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23 Cards in this Set
- Front
- Back
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What is the range of cells for Pediatrics?
RBC WBC Platelets Hgb Hct |
RBC: 3.8 - 5.5
WBC: 4.8 - 19 Platelets: 140 - 400 Hgb: 11.5 - 15.5 Hct: 30 ish |
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What are symptoms of low RBC's?
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Pt. reports weak & fatigued
Objective S&S: Pale, Bruising, Wounds, Cool Skin |
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What dietary considerations could impact RBC's/Iron?
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Red Meats are a good source of Iron.
High fat, coffee or caffeine intake impairs iron absorption. |
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What V/S's might you see with a hematological disorder?
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Decreased BP (esp. if low volume, late sign of shock)
Tachycardia (body compensating for hypovolemia) Peripheral pulse decreases Nuerological changes - fatigue, decreased LOC, HA, weakness. |
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What Dx tests might be ordered if a hematological disorder is suspected?
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CBC - looks at RBC, Hgb, Hct, RBC indexes (showing size & weight of RBC's, determines different types of anemia)
Bone Marrow Aspiration (not routine) - definitive test for leukemia & lymphomas |
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Explain Sickle Cell Anemia.
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It is a autosomal recessive gene genetic disorder (meaning that both parents are carriers & 25% of children will have the disease).
Hgb S is produced causing RBC's that have a sickle shape decreasing hemoglobin and oxygen carrying capacity of the cell. |
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What can trigger a sickle cell crisis?
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Any stress or traumatic event.
Infection Fever Acidosis Dehydration Physical Exertion Excessive Cole Exposure Hypoxia |
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What is the physiological result of sickling?
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A cascade of events:
Local tissue hypoxia -> ischemia -> infarct -> organ damage Pain at areas of hypoxia, increases metabolic need, tachycardia & sometimes tachypnea, causes further sickling, cells clump in lungs causing decreased gas exchange, producing, hypoxia, producing further sickling. Blood sequesters in the spleen causing splenomegaly & abdominal pain. Hemolysis occurs furthering anemia. Hemolysis makes the spleen work harder furthering splenomegaly, fibrosis & atrophy. Functional asplenia occurs from 6 mo. to 9 yrs 90% of the time. |
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What are the S&S of sickle cell anemia?
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** Joint Pain
** Anemia (hgb 6-9) Pallow Weakness Fatigue Jaundice Growth Retardation Cardiomegaly |
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What physical situations can trigger sickling?
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High altitudes
Poorly pressurized planes Hypoventilation Strenuous physical activity Emotional stress (more frequent during teen years) Infection Anesthesia (make sure they know sickle cell pt.) Basically, anything that increases demand for O2. |
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What are the types of sickle cell crisis? What is occurring physiologically? Which is most common?
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Vaso-Occlusive (most common)
Acute pain, tissue engorgement & fever. Treat with pain meds, O2, hydration, rest, blood if needed, antibiotics if infection. Splenic Sequestration (typical < 6 y.o.) Vascular occlusion in spleen causing liver/spleen enlargement. Severe anemia, tachycardia, weakness, dyspnia, pallor, eventually hypovolemic shock. Life threatening. Tx: Emergency Splenectomy, blood transfusions, ^ blood volume. Aplastic Crisis RBC production stops causing severe anemia. Associated with Parvo Virus (Fifth Disease). Anemia, weakness, pallor, tired, tachycardia, acutely ill, SOA. Tx: ^ blood volume so blood transfusions. |
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How is sickle cell anemia diagnosed?
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Initial screening is Sickledex (finger stick smear showing sickled RBC's)
Hgb Electrophoresis is definitive dx. Shows Hgb F & Hgb S in the infant OR Hgb SS in the older infant (> 6 mo's) or child. |
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What is the treatment (NI) for sickle cell patient?
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Bed Rest (cluster care)
Hydration (to decrease blood viscosity) Analgesics (for pain - ATC or PCA's) Blood transfusions (to treat anemia & make sickle cell less viscous) Prevent infections (prophylactic antibiotics, immunizations (flu & pneumonia), proper hand hygiene) Oxygen (keeping oxygenation up) |
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What are some complications/chronic affects of Sickle Cell Anemia?
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Spleen (filters abnormal RBC's & fight off infections) - if not functioning increases risk of infections.
Lungs - Pulmonary Hypertension or life threatening Acute Chest Syndrome (similar to pneumonia-chest pain, fever, dyspnea, cough, SOA) occurs. Brain - Stroke of SC's block blood flow. Abnormal behavior, slurred speech, vision changes or HA's. |
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What is the priority ND r/t Sickle Cell?
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Altered Tissue Perfusion.
Pain is also important but will improve if you treat the tissue perfusion. |
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What are the different types of Hemophilia?
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Hemophilia A or Classic Hemophilia (80% of cases) - missing clotting factor 8
Hemophilia B or Christmas Tree Hemophilia (20% of cases) - missing clotting factor 9 |
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How is Hemophilia inherited?
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Female - XX chromosomes
Male - XY chromosomes The clotting factor is associated with the X chromosome so if a male has the abnormal X chromosome he has the disease. However a female can have either one X or both X chromosomes affected (usually it is just one making them a carrier), if they have both X's affected then they will have the disease; however it is very rare. |
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Explain the pathophysiology of Hemophilia A.
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Factor VIII is the anti-hemophilic factor (AHF), produce in the liver.
A deficiency of factor VIII increases hemophilia. The level of AHF can be measured to determine severity: < 1% AHF = Severe Hemophilia 1-5% AHF = Moderate Hemophilia 6-50% AHF = Mild Hemophilia These patients don't bleed faster, it just takes longer for their blood to clot depending on how severe the disease is. |
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What are the general clinical manifestations of Hemophilia?
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Excessive (long term) bleeding & easy bruising. Most common location is internal bleeding to a joint (hemarthrosis).
These patients do not bleed greater or faster, just longer. |
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What are the S&S of hemarthrosis? What ND might you use for hemarthrosis?
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Tightness in the joint w/ no real pain (1st sign)
Tightness & pain (occurs before visible signs of bleeding) Swelling Hot to touch Difficulty with motion (a lot of pain) Bleeding will slow after several days once joint is full of blood. ND - Pain & Impaired Mobility |
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What S&S might you find with a history & assessment?
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Nosebleeds, bleeding gums, bloody urine or stools, prolonged bleeding, pain/joint stiffness & immobility.
Neurologic - slurred speech, HA's, decreased LOC indicating bleeding in the brain. Labs: Factor VIII & IX assays showing decrease, clotting factors PTT (prolonged), DNA testing for females who want to have children to detect the trait. |
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What is the medical management for a bleeding situation of a hemophiliac?
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1 - replace the missing clotting protein by administering Factor VIII pooled plasma via IV.
2 - DDAVP or Demopressin can be administered for Mild/Moderate Hemophilia. It stimulates the release of stored Factor VIII & Von Willebrand factor. Given by injection or nasal spray. Use limitedly b/c will lose potency. 3 - Hemiarthritis - treat appropriately. Give corticosteroids which decreases inflammation. As well as NSAIDS which help with inflammation & pain. Use with caution b/c can inhibit platelet function making bleeding worse. |
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What nursing management & education should be provided to the patient/family with hemophilia?
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*Prevent/Control bleeding episodes (infants in padded cribs & home safe, extra clothing on toddlers for padding & activity restrictions)
*Avoid unnecessary punctures (if must, hold pressure 5-10 minutes) *Supportive: RICE (Rest, Ice, Compression, Elevate) *Assess: S&S of bleeding in joints, head, GI tract *Limit joint involvement. Exercise therapy to prevent immobility *Manage pain *Education family - when to bring to hospital, bleeding that won't stop even with RICE. *Identify for school nurses who has hemophilia so they can respond appropriately |
