Hematology Flashcards
What symptoms suggest iron deficiency anemia (IDA)?
Pallor
Irritability
Poor feeding
Lethargy
What is the function of RBC? What is the lifespan?
Transport hemoglobin and oxygen and carry CO2 back to the lungs
90 days
Where are RBC formed? What is reticulocyte? Do RBC differ from each other?
Formed from bone marrow
Reticulocyte: immature RBC
Abnormal size, shape, or heme component
What s/s would make you suspect anemia? (7)
Pallor
Fatigue
Activity intolerance
Murmur
Tachycardia
Poor growth
Learning delays
If anemia is not severe, what would be the first clue that a patient is anemic?
CBC results
Decreased RBC
Decreased Hgb/Hct
Increased reticulocyte count
What are nutritional anemias?
Iron deficiency (IDA)
Lead poisoning
What are two disorders cause chronic anemia?
Autoimmune disease
Renal failure
What are three congenital hemolytic anemias
Alpha-thalassemia
Beta-thalassemia
Sickle cell disease
What is IDA caused by? Is it preventable?
Inadequate supply or loss of iron
Preventable
What needs to be in an infants diet to avoid IDA? When would they develop IDA if they did not have this?
Fortified iron
Between 9-24 months would develop IDA if not fortified iron
What are the risk factors for IDA? (5)
Decreased supply of iron (excessive milk intake, poor eating habits, feeding problems)
Impaired absorption of iron (iron inhibitors, malabsorption IBD, chronic diarrhea)
Increased need fir iron (prematurity, LBW, adolescence)
Blood loss - mensuration
Low SES, lead exposure, exclusively breasting feeding over 12 months)
What would cause a decreased supply of iron?
excessive milk intake, poor eating habits, feeding problems
What would cause impaired iron absorption?
iron inhibitors, malabsorption IBD, chronic diarrhea
What would cause an increase need for iron?
prematurity, LBW, adolescence
What is the management of IDA?
Prevention and screening is primary goal
Only breast milk or iron fortified formula for first 12 months
Iron supplement of 1mg/kg/day by 4-6 months in breastfed babies
Infants and toddler should have iron fortified cereal and solid foods containing iron
Toddlers formula intake should be limited to 20-24 ounces/day
What are nursing diagnoses for anemia?
Impaired gas exchange
Alteration in perfusion
Fatigue
Activity intolerance
What are nursing interventions that could help with activity intolerance r/t anemia?
Decrease tissue/metabolic oxygen needs by conserving energy and clustering nursing care
Supplemental oxygen
Nutritional support
What is hemophilia? Is there a cure?
X linked hereditary bleeding disorder
No cure
Who carries hemophilia? Who does it affect?
Mom carried the hemophilia A gene and passed it to baby boy
What is the most common type of hemophilia? What factor? Why is that needed?
Hemophilia A
Factor VIII
Protein that is necessary for thromboplastin in clot formation
How does hemophilia A present? (7)
Hemorrhage from minor trauma
Can be spontaneous
Hemathroses
Intracranial hemorrhage
Nose bleeds
Hematuria
IM hematoma –> compartment syndrome
What is the medical management for hemophilia A?
Replace factor VIII clotting factor vis DDAVP/desmopressin or factor VIII infusion
PRBC if H&H is decreased
What does DDAVP/desmopressin do for hemophilia A? When is it used?
Directly increased factor VIII
OKAY for mildly affect individuals
What are the types of factor VIII infusion?
Synthetic/recombinant formulation
Plasma based
What should you monitor in hemophilia A to determine if replacing the factor VIII for effective?
What drugs should be avoided in hemophilia A?
What are the nursing diagnoses r/t hemophilia A?
Risk for injury d/t bleeding
Acute pain - w/ bleed (joint) (RICE)
Risk for hypovolemic shock r/t hemorrhage
How do you decreased to risk for injury d/t bleeding in hemophilia A?
Prevention
Soft toys
Avoid contact sports
Protective pads/helmets
Early identification of the blood
Hold pressure for increase time after injections
What medical management needs to occur at home for a patient with hemophilia A?
Taught to administer factor VIII at home
Daily infusion via port
Sliding scale dose for acute bleeds
Education on s/s of hemorrhage (looking for a change in LOC)
Post injury care
Watching for neuro changes –> CVA
Educate on s/s that would indicate needing to come in for care
What are changes or safety precautions that should be taken at home for a patient with hemophilia A and ITP?
Avoid high risk behaviors such as contact sports, riding a bike, roller skating
Avoid medications that can affect bleeding such as NSAIDS and aspirin
Use an extra-soft bristle toothbrush
Establish age appropriate safe home environment such as pad table corners, pad crib rails, extra joint padding on clothes
What is the most effective test to diagnose hemophilia A?
PTT - partial thromboplastin time
Monitors bleeding times
Do you see petechia/purpura in hemophilia A?
NO, because platelets are not involved
Hemophilia A will just go straight to bleeding
What is the worst case scenario regarding hemophilia A?
Hemorrhage
If a joint is injured with hemophilia A what should be done for pain? Should they use their joint?
Tylenol
RICE to vasoconstrict and decrease bleeding
PROM for joint and weight bearing later on
What are platelets? What are their function?
Thrombocyte
Coagulation and capillary hemostatic
What is ITP? What does it usually follow?
Acquired hemorrhagic disorder
Autoimmune –> excessive destruction of platelets
Usually follows 1-3 weeks after a viral illness such as viral exanthema
Who does ITP affect most commonly? When is the recovery?
Most common bleeding disorder in childhood - ages 2-5
Most recover in 6 months
How do you diagnose ITP?
< 100,000 platelets is diagnostic
What are the clinical manifestations of ITP?
Mucocutaneous bleeding such as ecchymosis, petetchiae, purpura
Muscosal bleeding like oral, nasal, GI tract or GU tract
Hemorrhage
Does ITP go away on its own?
Usually self limiting - treatment is usually symptomatic
What is the treatment of ITP?
IV or oral corticosteroids (prednisone) initially
IVIG (intravenous immunoglobins) initially
Splenectomy if initial treatment ineffective and for chronic forms
When would you do a platelet transfusion in ITP?
Given only when active uncontrolled bleeding occurs
When should a patient with ITP begin to get treatment? What are you monitoring before getting to this point?
Less than 20,000 platelets
Monitoring platelets and H&H
What are some nursing consideration regarding ITP?
Avoid administering intramuscular injection (instead subQ if possible)
NO ASA or NSAIDS
NO rectal temps
Any invasive procedures need extreme caution
Defer Vaccines after patient is given IVIG
What should occur after a splenectomy r/t ITP?
Administer pneumococcal and HIB vaccine
Prophylactic PNC
What occurs to RBC in sickle cell?
Destruction of RBC
Loss of RBC
Malformation of heme portion of RBC
What is normal Hbg? What is sickle cell Hbg?
Normal Hbg: A or F
Sickle cell: S Hbg
Is sickle cell inherited? Is it apparent at birth? What is fetal Hbg?
Genetic mutation that is inherited and hemoglobin sickles
Usually not apparent until later infancy d/t fetal hemoglobin so difficult to see in newborn which is why use newborn screening nationwide
Fetal hemoglobin is a protective factor that does not sickle
What is the epigenetic adaptation regarding sickle cell?
Malaria protects against
What is the pathology of sickle cell?
Hemoglobin sickles under physiologic stress (generally asymptomatic unless under stress) such as exercise, illness, increase oxygen needs, emotional
Sickle cells obstruct blood vessels
Increased RBC destruction
Decreased tissue perfusion and oxygen to tissues
When do you see acute pain in sickle cell?
There is decreased perfusion so part of the body is deprived of oxygen and nutrients –> acute pain
Where do you usually see pain in sickle cell crisis? Why do you see this pain?
Abdominal pain
Long bone pain
Hands and feet
Joint pain (arthralgia)
Acute chest syndrome
Priapism (prolonged ejection w/o sexual arrousal)
Ischemia causing mild-severe pain
What are the complications of a sickle cell crisis?
Infections
Stroke (silent stoke occurs when a sickle cell gets stuck in brain and cuts off circulation)
URIs
Leg ulcers
Splenomegaly
What is a vaso-occulsive or sickle cell crisis? How long does it last?
Sickle shaped RBC block blood flow through the tiny blood vessels to chest, abdomen and joints –> pain
Lasts minutes to days
What is the acute management of sickle cell disease?
H- Hydration – aggressive to break sickle clots apart and restore perfusion (2-2.5 times maintenance)
O- Oxygenation
P- Pain control
Bedrest
Antibiotic if there is an infection (broad spectrum)
What is the chronic management of sickle cell disease? (6)
Enrollment into a SCD program
Prevent sickling
Immunize!!
PNC prophylactically daily and lifelong
Recognize early s/s of crisis
Any fever over 38.5 in an emergency
What is medical management of sickle cell disease?
Monthly blood transfusion w/ or w/o aphersis
Splenectomy
Daily hydroxyurea
Stem cell transplant (only potential cure and very high risk)
Why do monthly blood transfusions help? What is aphersis? When do you get blood transfusions?
Shuts down patients bone marrow from producing sickle Hbg. Not a cure b/c still very low Hbg
Apheresis cleanses the blood by removing sickled RBC
Used if hydroxyurea doesn’t work and still having several vaso-occulsive crisis in a year OR brain scan reveals infarct
What is a complication of receiving so many blood transfusions? How do you monitor for this? What is the treatment?
Hemosiderosis - iron storage in tissues b/c RBC that are transfused are packed with iron
Monitor by MRI of head, heart, kidney, and liver 1-2 times per year to look for overload. If overloaded –> failure of that organ
Treat by giving medication that binds to iron and poop it out
How does a daily hydroxyurea help with sickle cell?
Works in bone marrow to stimulate detal Hbg –> decreased sickled Hbg production
What are the s/s of acute chest syndrome? What should you start on these patients?
Presents like pneumonia
Cough, SOB, fever, retractions, increase RR, increase WOB, hypoxic, crackles
Draw cultures and start broad spectrum antibiotics until s/s improve or cultures come back negative
What should patients with sickle cell be educated about?
Avoid temp extremes
Hydrate!!
Recognize fatigue and any joint pain –> need rest
Avoid alcohol, smoking(increase risk of infections and clots), and drugs
Why are immunizations and prophylactic PNC important for patients with sickle cell?
Immunizations - high risk for infections b/c chronic anemic state
PNC daily - develop autosplenectomy b/c clogged up d/t so many damaged RBC –> stops functioning as immune support
