nursing exam

5/19/19

1

PATIENTS WITH

HEMATOLOGIC DISORDERS TA M M Y BROW N M SN, RN

UN IV ERSITY O F M O BILE

A D ULT H EA LTH II

ANEMIA

§ a condition that develops when the blood lacks enough healthy red blood cells, resulting in a lack of hemoglobin

§ Hemoglobin binds oxygen

§ Lack of oxygen delivered to body tissues / organs

§ Hemoglobin:

§ Females: 12-16 g/dL

§ Males: 14-18 g/dL

CLASSIFICATIONS

A N

E M

IA

HYPOPROLIFERATIVE

PERNICIOUS / VITAMIN B12 DEFICIENCY

FOLATE DEFICIENCY

IRON DEFICIENCY

CHRONIC DISEASE

APLASTIC

BLOOD LOSS ACUTE

CHRONIC

HEMOLYSIS

AUTOIMMUNE

THALASSEMIA

SICKLE CELL

5/19/19

2

GENERAL MANIFESTATIONS

§ DEPENDENT UPON:

§ SEVERITY

§ HOW QUICKLY IT DEVELOPS

§ HOW LONG IT LASTS

§ COMORBIDITIES

§ DAILY METABOLIC DEMANDS

VITAMIN B12

DEFICIENCY

§ Results from : § Inadequate dietary intake of B12 § M alabsorption of B12 in the GI tract secondary to

disease state and/or surgery § Lack of protein (intrinsic factor) that binds B12 so

that it can be absorbed through the intestinal tract

§ Pernicious anem ia

§ M anifestations § Fatigue / weakness

§ Jaundice § Sore, red tongue § NEUROLOGICAL SYMPTOMS

§ Treatm ent § Increase in dietary B12

§ B12 injections or nasal spray § B12 supplements § Folic acid supplements

FOLATE DEFICIENCY

ANEMIA

§ Results from : § Deficient dietary intake, especially raw

vegetables

§ Alcoholism § Pregnancy § Sm all bowel diseases (Celiac Sprue – sensitivity to

gluten)

§ M anifestations § Fatigue / weakness § M ood changes § Lacks severity of neurological sym ptom s seen

with B12 deficiencies

§ Treatm ent § Increased dietary intake of folic acid § Folic acid supplem ents

5/19/19

3

WHAT TYPE OF NURSING CARE IS IMPORTANT FOR

PATIENTS WITH B12 DEFICIENCIES?

IRON DEFICIENCY

§ Results from :

§ Insufficient dietary intake

§ Blood loss (**m ost com m on**)

§ Pregnancy / Childbirth

§ Alcoholism

§ M anifestations

§ Extrem e fatigue / weakness

§ Pallor / Tachycardia / dyspnea

§ Pica

§ Treatm ent

MO ST

COM MO

N A NEM

IA

WHAT TYPE OF NURSING CARE IS IMPORTANT FOR

PATIENTS WITH IRON DEFICIENCY ANEMIA?

5/19/19

4

CHRONIC DISEASES

§ Most common diseases:

§ Autoimmune diseases (Rheumatoid, Lupus)

§ Inflammatory bowel diseases (Chron’s, Ulcerative Colitis)

§ Chronic Infections (HIV / AIDS)

§ Chronic Kidney Disease

§ Manifestations

§ Mild – Moderate

§ Progress slowly § Stabilize quickly

§ Treatment

§ Treat the underlying disorder

BLOOD LOSS

§ ACUTE

§ Associated with acute GI bleeding, trauma, or surgery

§ Menstrual cycle

§ CHRONIC

§ Chronic GI bleeding

§ Manifestations

§ Hemoglobin may be normal at first, but levels will decrease as blood loss continues

§ Acute

§ Hypotension, dizziness, tachycardia, tachypnea

§ Chronic

§ Fatigue, pallor, dyspnea

§ Treatment

§ Blood Transfusion

§ Alternative treatments for religious considerations

THALASSEMIA

Hereditary Anemia

• Mediterranean, African, Southeast Asian population

• Thalassemia Major: both parents

• Thalassemia Minor: 1 parent (carrier)

Characteristics

• Too few and/or abnormal hemoglobin

• Smaller-than- normal RBCs

• Destruction of RBCs; marrow releases new RBCs too early, making them unable to function appropriately

Manifestations

• Alpha: mild form with few to no symptoms

• Beta: pallor, fatigue, poor appetite, splenomegaly, dark urine, jaundice, skeletal abnormalities

Life Expectancy

• 30 years on average (heart problems / iron overload)

Treatment

• Stem cell transplant

• Lifelong blood transfusion

• Chelation therapy for iron overload

5/19/19

5

SICKLE CELL

ANEMIA

§ Inherited disorder that causes RBCs to become misshapen (sickled) and break down § Become rigid and sticky § Obstruct vessels, limiting O2 to parts of the body

§ Most commonly seen in African-Americans and Hispanics

§ Manifestations § Symptoms of anemia § Sudden severe episodes of pain (crises)

§ Swelling of the hands and feet § Frequent infections § Vision problems

§ Complications § Stroke, blindness, priapism, blindness

§ Goals of Treatment: § Avoid Crisis § Relieve Symptoms § Prevent Complications

§ Treatment § Hydroxyurea § Vaccinations

§ Antibiotics § Pain medications

§ Blood transfusions § Daily folic acid

GENERAL NURSING

MANAGEMENT

§ FATIGUE § Frequent, short-periods of exercise § Frequent rest periods § Prioritize activities by importance and necessity

§ IM BALANCED NUTRITION § Balanced, healthy diet § Alcohol limitation / cessation § Individualize dietary plans based on food preferences,

religious / cultural concerns § Dietary supplements

§ PERFUSION § Supplemental O2 § IV fluids § Blood Transfusions § Monitor BP and hold anti-hypertensives when

necessary

5/19/19

6

THROMBOCYTOPENIA

§ LOW PLATELET LEVEL

§ Normal: 150,000 – 400,000

§ RESULT OF: § Decreased production

§ Increased destruction

§ Increased consumption

§ CAUSES:

§ Malignancy

§ Infection

§ Medications

§ Autoimmune

§ Disseminated Intravascular Coagulation (DIC)

• Treat the underlying causeINFECTION

• Stop the offensive medications if possible • If from chemotherapy, platelet transfusions

may be necessary Medications

• Penicillin • Cephalosporins • Vancomycin • Sulfa Drugs • Digoxin • Valproate • Aspirin • Zantac • Pepcid

Most common medications

causing thrombocytopenia

IM M

U N

E TH

RO M

BO CY

TO PE

N IA

PU RP

U RA

(I TP

)

§ Most commonly found in children and young women § Body produces antibodies against its own platelets

§ The antibodies attach to the platelets § The body destroys the platelets that carry the antibodies

§ Symptoms § Platelet count as low as 5,000 § Easy or excessive bruising

§ Petechiae (extremities and trunk) § Bleeding from gums or nose

§ Hematuria / Melena § Unusually heavy menstrual flow

§ Symptoms may begin vaguely, leading to an incidental finding of low platelet count

5/19/19

7

TREATMENT

§ Monitor for platelet counts 30,000 – 50,000

§ 1st Line:

§ < 30,000 or with severe bleeding

§ Begin oral prednisone (ITP is the result of an abnormally acting immune system / steroids suppress

the immune system)

§ < 5,000 and/or internal bleeding

§ Pulse corticosteroids (large doses given in intermittent infusions)

§ IV immunoglobulin (IVIG) (overwhelms the spleen with

antibody so that it cannot recognize the antibody- coated platelets)

§ 2nd Line:

§ Splenectomy (main site of platelet destruction)

§ Rituximab (kills the cells that produce the platelet destroying antibodies)

SPLENECTOMY

§ Patient is at risk for: § Infection § Death related to sepsis (50% of the cases)

§ If fever occurs: § Im m ediately adm inister antibiotics

§ Prior to surgery: § Vaccinations

§ Education: § Instruct patient to call provider at the first

sign of sickness

§ Tem p > 100.5 § Sore throat § Cough / cold that lasts longer than

norm al § Chills that do not go away § M alaise

WHAT IS THE APPROPRIATE NURSING MANAGEMENT FOR PATIENTS WITH ITP?

5/19/19

8

PLATELET DYSFUNCTION

§ PLATELET NUMBERS ARE WNL BUT FUNCTION IS ALTERED

§ MEDICATIONS (PREVENT PLATELETS FROM CLUMPING TOGETHER AND FORMING CLOTS)

§ ASPIRIN (LASTS 7-10 DAYS)

§ NSAIDS (LASTS 5-7 DAYS)

§ PLAVIX (2-3 DAYS)

§ BLEEDING MAY RANGE MILD – SEVERE

§ TRAUMA § DENTAL PROCEDURES

§ ECCHYMOSIS (BRUISING)

VON WILLEBRAND

DISEASE (“Free Bleeder”

§ a genetic disorder caused by m issing or defective von W illebrand factor, a clotting protein.

§ VW F binds factor VIII, a key clotting protein, and platelets in blood vessel walls, which help form a

platelet plug during the clotting process at the site of

vascular injury

§ Type 1

§ Sym ptom s are m ild

§ Type 2

§ Sym ptom s are m ild to m oderate

§ Type 3

§ Sym ptom s are severe

§ Spontaneous bleeding often occurs within joints and m uscles

ü Manifestations: ü Severe bleeding following

dental procedures, surgery, childbirth

ü Unusually heavy menses ü Large bruises that easily

appear ü nosebleeds

ü Platelets WNL ü PTT WNL ü INR WNL ü Bleeding Time Prolonged (> 10

minutes)

5/19/19

9

TREATMENT

§ Desmopressin

§ Injection or Nasal Spray

§ Stimulates release of stored VWF

§ Contraindicated with CAD (may cause MI)

§ May cause hyponatremia from fluid

retention

§ Fluid restriction 24 hours after dosing

§ Replacement Therapies

§ infusions of prepared doses of concentrated blood-clotting factors

containing VWF and factor VIII