Hemotologic Disorder s

1. Hemotologic Disorders By Diana Blum RN MSN Metropolitan Community College

2. Functions • Oxygenation • Hemostasis (control of bleeding) • If injury the vessel constricts • Platelets adheres to injured vessel • Them the coagulation cascade begins • Diagnosis of problems can be difficult

3. Anatomy • Bone Marrow: • Liver: manufactures clotting factors • Spleen: removes old RBCs from circulation • Blood: transports oxygen from lungs. • Maintains hemostasis • About 6 liters in body

4. Parts of the Blood • RBC: made in bone marrow • Transfers oxygen to from lungs to tissues • Hemoglobin makes the transfer of oxygen and carbon dioxide possible • 120 day life span • Iron and heme are recycled • Have proteins called antigens • 2 major parts are A and B and O • Rh + and Rh- • PLATELETS: made in bone marrow • Numbered I to XIII • Form stable fiber matrix over wound

5. Parts of the Blood • Plasma: clear straw colored fluid that carries red blood cells, platelets, and clotting factors • Primarily water • Other components are plasma proteins, albumin, and globulins

6. Age Related Changes • The bone marrow becomes less productive • Hemotologic function is not affected unless trauma, a chronic illness, or treatment for cancer

7. Nursing Assessment • Health Hx: Chief complaint, Hx of present illness • Past Medical Hx: Ca, HIV, liver dx, kidney dx, malabsorption dx, transfusions, clots • Family Hx: hemophilia, sickle cell dx • Review System: ask about change in color, skin dryness, pruritus(itching), vertigo, confusion, pain, headaches (duration, location, intensity), mental status changes, bleeding, heart palpitations • Functional assessment: occupation, hobbies, self concept, activity, exercise, sleep and rest, nutrition, relationships, stress, health perception See page 607

8. Physical Exam • Vs • Ht • Wt • Look for cracking of mouth • Monitor for SOB • Look for tachycardia, tachypnea, hypotension • Look for orthostatic changes, dehydration • Look for pale skin or jaundice • Look for bruising (ecchymosis) • Look for purpura: larger than petechiae • Can be from low platelet count • From large blood vessels breaking • Look for petechiae • (confused with a rash) • Reddish purple in nature • Severe coughing can cause

10. Blood tests • Blood cell count • Hemoglobin • Hematocrit-approximately 3 times the hemoglobin • Normal platelet ct is • PT and PTT measure bleeding time • Blood typing • To prepare the client tell them that they will feel a small prick

11. Procedures • Bone marrow Bx: measures how it is making blood • Explain the purpose and procedure • Obtain consent • No fasting is necessary • Procedure takes about 30 minutes • Transfusions see page 612-615

12. Blood compatibilities

13. Bleeding precautions • Minimize the # of invasive procedure • Avoid prolonged tourniquet use • Avoid IM injections • Instruct the client to use soft bristled tooth brush • No strait edge razor shaving only use electric razor • Avoid NSAIDS

14. products • PRBCs: 250-300ml/unit infuse over 2-4 hours • Platelets: 80-60ml/pack: usually 4-6 packs are pooled for transfusion infuse as quickly as the pt tolerates • FFP: 180-270ml/unit infuse in less than 4 hours • Cryoprecipitate:10-15ml/bag; usually 10 bags are pooled for transfusion infuse in less than 4 hours (contains factor 1 and 8)

15. Blood typing for transfusion • Universal donor= • Does not contain A, B, or Rh antigens • Universal recipients= • Blood contains A, B, and RH antigens • Usually blood banks exactly match the pt blood

16. adminstration on blood • Pt needs 18 or 20 gauge IV needle so cells are not lysed (destroyed) • Prior to administration, blood needs to be checked by 2 licensed nurses. Check the expiration date, name, medical record number, type of blood, blood band id, pt birthday • Check vitals prior to administration • **blood must be initiated with in 30 minutes of arrival from lab to floor • Use blood tubing for administration • Monitor for blood reactions • Monitor vitals continuously during administration

17. Blood reactions • Hemolytic: fever, chills, nausea, dyspnea, chest pain, back pain, hypotension • Antigen/antibody rx to transfusion • Happens shortly after initiation • Tx: stop the transfusion, call md, supportive therapy to maintain HR and BP • Anaphylactic: urticartia, wheezing, dyspnea, hypotension • Type 1 hypersensitivity rx to plasma proteins • Occurs within 30 minutes of initiation • Tx: stop transfusion, call md, be ready for epi and steroids • Febrile: fever, chills • Recipients antibodies rx to donor leukocyte • Occurs within 30-90minutes of initiation • Tx: stop infusion, call md • Circulatory overload: cough, frothy sputum, cyanosis, decreased BP • cardio system is unable to manage the additional fluid load • Occurs anytime during transfusion and up to several hours after completion • Tx: stop infusion, call for help, be prepared for code, be prepared to administer oxygen and Lasix

18. FFP Transfusion administration • Start IV of NS using at least 24 gauge needle • Platelets are smaller so a smaller needle can be used • Check just like you check blood • Prior to administration check vs • Continue to check vs during administration • Run platelets through blood tubing with a filter • Infuse as fast as the pt can tolerate • Monitor for rx • See page 614

19. Colony Stimulating Factors • Naturally occuring hormones that stimulate bone marrow to produce more blood cells • Ferrous sulfate: iron replacement • Iron dextran: iron replacement • Vitamin B12: Vitamin B12 replacement • Hydroxyurea: prevention of sickle cell crisis • Epogen: stimulates the bone marrow to produce more RBCs • Used frequently with hemodialysis • Used for anemia secondary to HIV or cancer See page 614

20. Polycythemia Vera • Too many RBCs produced • Blood more viscous • s/s: headache, dizziness, ringing in the ears, blurred vision, ruddy complexion • Tx:

21. Aplastic Anemia • Complete failure of the bone marrow • Low RBC count • Low WBC • Low Platelet cts • S/S: pallor, fatigue, tachycardia, sob, hypotension, prolonged/spontaneous bleed, frequent infections • Tx: transfusion of RBC and Platelet, antibiotics, corticosteroids, bone marrow transplant, ICU setting

22. Autoimmune Hemolytic Anemia • Enough RBCs made but they are destroyed once they are released into circulation • Causes: infection, drug reaction, cancer • s/s: pallor, fatigue, tachycardia, sob, hypotension, jaundice, high bilirubin levels • Postive direct coombs antiglobulin test • Tx: blood transfusions, corticosteroids • Recovery in few days to weeks

23. Iron Deficiency Anemia • Low RBC • Low HGB andHCT • Low serum Iron level • Low ferritin level • High TIBC level • Results from diet low in iron • Results from body not absorbing enough iron from GI tract • Not enough hemoglobin made as result • s/s: fatigue, pallor, orthostatic changes (in severe cases) • Tx: iron supplements, iron rich foods

24. Pernicious Anemia • Pt does not absorb vitamin B12 from stomach • Pt may lack intrinsic factor-essential for b12 absorption • Assess hx: gastrectomy, • s/s: weakness, sore tongue, numbness of hand and feet • Tx: B12 injections monthly

25. Sickle cell anemia • RBCs normally disc shaped • In sickle cell they are sickle shaped • Easily rupture • Obstruct blood flow • Genetic in nature • Most common in African Americans • Sickle cell is recessive (inherit from mom and dad)

26. Thrombocytopenia • Too few platelets • Causes: cancer treatment • Too many platelets being destroyed • Causes: idiopathic thrombocytopenic purpura, thrombic thrombocytopenic purpura (chptr 32) • s/s: petechiae, purpura, gingival bleed, epistaxis, prolonged bleeding

27. Hemophilia • Genetic • Lack of blood clotting factor • 1-2 cases per 20,000 persons • Types • A: factor VIII is missing-higher incidence • B: factor IX is missing • Trait is carried on x chromosome • Rare for women to have • s/s: uncontrolled bleed especially in joints, skin, GI tract • Tx: no cure. Transfusions, pain treatment (IV morphine is common)..monitor for addiction to opiods.

28. Nrsg diagnosis • Risk for injury r/t bleed. Goal: cessation of bleeding aeb no visual signs of bleeding and stable vs • Acute pain r/t bleeding into closed spaces(creating pressure on nerves. Goal: pain relief aeb: patient states pain is relieved and appears to be in relaxed manner. • Ineffective therapeutic regimen management r/t lack of knowledge about dx process and self care. Goal: effective management of condition aeb patient accurately describes condition and demonstrates self care measures.

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