Nursing Pathophysiology Note Cards

Upper airway obstruction condition in which sites of anatomic narrowing such as the hypo pharynx at the base of the tongue and the false and true vocal cords at the laryngeal opening are obstructed.

Vasogenic edema an accumulation of fluid in the cerebrum that is typically caused by an increase in capillary endothelial cell permeability and usually occurs near a tumor.

Ventilation-perfusion ratio (V/Q) the relationship between ventilation and blood flow in the lung that is measure by calculating the difference between the alveolar and arterial partial pressured of oxygen.

Ventricular septal defect a congenital malformation in which the wall between the left and right ventricles has a hole that allows blood to travel between the left and right ventricles potentially leading to congestive heart failure.

Vital capacity Maximal amount of air that can be taken in and exhaled with forceful expiration.

Von Willebrand disease an inherited disease in which the von Willebrand factor proteins that are made in the blood vessel and function to control platelet activity are abnormal or absent resulting in a tendency to hemorrhage.

Wheezing is a continuous, high pitched, whistling sound; it is significant for obstruction or tightness in the small airways.

Hemoglobin the iron-containing oxygen-transport metalloprotein in the red blood cells. Transports oxygen from the lungs to the rest of the body.

Hematocrit also called packed cell volume (PCV), is the proportion of blood volume that is occupied by RBCs. 47% in men, 42% in women.

Paroxysmal nocturnal dyspnea (PND) waking up suddenly during the night, feeling short of breath.

Parasympathetic nervous system division of the ANS cardiac muscle, smooth muscle, glandular tissue, “rest and repose”.

Ventilation, diffusion, perfusion, respiration 4 major processes occuring as you breathe.

Ventilation process of moving air into and out of the trachea, bronchi, and lungs

Diffusion process of moving and exchanging the oxygen acquired during ventilation and carbon dioxide waste across the alveolar capillary membranes.

Perfusion process of supplying oxygenated blood to the lungs and organ systems via the blood vessels.

Respiration process in which cells throughout the body use oxygen aerobically to make energy.

Tidal Volume is the amount of air that is exhaled after passive inspirations: this is the volume of air going in and out of the lungs at rest; in adults, this volume is approximately 500 cc.

Vital Capacity is the maximal amount of air that can be moved in and out of the lungs with forced inhalation and exhalation.

Forced vital capacity is the maximal amount of air that is exhaled from the lungs during a forced exhalation.

Forced expiratory volume in 1 second (FEV1) the maximal amount of air that can be expired from the lungs in 1 second.

Residual volume the volume of air that remains in the lungs after maximal expiration.

Total lung capacity (TLC) the total amount of air in the lungs when they are maximally expanded and is the sum of the vital capacity and residual volume.

Acute characterized by sudden onset

Aerobe An organism that lives in an oxygen environment

Ambulatory Able to walk

Anaerobe An ogranism that lives in an oxygen free environment

Anaplasia The irregular structural characteristics of a cell that identify it as a malignant cancer cell

Anomaly An abnormal occurance especially in reference to birth defects

Antiboiotic A chemical substance derivable from a mold or bacterium that kills microorganisms & cures infections

Antibody A protein produced by the body as part of its defense against foreign bacteria or blood cells

Antisepsis The prevention of sepsis by excluding or destroying microorganisms

Antiseptic A substance that kills or prohibits the growth of microorganisms

Asepsis Free from germs

Atrophy A wasting away or decrease in the size of cell, tissue, organ or part of the body caused by lack of nurishment, inactivity or loss of nerve supply

Autoimmunity A situation in which the body produces an immune response against its own organs or tissues, causing severe inflammation & chronic conditions

Bacteria Microorganisms capable of reproduction; some strains cause infection and some are benefical

Benign Referring to a tumor, or abnormal growth that is not cancerous & does not invade nearby tissues or spread to other parts of the body

Chronic Slow developing, reoccuring

Degenerative Characterized by diminishing capabilities

Diagnosis The identification of disease or trauma

Disease An impairment of health that interferes with the body's ability to function normally

Disinfect The prevention of sepsis by excluding or destroying microorganisms

Endemic Characterizing a disease that exists a location or group of people all the time

Epidemic A sudden outbreak of disease in numbers much higher than normal

Etiology The study of the cause and origin of disease

Exacerbation A marked increase in symptoms or severity of disease

Fungus A mold, yeast or mushrooms; some fungi are beneficial; some, such as ringworm and athlete's foot, are not

Hereditary Genetically passed from parent to child

Hyperplasia An increase in the number of cells in an organ or tissue

Idiopathic Of unknown origin

Infection The invasion & growth of mircroorganisms that may cause cellular injury in tissue

Inflammation A protective response from the body in response to infection or injury, characterized by swelling, heat, redness and pain

Local Affecting only one part

Malignant Cancerous, A growth with a tendency to invade and destroy nearby tissue & spread to other parts of the body

Morbid Diseased or sick

Morbidity Any departure, subjective or objective from a state of physiological or psyiological well being

Neoplasm An abnormal growth of tissue that may be benign or malignant

Pandemic An epidemic that affects an expanded demographical area

Pathogenesis The origin and development of disease

Pathology The study of disease

Pathophysiology The study of how disease and/or trauma alters the normal functioning of the body

Signs The evidence of disease as perceived by the doctor

Sterilize To destory bacteria and other micoorganisms

Symptoms The subjective evidence of disease as perceived by the patient

Syndrome A group of signs or symptoms characterized of a particular disease or abnormal condition

Systemic Affecting the whole body

Trauma A physical injury or wound caused by an external force of violence, which may cause death or permanent disability.

Trauma is also used to describe severe emotional or psychological shock or distress

Virulence The ability of an organism to cause disease

Virus An intracellular parasite that causes disease

Erythropoiesis is? The production of RBCs

What is the most common (yet acute) complication of polycythemia? CVA from thrombosis, vessel rupture can cause bleeding, increased uric acid (due to cell breakdown....could lead to gout) IF YOU LIVE! Cause the thrombosis could kill ya!

T/F cardiac problems occure at the same time the child stops breathing False - After a child stops breathing, cardiac problems appear which is opposite from adults

A newborn with oxygen distress can have up to ____ liters of oxygen but at this level can cause problems with ____. 6 liters can have problems with integument

6 cardinal signs of respiratory failure Restlessness, tachypnea, tachycardia, diaphoresis, decreased stridor or wheezing due to total obstruction, retractions without clinical improvement

Teaching plan for home care of BPD include: disease process, ability to evaluate infants respiratory status, feeding plan, meds, care of O2 equipment, special care needs and indications for emergency action with steps to be taken.

Prinzmetal Angina intermittent chest pain at rest, generally considered to be caused by vasosasm

unstable angina prolonged or recurrent pain at rest, often indicative of imminent MI

Stable angina most common, pain is precipitated by excertion and is releived by rest or NTG

Stable angina results from severe narrowing of artherosclerotic coronary vessles, which are thus unable to supply sufficient oxygen to support increased mocardial demands of exertion

MI characterized by myocardial coagulative necrosis caused by soronary artery occlusion, releases myocardial enzymes and other proteins by altered membrane permeability of necreotic cells

MI cells in the evolution are neutrophils, macrophages, and fibroblasts

MI transmural- involve enteric thickness of wall, subendocardial- inner 1/3 to 1/2 of wall

Complications of MI ventricular aneurysm, mural thrombosis, ruptured papillary muscles, myocardial rupture, myocardial fialure, arrythmia

arrythmia most common cause of death in the first several hours after MI

Myocardial failure can lead to CHF and/or shock

myocardial rupture usually occurs 4-7 days after MI and may result in death from cardiac tamponade

Rheumatic fever multisystem inflammatory disorder with major cardiac manifestations, aslo characterized by transient mild migratory polyarthritis

Rheumatic fever occurs 1-4 weeks after pharyngitis caused by group A beta hemolytic strep Aschoff Body classic lesions of rheumatic fever; is an area of focal interstitial myocarial inflammation that is characterized by large cells ( antischkow myocytes) and by occasional multinucleated giant cells

rheumatic fever carditis rheumatic endocarditis usually occurs in areas subject to greatest hemodynamic stress; in the early stages, the valve leaflets are red and swollen, and tiny warty, bead-like, rubbery vegetations form along lines of valve closure; valves become thickened,

manifestations of rheumatic fever (other than cardiac) fever, malaise, increased SED rate; joint involvement; skinlesions (subcutaneous nodules and erythema marginatum); CNS involvement( including sydenham chorea)

infective endocarditis bacterial or sometimes fungal infection of enocardium with prominent involvement of surfaces

infective endocarditis characterized by large, soft, friable, easily detached vegetation consisting of fibrin and intermeshed inflammatory cells and bacteria

infective endocarditis may be complincated by ulceration, often with perforation of valve cusps or rupture of one of the chordae tendineae

acute endocarditis is caused by staph aureus often secondary to infection elswhere in body

subacute endocarditis caused by less virulent oranisms such as strep viridans

subacute endocarditis tends to occur in patients with congenital heart disease or pre-existing valvular disease

endocarditis valvular involvment- mital (most frequent), mitral and aortic (40%), tricuspid (intravenous drug users)

enocarditis complication- distal embolization, septic infarcts, glomerulonephritis

nonbacterial thrombotic endocarditis associated with dibilitating disorders, such as metestatic cancer

nonbacterial thrombotic endocarditis characterized by small, sterile fibrin deposits randomly arranged along the line of closure of the valve leaflets; can result in peripheral embolization but, emboli are sterile libmen-sacks endocarditis ocurs in SLE, is characterized by small vegetations on either or both surfaces of the valve leaflets

endocarditis of carcinoid syndrome cuased by secretory products of carcinoid tumors (vasoactive peptides and amines, especially serotonin), results in thickened endocarcial plaques; rarely involves values on left side because lungs detoxify amines

mitral valve prolapse most frequent lesion, characterized by myxoid degenration of the ground substance of the valve

mitral valve prolapse results in stretching of posterior valve leaflet, producing a floppy cusp, produces systolic murmur with a midsystolic click

mitral valve prolapse usually benign, often associated with a variety of arrythmias, preidposes to infective endocarditis

mitral valve stenosis rheumatic heart disease

mitral valve insufficiency rheumatic hear diesase-, can result from mitral prolapse, inective endocarditis, or damage to a papillary muscle from MI, can be secondary to left ventricular dilation with stretching of the mitral valve ring

aortic stenosis often present as calcific aortic stenosis, caused by; otherwise normal valve(age related), a congenital bicuspid aortic vlave, a valve affected by rheumatic heart disease

tricuspid rarely invovled alone in rheumatic heart disease but may be involved together with mitral and aortic, may be involved in the carcinoid syndrome

pulmonary valve most commonly affected by congenital malformations, rarely involved in rheumatic heart disease, may be involved in carcinoid syndrome

Hypertrophy of right ventricle caused by left ventricle failure, chronic lung disease, mitral valve disease, congenital heart disease with left to right shunt

Hypertrophy of left ventricle most commonly caused by hypertension and aortic or mitral valvular disease

right sided heart failure caused by left sided failure, left sided lesion, pulmonary hypertension, cardiomyopathy, diffuse myocarditis, tricuspid or pulmonary valvular disease

right sided heart failure manifested by renal hypoxia leading fluid retention and peripheral edema, enlarged and congested liver and spleen, distention of neck viens

left sided heart failure caused by ischemic heart disease, hypertension, aortic and mitral valvular disease and mycoardial disease

left sided heart failure manifestated as dyspnea and orthopnea, pleural effusion, reduced renal perfusion

chronic pericarditis usually tuberculous or pyogenic staph etiology

chronic pericarditis thickening and scarring of pericardium with loss of elasticity

chronic pericarditis often micking signs and symptoms of right sided heart failure, proliferation of fibrous tissue

Serous pericarditis SLE, rhuematic fever, and a variety of viral infections-->clear straw colored protein rich exudate with small numbers of inflammator cells

ffibrinous or serofribrinous pericarditis caused by uremia, myocardial infarction, acute rheumatic fever--> fibrin rich exudate

purulent or suppurative pericarditis caused by bacterial infection--> grossly cloudy or frankly purulent inflammatory exudate

hemorrhagic pericarditis caused by tumor invastion of pericardium, TB or other bacterial infection--> bloosy inflammatory exudate

chronic pericarditis usually tuberculous or pyogenic staph etiology,causes proliferation of fibrous tissue and thickening and scarring of pericardium with loss of elasticity, often mimics the signs of right heart failure

left sided heart failure caused by ischemic heart disease, hypertension, aortic and mitral valvular disease and mycoardial disease

left sided heart failure manifestated as dyspnea and orthopnea, pleural effusion, reduced renal perfusion

right sided heart failure caused by left sided heart failure, left sided lesion, pulmonary hypertension, cardiomyopathy and diffuse myocarditis,tricuspid or pulmonary valvular disease

right sided heart failure manifested by renal hypoxia leading to fluid retention and peripheral edema, enlarged and congested liver and spleen, distention on neck veins

hypertrophy of left ventricle most commonly caused by hyoertensin or mitral valvular disease

hypertrophy of right ventricle caused by left ventricular failure, chronic lung disease, mitral valve lesions, congenital heart disease with left to right shunt

cor pulmonale right ventricular hypertrophy and or dilation secondary to lung disease, characerized by pulmonary hypertension

hypertrophic cardiomyopathy hypertrophy of all 4 chamber walls, especially the ventricular septum; characterized by disoriented tangled and hypertophied myocardial fibers, inherited autosomal dominant, may result in left ventricular outflow obstruction congested or dilated cardiomyopathy

most common myopathy, 4 chamber hypertrophy and dilation and both right and left sided failure; in some cases it is related to alcoholism, thiamine deficiency, or prior myocarditis

cardiomyopathy refers to diseases of the heart that are non inflammatory and are not associated with hypertension, congential heart disease, valvular disease, or coronayr artery disease; usually characterized by otherwise unexplained ventricular dysfunction restrictive cardiomyopathy caused by infiltrarive processes within myocardium that results in stiffining of heart muscle, wich inteferes with pumping action; exemplified by cardiac amyloidosis, which are reight and left sided failure

Circulation of heart to the lungs Cardiopulmonary Circulation Signs and symptoms of hyperventilation: Tachycardia, SOB, dizziness & kightheadedness, numbness, tinnitus, disorientation, chest pain, decreased concentration, tetany, muscle spasms

Signs & Symptoms of hypoventilation: Dizziness, disprientation, headache, lethargy, coma, & convulsions, heart arrythmias, cardiac arrest

Inadequate tissue O2 at cellular level Hypoxia

Hypoxia causes: Decreased Hgb levels, decreased in inspired O2 & impaired ventilation, inability of tissues to extract O2 from blood, inability of alveoli to perfuse O2 to blood

Signs and symptoms of hypoxia: SOB, dyspnea, apprehension, behavior changes, heart arrythmias, dizziness, tachycardia, tachypnea, restlessness, decreased level of consciousness, cyanosis

Cardiac pain in men: Sub sternal & radiates to left arm & jaw

Cardiac pain in women: Epigastric pain, c/o indigestion, chocking feeling, & dyspnea

Suctioning: After suctioning oro/ nasal Oxygenerate with 100% O2 for a few minutes

Tube from mouth or nose to trach Endotracheal tube

The last part to be suctioned: The mouth: always suction mouth last, clean technique

Suctioning: Endo/trach Use sterile technique - pre & post oxygenate 10 - 15 seconds

To clean Tracheal tubes: Hydrogen peroxide and sterile normal saline

Encouraging Fluids: Removes mucus and cellular debris from respiratory tract. Prevents thickening of pulmonary secretions

How would you treat a patient with hemophilia Blood transfusion if needed, NO ASPIRIN OR ANTI COAGULANTS, ice and rest for hemarthrosis as there will be pain, redness, and swelling

Monckeberg Arteriosclerosis calcification of tunica media, medium sized, muscular arteries

Obliterans Arteriosclerosis proliferation intima, small vessels, complete obliteration of artery lumen

Peripheral Arteriosclerosis arteriosclerosis of extremites

Arteriosclerosis arterial wall becomes thick & loses elasticity

Atherosclerosis form of arteriosclerosis - .y thickening of subintimal wall

Buerger's Male, smoker

Marfan's Dissecting aneurysm common

Prinzmetal angina at rest

Cor pulmonale right sided heart failure

Dissecting Aneurysm longitudinal cleavage of the arterial media by a column of blood

Dissecting Aneurysm tearing pain, acute onset

Berry Aneurysms Subarachnoid & intracerebral hemorrhage, hereditary, 40% ant. comm. art. (Circle of Willis)

Abdominal Aortic aneurysm can be asymptomatic, lumbar vertebrae pressure

Wolf's law change in anterior body from aneurysm

Patent Ductus Arteriosus congenital failure of closure between the pulmonary artery & aorta (up to 80% premature)

Cor Pulmonale can include emphysema & chronic bronchitis

Tetralogy of Fallot Dextrorotation of the aorta, Right ventricular hypertrophy, Intraventricular septal defect, Pulmonary artery stenosis

Rheumatic Fever Mitral & Aortic valves affected

Raynaud's Disease vasospastic disorder of small arteries (especially hands)

Microcytic Hypochromic Iron deficiency, Chronic hemmorrhage

Macrocytic normochromic Pernicious, Folate

Guiac Test hidden blood

portal hypertension esophageal varices

Helocobacter pylori ulceration in stomach

No Meissner's & Auerbach's Hirschsprung's

Air emboli deep sea diving

paradoxical embolism blockage of systemic artery by a thrombus originating in a systemic vein that has passed through a defect in the interatrial or interventricular septum

Miliary embolism affecting many small blood vessels

Stable angina pain with activity, emotional excitment or other increased cardiac work

Unstable angina close to MI, increased frequency, prolonged duration

Transmural infarction full of nearly full thickness of the ventricular wall

Subendocardial infarction inner 1/3 or at most 1/2 ventricular wall

Risk Factors for arteriosclerosis hypertension, inc. serum levels, smoking, DM, obesity & male

Angina Pectoris Chest pain due to inadequate oxygenation of the myocardium.

Cardiac Dysrythmias Abnormal elecrical condition in theheart or abnormal changes in heart rate and rhythm.

Fibrillation An irregular, quivering and ineffective type of myocardial contraction.

Congenial Heart Defect A heart defect present at birth but not necessarily hereditary.

Coronary Heart Disease A commonly used term for any disease affecting the heart.

Pericardis An inflammation of the pericardium.

Myocarditis An inflammation of the heart muscle.

Endocarditis An infalmmation of the endocardium, the heart valves and the inner lining of the blood vessels attached to the heart.

Myocardial Infarction A heart attack.

Rheumatic Fever An infectious disease generally caused by the Streptococcus bacteria. Valvular Heart Disease A dysfunction of any of the four valves.

Community acquired pneumonia (CAP) strep, hemophilus, influenza, staph

Hospital acquired pneumonia (HAP) (nosocomial) ecoli, MRSA Centilator acquired pneumonia with in 48 hours of intubation

aspiration pneumonia caused by gastric contents or oropharyngeal bacteria

S&S of pneumonia dyspnea, fever chills, cough, purulent sputum, .les, confusion and tachypnea, CBC, blood clutures, ABG's

people at risk for pneumonia age 65 or older, under 65 and have a chronic disease, psplenia or splenic dysfunction

Acute hypoxic injury ARDS

direct injury aspiration, pulmonary infection, near drowning, thoracic trauma or toxic inhalation

indirect injury shock, sepsis, hypothermia, DIC, multiple transfusion eclampsia, pancreatitis, burns

presentation of pneumonia bilater infiltrates on CRX, non cardiogenic pulmonary edema, hypoxemia despite increaseing FiO2, increasing peak ariway pressure and reduced lung compliance

early phase of pneumonia edematous-mediators released, damaging alveolar capillary membrane

late phase pneumonia fibrotic and brittle lungs

hypoxia tachypnea, restlessness and apprehension, moderate increased work of breating, all worsening until resp failure develops

leasing cause of hospital admission for children asthma

asthma a chronic inflammatory disease of the airways, hyperresponsiveness, variable ariway obstruction, resolves spontaneously or after using a bronchodilator

testing for asthma spirometry, pulmonary function testing

common triggers of asthma upper resp tract infection, allergens, exercise, medication (b-blockers), food (sulfites), emotions

S&S of PE dyspnea, sudden onset of pleuritic chest pain (worsens with breathing), cough, tachypnea, rales, tachycardia, sinus tachycardia

What is used to diagnosis PE spiral CT

Prevention of PE compression stockings, SCD's, ambulation, hydration, anticoagulation, inferior vena cava filter

What is PTT used to test for heparin

What is PT used to test for comadin warfarin

disruption of the nurmal negative pressure in the pleural space pleural space disease

pheumothorax air in the pleural space

hemothorax blood in the pleural space

pleural efusion accumulation of pleural fluid secondary to increased fluid formation empyema pus infection

What are the effects of anemia on the circulatory system? Hemodilution, decreased peripheral resistance, increased cardiac circulation and turbulence,(may have murmur, may lead to cardiac failure), cyanosis, growth retardation.

What is the theraputic management of anemia? transfusion after hemorrhage if needed, nutritional intervention for deficiency anemias, IV fluids to replace intravaxcular volume, O2, bedrest.

What are the nursing considerations for anemia? Prepare child & family for lab tests, decrease 02 demands, prevent complications, support family

What is the cause of iron deficient anemia? Inacequate supply of dietary iron.

How to prevent iron deficient anemia? iron-fortified cereals and formulas for infants, special needs of premature infants, adolescents at risk due to rapid growth and poor eating habits.

What is sickle cell Anemia? A hereditary hemoglobinopathy.

Who does sickle cell effect? primarily african americans,occasionally occurs in mediterranean decent, also seen in south americans, arabian, and east indian descent.

How often does sickle cell occur ? 1 in 375 infants born in the US, 1 in 12 have sickle cell trait,

What is the etiology of sickle cell? Autosomal recessive disorder, 1 in 12 african americans are carriers(have trait), if each parent has trait each offspring has 1 in 4 chance of having disease.

What is the patho of sickle cell? partial or complete replacement of normal Hgb with abnormal hemagloin S(Hgb S),

What do the blood cells look like with sickle cell? Hemaglobin in the RBCs takes on an elongated "sickle" shape

When the RBCs are sickled shape how does this effect blood flow? The sicked cells are rigid and obstruct capillary blood flow, microscopic obstructions lead to engorgement and tissue ischemia/death, hypoxia(lack of 02 ) occurs and causes sickling,large tissue infarctions occur, damaged tissue in organs impairs fuction
What types of surgery might occur with sickle cell? splenectomy at early age

What is the prognosis for pt. with sickle cell? No cure, supportive care/prevent sickling episodes, frequent bacterial infectionds due to immunocompromise, strokes in 5% to 10% of children with disease.

What is the leading cause of death for chidren with sickle cell? bacterial infections

What are the precipitating factors of a sickle cell crisis? anything that increases body's need for 02 or alters transport of 02, trauma, infection, fever, stress, dehydration(increased blood viscosity), hypoxia(from high altitude poorly pressurized)

What are the manifestations of a sickle cell crisis? Acute exacerbations that vary in severity and frequency

Types of sickle cell crisis? vaso-occlusive[VOC] thrombotic, splenic sequestion, aplastic crisis

What is Vaso-occlusive thromotic sickle cell crisis? most common type of crisis-very painful, stasis of blood with clumping of cells in microcirculation-ischemia-infarction

What are the signs of Vaso-occlusive thrombotic sickle cell crisis? fever, pain, tissue engorgement

What is splenic sequestration sickle cell crisis? life threatening-death can occur within hours, blood pools in the spleen

What are the signs of splenic sequestration sickle cell crisis? profound anemia, hypovolemia, and shock

What is aplastic crisis in sickle cell? diminished production and increased destruction of RBCs, triggered by viral infection or depletionof folic acid

What are the sighs of aplastic crisis in sickle cell? profound anemia, pallor

How do they diagnose sickle cell? cord blood, newborn screen (43 states) genetic testing to identify carriers and children who have disease, sickle turbidity test (quick screening purposes in children less than 6 months old)

What is the medical management for sickle cell? aggressive treatment of infection, possible prophylactic ATBs from 2 months to 5 years, monitor reticulocyte count regularyly to evaluate bone marrow function, blood transfusion(if given early in crisis may reduce ischemia)

What does frequent transfusion do in sickle cell crisis? decreases hemosiderosis ( iron in tissues)

How do you treat hemosiderosis(iron in tissues)in sickle cell crisis? treat with iron chelation such as feroxamine, + vitamin C to promote iron excretion

Rx medications used in sickle cell? hydoxyurea (cytotoxic) decreases production of abnormal blood cells and decreases pain

What is the nursing considerations for child with sickle cell crisis? Monitor childs growth-watch for failure to thrive, careful mutisystem assessment, assess pain, observe for presence of inflammation or possible infection, carefully monitor for signs of shock

What are the phychosocial needs of children/family with sickle cell? coping mechanisms, support with genetic counseling, financial needs, caregiver role strain, living with chronic illness int he family

What is thalassemia? inherited blood disorders of hemoglobin synthesis, classified by Hgb chain affected and by amount of effect, autosomal reciessive with varying expressivity(both parents must be carriers to have child with disease)

What is the pathophysiology of Thalassemia? Anemia results from defective sythesis of Hgb, structurally impaired RBCs, and shortened life of RBCs,chronic hypoxia, detected in infancy or toddlerhood.

What are the S/S of chroic hypoxia with thalassemia? headache, irritability, precordial and bone pain, exercise intolerance, anorexia, epistaxis.

Physical manifestations of thalassemia? pallor, FTT, Hepatosplenomegaly, severe anemia (Hgb less than 6)

Who does it affect/what happens in a-Thalassemia? Alpha chains affected, occurs in chinese, thai, african and mediterranean people

Who does b-Thalassemia occur in ? Occurs in greeks, italians and syrians, b is most common and has four forms.
Thalassemia minor asymptomatic silent carrier

Thalassemia trait mild microcytic anemia

Thalassemia intermediate moderate to severe anemia plus splenomegaly

Thalassemia major "Cooley's anemia" severe anemia requiring transfusions to survive

What are the four types of b-Thalassemia? Thalassemia minor, Thalassemia trait, Thalassemia intermediate, Thalassemia major "Cooley's anemia"
Diagnosis of Thalassemia? by hemoglobin electrophoresis, RBC changes often seen by 6 weeks of age, child presents with severe anemia, FTT

Medical management of Thalassemia? blood transfusion to maintain normal Hgb levels, side effect-hemosiderosis (treat with ironochelating drugs such as deferoxamine (Desferal-binds excess iron for excretion by kidneys))

Nursing care for Thalassemia? Observe for complications of transfusion, emotional support to family, encourage genetic counseling, parent and patient teaching for self-care

Prognosis for child with Thalassemia ? retarded growth, delayed or absent secondary sex characteristics, expect to live well into adulthood with proper clinical management, bone marrow transplant is potential cure

What is aplastic anemia? All formed elements of the blood are simultaneously depressed ("pancytopenia")

What is hypoplastic anemia? profound depression of RBCs but normal WBCs and platelets

Etiology of Aplastic anemia? primary (congenital) secondary (acquired), diagnostic evaluation, therapeutic management, nursing consideration

What is hemophilia? A group of hereditary bleeding disorders that result from deficiencies of specific clotting factors.

Types of hemophilia? Hemophilia A, Hemophilia B

What is hemophilia A? "Classic hemophilia", deficiency of factor Vlll, accounts for 80% of cases of hemophilia, occurence: 1 in 5000 males

What is Hemophilia B? Also known as christmas disease, caused by defiency of factor lX, accounts for 15% of cases of hemophilia

What is the etiology of hemophilia A? X-linked recessive trait, males are affected, females are carriers, degree of bleeding depends on the amount of clotting factor and severity of injury, up to 1/3 of cases have no known family hx (in these cases caused by a new mutation)

What are the manifestations of hemophilia? bleeding tendencies rage from mild to severe, symptoms may not occur until 6 months of age (mobility leads to injuries from falls and accidents), Hemarthrosis (bleeding into joint spaces of knee, ankle, elbow leading to impaired mobility, Ecchymosis

What clinical therapy can be done? Can be diagnosed through amniocnetesis, genetic testing of family members to identify carriers, diagnosis on basis of hx, labs and exam.

What labs help diagnose hemophilia? Low levels of factor Vll or lX, prolonged PTT, normal platelet count, PT and fibrinogen

Medical management of hemophilia? DDAVP- IV, causes 2 to 4 times increase in factor Vlll activity, used for mild hemophilia, replace missing clotting factors, transfusions-at home with prompt interventionto reduce complications, following major surgery or minor hemorrhage

Prognosis for hemophiliacs? Historically, most died by 5 years old, now mild to moderate hemophilia pt. live near normal lives, gene tharapy for future- infused carrier oranisms act on target cells to promote manufacture of deficient clotting factor.

Interventions for hemophiliacs? close supervision & safe invironment, dental procedures in controlled situation, shave only with electric razor, superficial bleeding -apply pressure for at least 15 minutes + ice to vasoconstrict, If significant bleeding -transfuse for factor replacement

Management of Hemarthrosis? During bleeding episoses, elevate and immobilize joint, ice, analgesics, ROM after bleeding stops to prevent contractures, PT, Avoid Obesity to minimize joint stress.

What is von Willebrand Disease? A hereditary bleeding disorder, involving deficiency of von Willebrand factor( a plasma protein, & the carrier for factor Vlll), von Willebrand factor is necessary for platelet adhesion, transmitted as autosomal dominant trait, males &females,chromosome12

What are the manifestations of von Willebrand disease? easy bruising, epistaxis, gingival bleeding, excessive bleeding with lacerations or surgeries, menorrhagia

Lab test for von Willebrands? decreased von Willebrand factor & antigen levels, decreased platelet agglutination, prolonged bleeding time, PTT may be normal or prolonged.

Treatment of von Willebrand disease? Infusion of von Willebrand's protein concentrate, DDAVP infusion before surgery or to treat bleeding episode, Aminocaproic acid to treat bleeding in mucous membranes(in some cases)

Interventions for von Willebrands disease? Avoid aspirin or NSAIDs (increase bleeding time and inhibit platelet fuction), manage bleeding episodes with prompt infusion therapy, children with von Willebrand have normal life expectancy if well managed. Idiopathic Thrombocytopenic Purpera (ITP) An acquired hemorrhagic disorder characterized by: thrombocytopenia(excessive destruction of platelets), Purpura(discoloration caused by petechiae beneath the skin

Forms of Idiopathic Thrombocytopenic Purpura? Acute self-limiting-often follows URI or other infections, chronic ( greater than 6 months duration)

What is Disseminated intravascular Coagulation (DIC)? Disorder of coagulation that occurs as complication of numerous pathologic processes such as hypoxia, acidosie, shock, endothelial damage

Pathophysiology of DIC? Coagulation process is abnormally stimulated, Excessive amounts of thrombin are generated, fibrinolytic mechanisms are activated and cause extensive destruction of clotting factors.

Process of DIC? Trigger mechanism, intrinsic coagulation process, consumption of platelets and clotting factors-(bleeding tendency) or fibrin deposits in microcirculation(secondary fibrinolysis)-( ischemic tissue damage and RBC damage and hemolysis)

ICP diagnostic evaluation, therapeutic management prognosis nursing considerations

DIC diagnostic evaluation, therapeutic management, nursing considerations

Epistaxis (Nosebleed) Isolated and transient epistaxis is common in childhood, Recurrent or severe episodes may indicate underlying disease- vascular abnormalities, leukemia, thrombocytopenia, clotting factor deficiency diseases(von Willebrand and hemophilia) Nursing considerations for Epistaxis? remain calm, keep child calm, have child sit up and lean forward pressure to nose further evaluation if bleeding continues

Neoplastic Disorders in children? leading cause of death from disease in children bast infancy, almost half of all childhood cancers involve blood or blood-forming organs

Leukemias in children Most common form of childhood cancer, 3 or 4 cases per 100,000 caucasian children less than 15 year old,more frequently in males greater than 1, peak onset between 2 & 6 years old, survivability.

Classification of Leukemias Leukemia: broad group of malignant diseases of bone marrow and lymphatic system, complex disease with varying heterogeneity, classifications are increasingly complex

Mophology of leukemia Acute lymphoid leukemia (ALL), Acute nonlymphoid (myelogenous) leukemia (ANLL or AML), Stem cell or blast cel leukemia

Symptoms of Leukemia? ALL: lymphatic, lymphocytic, lyphoblastic, and lymphoblastoid leukemia, AML: granulocytic, myelocytic, monocytic myelogenous, monoblasic, and nonmyeloblastic leukemia

Chromosome studies for leukemia children with trisomy 21: 20 times greater risk for developing ALL, Children with more than 50 chromosomes on the leukemic cells have the best prognosis, Specicic translocations of chromosmes on leukemic cells can affect prognosis.

Pathophysiology of leukemia Leukemia is a n unrestricted proliferation of immature WBCs in the blood-forming tissues of the body, liver & spleen ar the most severly affected organs

More pathophisiology of leukemia Although leukemia is an overproduction of WBCs, often acute form causes low leukocyte count, cellular destruciton takes place by infiltation and subsequent competition for metabolic elements.

Consequences of Leukemia Anemia from decreased RBCs, infection from neutropenia, bleeding tendencies from decreased platelet produciton, spleen, liver, and lymph glands sho marked infiltration, enlargement, and fibrosis

Diagnostic evaluation? Based on hx, physical manifestations, peripheral blood smear(immature leukocytes, frequently low blood counts), LP to evaluate CNS involvement, bone marrow aspiration or biopsy

Therapeutic Management Chemotherapeutic agents, cranial irradiation (in some cases)

Four phases of Therapy for leukemia Induction to therapy:4 to 6 weeks, CNS prophylactic therapy: intrathecal chemotherapy, Intensification(consolidation) therapy: to eradicate residual leukemic cells and prevent leukemic clones, Maintenance therapy: to preserve remission

Hematopoietic stem cell trsplatation(HSCT) for leukemia patients donors may be relatives or nonrelatives, antigen natched or mismatched, peripheral stem cells may be used, stem cells form umbilical cord blood.

Risks of HSCT for leukemia patients significant risk of morbitiy and mortality, graft vs. host disease(GVHD), overwhelming infection, severe organ damage, cure after HSCT: up to 60% or 70%

Prognosis of HSCT leuekemia patients If released after HSCT: dismal prognosis, identified factors for determining prognosis: initial WBC count, age at the time of diagnosis, type of cell involvement, gender, karyotype analysis

Nursing considerations for leukemia patients recieving HSCT Assessment, nusing diagnosis, planning, implementation: prepare child and family for procedures, pain management, prevent complications of myelosuppression

Increased susceptibility to infection in leukemia patients At time of diagnosis and relaspe, during immunosuppressive therapy, after prolonged antibiotic therapy that predisposes to the growth of resistant organism.

Infection control for leukemia patients hand washing, environment, visitor restriction

Managing chemotherapeutic agents with leukemia "Vesicants"-sclerosing agents even in minute amounts, interventions for extravasation, risk for anaphylaxis

Managing problems of drug toxiciy in leukemia Nausea/vomiting, Anorexia, mucosal ulceration, neuropathy, hemorrhagic cystitis, alopecia, mood changes, moon face

Nursing diagnoses for leukemia patients Risk for injury related to malignant process & treatment, risk for deficient fluid volume related to nausea/vomiting, risk for imbalanced nutrition, impaired skin integrity, altered family processes, fear related to diagnosis, procedures, treatments

Types of Lymphomas Hodgkin disease-15 to 19 years of age, non-hodgkin lyphoma(NHL)- more prevalent in children less than 14 years old.

Hodgkin disease neoplastic disease originating in lymph system, often metastasizes to spleen, liver, bone marrow, lungs, and other tissues.

What are the four types of hodgkin disease? lymphocytic predominance, nodular sclerosis, mixed cellularity, lyphocytic depletion, accurate staging is basis for treatment and prognosis

Classifications of Hodgkin disease? class A: asymptomatic, class B: temperature of 38C or higher for 3 consecutive days, night sweats, unexplained wt. loss of 10% or more over previous 6 months.

Diagnostic studies for Hodgkin disease? clinical manifestations of hodgkin disease, lymph node biopsy for diagnosis and staging, presence of Reed-Sternberg cells is characteristic of Hodgkins disease.

Therapeutic management of Hodgkin? Radiation, chemotherapy (alone or with radiation), prognosis, nursing considerations
Non-Hodgkin lymphoma Approximately 60% of pediatric lymphomas are NHL

Clinical appearance of Non-Hodgkin lymphoma Disease usually diffuse rather than nodular, cell type undefferentiated or poorly differentiated, dissemination occurs early, often, and rapidly, mediastinal involvementand invasion of meninges. Non-hodgkin lymphoma Diagnostic evaluation, therapeutic management, prognosis, nursing considerations

Immunologic Deficiency Disorders HIV/AIDS-Epidemiology, etiology and pathophysiology, clinical manifestions, diagnostics, therapeutic management

Sever combined Immunodificiency Disease (SCID) therapeutic management, nursing considerations

Wiskott-Aldrich Syndrome Thrombocytopenia, Eczema, Immunodeficiency of selective functions of B-lymphocytes and T-lyphocytes, x-linked recessive

Blood transfusion Therapy for Wiskott-Aldrich syndrome verify identity of recipient and verification of donor's blood group, monitor VS, use appropriate filter, Use blood within 30 minutes of arrival, infuse over 4 hours maximum. Transfusion Reactions with Wiskott-Aldrich syndrome Hemolytic:the most severe, but rare, Febrile reactions: fever chills, Allergic reaction: urticaria, pruritis, laryngeal edema, Air emboli: may occur when blood is transfused under pressure, Hypothermia, Electrolyte disturbances: hyperkalemia

Nursing responsibilities with Wiskott-Aldrich syndrome blood transfusion Identify donor and recipient blood types and groups before transfusing, transfuse slowly for first 15 to 20 minutes, observe carefully for patient response, stop transfusion immediately fi signs/symptoms of transfusion reaction; notify practitioner.

Delayed reactions to blood transfusion transmission of infection--Hepatitis, HIV, malaria, syphillis, other, blood banks test vigorously and discard units of infected blood.

Family concerns for wiskott-aldrich sydrome difficult decision for HTSC transplant- child faces death without transplant, preparing child for transplant places child at great risk, No "rescue" procedure if complications follow HTSC transplant, nursing considerations

Apheresis in Wiskott-Aldrich syndrome Definition: removal of blood from an individual; separation of the blood into its components
Hematopoietic stem cell transplantation(HSCT) Used to establish healthy cells in both malignant and nonmalignant disease, Ablative Therapy: high-dose combinatin chemo (with or without radiation) to eradicate unhealthy cells and suppress immune system to prevent rejection of transplanted marrow.

Hematopoietic stem Cell Transplantation(HSCT) Stem cells harvested from bone marrow, peripheral blood, or umbilical vein of placenta, stem cells given to patient by IV transfusion, Newly transfused stem cells repopulate ablative bone marrow.

Allogeneic Bone Marrow Transplant (BMT) Involves matching histocompatible donor with recipient, Limited by presence of suitable marrow donor

Umbilical Cord Blood Stem Cell Transplantation Rich source of hematopoietic cells for for kids with cancers, Stem cells found with high frequency in circulation of newborns

Benifit of umbilical cord blood blood's relative immunodeficiency at birth, allowing for partially matched unrelated cord blood transplants to be successful.

Autologous Bone marrow transfusion Uses pt. own marrow collected from disease-free tissue, frozen and somtimes treated to remover malignant cells, Has been used to treat neuroblastoma, Hodgkins, NHL, Wilms' tumor, rhabdomyosarcoma, and Ewing sarcoma

Peripheral Stem Cell Transplants (PSCT) A type of autologous transplant, different type of collection from pt., stimulate producitonof high number of stem cells, then collect stem cells by an apheresis machine, stem cells separated from whole blood
PSCT continued Remaining blood cells and plasma returned to pt. after apheresis, stem cells frozen for later transfusion to pt. What type of cells are lymphocytes and monocytes? * WBC’s * Norm: 4500-11000 cells/mm3

An elevated WBC count can indicate what? * Infection

what is the % of Neutrophils in a normal WBC count 60%

Where are lymphocytes and monocytes produced? * Bone Marrow * Lymphocytes also produced by the Spleen

What 2 cell groups make up lymphocytes? T cells and B cells

What is the purpose of the B cells? * B cells-when stimulated, produce 2 types of cells: * Plasma cells-antibody factories that produce large amount of immunoglobulin which when released becomes the antibody. * 4 types of Immunoglobulin * Antigen-binding proteins

What are the 4 major antibodies produced by B cells? * IgM-first to be secreted during primary immune response to antigen

* IgG-secreted during second immune response-more specific to antigen * IgA-present in mucus and mothers milk * IgE-triggers release of histamine; can create a hypersensitivity reaction

IgM first to be secreted during primary immune response to antigen

IgG secreted during second immune response-more specific to antigen

IgA present in mucus and mothers milk

IgE triggers release of histamine; can create a hypersensitivity reaction

* IgE-triggers release of histamine; can create a hypersensitivity reaction * An immune system disorder * This type of reaction is potentially harmful * There are 4 types of hypersensitivity reactions: Type I, II, III, IV

4 types of hypersensitivity reactions Type I, II, III, IV

Type I mediated by IgE-reactions to common allergens, i.e., dust, pollen, bee stings, animal dander * Local: edema, pain --Systemic: anaphylaxis

Type II mediated by antibodies-occurs with blood tx or medications

Type III causes tissue damage from precipitation of antibody-antigen rx-autoimmune reactions, occupational diseases or various drugs

Type IV delayed rx-contact dermatitis, measles rash TB skin testing, transplant tissue rejection
What are memory B cells? * remember specific antigens, which triggers plasma cells to react quicker in producing immunoglobulin

Innate immunity always working, starts at birth-it is the inflammatory response and can phagocytize foreign invaders

Acquired immunity occurs when a foreign antigen is introduced into the body

active Acquired immunity Vaccinations

passive acquired antibody immunity Chicken Pox as a child

Cell-mediated-T cytokines attach to defective cells (primarily viruses and cancer) and directly attack them. T cells are a type of lymphocyte
HIV is a virus that cause s destruction of T cells leading to a decreased immunity. How does this occur? * HIV copies the RNA into the host T cell’s DNA and then remains hidden until the host cell is activated to mount an immunologic response

How is HIV transmitted? * Sexually Contact with infected body fluids

Initial stage: primary HIV infection may last 4-8 weeks. Pt may have flu like symptoms

2nd stage or Latent stage may last 2-12 years and have no symptoms

when the CD4 count drops below 200 the pt enters into the final stage and is diagnosed with AIDS. Once this occurs, even if the CD4 count rises again above 200, the pt will still be diagnosed with AIDS.

Once the CD4 count drops below 200 illnesses, known as opportunistic infections, occur. Some of these are pneumocystic pneumonia, TB, and CMV.

What are the s/s of the final stage of HIV Infection? * Fever, night sweats, swollen lymph nodes, and symptoms specific to the infection

HIV Medical care primarily centers on minimizing infection.

What treatment is available for the pt with HIV? AZT

Nucleoside reverse transcriptase inhibitor-interferes and slows the replication of HIV

Crixivan Protease inhibitor-blocks the protease enzyme so that it can’t make any more HIV proteins

Viramune Non-nucleoside reverse transcriptase inhibitor-interferes with replication inside CD4/T cells

Other medical tx for HIV/AIDs pt: * Balanced diet * Avoid crowded areas * Regular exercise * Avoid illicit drug use Regular dental check-ups
What is another name for red blood cells? Erythrocytes

RBC’s total number of red blood cells found in a cubic mm (mm3) of blood. Norm Men-4.2-5.4 million cells/mm3 Women- 3.6-5.0 million cells/mm3

Hemoglobin (Hgb) indicates the oxygen-carrying capacity of the blood Norm: Men-14.0-17.4 g/dl Women-12.0-16.0 g/dl

Hematocrit (Hct) is the percentage of red blood cells in whole blood Norm: Men-42%-52% Women-36%-48%

What is the purpose of the spleen? * To prevent infection by filtering microorganisms from the blood * Produces lymphocytes * Destroy platelets and old RBC’s

What complications is the individual without a spleen at risk for? * Infections-specifically pneumonia * Can be given the pneumococcal vaccine prior to surgery to build own antibodies * Should be vaccinated in the future

Define ecchymosis * Large areas of bruising or hemorrhaging under the skin

What causes clubbing of the fingers? Long term hypoxia and anemia

universal donors? Donors: O negative

Universal recipients? * Recipients: AB positive

What IV solution is used when infusing blood? Why? * 0.9% saline-can be used to dilute blood if necessary * Any other type of fluid causes clotting
Describe the procedure necessary for hanging blood: * Start IV with an 18 or 20g cath only * 2 nurses at the bedside verify blood with patient ID * Baseline VS * VS q 15 min x4 per protocol * Hang with NS How long can blood hang? 4 hours

* Hemolytic * Chills, fever, nausea, dyspnea, chest pain, back pain, hypotension

Anaphylactic Itching, swelling, SOB, hypotension

Febrile * Chills, fever
* Circulatory overload * Cough, frothy sputum, .les, dyspnea, cyanosis, hypotension

transfusion reactions * First nursing intervention * STOP the infusion, keep line open ! * Call the Dr. immediately

Aplastic Anemia * Cause * complete failure of bone marrow * pt’s have extremely low red and white blood cells * Treatment * Epoeiten alfa

Autoimmune hemolytic anemia * bone marrow makes enough RBC’s, but they are destroyed as they are released. * Causes drug reaction certain cancers in newborn- Rh- mother, Rh+ baby * Treatment identify and treat the cause

Iron deficiency anemia Causes a. diet low in iron b. body not absorbing enough iron Treatment a. Iron tablets b. Foods rich in iron

Pernicious anemia -Causes a. unable to absorb Vit B12 due to lack of intrinsic factor produced by the parietal cells of the stomach b. cancer c. gastrectomy

Pernicious anemia - s/s a. Fatigue b. Pallor c. Weakness d. Beefy red tongue

Pernicious anemia - Diagnosis tests a. Schillings test-radioactive Vit B12 used to asses GI absorption of Vit B12

Pernicious anemia - treatment Vit B12 injections-monthly b. Monitor for stomach cancer as this dx carries high risk

Sickle cell anemia * normal number of RBC’s but they’re sickle shaped

* Polycythemia vera too many RBC’s

Define anemia * Decreased circulating RBC’s

Which foods are rich in iron? * Liver, red meats * Raisins * Dark, red fruits and juices such as cherry or cranberry * dark green, leafy veggies * iron enriched cereals

iron tablets side effects expected * C/O black stools * Constipation * Mild nausea

* Chronic leukemia * WBC increases over months or years * usually in adults * Each of the chronic types also have an acute form * s/s for final stage: fever and abnormal bleeding

Types of Acute Leukemias: * Acute Lymphocytic Leukemia (ALL) –occurs most often in kids * Acute Myelogenous Leukemia

(AML) – usually occurs in adults * WBC’s skyrocket over days, crowding out RBC’s and plts

What are the s/s of acute leukemia? * infection and fever, with night sweats * s/s of decreased RBC’s-fatigue, paleness, tachycardia * decreased platelets-petechiae or purpura, epistaxis, gingival bleeding, melena, meorrhagia

Describe the nursing care that would be important for a pt with leukemia? * prevent infection * if pt has thrombocytopenia-avoid needle sticks * treat N/V with meds: Zofran, assess for dehydration, avoid foods pt doesn’t like, no fluids with meals * anorexia-small frequent meals, frequent oral hygiene, monitor weight

What are some of the SE or complications of chemo? * neutropenia-decreased WBC’s * stomatitis-mouth infection * bone marrow suppression-decreased red and white blood cells causes anemia and inability to fight infection

* idiopathic thrombocytopenia purpura * autoimmune disorder * IgG mistakenly attacks plts. Meds can cause ITP * Pt’s with HIV at risk

thrombolytic thrombocytopenia purpura * exaggerated response of the body to vessel injury which leads to extensive clot formation

What is DIC? * Disseminated intravascular coagulation * hypercoagulable state where you have an over stimulation of the coag cascade which leads to simultaneous thrombosis and hemorrhage

DIC: treat primary illness or cause * Early treatment-Heparin to anticoags blood-stops the system from using up all of the clotting factors * Can give Vit K to prevent the lysis of fibrin and promote clotting

What is Hemophilia ? * Genetic disease * Carried on X chromosome as a recessive trait, passed from mothers to sons * person lacks clotting factors * A- missing factor VIII * Most common * B- missing factor IX

What are the s/s of hemophilia? * uncontrollable bleeding – either by trauma or spontaneous bleeding * severe pain in joints due to bleeding and swelling * can bleed to death

Describe the medical treatment necessary for Hemophilia * no cure * Treatment: replace missing factors * A-factor VIII found in FFP and cryoprecipitate * B-factor IX found in FFP * PRBC’s to replace blood loss * IV morphine for pain relief

What other immune disorders can affect patients? * Systemic Lupus Erythematosus * autoimmune disorder in which the body loses the ability to recognize itself * mounts an immune response against itself and causes damage to organs * most common in women, 3X more common in African Americans

What are S/S of SLE? * periods of remission alternating with symptom exacerbation * fatigue and malaise * anorexia and nausea * Fever * weight loss * musculoskeletal symptoms, joint pain * classic rash across bridge of nose and cheeks, called butterfly rash

How is SLE diagnosed? * positive ANA (antinuclear antibody)-makes dx more likely * primarily dx is based on s/s must have 4 of the following-classic rash, photosensitivity, oral ulcers, arthritis, organ involvement, hematologic disorder, immune system disorder

How is SLE treated? no cure b. prevent organ damage c. maintain quality of life d. take anti-inflammatory drugs e. antimalarials and corticosteroids f. Cytotoxic agents if other meds ineffective, or organ damage occurs Hodgkin’s Lymphoma * cancer of the lymph system characterized by Reed-Sterling cells in the lymph nodes * lymph nodes enlarge in chest and neck-may cause stridor * treatment consists of radiation and chemo * bone marrow or stem cell transplant * survival rate 81%

Non-Hodgkin’s Lymphoma * cancer of the lymph system * treatment consists of radiation and chemo-possibly treat bone marrow or peripheral stem cell transplant * overall survival rate 51%

Multiple Myeloma * cancer of plasma cells in the bone marrow * abnormally high levels immunoglobulin * bone pain from fx or bone destruction which causes hypercalcemia, hyperuricemia and anemia * drink plenty of fluids * twice as common in African Americans

What is transplant rejection & how is it prevented? * treated with Cyclosporine or Prograf * must take for the rest of their life * depresses risk of rejection, but also depresses the pt’s immune system increasing risk of infx. * rationale for tissue matching, donor to recipient

Primary functions of the hematologic system: * Oxygenation * Hemostasis * Control of bleeding

Structures & components of the hematologic system: * Bone marrow * Spongy center produces RBC’s & platelets *

Becomes less productive with aging * Liver * Manufactures clotting factors * Clears old or damaged RBC’s * Spleen * Also removes old RBC’s from circulation

RBC’s AKA Erythrocytes * Life cycle of RBC: 120 days * 45% of blood

Platelets AKA Thrombocytes * Life span: 7.5- 10 days * less than 1% of blood

Clotting Factors * Numbered I – XIII * Includes fibrinogen (factor I) * Thrombin (factor II)

Plasma * Clear, straw-colored fluid carries blood cells, etc * Primarily water. Also contains proteins, albumin & globulins * makes 55%of blood

* Upon injury to blood vessel: * The blood vessel constricts * Platelets adhere to it * Coagulation cascade is initiated * Forms scab Bone Marrow Biopsy * Evaluates production of blood cells & plts * Obtain consent * Posterior iliac crest site of choice,

using Jamshidi needle * Usually requires local + conscious sedation & monitoring * Pressure dressing applied X 2hrs
Platelets - lab values 150,000 - 450,000

RBC - lab values 4-6 million

WBC - lab values 5000 - 10000

T - Lymphocytes - lab values 60% - 80%

B - Lymphocytes - lab values 10% - 20% Risk for injury RT Anemia * O2 as Rx * Blood products * Meds: hematopoietic growth factor erythropoietin (epogen) * Alternate rest & activity * Elevate HOB * Keep warm * Teaching

* PRBCs * Adm for symptomatic low H&H * 250-300 ml/unit over 2-4 hours per unit * 4 hrs max

* Platelets * Adm as quick as tolerated for bleeding due to low plts * 60-80 ml/pack, usually 4-6 packs pooled

* FFP * Adm for clotting deficiencies, hemophilia, Coumadin reversal, & w/massive RBC transfusions * 180-270 ml/unit adm in < 4 hours * Cryoprecipitate (factors I & VIII) * Adm for hemophilia A, DIC * 10-15 ml/bag; 10 bags pooled, adm in < 4 hours

Adm PRBC transfusion * Start 18-20G IV of NS; use approp tubing with filter * Start within 30 mins from blood bank * Take baseline VS; stay w/pt X 15 mins

Platelet transfusions * Interventions same as PRBC transfusions no filters * May use 24G IV
* Scandinavians Higher risk of pernicious anemia

Immunologic Disorders Recognizes, isolates & destroys pathogens

* Lymph, lymphatics, & lymph nodes * Collects left over plasma & returns it to venous system * WBC’s also travel thru lymph system * Filters out microorganisms

Cytokines hormones secreted by cells to signal other cells

Leukocytes - * 5 major types: * neutrophils, lymphocytes, eosinophils, basophils, & monocytes * ID & destroy foreign antigens by ingesting them * This process also destroys the WBC * Pus formed if used old WBC’s build up faster than macrophages can get rid of them

* Neutrophils * Fight bacteria; most numerous: 60% of WBC’s

Monocytes * Circulate in bloodstream X1day, then enter tissue * Called macrophages once in tissue, can live months/years

* Eosinophils * Combat parasites; aid in allergic & inflammatory response

Basophils * Release histamine, a vasodilator, when IgE ID’s foreign antigen; this brings large # of WBC’s to area for combat
Lymphocytes * B cells produce plasma cells (antibody factories: IgM, IgG, IgA, IgE) * T cells: T helper & T cytotoxic; can directly destroy invaders

Innate immunity * Present at birth & operational at all times Includes: anatomic/physiological barriers, inflammation & phagocytosis

* Acquired immunity * Active only when needed; attacks specifically * Includes: antibody-mediated immunity, cell-mediated immunity

Tolerance * Immune system recognizes itself

Goldenseal Must be avoided by organ transplant recipients

Nursing Interventions for the Immune compromised pt: Private room Teach everyone (pt & visitors) to wash hands * Avoid invasive techs; remove tubes ASAP; use ASEPTIC tech; use designated stethoscope & thermometer * Diet: NO fresh fruit or veggies,milk products NO live plants, NO humidifiers in room

Apheresis done to collect pt’s stem cells, then these are given back to pt post chemo or radiation

* Bone marrow * Spongy center produces RBC’s & platelets * Becomes less productive with aging

Liver Manufactures clotting factors Clears old or damaged RBC’s

Spleen Also removes old RBC’s from circulation

Name 3 causes of anemia -Blood loss-impaired production of erythocytes-increased destruction of erythrocytes cardiopulmonary manifestations of anemia SOB - increased heart rate - increased pulse - tachypnea (fast breathing)

What drugs would you take when taking iron STOOL SOFTENERS - colace or docusate sodium

Foods high in iron......name em' Popeye! spinich, liver, muscle meat, whole grains, eggs

What are the four phases of normal hemaostasis? Vascular phase, platelet phase, coagulation phase and fibrinolytic phase

What is the best indicator of normal hemostasis? Hx of normal clotting after a recent challenge due to trauma or natural (better than any test)

What effect does nitrous oxide have on vessels? Keeps them open

What does Von Willibrans factor do? Prothrombotic when exposed to blood - Causes thromboxane A2 to be released which causes vasoconstriction

What is primary hemostasis? The formation of a platelet plug

What do fibrin and fibrinogen do? Fibrin stabilizes platelets, Fibrinogen makes the clot inactive (not sticky)

What are some causes of vessel defects? Vit C dificiency (Scurvy - causes defective collagen), Bacterial, fungal and viral

What is the difference between thrombocytopenia and thrombocytopathy? Thrombocytopenia - not enough platelets. Thrombocytopathy - poor quality platelets.

What drugs can cause thrombocytopenia? Drug induced (Alcholol, thiazide diauretics), bone marrow failure, chemo/rad tx, Aplastic anemia, nutritional deficiencies and hypersplenism (CLL)

How does hypersplenism affect clotting? Increased size of speeen leads to platelet trapping

Bone tumors occur more commonly in ___, with the highest incidence during the ____ rate of adolescence? Occur more commonly in males, with the highest incidence during the accelerated growth rate of adolescence

What are the clinical manifestations of bone tumors? Clinical manifestations are similar: Localized pain and swelling at tumor site, palpable mass, Limp or decreased ROM

Bone Tumors it is important to rule out __ or ___ as causes? Rule out trauma or infection

What are the definitive diagnosis of Bone tumors? Definitive diagnosis based on radiologic studies ( CT scans, bone scans) and bone biopsy

Blood Lab Values

Red Blood Cells - Men 4.2-6.0 x 10uL

Red Blood Cells - Women 3.6-5.0 x 10uL
Hematocrit - Men 40-50% Hematocrit - Women 40-47%

Platelets 150,000-400,000

White Blood Cells 5,000-10,000

Hemoglobin - Men 14.0-17.0 g/dL

Hemoglobin - Women 12.0-16.0 g/dL

Bleeding Time 1-9sec
Partial thromboplastin time (Ptt) 20-45sec

Prothrombin Time (Pt) 9.5-12.0sec

Sedimentation Rate (ESR) - Men 0-15mm/h

Sedimentation Rate (ESR) - Women 0-20 mm/h

Optimal Total Cholesterol