Flashcards in T3-Blueprint: Blood Disorders Deck (64):
What is key with sickle cell disease?
Hydration and oxygenation
Sickle cell disease is a genetic issue resulting ins ever reduction in ____
What is the average life span for sickle cell?
What is the hgb count in sickle cell? What is normal?
Norm is above 11
Sickle cell disease--RBC contain an abnormal form of HGB so what does that mean?
RBC are inflexible and rigid (sickle shape)
More fragile= hemolysis (death of cells)
Can sickle cell be cured?
What is treatment for sickle cell?
Controlling anemia and relieving pain
Ions and fluid replacement is needed
T/F: A small cold in a sickle cell patient will make them very ill
Nutrition: What do they need? What do they not need?
Need: folic acid, and can eat normal foods but don't need lots of sweets and fast foods
Sickle cell patients in crisis is a HIGH PRIORITY!--What are the normal complaints they are telling if they come to ED?
Severe pain and fever
Screening tests for sickle cell only detect the prescence of an abnormal hb. What are they NOT specific for?
Trait or anemia
What is the definitive test for sickle cell?
Sickle cell: What is the Hgb electrophoresis test?
Definitive test for sickle cell that separates and measures the various HGB aka differentiates between trait and anemia
Sickle cell: Ophthalmic complications. What does this mean
Sickle cell: What is a vaso-occlusive crisis?
Sickle cell: Vaso-occlusive crisis
Chest syndrome: If they have had this in the past, will they have it again?
They probably will
It can cause life threatening issues
Chest syndrome= chest pain, fever, cough (can worsen with or result from pneumonia)
Sickle cell: Vaso-occlusive crisis. What may be the first symptom of vaso-occlusion?
Hand and foot syndrome (dactylitis)
Sickle cell: Vaso-occlusive crisis. What is the most frequent complication?
Painful episodes occuring in joints and limbs
*Pain is there bc there is obstruction of blood flow due to sickled RBC
What are some other complications of sickle cell disease?
-Cardiomegaly & systolic flow murmurs
-Abdominal pain, GU dysfunction
What is a main issue of sickle cell disease?
SCD: Enlarged spleen is caused by picked blood. How is this treated? What can this cause?
Treat with blood transfusion
Can cause anemia, hypovolemic shock
SCD: What is the role of the spleen?
Filters out infection in the body
SCD: If the spleen is enlarged, there is risk for infection!!! Explain.
1. Normal cells last 3 months; sickled cells only last 1 month
2. Keep out of crowds
3. Penicillin is DOC for this issue
What is sickle cell crisis triggered by?
Viral infection or depletion of folic acid
Signs: profound anemia and pallor
What is aplastic crisis of SCD caused by?
Human parvo virus leading to lowered Hb, retake count
SCD: How is aplastic crisis treated?
Observation and transfusion
SCD: T/F: Once they've had one stroke, they will normally have another
Stroke affects 10% of children with sickle cell
What do we do to treat the SCD complications?
When is RBC transfusion used for sickle cell complications?
Aplastic, hyperhemolytic, and splenic sequestration crisis
SCD: Avoid heat or cold?
What is treatment for FE overload?
Chelating agent that binds with iron in the bloodstream and enhances its elimination in the urine and feces
Who is more likely to get hemophilia: boys or girls?
*this is an X linked recessive disorder--girls have 2x, boys only have 1
Hemophilia A is a deficiency in factor ?
Factor VIII defiency
"classic hemophilia"--80% of cases
Hemophilia B is a deficiency of what factor?
Hemophilia: What is there difficulty controlling bleeding?
Bleeding time is exteneded due to lack of factor required for blood to clot
Hemophilia: When may difficulities become apparent?
Male circumcision...but may not become apparent until infant becomes more active and prone to injuries during toddler years
Hemophilia: What are s/s?
Hemophilia: Avoid hot or cold?
What are some tests for hemophilia?
Factor specific assays
Platelet and prothrombin time
Whole blood clotting time
Hemophilia: Ok to take rectal temps?
Avoid this, as well as skin punctures and use surgical aseptic technique
What are Factor VIII and IX ?
Clotting factors necessary to begin clotting cascade
Why do we give factor VIII and IX to hemophilia patients?
This is given via port-a-acth bc this is what hemophilia pts ar deficient in
What is the key sign to prolonged bleeding to the joints in hemophilia pts?
What are s/s of prolonged bleeding to the joints in hemophilia pts.?
-Joint pain stiffness
-Loss of ROM
Hemophilia: How do we treat prolonged bleeding to joints?
Must treat immediaely!
Active ROM after bleeding controlled
Maintain ideal weight to avoid stress to joints
Encourage regular exercise and PT
Hemophilia: If we need to give injections or perform an invasive procedures, how should we pretreat?
Pretreat with their specific factor (VIII or IX)
Hemophilia: How do we prevent bleeding at home?
Hemophilia: How do we prevent bleeding for toddlers?
Environment free of clutter--padding corners of furniture
Dress toddlers in extra layers of clothing to provide padding
Hemophilia: How do we prevent bleeding for preschool?
Hemophilia: How do we prevent bleeding in school aged?
Set activity restrictions
Low contact sports!!! (tennis, swimming)--use protective equipment
Hemophilia basics--Whats important for client educatin?
-Use soft bristle toothbrush
-Up-to-date on immunizations
-Med ID wristband or tags need to be worn
Why is iron deficiency anemia common in infancy?
Ages 12-36 months are most at risk due to consuming a diet high in cow's milk without adequate intake of foods high in iron
*Hgb requires iron...Low iron=low hgb
Why are adolescents at risk for iron deficiency anemia?
How should we administer iron for iron deficiency anemia?
Give on empty stomach (with juice, NOT MILK)
Give with meals and start with reduced dose and gradually increase if GI distrubce occurs
Give with Vit. C to help increase absorption
If we are given liquid iron, how should child drink it?
Through a straw
*brush teeth to prevent staining
What should we tell parents about stools of children who are taking iron?
Stools with be tarry green--this is normal
If we give iron IM, how should it be given?
z-track method--do not massage afterward!
What is epistaxis?
How do you stop a nose bleed?
Sit up and slightly lean forward and pinch bridge of nose for 10 min
A secondary disorder of coagulation that occurs as a complication of a number of pathologic processes (hoopla, acidosis, shock, burns)
Disseminated intravascular coagulation (DIC)
What are the hallmarks of DIC?
Bleeding and clotting
DIC: What is the patho and why is this bad?
Starts when the clotting process gets started abnormally--thrombin is made faster than body can neutralize it...bad bc child is susceptible to uncontrollable hemorrhage into vital organs
S/S of DIC?
-Bleeding form openings
-Bleeding from umbilicus or trachea (newborn)
-Bleeding evidence in GI
-Organ dysfunction from infarction and ischemia