Pediatric Hematology

What are the components of blood?

RBC, platlets, WBC, plasma

What is necessary to assess hematologic fx?

hx, physical exam, CBC

What is anemia caused by an inadequate supply of dietary iron?

iron deficiency anemia

What cause iron deficiency anemia?

decrease supply of iron, impaired iron absorption, increase need for iron, affect the synthesis of hgb

How long do a mothers iron stores last in a full term infant?

5-6 months

How long do a mothers iron stores last in preterm infant or multiple births?

2-3 months

Why are children 12-36 months old at risk for low iron intake?

drinking cows milk

What are s/s of iron deficiency anemia?

lack of energy,

What does testing for iron deficiency anemia show?

decreased hgb and hct

What management is needed for iron deficiency anemia?

add iron supplement to diet and oral iron supplement with vitamin C

When do you give iron supplements?

BID between meals

What diet changes are needed for iron deficiency anemia?

avoid cows milk

What type of anemia is characterized by normal adult hgb is partly or complete.y replaced by abnormal hgb, causing sickled RBCs?

sickle cell anemia

What are the clinical features of sickle cell anemia?

obstruction caused by RBCs, vascular inflammation, increased RBC destruction

What are sickle cell risk factors?

genetics, African American, hispanic American, middle eastern, Asian, Indian, and Mediterranean

What is the inheritance pattern for sickle cell diease?

autosomal recessive

What is needed for sickle cells diagnosis?

hbg electrophoresis and family hx

What are sickle cell complications?

crisis, acute chest syndrome, cerebrovascular accident

What are precipitating factors for sickle cell crisis?

anything that increases body’s need for oxygen or laters transport of oxygen, trauma, infection, fever, physical and emotional stress, increased blood viscosity caused by dehydration, hypoxia

What are s/s of Sickle Cell Crisis?

pain, SOB, fatigue, HA, dizziness, weakness, jaundice

What is given to those to prevent sickle cell crisis?

folic acid and hydroxyurea

What is the treatment for sickle cell disease?

blood transfusion

What is needed to help with pain with those who have sickle cell?

avoid dehydration, hydroxyurea

What is the treatment for sickle cell disease?

IV fluids, NSAIDs, opoids

What disease is characterized by sickle shaped cells sticking together and block the flow of oxygen in the vessels in the lungs?

acute chest syndrome (ACS)

What is ACS triggered by?

asthma crisis or infection

What can ACS progress to?

respiratory failure

What are s/s of ACS?

chest pain, fever, cough, SOB

What can you do to prevent ACS in sickle cell patients?

IS and ATBs

What disease is characterized by deficiency of one of the factors necessary for coagulation of blood?

hemophilia

What is the inheritance pattern for hemophilia?

x-linked recessive

What are the types of hemophilia?

factor VIII deficiency, factor IX deficiency, Von Willenbrand Disease

What does hemophilia result from?

congenital deficiency, dysfunction, absence of specific coagulation proteins or factors

What lab findings are likely for hemophilia?

factor VII and IX assay, PTT, carrier detection

What are the manifestations of hemophilia?

prolonged bleeding anywhere in the body, hemorrhage from any trauma, excessive bruising, subq and IM hemorrhage, bleeding in joints, hematomas, hematuria

What is the therapeutic management needed for hemophilia?

replace the deficient clotting factor

What is the treatment for hemophilia?

gene therapy

What nursing care is needed for hemophilia?

support the family, prevent bleeding, prevent effects of bleeding, recognize and control bleeding

What is an acquired hemorrhagic disorder characterized by thrombocytopenia, minimal signs of bleeding, normal bone marrow with normal or increased number of immature platelets and eosinophils?

idiopathic thrombocytopenic purpura (ITP)

What causes ITP?

evolution of ATBs against multiple plt antigens - reduced plt survival and impaired plt production

What does the diagnosis for ITP include?

ruling out other disorders

What therapeutic management is needed for ITP?

supportive measures, IVIG

What nursing management is needed for ITP?

bed rest

What disease is characterized by diffused fibrin deposition in microvasculature where the first stage of coagulation process is inappropriately stimulated causing bleeding and clotting to occur simultaneously?

disseminated intravascular coagulation (DIC)

What management is needed for DIC?

control initiating causes