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226 Cards in this Set

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When is sickle cell disease diagnosed?
After age 4 months
What type of hemoglobin do well people have?
HgbA
What type of hemoglobin do infants have?
HgbF
What type of hemoglobin is found in a person who has sickle cell disease?
HgbS
Explain why sickle cell disease affects males and females alike:
It is autosomal: the affected gene is located on one of the 1st 22 pairs of chromosomes that do not determine gender
If a child inherits only one abnormal "sickle cell" gene, does he or she have the disease?
No; only the trait
What type of disorder is sickle cell disease classified as?
Autosomal, recessive disorder
In order for a child to inherit sickle cell disease, what must happen?
Both parents must have the defective genes
What is the lifespan of a sickled red blood cell?
7 - 20 days
What is the lifespan of a normal red blood cell?
120 days
What can cause sickling of the RBC's?
*Dehydration
*Acidosis
*Infection
*Hypoxia
*Venous stasis
*Strenuous exercise
*Anesthesia
What are the symptoms of sickle cell?
*Growth retardation
*Chronic anemia
*Susceptibility to sepsis
*Delayed sexual maturation
*Fever
*Pain
What is the most common and most distressing symptom of sickle cell?
Pain
What is priapism?
A nocturnal penile erection that lasts as long as 4 or more hours. Very painful
How is priapism treated?
*Estrogens
*Vasodilators
What are reticulocytes?
Immature RBC's
What is the WBC count like in a patient with sickle cell?
Elevated
What is the total bili count like in a patient with sickle cell?
Elevated
What is the sickle cell turbidity test?
A screening tool only
How is sickle cell disease determined for sure?
Hemoglobin electrophoresis
How are results from a hemoglobin electrophoresis test interpreted?
*Less than 40% HgbS = Trait
*More than 40% HgbS = Disease
What is the most common sickle cell crisis?
Vaso-occlusive crisis
Which sickle cell crisis causes lung/chest pain in the elderly?
Vaso-occlusive crisis
Which sickle cell crisis causes painful swelling of the hands/ feet and extreme pain in infants?
Vaso-occlusive crisis
Which sickle cell crisis commonly affects the knees/back of adults, and can cause infarcts of the small bones?
Vaso-occlusive crisis
Which sickle cell crisis is also known as splenic sequestration?
Sequestration crisis
Describe sequestration crisis:
*Blood is blocked into spleen
*Spleen enlarges
*Spleen can become scarred/useless
Why can a patient become "shocky" during a sequestration crisis?
Blood is trapped in the spleen and therefore not circulating throughout the body
In patients with sequestration crisis, what can happen by age 1?
Spleen can lose up to 29% of function
In patients with sequestration crisis, what can happen by age 2?
Spleen can lose greater than 40% of functionality
What is G6PD stand for?
Glucose 6 Phosphate Deficiency
What type of sickle cell crisis is caused by G6PD?
Hyperhemolytic Crisis
What does G6PD's function in the body?
It is an enzyme that stimulates the RBC's (gives them energy)
How much fluid intake is encouraged for patients who have sickle cell disease?
4-6 quarts per day
What is G6PD stand for?
Glucose 6 Phosphate Deficiency
How much fluid intake is encouraged for patients who are in sickle cell CRISIS?
6-8 quarts per day
What type of sickle cell crisis is caused by G6PD?
Hyperhemolytic Crisis
List some drugs that are used to manage pain in sickle cell patients:
*Methadone
*Morphine
*Hydroxyuria
*Dilaudid
*Fentanyl patch
What does G6PD's function in the body?
It is an enzyme that stimulates the RBC's (gives them energy)
What treatment for sickle cell disease is aimed at diluting the HgbS levels?
Chronic transfusions
How much fluid intake is encouraged for patients who have sickle cell disease?
4-6 quarts per day
What lab levels should be monitored in a patient who is undergoing chronic transfusions?
*Iron
*TIBC
*Ferritin
How much fluid intake is encouraged for patients who are in sickle cell CRISIS?
6-8 quarts per day
What should be avoided in patients with sickle cell disease?
*Caffeine
*Cold liquids
List some drugs that are used to manage pain in sickle cell patients:
*Methadone
*Morphine
*Hydroxyuria
*Dilaudid
*Fentanyl patch
In the pathophysiology of sickle cell disease, what happens to the Hgb S cell?
It assumes a sickled appearance
What happens to the blood's viscosity when the RBC's sickle?
The viscosity is increased
True or False: sickled cells increase RBC destruction
True
What treatment for sickle cell disease is aimed at diluting the HgbS levels?
Chronic transfusions
What lab levels should be monitored in a patient who is undergoing chronic transfusions?
*Iron
*TIBC
*Ferritin
What should be avoided in patients with sickle cell disease?
*Caffeine
*Cold liquids
In the pathophysiology of sickle cell disease, what happens to the Hgb S cell?
It assumes a sickled appearance
What happens to the blood's viscosity when the RBC's sickle?
The viscosity is increased
What happens to the oxygen carrying capacity of red blood cells when they are sickled?
It is decreased
True or False: sickled cells increase RBC destruction
True
What happens to circulation when the RBC's are sickled?
Circulatory stasis occurs
What happens to the oxygen carrying capacity of red blood cells when they are sickled?
It is decreased
What is a critical intervention for a patient in sickle cell crisis?
Bedrest
What happens to circulation when the RBC's are sickled?
Circulatory stasis occurs
What is a critical intervention for a patient in sickle cell crisis?
Bedrest
What kind of gene is affected in hemophilia A?
Sex-linked recessive gene
Why is almost 100% of hemophilia A found in men?
The gene is linked to the X chromosome, and males only have one X chromosome.
What clotting factor is deficient in hemophilia A?
Factor VIII
What is the preventive treatment for children with sickle cell disease?
*Penicillin at age 3 mo.
*Pneumococcal vaccine @ 2, 4 & 6 mo.
*Flu shot @ 6 mo.
What should be taught to parents of sickle cell patients?
*Recognizing s/sx
*Charting growth/development
*Give folic acid early
*Give protein supplements
*Get eyes tested early
What is Partial chimerism?
A transfusion in which a donor's bone marrow is mixed with the patient's bone marrow and administered to the patient
What clotting factor is deficient in hemophilia B?
Factor IX
What clotting factor is deficient in hemophilia C?
Factor XI
What is the difference between hemophilia and DIC?
Hemophilia: involves only intrinsic clotting factors

DIC: involves both intrinsic and extrinsic clotting factors
What is a good physical activity to suggest for a patient with hemophilia?
Swimming
When is hemophilia usually diagnosed?
In early childhood (when the child begins to walk)
What is the usual treatment for hemophilia?
Replacement of coagulation factor that is affected
What type of deformities are a complication of hemophilia?
Joint deformities
What are the signs/symptoms of hemophilia?
*Prolonged bleeding anywhere in the body
*Bruising
*Hematuria
What laboratory finding will be abnormal in a patient with hemophilia?
PTT
Will the PT lab value be normal or abnormal in a patient with hemophilia?
Normal
What is DIC?
Disseminated Intravascular Coagulation
How will the PT / INR reflect in lab reports of a patient with DIC?
Prolonged
How will the activated partial thromboplastin time (APTT) reflect in lab reports of a patient with DIC?
Prolonged
How will the platelet count reflect in lab reports of a patient with DIC?
Decreased
How will the fibrinogen levels reflect in lab reports of a patient with DIC?
Decreased
How will the fibrinogen degradation product levels reflect in lab reports of a patient with DIC?
Increased
How will the D-Dimer test reflect in lab reports of a patient with DIC?
Increased
What is the cure for DIC?
Treat the cause
True or False: Once you have DIC, you are prone to get it again
False
What are some nursing priorities in patients with DIC?
*Maintain fluid balance
*Control bleeding
*Restore normal clotting factors
What are some important things to remember when caring for a DIC patient?
*No blood pressure cuffs
*Turn carefully
*Be careful with catheters, ET tubes, etc.
What level of D-Dimer proteins indicate DIC?
Greater than 250 per ml
Explain erythropoesis:
Kidneys respond to low O2 and release erythropoetin, which travels to the bone marrow to stimulate RBC production
What are the 5 types of WBC's?
*Neutrophils
*Lymphocytes
*Eosinophils
*Monocytes
*Basophils
What is the largest components of white blood cells?
Neutrophils
In regards to WBC's, what indicates a patient's ability to fight infection?
The number and percentage of neutrophils
How is the absolute neutrophil count calculated?
*Add the neutrophils plus the bands
*Write the sum as a decimal or percentage
*Multiply this number by the total WBC number
With regards to WBC's, what is a "left shift?"
The number of bands is greater than everything else
What do the granules of basophils and mast cells release?
Histamines
What happens to the lungs when histamines are released?
Airways swell
What happens in the nasal passages when histamines are released?
*Vasodilation
*Stuffy, runny nose
What is the normal range for platelets?
150-440
What happens to platelets in a patient with sickle cell disease?
They are increased
When is AIDS diagnosed?
When the body loses the ability to fight opportunistic infections
What must the CD4 count be in order to be classified as AIDS?
Below 200
What does the HIV retrovirus have that helps viral replication?
Reverse transcriptase (RT)
What does Reverse Transcriptase do?
Forces DNA to use the virus as a "pattern"
After the Reverse Transcriptase forces the body's DNA to use HIV as a pattern, what takes place?
The new viral DNA gets into the body's DNA
In AIDS, how many viral particles per day can synthesize?
2 billion
What does the HIV virus do to the CD4 receptors?
Attaches to, infects and kills all immune cells with the CD4 receptors
What is significant of AIDS as opposed to HIV?
Profound immunodeficiency
What is the most common life threatening opportunistic infection in AIDS patients?
Pneumocystis Carinii (PCP)
What type of infection is pneumocystis carinii?
Protozoa and fungal infection
How is pneumocystis carinii (PCP) transmitted?
Via air, food, water
What percentage of AIDS patients have PCP as their first bout of AIDS-related opportunistic infection?
60%
What are the symptoms of pneumocystis carinii (PCP)?
*Fever
*Fatigue
*Weight loss
*SOB
*Crackles
*Cough
What is the treatment for pneumocystis carinii (PCP)?
*Pentamidine (aerosol)
*Mechanical ventilation
*aerosolized bronchodilators
*TMP-SMX (sulfa compound)
*Steroids
What is a serious side effect of TMP-SMX?
Nephrotoxicity
What is the most common malignancy in HIV patients?
Kaposi's Sarcoma
Besides skin lesions, what are some other symptoms of Kaposi's Sarcoma?
*Diarrhea
*Blockage of lymph leading to edema of face or extremities
*Respiratory distress
*Cognitive changes
What is the treatment for Kaposi's Sarcoma?
*Radiation
*Chemotherapy
*Interferon
What is Cryptosporidium?
A parasite
How is Cryptosporidiosis transmitted?
Animal to human or human to human
What is the most common site of cryptosporidiosis infection?
Small intestine
What is the most distressing symptom of cryptosporidiosis?
Copious explosive diarrhea (15-20 liters/day)
True or False: cryptosporidium can be killed by bleach
False
By what cycle is cryptosporidium transmitted?
Fecal-oral cycle
Besides the diarrhea, what are some other symptoms of cryptosporidiosis?
*Weight loss
*Dehydration
Is there an effective treatment for cryptosporidiosis?
No
What drug can be given to patients with cryptosporidiosis?
Octreotide (to lessen diarrhea)
What is significant of Octreotide?
It has lots of side effects
What is the screening test that measures HIV antibodies?
ELISA
What is the confirming test for HIV?
Western Blot
Why is the CD4 count measured in HIV patients?
This is used to measure the progression of the disease and the effectivity of the medication
How often should an HIV patient have CD4 counts measured initially?
Every 3-6 months
What is the WBC count like in a patient with HIV/AIDS?
Low
What does the Viral culture/Viral Load Test measure?
The genetic material of HIV; this indicates amount of disease progression and effectivity of medication
How often should an AIDS patient have a Viral Load Test?
Every 2-8 weeks
What is the drug regimen for HIV patients who are pregnant?
ZDV (a combination of 3 drugs)
When is ZDV therapy started in a pregnant woman with HIV?
14-34 weeks gestation
When is ZDV administered IV in a pregnant woman with HIV?
During delivery
ZDV is given in liquid form to babies of HIV mothers at what frequency and duration?
*Every 6 hours
*For the 1st 6 weeks of life
How are babies tested for HIV?
By looking for the virus itself (not the antibodies as in adults)
ZDV therapy in mothers with HIV is shown to reduce the risk of passing HIV to the baby by what percentage?
70%
When is a c-section delivery recommended for pregnant women with HIV?
*If viral load is greater than 1000
*If membranes are ruptured
*If no HIV drug therapy
*if no prenatal care
How often should a room/bathroom be cleaned for immunocompromised patients?
Every day
How often should the vital signs be checked in an immunocompromised patient?
Every 4 hours
A minor elevation in temperature for an immunocompromised patient can indicate what?
Sepsis
How often should the mouth and skin of an immunocompromised patient be assessed?
Every 8 hours
How often should open areas/IV sites, etc. be assessed in an immunocompromised patient?
Every 4 hours
How often should IV tubing be changed for an immunocompromised patient?
Every day
How often should wound dressings be changed in patients who are immunocompromised?
Every day
How often should WBC's (esp. ANC) be monitored in immunocompromised patients?
Every day
Immunocompromised patients should not drink water that has been standing for how long?
Longer than 15 minutes
What do the initials HAART stand for?
Highly Active Antiretroviral Therapy
What is HAART?
A combination of 3 or more anti-HIV medications in a daily regimen
How do the Nonnucleoside Reverse Transcriptase Inhibitors (NNRTI's) work?
Bind to and disable reverse transcriptase
How do Nucleoside Reverse Transcriptase Inhibitors (NRTI's) work?
*NRTI's are faulty versions of building blocks HIV needs to make more copies of itself

*When HIV uses an NRTI instead of a normal building block, reproduction of the virus is stalled
How do Protease Inhibitors (PI's) work?
Disable protease, a protein that HIV needs to make more copies of itself
How do Fusion Inhibitors work?
By blocking HIV entry into cells
What does HIV do to CD4 cells?
Destroys them
In the morphology of leukemia, what does "lympho-" indicate?
Originating from lymphoid or lymphatic system
In the morphology of leukemia, what does "Myelo-" indicate?
Originating from bone marrow
In the morphology of leukemia, what is indicated by the words "blastic" and "acute"?
Sudden; involves immature white cells
In the morphology of leukemia, what is indicated by the words "cytic" and "chronic?"
Over time; involves mature white cells
To what area/body system is leukemia prone to travel, yet chemotherapy does not reach this area/body system?
Brain/CNS
From what cells does Acute Lymphocytic Leukemia (ALL) arise?
Lymphoblasts
80% of ALL diagnoses are what age group
children
What is the peak age of diagnosis of ALL?
2-4 years
There is a sharp decline in diagnosis of ALL after what age?
10 years
What percentage of children with ALL can be cured?
50%
ALL is more common in what gender?
Males
In patients with ALL, a higher WBC indicates what?
A poorer prognosis
In regards to ALL prognosis, which diagnosis age groups do better?
*Diagnosed at age 2-9: better prognosis
*Diagnosed before age 2 or after age 10, poorer prognosis
Which gender has a better prognosis with ALL?
Females
What should be done for patients who have an ANC of less than 500?
*Neutrapenic precautions
*Reverse isolation
What is a normal ANC level?
Greater than 500
From what kind of cells does Acute Myelogenous Leukemia (AML) arise?
Single myeloid stem cell (myeloblast)
What is a myeloblast?
Precursor to a granulocyte
There is a sharp decline in diagnosis of ALL after what age?
10 years
What percentage of children with ALL can be cured?
50%
AML causes the development of what in the bone marrow?
Immature myeloblasts
At what age does AML occur more frequently?
*In adolescence (12-20 years)
*After age 55
ALL is more common in what gender?
Males
What is the common treatment for AML?
Bone marrow transplants
In patients with ALL, a higher WBC indicates what?
A poorer prognosis
From what type of cells does Chronic Lymphocytic Leukemia (CLL) arise?
Lymphocytes
In regards to ALL prognosis, which diagnosis age groups do better?
*Diagnosed at age 2-9: better prognosis
*Diagnosed before age 2 or after age 10, poorer prognosis
Which gender has a better prognosis with ALL?
Females
CLL is more common in what age group?
50-70 years old
What should be done for patients who have an ANC of less than 500?
*Neutrapenic precautions
*Reverse isolation
What is a normal ANC level?
Greater than 500
From what kind of cells does Acute Myelogenous Leukemia (AML) arise?
Single myeloid stem cell (myeloblast)
What is a myeloblast?
Precursor to a granulocyte
AML causes the development of what in the bone marrow?
Immature myeloblasts
At what age does AML occur more frequently?
*In adolescence (12-20 years)
*After age 55
What is the common treatment for AML?
Bone marrow transplants
From what type of cells does Chronic Lymphocytic Leukemia (CLL) arise?
Lymphocytes
CLL is more common in what age group?
50-70 years old
CLL is more common in which gender?
Males
What are the WBC levels like in a patient with CLL?
High
What is the survival rate for those diagnosed with CLL?
4-5 years
From what cells does Chronic Myelogenous Leukemia (CML) arise?
Granulocytes
Which leukemia has the Philadelphia chromosome marker?
CML
What is the survival rate for those with CML?
5-6 years
Which leukemia is associated with "blast crisis"?
CML
What is the prognosis for a CML patient in blast crisis?
2-4 months
What is the treatment for CML patients?
Bone marrow transplant
What are some symptoms of CML?
*Fatigue
*Anorexia
*Splenomegaly
*Weakness
*Weight Loss
How should the nurse explain the pain of bone marrow aspiration procedure?
"you will experience a brief, sharp pain"
What is involved in the diagnosis of CML?
*Elevated WBC (15,000-500,000)
*Granulocytic hyperplasia in the bone marrow
What should the nurse monitor in leukemia patients in regards to oxygenation and circulation?
*Anemia
*Hemorrhage
What is the hallmark for Hodgkin's Disease?
Reed-Sternberg cell
What age groups are affected by Hodgkin's Disease?
Any age group
What are the major symptoms of Hodgkin's Disease?
*Painless enlargement of a lymph node
*Fatigue
*Anorexia
*Unexplained fever
*Night sweats
What are some diagnostic tools for Hodgkin's disease?
*Lymph node biopsy
*Chest X-Ray
*CT scan
What is Multiple Myeloma?
Bone cancer; cancer of the plasma cells
Multiple myeloma is a WBC cancer that involves what kind of cells?
More mature cells
What is the onset of multiple myeloma like?
Slow and insiduous
What are the main problems with multiple myeloma?
*Pathologic bone fractures

*Renal failure
What are some other symptoms of multiple myeloma?
*Back pain
*Paralysis
*Constipation
*Hypercalcemia
*Hyperuricemia
*URI
Is multiple myeloma common?
No; very uncommon
At what age is multiple myeloma usually diagnosed?
Around age 50
What is a very important treatment for people with multiple myeloma?
Hydration: approx. 3000 cc's per day
Besides hydration, what are some other treatment measures for multiple myeloma?
*Glucocorticoids
*Calcitonin
*Chemotherapy
*Radiation
*Bone marrow transplant
Which multiple myeloma treatment has a poor prognosis?
Bone marrow transplant
What is the percentage of remission with multiple myeloma?
50%
What is the hallmark of multiple myeloma?
Bence Jones protein
What type of diet is recommended to prevent calculus formation in a multiple myeloma patient?
Low calcium
What lab values should be checked often in multiple myeloma patients?
BUN/Creatinine