Peds Exam 2: Hematology

Blood clotting. The extremely complex clotting mechanism can be combined into 3 basic steps:

(1) release of clotting factors from both injured tissue cells and sticky platelets at the injury site (which form a temporary platelet plug) (2) a series of chemical reactions that eventually result in the formation of thrombin; and (3) formation of fibrin and trapping of RBCs to form a clot.

CARE MANAGEMENT OF SICKLE CELL CRISIS

***Educate the family and child: A. Seek early intervention for fever B. Give penicillin as ordered C. Recognize signs and symptoms of splenic sequestration ***Provide supportive therapies during crisis ***Meet psychosocial needs of the family

Data to support iron deficiency anemia: Lab data

**Know normal values in order to see when deficits occur -CBC: Decreased RBC, Hgb., Hct. -RBC: Decreased indicating Microcytic/hypochromic RBC's -Reticulocyte count: May be decreased indicating bone marrow production of rbcs -Decreased serum ferritin level (indicator of iron stores) -Total iron binding: ELEVATED Lab Data: Chart in ATI pg. 220??

PROGNOSIS OF SICKLE CELL ANEMIA

**No cure (except possibly bone marrow transplantation). -Supportive care/prevention of sickling episodes. -Frequent bacterial infections because of immunocompromised state ******Bacterial infection: leading cause of death in young children with sickle cell disease. -Usual life span: into the fifth decade.

What are dietary sources of iron

*For infants: iron fortified cereals and formula *Older children: dried beans and lentils, peanut butter, green, leafy veggies, iron fortified breads and flour, red meat

Sickle Cell Anemia

*One of a group of diseases termed hemoglobinopathies *Partial or complete replacement of normal Hgb. with abnormal Hgb. **A group of disorders that cause red blood cells to become misshapen and break down. Sickle cell diseases include: -Sickle cell anemia -Sickle cell-C disease -Sickle cell-hemoglobin E disease -Sickle cell ischemia disease

PROGNOSIS OF HEMOPHILIA/ complications:

*Prognosis: -Mild to moderate hemophilia: patients live near-normal lives -Control of symptoms and limiting joint damage improve quality of life Complications: A. Uncontrolled Bleeding: Intracranial, airway obstruction from bleeding in mouth, neck or chest B. Joint Deformity: Repeated episodes of hemarthrosis

Red blood cell disorders: Anemia

*The most common hematologic disorder of childhood *Decrease in number of RBCs or hemoglobin (Hgb) concentration below normal, or both *Decreased oxygen-carrying capacity of blood *This diminishes the O2-carrying capacity of the blood, causing a reduction in the oxygen available to tissues.

EPITAXIS

*nose bleeds -short, Isolated and transient/acute epistaxis is common in childhood -Although epistaxis is rarely an emergency, it causes anxiety for the child and the parents -Recurrent or severe episodes may indicate underlying disease (vascular abnormalities: leukemia, idiopathic thrombocytopenia purpura) or (Clotting factor deficiency disease: hemophilia, , von willebrand disease, ) **The nose (especially the septum) is highly vascular; bleeding results from direct trauma, including direct blows to the nose, foreign bodies, and nose picking, rubbing, or from mucosal inflammation associated with rhinitis and URIs

Hemophilia

-A group of hereditary bleeding disorders that result from deficiencies of specific clotting factors and difficulty in controlling bleeding -a medical condition in which the ability of the blood to clot is severely reduced, causing the sufferer to bleed severely from even a slight injury. The condition is typically caused by a hereditary lack of a coagulation factor, most often factor VIII....blood doesnt clot normally *In 80% of cases, the inheritable pattern is X-linked recessive -Identification of a specific factor deficiency allows for definitive treatment

Iron Deficiency Anemia: Home care

-Anticipatory Guidance: -Iron Supplements: Teach about Iron Administration A. Give 1 hr. before or 2 hr. after milk or antacid to prevent decreased absorption *If tolerated, give on empty stomach to promote absorption. Give with meals and start with reduced dose and gradually increasae if GI distress occurs B. GI side effects: Diarrhea, constipation, nausea *GI upset common with start of therapy but decrease over time. *Educate child and family to expect stools to turn a tarry green color if dose is adequate. C. Vitamin C helps absorption D. Use straw with liquid administration and rinse mouth to prevent staining of teeth. E. May be given IM (Z-track method) *IM admin reserved for children who have iron malabsorption or chronic hemoglobinuria. (Do not massage post injection to prevent skin staining and irritation) -Increase fiber and fluids to prevent constipation -Promote dietary sources of iron **May take 3 months to correct low Hgb. *Prognosis is very good but if severe or chronic may cause cognitive, behavioral, and motor impairment Education: educate the child and family to expect stools to turn a tarry green color if dose is adequate *Instruct child to brush teeth after oral dose to minimize or prevent straining -increase fiber and fluids to prevent constipation

Sickle Cell Crisis: Precipitating factors/ labs

-Anything that increases body's need for oxygen or alters transport of oxygen -Trauma -Infection, fever -Physical and emotional stress -Increased blood viscosity caused by dehydration -Hypoxia *HGB: decreased *WBC: elevated *Bilirubin and reticulocyte(an immature red blood cell) levels: elevated *Peripheral blood smear: revels sickled cells

Cause of sickle cell anemia

-Autosomal recessive disorder *9% of African Americans are carriers (have sickle cell trait) *40% of native Africans are carriers **If both parents have trait, each of their children has a 25% chance of having disease Clinical Features: -Obstruction caused by sickled RBCs -vascular inflammation -Increased RBC destruction -Abnormal adhesion, entanglement, and meshing of rigid sickle-shaped cells -Local hypoxia -Cellular death

Iron Deficiency Anemia: Nursing Care

-Collaborative care -Iron Supplements for pre term 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 -Recommend Iron-fortified formula for infants when solids are introduced -Diet: include iron, vitamin C, and protein as child grows -Restrict milk in toddlers: Limit milk intake to 32 oz. per day; delay giving milk until AFTER a meal; DO NOT allow toddlers to carry bottles or cups of milk. -encourage intake of iron rich foods -Frequent rest periods -May require packed RBC administration ***Provide iron supplements for preterm and LBW infants by 2m of age and to exclusively breastfed infants by 4m of age.

Key Factors of Hemophilia

-Impaired ability to control bleeding. -Lack of clotting factors -Internal or External bleeding -May not see signs until infant teeths, sits up or crawls. -Parents observe bruising with minor falls or contact

THERAPEUTIC MANAGEMENT OF SICKLE CELL CRISIS

-Possible prophylactic antibiotics for 2 months to 5 years -Monitoring of reticulocyte count regularly to evaluate bone marrow function -Blood transfusion: if given early in crisis, may reduce ischemia **Treat the medical emergencies of sickle cell crisis: -Rest to minimize energy loss/ oxygen consumption -Hydration through oral or IV therapy -Electrolyte replacement -Analgesia for pain -Blood replacement for anemia -Antibiotics for infection -Monitor lab results

CARE MANAGEMENT OF EPISTAXIS

-Remain calm; keep child calm keep the family calm -Bleeding usually stops within 10 minutes after nasal pressure -Have child sit up with the head titled slilghly foward to prevent aispiration of blood -Apply pressure to the soft lower part of the nose with the thumb and forefinger for at least 10 mins -if needed, pack cotton or tissue into the side of the nose that is bleeding -encourage the child to breathe through her mouth while her nose is bleeding -apply ice across the bridge if nose bleeding continues -Evaluate further if bleeding continues -Bleeding usually stops spontaneously or with minimal pressure and requires no medical eval. or therapy. **Persistent episodes of epistaxis require medical attention (repeated episodes lasting longer than 30 minutes).

Name 9 Risk factors for iron deficiency anemia

1. Prematurity (premature birth resuluts in decreased iron stores) 2. Toddlers: Excessive intake of cow's milk *milk IS NOT a good source of iron so its a problem when milk takes the place of iron rich solid foods) 3. Prolonged diarrhea 4. Poor dietary intake of iron 5. Adolenscents: poor diet, rapid growth, menses, strenuous activities, and obesity 6. increased iron requirements (Blood loss) 7. Infection 8.Chronic disorders: (ie. Sickle cell, hemophilias, folate) 9. Malabsorption

1.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 the head up. c. lie in a supine position. D. lie in a prone position.

A 1. .a.CORRECT:the nurse should instruct the child to sit up and lean to prevent aspiration when experiencing a nosebleed. B. sitting up and tilting the head up could cause aspiration of blood and is not an appropriate position when experiencing a nosebleed. c. lying in a supine position could cause aspiration of blood and is not an appropriate position when experiencing a nosebleed. D. lying in a prone position could cause aspiration of blood and is not an appropriate position when experiencing a nosebleed.

2.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,C, 2. a.CORRECT: Pressing the nares together for at least 10 min is an appropriate action to take when managing an episode of epistaxis. B. the child should breathe through the mouth until the bleeding stops. c.CORRECT: Packing cotton or tissue into the naris that is bleeding is an appropriate action when managing an episode of epistaxis .D. applying an ice pack across the bridge of the nose is an appropriate action when managing an episode of epistaxis. e. inserting petroleum into the naris after the bleeding stops is not an appropriate action when managing an episode of epistaxis

When assessing a child for anemia why is it important to know their age? Race?

A. Age provides some clues regarding possible cause of the anemia. (i.e. iron deficiency anemia (IDA) occurs more frequently in toddlers between 12 and 36 months and during the growth spurt of adolescence. ) * are at risk for anemia as a results of primarily cow's milk intake and not eating an adequate amount of iron-containing food. *-Adolescents at risk because of rapid growth and poor eating habits, menses, obesity, or strenuous activities B. : Certain anemias are more common in particular races (Southeast Asians and persons of African or Mediterranean ancestry).

Care Management of anemia

A. Assessment: -Age of the child -Racial or ethnic background -Careful history: "my child drinks lots of milk" (frequent finding in toddlers with IDA.) -Stool for occult blood: test for chronic intestinal bleeding B. Prepare child and family for laboratory tests C. Decrease tissue oxygen needs: O2 may be needed to reduce tissue hypoxia, prevent infection D. Prevent complications: Immunocompromised, observe for s/s of infection (temp)

TECHNOLOGIC MANAGEMENT OF HEMATOLOGIC DISORDERS

A. Blood transfusion therapy: -Verify identity of recipient and donor's blood group -Monitor vital signs -Use appropriate filter -Use blood within 30 minutes of its arrival -Infuse over 4 hours maximum -Monitor for transfusion reaction

SICKLE CELL ANEMIA: COMPLICATIONS

A. CVA: Monitor for Signs and Symptoms and report *Manifestations of CVA: seizures, abnormal behavior, weakness of or inability to move an extremity, slurred speech, visual changes, vomiting, severe headache. *educate client that blood transfusions are usually every 3 to 4 weeks to prevent CVA B. Acute Chest Syndrome: -Can be Life-threatening!! **Symptoms: Chest, back, or abdominal pain, fever (101.3 F or higher), congested cough, tachypnea, dyspnea, retractions, decreased O2 saturation.

Anemia: Causes, Morphology, and diagnostic evaluation

A. Causes and physiology: Depletion of RBCs or Hgb, or both B. Morphology: Characteristic changes in RBC size, shape, or color, or a combination of these *Morphology is important for diagnosis of type of anemia. C. Diagnostic evaluation: Sometimes defined as Hgb <10 or 11 g/dL; however, this cutoff may be inappropriate for children (levels vary with age) *suspected anemia may be based on Hx and PE; unless severe first clue may be alterations in the CBC (hence screenings).

What are factors that would indicate hematologic and immunologic dysfunction?

A. Complete blood cell count **CBC performed to assess hematologic function. B. History and physical examination History and physical exam are essential for identifying hematologic dysfunction C. Comments by the parent regarding child's lack of energy *Comments from parents that offer clues may include: Lack of energy, food diary of poor sources of iron, frequent infections, and bleeding that is difficult to control D. Food diary of poor iron sources E. Frequent infections F. Bleeding *The nurse if often the first person to suspect a problem.

Classifications of anemia: Decreased red blood cell production -s/s -types

A. Decreased red blood cell production s/s: pallor, tachycardia/headache, muscle weakness, systolic heart murmur *Pica (eating clay, paper, paste) Types: i. Nutritional Deficiency: iron, folate, b 12, copper, chronic disease, chronic blood loss ii. Bone marrow failure: Aplastic anemia, red cell aplasia, malignancy, ALL/neuroblastoma infection (parvovirus, CMV)

Effects of anemia on the circulatory system

A. Hemodilution: increase in fluid content of blood (plasma) in relation to RBCs, resulting in lowered concentration of formed elements/ RBCs in the circulation. *Hemodilution of severe anemia > decreased peripheral resistance > greater quantities of blood to return to the heart > increased circulation and turbulence within the heart may produce a murmur > increased cardiac workload (usually during exercise, infection, or emotional stress) > may lead to cardiac failure. B. Decreased peripheral resistance C. Increased cardiac circulation and turbulence -Such increases may produce a murmur -Cardiac failure may ensue D. Cyanosis *Children may function well despite low levels of Hgb. Cyanosis is typically not evident. E. Growth retardation *Growth retardation is a common finding in chronic severe anemia and often accompanies delayed sexual maturation in older children -When anemia develops slowly the child usually adapts to declining Hgb level. -Effects of anemia can be very profound on the circulatory system. *Viscosity of blood depends almost entirely on the concentration of RBCs.

Transfusion reactions

A. Hemolytic: the most severe, but rare B. Febrile reactions: fever, chills C. Allergic reaction: urticaria, pruritus, laryngeal edema D. Air emboli: may occur when blood is transfused under pressure E.Hypothermia

Types of hemophilia

A. Hemophilia A -"Classic hemophilia" -Deficiency of factor VIII -accounts for 80% of cases B. Hemophilia B -Also known as "Christmas disease" -Caused by deficiency of factor IX C. Von Willebrand disease Deficiency of von Willebrand factor and factor VIII

EVALUATION OF HEMOPHILIA

A. History of bleeding episodes -Overt prolonged bleeding -Hemarthrosis: is a bleeding into joint spaces. -Ecchymosis: a discoloration of the skin resulting from bleeding underneath, typically caused by bruising B. X-linked inheritance C. Laboratory findings -Low levels of factor VIII or IX, prolonged partial thromboplastin time -Normal: platelet count, parathormone level, and fibrinogen level ***DNA testing: detects classic hemophilia trait in females

Preparation and Nursing care of a patient with Anemia

A. Preparation: nurse is responsible for (1) explaining significance of each test, particularly why the test are not done all at once; (2) encouraging parents or another supportive person to be with the child during the procedure; (3) allowing the child to play with the equipment on a doll or participate in the actual procedure. B. Nursing care: assess the child's energy level and minimize excess demands (assess child's level tolerance for ADLs and play). Assess VS during rest and activity for comparison. (handwashing, selecting a room in a noninfectious area, restricting visitors or hosp. personnel with active infection, maintaining adequate nutrition -O2 may be needed to reduce tissue hypoxia, prevent infection -Immunocompromised, observe for s/s of infection (temp)

Care Management of hemophilia

A. Prevent bleeding -Safe environment -Dental hygiene B. Recognize and control bleeding -RICE C. Prevent the crippling effects of bleeding D. Support the family and home care -Genetic counseling -Support groups **Padded crib, safe home and play environment free of clutter. **Set activity restrictions to avoid injury (low-contact sports). **Encourage use of soft bristled toothbrushes. **UTD immunizations ***Medical identification wristband or tags.

Data to support iron deficiency anemia: subjective vs objective

A. Subjective: *SOB *Dizziness or fainting with exertion *Fatigue, irritability, muscle weakness B. Objective: *Pallor *tachycardua *Brittle, spoon-shaped fingernails *Systolic heart murmur *Enlarged heart *****Complication: Heart Failure!!

Therapeutic management of anemia

A. Treatment of underlying cause -Transfusion after hemorrhage if needed -Nutritional intervention for deficiency anemias B. Supportive care -Intravenous (IV) fluids to replace intravascular volume -Oxygen therapy -Bed rest *Objective of medical management is to reverse the anemia by treating underlying cause and to make up for any deficiency. **For severe anemia, supportive care may include O2 therapy, bed rest, and replacement of intravascular volume with IV fluids. -Prognosis depends on the correction of the cause

THERAPEUTIC MANAGEMENT OF HEMOPHILIA

A.Replace missing clotting factors -Factor VII aggressive replacement therapy for hemorrhage -Home infusion B. Desmopressin (DDAVP) -IV administration or nasal spray -Causes two to four times' increase in factor VIII activity -Used for mild hemophilia and prior to dental or surgery

Hemophilia nursing care: Additional medications

Additional Medications: 1. Corticosteroids: Treats hematuria, hemarthrosis, chronic synovitis 2. NSAIDS treat chronic synovitis aka inflammation of a synovial membrane; administer cautiously due to potential inhibition of platelet function

What is the most common hematologic disorder of childhood?

Anemia

This diminishes the O2-carrying capacity of the blood, causing a reduction in the oxygen available to tissues.

Anemia *Decrease in number of RBCs or hemoglobin (Hgb) concentration below normal, or both

4.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 4.a. the nurse should administer the dose in to a large muscle mass .B.CORRECT:the nurse should use the Z‑track method when administering the dose. c. the nurse should avoid injecting more than 1 ml with each dose. D. to reduce irritation and skin staining, the nurse should not massage the injection site after administering the dose.

5.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 5.a. the sickle solubility test is a screening tool that detects the presence of abnormal hemoglobin, but does not distinguish between the trait and the disease. B.CORRECT:the hemoglobin electrophoresis test should be performed to distinguish if the infant has the trait or the disease. c. a complete blood count tests for anemia. it indicates the average size of the red blood cells, and the amount of hemoglobin in the red blood cells. it will not distinguish between sickle cell disease and sickle cell trait. D. the transcranial Doppler is performed to assess intracranial vascular flow and detect the risk for cerebrovascular accident. it will not distinguish between sickle cell disease and sickle cell trait.

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 FURTHER TEACHING IS NEEDED? A. "My child's stool will be dark with adequate dosing." B. "My child should not take the supplement through a straw." C. "My child should eat citrus fruits while taking the supplements." D. "I should not give the iron with milk to help prevent an upset stomach."

B.

Classifications of anemia: Increased red blood cell loss

Classifications of anemia: Increased red blood cell loss s/s: pallor, fatigue/headache, cool skin, tachycardia, decreased peripheral pulses, low blood pressure (late sign of shock) Type: i. Acute blood loss: epistaxis, hemophilia, hypersplenism, ITP, DIC

3.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 emergency department if his stools become 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."

D. 3 .a. the child's stools will become a tarry‑green color if the iron supplement dose is adequate. B.Vitamin c increases absorption of the iron and should be encouraged while taking the supplement. c. milk prevents absorption of the iron. the supplement should be given 1 hr before or 2 hr after consuming milk. D.CORRECT:the child should take the supplement through a straw to prevent or minimize staining of the teeth.

DEFECTS IN HEMOSTASIS: Coagulation process is complex and depends on these 3 factors.

Dysfunction in clotting mechanisms 1. Vascular influence 2. Role of platelets 3. Clotting factors

What would you use to definitively diagnose someone with sickle cell anemia

Hemoglobin (HGB) electrophoresis: separates the various forms of HGB and is the definitive diagnosis of sickle cell anemai

RBC Sickling =

Increased Blood viscosity!!! Which leads to Obstruction of blood flow and Tissue hypoxia *Tissue hypoxia causes tissue ischemia, which results in pain * Increased destruction of RBC occurs *Not apparent until late infancy due to presence of fetal Hgb.

Iron Deficiency anemia is caused by

Iron Deficiency anemia is caused by an inadequate supply of dietary iron, and is the most preventable mineral disturbances *Generally preventable: -Iron-fortified cereals and formulas for infants: Prevalence in infants has decreased probably due to screenings and WIC. -Special needs of premature infants *Preterm infants are particularly at risk due to reduced fetal iron supply; *Children age 12-36 months of age are at risk for anemia as a results of primarily cow's milk intake and not eating an adequate amount of iron-containing food. -Adolescents at risk because of rapid growth and poor eating habits, menses, obesity, or strenuous activities Most prevalent nutritional disorder in the U.S. and most preventable mineral disturbance.

________ is the most common anemia in the US

Iron deficiency anemia

Hemophilia: nursing care/ Home care

Nursing Care: A. Managing Bleeding **No rectal temps -Avoid unnecessary skin punctures and use surgical aspectic technique **5 minute pressure after injections, venipuncture, needle sticks -Monitor urine, stool, and masogastric fluid for occult blood **Control local bleeding: Administer factor replacement, Ice to joints, and immobilize them * administer injections via subQ instead of the intramuscular route whenever possible -venipunctures are preferred over finger or heel sticks for blood sampling *****DO NOT ADMINISTER ASPIRIN or products containing aspirin. Acetametapine is an appropriate substitute for aspirin Home Care: -make sure house is to prevent bleeding -up to date Immunizations -Support groups -limit activites that could cause injury. Acceptable activites include low contact sports...bowling, fishing, swimming, gold -WEAR MEDICAL ID -know manifestations of internal bleeding and hemarthrosis -To help control bleeding episodes RICE

Hemophilia labs

Objective: 1. Prolonged PTT 2. Factor specific assays to determine deficiency 3.Platelets and prothrombin time: normal range

SICKLE CELL CRISIS: VASO-OCCLUSIVE *Acute: What are the Nursing Priorities?

Oxygen, Fluids, Pain Management, Infection Prevention, Rest

Risk Factors for epistaxis/ expected findings

Risk Factors: * trauma, such as picking, 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 infections, blunt injury, or a foreign body in the nose can precipitate a nosebleed *medications that affect clotting factors can increase bleeding *Epistaxis can be the result of underlying diseases (von willebrand disease, hemophillia, idopathic thrombocytopenia purpura, leukemia) Expected findings: history of bleeding gums or blood in body fluids or stool -history of trauma, illness, allergies, or placing foreign bodies in ones nose Physical assessment findings: active bleeding nose, restlessness and agitation

What screening tool DETECTS HbS? What lab/diagnositcs would indicate a definitve diagnosis of sickle cell anemia?

Screening for sickle cell anemia in newborns is manditory in most states ***Sickledex : Screening tool detects the prescence of HbS ***Hgb electrophoresis: Definitive diagnosis of Sickle Cell Anemia *CBC would indicate anemia *Transcranial Doppler test: Assesses intracranial vascular flow and risk for CVA **People with Sickle Cell Anaemia have Sickle haemoglobin (HbS) which is different from the normal haemoglobin (HbA). When sickle haemoglobin gives up its oxygen to the tissues, it sticks together to form long rods inside the red blood cells making these cells rigid and sickle-shaped. Normal red blood cells can bend and flex easily.

SICKLE CELL ANEMIA: SUBJECTIVE/OBJECTIVE

Subjective: -Family history: Sickle cell anemia or trait -Reports pain: Due to tissue hypoxia and tissue ischemia -SOB/Fatigue Objective Physical symptoms: -Pallor, pale mucous membranes -Jaundice -Hands and feet cool to touch -Dizziness -Headache

Hemophilia: subjective/objective

Subjective: Reports episodes of bleeding, excessive bleeding, joint pain and stiffness, impaired mobility, easy bruising, and activity intolerance. Objective: 1. Active bleeding (gums, epistaxis, hematuria, and/or tarry stools) 2. Hematomas/bruising (even with minor injuries) 3. Hemarthrosis: is a bleeding into joint spaces. The presence of it excludes the Idiopathic thrombocytopenic purpura (ITP) 4. Headache, slurred speech, decreased level of consciousness

Epistaxis: client education and complications

Teaching: -For recurrences remind the child to sit up and slightly lean foward so blood does not flow down the throat and cause choking -inform the family that bleeding usually stops within ten mins Complications: -HEMORRHAGE *Nursing actions: Provide support to the child during cauterization (burning the skin to stop the bleeding and prevent infection) or packing **Education: Instruct child and family to seek medical care if bleeding lasts longer than 30 mins, and that repeated episodes require further evaluation for bleeding diseoders

Iron and hemoglobin

The production of hemoglobin (Hgb) requires iron. Irom deficiency will result in decreased Hgb levels *Iron Deficiency anemia is caused by an inadequate supply of dietary iron, and is the most preventable mineral disturbances

Unless severe, the first clue that a client may have anemia may be

alterations in the CBC (hence screenings)

Classifications of anemia: Increased red blood cell destruction

clasifications of anemia: Increased red blood cell destruction s/s: Iceteric sclera/pallor, fatigue/headache, dark urine, splenomegaly, hepatomegaly, frontal bossing Types: i. intracorpuscular: hemoglobinopathies (sickle cell disease, thalassemia) Enzymopathies (G6PD) membrane defects (hereditary spherocytosis) ii. Extracorpuscular: Immunologic (AIHA, isoimmunization) Drugs/toxic substances (chemotherapy, radiation infection)

Viscosity of blood depends almost entirely on the

concentration of RBCs

Blood disorders that can affect children include...

epitaxis, iron deficiency anemia, sickle cell anemia, and hemophilia

corticosteroids can be used to treat ....

hematuria, acute episodes of hemarthrosis and chronic synovitis *NSAIDS may also be used to treat chronic synovitis...take med with food *monitor for infection and bleeding *encourage family to maintain good hand hygiene and avoid individuals with colds/infections/viruses

SICKLE CELL CRISIS: VASO-OCCLUSIVE

is the exacerbation of SCA; usually last 4 to 6 days Acute: -Severe Pain: bones, joints, abdomen -Swollen Joints, hands, feet -Anorexia, vomiting, fever -Hematuria -Obstructive jaundice -Visual disturbances

Hemoarthrosis s/s with pic

joint pain, stiffness, warmth, swelling, redness, loss of ROM, and deformities.