nursing exam

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PATIENTS WITH

HEMATOLOGIC DISORDERS (PART 2)

TA M M Y BROW N M SN, RN

UN IV ERSITY O F M O BILE

A D ULT H EA LTH II

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

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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)

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

ACQUIRED COAGULATION

DISORDERS

LIVER DISEASE

§ CIRRHOSIS

§ HEPATITIS

§ ADMNISTER FRESH FROZEN PLASMA (FFP)

VITAMIN K DEFICIENCY § MALNOURISHMENT

§ ALCHOLISM

§ DIETARY DEFICIENCY

§ ADMINISTER VITAMIN K (MEPHYTOM) SUBQ OR ORALLY

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vA unit of FFP is usually administered over 15 - 30 minutes

vA standard blood administration set is used

vOnce thawed, FFP must not be re-frozen and should be used immediately

vIf delay is unavoidable, the component should be stored at ambient temperature (about 73° F) and used within 4 hours.

vThere is a rare risk of anaphylactic reaction with IM / IV Vitamin K

vOral doses work better with prolonged bleeding time with Coumadin administration (INR)

vTakes 6-24 hours to reach full effect

DISSEMINATED INTRAVASCULAR COAGULATION (DIC)

Triggered by an underlying condition:

Sepsis

Traum a / Shock

Mortality rate is >80%

Early recognition is essential for improved outcomes

Precipitating factor MUST be successfully treated

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§ Clotting is altered, causing sm all blood clots to form in the blood vessels, som e of which can clog the vessels and cut off the norm al blood supply to organs such as the liver, brain, or kidneys

§ Lack of blood flow can dam age and cause m ajor injury to the organs

§ During the clotting process, platelets & clotting factors are consum ed, leaving the patient at high risk of serious bleeding, even from a m inor injury or without injury

§ Bleeding m ay also start spontaneously

§ DIC can also cause your healthy red blood cells to fragm ent and break up when they travel through the sm all vessels that are filled with clots.

Lab Changes in a patient with DIC Platelets Decreased

PT Increased PTT Increased Fibrinogen Decreased D-Dimer Increased

MANIFESTATIONS

§ Initially there m ay be no physical sym ptom s / only subtle changes in the platelet count

§ As m icro-clots occur: § Organ involvem ent

§ Kidney failure / Liver failure § Stroke-like sym ptom s § Sym ptom s of Pulm onary Em bolus

§ As clotting factors / platelets are depleted: § Petechiae § Oral bleeding / epistaxis / conjunctival

hem m orhage

§ Increased bleeding from puncture sites (IV, drainage tubes, etc.)

§ Hem atem esis / Hem aturia / M elena § Profuse bleeding from all orifices § Internal bleeding (increased abdom inal girth)

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TREATMENT

§ PRIMARY

§ Treat the underlying cause

§ SECONDARY § Treat the symptoms

§ Oxygen / IV fluids / Electrolytes

§ Transfusions (PRBCs, FFP, and platelets)

§ Controversial Treatments

§ Heparin or Lovenox to disrupt the formation of micro-clots

NURSING MANAGEMENT

§ Monitor those at risk (Sepsis / Trauma)

§ Early recognition and reporting is essential

§ Monitor for s/s clots

§ Monitor lab values

§ Monitor for bleeding

§ Administer O2 / IV fluids / electrolytes

§ Most patients will have a central line / monitor closely

§ If kidney failure occurs, dialysis may be necessary

§ Transfuse FFP & platelets

§ Monitor dialysis catheter for bleeding

§ Monitor liver function tests

§ Monitor neurological function

ESSENTIAL THROMBOCYTHEMIA

§Occurs most often in women > 50yo

§Caused by an excessive production of platelets that leads to abnormal clotting or bleeding

§Occurs as a result of acquired gene mutation or infections

§Average survival rate post- diagnosis is 20 years

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TREATMENT

§ GOALS: § REDUCE THE RISK OF OCCLUSIONS RELATED TO

CLOTS (THROMBOTIC RISKS) § ALLEVIATE SYMPTOMS

§ Assess for factors that make patient high risk for complications: § History of clots § PVD

§ Atherosclerosis § Obesity § History of smoking

§ High risk: § Hydroxyurea § IV / SubQ coagulation

§ Extreme Cases § Plateletpheresis

APLASTIC ANEMIA

§ Results from damage to the stem cells within the bone marrow

§ Leads to pancytopenia (decrease in RBCs, WBCs, & platelets)

§ Most cases are idiopathic (no known cause) § Viral infection § Pregnancy § Some medications § Chemical exposures § Chemotherapy

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MANIFESTATIONS

§ Often insidious (come on slowly and progress gradually)

§ Fatigue

§ Shortness of breath with exertion

§ Rapid or irregular heart rate

§ Pale skin

§ Frequent or prolonged infections

§ Unexplained or easy bruising

§ Nosebleeds and bleeding gums

§ Prolonged bleeding from cuts

§ Skin rash

§ Dizziness

§ Headache

§ Neutropenia / Thrombocytopenia / Anemia

TREATMENT

§PRBCS (symptomatic anemia)

§Platelet transfusion (thrombocytopenia) §Antibiotics (neutropenia)

§<40yo with a matched sibling donor §Stem cell transplant (curative)

§>40yo or no matched sibling donor §Immunosuppressive therapy (IST)

§If IST is not successful, unmatched/unrelated stem cell donor should be considered

§High mortality rate from rejection

NURSING MANAGEMENT

§ Infection prevention

§ M onitor for s/s bleeding

§ Avoid ASA and aspirin-containing products

§ Birth control for wom en to dim inish loss of blood during

m enstrual cycle

§ Educate on IST

§ Close m onitoring of blood pressure

§ Educate on hirsutism and gingival hyperplasia (body im age)

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LEUKEMIA

§ A rapid increase in the number of white blood cells (proliferation) with abnormal maturation of these cells; results in suppression of other cells

§ Bone marrow begins to over-fill with white blood cells, leading them to overflow into the circulatory system prematurely

§ Increase in WBCs (leukocytes) = leukocytosis

§ Usually only involves 1 type of WBC § Neutrophils – Myeloblast § Lymphocytes - Lymphoblast

ACUTE VS

CHRONIC LEUKEMIA

§ Acute § Immature leukocytes that do not function

normally

§ Onset is rapid § Symptoms progresses quickly

§ Death can occur within weeks to months without aggressive treatment

§ Chronic § Most leukocytes are mature and can still

function normally § Symptoms take months to years to progress § Patients live longer and often die of

secondary conditions in late age

A M L (AC UTE

M Y E LO ID LE UK E M IA )

A LL (AC UTE

LY M P H O C Y TIC LE UK E M IA )

C M L (C H RO N IC M Y E LO ID

LE UK E M IA )

C LL (C H RO N IC

LY M P H O C Y TIC LE UK E M IA )

A ffects all age groups C om m on in young

children

C om m on adults > 60 C om m on in adults > 60

G row s quickly G row s quickly G row s slow ly G row s slow ly

Prognosis is directly

related to age – M ost lethal of the leukem ias

90% survival rate in

children < 5 / 60% in adolescents

80% 5 year survival rate w ith early

detection; unless disease progresses to “blast crisis” phase (3-6 m onths)

90% 5 year survival rate

D evelops w ithout

war ning – sym ptom s appear over weeks to

m onths

Sym ptom s com e on

quickly but expected outcom e w ith

treatm ent is com plete rem ission

Sym ptom s progress slow ly and in

phases – early detection is m ost com m on and leads to high survival

rates

Sym ptom s progress

slow ly – often req uires m onitoring w ith no

treatm ent

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CML

• Mostly asymptomatic • Incidental finding in blood work

(increased WBCs)

Chronic phase

• Fatigue, fever, night sweats, abdominal pain, weight loss

• Increased WBSs / Low platelets

Accelerated phase

• Bone pain, enlarged spleen, bleeding, infections

• Confusion, vision changes, SOB • Very high WBCs / very low platelets

Blast crisis

AML

NEUTROPENIA Fever / infection

ANEMIA Weakness / fatigue

THROMBOCYTOPENIA Bleeding

Enlarged liver / spleen with pain / bone pain

Anorexia / nausea / vomiting / diarrhea

ALL

PAIN FROM ENLARGED

LIVER / SPLEEN

BONE PAIN

TESTICULAR SWELLING /

PAIN

VISUAL CHANGES

NAUSEA / VOMITING

ALTERED LOC

CONFUSION

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NURSING MANAGEMENT

CML

Focuses on medications (TKI) • Take before meals • Do not take with antacids,

PPIs (protonix), grapefruit juice

AML

Administer blood products as ordered

Administer antibiotics Infection / bleeding

prevention is priority!

Educate to for self- management of disease and to decrease anxiety

Monitor for increased potassium and phosphates

Monitor uric acid levels

Increased fluid intake

ALL

Educate men on testicular exams

Prevent infection / bleeding Discuss fertility preservation with young girls

**With all, monitory and treat side effects of chemotherapeutic treatment • Nausea • Mucositis

LYMPHOMAS

HODGKINS

§ RARE

§ HIGH CURE RATE

§ M ORE COM M ON IN YOUNG M EN

NON-HODGKINS

§ > 70,000 CASES ANNUALLY

§ RATE OF CURE IS LOW ER THAN W ITH HODGKINS / DIAGNOSED AT A M ORE

ADVANCED STAGE

§ M ORE COM M ON IN OLDER M EN

NURSING MANAGMENT

§ TREATMENT

§ CHEMOTHERAPY

§ RADIATION

§ MONITOR AND TREAT

§ NAUSEA

§ HAIR LOSS

§ INFECTION

§ WEIGHT LOSS / ANOREXIA

§ ORAL ULCERS

§ BE AWARE OF TUMOR LOCATION

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BLOOD TRANSFUSIONS

§ Each unit should be given over 2-4 hours unless it is an emergency

§ Should be started within 30 minutes after picking up from blood bank / run over no longer than 4 hours

§ Checks (name, DOB, medical record #, Unit #, blood type, expiration date) § Blood bank § Patient bedside with second RN

§ Vital signs generally are taken prior to starting blood, 15 minutes after starting blood, and at completion of blood

§ Monitor for transfusion reaction

§ back pain / flank pain § dark urine. § Chills / fever / skin flushing § fainting or dizziness.

§ shortness of breath / restlessness § Itching

ALTERNATIVES TO BLOOD FOR RELIGIOUS OR OTHER REASONS

§ VOLUME EXPANDERS § NORMAL SALINE

§ ERYTHROPOEITIN § STIMULATES PRODUCTION

OF RBCS

§ BLOOD SALVAGE DURING SURGERY

§ AUTOLOGUS DONATION (WHEN POSSIBLE)

§ ARTIFICIAL BLOOD § REMAINS EXPERIMENTAL

TYPESOFDONATION

• Friends and family donate

Directed donation

• Blood is received from donors at community / school / occupational blood drives

Standard donation

• Patient donates their own blood

Autologous

• Collected in cell-saver and re-transfused to patient

Intraoperative blood salvage

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§ INFECTION

§ HEPATITIS B OR C

§ HIV

§ CMV

§ IRON OVERLOAD § TREAT WITH CHELATION THERAPY

§ TRANSFUSION REACTION § ALLERGIC-TYPE REACTIONS

§ SENSITIZATION

§ INCORRECT BLOOD TYPE AND/OR RH FACTOR

§ RH – (RH – ONLY) § RH + (RH – OR +)

§ EMERGENCY OR UNKNOWN BLOOD TYPE (O)

COMPLICATIONS OF

TRANSFUSIONS