Hematologic Conditions Flashcards
______ is the most prevalent / preventable nutritional disorder
Iron deficiency anemia
What populations of children have highest risk of developing iron deficiency anemia? (2)
- Low socioeconomic status
- 6 months - 2 years old
What is the primary risk factor of iron deficiency anemia in young children?
Inadequate intake of dietary iron
What is the primary risk factor of iron deficiency anemia in school-aged children?
Blood loss
______ decrease the incidence of iron deficiency anemia
WIC programs
What is heme iron?
Iron found in animal sources (best form, easily absorbed)
What are some examples of foods rich in heme iron? (4)
- Red meat
- Seafood
- Chicken
- Eggs
What is non-heme iron?
Iron found in plant sources
What are some examples of foods rich in non-heme iron? (3)
- Dark, leafy greens
- Iron-fortified cereals
- Iron-fortified formulas
When is iron normally transferred from mother to fetus?
During the last trimester of pregnancy
A full-term infant has adequate iron stores for ______
5 - 6 months
A pre-term infant has adequate iron stores for ______
2 - 3 months
Iron is needed for the formation of ______
Hemoglobin
Cow’s milk should not be given to a child until ______
12 months
Describe the effects of early consumption of cow’s milk
Causes microscopic GI bleeding –> iron deficiency anemia
Manifestations of iron deficiency anemia are related to …
Degree of tissue hypoxia (from lack of hemoglobin)
______ bonds to stored iron
Ferritin
What type of iron supplement is most easily absorbed?
Ferrous iron
______ converts iron from ferritin into hemoglobin
Folic acid
What are the manifestations of iron deficiency anemia? (11)
- SOB
- Tachycardia
- Muscle weakness
- Fatigue
- Poor feeding
- Pale skin / mucous membranes
- Hair loss
- Headache
- Decreased attention span
- Depression
- Dizziness
What laboratory findings are associated with the diagnosis of iron deficiency anemia? (4)
- Decreased ferritin
- Decreased RBC
- Decreased H & H
- Decreased MCV
Describe the management of iron deficiency anemia (3)
- Iron-fortified cereal
- Oral iron supplements (ferrous iron)
- Monitor for bleeding / malabsorption
Describe the management of iron deficiency anemia for infants (4)
- Encourage breast milk
- Iron-fortified formula
- 2 servings of iron-rich food per day (after 6 months)
- No cow’s milk until 12 months
Why is breast milk encouraged for infants?
Provides better iron absorption than formula
Describe education associated with iron supplementation (4)
- Give BID between meals (may give with / after meals if unable to tolerate)
- Take with vitamin C to improve absorption
- Increase protein / folic acid
- Liquid form may stain teeth
What is the primary side effect associated with iron supplementation?
Dark stools
What are the possible results of severe / prolonged iron deficiency anemia?
Cognitive / behavioral impairment
What is sickle cell disease?
C-shaped RBCs due to abnormal hemoglobin gene (Hgb S)
What type of disorder is sickle cell disease?
Autosomal recessive disorder
What population has highest risk of developing sickle cell disease?
African Americans
Describe the pathophysiology of sickle cell disease (2)
- Sickled RBCs are unable to pass through circulation
- Vaso-occlusion causes hypoxia / ischemia
Most sickled cells …
Regain their shape, however irreversible sickling is possible
Manifestations of sickle cell disease are related to … (3)
- Obstruction
- RBC destruction
- Vascular inflammation
Describe the occurrence of manifestations of sickle cell disease (2)
- Asymptomatic for first 4 - 6 months
- Symptoms present during period illness (causes exacerbation)
Why are children with sickle cell disease asymptomatic for the first 4 - 6 months of life?
Presence of fetal hemoglobin (HbF) - does not sickle
Describe the pathophysiology of a vaso-occlusive crisis (2)
- Oxygen cannot reach tissues, leading to ischemia
- Causes extreme pain
How long does vaso-occlusive crisis pain typically last?
4 - 6 days
What is the first sign of a vaso-occlusive crisis in young children?
Hand-foot syndrome
Describe hand-foot syndrome
Tender / warm / swollen hands and feet
What are the manifestations of a vaso-occlusive crisis? (6)
- CVA
- SOB
- Fatigue
- Dizziness
- Pneumonia symptoms
- Priapism
When is the peak incidence of CVAs associated with sickle cell disease?
Birth - 5 years
Where are some common areas for pain associated with sickle cell disease? (5)
- Lumbosacral spine
- Shoulder
- Elbow
- Knee
- Femur
Describe the pathophysiology of a sequestration crisis (2)
- Blood pooling in spleen / liver
- Decreased circulation causes shock / cardiac arrest
A sequestration crisis is typically seen in children of what age?
< 3 years old
What is the mortality rate of a sequestration crisis?
50%
When is sickle cell disease usually diagnosed?
Newborn screening
If sickle cell disease is not diagnosed in infancy, when does it usually manifest?
Toddler / pre-school years due to secondary illness causing excessive stress in the body leading to sickling
What tests are used for the diagnosis of sickle cell disease? (3)
- CBC
- Sickledex
- Hemoglobin electrophoresis
Describe the prevention of sickle cell disease crisis (3)
- Immunizations
- Prophylactic antibiotics
- Avoid stress on the body (fever, infection, acidosis, dehydration, hypothermia)
Prophylactic antibiotics for sickle cell disease are especially important for …
Children who have had their spleen removed
Describe the management of sickle cell disease (7)
- Blood transfusions
- Bedrest
- Oxygen
- NSAIDS
- Hydroxyurea
- Fluid / electrolyte replacement
- Warm compress
What are the functions of blood transfusions for sickle cell disease? (2)
- Decrease blood viscosity
- Replace lost RBCs
What is the function of hydroxyurea for sickle cell disease?
Increases fetal hemoglobin (HbF) which does not sickle
It is important to maintain an oxygen saturation of ______ in children with sickle cell disease
> 93%
Hypoxia related to sickle cell disease causes ______
Metabolic acidosis
Why is a warm compress used for children with sickle cell disease?
Vasodilation to prevent vaso-occlusion
DO NOT ______ to hot / swollen hands in sickle cell disease
Apply ice
What pain medications are used for management of sickle cell disease? (3)
- Codeine
- Oxycodone
- Morphine
______ may be used for pain management of sickle cell disease
PCA
Which pain medication is contraindicated for sickle cell disease?
meperidine (Demerol) - risk of seizures
Most patients with sickle cell disease live into the ______ with optimal management
5th decade
What age has the greatest risk for complications from sickle cell disease?
< 5 years old
Most deaths from sickle cell disease in children < 5 years old are related to ______
Infection
What is hemophilia A?
Deficiency of factor VIII
What is the function of factor VIII?
Formation of thromboplastin (essential for blood clotting)
What type of disorder is hemophilia A?
X-linked recessive disorder
Describe the pathophysiology of hemophilia A (2)
- Lack of factor VIII impairs coagulation
- Causes fatal hemorrhage
Where is the most common site of internal bleeding from hemophilia A?
Bleeding into the joint cavities / muscles
Describe the risk of bleeding in various locations from hemophilia A (3)
- GI tract bleeding = anemia
- Neck / mouth / thorax bleeding = compromised airway
- Intracranial bleeding = death
______ occur after repeated bleeding episodes from hemophilia A
Bony changes / crippling deformities
Describe the occurrence of bleeding associated with hemophilia A (2)
- As child learns to walk, bruising may cause bleeding into joints (hemarthrosis)
- By age 3 - 4, minor lacerations may cause persistent bleeding
What are common forms of bleeding in hemophilia A? (2)
- Hematuria
- Epistaxis
Describe the effects of hemarthrosis (2)
- Limited movement in elbows, knees, and ankles
- Risk of degenerative joint changes later in life
______ is rare in hemophilia A during the first year of life
Spontaneous bleeding
Injections / firm holding can result in ______ is hemophilia A
Hematomas
What laboratory findings are associated with the diagnosis of hemophilia A? (2)
- Decreased factor VIII
- Prolonged PT / PTT
Describe the management of hemophilia A (7)
- Factor VIII concentrate
- DDAVP - synthetic antidiuretic (increases factor VIII)
- Exercise / physical therapy to maintain joint mobility
- Rest
- Ice
- Compression
- Elevation
Why is ice used for children with hemophilia A?
Vasoconstriction to stop bleeding
Children with hemophilia A must wear ______
A medic alert bracelet
Describe the prognosis of hemophilia A
With proper monitoring / treatment children lead relatively normal lives
Patients treated with plasma products for hemophilia A prior to 1985 …
Have likely HIV exposure