Curriculum Update: The Hematopoietic System, Patients with Special Challenges, Interventions For The Patient With Chroni

1. Curriculum Update:TheHematopoietic System,Patients with Special Challenges,Interventions For The Patient With Chronic Care Needs Condell Medical Center EMS System August 2006 Site Code #10-7200-E1206 Program revised by: Sharon Hopkins, RN, BSN EMS Educator

2. Objectives • Upon successful completion of this CE module, the EMS provider should be able to: • discuss insults to the hematopoietic system • discuss the uniqueness when caring for patients with special challenges • review acute interventions necessary for the chronic care patient • discuss medications used in Region X • participate in case scenario review • successfully complete the post quiz with a score of 80% or better

3. Hematology • Definition - the study of blood and blood-forming organs collectively known as the hematopoetic system. • Prehospital care for most patients with hematological disorders is mainly supportive. • Understanding some of the hematological disorders enhances EMS assessment skills

4. Blood Cells • Produced mainly in bone marrow - the spongy material in the center of bone • Assistance to regulation of production, destruction and differentiation (development of a specific function) of cells carried out in the: • lymph nodes • spleen • liver

5. Components of Blood • Plasma • Platelets • thrombocytes • White blood cells • leukocytes • Red blood cells • erythrocytes

6. Components by Volume -WBC’s & platelets together form <5% of formed elements - RBC’s 95% of volume of formed elements

7. Components of Blood • 78% water • 22% solids (plasma, RBC’s, WBC’s, platelets) • Plasma • Clear fluid part of blood; about 92% water • Plasma contains various cellular materials in solution and suspension: • proteins (albumin, globulins, fibrinogen) • salts • metals • inorganic compounds

8. Red Blood Cells (RBC’s) - Erythrocytes • Mainly comprised of water and hemoglobin • Hemoglobin (a protein) - oxygen carrying component of the RBC; gives blood its red color; carries oxygen • Primary function of RBC: • transport oxygen from the lungs to various body tissue • transport carbon dioxide from various tissues to the lungs • Average life expectancy RBC 90-120 days

9. Pulse Oximetry • Estimates the amount of oxygen carried in the bloodstream using infrared technology across the skin

10. White Blood Cells (WBC’s) - Leukocytes • Helps fight infection and aids in the immune process • Helps heal wounds by ingesting matter (dead cells, tissue debris, old RBC’s) • Protects the body from foreign material (antigens) • Involved in the protection from mutated cells (ie: cancer) • Includes: lymphocytes, monocytes, eosinophils, basophils, neutrophils

11. Platelets - Thrombocytes • Small, sticky cells • Helps in blood clotting • Groups together to form clumps to plug holes and leaks to stop the bleeding • WBC’s, RBC’s, and platelets make up about 45% of the blood volume • Plasma takes up about 55% of the blood volume

12. More Blood Components • Fat globules • Chemical substances • carbohydrates • proteins • hormones • Gases including • oxygen • carbon dioxide • nitrogen

13. Complete Blood Count - CBC • A particular and common blood test • Measures the size, number, and maturity of different blood cells in a specific volume of blood • Can be used to determine abnormalities • in production of blood cells • in destruction of blood cells

14. Blood Groups • Surfaces of RBC’s contain glycoproteins and glycolipids • Based on the presence or absence of isoantigens (an antigen substance that can stimulate antibody production), we can classify blood into groups • Most common blood grouping is ABO and Rh • A, B, AB, and O blood types • Person can be Rh+ or Rh-

15. Blood Types • Type AB blood- universal recipient • Can receive any of the 4 types of blood • Carries both A & B antigens but does not have antibodies to A or B blood • Type O blood - universal donor • Can be given to patients regardless of blood type • Has both anti-A & anti-B antibodies but no antigens • Type A blood has A antigens & anti-B antibodies • Can receive type A or O blood only • Type B blood has B antigens & anti-A antibodies • Can receive type B or O blood only

16. Blood groups • Rh blood groups • Discovered in the blood of the rhesus monkey in 1940’s • If the blood has Rh antigen they are Rh+ • If the blood lacks the antigen they are Rh- • Rh+ & Rh- blood is incompatible; the immune system makes antibodies if mixed • When a subsequent infusion of Rh+ blood is received the immune response causes a severe reaction (hemolysis - breakdown of RBC’s and release of hemoglobin)

17. Rh Acquired Factor Deficiencies • Hemolytic disease of the newborn • Arises during pregnancy • Maternal and fetal blood may be incompatible if Rh factor discrepancy is present • Usually the first born is unaffected • Future problems in pregnancy prevented by Rhogam injections given after birth to Rh- mother delivering an Rh+ baby • Rhogam given to Rh- mother binds to any Rh+ antigens left by the fetus and prevent any production of antibodies by the mother that would affect future pregnancies of Rh+ baby

18. Blood Transfusions • Blood is the most easily shared human tissue • Many lives are saved each year • Most often used to alleviate anemia • An antibody-antigen reaction can occur causing the transfused blood to hemolyze or burst • The liberated hemoglobin causes kidney damage from sludging

19. Hemostasis • The sequence of clot formation that stops bleeding • Quick, localized and carefully controlled • Three mechanisms reduce blood loss: • Vascular spasm - local vasoconstriction • Platelet plug formation • Blood clotting (coagulation) • Failure of these mechanisms could result in hypoperfusion and shock

20. Mechanisms of Hemostasis • Vascular spasm • Contraction of the smooth muscle around the injured vessel • Plug formation • Platelets stick to the walls (platelet adhesion) • Platelets physically change to have projections that hold onto one another • Enzymes released that make other platelets more sticky in the same area

21. Mechanisms of Hemostasis • Blood clotting • A ruptured blood vessel exposes collagen and other structural proteins to the blood • Proteins activate an enzyme reaction & certain blood proteins are changed into long fibrin strands • A gelatinous mass is formed that further occludes vessel opening • Clot reabsorbed by body when no longer needed

22. General Patient Assessment • Most patients with hematological problems are dealing with a chronic condition • Most patients need help due to a change in their baseline condition • Most care administered by EMS providers will be supportive in nature - treat the symptoms • When possible, honor patient requests for hospital destination • improved continuity of care • have release signed if necessary (not closest)

23. Patient Assessment • Scene size-up, BSI precautions, general impression • Initial assessment • Assess airway, breathing, circulation, mental status (AVPU) • Check for life threats • common issues could include life-threatening bleeds and massive infections with septic shock

24. Patient Assessment cont’d • Focused history and physical exam • Patient categorized as: • responsive or unresponsive medical patient • trauma patient with significant or non-significant mechanism of injury • Categorization determines which format is used to complete history and physical

25. Patient Assessment cont’d • SAMPLE history • anemia - increased heart rate & respirations • poor tissue oxygenation - fatigue, malaise, apprehension, confusion, change in skin color • clotting problem - excessive bruising, skin discoloration • infection - lymph node enlargement (swollen glands), sore throat, pain on swallowing • bleeding abnormalities - nausea, anorexia, bloody vomiting or diarrhea, bleeding gums • Vital signs, pulse oximetry, pain scale, EKG

26. Patient Assessment cont’d • Physical exam • eye problems • especially with autoimmune disorders (immune system can’t determine which tissues are self and which are not) and sickle cell anemia • skin condition • liver disease or hemolysis of RBC’s - jaundice • polycythemia - reddish tone • anemia - pale • bleeding under the skin - petechia (tiny red dots), purpura (large purple blotches), bruising • pruritis (itching) - excess of bilirubin • prolonged bleeding of relatively minor injuries

27. Patient Assessment cont’d • GI system • Low platelet counts & blood clot abnormalities • epistaxis (nosebleed) is common • may swallow excessive blood causing nausea and loose, dark bowel movements • bleeding of the gums • abdominal pain especially associated with problems of the spleen and/or liver • Musculoskeletal system • Pain & swelling in joints • autoimmune diseases (rheumatoid arthritis), hemophilia (bleeding into joint)

28. Patient Assessment cont’d • Cardiorespiratory system • Anemia • dyspnea, tachycardia, chest pain • Genitourinary system • Hematuria - blood in the urine • Heavy menstrual bleeding • Frank vaginal bleeding

29. General Treatment • Airway & breathing • Supplemental oxygen usually with non-rebreather • Watch for dyspnea and fatigue • Circulation • Fluid volume replacement with crystalloid solution (ie: NS or LR) does not carry oxygen • Watch for & treat dysrhythmias • Comfort measures • Morphine, oxygen, positioning • Psychological support for patient & family

30. Diseases of Red Blood Cells • Anemias • The most common disease of RBC’s • Defined as a hematocrit less than 37% in women; 40% in men • Sickle cell anemia = sickle cell disease • Disorder in production of red cells (sickle shape when oxygen levels become low) • Polycythemia • Excess production in red blood cells

31. Anemia • A sign and not a disease process • Bone marrow must be able to keep up with the destruction of older RBC’s to maintain adequate percentages • Anemia is a significant drop in the percentage of red blood cells (RBC’s) (low hematocrit) • Oxygen carrying capacity of the blood is reduced due to decrease in hemoglobin • Females may temporarily decrease RBC levels during menstruation

32. Anemia • Signs and symptoms • Fatigue, intolerance of cold, pale skin, dizziness, irritability, tachycardia, shortness of breath, chest pain • Many actual types of anemia: • Iron deficiency - inadequate iron intake • Pernicious - poor absorption vitamin B12 • Hemolytic - RBC’s destroyed faster than produced • Aplastic - bone marrow fails to produce blood cells

33. Sickle Cell Anemia/Disease • Inherited disorder of red blood cell production • RBC’s have sickle shape when oxygen levels are low; red blood cells become rigid • Average sickled RBC life span 10-20 days (normal is 120 days) • Generation of new RBC’s can’t keep up with the hemolysis (destruction) • Blood viscosity increased • sludging of blood • obstruction of capillaries & small blood vessels • blood flow to tissues & organs disrupted • tissues & organs eventually damaged • adults often have multiple organ problems • cardiopulmonary, renal, neurological diseases

34. Sickle Cell Crisis • Disease not limited to 1 ethnicity but can affect many including Caucasian • 3 presentations of crisis common: • Vasoocclusive crisis • musculoskeletal pain • abdominal pain • priapisms • pulmonary problems • renal crisis (renal infarction) • central nervous system crisis (cerebral infarctions)

35. Sickle Cell Crisis cont’d • Hematological Crisis • fall in hemoglobin level • stagnation of red blood cells in spleen • problems with bone marrow function • Infectious crisis • patient functionally immunosuppressed • vulnerable to infection & sepsis • loss of splenic function makes patient susceptible to massive bacterial infection

36. Sickle Cell Complications • Sepsis - due to immunosuppression • Acute chest syndrome • infection or sickled red cells trapped in lungs • dyspnea, coughing, chest pain • Hand-and-foot syndrome • painful swelling due to severe vascular occlusion • Splenic sequestration crisis • accumulation of sickled cells in spleen • pale, enlarged spleen, abdominal pain, shock

37. Sickle Cell Complications cont’d • Aplastic crisis • severe anemia when bone marrow temporarily stops producing red blood cells • pale, tired, less active than normal • Stroke • cerebral vascular occlusion due to sickled cells • can affect any age • extremity weakness, change in level of consciousness • Painful episode • acute and severe pain anywhere but most often hands, arms, chest, legs, feet

38. Prehospital Care Sickle Cell Anemia/Disease • Care is primarily supportive • Oxygen via nonrebreather mask to increase the saturation of the circulating RBC’s - evaluate pulse oximetry • IV of normal saline to hydrate patient • Pain relief with analgesics • larger than normal amounts of morphine are often required for pain control • involve medical control for higher dosing

39. Polycythemia • Abnormally high percentage of RBC’s (high hematocrit) • Rare disorder; typically in people over 50 • No cure but can be treated • Can be caused by: • Unregulated increase in RBC production • Tissue hypoxia • Dehydration • Blood doping - athlete training at high altitudes to increase RBC production • Makes it harder to pump blood through the body because it’s thicker • Increases risk of thrombosis

40. Polycythemia • Signs and symptoms • Bleeding abnormalities • epistaxis, spontaneous bruising, GI bleeding • Headache, dizziness, blurred vision • Itching • Severe cases with congestive heart failure • Treatment • Supportive • IV - O2 - monitor • Hospital treatment - phlebotomy (“blood letting”) to remove excess red blood cells

41. Diseases of White Blood Cells (WBC’s) • White blood cells are body’s principal defense system against infection • Problems could include: • Leukopenia/neutropenia • decrease in number of WBC’s • Leukocytosis • increase in number of circulating WBC’s • could indicate: bacterial infection, rheumatoid arthritis, DKA, leukemia, pain, excercise • Leukemia • Lymphomas

42. Leukemia • Malignant disease (cancer) of blood forming tissue • Classifications • Acute lymphocytic leukemia (ALL) • primarily of children and young adults • Acute myelogenous leukemia (AML) • primarily of older people in their 60’s and 70’s • Chronic lymphocytic leukemia (CLL) • primarily of older people in their 60’s and 70’s • Chronic myelogenous leukemia (CML) • occurs in children and adults

43. Leukemia • Common presentations: • moderate to severe anemia • abnormal decrease in platelets • patient appears acutely ill; febrile, weak, & fatigued; lymph node enlargement; history of weight loss & anorexia; enlarged liver & spleen with abdominal tenderness; tender sternum • Common complication - infection • primarily due to low number of circulating neutrophils (main blood component protecting against bacterial or fungal infection)

44. Leukemia & EMS Care • Patient at risk for infection • Isolation techniques (gloves and masks) to prevent spread of your germs to the patient • Care primarily supportive • Position of comfort • Oxygen via nonrebreather mask if needed • IV (fluid bolus if patient is dehydrated) • Analgesics (ie: morphine) for pain

45. Lymphomas • Cancer of the lymphatic system • Most prominent in the lymph nodes • Malignant lyphoma classification: • Hodgkin’s lymphoma • 7,500 diagnosed annually in USA • long-term survival better • treatable with radiation, chemotherapy, or both • can be curable • Non-Hodgkin’s lymphoma • 40,000 diagnosed annually in USA • cure rate depends on type of lymphoma identified

46. Lymphoma • Presenting signs & symptoms • may report fever, night sweats, anorexia, weight loss, fatigue, itching • many with Hodgkin’s have no symptoms • non-Hodgkin’s most commonly have swollen lymph nodes • Field treatment • supportive • consider IV, O2, pain control if necessary • isolation techniques • gloves & mask

47. Diseases of Platelets & Blood Clotting Abnormalities • Thrombocytosis - increased platelets • patients usually asymptomatic • Thrombocytopenia - decrease in platelets • easy bruising & bleeding • Hemophilia • absence of protein necessary for blood clotting • von Willebrand’s disease • inherited disease affecting both sexes • excessive bleeding after injury or surgery • aspirin contraindicated

48. Hemophilia • Inherited clotting deficiency • Females are carriers • 1 in 10,000 males have the disease • Bleeding may occur spontaneously or after minor trauma • Can be caused by deficiency of different clotting factors - factor VIII or factor IX • Bleeding takes longer to stop because body cannot form stable fibrin clots

49. Hemophilia • Signs and symptoms • numerous bruises • deep muscle bruising • joint bleeding - hemarthrosis • Characterized by blood in the urine, bloody noses and painful, swollen joints • Permanent joint damage with repeated bleeding • Transfusion of specific clotting factor near time of injury may lessen the bleeding but will never make the disease go away

50. Field Treatment Hemophilia • The platelet plug is not stable but the mechanisms of vasoconstriction and platelet aggregation will still occur • Patients may have prolonged bleeding and possible rebleeding • IV - O2 via nonrebreather- monitor • Ice application • Superficial injuries - patience & pressure • If joint injury, splinting will reduce pain • Transport to ED necessary for replacement of deficient factor