Practice nursing care for Clients
with Disorders of Hemostasis
Hemostasis is derived from a Greek word, which means stoppage of blood flow. The process is a combination of cellular and biochemical events that function together to keep blood in the liquid state within the veins and arteries and prevent blood loss following injury through the formation of a blood clot. It consists of a complex regulated system which is dependent on a delicate balance among several systems. The systems involved in the hemostatic process include the vascular system, coagulation system, fibrinolytic system, platelets, kinin system, serine protease inhibitors, and the complement system.
The systems work together when the blood vessel endothelial lining is disrupted by mechanical trauma, physical agents, or chemical trauma to produce clots. The clots stop bleeding and are eventually dissolved through the fibrinolytic process. As a result, there is a delicate balance between the production and dissolution of clot during the hemostatic process. A disruption of this balance may precipitate thrombosis or hemorrhage as a result of hypercoagulation or hypocoagulation, respectively.
Hemostasis is categorized as either a primary or secondary process. Primary hemostasis involves the response of the vascular system and platelets to vessel injury. It takes place when there are injuries to small vessels during which the affected vessels contract to seal off the wound and platelets are mobilized, aggregate, and adhere to components of the subendothelium of the vasculature.
Platelet adhesion requires the presence of various factors such as von Willebrand factor (vWF) and platelet receptors (IIb/IIIa and Ib/IX). Additional platelets are attracted to the site of injury by the release of platelet granular contents, such as adenosine diphosphate (ADP). The platelet plug is stabilized by interaction with fibrinogen. Thus a defect in platelet function or von Willebrand’s disease (vWD) may result in debilitating and sometimes fatal hemorrhage.
Secondary hemostasis involves the response of the coagulation system to vessel injury. It is required to control bleeding from large wounds and is a continuation of the primary hemostatic mechanisms.
Whereas the outcome of primary hemostasis is the formation of the platelet plug, the outcome of secondary hemostasis is the formation of a thrombus.
Hemostasis is one of the most signifi cant maintenance systems of human bodily homeostasis; hemostasis plays two roles in the organism, namely:
1. to provide blood fl ow through blood vessels, i.e., to maintain the liquid state of circulating blood and
2. to prevent bleeding that results from blood vessel damage.
Hemostasis is a complex system that includes the participation of several factors:
– blood vessel endothelium
– platelets
– blood coagulation
– fibrinolytic process
– coagulation inhibitors.
The basic function of normal hemostasis is to prevent blood loss from undamaged blood vessels and to inhibit massive bleeding from damaged blood vessels. Blood loss from undamaged blood vessels is prevented by the normal blood vessel structure and normal platelet function. Bleeding after injury is stopped in three stages:
1. vascular stage
2. platelet stage
3. blood coagulation.
Coagulation Cascade
to stabilize and reinforce the weak platelet plug
fibrinogen → fibrin
3 main steps:
1. formation of prothrombin activator
2. conversion of prothrombin into thrombin
3. conversion of fibrinogen to fibrin
Platelets
Platelets are anuclear fragments derived from the bone marrow megakaryocytes. They have a complex internal structure, which reflects their hemostatic functions. The 2 major intracellular granules present in the platelets are the α-granules and the dense bodies. The α-granules contain platelet thrombospondin, fibrinogen, fibronectin, platelet factor 4, vWF, platelet derived growth factor, β-thromboglobulin, and coagulation factors V and VIII. The dense granules contain ADP, adenosine triphosphate (ATP), and serotonin. When stimulated, platelets release both the α-granules and the dense bodies through the open canalicular system. When platelets aggregate, they expend their stored energy sources, lose their membrane integrity, and form an unstructured mass called a syncytium. In addition to the plug formation, platelet aggregates release micro-platelet membrane particlesrich in phospholipids and various coagulation proteins which provide localized environment that support plasma coagulation.
Platelets and ECs have biochemical pathways involving the metabolism of arachidonic acid (AA), which is released from membrane phospholipids by phospholipase A2. Subsequently, cyclooxygenase converts AA to cyclic endoperoxides. The endoperoxides are then converted by thromboxane synthetase to thromboxane A2. Thromboxane A2 is a potent agonist that induces platelet aggregation. Endothelial cells also contain AA and preferentially convert cyclic endoperoxides to prostacyclin, which is a potent inhibitor of platelet aggregation.
During primary hemostasis, platelets interact with elements of the damaged vessel wall leading to the initial formation of the platelet plug. The platelet/injured vessel wall interaction involves a series of events that include platelet adhesion to components of the subendothelium, activation, shape change, release of platelet granules, formation of fibrin stabilized fibrin platelet aggregates, and clot retraction. In this process, the activation of platelets with exposure of negatively charged phospholipids facilitates the assembly of coagulation factors on the activated platelet membrane, leading to the generation of thrombin and subsequent fibrin deposition.
Platelet Function
In some disorders, platelets may be normal iumber, yet hemostatic plugs do not form normally, and therefore, bleeding time will be long. Platelet dysfunction may stem from an intrinsic platelet defect or from an extrinsic factor that alters the function of otherwise normal platelets. Defects may be hereditary or acquired. Tests of coagulation phase of hemostasis such as activated partial thromboplastin time (APTT) and prothrombin time (PT) are normal in most circumstances but not all.10 When a patient’s childhood history reveals easy bruising and bleeding after tooth extraction, tonsillectomy, or other surgical procedures, the finding of normal platelet count but a prolonged bleeding time suggests a hereditary disorder affecting platelet function. The cause is eithervWD (which is the most common cause of hereditary hemorrhagic disease) or a hereditary intrinsic platelet disorder.
Whatever the cause of platelet dysfunction, drugs that may further impair platelet function should be avoided such as aspirin and other non-steroidal antiinflammatory drugs (NSAIDs).
Laboratory Tests for Assessing Hemostasis
Disorders of the hematologic system can occur as a result of problems in the production, function, or normal destruction of any type of blood cell. The type and severity of the specific disorder determine the degree of threat to the client’s wellbeing.
BONE MARROW TRANSPLANTATION.
Once viewed as a treatment of last resort, bone marrow transplantation (BMT) is now considered a standard treatment for the client with leukemia. This treatment modality began more than 25 years ago (Johns, 1998). BMT is the treatment of choice for the client with leukemia who has a closely matched donor and who is experiencing temporary remission with induction therapy.
Because of the success of BMT in the client with leukemia, this therapy is now being used for lymphoma, aplastic anemia, inborn errors of metabolism, and many solid tumors (Wolf, 1999). The bone marrow is the actual site of production of leukemic cells. Because it can be difficult to ensure that all leukemic cells have been eradicated during induction therapy, the goal is for extremely high doses of chemotherapy to destroy all of the affected marrow.
The new, healthy marrow then begins the process of hematopoiesis, which results in normal, properly functioning cells and, it is hoped, a permanent cure. For many malignant disorders, the dose-limiting toxicity of treatments is bone marrow suppression.
The aim of bone marrow or stem cell transplantation is to rid the client of all leukemic or other malignant cells through high doses of chemotherapy, often in conjunction with whole-body irradiation. These treatments are lethal to the bone marrow, and without replacement of bone marrow function through transplantation of progenitor cells of the hematopoietic system, the client would die of infection or hemorrhage. Advances in the field of transplantation have been remarkable. Even as recently as the late 1980s, the client undergoing transplantation would have been seen only in major medical centers. Today, transplant units are becoming commonplace, even in community hospital settings. With long-term survival after transplantation increasing, nurses can expect to be caring for these people, if not during the actual transplantation or recovery period, then during the post-transplantation period, in a variety of health care settings.
SOURCES OF STEM CELLS.
BMT originated with the use of allogeneic bone marrow transplantation (transplantation of identical bone marrow from a sibling) and has advanced to the use of human leukocyte antigen (HLA)-matched stem cells from the umbilical cords of unrelated donors (Wolf, 1999). Transplants can be classified based on the source of stem cells.
In autologous transplants, the clients receive their own stem cells, which were collected before therapy.
Syngeneic transplants are rare and involve the client’s own identical twin as the donor of stem cells.
In allogeneic transplants, a closely HLA-matched sibling or an unrelated donor provides the stem cells. Stem cells for transplantation may be obtained by one of the following methods: bone marrow harvest, peripheral stem cell pheresis, or umbilical cord blood stem cell banking. Table 40-5 provides an overview of the types of transplants. Transplantation procedures have five phases: stem cell procurement, conditioning regimen, transplantation, engraftment, and post-transplantation recovery.
OBTAINING STEM CELLS.
Stem cells for transplantation are obtained either by harvest of bone marrow, by pheresis for peripheral blood stem cells, or by collection of umbilical cord stem cells. Bone marrow is harvested either from the client directly (autologous marrow) or from an HLA-matched person (allogeneic marrow).
For allogeneic marrow, a suitable donor is selected after family members are tested for HLA type. Preferred transplantations are those between HLA-identical siblings, but transplantation can also be successful between those with closely matched HLA types. The chance of matching with any given sibling is 25%. Several donor registries have been formed that keep records of people willing to donate marrow to provide marrow for clients who do not have a family member HLA match. The chance of matching with an unrelated donor is one in 5000 (Alcoser & Burchett, 1999).
After a suitable donor is identified by tissue typing, the donor is taken to the operating room, where sufficient marrow for transplant is harvested through multiple aspirations aspirated; this amount is approximately 5% to 10% of the donor’s marrow supply and will be replenished in a few weeks (Poliquin, 1997).
The marrow is then filtered and may be further processed to purge the autologous marrow of any residual cancer cells or to deplete the allogeneic marrow of T-cells, which may later cause graft-versus-host disease (GVHD) (see p. 855). Allogeneic marrow is transfused into the recipient immediately; autologous marrow is frozen for later use. The nurse monitors the donor for signs and symptoms of fluid loss, assesses for complications of anesthesia, and manages postoperative pain. During surgery, donors may lose a significant amount of fluid in addition to the volume of marrow donated. Donors are often hydrated with saline infusions before and immediately after surgery. Occasionally the donor may require an infusion of autologous salvaged red blood cells (RBCs) (Poliquin, 1997).
The nurse assesses the harvest sites to ensure that the dressings are dry and intact and that the donor is not bleeding excessively. Marrow donation is usually a same-day surgical procedure. At home the donors are taught to inspect the harvest sites for bleeding and to take analgesics for pain. Pain is often experienced at the harvest sites (hip) and is usually managed effectively with oral non-aspirin-containing analgesics. Individual differences do occur, however. Some donors refuse pain medication, but others require opioid analgesics.
There are three phases to obtaining peripheral blood stem cells (PBSCs): mobilization, collection, and reinfusion. During the mobilization phase, chemotherapy or hematopoietic growth factors are administered to the client (Wagner & Quinones, 1998). These agents cause stem cells to circulate in the peripheral blood and the number of WBCs to increase. The stem cells are then collected by pheresis (withdrawing whole blood, filtering out the cells, and returning the plasma to the client). One to five pheresis procedures, each lasting 2 to 4 hours, are usually required to obtain enough stem cells for PBSC transplantation.
The stem cells are then frozen and stored for reinfusion after the conditioning regimen (Wolf, 1999). The nurse must monitor the client closely during pheresis. Common complications include catheter clotting, which may delay pheresis, and hypocalcemia caused by anticoagulants (Poliquin, 1997). The client with hypocalcemia may experience chills, paresthesia, abdominal or muscle cramping, or chest pain, and the nurse may need to administer oral calcium supplements to manage these symptoms.
The nurse must also monitor vital signs frequently. The client may experience hypotension as a result of fluid volume changes during the procedure. Stem cells may also be obtained from umbilical cords. The first cord blood transplant was done in France in 1988 (Wolf, 1999). Umbilical stem cells are obtained via a simple phlebotomy procedure. After birth, before the placenta detaches, a syringe is used to withdraw 40 to 150 mL of blood from the umbilical vein. The syringes are placed in a kit, which is returned to the Cord Blood Registry for processing and storage (Wolf, 1999). The stem cells may be used later for an unrelated recipient or stored in case the infant develops a serious illness later in life and needs them. The cost of banking and processing umbilical cord stem cells is approximately $1500, with an additional charge of $100 per year for storage. Umbilical stem cells can last for years when stored properly in liquid nitrogen. The oldest viable sample is nearly 20 years old (Wolf, 1999).
CONDITIONING REGIMEN.
Figure 40-3 outlines the timing and steps typically involved in BMT. The day the client receives the bone marrow is considered day T-0.
Pretransplantation conditioning days are counted in reverse chronologic order from T-0, just like a rocket countdown. Post-transplantation days are counted in chronologic order from the day of transplantation. The client must first undergo a conditioning regimen, which varies with the diagnosis and type of transplant to be received. The conditioning regimen serves two purposes: (1) to obliterate, or “wipe out,” the client’s own bone marrow, thus preparing the client for optimal graft take, and (2) to give higher thaormal doses of chemotherapy and/or radiotherapy to obliterate, or wipe out, a malignancy, such as breast cancer. Usually a period of 5 to 10 days is required. The conditioning regimen always includes intensive chemotherapy and sometimes includes radiotherapy, usually total-body irradiation (TBI). Each conditioning regimen is individually tailored, with the client’s specific disease, overall health, and previous treatment taken into account (Poliquin, 1997). A typical conditioning regimen for an adult client receiving an allogenic BMT for treatment of acute myelogenous leukemia (AML) is as follows:
Days T-7 through T-5: High-dose chemotherapy to obliterate the client’s own bone marrow cells and to eradicate any remaining leukemic cells. Specific agents include busulfan, carmustine, cyclophosphamide, cytosine arabinoside, etoposide, and melphalan. The dosages are many times higher than those used for normal chemotherapy.
Days T-4 through T-2: Delivery of fractionated TBI (smaller doses of radiation given over a period of time instead of one larger dose). The typical radiation dose for TBI is 1200 rad. The client usually receives no cellkilling treatment on day T-l. During conditioning, bone marrow and normal tissues begin to respond immediately to the chemotherapy and radiotherapy. The client experiences all of the expected side effects associated with both therapies. Because the chemotherapy is administered in such high doses, these side effects are much more intense than those seen with either standard chemotherapy or radiation. These side effects include severe nausea and vomiting, mucositis, capillary leak syndrome, diarrhea, and bone marrow suppression (Poliquin, 1997). Late effects from the conditioning regimen are also common, occurring as late as 3 to 10 years after transplantation, and include veno-occlusive disease (VOD), skin toxicities, cataracts, fibrotic pulmonary disease, secondary malignancies, cardiomyopathy, endocrine complications, and neurologic complications (Poliquin, 1997).
TRANSPLANTATION.
Day T-0, the day of transplantation, is separated from the chemotherapy conditioning by at least 2 days to ensure that the chemotherapeutic agent has cleared and will not exert any cytotoxic effects on the transplanted stem cells. The client should have few, if any, circulating white blood cells (WBCs) at this point, indicating successful conditioning. The transplantation itself is very simple. Frozen marrow, PBSCs, or umbilical cord blood cells are thawed in a temperature- controlled warm water bath (D’Andrea et al., 1997). The bone marrow is administered through the client’s central catheter like an ordinary blood transfusion, but not using blood administration tubing. Usually the marrow is infused over a 30-minute period, although it may also be administered by IV push directly into the central catheter with syringes. Side effects of all types of stem cell transfusions are similar. The client may experience fever and hypertension as a result of a reaction to the preservative used for storage of stem cells (D’Andrea et al., 1997). To prevent these reactions, the nurse administers acetaminophen (Tylenol), hydrocortisone, and diphenhydramine (Benadryl) before the transfusion (Johns, 1998). Antihypertensives or diuretics may also be required to treat fluid volume changes. The client may experience red urine secondary to hemolysis of erythrocytes in the infused product.
ENGRAFTMENT.
The transfused PBSCs and marrow cells circulate only briefly in the peripheral blood. Most of the cells, especially the stems cells, find their way to the marrowforming sites of the recipient’s bones and establish residency there. The mechanism by which the donated marrow cells “home in” on the appropriate sites is not yet understood. Engraftment, successful “take” of the transplanted cells in the recipient’s bone marrow, is key to the whole transplantation process. For the donated marrow or stem cells to “rescue” the client after large doses of chemotherapy and/or radiotherapy wipe out his or her own bone marrow, the transfused stem cells must survive and grow in the clients’ bone marrow sites. The engraftment process takes 8 to 12 days for peripheral blood stem cells and 12 to 28 days for bone marrow stem cells (Poliquin, 1997). To facilitate engraftment, hematopoietic growth factors, such as granulocyte colony-stimulating factor or granulocyte-macrophage colonystimulating factor, may be administered (Alcoser & Burchett, 1999). When engraftment is successful, the client’s WBC, erythrocyte, and platelet counts begin to rise.
PREVENTION OF COMPLICATIONS.
The post-transplantation period is difficult. Because the client remains without any natural immunity until the transfused stem cells begin to proliferate and engraftment occurs, infection and severe thrombocytopenia are major problems. The nursing care requirements for this client are virtually identical to those for the client undergoing aggressive induction therapy for AML. Helping the client to maintain hope through this long recovery period is difficult (Campbell, 1999). Complications are often severe and life threatening.
The nurse should try to encourage the client to maintain a positive attitude and be involved in his or her own recovery. In addition to the problems related to the period of pancytopenia (too few circulating blood cells), other immediate hazards associated with BMT include failure to engraft, development of GVHD, and VOD.
Failure to Engraft.
Sometimes the donated marrow or stem cells fail to engraft. This possibility is discussed in advance with the client and the donor. Failure to engraft occurs more often among allogeneic stem cell recipients than among autologous stem cell recipients. The causes may be related to insufficient numbers of cells transplanted, attack or rejection of donor cells by the remaining immunologically competent recipient cells, infection of transplanted cells, and unknown biologic factors. If the transplanted stem cells fail to engraft, the client will die unless another transplantation is successful.
Graft-Versus-Host Disease.
Graft-versus-host disease (GVHD) is an immunologic event that occurs in allogeneic transplants. The immunocompetent cells of the donated marrow recognize the client’s (recipient) cells, tissues, and organs as foreign and mount an immune offense against them. The graft is actually trying to attack the host (Alcoser & Burchett, 1999). Although all host tissues can be attacked and harmed, the tissues most commonly damaged are the skin, gastrointestinal tract, and liver. Approximately 25% to 50% of all allogeneic BMT recipients experience some degree of GVHD, and more than 15% of the clients who experience GVHD die of its complications (Johns, 1998). The presence of GVHD indicates that the transplanted cells are competent and have successfully engrafted. Management of GVHD is achieved by limiting the activation of donor T-lymphocytes through the administration of immunosuppressive agents such as cyclosporine, methotrexate, corticosteroids, and antithymocyte globulin (Alcoser & Burchett, 1999). Care is taken to avoid suppressing the new immune system to the extent that either the client becomes more susceptible to infection or the transplanted cells stop engrafting.
Veno-occlusive Disease.
Veno-occlusive disease (VOD) involves occlusion of the hepatic blood vessels by clotting and inflammation (phlebitis). This condition occurs in up to 20% of clients who receive either an autologous or an allogeneic transplant, and symptoms usually occur within the first 30 days after transplantation (Johns, 1998). Clients who have received high doses of chemotherapy, especially alkylating agents, are at risk for life-threatening hepatic complications. Clinical signs include jaundice, pain in the right upper quadrant, ascites, weight gain, and liver enlargement. Because there is no known way of opening the hepatic vessels, treatment is supportive. Early detection enhances the chances of survival. Fluid management is also crucial. The nurse assesses the client daily for weight gain, fluid accumulation, increases in abdominal girth, and hepatomegaly.
RISK FOR INJURY
Because normal bone marrow production is severely limited with acute myelogenic leukemia (AML), the number of circulating platelets is severely diminished, causing thrombocytopenia. This condition puts the client with AML at a greatly increased risk for excessive bleeding in response to minimal trauma. Thrombocytopenia can also be induced by induction therapy for AML or high-dose chemotherapy for transplantation (Rust, Wood, & Battiato, 1999).
PLANNING: EXPECTED OUTCOMES.
The client with leukemia is expected to remain free from bleeding.
INTERVENTIONS.
As a result of chemotherapy-induced pancytopenia, the client’s platelet count is decreased. During the period of greatest bone marrow suppression (the nadir), the platelet count may be extremely low (<10,000/mm3). The client is at great risk for bleeding once the platelet count falls below 50,000/mm3, and spontaneous bleeding often occurs when the platelet count is lower than 20,000 (Harrahill & DeLoughery, 1998).
BLEEDING PRECAUTIONS.
The nurse’s major objectives are to protect the client from situations that could lead to bleeding and to closely monitor any bleeding that does occur. The nurse assesses the client frequently for evidence of bleeding: oozing, confluent ecchymoses, petechiae, or purpura. All stools, urine, nasogastric drainage, and vomitus are examined visually for blood and tested for occult blood. The nurse measures any blood loss as accurately as possible and measures the client’s abdominal girth daily. Increases in abdominal girth can indicate internal hemorrhage. Bleeding precautions are instituted (Chart 40-11).
The nurse also monitors laboratory values daily. Complete blood count (CBC) results are reviewed daily to determine the risk for bleeding, as well as actual blood loss. The client with a platelet count below 20,000/mm3 may need a platelet transfusion. An alternative treatment is the administration of oprelvekin (Neumega), a platelet (thrombopoietic) growth factor. The recommended dose is 50 xg/kg/day subcutaneously starting 6 to 12 hours after the completion of chemotherapy (Rust, Wood, & Battiato, 1999). For the client with severe blood loss, packed RBCs may be ordered.
FATIGUE
Because normal bone marrow production is severely limited in leukemia, the number of circulating erythrocytes is severely diminished, creating a condition of anemia, leading in turn to fatigue. Because leukemic cells tend to have higher rates of metabolism and greater utilization of oxygen, the anemic client with leukemia is at risk for severe fatigue. Anemia also occurs secondary to chemotherapy treatment (Richardson, Ream, & Wilson-Barnett, 1998).
PLANNING: EXPECTED OUTCOMES.
The client with leukemia is expected to:
• Experience no increase in fatigue
• Recognize symptoms of fatigue and alter activity before fatigue becomes excessive
INTERVENTIONS
ENERGY MANAGEMENT.
Energy management aims at decreasing the effects of anemia and conserving energy expenditure (see Chart 40-10).
DIET THERAPY.
Diet therapy is indirectly related to fatigue and subsequent activity intolerance. The client must ingest enough calories to meet at least basal energy requirements, but increasing dietary intake can be difficult when the client is extremely fatigued. The nurse thus provides small, frequent meals high in protein and carbohydrates. Food items that are liquid or easy to chew also require less effort to eat.
BLOOD REPLACEMENT THERAPY.
Blood transfusions are sometimes indicated for the client with fatigue. Transfusions increase the blood’s oxygen-carrying capacity and replace missing red blood cells (RBCs) and some coagulation factors (see Table 40-2). For the client with leukemia who is experiencing fatigue related to anemia, packed RBCs are usually the blood component of choice. (See Transfusion Therapy, p. 862, for a discussion of nursing care during transfusions.)
DRUG THERAPY.
Clients may receive subcutaneous injections of epoetin alfa (Epogen or Procrit) 50 to 100 units/kg three times per week (Cella & Bron, 1999). This growth factor is naturally secreted by the kidney and boosts the production of RBCs. Epoetin alfa has previously been used in anemia associated with chronic renal failure and in clients with human immunodeficiency virus (HIV) who are receiving zidovudine and is now approved for use in anemia associated with chemotherapy. The nurse administers injections three times a week and assesses for side effects such as hypertension, headaches, fever, myalgia (muscle aches), and rashes.
CONSERVATION OF ENERGY.
The nurse examines the hospitalized client’s schedule of prescribed and routine activities. Those activities that do not have a direct positive effect on the client’s condition are assessed in terms of their usefulness. If the actual or potential benefit of an activity is less than its actual or potential worsening of fatigue, the nurse consults with other members of the health care team about eliminating or postponing it. Candidates for cancellation or postponement include physical therapy and certain invasive diagnostic tests not required for assessment or treatment of current problems.
Community-Based Care
The client with leukemia is discharged after induction chemotherapy and recovery of blood cell-producing function. Follow-up care is provided on an outpatient basis. Although the majority of transplant centers discharge clients following engraftment, some centers administer high-dose chemotherapy and stem cell infusion on an outpatient basis. This plan involves daily clinic visits and frequent follow-up by nurses in the home care setting (D’Andrea et al., 1997).
HOME CARE MANAGEMENT
Planning for home care for the client with leukemia begins as soon as a client achieves remission. He or she will need assistance at home until the condition improves. The nurse assesses the available support mechanisms. Many clients require the services of a visiting nurse to assist with dressing changes for central venous catheters, to assist with hyperalimentation infusions, to transfuse platelets, and to answer questions. Occasionally they may also require home transfusion therapy for one or more blood components (Bean, 1998). The home care team is critical for the client receiving stem cell transplantation in the home setting. Potential candidates are evaluated in advance. Criteria include a knowledgeable caregiver, a clean home environment, close proximity to the hospital, telephone access, and emotional stability on the part of the client and caregiver (Herrmann et al., 1998). In one sample program, clients receive their daily dose of chemotherapy in the outpatient clinic in the morning and then receive a home visit in the evening. Home care nurses administer chemotherapy and monitor the client for complications. Nurses visit the client once or twice a day and spend between 4 and 8.5 hours per day in the home (D’Andrea et al., 1997). The client receives the stem cell transplant infusion in the outpatient clinic. Nursing care is similar to that provided in the hospital. If serious complications such as sepsis or veno-occlusive disease occur, the client is admitted to the inpatient facility
HEALTH TEACHING
The client and the family need to be educated about the importance of continuing therapy and appropriate medical follow-up, despite the unpleasant side effects of therapy. Many clients go home with a central venous catheter in place and require instructions about its care and maintenance (Bean, 1998). Chart 40-12 lists general guidelines for central venous catheter care at home.
These guidelines may be altered depending on the home setting, assistance available, and agency policy. Protecting the client from infection after discharge from the hospital is just as important as it was during hospitalization. The nurse urges the client to use proper hygiene and to avoid crowds or others with infections. Neither the client nor any household member should receive live virus immunization (poliomyelitis, measles, or rubella) for 2 years after transplantation (Alcoser & Burchett, 1999). The client should continue mouth care regimens at home. The nurse emphasizes that the client should immediately notify the physician if he or she experiences fever or any other sign of infection.
Chart 40-13 lists guidelines for clients for the prevention of infection.
Because platelet recovery is usually slower than recovery of white blood cells (WBCs), many clients return home still at risk for bleeding. Thrombocytopenia may be present for 6 months following transplantation (Hurley, 1997). The nurse reinforces the safety and bleeding precautions initiated in the hospital, emphasizing that the client must follow these precautions until the platelet count is above 50,000. The client and family are instructed to assess for petechiae, avoid trauma and sharp objects, apply pressure to wounds for 10 minutes, and report any unusual symptoms, including blood in the stool or urine, or headache that does not respond to acetaminophen. Chart 40-14 lists guidelines for clients at risk for bleeding.
PSYCHOSOCIAL PREPARATION
The nurse’s responsibility in psychosocial preparation of the client before discharge is very important. A diagnosis of leukemia threatens self-esteem and the family role. The client is confronted with the reality of death, and treatment causes major adjustments in self-image. The client and family also experience changes in the client’s body image, level of independence, and lifestyle. Some feel threatened by their environment, seeing everything as potentially infectious. Clients who are cared for in protective isolation may experience loneliness and loss of contact with the outside world (Campbell, 1999). The nurse helps the client and family redefine priorities, understand the illness and its treatment, and find hope. The nurse makes referrals to support groups sponsored by organizations such as the American Cancer Society (“I Can Cope” and “Make Today Count”), which can be enormously beneficial to both the client and the family.
COAGULATION DISORDERS
Coagulation disorders are synonymous with bleeding disorders and are characterized by abnormal or increased bleeding resulting from defects in one or more components regulating hemostasis. Bleeding disorders may be spontaneous or traumatic, localized or generalized, lifelong or acquired. They can originate from a defect in the hemostatic processes at the vascular, platelet, or clotting factor level. Figure 40-4 outlines blood-clotting cascades and sites where specific defects and drugs disrupt the hemostatic processes.
PLATELET DISORDERS
Platelets play a vital role in hemostasis. For both the intrinsic and the extrinsic pathways, blood clotting starts with platelet adhesion and the formation of a platelet plug. Any condition that either reduces the number of platelets or interferes with their ability to adhere (to one another, blood vessel walls, collagen, or fibrin threads) can be manifested as increased bleeding. Platelet disorders can be inherited, acquired, or temporarily induced by the ingestion of substances that limit platelet production or inhibit aggregation. A drop in the number of platelets below the level needed for normal coagulation is called thrombocytopenia.
Causes of Thrombocytopenia
Platelets and other blood cells are made in the bone marrow. This is the soft, spongy part inside bones. Thrombocytopenia can result when:
· The bone marrow doesn’t make enough platelets.
· Platelets are destroyed by the body at a rate faster than they can be made in the bone marrow.
· Platelets become trapped in an enlarged spleen.
These problems can occur due to many reasons, including:
· Certain conditions that affect how platelets are made in the bone marrow, such as aplastic anemia, leukemia, and lymphoma
· Certain medications, such as some types of antibiotics, anti-seizure medications, and chemotherapy drugs
· Certain viral infections, such as varicella (chicken pox), HIV, and Epstein-Barr virus
· Certain autoimmune problems, such as lupus and immune thrombocytopenic purpura (ITP)
· Certain conditions that can cause an enlarged spleen, such as cirrhosis and cancer
· Alcohol abuse
· Pregnancy
Symptoms of Thrombocytopenia
Possible symptoms include:
· Severe bruising or bleeding
· Small red or purple spots (petechiae) on the skin
· Bleeding gums
· Nosebleeds
· Bleeding from a wound that stops and starts again
· Bloody urine or stool
· Heavy menstrual flow (women only)
Diagnosing Thrombocytopenia
Your doctor will ask about your symptoms and health history. You will also be examined. Tests will be done to confirm the problem as well. These may include:
· Acomplete blood cell count (CBC). This test measures the amounts of the different types of cells in the blood. This includes the number of platelets in the blood (platelet count).
· A blood smear. This test checks for the different types of blood cells in the blood and how they appear. A sample of your blood is spread on a glass slide and viewed under a microscope. A stain is used so the blood cells can be seen.
· A bone marrow aspiration and biopsy. This test checks for problems with how the bone marrow makes blood cells. A needle is used to remove a sample of the bone marrow in your hip bone. The sample is then sent to a lab to be tested for problems.
Treating Thrombocytopenia
Often, no treatment is needed for thrombocytopenia. Your doctor will monitor your symptoms to see if they improve. Blood tests will also be done to check whether your platelet count returns to normal on its own. If treatment is needed, this may involve:
· Treatment of the underlying cause. For instance, if a medication is the cause, it may be stopped or changed.
· Platelet transfusions. These help raise the number of healthy platelets in the body.
· Blood transfusions. These help treat blood loss that may occur because of low platelets.
· Medications. These may be given to help prevent platelets from being destroyed. These may also be given to help the bone marrow make more platelets.
· Surgery to remove the spleen. The spleen helps filter the blood. It also stores some blood cells, including platelets. If the spleen is enlarged, it can store too many platelets. This causes there to be fewer platelets in the blood thaormal. Though done less often, removing the spleen may help treat thrombocytopenia in certain cases.
Thrombocytopenia may occur as a result of other conditions or treatments that suppress general bone marrow activity. It also can occur through processes that specifically limit platelet formation or increase the rate of platelet destruction. The two thrombocytopenic conditions affecting adults are autoimmune thrombocytopenic purpura and thrombotic thrombocytopenic purpura.
Autoimmune Thrombocytopenic Purpura
OVERVIEW
Before the underlying cause of autoimmune thrombocytopenic purpura was identified, this condition was known as idiopathic thrombocytopenic purpura (ITP). Although the cause is now thought to be an autoimmune reaction, the condition is still commonly known as ITP. The total number of circulating platelets is greatly reduced in ITP, even though platelet production in the bone marrow is normal. Clients with idiopathic thrombocytopenic purpura make an antibody directed against the surface of their own platelets (an antiplatelet antibody). This antibody coats the surface of the platelets, making them more susceptible to attraction and destruction by phagocytic leukocytes, especially macrophages. Because the spleen contains a large concentration of macrophages and because the blood vessels of the spleen are long and twisted, antibody-coated platelets are destroyed primarily in the spleen. When the rate of platelet destruction exceeds that of production, the number of circulating platelets decreases and blood clotting slows. Although the cause of this disorder appears to be autoimmune, the exact mechanism initiating the production of autoantibodies is unknown. ITP is most common among women between the ages of 20 and 40 and among people with a preexisting autoimmune condition, such as systemic lupus erythematosus (Cotran, Kumar, & Robbins, 1999).
A 36-year-old male with idiopathic thrombocytopenia purpura
and a platelet count of 5,000/mm3. Supportive platelet transfusions and
immunoglobulin therapy were used to control bleeding.
A, Labial and tongue ecchymoses;
B, palatal ecchymoses;
C, buccal ecchymoses and fibrinous clot
COLLABORATIVE MANAGEMENT
Assessment Clinical manifestations associated with ITP are generally limited to the skin and mucous membranes: large ecchymoses (bruises) on the arms, legs, upper chest, and neck or a petechial rash. Mucosal bleeding occurs easily. If the client has experienced significant blood loss, signs of anemia may also be present. A rare complication is an intracranial bleeding-induced stroke. The nurse assesses for neurologic function and mental status. Family members or significant others are asked if the client’s behavior and responses to the mental status examination are typical or represent a change from usual reactions. Idiopathic thrombocytopenic purpura is diagnosed by a decreased platelet count and large numbers of megakaryocytes in the bone marrow. Antiplatelet antibodies may be present in detectable levels in peripheral blood. If the client experiences any episodes of bleeding, hematocrit and hemoglobin levels also are low.
Interventions
NONSURGICAL MANAGEMENT.
As a result of the decreased platelet count, the client is at great risk for bleeding. Interventions include therapy for the underlying condition, as well as protection from trauma-induced bleeding episodes.
DRUG THERAPY.
Agents used to control ITP include drugs that suppress immune function to some degree. The premise for the use of agents such as corticosteroids and azathioprine (Imuran) is to inhibit immune system synthesis of antiplatelet autoantibodies. More aggressive therapy can include low doses of chemotherapeutic agents, such as the antimitotic agents and cyclophosphamide.
BLOOD REPLACEMENT THERAPY.
For the client with a platelet count of less than 20,000/mm3 who is experiencing an acute life-threatening bleeding episode, a platelet transfusion may be required. Platelet transfusions are not performed routinely, because the donated platelets are just as rapidly destroyed by the spleen as the client’s own platelets (see later discussion under Platelet Transfusions, p. 865).
MAINTAINING A SAFE ENVIRONMENT.
The nurse’s major objectives are to protect the client from situations that can lead to bleeding and to closely monitor the amount of bleeding that is occurring. (For nursing care actions, see Risk for Injury [Leukemia], p. 856.)
SURGICAL MANAGEMENT. For the client who does not respond to drug therapy, splenectomy may be the treatment of choice. Because the leukocytes in the spleen perform many different immunodefensive functions, the client who has undergone a splenectomy is at increased risk for infection.
Thrombotic Thrombocytopenic Purpura
OVERVIEW
Thrombotic thrombocytopenic purpura (TTP) is a rare disorder in which platelets clump together inappropriately in the microcirculation and insufficient platelets remain in the systemic circulation. The client experiences inappropriate clotting, yet the blood fails to clot properly when trauma occurs. The underlying cause of TTP appears to be an autoimmune reaction in blood vessel cells (endothelial cells) that makes platelets clump together in very small blood vessels. As a result, tissues become ischemic. Common manifestations include renal failure, myocardial infarction, and stroke. If left untreated, this condition is fatal within 3 months in 90% of clients (McBrien, 1997).
COLLABORATIVE MANAGEMENT
Treatment for the client with TTP focuses on inhibiting the inappropriate platelet aggregation and disrupting the underlying autoimmune process. Primary treatment consists of plasma pheresis with the infusion of fresh frozen plasma. This treatment provides the necessary platelet aggregate inhibitors (McBrien, 1997). Drugs that inhibit platelet clumping, such as aspirin, alprostadil (Prostin), and plicamycin, also may be helpful. Immunosuppressive therapy reduces the intensity of this disorder.
CLOTTING FACTOR DISORDERS
Coagulation or bleeding disorders can result from a clotting factor defect, including the inability to produce a specific clotting factor, production of insufficient quantities, or a less active form of clotting factor. Most clotting factor disorders are congenitally transmitted gene abnormalities of one clotting factor. The few acquired clotting factor disorders are related to an inability to synthesize many clotting factors at the same time as a result of liver damage or an insufficiency of clotting cofactors and precursor products. Common congenital disorders that result in defects at the clotting factor level include hemophilias A and B and von Willebrand’s disease. Disseminated intravascular coagulation (DIC) may be considered an acquired clotting disorder but is more closely associated with septic shock.
Hemophilia
OVERVIEW
Hemophilia comprises two hereditary bleeding disorders resulting from deficiencies of specific clotting factors. Hemophilia A (classic hemophilia) results from a deficiency of factor VIII and accounts for 80% of cases of hemophilia. Hemophilia B (Christmas disease) is a deficiency of factor IX and accounts for 20% of cases. The incidence of both is 1 in 10,000. Hemophilia is an Xlinked recessive trait. Female carriers have a 50% chance of transmitting the gene for hemophilia to their daughters (who then are carriers) and to their sons (who will have overt hemophilia).
Hemophilia A is, with rare exceptions, a disease affecting males, none of whose sons will have the gene for hemophilia and all of whose daughters will be carriers (able to pass on the gene without actually having the disorder). In about 30% of clients with hemophilia, there is no family history, and it is presumed that their disease is the result of a new mutation (Cotran, Kumar, & Robbins, 1999).
The bleeding disorder associated with hemophilia A is so severe that before blood transfusions were available, children with hemophilia rarely survived past age 3 years. With the availability of blood transfusion and factor VIII therapy, mean survival time has increased so greatly that hemophilia now is commonly seen among adult clients. The clinical pictures of hemophilia A and B are identical. The client has abnormal bleeding in response to any trauma because of an absence or deficiency of the specific clotting factor. Clients with hemophilia do not bleed more frequently or even more rapidly that those without the disease, but they do bleed for a longer period (Ligda, 1998). Hemophiliacs form platelet plugs at the bleeding site, but the clotting factor deficiency impairs the hemostatic response and the capacity to form a stable fibrin clot. This produces abnormal bleeding, which may be mild, moderate, or severe, depending on the degree of factor deficiency.
COLLABORATIVE MANAGEMENT
Assessment
Assessment of the client with hemophilia reveals the following:
• Excessive hemorrhage from minor cuts or abrasions caused by abnormal platelet function
• Joint and muscle hemorrhages that lead to disabling long-term sequelae
• A tendency to bruise easily
• Prolonged and potentially fatal postoperative hemorrhage
The laboratory test results for a client with hemophilia demonstrate a prolonged partial thromboplastin time (PTT), a normal bleeding time, and a normal prothrombin time (PT) (Cotran, Kumar, & Robbins, 1999). The most common health problem associated with hemophilia is degenerating joint function resulting from chronic bleeding into the joints, especially at the hip and knee.
• Interventions
The bleeding problems of hemophilia A can be well managed by either regularly scheduled IV administration of factor VIII cryoprecipitate or intermittent administration as needed, depending on the individual’s activity level and injury probability (see Cryoprecipitate, p. 865). However, the cost of cryoprecipitate is prohibitive for many people with hemophilia. In addition, because the precipitated clotting factors are derived from pooled human serum, a risk of viral contamination remains, even with the use of heat-inactivated serum (Ligda, 1998). Major complications of hemophilia therapy during the 1980s were infection with hepatitis B virus, cytomegalovirus, and human immunodeficiency virus (HIV). Although heat-inactivated serum and the elimination of HIV-positive donors have reduced these risks, they have not yet been eliminated.
Side Effects of Hemophilia Treatment
Due to the great progress in the prevention of infectious bloodborne viruses, careful donor selection, viral inactivation processes, and new fractionation procedures, the risk of complications has been drastically reduced; however, it has not be completely eliminated. The possibility of viral disease transmission by blood derivatives still exists.
TRANSFUSION THERAPY
Any blood component may be removed from a donor and transfused to benefit a recipient. Components may be transfused individually or collectively, with varying degrees of benefit to the recipient.
PRETRANSFUSION RESPONSIBILITIES
Nursing actions during transfusions aim largely at prevention or early recognition of adverse transfusion reactions. Preparation of the client for transfusion therapy is imperative, and in stitutional blood product administration procedures should be carefully followed. Before administering any blood product, the nurse reviews the agency’s policies and procedures. Chart 40-15 presents best practices for transfusion therapy.
Legally, a physician’s order is needed to administer blood or its components. The order specifies the type of component to be delivered, the volume to be transfused, and any special conditions the physician judges to be important. The nurse verifies the order for accuracy and completeness. The nurse also evaluates the need for transfusion, considering both the client’s clinical condition and the laboratory values. In many hospitals a separate consent form must be obtained for the administration of blood products before a transfusion is performed.
A blood specimen is obtained for crossmatching (testing of the donor’s blood and the recipient’s blood for compatibility). The procedure and responsibility for obtaining this specimen are specified by hospital policy. The laboratory requires at least 45 minutes to complete the crossmatch testing. In most hospitals a new crossmatching specimen is required at least every 48 hours (Dreger & Tremback, 1998).
Because of the viscosity of blood components, a 19-gauge needle or larger is used, whenever possible, for venous access. Both Y-tubing and straight tubing sets are available for blood component administration. A blood filter (approximately 170 xm) to remove aggregates from the stored blood products is included with component administration equipment and must be used to transfuse all blood products. In massive transfusion, a microaggregate filter (20 to 40 jam) may be used.
Normal saline is the solution of choice for administration. Ringer’s lactate and dextrose in water are contraindicated for administration with blood or blood products because they cause clotting or hemolysis of blood cells.
Medications are never added to blood products.
Before the transfusion is initiated, it is essential to determine that the blood component delivered is correct. Two registered nurses simultaneously check the physician’s order, the client’s identity, and whether the hospital identification band name and number are identical to those on the blood component tag. The blood bag label, the attached tag, and the requisition slip are examined to ensure that the ABO and Rh types are compatible. The expiration date is also checked, and the product is inspected for discoloration, gas bubbles, or cloudiness—indicators of bacterial growth or hemolysis.
TRANSFUSION RESPONSIBILITIES
The nurse takes the vital signs, including temperature, immediately before initiating the transfusion. Infusion begins slowly. A nurse remains with the client for the first 15 to 30 minutes. Any severe reaction usually occurs with administration of the first 50 mL of blood. The nurse assesses vital signs 15 minutes after initiation of the transfusion to detect signs of a reaction. If there are none, the infusion rate can be increased to transfuse 1 unit in about 2 hours (depending on the client’s cardiovascular status). The nurse takes the vital signs every hour throughout the transfusion or as specified by agency policy. Blood components without large amounts of red blood cells (RBCs) can be infused more quickly. The identification checks are the same as for RBC transfusions. Physiologic changes in older clients may necessitate that blood products be transfused at a slower rate. Best practices related to the nursing care needs of older clients undergoing transfusion therapy are provided in Chart 40-16.
TYPES OF TRANSFUSIONS
Red Blood Cell Transfusions
RBCs are administered to replace erythrocytes lost as a result of trauma or surgical interventions. Clients with clinical conditions that result in the destruction or abnormal maturation of RBCs may also benefit from RBC transfusions. Packed RBCs, supplied in 250-mL bags, are a concentrated source of RBCs and are the most common component administered to RBCdeficient clients (Dreger & Tremback, 1998). Packed RBCs are administered to individuals with a hemoglobin concentration less than 6 g/dL (or a hemoglobin value of 6 to 10 g/dL if clinical symptoms are present) (Kennedy, 1999). Blood transfusions are actually transplantations of tissue from one person to another. The donor and recipient blood must thus be carefully checked for compatibility to prevent potentially lethal reactions (Table 40-7).
Compatibility is determined by two different types of antigen systems (cell surface proteins): the ABO system antigens and the Rh antigen, present on the membrane surface of RBCs (Dreger & Tremback, 1998). RBC antigens are inherited.
For the ABO antigen system, a person inherits one of the following:
• A antigen (type A blood)
• B antigen (type B blood)
• Both A and B antigens (type AB blood)
• No antigens (type O blood)
Within the first few years of a child’s life, circulating antibodies develop against the blood type antigens that were not inherited. For example, a child with type A blood will form antigens against type B blood. A child with type O blood has not inherited either A or B antigens and will form antibodies against RBCs that contain either A or B antigens. If erythrocytes that contain a foreign antigen are infused into a recipient, the donated tissue can be recognized by the immune system of the recipient as non-self, and the client may have a reaction to the transfused products. The mechanism of the Rh antigen system is slightly different. An Rh-negative person is born without the antigen and does not form antibodies unless he or she is specifically sensitized to it. Sensitization can occur with RBC transfusions from an Rh-positive person or from exposure during pregnancy and birth. Once an Rh-negative person has been sensitized and antibody development has occurred, any exposure to Rh-positive blood can cause a transfusion reaction. Antibody development can be prevented by administration of Rh-immune globulin as soon as exposure to the Rh antigen is suspected. People who have Rh-positive blood can receive an RBC transfusion from an Rh-negative donor, but Rh-negative people must never receive Rh-positive blood.
Platelet Transfusions
Platelets are administered to clients with platelet counts below 20,000 mm3 and to clients with thrombocytopenia who are actively bleeding or are scheduled for an invasive procedure (Dreger, & Tremback, 1998). Platelet transfusions are usually pooled from as many as 10 donors and do not have to be of the same blood type as the client. For clients who are candidates for bone marrow transplantation (BMT) or who require multiple platelet transfusions, single-donor platelets may be ordered. Single-donor platelets are obtained from one person and decrease the amount of antigen exposure to the recipient, helping prevent the formation of platelet antibodies. The chances of allergic transfusion reactions to future platelet transfusions are thus reduced. Platelet infusion bags usually contain 300 mL for pooled platelets and 200 mL for single-donor platelets. Because the platelet is a fragile cell, platelet transfusions are administered rapidly after being brought to the client’s room, usually over a 15- to 30-minute period. A special transfusion set with a smaller filter and shorter tubing is used. Standard transfusion sets are not used with platelets because the filter traps the platelets, and the longer tubing increases platelet adherence to the lumen. Additional platelet filters help remove white blood cells (WBCs) in the platelet concentrate. These filters are connected directly to the platelet transfusion set and are used for clients who have a history of febrile reactions or who will require multiple platelet transfusions. The nurse takes the vital signs before the infusion, 15 minutes after the infusion is initiated, and at its completion. The client may be premedicated with meperidine (Demerol) or hydrocortisone to minimize the chances of a reaction. He or she can become febrile and experience rigors (severe chills) during transfusion, but these symptoms are not considered a true transfusion reaction. IV administration of amphotericin B (Amphotec, Fungizone), an antifungal agent given to many clients with leukemia, is discontinued during platelet transfusion and not resumed for at least 1 hour after transfusion. Amphotericin B can cause severe allergic reactions that are difficult to distinguish from transfusion reactions.
Plasma Transfusions
Historically, plasma infusions have been administered to replace blood volume, and they are occasionally still used for this purpose. It is more common for plasma to be frozen immediately after donation. Freezing preserves the clotting factors, and the plasma can then be used for clients with clotting disorders (Kennedy, 1999).
Fresh frozen plasma (FFP) is infused immediately after thawing while the clotting factors are still viable. Clients who are actively bleeding with a prothrombin time (PT) or partial thromboplastin time (FIT) greater than 1.5 times normal are candidates for an FFP infusion (Harrahill & DeLoughery, 1998). ABO compatibility is required for transfusion of plasma products. The volume of the infusion bag is approximately 200 mL. The infusion takes place as rapidly as the client can tolerate, generally over a 30- to 60-minute period, through a regular Y-set or straight-filtered tubing.
Cryoprecipitate is a product derived from plasma. Clotting factors VIII and XIII, von Willebrand’s factor, fibronectin, and fibrinogen are precipitated from pooled plasma to produce cryoprecipitate. Clients with a fibrinogen level less than 100 mg/dL are candidates for a cryoprecipitate infusion (Harrahill & DeLoughery, 1998). This highly concentrated blood product is administered to clients with clotting factor disorders at a volume of 10 to 15 mL/unit. Although cryoprecipitate can be infused, it is usually given by IV push within 3 minutes. Dosages are individualized, and it is best if the cryoprecipitate is ABO compatible.
Granulocyte Transfusions
At some centers, neutropenic clients with infections receive granulocyte transfusions for WBC replacement. However, this practice is highly controversial because the potential benefit to the client must be weighed against the potential severe reactions that often accompany granulocyte transfusions. The surfaces of granulocytes contaiumerous antigens that can cause severe antibody-antigen reactions when infused into a recipient whose immune system recognizes these antigens as non-self. In addition, transfused granulocytes have a very short life span and are probably of minimal benefit to the client (see Chapter 20). There is some evidence that treatment with antibiotics alone results in better survival rates. Granulocytes are suspended in 400 mL of plasma and should be transfused over a 45- to 60- minute period. Institutional policies often require more stringent monitoring of clients receiving granulocytes. A physician may need to be present in the hospital unit, and vital signs may need to be taken every 15 minutes throughout the transfusion. Administration of amphotericin B and granulocyte transfusions should be separated by 4 to 6 hours.
TRANSFUSION REACTIONS
Clients can experience any of the following transfusion reactions: hemolytic, allergic, febrile, or bacterial reactions; circulatory overload; or transfusion-associated graft-versus-host disease (GVHD). The nurse is vigilant to prevent serious complications through early detection and initiation of appropriate treatment.
Hemolytic Transfusion Reactions
Hemolytic transfusion reactions are caused by blood type or Rh incompatibility. When blood containing antibodies against the recipient’s blood is infused, antigen-antibody complexes are formed and released into the circulation. These complexes can destroy the transfused cells and initiate inflammatory responses in the recipient’s blood vessel walls and organs. The ensuing reaction may be mild, with fever and chills, or life threatening, with disseminated intravascular coagulation (DIC) and circulatory collapse (Robb, 1999).
Other clinical signs include the following:
• Apprehension
• Headache
• Chest pain
• Low back pain
• Tachycardia
• Tachypnea.
• Hypotension
• Hemoglobinuria
• A sense of impending doom
The onset of a hemolytic transfusion reaction may be immediate or may not occur until subsequent units have been transfused.
Allergic Transfusion Reactions
Allergic transfusion reactions are most often seen in clients with a history of allergy. They may have urticaria, itching, bronchospasm, or occasionally anaphylaxis. Onset of this type of reaction usually occurs during or up to 24 hours after the transfusion. Clients with a history of allergy can be given buffy coat-poor or washed red blood cells (RBCs) in which the white blood cells (WBCs) and plasma have been removed. This procedure minimizes the possibility of an allergic reaction.
Febrile Transfusion Reactions
Febrile transfusion reactions occur most commonly in the client with anti-WBC antibodies, a situation seen after multiple transfusions. The recipient experiences the following: • Sensations of cold • Tachycardia • Fever • Hypotension • Tachypnea Again, the physician can order buffy coat-poor RBCs or single-donor HLA-matched platelets. Leukocyte filters may also be used to trap WBCs and prevent their transfusion into the client.
Bacterial Transfusion Reactions
Bacterial transfusion reactions are seen after transfusion of contaminated blood products. Usually a gram-negative organism is the source because these bacteria grow rapidly in blood stored under refrigeration. Symptoms include the following: • Tachycardia • Hypotension • Fever • Chills • Shock The onset of a bacterial transfusion reaction is rapid.
Circulatory Overload
Circulatory overload can occur when a blood product is administered too quickly (Goldy, 1998). This complication is most common with whole-blood transfusions or when the client requires multiple transfusions. Older adults are most at risk for this condition (see Chart 40-16). Symptoms include the following: • Hypertension • Bounding pulse • Distended jugular veins • Dyspnea • Restlessness • Confusion The nurse can both manage and prevent this complication by monitoring intake and output, transfusing blood products more slowly, and administering diuretics.
Transfusion-Associated Graft-Versus-Host Disease
Transfusion-associated graft-versus-host disease (TA-GVHD) is an infrequent but life-threatening complication that can occur in both immunosuppressed and immunocompetent clients. Its cause in immunosuppressed clients is similar to that of GVHD associated with allogeneic bone marrow transplantation (BMT), discussed on p. 855, in which donor T-cell lymphocytes attack host tissues. The cause of TA-GVHD in immunocompetent hosts is uncertain. Reactions are more common when the host and donor share similar human leukocyte antigens (HLAs), such as in first-degree relatives or individuals with a similar ethnic background. Symptoms typically occur within 1 to 2 weeks and include thrombocytopenia, anorexia, nausea, vomiting, chronic hepatitis, weight loss, and recurrent infection. TA-GVHD has a 90% mortality rate but can be prevented by using irradiated blood products, which destroy T-cells and their cytokine products (Dreger & Tremback, 1998).
AUTOLOGOUS BLOOD TRANSFUSIONS
Autologous blood transfusions involve collection and transfusion of the client’s own blood. Advantages of this type of transfusion are guaranteed compatibility and elimination of the risk of transmitting diseases such as hepatitis or HIV. The four types of autologous blood transfusions are preoperative autologous blood donation, acute normovolemic hemodilution, intraoperative autologous transfusion, and postoperative blood salvage. Preoperative autologous blood donation, the most common type of autologous blood transfusion, involves collection of whole blood from the client, division into components, and then storage for later use (such as after a scheduled surgical procedure).
As long as hematocrit and hemoglobin levels are within a safe range, the client can donate blood on a weekly basis until the prescribed amount of blood is obtained. Fresh packed RBCs may be stored for 42 days. For individuals with rare blood types, blood may be frozen for up to 10 years. Platelets and plasma may be collected via pheresis. Some cardiovascular problems and bacteremia are contraindications for autologous blood donation. Acute normovolemic hemodilution involves withdrawal of a client’s RBCs and volume replacement just before a surgical procedure. The goal is to decrease RBC loss during surgery. The blood is stored at room temperature for up to 6 hours and reinfused after surgery. This type of autologous transfusion is appropriate for healthy clients but is contraindicated for individuals who are anemic or who have poor renal function. Intraoperative autologous transfusion and postoperative blood salvage involve the recovery and reinfusion of a client’s own blood, collected either from an operative field or postoperatively from a wound.
Several commercial products are available that collect, filter, and drain the blood into a transfusion bag. This autologous blood is often used for trauma or surgical clients with severe blood loss and must be reinfused within 6 hours. The nurse transfuses autologous blood products using the guidelines previously described. Although the client receiving autologous blood is not at risk for most types of transfusion reactions, the nurse must still assess for circulatory overload or bacterial transfusion reactions that can occur as a result of contamination.
