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

1
Curriculum UpdateThe Hematopoietic
System,Patients with Special Challenges,Interven
tions 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
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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

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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

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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

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Components of Blood

• Plasma
• Platelets
• thrombocytes
• White blood cells
• leukocytes
• Red blood cells
• erythrocytes

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Components by Volume
-WBCs platelets together form elements
- RBCs 95 of volume of
formed elements
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Components of Blood

• 78 water
• 22 solids (plasma, RBCs, WBCs, 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

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Red Blood Cells (RBCs) - 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

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Pulse Oximetry

• Estimates the amount of oxygen carried in the
  bloodstream using infrared technology across the
  skin

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White Blood Cells (WBCs) - Leukocytes

• Helps fight infection and aids in the immune
  process
• Helps heal wounds by ingesting matter (dead
  cells, tissue debris, old RBCs)
• Protects the body from foreign material
  (antigens)
• Involved in the protection from mutated cells
  (ie cancer)
• Includes lymphocytes, monocytes, eosinophils,
  basophils, neutrophils

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Platelets - Thrombocytes

• Small, sticky cells
• Helps in blood clotting
• Groups together to form clumps to plug holes and
  leaks to stop the bleeding
• WBCs, RBCs, and platelets make up about 45 of
  the blood volume
• Plasma takes up about 55 of the blood volume

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More Blood Components

• Fat globules
• Chemical substances
• carbohydrates
• proteins
• hormones
• Gases including
• oxygen
• carbon dioxide
• nitrogen

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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

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Blood Groups

• Surfaces of RBCs 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-

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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

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Blood groups

• Rh blood groups
• Discovered in the blood of the
  rhesus monkey in 1940s
• 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 RBCs and
  release of hemoglobin)

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Acquired Factor Deficiencies
Rh

• 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

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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

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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
  

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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

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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

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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)

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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

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Patient Assessment contd

• 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

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Patient Assessment contd

• 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

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Patient Assessment contd

• Physical exam
• eye problems
• especially with autoimmune disorders (immune
  system cant determine which tissues are self and
  which are not) and sickle cell anemia
• skin condition
• liver disease or hemolysis of RBCs - 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

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Patient Assessment contd

• 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)

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Patient Assessment contd

• Cardiorespiratory system
• Anemia
• dyspnea, tachycardia, chest pain
• Genitourinary system
• Hematuria - blood in the urine
• Heavy menstrual bleeding
• Frank vaginal bleeding

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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

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Diseases of Red Blood Cells

• Anemias
• The most common disease of RBCs
• 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

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Anemia

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

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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 - RBCs destroyed faster than produced
• Aplastic - bone marrow fails to produce blood
  cells
• 
  

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Sickle Cell Anemia/Disease

• Inherited disorder of red blood cell production
• RBCs 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 RBCs cant 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
• 
  

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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)

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Sickle Cell Crisis contd

• 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

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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

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Sickle Cell Complications contd

• 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

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Prehospital Care Sickle Cell Anemia/Disease

• Care is primarily supportive
• Oxygen via nonrebreather mask to increase the
  saturation of the circulating RBCs - 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

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Polycythemia

• Abnormally high percentage of RBCs (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 its thicker
• Increases risk of thrombosis

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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

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Diseases of White Blood Cells (WBCs)

• White blood cells are bodys principal defense
  system against infection
• Problems could include
• Leukopenia/neutropenia
• decrease in number of WBCs
• Leukocytosis
• increase in number of circulating WBCs
• could indicate bacterial infection, rheumatoid
  arthritis, DKA, leukemia, pain, excercise
• Leukemia
• Lymphomas

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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 60s and 70s
• Chronic lymphocytic leukemia (CLL)
• primarily of older people in their 60s and 70s
• Chronic myelogenous leukemia (CML)
• occurs in children and adults

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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)

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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

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Lymphomas

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

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Lymphoma

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

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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 Willebrands disease
• inherited disease affecting both sexes
• excessive bleeding after injury or surgery
• aspirin contraindicated

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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

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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

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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

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Other Hematopoietic Disorders

• Disseminated intravascular coagulation - DIC
• Disorder of coagulation
• High mortality rate
• Most commonly results from
• sepsis, hypotension, obstetric complications,
  severe tissue injury, brain injury, cancer, major
  hemolytic blood transfusion reactions
• Fibrin clots formed throughout circulation
  instead of just at the affected area of need
• Simultaneous clotting and bleeding
• Patient experiences widespread thrombosis and
  end-organ ischemia

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DIC

• Signs symptoms
• Oozing blood at
  venipuncture and wound sites
• Purpuric rash often over chest and abdomen
• Minute hemorrhages just under skin
• Prehospital care
• Supportive and focused on symptoms
• IV - O2- monitor
• Patient often hemodynamically unstable
• Hospital treatment
• Fresh frozen plasma platelets at hospital

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Additional Hematopoietic Disorders

• Multiple myeloma
• Cancer disorder of plasma cells (cells that
  produce antibodies)
• Rarely found in persons under 40
• Approximately 14,000 new cases every year
• Cancer plasma cells overcrowd healthy cells with
  a reduction in blood cell production
• Patient becomes anemic and is prone to infection

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Multiple Myeloma

• Signs symptoms
• Pain in back or ribs
• Diseased marrow weakens bones and pathological
  fractures occur (fractures with minimal or no
  trauma)
• Fatigue
• Risk for bleeding due to decrease in platelets
• Renal failure from elevated calcium levels
• Increased risk for infection due to a lack of
  certain antibody secretion

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Multiple Myeloma Treatment

• Prehospital care
• Supportive
• IV if signs of dehydration
• Pain control - disease can be painful and pain in
  presence of pathological fractures
• Hospital care
• Chemotherapy
• Radiation
• Bone marrow transplants

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What Is Bone Marrow?

• Tissue in the center of large bones where new
  blood cells are produced
• 2 types of stem cells produced
• hematopoietic stem cells - forms blood cells
• WBCs - leukocytes
• RBCs - erythrocytes
• platelets - thrombocytes
• stromal stem cells - mix of cells to generate
• fat
• cartilage
• bone
• can differentiate into many kinds of tissue (ie
  nervous)

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Bone Marrow
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Bone Marrow Transplant

• Pioneered in 1970
• Transplant of hematopoietic stem cells
• Most often done for life-threatening diseases of
  blood or bone marrow
• acute lymphocytic leukemia
• acute myelogenous leukemia
• aplastic anemia
• chronic myelogenous leukemia
• Hodgkins and non-Hodgkins diseases
• multiple myeloma
• radiation poisoning

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Types of Bone Marrow Transplant

• Autologous bone marrow transplant
• stem cells obtained months in advance of use
• stem cells isolated from patient stored in
  freezer patient treated to destroy remaining
  stem cells harvested stem cells reinfused
• Allogenic bone marrow transplant
• involves donor recipient must match tissue
  type
• recipient requires immunosuppressant drugs
• donor bone marrow harvested under general
  anesthesia (100 needle sticks in large bone)

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Bone Marrow Transplant Contd

• Peripheral blood stem cell process - apheresis
• donor blood withdrawn via needle blood passes
  thru machine to remove WBCs RBCs returned to
  donor WBC stem cells can be stored frozen
• recipient gets injection to boost stem cell yield
• Cord blood
• donation of umbilical cord placenta after
  infant delivered
• cord blood yields higher concentration of stem
  cells but limited volume (50 ml - 2 T)
• usually transplanted into children, not adults
• cord blood can be stored frozen for years

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Bone Marrow Infusion

• Stem cells (bone marrow transplant) given IVPB
• stem cells circulate in the blood stream
• stem cells migrate into bone marrow spaces
• stem cells grow start to produce new blood
  cells
• takes several weeks to grow enough new cells
• Bone marrow transplant mortality rate is high -
  10
• reserved for life-threatening conditions

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Case Scenario 1

• Your 22 year-old patient with hemophilia presents
  after falling off his motorcycle at low speed.
• There are some minor abrasions over the
  extremities and the patient complains of pain and
  swelling to the left elbow.
• Vital signs B/P 132/70 P - 96 R - 18
• What is the order of the assessment steps to
  take?
• What interventions are necessary?

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Case Scenario 1

• Assessment steps
• Scene size-up, BSI, general impression
• Initial assessment
• ABCs, AVPU, check for life threats
• Focused history physical exam
• trauma without significant mechanism of injury
• perform a head-to-toe
• SAMPLE, vital signs, pulse ox, pain scale

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Case Scenario 1

• Interventions
• Manual c-spine control
• Follow SOP Spinal clearance, In-field
• evaluate ?mechanism of injury, ?signs symptoms,
  ?patient reliability
• IV-O2-monitor as applicable
• Application of ice
• Splinting of injured/swollen extremities will
  help with pain control
• Direct pressure for bleeding wounds

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Case Scenario 2

• Your 65 year old patient undergoing treatment for
  leukemia calls for transport.
• What is a common complication for this group of
  patients?
• What method of infectious control needs to be
  exercised by the EMS responders?

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Case Scenario 2

• A common complication for patients with leukemia
  is infection
• primarily due to the low number of circulating
  neutrophils (WBC component responsible for
  protecting against bacterial and fungal
  infection)
• BSIs necessary for providers to wear include
• gloves
• mask