Nur 421 Final exam Q%A SOLVED 100%
CORRECT!!!
Thalassemia- What is it?
Inherited autosomal recessive blood disease
Abnormal production of hemoglobin=damages RBCs causing profound anemia
Bone marrow compensates with ↑ production of erythrocytes (bone changes)
Excess iron (hemosiderosis)
Thalassemia- S/Sx
Pallor
FTT
Severe anemia
Hepatosplenomegaly
Thalassemia- Tx/Interventions
blood transfusions
chelation (deferoxamine or deferasirox)
Splenectomy/antibiotics
A child has iron overload from receiving multiple blood transfusions for treating thalassemia.
A nurse should anticipate the physician will likely:
Order IV fluids to dilute the excess iron and increase urinary excretion
Change the type of blood product being transfused
Reduce the frequency of blood transfusion
Begin chelation therapy
Begin chelation therapy
Hemophilia- What is it?
A deficiency of a clotting factor; Typically x-linked recessive
,Hemophilia- S/Sx
Bleeding can be spontaneous
Hematuria
Bruising
Nosebleeds
Intracranial bleeds and retinal bleeds
Hemarthrosis
Hemophilia- Tx/Interventions
Medications: Factor VIII infusions → #1 but it takes time !
DDAVP (increases the level of factor VIII) → pt whos liver doesn't make enough factor VIII →
stimulates liver to make more; May not be enough but can help slow the bleeding
Medic-Alert bracelet
Injury prevention
Regular dental care → even a small nick on the gum
No aspirin or aspirin-like meds
Sickle cell disease- what is it?
Abnormal HgbS cause crescent or sickle-shaped RBCs → abnormal protein in RBCs → stiff cells
At low oxygenation → HgbS molecules polymerize and stick together
Sickle cell disease- s/sx
Vaso‐occlusive ("pain episode")
SoB
fatigue
sx when HgbF changes to Hgb SS (2-3 months of age)
Sickle cell disease- tx/interventions
Toradol and Morphine
Penicillin started by 2 months of age
,All immunizations including influenza and meningococcal
Monthly transfusions for severe pts
Iron-chelation for hemosiderosis
Hydroxyurea to increase production of HgbF
Genetic counseling
Sickle cell disease- complications
SEQUESTRATION (spleen)
Aplastic crisis
Acute chest syndrome
Hyperhemolytic crisis
Stroke
Anemia (Iron)- what is it?
Hgb <10, MOST common → easily diagnosed, easily treated (still significant)
Anemia (Iron)- s/sx
General lethargy, Tachycardia, Tachypneic
Anemia (Iron)- tx/interventions
Avoid milk
Iron supplements (AAP says start at 4 months in exclusively breastfed infants) → formula is iron
fortified
Give with vitamin C to aid in absorption
Give on empty stomach (absorbed better in acidic environment) or with some acidic food
Type I DM- what is it?
Lack of insulin, hyperglycemia d/t autoimmune, genetic, and environmental factors
Type I DM- s/sx
, Polyuria: excessive urination
Polydipsia: excessive thirst
Polyphagia: excessive appetite
Intense vomiting, diarrhea, electrolyte imbalance (acidotic)
Weight loss
Type I DM- tx/interventions
insulin
Type II DM- what is it?
insulin resistance and impaired glucose regulation d/t weight gain, fatty liver, high BP, high
lipids or Metabolic Syndrome
Type II DM- s/sx
Gradual onset
Hyperglycemia
Polyuria, ketonuria can be present
Little or no weight loss
Acanthosis nigricans → skin condition characterized by dark, thick, velvety patches of skin,
typically appearing in body folds like the neck, armpits, and groin
Type II DM- tx/interventions
exercise, weight loss, meds (metformin)
Weight loss can be hard to implement and if unsuccessful, Rx meds
DM Risks- acute
Diabetic Ketoacidosis (DKA) → body produces ketones due to lack of insulin (type I)
Hyperosmolar hyperglycemic state (HHS) → Blood glucose levels are too high leading to
dehydration (type II)
Both are treated with: Fluids, electrolytes, insulin
CORRECT!!!
Thalassemia- What is it?
Inherited autosomal recessive blood disease
Abnormal production of hemoglobin=damages RBCs causing profound anemia
Bone marrow compensates with ↑ production of erythrocytes (bone changes)
Excess iron (hemosiderosis)
Thalassemia- S/Sx
Pallor
FTT
Severe anemia
Hepatosplenomegaly
Thalassemia- Tx/Interventions
blood transfusions
chelation (deferoxamine or deferasirox)
Splenectomy/antibiotics
A child has iron overload from receiving multiple blood transfusions for treating thalassemia.
A nurse should anticipate the physician will likely:
Order IV fluids to dilute the excess iron and increase urinary excretion
Change the type of blood product being transfused
Reduce the frequency of blood transfusion
Begin chelation therapy
Begin chelation therapy
Hemophilia- What is it?
A deficiency of a clotting factor; Typically x-linked recessive
,Hemophilia- S/Sx
Bleeding can be spontaneous
Hematuria
Bruising
Nosebleeds
Intracranial bleeds and retinal bleeds
Hemarthrosis
Hemophilia- Tx/Interventions
Medications: Factor VIII infusions → #1 but it takes time !
DDAVP (increases the level of factor VIII) → pt whos liver doesn't make enough factor VIII →
stimulates liver to make more; May not be enough but can help slow the bleeding
Medic-Alert bracelet
Injury prevention
Regular dental care → even a small nick on the gum
No aspirin or aspirin-like meds
Sickle cell disease- what is it?
Abnormal HgbS cause crescent or sickle-shaped RBCs → abnormal protein in RBCs → stiff cells
At low oxygenation → HgbS molecules polymerize and stick together
Sickle cell disease- s/sx
Vaso‐occlusive ("pain episode")
SoB
fatigue
sx when HgbF changes to Hgb SS (2-3 months of age)
Sickle cell disease- tx/interventions
Toradol and Morphine
Penicillin started by 2 months of age
,All immunizations including influenza and meningococcal
Monthly transfusions for severe pts
Iron-chelation for hemosiderosis
Hydroxyurea to increase production of HgbF
Genetic counseling
Sickle cell disease- complications
SEQUESTRATION (spleen)
Aplastic crisis
Acute chest syndrome
Hyperhemolytic crisis
Stroke
Anemia (Iron)- what is it?
Hgb <10, MOST common → easily diagnosed, easily treated (still significant)
Anemia (Iron)- s/sx
General lethargy, Tachycardia, Tachypneic
Anemia (Iron)- tx/interventions
Avoid milk
Iron supplements (AAP says start at 4 months in exclusively breastfed infants) → formula is iron
fortified
Give with vitamin C to aid in absorption
Give on empty stomach (absorbed better in acidic environment) or with some acidic food
Type I DM- what is it?
Lack of insulin, hyperglycemia d/t autoimmune, genetic, and environmental factors
Type I DM- s/sx
, Polyuria: excessive urination
Polydipsia: excessive thirst
Polyphagia: excessive appetite
Intense vomiting, diarrhea, electrolyte imbalance (acidotic)
Weight loss
Type I DM- tx/interventions
insulin
Type II DM- what is it?
insulin resistance and impaired glucose regulation d/t weight gain, fatty liver, high BP, high
lipids or Metabolic Syndrome
Type II DM- s/sx
Gradual onset
Hyperglycemia
Polyuria, ketonuria can be present
Little or no weight loss
Acanthosis nigricans → skin condition characterized by dark, thick, velvety patches of skin,
typically appearing in body folds like the neck, armpits, and groin
Type II DM- tx/interventions
exercise, weight loss, meds (metformin)
Weight loss can be hard to implement and if unsuccessful, Rx meds
DM Risks- acute
Diabetic Ketoacidosis (DKA) → body produces ketones due to lack of insulin (type I)
Hyperosmolar hyperglycemic state (HHS) → Blood glucose levels are too high leading to
dehydration (type II)
Both are treated with: Fluids, electrolytes, insulin
