Monday, March 9, 2009

Pediatric nursing: hematology

Hematology Lecture notes


Blood



Blood is the fluid of life


Blood is composed of:


Plasma


RBC


WBC


Platelets






Plasma

Plasma consists of:


90% water.


10 % solutes: albumin, electrolytes and proteins.


Proteins consist of clotting factors, globulins, circulating antibodies and
fibrinogen.





Red Blood Cells

RBC’s travel through the body delivering oxygen and removing waste.



RBC’s are red because they contain a protein chemical called hemoglobin
which is bright red in color.


Hemoglobin contains iron, making it an excellent vehicle for transporting
oxygen and carbon dioxide.




RBC’s


Average life cycle is 120 days.


The bones are continually producing new cells.


White Blood Cells


The battling blood cells.


The white blood cells are continually on the look out for signs of disease.


When a germ appears the WBC will:



Produce protective antibodies.


Surround it and devour the bacteria.



WBC’s


WBC life span is from a few days to a few weeks.



WBC’s will increase when fighting infection.


Platelets


Platelets are irregularly-shaped, colorless bodies that are present in
blood.


Their sticky surface lets them form clots to stop bleeding.



Blood Values


CBC with differential and platelet count.


Hgb:

Normal levels are 11 to 16 g / dl


Panic levels are:


Less than 5 g / dl


More than 20 g / dl


Hematocrit


Normal hematocrit levels are 35 to 44%.


Panic levels:

Hmct less than 15 %



Hmct greater than 60%


Hemoglobin and Hematocrit


Can be used as a simple blood test to screen for anemia.


The CBC with differential would be used to help diagnose a specific
disorder.


A bone marrow aspiration would be the most conclusive in determining cause
of anemia – aplastic / leukemia.


Bone Marrow


Bone marrow is the spongy substance found in the center of the bones.


It manufactures bone marrow stem cells, which in turn produce blood cells.



Red blood cells – carry oxygen to tissue


Platelets – help blood to clot


White blood cells – fight infection


Bone Marrow Transplant


Donor is placed under anesthesia.


Marrow is aspirated out of the iliac crest.


Marrow is filtered and treated to remove bits of bone and other unwanted
cells and debris, transferred to a blood bag, and is infused into the
patient’s blood just like at transfusion.


Bone Marrow Aspiration


Treatment Modalities


Transfusion:



Packed red blood cells – anemia


Platelets – platelet dysfunction


Fresh frozen plasma – coagulation factors


Blood Transfusions


3 types of transfusion reactions



Hemolytic


Allergic


Febrile



Hemolytic Reaction


Refers to an immune response against transfused blood cells.


Antigens, on the surface of red blood cells, are recognized as "foreign
proteins" and can stimulate B lymphocytes to produce antibodies to the red
blood cell antigens.



Hemolytic reaction


Flank pain


Fever


Chills


Bloody urine


Rash


Low blood pressure


Dizziness / fainting


Nursing Management


Stop the blood transfusion.


Start normal saline infusion.


Take vital signs with blood pressure


Call the MD


Obtain blood sample and urine specimen.


Return blood to blood bank.


Document


Febrile Reaction


Often occurs after multiple blood transfusions.


Symptoms:fever, chills, and diaphoresis.


Interventions:



Slow transfusion and administer antipyretic.


Administer antipyretic prior to administration.


Allergic Reaction


Symptoms: rash, urticaria, respiratory distress, or anaphylaxis.


Interventions:



administer antihistamine before transfusion


Physician may order washed rbc’s

Hematologic Conditions


Alteration in Hematologic Status


Disorders of hemostasis or clotting factors


Structural or quantitative abnormalities in the hemoglobin.


Anemias


Aplastic Anemia


Genetic Implications


The following have a genetic link: implications for genetic screening and
fetal diagnosis



Sickle cell anemia


Thalassemia


Hemophilia


Bleeding Disorders


Three types Hemophilia: males only



Type A most common – factor VIII deficiency


Type B - lack of factor IX (Christmas Disease)


Type C – lack of factor XI


Von Willebrand Disease – 1% of population – men or women – prolonged
bleeding time



Hemophilia Type A


Hemophilia type A is the deficiency of clotting factor VIII.



A serious blood disorder


Affects 1 in 10,000 males in the US


Autoimmune disorder with lowered level of clotting factor


All races and socio economic groups affected equally



Hemophilia

Hemophilia is a sex-linked hereditary bleeding disorder


Transmitted on the X chromosome


Female is the carrier


Women do not suffer from the disease itself


Historical Perspective


First recorded case in Talmud Jewish text by an Arab physician –
documentation of two brothers with bleeding after circumcision.


Queen Victoria is carrier and spread the disease through the male English
royalty.


Goals
of care


Provide factor VIII (IX) to aid blood in clotting.


To decrease transmission of infectious agents in blood products; hepatitis
& AIDS.


Future: gene therapy to increase production of clotting factor.


Symptoms


Circumcision may produce prolonged bleeding.


As child matures and becomes more active the incidence of bleeding due to
trauma increases

Symptoms



May be mild, moderate or severe


Bleeding into joint spaces, hemarthrosis


Most dangerous bleed would be intracranial.



Diagnosis

Presenting symptoms


Prolonged activated aPTT and decreased levels of factor VIII or IX.


Genetic testing to identify carriers



Treatment

Products used to treat hemophilia are:



Fresh frozen plasma and cryoprecipitate which are from single blood donors
and require special freezing.


Second generation of factor VIII are made with animal or human proteins.=


Nursing Diagnoses


Risk for injury


Pain with bleed especially into a joint


Impaired physical mobility


Knowledge deficit regarding disease and management of disease


Nursing interventions


No rectal temps.


Replace the factor as ordered by physician.


Manage pain utilizing analgesics as ordered.


Maintaining joint integrity during acute phase: immobilization, elevation,
ice.


Physical therapy to prevent flexion contraction and to strengthen muscles
and joints.


Provide opportunities for normal growth and development.


Teaching


Avoid aspirin which prolongs bleeding time in people with normal levels of
factor VIII.


A fresh bleeding episode can start if the clot becomes dislodged.


Natural reactions in the body cause the clot that is no longer needed to
"break down. This process occurs 5 days after the initial clot is formed.



Family Education


Medic-Alert bracelet


Injury prevention appropriate for age


Signs and symptoms of internal bleeding or hemarthrosis


Dental checkups


Medication administration


Long Term Complications


20% develop neutralizing antibodies that make replacement products less
effective.


Gene therapy providing continuous production of the deficient clotting
factor could be the next major advance in hemophilia treatment.


Disseminated Intravascular Coagulation or DIC



DIC is an acquired coagulopathy that is characterized by both thrombosis
and hemorrhage.



DIC is not a primary disorder but occurs as a result of a variety of
alterations in health.


Assessment


The most obvious clinical feature of DIC is bleeding.


Renal involvement = hematuria, oliguria, and anuria.


Pulmonary involvement = hemoptysis, tachypnea, dyspnea and chest pain.


Cutaneous involvement = petechiae, ecchymosis, jaundice, acrocyanosis and
gangrene.



Management of DIC


Treatment of the precipitating disorder.


Supportive care with administration of platelet concentration and fresh
frozen plasma and coagulation factors.


Administration of heparin (controversial in children).


Heparin potentates anti-thrombin III which inhibits thrombin and further
development of thrombosis.


Nursing Diagnoses


Altered tissue perfusion


Risk for injury

Anxiety



Nursing Interventions


Rigorous ongoing assessment of all body systems


Monitor bleeding


No rectal temps


Avoid trauma to delicate tissue areas


All injections sites and IV sites need to be treated like an arterial
stick.


Prognosis


Depends on the underlying disorder and the severity of the DIC.



ITP


Idiopathic thrombocytopenic purpura

Idiopathic = cause is unknown


Thrombocytopenic = blood does not have enough platelets


Purpura = excessive bleeding / bruising


Immune Thrombocytopenic Purpura


Antibodies destroy platelets


Antibodies see platelets as bacteria and work to eliminate them


ITP is preceded by a viral illness



URI


Varicella / measles vaccine


Mononucleosis


Flu

Symptoms


Random purpura


Epistaxis, hematuria, hematemesis, and menorrhagia



Petechiae and hemorrhagic bullae in mouth



Diagnostic Tests


Low platelet count


Peripheral blood smear


Antiplatelet antibodies

Normal platelet count: 150,000 to 400,000


Management


IV gamma globulin to block antibody production, reduce autoimmune problem


Corticosteroids to reduce inflammatory process


IV anti-D to stimulate platelet production



Sickle Cell Anemia


Autosomal recessive disorder



Defect in hemoglobin molecule


Cells become sickle shaped and rigid


Lose ability to adapt shape to surroundings.


Sickling may be triggered by fever and emotional or physical stress



Pathophysiology


When exposed to diminished levels of oxygen, the hemoglobin in the RBC
develops a sickle or crescent shape; the cells are rigid and obstruct
capillary blood flow, leading to congestion and tissue hypoxia; clinically,
this hypoxia causes additional sickling and extensive infarctions.

Crescent Shaped Cells


Body Systems Affected by SS




Brain: CVA – paralysis - death


Eyes: retinopathy – blindness


Lungs: pneumonia


Abdomen: pain, hepatomegaly, splenomegaly (medical emergency due to
possible rupture


Skeletal: joint pain, bone pain – osteomyelitis


Skin: chronic ulcers – poor wound healing


Vaso-occlusive Crisis


Stasis of blood with clumping of cell in the microcirculation, ischemia,
and infarction


Most common type of crisis; painful


Signs include fever, pain, tissue engorgement


Splenic Sequestration


Life-threatening / death within hours


Pooling of blood in the spleen


Signs include profound anemia, hypovolemia, and shock


Abdominal distention, pallor, dyspnea, tachycardia, and hypotension


Aplastic Crisis


Diminished production and increased destruction of red blood cells


Triggered by viral infection or depletion of folic acid


Signs include profound anemia, pallor


Nursing Diagnoses


Altered tissue perfusion


Pain


Risk for infection


Knowledge deficit regarding disease process


Nursing Management - Hospital


Increase tissue perfusion



Oxygen


Blood transfusion if ordered


Bed rest


Pain management


Hydration


IV fluids as ordered


Oral intake of fluids



Nursing Management


Adequate nutrition


Emotional Support


Discharge instructions



Information about disease management


Daily folic acid


Control of triggers


Prophylactic antibiotics


Immunizations / Pneumococcal


Patient Education

Necessity of following plan of care


Signs and symptoms of impending crisis.


Signs and symptoms of infection


Preventing hypoxia from physical and emotional stress


Proving adequate rest



Beta-Thalassemia

Hereditary / autosomal defect


Genetic defect on chromosome 11


Mediterranean descent


Defect in the beta globin gene


Beta globin chains are required for synthesis of hemoglobin A


RBC Characteristics


Microcytosis = small in size



Hypochromia = decrease hemoglobin


Poikilocytosis = abnormal shape


Treatment / Prognosis


Supportive


Blood transfusions as needed


Bone marrow transplant


Iron Deficiency Anemia


Most common nutritional deficiency


Depletion of iron stores


Abnormal Laboratory Values


Hemoglobin levels less than 8 g/dL


Decreased levels of Serum Iron or Total Iron Binding or Serum Ferritin


Microcytic and hypochromic red blood cells



IDA

Occurs in children experiencing:



Rapid physical growth


Low iron intake


Inadequate iron absorption


Loss of blood



Symptoms

Associated with low oxygenation of tissue:



Pallor


Fatigue


Shortness or breath


Irritability


Intolerance of physical work / exercise



Management

Iron supplementation


Given in a.m. on an empty stomach


To avoid staining of teeth, give using a syringe, dropper or straw


Instruct caretaker that child may have dark-colored stools


Management




Nutritional counseling


Infants younger than 12 months should be on formula until around 12 months
of age


Infants 12 months or older


Decrease intake of milk


Introduce solid foods


Children: iron fortified cereals, foods, meat, green leafy vegetables


Teenagers: reduce junk food




Aplastic Anemia


Acquired or inherited


Normal production of blood cells in the bone marrow is absent or decreased.


A marked decrease in RBC’s, WBC’s and platelets.



Causes

Exposure to drugs


Exposure to chemicals


Exposure to toxins


Infection


Idiopathic in nature



Blood Characteristics


Neutophil less than 500



Platelet less than 20,000


Hemoglobin less than 7


Reticulocytes 1%




Nursing Diagnosis?




Management

Immunosuppressive therapy

Antithymocyte globulin




Administered IV over 4 days


Response seen within 3 months

Bone Marrow Transplant



Hyperbilirubinemia

Many babies have some jaundice. When they are a few days old, their skin
slowly begins to turn yellow. The yellow color comes from the color of
bilirubin. When red blood cells die, they break down and bilirubin is left.
The red blood cells break down and make bilirubin. In newborns, the liver may
not be developed enough to get rid of so much bilirubin at once. So, if too
many red blood cells die at the same time, the baby can become very yellow or
may even look orange. The yellow color does not hurt the baby's skin, but the
bilirubin goes to the brain as well as to the skin. That can lead to brain
damage.


Signs and symptoms


Very yellow or orange skin tones (beginning at the head and spreading to
the toes)


Increased sleepiness, so much that it is hard to wake the baby


High-pitched cry


Poor sucking or nursing


Weakness, limpness, or floppiness

Photo Therapy


Fiberoptic Blanket


Nursing Interventions


Monitor bilirubin levels


Assess activity level – muscle tone – infant reflexes


Encourage po intake: May need to supplement with formula if inadequate
breastfeeding


Weight daily to assess hydration status


Monitor stools – amount and number


Cover eyes while under bili-lights


Facilitate parent - infant bonding



Loss of moro or startle reflex can indicate possible brain damage due to
Kernicterus