Competency 6- Pediatric Hematologic & Immunologic Dysfunction

What are the two main approaches to therapeutic management of AA?

(1) immunosuppressive therapy to remove the presumed immunologic functions that prolong aplasia (2) replacement of the bone marrow through transplantation. Bone marrow transplantation is the treatment of choice for severe AA when a suitable donor exists

What is the objective of nursing care for β-Thalassemia?

(1) promote compliance with transfusion and chelation therapy (2) assist the child in coping with the anxiety-provoking treatments and the effects of the illness (3) foster the child's and family's adjustment to a chronic illness (4) observe for complications of multiple blood transfusions.

What group of people does thalassemia usually affect?

- Descendants of or people living near the Mediterranean Sea, who have the highest incidence of the disease, namely Italians, Greeks, and Syrians. -However, the disorder has a wide geographic distribution, probably as a result of genetic migration through intermarriage or possibly as a result of spontaneous mutation.

What are other therapeutic measures for hemophilia patients?

-A regular program of exercise and physical therapy is an important aspect of management. -Treatment without delay results in more rapid recovery and a decreased likelihood of complications; therefore most children are treated at home. -The family is taught the technique of venipuncture and to administer the AHF to children older than 2 to 3 years. -The child learns the procedure for self-administration at 8 to 12 years of age. -Primary prophylaxis in hemophilia patients has proved to be effective in preventing bleeding complications by administrating periodic factor replacement.

Why may a splenectomy be necessary for children with severe splenomegaly?

-A splenectomy may be necessary to decrease the disabling effects of abdominal pressure and to increase the life span of supplemental RBCs. -After a splenectomy, children generally require fewer transfusions, although the basic defect in Hgb synthesis remains unaffected

What is RICE?

-A supportive measurement for controlling bleeding. -Stands for rest, ice, compression, and elevation.

Clinical Manifestations of β-Thalassemia?

-Before diagnosis: Anemia • Pallor • Unexplained fever • Poor feeding • Enlarged spleen or liver -Progressive Anemia • Signs of chronic hypoxia which are: •Headache •Precordial and bone pain •Decreased exercise tolerance •Listlessness •Anorexia -Bone changes (Older Children If Untreated) • Enlarged head • Prominent frontal and parietal bossing • Prominent malar eminences • Flat or depressed bridge of the nose • Enlarged maxilla • Protrusion of the lip and upper central incisors and eventual malocclusion • Generalized osteoporosis -Other features • Small stature • Delayed sexual maturation • Bronzed, freckled complexion (if not receiving chelation therapy)

What are three manifestations of SCD that may appear in the first 2 years old life? What are they predictors of?

-Dactylitis, severe anemia, leukocytosis -an be predictors of disease severity

Symptoms of a bleed?

-Headache -Slurred speech -Loss of consciousness -Black tarry stools (rectal bleed)

What are the general guideline of a blood transfusion? (Nurse's role)

-ID blood with blood bank staff, check ID of child. -Vital signs before and 15 minutes into transfusion, then hourly. -Administer first 50 mL of blood, or 20% of NS while staying with child. -Administer blood through appropriate filter (gently shake container frequently) -Use blood within 30 minutes of arrival -Infuse specified amount within 4 hours -Observe for rash or other reactions: Stop infusion, re-assess vitals, keep IV line open with normal saline.

What is the onset of thalassesmia major?

-It may be insidious and not recognized until the latter half of infancy.

What is involved in the management of pain in children? What is the issue with it?

-Management of pain is an especially difficult problem and often involves experimenting with various analgesics, including opioids, and schedules before relief is achieved. -Unfortunately, these children tend to be undermedicated, resulting in their "clock watching" and demands for additional doses sooner than might be expected. -Often this incorrectly raises suspicions of drug addiction, when in fact the problem is one of improper dosage

What are the four types of thalassemia?

-Minor: asymptomatic silent carrier -Trait: Mild microcytic anemia -Intermediate: Moderate to serve anemia plus splenomegaly -Major (also known as Cooley's anemia): severe anemia necessitating transfusions to survive

What may recurrent epistaxis indicate?

-Recurrent epistaxis and severe bleeding may indicate an underlying disease, particularly vascular abnormalities, leukemia, thrombocytopenia, and clotting factor deficiency diseases (e.g., hemophilia, vWD). -Nosebleeds are sometimes associated with administration of aspirin, even in normal amounts.

Care management of Epistaxis?

-Remain calm, keep child calm. Otherwise, the child will become more agitated, the blood pressure will increase, and the child will not cooperate. -Have child sit up and lean forward -Apply pressure to the lateral aspect of the affect side of the nose, against bony prominences. -Ice pack can help to close vessel -Evaluate further if bleeding continues.

Care management of Hemophilia?

-The earlier a bleeding episode is recognized, the more effectively it can be treated. -Signs that indicate internal bleeding are especially important to recognize. -Children are aware of internal bleeding and are reliable in telling the examiner where an internal bleed is.

What is the primary therapy for hemophilia?

-The primary therapy for hemophilia is replacement of the missing clotting factor. -The products available are factor VIII concentrates, either produced through genetically engineering (recombinant) or derived from pooled plasma, which are reconstituted with sterile water immediately before use.

What is Von Willebrand disease?

-Von Willebrand disease (vWD) is another hereditary bleeding disorder characterized by a deficiency, abnormality, or absence of the protein called von Willebrand factor (vWF) and a deficiency of factor VIII. -Unlike hemophilia, vWD affects both males and females.

What is thalassemia?

-a common genetic disorder, affecting as many as 15 million people. -applied to a variety of inherited blood disorders characterized by deficiencies in the rate of production of specific globin chains in Hgb.

List 3 essential guidelines for the effective administration of oral iron supplements to small children.

1. Administer between meals 2. Administer with a fruit juice 3. Administer through a straw or with a medication syringe

What are some medical management goals during a crisis of SCD?

1. Rest- to minimize energy expenditure and improve oxygen utilization 2. Hydration through oral and IV therapy 3. Electrolyte replacement because hypoxia results in metabolic acidosis 4. Analgesia for severe pain 5.blood replacement to treat anemia and reduce viscosity 6. antibiotics to treat any existing infection

When both parents have sickle cell trait, there is a ____%chance of each pregnancy producing an offspring with SCA

25%

What is aplastic anemia?

A bone marrow failure condition in which the formed elements of the blood are simultaneously depressed.

What is a curative treatment for children with β-Thalassemia?

A curative treatment for some children is HSCT (Hematopoietic Stem Cell Transplant). Children younger than 16 years who undergo allogeneic HSCT have a high rate of complication-free survival; approximately 80% of these children are cured

What is hemophilia?

A group of bleeding disorders in which there is a deficiency of one of the factors (proteins) necessary for coagulation of the blood.

What is a major postsplenectomy complication?

A major postsplenectomy complication is severe and overwhelming infection. Therefore these children continue to receive prophylactic antibiotics with close medical supervision for many years and should receive the pneumococcal and meningococcal vaccines in addition to the regularly scheduled immunizations.

Name some complications of sickle cell anemia

A result of RBC "sickling" is increased blood viscosity, obstruction of blood flow, tissue hypoxia which causes pain, decreased RBCs

What is Disseminated Intravascular Coagulation?

A serious disorder in which the proteins that control blood clotting become overactive.

What is Sickledex?

A sickle-turbidity test conducted on infants to detect SCD, which can be done by finger stick and results are available in 3 minutes. If a Sickledex is positive, an Hgb electrophoresis test is necessary to distinquish between children with the trait and those with the disease.

At birth the healthy full-term newborn has maternal stores of iron sufficient to last: A. 5 to 6 months B. 2 to 3 months C. 8 months D. less than 1 month

A. 5 to 6 months

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? (SATA) A. Press the nares together for at least 10 minutes. B. Breathe through the nose until it stops bleeding C. Pack cotton or tissue into the nares that is bleeding D. Apply a warm cloth across the bridge of nose E. Insert petroleum into the naris after the bleeding stops.

A. Pressing the nares together for at least 10 minutes is an appropriate action to take when managing an episode of epistaxis. C. Packing cotton or tissue into the nares that is bleeding is an appropriate action when managing an episode of epistaxis.

A nurse is providing teaching about the management of epistaxis to a child and his family. Which of the following positions should the nurse instruct the child to take when experiencing a nosebleed? A. Sit up and lean forward B. Sit up and tilt head up C. Lie in a supine position D. Line in a prone position

A. The nurse should instruct the child to sit up and lean forward to prevent aspiration when experiencing a nosebleed.

A common term used in describing an abnormal CBC is shift to the left, which is usually caused by a(n): A. infection B. anemia C. hemolysis D. bleeding

A. infection

A 5-year old with sickle cell anemia is admitted because of diminished RBC production triggered by a viral infection. The episode is characterized by distal ischemia and pain. The crisis the child is most likely to be experiencing is: A. Vasooclusive crisis B. Splenic Sequestration crisis C. Aplastic crisis D. Hyperhemolytic crisis

A. vasooclusive crisis

What should you advise parents on dehydration?

Advise parents to be particularly alert to situations in which dehydration may be a possibility, such as hot weather, and to recognize early signs of reduced intake, such as decreased urinary output (e.g., fewer wet diapers) and increased thirst.

Which ethnic group is most affected by sickle cell disease?

African-American

What should you do after the child has a nose bleed?

After a nosebleed, petroleum or water-soluble jelly can be inserted into each nostril to prevent crusting of old blood and to lessen the likelihood of the child's picking at the nose and restarting the hemorrhage.

Are levels of RBCs, Hgb and Hct increased or decreased in patients with Iron deficiency anemia?

All decreased

What is the prognosis of hemophilia?

Although there is no cure for hemophilia, its symptoms can be controlled and its potentially crippling deformities greatly reduced or even avoided. Today many children with hemophilia function with minimal or no joint damage.

What is vaso-occlusive crisis?

An episode of SCA known as "painful episode" that causes severe pain in joints, abdomen, hematuria, visual disturbances

Clinical manifestations of AA?

Anemia, leukopenia, and decreased platelet count, is usually insidious

What is the principal drug treatment used for AA?

Antilymphocyte globulin (ALG) or antithymocyte globulin (ATG)

Is aplastic anemia congenital or acquired?

Aplastic anemia can be primary (congenital, or present at birth) or secondary (acquired).

A serious complication of sickle cell crisis is ACS. What is that and name some symptoms.

Acute chest syndrome. It mimics pneumonia with symptoms like chest pain, fever, cough, tachypnea, wheezing and hypoxia

A common childhood anemia that occurs more frequently in toddlers between the ages of 12 and 36 months is__________.

iron deficiency

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

B. The hemoglobin electrophoresis test should be performed to distinguish if the infant has the trait or the disease.

A nurse is preparing to administer iron dextran IM to a school age child who has an iron deficiently anemia. Which of the following actions by the nurse is appropriate? A. Administer the dose in deltoid muscle. B. use a SZ-track method when administering the dose C. Avoid injecting more than 2ml with each dose. D. Massage the injections site for 1 minute after administering

B. The nurse should use the Z-track method when administering the dose.

Why does DIC occur?

DIC is a secondary disorder of coagulation that occurs as a complication of a number of pathologic processes, such as hypoxia, acidosis, shock, and endothelial damage.

When is DIC suspected?

DIC is suspected when the patient has an increased tendency to bleed. Hematologic findings include prolonged prothrombin time, PTT, and thrombin time. There is a profoundly depressed platelet count, fragmented RBCs, and depleted fibrinogen.

In controlling sever pain related to vasooclusive sickle cell crisis, the plan of care will most likely include: A. administration of long-term oxygen B. Application of cold compresses to the area C. Intramuscular meperidine (Demerol) D. Intravenous or oral opioids

D. Intravenous or oral opioids

How is AA diagnosed?

Determined from bone marrow examination, which demonstrates the conversion of red bone marrow to yellow, fatty bone marrow

Therapeutic management of sickle cell crisis generally includes: A. long term oxygen use to enable the oxygen to reach sickled RBCs B. An increase in activity to promote circulation in the affected area C. A diet high in iron to decrease anemia D. Oral or intravenous hydration for hemodilution

D. Oroal or intravenous hydration for hemodilution

A nurse is providing teaching to the parent of a child who has a new prescriptions 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 emergency department if his tools become dark". B. "My child should avoid eating citrus fruits while taking the supplements". C. "I should give the iron with milk to prevent an upset stomach. D. "My child should take the supplement through a straw."

D. The child should take the supplement through a straw to prevent staining of the teeth.

Treatment for the child with aplastic anemia will most likely include: A. administration of testosterone B. administration of iron-chelating agents C. irradiation D. bone marrow transplant

D. bone marrow transplant

Name some dietary sources of iron

Dried beans, lentils, peanut butter, green leafy vegetables, poultry, red meat,

TRUE or FALSE A child with nose bleed should be instructed to sit up and title head back while holding pressure on nose

False- child should tilt head forward so blood does not get swallowed

True or False: In about 80% of all cases of hemophilia, the inheritance pattern is demonstrated as X-linked dominant.

False: In about 80% of all cases of hemophilia, the inheritance pattern is demonstrated as X-linked recessive.

What is the best-known congenital disorder of which AA is an outstanding feature

Fanconi syndrome, a rare hereditary disorder characterized by pancytopenia, hypoplasia of the bone marrow, and patchy brown discoloration of the skin resulting from the deposit of melanin and associated with multiple congenital anomalies of the musculoskeletal and genitourinary systems.

What is hemarthrosis?

Hemarthrosis, which is bleeding into a joint space, is the most frequent type of internal bleeding.

What is hemostasis?

Hemostasis is the process that stops bleeding when a blood vessel is injured.

What is hypoplastic anemia?

Hypoplastic anemia is characterized by a profound depression of RBCs but normal or slightly decreased white blood cells (WBCs) and platelets.

What are potential complications of frequent blood transfusions?

Iron overload (hemosiderosis). Because the body has no effective means of eliminating the excess iron, the mineral is deposited in body tissues.

When should iron supplements be given for best absorption?

Iron should be given 1 hour before or 2 hours after milk or antacids.

What is the prognosis of β-Thalassemia?

Most children treated with blood transfusion and early chelation therapy survive well into adulthood.

What are side effects associated with chemotherapy for AA?

Nausea and vomiting, alopecia, and mucositis

What is Epistaxis?

Nose bleeding

What is pancytopenia?

Pancytopenia is a medical condition in which there is a reduction in the number of red and white blood cells, as well as platelets.

Clinical manifestations of DIC?

Petechiae • Purpura • Bleeding from openings in the skin such as: •Venipuncture site •Surgical incision • Bleeding from umbilicus, trachea (newborn) • Evidence of gastrointestinal bleeding • Hypotension • Organ dysfunction from infarction and ischemia

What preventative treatments should be given to patients with SCD?

Pneumococcal and meningococcal vaccines, influenza vaccine, oral penicillin prophylaxis by age 2 to reduce risk of pneumococcal sepsis

What are the clinical effects of thalassemia major?

Primarily attributable to (1) defective synthesis of HbA, (2) structurally impaired RBCs, and (3) shortened life span of erythrocytes

What should the nurse instruct her patient/family about oral iron supplements?

Take with straw to prevent teeth staining

Define Anemia

Reduction of RBCs or Hgb concentration to levels below normal for age

Why can sickle cell crisis cause a CVA?

Sickled cells block major blood vessels in the brain, resulting in cerebral infarction, which causes impairment.

Signs of hemarthrosis?

Signs of hemarthrosis are swelling, warmth, redness, pain, and loss of movement.

Name some expected findings of Iron deficient anemia

Tachycardia Pallor Brittle, spoon shaped fingernails fatigue, irritability, muscle weakness systolic heart murmur

What are advantages of transfusions for β-Thalassemia?

The advantages of this therapy include: (1) improved physical and psychologic well-being because of the ability to participate in normal activities (2) decreased cardiomegaly and hepatosplenomegaly, (3) fewer bone changes (4) normal or near-normal growth and development until puberty (5) fewer infections.

What is included in the care management of a child with AA?

The care of the child with AA is similar to that of the child with leukemia and includes preparing the child and family for the diagnostic and therapeutic procedures, preventing complications from the severe pancytopenia, and emotionally supporting them in the face of a potentially fatal outcome.

How is hemophilia diagnosed?

The diagnosis is usually made from a history of bleeding episodes, evidence of X-linked inheritance (only one third of the cases are new mutations), and laboratory findings.

What are the most common causes of death with β-Thalassemia?

The most common causes of death are heart disease, postsplenectomy sepsis, and multiple-organ failure secondary to hemochromatosis

What causes epistaxis?

The nose, especially the septum, is a highly vascular structure, and bleeding usually results from direct trauma, including: -Blows to the nose -Foreign bodies -Nose picking, -Mucosal inflammation associated with allergic rhinitis -Upper respiratory tract infections.

What is the objective of supportive therapy of β-Thalassemia?

The objectives of supportive therapy are to maintain sufficient Hgb levels to prevent bone marrow expansion and the resulting bony deformities and to provide sufficient RBCs to support normal growth and normal physical activity.

Why is AA treatment focused on restoring the function to the marrow?

The objectives of treatment are based on the recognition that the underlying disease process is failure of the bone marrow to carry out its hematopoietic functions. Therefore therapy is directed at restoring function to the marrow.

What are the two most common forms of hemophilia?

The two most common forms of the disorder are factor VIII deficiency (hemophilia A, or classic hemophilia) and factor IX deficiency (hemophilia B, or Christmas disease).

How do you minimize the development of hemosiderosis?

To minimize the development of hemosiderosis, the oral iron chelator deferasirox has been shown to be a safe equivalent to deferoxamine (Desferal)

What are some therapeutic management for β-Thalassemia?

Transfusions are the foundation of medical management with the goal of maintaining the Hgb level above 9.5 g/dL, an aim that may require transfusions as often as every 3 to 5 weeks.

Name some risk factors for Epistaxis

Trauma, blunt Picking or rubbing Low humidity Allergic rhinitis Foreign body in nose

Therapeutic management for DIC?

Treatment of DIC is directed toward control of the underlying or initiating cause, which in most instances stops the coagulation problem spontaneously.

True or False Iron should be given with Vitamin C

True

The best dietary sources of iron for a 7 month old infant are? I. Whole Milk II. Oral iron supplements III. Iron fortified rice cereal IV. Iron-fortified commercial formula a. I and II b. III and IV c. I,II, III, and IV

b. III and IV

Primary prophylaxis in hemophilia patients involves the infusion of factor VIII: a. regularly at the emergency room before joint damage occurs b. regularly at home before the onset of joint damage c. whenever bleeding into a joint occurs d. when bleeding begins to impair joint function

b. regularly at home before the onset of joint damage

An acquired hemorrhagic disorder characterized by excessive destruction of platelets and a discoloration caused by petechiae beneath the skin with normal n=bone marrow is _______.

immune thrombocytopenia

Children who develop moon face from short term steroid therapy used to treat cancer may experience symptoms of: a. acute toxicities b. decreased appetite c. permanent facial change d. altered body image

d. altered body image

What is sequestration in SCA?

excessive pooling of blood, primarily in the spleen, which causes reduced circulation

Why are adolescents at high risk for iron deficiency?

poor diet, rapid growth, menses, strenuous exercise, obesity

Name some risk factors for iron deficiency anemia

premature birth excessive intake of cow's milk in toddlers malabsorption disorders poor dietary intake of iron rich foods increased iron requirements (due to blood loss)

Describe some symptoms of acute vasooclusive crisis

severe pain in joints, bones, and abdomen, swollen hands and feet, hematuria, visual disturbances

Name some risk factors for epistaxis

trauma( nose picking) low humidity, allergic rhinitis, URI foreign body in the nose medications that effect clotting factors

What are common causes of Acquired Aplastic Anemia?

• Human parvovirus infection, hepatitis, or overwhelming infection • Irradiation • Immune disorders such as hypoimmunoglobulinemia and eosinophilic fasciitis • Drugs such as certain chemotherapeutic agents, anticonvulsants, and antibiotics • Industrial and household chemicals, including benzene and its derivatives, which are found in petroleum products, dyes, paint remover, shellac, and lacquers • Infiltration and replacement of myeloid elements, such as in leukemia or the lymphomas • Idiopathic (In most cases, no identifiable precipitating cause can be found.)

What are the signs of CVA (stroke)?

•Severe, unrelieved headaches •Severe vomiting •Jerking or twitching of the face, legs, or arms •Seizures •Strange, abnormal behavior •Inability to move an arm or leg •Stagger or an unsteady walk •Stutter or slurred speech •Weakness in the hands, feet, or legs •Changes in vision