Acute care handbook for physical therapists (fourth edition) chapter 7 vascular system and hematology

Acute care handbook for physical therapists (fourth edition) chapter 7 vascular system and hematology Acute care handbook for physical therapists (fourth edition) chapter 7 vascular system and hematology Acute care handbook for physical therapists (fourth edition) chapter 7 vascular system and hematology Acute care handbook for physical therapists (fourth edition) chapter 7 vascular system and hematology Acute care handbook for physical therapists (fourth edition) chapter 7 vascular system and hematology Acute care handbook for physical therapists (fourth edition) chapter 7 vascular system and hematology Acute care handbook for physical therapists (fourth edition) chapter 7 vascular system and hematology

CHAPTER Vascular System and Hematology Falguni Vashi CHAPTER OUTLINE CHAPTER OBJECTIVES Body Structure Vascular System Structure Hematologic System Structure Lymphatic System Structure Body Function Physical Examination Vascular Evaluation Hematologic Evaluation Lymphatic Evaluation Health Conditions Vascular Disorders Hematologic Disorders Lymphatic Disorders Management Pharmacologic Therapy Anticoagulation Therapy Blood Product Transfusion Vascular Surgical Procedures Physical Therapy Management for Patients with Vascular and Hematologic Disorders The objectives of this chapter are to provide the following: Review the structure and function of blood and blood vessels Review the vascular and hematologic evaluation, including physical examination and diagnostic and laboratory tests Describe vascular, hematologic, and lymphatic health conditions, including clinical findings, medical and surgical management, and physical therapy intervention PREFERRED PRACTICE PATTERNS The most relevant practice patterns for the diagnoses discussed in this chapter, based on the American Physical Therapy Association’s Guide to Physical Therapist Practice, second edition, are as follows: • Arterial Disorders (Atherosclerosis, Aneurysm, Aortic Dissection, Hypertension, Raynaud’s Disease), Chronic Regional Pain Syndrome, Compartment Syndrome: 4C, 4J, 6D, 7A • Venous Disorders (Varicose Veins, Venous Thrombosis, Pulmonary Embolism, Chronic Venous Insufficiency): 4C, 6D, 7A • Combined Arterial and Venous Disorders (Arteriovenous Malformations): 4C, 6D, 7A • Hematologic Disorders (Erythrocytic Disorders [Anemia], Polycythemia), Thrombocytic Disorders: 4C, 6D • Lymphatic Disorders (Lymphedema): 4C, 6D, 6H Please refer to Appendix A for a complete list of the preferred practice patterns, as individual patient conditions are highly variable and other practice patterns may be applicable Alterations in the integrity of the vascular and hematologic systems can alter a patient’s activity tolerance The physical therapist must be aware of the potential impact that a change in blood composition or blood flow has on a multitude of body functions, including cardiac output, hemostasis, energy level, and healing Body Structure The network of arteries, veins, and capillaries composes the vascular system Living blood cells and plasma within the blood vessels are the structures that compose the hematologic system The lymphatic system assists the vascular system by draining unabsorbed plasma from tissue spaces and returning this fluid (lymph) to the heart via the thoracic duct, which empties into the left jugular vein The flow of lymph is regulated by intrinsic contractions of the lymph vessels, muscular contractions, respiratory movements, and gravity.1 Vascular System Structure All blood vessels are composed of three similar layers (Figure 7-1 and Table 7-1) Blood vessel diameter, length, and wall thickness vary according to location and function (Table 7-2) Note 161 162 CHAPTER 7    Vascular System and Hematology Artery Vein Endothelium (tunica intima) Valve Elastic membrane (thinner in veins) Smooth muscle layer (tunica media) (thinner in veins) Connective tissue (tunica advenitia) FIGURE 7-1  Structure of the arteries and veins (From Lewis SL, Heitkepmer M, Dirksen S et al: Medical-surgical nursing: assessment and management of clinical problems, ed 7, St Louis, 2007, Mosby.) TABLE 7-1  Blood Vessel Layers Layer Description Function Tunica intima Innermost layer: Endothelial layer over a basement membrane Tunica media Middle layer: Smooth muscle cells and elastic connective tissue with sympathetic innervation Outermost layer: Composed of collagen fibers, lymph vessels, and the blood vessels that supply nutrients to the blood vessel Provides a smooth surface for laminar blood flow Constricts and dilates for blood pressure regulation Protects and attaches blood vessels to nearby structures Tunica adventitia Data from Marieb EN: Human anatomy and physiology, ed 3, Redwood City, CA, 1995, Benjamin-Cummings TABLE 7-2  Characteristics of Blood Vessels Vessel Description Artery Large or elastic arteries—aorta and its large branches and pulmonary artery Medium or muscular arteries—composing other branches of aorta (i.e., coronary arteries) Small arteries and arterioles Thick tunica media layer allows arteries to readily accommodate to pressure changes from the heart Small, medium, or large in diameter Thin tunica media and thick tunica adventitia Valves prevent backflow of blood to maintain venous return to the heart The interface of the arterial and venous systems where blood cells, fluids, and gases are exchanged Capillary beds can be open or closed, depending on the circulatory requirements of the body Vein Capillary network Data from Marieb EN: Human anatomy and physiology, ed 3, Redwood City, CA, 1995, Benjamin-Cummings; Kumar V: Robbins and Cotran pathologic basis of disease, ed 7, Philadelphia, 2005, Saunders that the arteries are divided into three types, depending on their size and structural features Hematologic System Structure Blood is composed of living cells (Table 7-3) in a nonliving plasma solution and accounts for 8% of total body weight, or to liters in women and to liters in men Plasma is composed almost completely of water and contains more than 100 dissolved substances The major solutes include albumin, fibrinogen, protein globules, nitrogenous substances, nutrients, electrolytes, and respiratory gases.2 Lymphatic System Structure The lymphatic system includes lymph vessels, lymph fluid, and lymph tissues and organs (lymph nodes, tonsils, spleen, thymus, and the thoracic duct) The lymphatic system is parallel to and works in concert with the venous system Lymphatics are fragile and are more likely to collapse under pressure than the veins Lymphatics are located in all portions of the body except the central nervous system and cornea Lymph moves throughout the body through a number of mechanisms, and the excess CHAPTER 7    Vascular System and Hematology TABLE 7-3  Blood Cell Types Cell Description Erythrocyte (red blood cell; RBC) Contains hemoglobin molecules responsible for oxygen transport to tissues Composed of four protein chains (two alpha and two beta chains) bound to four iron pigment complexes An oxygen molecule attaches to each iron atom to become oxyhemoglobin Five types of WBCs (neutrophils, basophils, eosinophils, lymphocytes, and monocytes) are responsible for launching immune defenses and fighting infection WBCs leave the circulation to gain access to a site of infection Cell fragment responsible for clot formation Leukocyte (white blood cell; WBC) Thrombocyte (platelet; Plt) Data from Marieb EN: Human anatomy and physiology, ed 3, Redwood City, CA, 1995, Benjamin-Cummings TABLE 7-4  Functions of Blood Function Method Oxygen and carbon dioxide transport Nutrient and metabolite transport Hormone transport Transport of waste products to kidneys and liver Transport of cells and substances involved in immune reactions Clotting at breaks in blood vessels Maintenance of fluid balance Body temperature regulation Binding to hemoglobin; dissolved in plasma Bound to plasma proteins; dissolved in plasma In plasma In plasma Maintenance of acid-base balance In plasma to site of infection or foreign body Hemostasis Blood volume regulation Peripheral vasoconstriction or dilation Acid-base regulation Data from Nettina S: The Lippincott manual of nursing practice, ed 8, Philadelphia, 2005, Lippincott Williams & Wilkins lymph is transported to the thoracic duct and emptied into the jugular vein trunks Lymph fluid is first absorbed at the capillary level, then channeled through the small vessels and finally picked up by the larger valved vessels.3 Body Function The function of the blood vessels is to carry blood throughout the body to and from the heart (Table 7-4) Normal alterations in the vessel diameter will occur, depending on circulating blood volume and the metabolic needs of the tissues The function of the lymphatic system is to (1) protect the body from infection and disease via the immune response and 163 (2) to facilitate movement of fluid back and forth between the bloodstream and the interstitial spaces, removing excess fluid, blood waste, and protein molecules in the process of fluid exchange.3 The vascular and hematologic systems are intimately linked, and the examination of these systems is often similar For the purpose of this chapter, however, the evaluations of the vascular and hematologic systems are discussed separately Physical Examination Vascular Evaluation History In addition to the general chart review (see Chapter 2), it is important to gather the following information during examination of the patient with a suspected vascular disorder4-8: • Relevant medical history that includes diabetes mellitus, hypertension, hyperlipidemia, syncope or vertigo, and nonhealing ulcers • Relevant social history that includes exercise and dietary habits, as well as the use of tobacco or alcohol • History of recent prolonged bed rest and/or surgery or a long flight • Pain in arms and legs (Visceral pain and arthritis pain may radiate to the extremities.) • Presence of intermittent claudication (pain, ache, sense of fatigue, or other discomfort that occurs in the affected muscle group with exercise, particularly walking, and resolves with rest.) The speed, distance, and the site of the pain, including what relieves the pain, should be noted • Buttock, hip, or thigh claudication typically occurs in patients with obstruction of the aorta and iliac arteries Calf claudication characterizes femoral and popliteal artery stenosis The gastrocnemius muscle consumes more oxygen during walking than other muscle groups in the leg and hence causes the most frequent symptom reported by patients • Presence of nocturnal pain that can develop as the vascular occlusion worsens This type of pain occurs when the patient is in bed and is caused by a combination of leg elevation and reduced cardiac output • Presence of rest pain refers to pain that occurs in the absence of activity and with legs in a dependent position Rest pain signals advanced occlusive disease, typically greater than 90% occlusion • Presence or history of acute or chronic peripheral edema If chronic, what is the patient’s baseline level of edema? • Precautions, such as weight bearing or blood pressure parameters after vascular surgery   CLINICAL TIP Intermittent claudication is often abbreviated in the clinical setting as IC 164 CHAPTER 7    Vascular System and Hematology Inspection Observation of the following features can help delineate the location and severity of vascular disease and help determine whether these manifestations are arterial or venous in origin1,4,5,9: • Skin color: Note the presence of any discoloration of the distal extremities/nail bed, which is indicative of decreased blood flow (e.g., mottled skin) • Hair distribution: Patchy hair loss on the lower leg may indicate arterial insufficiency • Venous pattern: Dilation or varicosities—dilated, purplish, ropelike veins, particularly in the calf • Edema or atrophy: Peripheral edema from right-sided congestive heart failure occurs bilaterally in dependent areas; edema from trauma, lymphatic obstruction, or chronic venous insufficiency is generally unilateral Refer to Table 3-5 for grading of pitting edema Measurement of the extremities may help to identify the edema or atrophy With a flexible tape, measure: • The forefoot • The smallest possible circumference above the ankle • The largest circumference at the calf • The mid-thigh, a measured distance above the patella with the knee extended A difference greater than 1 cm just above the ankle or 2 cm at the calf is suggestive of edema • Presence of cellulitis • Presence of petechiae: Small, purplish, hemorrhagic spots on the skin • Skin lesions: Ulcers, blisters, or scars • Digital clubbing: Could be indicative of poor arterial oxygenation or circulation • Gait abnormalities Palpation During the palpation portion of the examination, the physical therapist can assess the presence of pain and tenderness, strength and rate of peripheral pulses, respiratory rate, blood pressure, skin temperature, and limb girth (if edematous) Changes in heart rate, blood pressure, and respiratory rate may correspond to changes in the fluid volume status of the patient For example, a decrease in fluid volume may result in a decreased blood pressure that results in a compensatory increase in heart and respiratory rates The decreased fluid volume and resultant increased heart rate in this situation may then result in a decreased strength of the peripheral pulses on palpation A decreased or absent pulse provides insight into the location of arterial stenoses.7 In patients with suspected or diagnosed peripheral vascular disease, monitoring distal pulses is more important than monitoring central pulses in the larger, more proximal vessels.4 The following system/scale is used to grade peripheral pulses10: • 0: Absent, not palpable • 1: Diminished, barely palpable • 2: Brisk, expected • 3: Full, increased • 4: Bounding Peripheral pulses can be assessed in the following arteries (see Chapter 3, Figure 3-6): • Temporal • Carotid • Brachial • Ulnar • Radial • Femoral • Popliteal • Posterior tibial • Dorsalis pedis   CLINICAL TIP A small percentage of the adult population may normally have absent peripheral pulses; 10% to 17% lack dorsalis pedis pulses.1 Peripheral pulse grades are generally denoted in the medical record by physicians in the following manner: dorsalis pedis +1 The aorta can also be palpated in thin people Physical Therapy Considerations • In patients who have disorders resulting in vascular compromise (e.g., diabetes mellitus, peripheral vascular disease, or hypertension), pulses should be monitored before, during, and after activity not only to determine any rate changes, but, more important, to determine any changes in the strength of the pulse • Notation should be made if the strength of pulses is correlated to complaints of pain, numbness, or tingling of the extremity • Compare the two extremities for color, temperature, and swelling Bilateral coldness is most often due to cold environment and/or anxiety.5 • Carotid arteries should never be palpated simultaneously, as excessive carotid sinus massage can cause slowing of the pulse, cause a drop in blood pressure, and compromise blood flow to the brain If the pulse is difficult to palpate, the patient’s head should be rotated to the side being examined to relax the sternocleidomastoid.10 Auscultation Systemic blood pressure and the presence of bruits (whooshing sound indicative of turbulent blood flow from obstructions) are assessed through auscultation.4 Bruits are often indicative of accelerated blood flow velocity and flow disturbance at sites of stenosis.7 Bruits are typically assessed by physicians and nurses (see Chapter for further details on blood pressure measurement) Vascular Tests Various tests that can be performed clinically to evaluate vascular flow and integrity are described in Table 7-5 These tests can be performed easily at the patient’s bedside without the use of diagnostic equipment The Wells Clinical Decision Rule for Deep Venous Thrombosis is described in Table 7-6 165 CHAPTER 7    Vascular System and Hematology TABLE 7-5  Vascular Tests Test Indication Description Normal Results and Values Capillary refill time* To assess vascular perfusion and indirectly assess cardiac output Blanching should resolve (capillary refill) in less than seconds Elevation pallor To assess arterial perfusion Normally color should not change A gray (darkskinned individuals) or pale/pallor (fair-skinned individuals) discoloration will result from arterial insufficiency or occlusion Nail beds of fingers or toes are squeezed until blanching (whitening) occurs, and then they are released A limb is elevated 30-40 degrees for 15-60 seconds, and color changes are observed over 60 seconds Trendelenburg’s test/ Retrograde filling test To determine if superficial or deep veins and their valves are involved in causing varicosities Manual compression test To detect competent valves in the veins Allen’s test To assess the patency of the radial and ulnar arteries, to ensure the collateral circulation of the hand Homans’ sign† To detect the presence of deep vein thrombosis To Trendelenburg’s test, mark the distended veins with a pen while the patient stands Then have the patient lie on the examination table and elevate his or her leg for about a minute to drain the veins Next, have the patient stand while you measure venous filling time If the veins fill in less than 30 seconds, have the patient lie on the examination table again, and elevate his or her leg for minute Then apply a tourniquet around his or her upper thigh Next, have the patient stand Next, remove the tourniquet To pinpoint incompetent valve location, repeat this procedure by applying the tourniquet just below the knee and then around the upper calf Palpate the dilated veins with the fingertips of one hand With the other hand, firmly compress the vein at a point at least inches (20.3 cm) higher Palpate the impulse under your finger Flex the patient’s arm with the hand above level of the elbow Then compress the radial and ulnar arteries at the level of the wrist while the patient clenches his or her fist The patient then opens his or her hand and either the radial or the ulnar artery is released The process is repeated for the other artery The calf muscle is gently squeezed, or the foot is quickly dorsiflexed Observe the amount of time it takes for pallor to appear: Pallor within 25 seconds indicates severe occlusive disease Pallor within 25-40 seconds indicates moderate occlusive disease Pallor within 40-60 seconds indicates mild occlusive disease Competent valves take at least 30 seconds to fill If leg veins still fill in less than 30 seconds, suspect incompetent perforating vein and deep vein valves (functioning valves block retrograde flow) If the veins fill again in less than 30 seconds, suspect incompetent superficial vein valves that allow backward blood flow With a competent valve, there will be no detectable impulse A palpable impulse indicates incompetent valves in the vein segment between the two hands When the patient opens the hand, the blanched area should flush within seconds if collateral circulation is adequate If the blanched area does not flush quickly, then it may indicate that collateral circulation is inadequate to support circulation to the hand Pain that is elicited with either squeezing or dorsiflexing may indicate a deep vein thrombosis Continued 166 CHAPTER 7    Vascular System and Hematology TABLE 7-5  Vascular Tests—cont’d Test Indication Description Normal Results and Values Ankle-brachial index (ABI)‡ To compare the perfusion pressures in the lower leg with the upper extremity using a blood pressure cuff and Doppler probe This test is commonly used to screen patients for evidence of significant arterial insufficiency Place the patient in supine at least 10 minutes before the test Obtain the brachial pressure in each arm, and record the highest pressure Obtain the ankle pressure in each leg Place the cuff around the lower leg 2.5 cm above the malleolus Apply acoustic gel over the dorsalis pedis or posterior tibialis pulse location Hold the Doppler probe lightly over the pedal pulse Inflate the cuff to a level 20-30 mm Hg above the point at which the pulse is no longer audible Slowly deflate the cuff while monitoring for the return of the pulse signal; the point at which the arterial signal returns is recorded as the ankle pressure Calculate the ABI by dividing the higher of the two ankle systolic pressures by the higher of the brachial systolic pressures The diagnosis of peripheral arterial disease is based on the limb symptoms or an ABI Interpretation of ABI ABI ≥ 1.0-1.3: normal range.§ ABI ≥ 0.7-0.8: borderline perfusion ABI ≤ 0.5: severe ischemia, wound healing unlikely ABI ≤ 0.4: critical limb ischemia Data from Seidel HM, Ball JW, Dains JE et al: Mosby’s guide to physical examination, ed 7, St Louis, 2010, Mosby; Lanzer P, Rosch J, editors: Vascular diagnostics: noninvasive and invasive techniques, peri-interventional evaluations, Berlin, 1994, Springer Verlag; Springhouse: Handbook of medical-surgical nursing, ed 4, Philadelphia, 2005, Lippincott Williams & Wilkins; Newberry L, Sheehy S, editors: Sheehy’s emergency nursing: principles and practice, ed 6, St Louis, 2009, Mosby; Dormandy JA, Rutherford RB: Management of peripheral arterial disease (PAD) TASC Working Group, J Vasc Surg 31:S1–S296, 2000; Hallet JW, Brewster DC, Darling RC, editors: Handbook of patient care in vascular surgery, ed 3, Boston, 1995, Little, Brown; Goodman CC: The hematologic system In Goodman CC, Boisonnault WG, editors: Pathology: implications for the physical therapist, ed 3, Philadelphia, 2009, Saunders *Variability is found in defining the time by different individuals, so capillary refill time should not be considered an observation with exquisite sensitivity and specificity and should be used more to confirm clinical judgment †A 50% false-positive rate occurs with this test Vascular laboratory studies are more sensitive ‡ABI measurements may be of limited value in anyone with diabetes because calcification of the tibial and peroneal arteries may render them noncompressible §An ABI of less than 0.95 is considered abnormal and is 95% sensitive for the angiographically verified peripheral arterial stenosis Diagnostic Studies Noninvasive Laboratory Studies.  Various noninvasive proce- dures can examine vascular flow The phrases lower-extremity noninvasive studies and carotid noninvasive studies are general descriptions that are inclusive of the noninvasive tests described in Table 7-7 Invasive Vascular Studies.  The most common invasive vascular study is arteriography, typically referred to as contrast angiography (Figure 7-2) This study is performed by injecting radiopaque dye into the femoral, lumbar, brachial, or axillary arteries, followed by radiographic viewing Blood flow dynamics, abnormal blood vessels, vascular anomalies, normal and abnormal vascular anatomy, and tumors are easily seen during the radiographic viewing With the use of digital-subtraction angiography (DSA), bony structures can be obliterated from the picture DSA is useful when adjacent bone inhibits visualization of the blood vessel to be evaluated.11 An angiogram is a picture produced by angiography Angiography is generally performed before or during therapeutic interventions, such as percutaneous angioplasty, thrombolytic therapy, or surgical bypass grafting Postangiogram care includes the following12: • Bed rest for to hours • Pressure dressings to the injection site with assessment for hematoma formation • Intravenous fluid administration to help with dye excretion Blood urea nitrogen (BUN) and creatinine are monitored to ensure proper renal function (refer to Chapter for more information on BUN and creatinine) • Frequent vital sign monitoring with pulse assessments • If a patient has been on heparin before angiography, the drug is not resumed for a minimum of hours.12 The complications of arteriography can be due to the catheterization or due to the contrast agent that is injected (Table 7-8) Hematologic Evaluation The medical workup of the patient with a suspected hematologic abnormality includes the patient’s medical history and laboratory studies, in addition to the patient’s clinical presentation History In addition to the general chart review (see Chapter 2), the following questions are especially relevant in the evaluation of the patient with a suspected hematologic disorder13-15: • What are the presenting symptoms? • Was the onset of symptoms gradual, rapid, or associated with trauma or other disease? CHAPTER 7    Vascular System and Hematology 167 TABLE 7-6  Wells Clinical Decision Rule (CDR) for Deep Venous Thrombosis Clinical Characteristic Score* Active cancer (treatment ongoing within previous 6 mo or palliative) Paralysis, paresis, or recent plaster immobilization of the lower extremities Recently bedridden for more than days or major surgery, within weeks Localized tenderness along the distribution of the deep venous system Entire leg swollen Calf swelling by more than 3 cm when compared with the asymptomatic leg (measured 10 cm below tibial tuberosity) Pitting edema (greater in the symptomatic leg) Collateral superficial veins (nonvaricose) Alternative diagnosis as likely as or more likely than deep vein thrombosis 1 1 1 1 −2 From Wells PS, Anderson DR, Bormanis J et al: Value of assessment of pretest probability of deep-vein thrombosis in clinical management, Lancet 350(9094):17951798, 1997 *−2 to 0: Low probability of DVT (3%); to 2: Moderate probability of DVT (17%); or more: High probability of DVT (75%) Medical consultation is advised in the presence of low probability; medical referral is required with moderate or high score TABLE 7-7  Noninvasive Vascular Studies Test Description Doppler ultrasound High-frequency and low-intensity (1-10 MHz) sound waves are applied to the skin with a Doppler probe (and acoustic gel) to detect the presence or absence of blood flow, direction of flow, and flow character over arteries and veins with an audible signal Low-frequency waves generally indicate low-velocity blood flow Ultrasound examination has a sensitivity and specificity of approximately 95% Velocity patterns of blood flow along with visual images of vessel and plaque anatomy can be obtained by combing ultrasound with a pulsed Doppler detector Distinctive color changes indicate blood flow through a stenotic area Plethysmography is a noninvasive test that provides measurement of changes in the volume of the blood distal to the affected area indicating an occlusion and specifically for the amount of time required for the veins to refill after being emptied Exercise testing is performed to assess the nature of claudication by measuring ankle pressures and pulse volume recordings (PVRs) after exercise A drop in ankle pressures can occur with arterial disease This type of testing provides a controlled method to document onset, severity, and location of claudication Screening for cardiorespiratory disease can also be performed, as patients with peripheral vascular disease often have concurrent cardiac or pulmonary disorders (see Chapter 3) CT is used to provide visualization of the arterial wall and its structures Indications for CT include diagnosis of abdominal aortic aneurysms and postoperative complications of graft infections, occlusions, hemorrhage, and abscess MRI has multiple uses in evaluating the vascular system and is now more commonly used to visualize the arterial system than arteriograms Specific uses for MRI include detection of deep venous thrombosis and evaluation of cerebral edema (Serial MRIs can also be used to help determine the optimal operative time for patients with cerebrovascular accidents by monitoring their progression.) MRA uses blood as a physiologic contrast medium to examine the structure and location of major blood vessels and the flow of blood through these vessels The direction and rate of flow can also be quantified MRA minimizes complications that may be associated with contrast medium injection Figure 7-2 illustrates the MRA of the aorta and lower extremity Color duplex scanning or imaging Plethysmography Exercise testing Computed tomography (CT) Magnetic resonance imaging (MRI) Magnetic resonance angiography (MRA) Data from Black JM, Matassarin-Jacobs E, editors: Luckmann and Sorensen’s medical-surgical nursing: a psychophysiologic approach, ed 4, Philadelphia, 1993, Saunders, p 1286; Bryant RA, Nix DP: Acute and chronic wounds Current management concepts, ed 4, St Louis, 2012, Mosby; Lanzer P, Rosch J, editors: Vascular diagnostics: noninvasive and invasive techniques, peri-interventional evaluations, Berlin, 1994, Springer Verlag; Kee JL, editor: Laboratory and diagnostic tests with nursing implications, ed 8, Stamford, CT, 2009, Appleton & Lange, p 606; Malarkey LM, Morrow ME, editors: Nurses manual of laboratory tests and diagnostic procedures, ed 2, Philadelphia, 2000, Saunders, p 359; Mettler FA: In Essentials of radiology, ed 2, Philadelphia, 2005, Saunders; Fahey VA, editor: Vascular nursing, ed 4, Philadelphia, 2003, Saunders; McCance KL, Huether SE, editors: Pathophysiology: the biologic basis for disease in adults and children, ed 6, St Louis, 2009, Mosby, p 1001; George-Gay B, Chernecky CC: In Clinical medical-surgical nursing: a decision-making reference, ed 1, Philadelphia, 2002, Saunders; Schroeder ML: Principles and practice of transfusion medicine, ed 10 In Lee GR, Foerster J, Lukens J et al, editors: Wintrobe’s clinical hematology, vol 1, Baltimore, 1999, Lippincott Williams & Wilkins, pp 817-874 168 CHAPTER 7    Vascular System and Hematology • • • • Have there been night sweats, chills, or fever? Is the patient easily bruised? Is wound healing delayed? Is there excessive bleeding or menses? Other relevant data include the patient’s diet (for the evaluation of vitamin- or mineral-deficiency anemia), history of weight loss (as a warning sign of cancer or altered metabolism), whether the patient abuses alcohol (a cause of anemia with chronic use), and race (some hematologic conditions have a higher incidence in certain races) Inspection During the hematologic evaluation, the patient is observed for the following13,16,17: • General appearance (for lethargy, malaise, or apathy) • Degree of pallor or flushing of the skin, mucous membranes, nail beds, and palmar creases Pallor can be difficult to assess in dark-skinned individuals In these individuals, lips, tongue, mucosa, and nail beds should be monitored • Presence of petechiae (purplish, round, pinpoint, nonraised spots caused by intradermal or subcutaneous hemorrhage) or ecchymosis (bruising) • Respiratory rate FIGURE 7-2  Magnetic resonance angiography (MRA) of the aorta and lower extremity arterial circulation (From Adam A: Grainger & Allison’s diagnostic radiology, ed 5, London, 2008, Churchill Livingstone.) TABLE 7-8  Complications of Contrast Arteriography Cause Complication Puncture site or catheter related Hemorrhage/hematoma Pseudoaneurysm Arteriovenous fistula Atheroembolism Local thrombosis Major (anaphylactoid) sensitivity reaction Minor sensitivity reactions Vasodilation/hypotension Nephrotoxicity Hypervolemia Contrast agent related From Belkin M, Owens CD, Whittemore AD et al: Peripheral arterial occlusive disease In Townsend CM, Beauchamp RD, Evers BM et al, editors: Sabiston textbook of surgery: the biological basis of modern surgical practice, ed 18, Philadelphia, 2007, Saunders • Is the patient unable to complete daily activities secondary to fatigue? • Is there a patient or family history of anemia or other blood disorders, cancer, hemorrhage, or systemic infection? • Is there a history of blood transfusion? • Is there a history of chemotherapy, radiation therapy, or other drug therapy? • Has there been an environmental or occupational exposure to toxins? Palpation The examination performed by the physician includes palpation of lymph nodes, liver, and spleen as part of a general physical examination For specific complaints, the patient may receive more in-depth examination of a body system Table 7-9 summarizes abnormal hematologic findings by body system on physical examination The physical therapist may specifically examine the following: • The presence, location, and severity of bone or joint pain using an appropriate pain scale (see Chapter 21) • Joint range of motion and integrity, including the presence of effusion or bony abnormality • Presence, location, and intensity of paresthesia • Blood pressure and heart rate for signs of hypovolemia (see Palpation in the Vascular Evaluation section for a description of vital sign changes with hypovolemia) Laboratory Studies In addition to the history and physical examination, the clinical diagnosis of hematologic disorders is based primarily on laboratory studies Complete Blood Cell Count.  The standard complete blood cell (CBC) count consists of a red blood cell (RBC) count, white blood cell (WBC) count, WBC differential, hematocrit (Hct) measurement, hemoglobin (Hgb) measurement, and platelet (Plt) count (Table 7-10) Figure 7-3 illustrates a common method used by the medical-surgical team to document portions of the CBC in progress notes If a value is abnormal, it is usually circled within this “sawhorse” figure Physical Therapy Considerations • The most important thing to consider when looking at hemoglobin values is the patient’s oxygen supply versus CHAPTER 7    Vascular System and Hematology 169 TABLE 7-9  Signs and Symptoms of Hematologic Disorders by Body System Body System Sign/Symptom Associated Condition Cardiac Tachycardia Palpitations Murmur Angina Dyspnea Orthopnea Back pain Bone pain Joint pain Sternal tenderness Headache Syncope Vertigo, tinnitus Paresthesia Confusion Visual disturbances Blindness Dysphagia Abdominal pain Splenomegaly or hepatomegaly Hematemesis, melena Hematuria Menorrhagia Petechiae Ecchymosis Flushing Jaundice Pallor Anemia, hypovolemia Anemia, hypovolemia Anemia, hypovolemia Anemia, hypovolemia Anemia, hypovolemia Anemia, hypovolemia Hemolysis Leukemia Hemophilia Leukemia, sickle cell disease Severe anemia, polycythemia, metastatic tumor Severe anemia, polycythemia Severe anemia Vitamin B12 anemia, malignancy Severe anemia, malignancy, infection Anemia, polycythemia Thrombocytopenia, anemia Iron-deficiency anemia Lymphoma, hemolysis, sickle cell disease Hemolytic anemia Thrombocytopenia and clotting disorders Hemolysis and clotting disorders Iron-deficiency anemia Iron-deficiency anemia Hemolytic, pernicious anemia Iron-deficiency anemia Hemolytic anemia Conditions with low hemoglobin Respiratory Musculoskeletal Nervous Visual Gastrointestinal, urinary, and reproductive Integumentary Data from Black JM, Matassarin-Jacobs E, editors: Medical-surgical nursing: clinical management for continuity of care, ed 5, Philadelphia, 1997, Saunders FIGURE 7-3  Illustration of portions of the complete blood cell count in shorthand format Hct, Hematocrit; Hgb, hemoglobin; Plt, platelet; WBC, white blood cell demand Decreased Hgb levels can reduce oxygen transport capacity and subsequently reduce the oxygen supply, which can reduce a patient’s endurance level • It is important to consider the trends in the Hgb and Hct levels If Hct/Hgb levels are low at baseline, these patients may be able to tolerate activity However, patients with acutely low levels of Hct/Hgb may or may not tolerate increased activity • A physical therapist should be aware of signs and symptoms of hypoxia to major organs: brain, heart, and kidneys • Monitoring of tolerance and modifications in the therapeutic plan may be indicated with low levels of Hct/Hgb.18 Hct is accurate in relation to fluid status; therefore Hct may be falsely high if the patient is dehydrated and falsely low if the patient is fluid overloaded.11 • Hct is approximately three times the Hgb value • A low Hct may cause the patient to experience weakness, dyspnea, chills, or decreased activity tolerance, or it may exacerbate angina • Patients with cancer such as leukemia or patients who are receiving cancer treatment will most likely present with lower Hct and Hgb values; therefore the therapist should proceed with caution in these patients • The term pancytopenia refers to a significant decrease in RBCs, all types of WBCs, and platelets • The term neutropenia refers to an abnormal decrease in WBCs, particularly neutrophils • The term leukocytosis refers to an abnormal increase in circulating WBCs • The term thrombocytopenia refers to a significant decrease in platelets • The term thrombocytosis refers to an abnormal increase in platelets Erythrocyte Indices.  RBC, Hct, and Hgb values are used to calculate three erythrocyte indices: (1) mean corpuscular volume (MCV), (2) mean corpuscular Hgb, and (3) mean corpuscular Hgb concentration (Table 7-11) At most institutions, these indices are included in the CBC Erythrocyte Sedimentation Rate.  The erythrocyte sedimentation rate (ESR), often referred to as the sed rate, is a 170 CHAPTER 7    Vascular System and Hematology TABLE 7-10  Complete Blood Cell Count: Values and Interpretation* Test Description Value Indication/Interpretation Red blood cell (RBC) count Number of RBCs per µl of blood Female: 4.2-5.4 million/µl Male: 4.7-6.1 million/µl White blood cell (WBC) count Number of WBCs per àl of blood 5-10 ì 103 (5000-10,000) WBC differential Proportion (%) of the different types of WBCs (out of 100 cells) Hematocrit (Hct) Percentage of RBCs in whole blood Neutrophils 55%-70% Lymphocytes 20%-40% Monocytes 2%-8% Eosinophils 1%-4% Basophils 0.5%-1% Female: 37%-47% Male: 42%-52% Blood loss, anemia, polycythemia Elevated RBC count may increase risk of venous stasis or thrombi formation Increased: polycythemia vera, dehydration, severe chronic obstructive pulmonary disease, acute poisoning Decreased: anemia, leukemia, fluid overload, recent hemorrhage Presence of infection, inflammation, allergens, bone marrow integrity Monitors response to radiation or chemotherapy Increased: leukemia, infection, tissue necrosis Decreased: bone marrow suppression Presence of infectious states Detect and classify leukemia Hemoglobin (Hgb) Amount of hemoglobin in 100 ml of blood Female: 12-16 g/100 ml Male: 14-18 g/100 ml Platelets (Plt) Number of platelets in àl of blood 150-450 ì 109 150,000-450,000àl Blood loss and fluid balance Increased: polycythemia, dehydration Decreased: anemia, acute blood loss, hemodilution Blood loss, bone marrow suppression Increased: polycythemia, dehydration Decreased: anemia, recent hemorrhage, fluid overload Thrombocytopenia Increased: polycythemia vera, splenectomy, malignancy Decreased: anemia, hemolysis, DIC, ITP, viral infections, AIDS, splenomegaly, with radiation or chemotherapy Data from Elin RJ: Laboratory reference intervals and values In Goldman L, Bennett JC, editors: Cecil textbook of medicine, vol 2, ed 21, Philadelphia, 2000, Saunders, p 2305; Matassarin-Jacobs E: Assessment of clients with hematologic disorders In Black JM, Matassarin-Jacobs E, editors: Medical-surgical nursing: clinical management for continuity of care, ed 5, Philadelphia, 1997, Saunders, p 1465; Mosby’s diagnostic and laboratory test reference, ed 8, St Louis, 2007, Mosby AIDS, Acquired immunodeficiency syndrome; DIC, disseminated intravascular coagulation; ITP, idiopathic thrombocytopenic purpura *Lab values vary among laboratories RBC, hemoglobin, and platelet values vary with age and gender TABLE 7-11  Erythrocyte Indices: Values and Interpretation* Test Description Value Interpretation Mean corpuscular volume (MCV) (Hct × 10/RBC) Mean size of RBCs in a sample of blood 80-100 µm Mean corpuscular hemoglobin (MCH) (Hgb × 10/RBC) Amount of Hgb in one RBC 26-34 pg/cell Mean corpuscular hemoglobin concentration (MCHC) (Hgb/Hct × 100) Proportion of each RBC occupied by Hgb 31-37 g/dl Increased by macrocytic, folic acid, or vitamin B12 deficiency anemias; liver disease; and recent alcohol use Decreased by microcytic, iron-deficiency, and hypochromic anemias; thalassemia; and lead poisoning Increased by macrocytic anemia Decreased by microcytic anemia Low mean corpuscular hemoglobin indicates Hgb deficiency Increased by spherocytosis (small round RBC) Decreased by microcytic, hypochromic, and iron-deficiency anemias and thalassemia Data from Elin RJ: Laboratory reference intervals and values In Goldman L, Bennett JC, editors: Cecil textbook of medicine, vol 2, ed 21, Philadelphia, 2000, Saunders, p 2305; Matassarin-Jacobs E: Assessment of clients with hematologic disorders In Black JM, Matassarin-Jacobs E, editors: Medical-surgical nursing: clinical management for continuity of care, ed 5, Philadelphia, 1997, Saunders, p 1466; and Pagana KD, Pagana TJ: Mosby’s diagnostic and laboratory test reference, ed 10, St Louis, 2011, Mosby, pp 830-833 Hct, Hematocrit; Hgb, hemoglobin; RBC, red blood cell *Lab values vary among laboratories CHAPTER 7    Vascular System and Hematology • • • • Fever and chills Decreased urine output Jaundice Abdominal or back pain and splenomegaly (intravascular only) Management of hemolytic anemia may include any of the following: investigation and removal of the causative factor, fluid replacement, blood transfusion, corticosteroids, or splenectomy.14,84,86 Sickle Cell Anemia.  Sickle cell anemia is an autosomal homozygous (hemoglobin SS or HgSS) recessive trait characterized by RBCs that become sickle (crescent)-shaped when deoxygenated Over time, cells become rigid and occlude blood vessels, thus causing tissue ischemia and infarction The risk of cerebrovascular accident and infarction of other organs or muscles is high Symptoms and physical findings of sickle cell anemia may include: • Jaundice, nocturia, hematuria, pyelonephritis, renal failure, splenohepatomegaly • Retinopathy or blindness • Chronic nonhealing ulcers of the lower extremity • Systolic murmur and an enlarged heart • Paresthesias • Acute painful episodes that affect long bones and joints, with the low back being the most frequent site of pain Other parts of the body affected are the scalp, face, jaw, abdomen, and pelvis The pain is usually nociceptive (secondary to tissue damage) and is sharp and throbbing in nature.97 A complication of sickle cell anemia that may require hospitalization is pain crisis, intense pain in any major organ or body area Pain crisis is usually treated with parenteral opioids.97 Lasting from to days to weeks, pain crisis can be precipitated by infection, dehydration, hypoxia, sleep apnea, exposure to cold, or menstruation, or it may be of unknown etiology.98   CLINICAL TIP Tachycardia may be the first change observed when monitoring vital signs during sickle cell crisis, usually accompanied by a sense of fatigue, generalized weakness, loss of stamina, and exertional dyspnea.24 Acute chest syndrome (ACS) is a situation that requires hospital admission The patient presents with chest pain, dyspnea, hypoxemia, and infiltrates on chest x-ray, perhaps with pleural effusion.99 ACS may be caused by intrapulmonary sickling, sickle cell emboli, or bone marrow or fat embolism, or by infection.99 The ACS is currently defined as a new infiltrate on chest radiograph associated with one or more symptoms, such as fever, cough, sputum production, tachypnea, dyspnea, or new-onset hypoxia.100 ACS is the leading cause of death in adolescents and adults with sickle cell disease.16 Management of sickle cell anemia may include the prevention or supportive treatment of symptoms with rest; hydration; analgesia; supplemental oxygen; incentive spirometry; use of corticosteroids or cytotoxic agents, such as hydroxyurea62; 185 partial RBC exchange; and psychosocial support.14,84,86,94,100 The average life expectancy of a patient with sickle cell anemia is 40 to 50 years.90 Note that sickle cell anemia is differentiated from sickle cell trait A patient with sickle cell trait has a heterozygous trait of Hgb that is asymptomatic for the signs and symptoms of anemia, although RBCs may sickle under the conditions of high altitude, strenuous exercise, or anesthesia.94 No treatment is usually necessary; however, genetic counseling is a reasonable strategy.39   CLINICAL TIP The use of oximetry can help the physical therapist and patient monitor RBC oxygenation and gauge exercise intensity Anemia of Chronic Diseases.  The term anemia of chronic disease (ACD) designates an anemia syndrome typically found in patients with chronic infections, inflammatory disorders, or neoplastic disorders ACD occurs in approximately 50% of hospitalized patients, as identified by laboratory studies ACD has also been observed in acute trauma and critical care patients The clinical features are those of the causative condition The diagnosis should be suspected in patients with chronic diseases and is confirmed by low serum level, low total iron-binding capacity, and normal or increased serum ferritin In most cases, no treatment is necessary Purified recombinant erythropoietin is effective for treatment of anemia of renal failure or anemia related to cancer and inflammatory conditions.87 Polycythemia.  Polycythemia is a chronic disorder characterized by excessive production of RBCs, platelets, and myelocytes As these increase, blood volume, blood viscosity, and Hgb concentration increase, causing excessive workload for the heart and congestion of some organs.16 The three types of polycythemia are primary polycythemia (polycythemia vera), secondary polycythemia, and relative polycythemia Primary polycythemia, or polycythemia vera, is an acquired myeloproliferative disorder39 that causes an increase in the number of RBCs, WBCs, and platelets.101 The origin of this disease is unknown; however, there is an autonomous overproduction of erythroid stem cells from bone marrow leading to increased blood viscosity and expanded blood volume Thus there is a risk for thrombus formation and bleeding.90 Primary polycythemia may convert to chronic myelogenous leukemia or myelofibrosis The hallmark of polycythemia vera is a hematocrit above normal, at times greater than 60%.39 Secondary polycythemia is the overproduction of RBCs owing to a high level of erythropoietin The increased erythropoietin level is a result of either altered stem cells (which automatically produce erythropoietin or erythropoietinsecreting tumors, such as hepatoma or cerebellar hemangioblastoma)89 or chronic low oxygenation of tissues, in which the body attempts to compensate for hypoxia The latter is common in individuals with chronic obstructive pulmonary disease, cardiopulmonary disease, or exposure to high altitudes 186 CHAPTER 7    Vascular System and Hematology Relative polycythemia is the temporary increase in RBCs secondary to decreased fluid volume (dehydration), as with excessive vomiting, diarrhea, or excessive diuretic use, or after a burn injury Signs and symptoms of polycythemia may include42: • Headache, dizziness, blurred vision, and vertigo, all a result of hypervolemia • Venous thrombosis, a result of hyperviscosity • Bleeding from the nose, gastrointestinal bleeding, and spontaneous bruising, all a result of platelet dysfunction • Fatigue • Paresthesia in the hands and feet • Splenomegaly (polycythemia vera only) Management of polycythemia includes phlebotomy as the main therapy Blood is withdrawn from the vein to decrease blood volume and decrease hematocrit to 45% Every to days, 250 to 500 ml of blood is removed, depending on the age of the patient, with a goal for the hematocrit to be below 42% for females and 45% for males.16,56 Other treatments used in polycythemia are myelosuppressive therapy (hydroxyurea); interferon30; antiplatelet therapy (aspirin); radiophosphorus (for primary polycythemia); smoking cessation; and fluid resuscitation (for relative polycythemia) Neutropenia.  Neutropenia is defined as an absolute neutrophil count (ANC) of less than 1500/µl and is calculated from the WBC differential: ANC = WBC (cells/àl) ì percent (polymorphonuclear neutrophils + bands) divided by 100.102 A variety of bone marrow disorders (e.g., leukemia) and nonmarrow conditions (e.g., immunologic peripheral destruction, sepsis, or hypersplenism) or chemotherapy may cause neutropenia Neutropenia is considered mild (ANC between 1000 and 1500/µl), moderate (ANC between 500 and 1000/µl), or severe (ANC less than 500/µl).103 The risk of infection, typically bacterial, is related to the severity of the neutropenia, with the risk of infection increasing at 1000/µl.103 Usual signs of inflammatory responses to infection may be absent in a neutropenic patient; however, fever in a neutropenic patient should always be assumed to be of infectious origin Refer to Chapter 14 for more information on neutropenia Medical therapy consists of symptomatic management of fever, discontinuation of causative drugs, broad-spectrum antibiotics or antifungal agents to treat infection, good dental hygiene, the administration of myeloid growth factor, and hematopoietic cell transplantation.39,103 Thrombocytic Disorders Disseminated Intravascular Coagulation.  DIC involves the introduction of thromboplastic substances into the circulation that initiate a massive clotting cascade accompanied by fibrin, plasmin, and platelet activation It is a complex and paradoxic disorder characterized by both hemorrhage and thrombus formation (Table 7-19) First, fibrin is deposited in the microcirculation, leading to organ ischemia and the destruction of RBCs as they pass through these deposits Second, platelets and clotting factors are consumed and hemorrhage occurs Plasmin is activated to further decrease clotting factor, and TABLE 7-19  Common Signs and Symptoms of Disseminated Intravascular Coagulation System Related to Hemorrhage Related to Thrombi Peripheral cyanosis, Bleeding from gangrene gums, venipunctures, and old surgical sites; epistaxis; ecchymoses Cardiopulmonary Hemoptysis Dysrhythmias, chest pain, acute myocardial infarction, pulmonary embolus, respiratory failure Renal Hematuria Oliguria, acute tubular necrosis, renal failure Gastrointestinal Abdominal Diarrhea, constipation, distention, bowel infarct hemorrhage Neurologic Subarachnoid Altered level of hemorrhage consciousness, cerebral vascular accident Integumentary From Urden LD, Stacy KM, Lough ME: Thelan’s critical care nursing: diagnosis and management, ed 5, St Louis, 2006, Mosby fibrin further inhibits platelet function, which further increases bleeding DIC, either acute or chronic, is always a secondary process mediated by inflammatory cytokines.104 DIC may be mild and self-limiting or may be severe, and it is often associated with critical illness.105 The onset of acute DIC usually occurs in the presence of illness within hours or days of the initial injury or event This condition is associated with severe infection and gram-negative sepsis Other causes of DIC include trauma, burn injury, shock, tissue acidosis, antigen-antibody complexes, or the entrance of amniotic fluid or placenta into the maternal circulation.89 Organ failure is common.6 Chronic DIC is associated with hemangioma and other cancers (particularly pancreatic or prostate cancer), systemic lupus erythematosus, or missed abortion DIC is a life-threatening condition with high mortality and requires immediate medical attention.6 The diagnostic workup for DIC includes platelet count (thrombocytopenia), PT and activated partial thromboplastin time (aPTT) (prolonged), INR (elevated), fibrinogen level (decreased), and presence of D-dimer.106 The D-dimer has high sensitivity and specificity for diagnosing DIC.6   CLINICAL TIP Inflate the blood pressure cuff only as high as needed to obtain a reading Frequent blood pressure readings may cause bleeding under the cuff, particularly for patients with thrombocytic disorders, so rotate arm use to reduce repeated trauma CHAPTER 7    Vascular System and Hematology Management of DIC may include treatment of the causative condition; hemodynamic and cardiovascular support, which includes fluid management, oxygen supplementation, and invasive monitoring; blood and blood product transfusion for active bleeding; heparin therapy (this is controversial); and recombinant protein factor therapy (experimental).16,104 Hemophilia.  Hemophilia is a disease characterized by excessive spontaneous hemorrhaging at mucous membranes, into joint spaces (hemarthrosis) and muscles, or intracranially It is the result of a genetic deficiency of a clotting factor There are four basic types107: Hemophilia A is characterized by the lack of factor VIII and is inherited as an X-linked recessive trait Hemophilia B (Christmas disease) is characterized by the lack of factor IX and is inherited as an X-linked recessive trait Hemophilia C is characterized by the lack of factor XI and is inherited as an autosomal recessive trait von Willebrand’s disease is characterized by the lack of factor VIII and is inherited as an autosomal dominant trait Patients with mild hemophilia experience bleeding only with trauma or after surgical procedures, whereas patients with severe hemophilia may bleed with minor trauma or spontaneously.90 Hemophilia A, when severe, is characterized by excessive bleeding into various organs of the body Soft-tissue hematomas and hemarthroses leading to severe, crippling hemarthropathy are highly characteristic of the disease Muscle hemorrhages can be more insidious and massive than joint bleeding and most often involve the flexor muscle groups.24 Bleeding into joints accounts for approximately 75% of bleeding episodes in severely affected patients with hemophilia A.108 The knee is the most frequently affected joint, followed by the ankle, elbow, hip, shoulder, and wrist Joints with at least four bleeds in months are called target joints When the blood is introduced into the joint, the joint becomes distended, causing swelling, pain, warmth and stiffness.24 In seriously affected patients, major hemorrhages may dissect through tissue planes, ultimately leading to compromise of vital organs However, bleeding episodes are intermittent, and some patients not hemorrhage for weeks or months.109 Symptoms and physical findings of bleeding episode from hemophilia may include: • Petechiae, purpura, and ecchymosis • Hematoma (Intramuscular hematomas are common in hemophilia A, bleeding into the psoas muscle is common,   CLINICAL TIP Patients with psoas hematoma will present with severe pain, usually around the buttocks, and their primary complaint is usually the inability to get comfortable in any position A large iliopsoas hemorrhage can cause displacement of the kidney and ureter and can compress the neurovascular bundle Iliopsoas bleeds are considered a medical emergency requiring immediate physician referral.24 187 FIGURE 7-7  Acute hemarthrosis of the knee is a common complication of hemophilia (From Forbes CD, Jackson WF: Color atlas and text of clinical medicine, ed 3, London, 2003, Mosby.) and femoral nerve involvement may be partial or complete.110) • Disorientation • Convulsions • Tachycardia, tachypnea, and hypotension • Intracranial bleeding is the major cause of death for all age groups of hemophiliacs.6 • Pain Patients with acute hemarthrosis have pain, with objective findings of warmth, a tense effusion, tenderness, and limitation of motion, and the joint is usually held in flexion Acute hemarthrosis of the knee is a common complication of hemophilia (Figure 7-7) It may be confused with acute infection unless the patient’s coagulation disorder is known Patients with subacute or chronic arthritis (related to hemophilia) have chronically swollen joints, which are usually painless and slightly warm to touch.111 Repeated hemarthrosis causes joint deformity and radiologic changes Management of hemophilia may include any of the following: methods to stop active bleeding (e.g., direct pressure), supportive therapy depending on the location of the bleed (e.g., joint debridement), factor replacement therapy, and pain management.94   CLINICAL TIP Watch for signs of joint effusion (warmth and edema) in patients with hemophilia who are prone to hemarthrosis, especially during weight-bearing activities Thalassemia.  Thalassemia is an autosomal-recessive disease characterized by abnormal formation of Hgb chains in RBCs, resulting in RBC membrane damage and abnormal erythropoiesis and hemolysis Hgb is composed of two alpha and two beta chains α-Thalassemia is a defect in alpha-chain synthesis in which one (alpha trait), two (α-thalassemia minor), or three (Hgb H disease) genes are altered Each type of α-thalassemia varies in presentation from a lack of symptoms (alpha trait and minor) to chronic severe hemolytic anemia (Hgb H).89 β-Thalassemia minor is a defect in beta-chain synthesis in one of two beta 188 CHAPTER 7    Vascular System and Hematology chains and is usually asymptomatic β-Thalassemia major is a severe reduction or absence in beta-chain production that results in severe anemia, growth failure, bony deformities, hepatosplenomegaly, and jaundice with a life expectancy of 20 to 30 years from complications of heart failure, cirrhosis, and endocrinopathy.90 Patients with thalassemia are classified as having thalassemia minor, thalassemia intermedia, or thalassemia major, depending on the severity of their anemia Patients with thalassemia minor generally have little or no hematologic disease Patients with thalassemia intermedia may require occasional transfusions Patients with thalassemia major require lifelong chronic RBC transfusion to maintain adequate hemoglobin levels.58 Management of the thalassemia may include folate supplementation, blood transfusion, iron-chelating agents, and splenectomy.90 Thrombocytopenia.  Thrombocytopenia is an acute or chronic decrease in the number of platelets (less than 150,000/µl) in the circulation It can result from decreased platelet pro­ duction (caused by infection, drug or immune responses, or blood vessel damage), increased platelet destruction (caused by malignancy, antiplatelet antibodies, or the use of myelosuppressive drugs), or altered platelet distribution (caused by cardiac surgery–induced hypothermia, portal hypertension, or splenomegaly).107 Signs and symptoms of thrombocytopenia may include112: • Bleeding of nose, gums, or puncture sites or blood in emesis, urine, or stool • Ecchymosis and petechiae • Tachycardia and tachypnea • Signs of increased intracranial pressure if a cranial bleed is present • Renal failure • Splenomegaly Management of thrombocytopenia may include treatment of the causative factor; immunosuppressive therapy; anticoagulants in plasma transfusion or plasmapheresis; corticosteroids; or splenectomy.107 Physical Therapy Considerations.  A physical therapist should implement fall precautions for the patient who is thrombocytopenic during all physical therapy interventions; patients with thrombocytopenia should be educated about the fall risk precautions and the potential for bleeding Blood pressure cuffs and similar devices must be used with caution Elastic support stockings must be thigh high, never knee high Mechanical compression with a pneumatic pump and soft-tissue mobilization are avoided unless approved by a physician.24 Heparin-Induced Thrombocytopenia Heparin-induced throm­bocytopenia (HIT) is the most common type of druginduced thrombocytopenia and one of the most common causes of thrombocytopenia in hospitalized patients.39 Heparin can have a dramatic thrombocytopenic effect, usually to 10 days after the initiation of heparin therapy The clinical presentation of HIT is distinct and may include113: • Large, bilateral lower-extremity DVT • Upper-extremity DVT at a venous catheter site • • • • Skin lesions at the injection site Aortic or ileofemoral thrombus with limb ischemia Pulmonary embolism An acute systemic reaction to heparin HIT may be type I (the asymptomatic aggregation of platelets) or type II (an immune response resulting in platelet activation and venous or arterial thrombi).114 Type I is more common than type II, and type II is associated with greater risk of lifethreatening thrombosis.115 HIT is diagnosed by clinical presentation, a platelet count less than 100 × 109/L, and a positive platelet aggregation test Heparin-PF antibody testing is recommended for patients suspected to have HIT.116 Management of HIT starts with the immediate discontinuation of heparin and initiation of fast-acting, nonheparin alternative anticoagulation The direct thrombin inhibitors argatroban, lepirudin, and bivalirudin are nonheparin anticoagulants that inhibit thrombin without interaction with heparin-PF4 antibodies.116 Management of HIT also includes plasmapheresis and immunoglobulin therapy, in addition to supportive therapies for alteration in skin integrity and pain.114 Thrombotic Thrombocytopenic Purpura.  Thrombotic thrombocytopenic purpura (TTP) is the rapid accumulation of thrombi in small blood vessels TTP is primarily seen in young adults 20 to 50 years of age.90 The etiology of TTP is unknown; however, it is associated with bacterial or viral infections, estrogen use, certain drug use, pregnancy, and autoimmune disorders such as acquired immunodeficiency syndrome.90 The diagnosis of TTP is made by thrombocytopenia, anemia, and elevated serum lactate dehydrogenase The coagulation tests are normal.39 Signs and symptoms of TTP may include: • Hemolytic anemia, thrombocytopenia • Fatigue and weakness • Fever • Pallor, rash, petechiae • Waxing and waning headache, confusion, altered consciousness from lethargy to coma • Hemiparesis and seizures • Abdominal pain and tenderness due to pancreatitis • Acute renal failure Management of TTP may include emergent large-volume plasmapheresis; plasma exchange; antiplatelet agents; corticosteroids; immunosuppressive agents; or splenectomy if not refractory to initial therapy or if the condition recurs.90 Idiopathic Thrombocytopenic Purpura.  Idiopathic thrombocytopenic purpura (ITP) is an autoimmune disorder in which an IgG autoantibody is formed that binds to the platelets Platelets are not destroyed by direct lysis; rather, destruction takes place in the spleen without subsequent enlargement (splenomegaly) An enlarged spleen should lead one to doubt the diagnosis of ITP Patients are systemically well and usually not febrile The hallmark of the disease is thrombocytopenia The bone marrow will appear normal, and coagulation studies will be entirely normal The common symptoms are of bleeding and include epistaxis, oral bleeding, menorrhagia, purpura, and petechiae Most patients will require treatment, but some patients will CHAPTER 7    Vascular System and Hematology have spontaneous remissions Treatment is usually with corticosteroids, most commonly with prednisone and in some cases with immunoglobulins Splenectomy is the most definitive treatment for ITP, and most patients will ultimately undergo splenectomy Platelet transfusions are rarely used in the treatment of ITP because exogenous platelets will survive no better than the patient’s own and will usually last less than a few hours.39 Lymphatic Disorders Lymphedema Lymphedema is a chronic disorder characterized by an abnormal collection of lymph fluid in the tissues of one or more body regions The most common cause for the accumulation of the fluid is a mechanical insufficiency of the lymphatic system Lymphedema can be classified as primary or secondary Primary (idiopathic) lymphedema is caused by a condition that is congenital or hereditary In this case, the lymph node or the lymph vessel formation is abnormal Secondary (acquired) lymphedema is caused by injury to one or more components of the lymphatic system in some other manner, when some of the lymphatic system is blocked, dissected, fibrosed, or damaged Lymphedema can develop in any part of the body or limb(s) The severity of lymphedema is graded using the scale from the International Society of Lymphology, as described in Table 7-20 Signs and symptoms of lymphedema include: • Swelling distal to or adjacent to the area where lymph system function has been impaired • Symptoms usually not relieved by elevation TABLE 7-20  Stages of Lymphedema Stage (latent lymphedema) Stage Stage Stage (lymphostatic elephantiasis) Lymph transport capacity is reduced; no clinical edema is present Accumulation of protein-rich pitting edema Reversible with elevation; area affected may be normal size on waking in the morning Increases with activity, heat, and humidity Accumulation of protein-rich nonpitting edema with connective scar tissue Irreversible; does not resolve overnight; increasingly more difficult to pit Clinical fibrosis is present Skin changes present in severe stage Accumulation of protein-rich edema with significant increase in connective tissue and scar tissue Severe nonpitting fibrotic edema Atrophic changes (hardening of dermal tissue, skin folds, skin papillomas, and hyperkeratosis) Data from Goodman CC: The hematologic system In Goodman CC, Boisonnault WG, editors: Pathology: implications for the physical therapist, ed 3, Philadelphia, 2009, Saunders 189 • Pitting edema in early stages and nonpitting edema in later stages, when fibrotic changes occur • Fatigue and heaviness, pressure, tightness, tingling, and numbness in the affected region—causing tremendous discomfort • Fibrotic changes in the skin • Increased circumferential limb girth • Loss of range of motion Diagnosis of lymphedema can be made without the use of special diagnostic tests It is beyond the scope of this text to discuss the diagnosis of lymphedema in detail However, when evaluating a patient with suspected lymphedema, cardiac, renal, thyroid, and arteriovenous disease must be ruled out medically Management includes manual lymphatic drainage and lymphedema bandaging Early intervention is of significance The physical therapist should have special training to treat this patient population Manual lymphatic drainage is a specialized manual therapy technique that affects primarily superficial lymphatic circulation Manual lymphatic drainage improves lymph transport capacity, redirects lymph flow toward collateral vessels, and mobilizes excess lymph fluid   CLINICAL TIP Patients, particularly those with primary lymphedema of the lower extremities, should be evaluated for abdominal and genital edema before undergoing any treatment to reduce the extremity lymphedema to avoid the complication of moving more fluid to an already overloaded abdominal area.24 Lymphedema bandaging is a highly specialized form of bandaging that uses multiple layers of unique padding materials and short stretch bandages to create a supportive structure for edematous and lymphedematous body segments It is commonly used in between the manual lymphatic drainage treatments.3 Management The management of vascular disorders includes pharmacologic therapy and vascular surgical procedures Hematologic disorders may be managed with pharmacologic therapy, as well as with nutritional therapy and blood product transfusion Pharmacologic Therapy Common drug classifications for the management of vascular and hematologic disorders include: • Anticoagulants (see Chapter 19, Table 19-2) • Antiplatelet agents (see Chapter 19, Table 19-4) • Thrombolytic agents (see Chapter 19, Table 19-7) • Colony-stimulating factors (Chapter 19, Table 19-24) Anticoagulation Therapy The standard INR goal for anticoagulation therapy with warfarin (Coumadin) is 1.5 to 2.5 times a control value and is 190 CHAPTER 7    Vascular System and Hematology TABLE 7-21  Therapeutic Values for International Normalized Ratio (INR) INR 2.0-3.0 INR 2.5-3.5 Prophylaxis of venous thrombosis (high-risk surgery) Treatment of venous thrombosis and pulmonary embolism Prevention of systemic embolism Tissue heart valves Valvular heart disease Atrial fibrillation Recurrent systemic embolism Cardiomyopathy Acute myocardial infarction Mechanical prosthetic heart valve replacement Data from Goldman L, Ausiello D: Cecil textbook of medicine, ed 24, St Louis, 2012, Saunders; Gibbar-Clements T, Shirrell D, Dooley R et al: The challenge of warfarin therapy, Am J Nurs 100:38-40, 2000 categorized by condition or clinical state117 according to the values in Table 7-21 Physical Therapy Considerations The physical therapist should understand some basic concepts of anticoagulation therapy to intervene safely and estimate length of stay • The physician will determine the PT/INR and PTT goal for each patient This goal is documented in the medical record The patient remains in a hospital setting until the goal is reached • The therapeutic effect of heparin or LMWH is reached within minutes or hours, whereas the effect of warfarin is reached in to days; thus heparin or LMWH is usually prescribed before warfarin The dose of heparin or LMWH is increased or decreased depending on the PTT goal, then the patient is transitioned to warfarin as indicated • The terms subtherapeutic and supertherapeutic imply a coagulation level below or above the anticoagulation goal, respectively • A subtherapeutic PT/INR or PTT indicates a risk for thrombus formation, whereas a supertherapeutic level indicates a risk for hemorrhage • Supertherapeutic anticoagulation is rapidly reversed by vitamin K or fresh-frozen plasma • Anticoagulant agents are temporarily discontinued before surgery to minimize bleeding intraoperatively or postoperatively • The physical therapist should always monitor the patient who is taking anticoagulants for signs and symptoms of bleeding, as bleeding can occur even if the PT/INR is therapeutic.117 Blood Product Transfusion Blood and blood products are transfused to replenish blood volume, maintain oxygen delivery to tissues, or maintain proper coagulation.118 The need for blood transfusion should be dependent on the patient’s symptoms It has been reported that refrigerated, banked blood loses its ability to deliver oxygen in a short period of time.119 Table 7-22 lists the most common transfusion products and the rationale for their use Blood may be autologous (patient donates own blood) or homologous (from a volunteer donor) Before transfusing blood or blood products, the substance to be given must be typed and crossed This process ensures that the correct type of blood is given to a patient to avoid adverse reactions A variety of transfusion reactions can occur during or after the administration of blood products (Table 7-23) These blood transfusion reactions are usually not fatal, but they can extend a patient’s length of stay Blood transfusions are a form of liquid tissue transplant and it can cause significant immunosuppression This effect is known as TRIM (transfusion-related immunomodulation), and it happens every time a patient receives blood Transfusion reactions can be nonimmunologic and are caused by the physical and chemical properties of the transfused blood.120 There is a threat of transmitting infectious diseases via a blood transfusion.119 In addition to these reactions, complications of blood transfusion include air embolism (if the blood is pumped into the patient) or circulatory overload (from a rapid increase in volume) Circulatory overload occurs when the rate of blood (fluid) transfusion is greater than the circulation can accommodate Signs and symptoms include tachycardia, cough, dyspnea, crackles, headache, hypertension, and distended neck veins To prevent circulatory overload during a transfusion, intravenous fluids may be stopped, or a diuretic (e.g., furosemide [Lasix]) may be given Delayed adverse transfusion reactions include iron overload, graft-versus-host disease, hepatitis, human immunodeficiency virus infection, or delayed hemolytic reaction (approximately to 14 days posttransfusion).121 Physical Therapy Considerations • If the patient is receiving blood products, the physical therapist should observe the patient for signs or symptoms, or both, of transfusion reaction before initiating physical therapy intervention • Depending on the medical status of the patient, the therapist may defer out-of-bed or vigorous activities until the transfusion is complete On average, the transfusion of a unit of blood takes to hours • Defer physical therapy intervention during the first 15 minutes of a blood transfusion as most blood transfusion reactions occur within this time frame • During a blood transfusion, vital signs are usually taken every 15 to 30 minutes by the nurse and posted at the bedside • After a blood transfusion, it takes 12 to 24 hours for the Hgb and Hct to increase.50 Vascular Surgical Procedures Surgical management of coronary vascular disorders, such as angioplasty, arthrectomy, and stent placement, is described in Chapter under Percutaneous Revascularization Procedures These same techniques are also used in peripheral arteries rather than coronary arteries This section therefore concentrates on embolization therapy, transcatheter thrombolysis/thrombectomy, CHAPTER 7    Vascular System and Hematology 191 TABLE 7-22  Common Blood Products and Their Clinical Indications and Outcomes Product Content Clinical Indications Outcome Whole blood Blood cells and plasma Resolution of signs and symptoms of hypovolemic shock or anemia Hct should increase 3% in a nonbleeding adult (per unit transfused) Red blood cells (RBCs) RBCs only Platelets (Plts) Concentrated platelets in plasma Fresh-frozen plasma (FFP) All plasma components, namely blood factors and protein Albumin Albumin cells with few globulins and other proteins Plasma protein fraction (PPF) Cryoprecipitate Albumin, globulins, and plasma proteins Factors VIII and XIII, von Willebrand’s factor, and fibrinogen in plasma Acute major blood loss in setting of hypotension, tachycardia, tachypnea, pallor, and decreased Hct and Hgb To treat oxygen-carrying deficit (RBC) and volume expansion (plasma) When more than 10 units of blood are required in a 24-hour period Whole blood is rarely used Acute or chronic blood loss To treat oxygen-carrying deficit in setting of tachycardia, tachypnea, pallor, fatigue, and decreased Hct and Hgb Anemia without need for volume expansion To restore clotting function associated with or after blood loss To increase platelet count in a bleeding patient with platelet