 minutes after starting the infusion. · If fever, chill, and headache occur, the nurse should give acetaminophen, diphenhydramine, or hydrocortisone and monitor the child for another hour § Infusions of IVIG ONLY IF active bleeding (intravenous immunoglobulin) § Splenectomy is used for patients not responding to meds and have increased risk for severe hemorrhage. Children should be at least age 5, and have pneumococcal, meningococcal, and H. influenzae vaccines. They will get prophylactic antibiotics after surgery usually for a minimum of 3 years. § Protect from injury/safety teaching to parents § NO contact sports, bike riding, skateboarding, in-line skating, gymnastics, climbing or running § Parents are encouraged to engage children in quiet activities and to prevent injuries (especially to head) § Immediate medical evaluation for head or ABD trauma

§ Fe+ deficiency § Folic Acid deficiency § Pernicious anemia (deficiency in B12)

Types of Anemia: · Nutritional Anemia: - - - · Toxin & Aplastic Anemia: § Lead Poisoning § Medication reaction · Hemolytic Anemia: § Sickle Cell anemia § Thalassemia

factor VIII concentrates given IV (often numerous doses)

· Therapeutic Management of Hemophilia: § Factor Replacement: __________________________________ § Desmopressin (DDAVP) to increase plasma factor VIII activity (not effective for hemophilia). Works well for mild hemophilia, NOT for severe hemophilia § Corticosteroids are given for hematuria, acute hemarthrosis, and chronic synovitis § NSAIDs can relieve pain, but use with caution due to bleeding (take with food) § Exercise program and physical therapy to increase muscle strength and decrease bleeding risk § Family/Patient teaching on venipuncture to administer meds at home § Genetic counseling and family planning

a. Activity intolerance related to generalized weakness a. Milk is a poor source of iron. c. Adequate dosage will turn the stools a tarry green color.

What is the most appropriate nursing diagnosis for a child diagnosed with moderate anemia? a. Activity intolerance related to generalized weakness b. Decreased cardiac output related to abnormal hemoglobin c. Risk for injury related to depressed sensorium d. Risk for Injury related to dehydration and abnormal hemoglobin Which statement best explains why iron deficiency anemia is common during toddlerhood? a. Milk is a poor source of iron. b. Iron cannot be stored during fetal development. c. Fetal iron stores are depleted by age 1 month. d. Dietary iron cannot be started until age 12 months. When teaching the mother of a 9-month-old infant about administering liquid iron preparations, the nurse should include that information? a. They should be given with meals. b. They should be stopped immediately if nausea and vomiting occur. c. Adequate dosage will turn the stools a tarry green color. d. Preparation should be allowed to mix with saliva and bathe the teeth before swallowing.

b. Notify the health care provider if a fever of 38.5° C (101.3° F) or greater occurs. c. Give penicillin as prescribed. e. Notify the health care provider if your child begins to develop symptoms of cold.

Which should the nurse teach about prevention of sickle cell crises to parents of a preschool child with sickle cell disease? (SATA) a. Limit fluids at bedtime. b. Notify the health care provider if a fever of 38.5° C (101.3° F) or greater occurs. c. Give penicillin as prescribed. d. Use ice packs to decrease the discomfort of vaso-occlusive pain in the legs. e. Notify the health care provider if your child begins to develop symptoms of cold.

c. Increased incidence occurs in families of Mediterranean extraction. b. Elimination of excess iron. a. Aplastic anemia

Which statement correctly describes β-thalassemia major (Cooley's anemia)? a. All formed elements of the blood are depressed. b. Inadequate numbers of red blood cells are present. c. Increased incidence occurs in families of Mediterranean extraction. d. Increased incidence occurs in persons of West African descent. Chelation therapy is begun on a child with β-thalassemia major with what expected result? a. Treatment of the disease. b. Elimination of excess iron. c. Decreasing the risk of hypoxia. d. Managing nausea and vomiting. In which condition are all the formed elements of the blood simultaneously depressed? a. Aplastic anemia b. Sickle cell anemia c. Thalassemia major d. Iron deficiency anemia

c. Increased red blood cell destruction occurs d. Painful swelling of hands and feet, painful joints b. Narcotics rarely cause addiction when they are medically indicated.

Which statement most accurately describes the pathologic changes of sickle cell anemia? a. Sickle-shaped cells carry excess oxygen b. Sickle-shaped cells decrease blood viscosity c. Increased red blood cell destruction occurs d. Decreased red blood cell destruction occurs Which clinical manifestation should the nurse expect when a child diagnosed with sickle cell anemia experiences an acute vaso-occlusive crisis? a. Circulatory collapse b. Cardiomegaly, systolic murmurs c. Hepatomegaly, intrahepatic cholestasis d. Painful swelling of hands and feet, painful joints The parents of a child hospitalized with sickle cell anemia tell the nurse that they are concerned about narcotic analgesics causing addiction. The nurse should provide what explanation in response to the parent's concern? a. Narcotics are often ordered but not usually needed. b. Narcotics rarely cause addiction when they are medically indicated. c. Narcotics are given as a last resort because of the threat of addiction. d. Narcotics are used only if other measures such as ice packs are ineffective.

· Beta-Thalassemia (Cooley's anemia)

___________________: a genetic disorder characterized by reduced production of normal hemoglobin. Defective hemoglobin is formed. When it disintegrates, it damages RBCs, causing severe anemia. § To compensate for this, an overabundance of erythrocytes is formed in the bone marrow (unless bone marrow is suppressed by transfusion therapy) § Excess iron from packed RBC transfusions and from the rapid destruction of defective cells is stored and produces hemosiderosis. § Common in Italian, Greek, Syrian, African & Asians

1. Less than 500 absolute granulocyte count 2. less than 20,000 platelet count 3. less than 40,000 absolute reticulocyte count

· Aplastic Anemia: Rare life-threatening condition of bone marrow failure (pancytopenia = profound anemia, leukopenia, and thrombocytopenia) · Two Forms - Congenital (Fanconi Syndrome) or acquired as a result of a virus, infection, an immune disorder, chemo agents, certain cleaning agents, cancers, or other unknown causes · Manifestations/Diagnosis of Aplastic Anemia: § Anemia, leukopenia, thrombocytopenia § Bone marrow examination: shows conversion of red bone marrow to yellow, fatty bone marrow § LABS: 1. ______________________________ 2. ______________________________ 3. ______________________________

Transfusions of PRBCs to keep Hgb >9.5 g/dL (as often as every 3-5 weeks) · Improves well-being because of increased ability to participate in normal activities · Decreased cardiomegaly and hepatosplenomegaly · Fewer bone changes · More normal growth and development until puberty · Fewer infections

· Assessment Beta-Thalassemia (Cooley's anemia): § Anemia (before diagnosis: pallor, unexplained fever, poor feeding, enlarged spleen/liver § Progressive anemia: chronic hypoxia, headache, bone pain, low exercise tolerance, listlessness, anorexia § Other features: small stature, delayed sexual maturation, bronzed/freckled complexion (if not getting chelation therapy) § Bone changes (in older children if untreated): · Frontal & Maxillary Bone Bossing · Jaundice & pallor · Enlarged head · Prominent malar eminences · Flat or depressed bridge of nose · Enlarged maxilla · Protrusion of lip and upper incisors and malocclusion · Generalized osteoporosis · TREATMENT for Beta-Thalassemia (Cooley's anemia): § 1. ________________________________________________________ - - - § Deferoxamine/Chelation therapy: needed for resulting iron overload (hemosiderosis) from multiple blood transfusions § Splenectomy if splenomegaly due to repeated transfusions. After surgery, a severe complication can be overwhelming infection, so children may be put on prophylactic antibiotics with close supervision for many years (they also need immunizations)

Beta-Thalassemia (Cooley's anemia)

· Assessment of ______________________________________: § Anemia (before diagnosis: pallor, unexplained fever, poor feeding, enlarged spleen/liver § Progressive anemia: chronic hypoxia, headache, bone pain, low exercise tolerance, listlessness, anorexia § Other features: small stature, delayed sexual maturation, bronzed/freckled complexion (if not getting chelation therapy) § Bone changes (in older children if untreated): · Frontal & Maxillary Bone Bossing · Jaundice & pallor · Enlarged head · Prominent malar eminences · Flat or depressed bridge of nose · Enlarged maxilla · Protrusion of lip and upper incisors and malocclusion · Generalized osteoporosis

Iron Deficiency Anemia

· Assessment of ________________________________________: § Unsteady gait § Weakness § Fatigue, irritability § Dizziness § Pallor § Shortness of breath § Tachycardia, systolic heart murmur § Brittle, spoon-shaped fingernails § Cravings for non-nutritive substances (ice, dirt, paper) § CBC: decreased RBC, Hgb, and Hct § Reticulocyte Count: can be decreased § Transferrin: 10% indicative of Anemia § Total Iron binding capacity: elevated

Human immunodeficiency virus (HIV & AIDS)

· Common Manifestations of __________________________ in children: § Lymphadenopathy § Hepatosplenomegaly § Recurrent URIs, sinusitis, otitis media § Oral candidiasis § Chronic or recurrent diarrhea § Failure to thrive § Developmental delay § Parotitis § Progressive disease: anemia, bacterial meningitis, pneumonia, sepsis, oropharyngeal candidiasis, cytomegalovirus, cardiomyopathy, herpes zoster, HSV, bronchitis, hepatitis, esophagitis, other serious infections § Severe: multiple serious infections, Kaposi's sarcoma, TB, encephalopathy, developmental delays, cryptosporidiosis, pneumocystis carinii pneumonia, septicemia, wasting syndrome, lymphoma · AIDs diagnosis is associated with certain illnesses or conditions and diagnostics/labs; they many show short stature, malnutrition, and cardiomyopathy · Diagnosis of HIV: Enzyme-linked immunosorbent assay (ELISA) and Western Blot immunoassay

Cerebrovascular Accident (CVA/stroke)

· Complications of Sickle Cell Anemia: § Vaso-occlusive, hyperhemolytic, aplastic, and Sequestrian crisis's § Sepsis § Delayed growth & development § ___________________________________: sudden, severe complication of SCA, where sickled cells block major blood vessels in the brain, resulting in cerebral infarction and varying degrees of neuro impairment · S/S: severe headache, vomiting, twitching of face or limbs, seizures, abnormal behavior, inability to move an arm/leg, stagger, unsteady gait, stutters or slurred speech, weakness in hand/foot/leg, vision changes

Acute Chest Syndrome (ACS)

· Complications of Sickle Cell Anemia: § Vaso-occlusive, hyperhemolytic, aplastic, and Sequestrian crisis's § Sepsis § Delayed growth & development § ________________________________________: the presence of a new pulmonary infiltrate and may be associated with chest pain, fever, cough, tachypnea, wheezing, and hypoxia · S/S: chest/back/ABD pain, fever (101.3F), cough, dyspnea, tachypnea, retractions, declining O2 saturation

hemoglobin electrophoresis

· Diagnosis of Sickle Cell Anemia: § Gold Standard: ____________________________ -> a blood test that separates hemoglobins by high voltage § Can screen newborns with sickle-turbidity test (Sickledex). If positive, they need hemoglobin electrophoresis to confirm diagnosis. § If Crisis: Hgb decreased, WBC elevated, bilirubin/reticulocyte elevated, blood smear shows sickled cells

contact sports with severe hemophilia. Always use protective equipment during activities. Preferred activities include low-contact sports (bowling, fishing, swimming, golf)

· Discharge Teaching for Hemophilia: Assess for and Prevent Bleeding § Monitor for bleeding (dark, tarry stool, hematuria, or active bleeding) § Prevent bleeding: use subQ (rather than IM); use venipuncture (rather than finger/heel sticks) § Control bleeding: factor replacement, RICE on affected joints § Physical activity/exercise to promote and strengthen muscles and joints. Infant's and toddler's high activity level places them at risk for falls, bruises, or minor wounds, but restraining the child from mastering motor development can bring more serious problems that allowing the before. MAKE THE ENVIRONMENT SAFE and SUPERVISE! § Refrain from ____________________________________ § Dental Hygiene: soft toothbrush, sponge-tipped disposable toothbrush, water irrigating devices § Wear medical ID band § Don't use aspirin; instead use acetaminophen § Protect furniture edges, carpet in the home (padding) § During hemarthrosis episodes, elevate the joint and immobilize. Active ROM used after acute phase § Maintain immunizations § Genetic counseling, support groups for family

Hemophilia

· Manifestations of ____________________________: § Prolonged bleeding anywhere; hemorrhage from any trauma (loss of baby teeth, circumcision, cuts, nose bleeds, injections) § Excessive bruising, even from slight injury § Hematomas: pain, swelling, limited motion § Spontaneous hematuria or tarry stools § Sub-Q and IM hemorrhages are common § Hemarthroses (bleeding in joint cavities) in knees, elbows, and ankles are common and can cause bony changes and crippling deformities after repeated episodes. · Early signs of this include stiffness, tingling, or achiness in the joint, followed by decrease in joint movement · Joints are warm, red, and swollen with pain and decreased movement. § Bleeding into the neck, mouth, or thorax is serious because it can obstruct airway § Intracranial hemorrhage can be fatal: HA, slurred speech, decreased LOC § Hemorrhage anywhere in the GI tract can lead to anemia, and bleeding into the retroperitoneal cavity is especially hazardous because of the large space for blood to accumulate § Hematomas in the spinal cord can cause paralysis Diagnosis: § History of bleeding episodes, evidence of X-linked heritance, and lab results § Factor VIII and IX assay § aPTT prolonged; platelets and prothrombin time are within expected range § Genetic counseling: DNA testing

1 platelet infusions to counteract bleeding 2 IVIG to provide passive immunity 3 prophylactic antibiotics to prevent/control infection 4 topicals for eczema 5 stem-cell transplant (HSCT) for cure as early as possible

· Medical Management of Wiskott-Aldrich Syndrome: 1 2 3 4 5 § Main nursing intervention is supporting the family in the care of the terminally ill child (prognosis is usually poor) § Control bleeding, prevent/treat infections, manage eczema § Genetic counseling

enuresis, so teach parents that they need to encourage the child to urinate frequently and prior to bed (not limit fluids at night or punish them)

· Nursing Care for Sickle Cell Anemia: § Treatment of any crises: Rest, fluids, transfusions, prevent infection, and pain management! § Education for family on disease § Teach patient to seek early intervention for problems (fever over 101.3F), give penicillin as ordered, recognize s/s of stroke, splenic sequestration, respiratory problems that can lead to hypoxia, and treat child normally (they are normal, but they can get sick in ways other children cannot) § Adequate hydration to prevent sickling and delay vaso-occlusion and hypoxia/ischemia. Provide instructions on how many glasses of water to drink, and teach that some foods have a lot of water (soups, popsicles, ice cream, gelatin, puddings). Don't encourage forcing fluids § Increased fluids can lead to _______________________________________________________ § Advise parents to be aware of situations that can cause dehydration, like hot weather or sports, and recognize s/s of reduced fluid intake like decreased urine output and increased thirst § Prevent pain: analgesics on schedule. Implement a pain program combined with psychologic support to help the child deal with depression, anxiety, or fear that may accompany the disease. § If giving a transfusion, monitor for reaction and hypervolemia § If splenic sequestration occurs, measure spleen size (increasing size is bad). § Monitor for signs of ACS and CVA, which can be fatal · CVA (stroke): seizures, abnormal behaviors, weakness (extremities), slurred speech, visual changes, vomiting, headache · Acute chest syndrome (ACS): chest/back/ABD pain, fever, cough, tachypnea, dyspnea, wheezing, retractions, decreased O2 sat

Rest, fluids, transfusions, prevent infection, and pain management!

· Nursing Care for Sickle Cell Anemia: § Treatment of any crises: ___________________________ § Education for family on disease § Teach patient to seek early intervention for problems (fever over 101.3F), give penicillin as ordered, recognize s/s of stroke, splenic sequestration, respiratory problems that can lead to hypoxia, and treat child normally (they are normal, but they can get sick in ways other children cannot) § Adequate hydration to prevent sickling and delay vaso-occlusion and hypoxia/ischemia. Provide instructions on how many glasses of water to drink, and teach that some foods have a lot of water (soups, popsicles, ice cream, gelatin, puddings). Don't encourage forcing fluids § Increased fluids can lead to enuresis, so teach parents that they need to encourage the child to urinate frequently and prior to bed (not limit fluids at night or punish them) § Advise parents to be aware of situations that can cause dehydration, like hot weather or sports, and recognize s/s of reduced fluid intake like decreased urine output and increased thirst § Prevent pain: analgesics on schedule. Implement a pain program combined with psychologic support to help the child deal with depression, anxiety, or fear that may accompany the disease. § If giving a transfusion, monitor for reaction and hypervolemia § If splenic sequestration occurs, measure spleen size (increasing size is bad). § Monitor for signs of ACS and CVA, which can be fatal · CVA (stroke): seizures, abnormal behaviors, weakness (extremities), slurred speech, visual changes, vomiting, headache · Acute chest syndrome (ACS): chest/back/ABD pain, fever, cough, tachypnea, dyspnea, wheezing, retractions, decreased O2 sat

Chronic Vaso-Occlusive Phenomenon

· Sickle cell crises: Vaso-Occlusive Crisis: characterized by ischemia causing mild to severe pain that may last from minutes to days or longer. Most common, severe, painful form requiring round the clock opioids; complications include CVA, Acute Chest Syndrome § Signs and Symptoms of __________________________________________________: · Heart: cardiomegaly, systolic murmurs · Lungs: altered pulmonary function, susceptibility to infection, pulmonary insufficiency · Kidneys: inability to concentrate urine, enuresis, progressive renal failure · Liver: hepatomegaly, cirrhosis, intrahepatic cholestasis · Spleen: splenomegaly, susceptibility to infection, functional reduction in splenic activity progressing to autosplenectomy · Eyes: eye abnormalities, visual disturbances, retinal detachment, blindness · Extremities: avascular necrosis of hip/shoulder, skeletal deformities, lordosis, kyphosis, chronic leg ulcers, susceptibility to osteomyelitis · CNS: hemiparesis, seizures

Sequestrian Crisis

· Sickle cell crises: § Aplastic crisis: diminished RBC production, usually triggered by viral infection that can result in profound anemia § Hyperhemolytic crisis: an accelerated RBCs destruction characterized by anemia, jaundice, and reticulocytosis. § ____________________________________________: Pooling of blood in the liver spleen and liver leading to decreased circulating blood volume, circulatory collapse, and ultimately shock · S/S: blood pooling, hepatomegaly, splenomegaly, circulatory collapse (shock), hypovolemic shock (irritability, tachycardia, pallor, decreased UO, tachypnea, cool extremities, thready pulse, hypotension) · Splenectomy may be performed in children with recurrent life-threatening SCA, but the spleen usually atrophies on its own (functional asplenia) by 6yrs of age

tested for the trait (genetic counseling)

· Treatment for Beta-Thalassemia (Cooley's anemia): § 1. Transfusions of PRBCs to keep Hgb >9.5 g/dL (as often as every 3-5 weeks) · Improves well-being because of increased ability to participate in normal activities · Decreased cardiomegaly and hepatosplenomegaly · Fewer bone changes · More normal growth and development until puberty · Fewer infections § Deferoxamine/Chelation therapy: needed for resulting iron overload (hemosiderosis) from multiple blood transfusions § Splenectomy if splenomegaly due to repeated transfusions. After surgery, a severe complication can be overwhelming infection, so children may be put on prophylactic antibiotics with close supervision for many years (they also need immunizations) · Nursing Interventions for Beta-Thalassemia (Cooley's anemia): § Promote compliance with transfusion therapy and chelation therapy § Assist the child in coping with the anxiety-provoking treatments and effects of illness § Foster the child's/family's adjustments to illness § Observe for complications from transfusions § All families with a child with thalassemia should be ______________________________ § Family must understand that they need to immediately report fever (101.3F) and symptoms of infection due to risk of sepsis

Transcranial Doppler (TCD)

· Treatments for Sickle Cell Anemia and Crises: § Rest to minimize energy expenditure and improve O2 use § Oral and IV Hydration; monitor I/O (caution with potassium replacement) § Electrolyte replacement because hypoxia causes metabolic acidosis, which promotes sickling § Analgesia for pain due to vaso-occlusive crisis (acetaminophen, IV ketorolac, Opioids, morphine PCA 0.1-0.2mg/kg/dose, comfort measures) · *Meperidine is NOT recommended because it can cause seizures, tremors, myoclonus, and anxiety · Schedule pain mains on regular basis for pain control § Blood replacement transfusion to treat severe anemia and to reduce viscosity of sickled blood. Exchange transfusion is a successful way to reduce sickled cell number and prevent injury. It is used in life-threatening ACS and after stroke. · _______________________ monitors intracranial vascular flow and is recommended yearly for children 2-16 years of age. If abnormal _______________, children should have chronic transfusion treatment · Chelation therapy PRN to prevent Fe+ overload from chronic transfusion therapy · Possible Hematopoietic stem cell transplant: permanent solution for SCD, but high risk of neuro complications § Antibiotics for any infections ( and hand washing, prophylactic penicillin) § O2 if documented hypoxia only (severe hypoxia = massive sickling that can be fatal). O2 administration is usually not effective in reversing sickling or reducing pain because O2 can't reach the sickled RBCs in clogged vessels § Rest, fluids, transfusions, prevent infection, and pain management! § Pneumococcal, Influenza, meningococcal vaccines recommended due to susceptibility to infection § Consult child life specialist

Hemophilia

· _____________________: a group of bleeding disorders resulting from congenital deficiency, dysfunction, or absence of specific coagulation proteins/factors § Two most common forms are Factor VIII deficiency (hemophilia A/classic) and Factor IX (hemophilia B/ Christmas disease) deficiency § Von Willebrand is another type of hemophilia characterized by a lack of the von Willebrand factor protein and inability of platelets to aggregate § X-Linked Recessive § People with hemophilia have 3 of the 3 factors required for coagulation (vascular influence and platelets), thus they may bleed for longer periods but not at a faster rate.

Human immunodeficiency virus (HIV & AIDS)

· __________________________: HIV is a retrovirus that is found in blood, semen, vaginal secretions, and breast milk. The virus mainly infects T lymphocytes (CD4 T cells), which increases risk for opportunistic infections § 90% of children are born to HIV-infected mothers · Risk Factors: § Infants can get infected perinatally or by ingesting milk from HIV infected mother § Exposure to blood or body fluids that contain HIV virus § Sexual Assault § Risky Behaviors (IV drug use, unprotected sexual activity -> adolescent high risk) § STIs § Lack of awareness of risk factors and modes of transmission

Idiopathic Thrombocytopenic Purpura (immune thrombocytopenia)

· __________________________: an acquired hemorrhagic disordered characterized by: 1. Thrombocytopenia, 2. Absence of minimal signs of bleeding (easy bruising, mucosal bleeding, petechiae) in most childhood cases, and 3. Normal bone marrow with a normal/increased number of immature platelets (megakaryocytes) and eosinophils. § __________________________ involves the evolution of antibodies against multiple platelet antigens, leading to reduced platelet survival and impaired platelet production § Can be acute and self-limiting or chronic (more than 12mth)

Wiskott-Aldrich Syndrome

· ___________________________: an X-linked recessive disorder characterized by a triad of abnormalities: § 1. Thrombocytopenia § 2. Eczema § 3. Deficiency of B and T lymphocytes · At birth the presenting feature may be an increased bleeding at circumcision or bloody diarrhea due to thrombocytopenia. As child grows, eczema gets more severe and recurrent infections occur, with bleeding becoming less frequent · Chronic pulmonary disease, sinusitis, and otitis media may result from recurrent infections · Chronic herpes simplex infection can lead to keratitis of eye with vision loss

Sickle Cell Anemia

· ______________________________________: a group of diseases termed hemoglobinopathies in which normal Hgb is partly or completes replaced by abnormal sickle Hgb (HgS). Sick cell disease refers to a group of hereditary disorders which are related to the presence of HgS § When both parents have sickle cell trait, there is a 25% chance with each pregnancy of producing a child with SCA § Most acute s/s are produced during crises · Pathophysiology of ______________________________________: § 1. Obstruction caused by sickled RBCs with other cells, 2. Vascular inflammation, 3. Increased RBC destruction § Cells sickle -> vaso-occlusion § Tissue Hypoxia § Become viscous from clumping § Ischemia & Infarction (cell death) § Prevents normal blood flow to tissues § Common in African American