Nursing Assessment Relevance in the Pediatric Patient - Essay Example

Nursing Assessment Relevance in the Pediatric Patient with Hepatic Veno Occlusive Disease Post Stem Cell Transplant Introduction Nurses are vital partners to all healthcare team of professionals. They must therefore be equipped with knowledge and expert skills of caring which they provide during illnesses and at the end of life (Jackson, 2005). In clinical practice, nurses make several assessments and judgments during the course of a shift. A careful and timely assessment therefore is crucial in the early treatment and correction of patients at risk for increased morbidity and mortality so that appropriate interventions can be targeted accordingly. It also aids in the timely identification of patients most in need of more rigorous measures (Green, 1990). Hepatic veno occlusive disease is an important cause of post stem cell transplantation high morbidity and mortality when severe (Ribaud and Gluckman, 1999). The incidence of this post-transplant complication is approximated to be as high as 4% in pediatric patients. Post-transplant hepatic veno occlusive disease is recorded to be the third leading cause of death in pediatric patients (Kist-van Holthe et al., 1998). And because of this, it has assumed importance in the management of the stem cell transplantation pediatric patient. Literature Review Stem Cell Transplantation Stem cell transplantation (term now used to mean bone marrow transplantation) is a very complex therapeutic procedure used in patients with damaged or defective bone marrow (Filipovich, 2005). It involves the administration of high-dose chemoradiotherapy to suppress the recipients immune system, wipe out cancer cells left behind, and provide space for the new marrow to grow. It is followed by the intravenous infusion of healthy hemopoietic stem cells to restore blood cell production. After transplant, the new cells find their way into the sites in the bone and begin to establish itself and reproduce (Muscaritoli et al., 2002). There are two types of stem cell transplantation. Autologous bone marrow transplantation entails the use of the patient’s own stem cells collected prior to the treatment. Allogeneic bone marrow transplantation on the other hand, involves the transfer of marrow to a recipient from a donor (Rowe et al., 1994). Hepatic Veno Occlusive Disease Along with infections, hepatic veno occlusive disease clearly remains a common and most serious complication related to the high-dose chemotherapy regimen after stem cell transplantation that results to liver dysfunction. It is also said to be a result of a regimen-related toxicity post allogeneic and autologous hematopoietic stem cell transplantation (Okamoto et al., 2003). In post autologous bone marrow transplantation, the risks of developing hepatic veno occlusive disease are less than those associated with an allogeneic bone marrow transplantation. Veno occlusive disease in post autologous bone marrow transplantation, occurs in about 10% and 20% post allogeneic stem cell transplantation (Litzow et al., 2002). The clinical syndrome of the hepatic veno occlusive disease is characterized by tender hepatomegaly, hyperbilirubinemia (total serum bilirubin > 34.2 µmol/L), right upper quadrant pain of liver origin, jaundice, ascites and sudden unexplained weight gain (> 2% of baseline body weight) because of fluid retention, and elevated levels of alkaline phosphatase and gamma-glutamyltransferase (Ho et al., 2004a). Transplant recipient patients usually manifest the syndrome of hepatic veno occlusive disease within the days preparative chemoradiation regimen is initiated and by the first 30 days after stem cell transplantation. Also, the symptoms may occur before the marrow is infused (Rappeport, 1996). In addition, the clinical manifestations appear more frequently during the period of pancytopenia prior to bone marrow recovery (Shulman and Hinterberger, 1992). Histologically, in veno occlusive disease, the hepatic venules become narrowed, clogged and swollen damaging the surrounding sinusoids and necrosis of hepatocytes in zone 3 of the liver acinus as a result of the toxic effects of chemotherapy (MacQuillan and Mutimer, 2004). Liver engorgement is the result of the occlusion to hepatic and portal outflow. Liver dysfunction results in inability to break down toxic substances, drugs and waste products. As a consequence, the liver becomes inflamed and eventually affects the kidney function leading to water accumulation (Ribaud and Gluckman, 1999). Diagnosis of hepatic veno occlusive is a challenging one because the clinical features are non-specific. The signs and symptoms must be discerned from a number a conditions that causes hepatic dysfunction in stem cell transplant patients that mimic the clinical features of hepatic veno occlusive such as hepatic congestion, fungal infiltration, acute graft-versus-host disease, bacterial sepsis, drug toxicity and total-parenteral-nutrition hepatitis. Although there are diagnostic tests specific for hepatic veno occlusive disease, elevated levels of PAI-1 have been found to be quite a specific marker (Ho et al., 2004b). Not all patients exhibit the full spectrum of the syndrome, but a clinical definition of hepatic veno occlusive calls for the involvement of any two of the clinical manifestations mentioned above that occurs within 14 days from the bone marrow infusion or reinfusion (Ho et al., 2004a). Patients exhibiting the symptoms are classified as having mild, moderate or severe hepatic veno occlusive disease based on the reversibility of the veno occlusive disease. The initial clinical features of liver toxicity are tender hepatomegaly and sudden weight gain. Patients with mild veno occlusive disease showed no overt adverse effect from liver disease and do not necessitate medications for excessive fluid and hepatic pain. On the other hand, patients showing an adverse effect form the liver disease are classified as having moderate veno occlusive disease. Management includes sodium restriction and diuretics to resolve fluid excess and use of medication to alleviate hepatomegaly pain. Signs and symptoms of liver damage manifested in both are reversible. Lastly, patients classified as severe demonstrate adverse effect from the liver disease with irreversible signs and symptoms before day 100 after transplantation. Fluid may enter the abdominal cavity resulting to ascites and the lungs may be compromised making it potentially fatal. Severe cases of veno occlusive disease culminates in multisystem organ failure such as renal failure, cardiac failure, pleural effusions and pulmonary failure and eventually leads to death (Carreras, 2000). Clinical improvement of patient with hepatic veno occlusive disease is evidenced by decrease in bilirubin, reduction in the right upper quadrant, excess fluid mobilization, and improvement in coagulopathy, reduction in hepatomegaly and reduction in other end-organ dysfunction (Ho et al., 2004b). Management of Post-transplant Pediatric Patients Complications such as hepatic veno occlusive disease is an experience outside the usual occurrences of childhood and which pediatric patients have little knowledge about require the attention of healthcare professionals. How a child responds to an illness or disease largely depends on his cognitive development and level of knowledge. It is therefore important to put into consideration how children view illness or his disease in planning specific nursing care (Pilliterri, 2003). Illness may be more disturbing for children than for adults because of their lack of ability in communicating and monitoring their own care. Children have difficulty expressing discomforts; therefore, closer assessment is essential to reveal how they really feel. Nurses should be aware of not only what the child’s reports but what their facial expressions may be indicating by careful observation. Keen, astute assessment is necessary to ascertain the extent of the child’s illness at any given time (Jackson, 2005). In the clinical management of patients with hepatic veno occlusive disease, the most crucial step is to assess the patient. Assessment of appearance is a quick gauge of the child's overall condition. The nurse can see from across the room whether or not a child is alert, responsive of his surroundings, and interacting with those around him, or irritable and withdrawn (Hewitt-Taylor, 2002). Treatment of patients with established hepatic veno occlusive disease is primarily supportive. Nurses should carefully plan nursing management plan and cautiously and vigilantly monitor vital functions because veno occlusive disease is a serious and potentially life threatening complication that may be compounded with other end-organ damage. Early intervention is essential in minimizing the risk of loss of life due to multiorgan failure (McDonald et al., 1993). A nurse’s obligation to manage pain and relieve patients’ anguish is central to commitment to pediatric patient care. Nurses should perform a number of interventions that will promote comfort, safety and security for patients under their care. Their responsibilities will vary with the type, extent and seriousness of the child’s illness, age and individual circumstances (Green, 1990). The first 100 days post stem cell transplantation is the most critical period. Recipients are cared for in special isolation because there is a high risk of infection due to the absence of functioning immune system and risks for other complications as a result of the pre-transplant chemotherapy. The nurse should ascertain that stringent precautions are taken are taken during this time to minimize exposure to viruses, bacteria and fungi often found in fresh fruits and vegetables, plants and cut flowers to avoid infection (Baron et al., 1997). After the transplant up to the time of engraftment in which new infused cells find their way to the marrow spaces in the bones and begin to reproduce, the patient is often quite ill. Patients require intensive and rigorous monitoring for post-transplant complications because during this period, the patient is neutropenic and his immune system has been destroyed prior to the transplant (Herbetko et al., 1992). The initial signs of liver toxicity are sudden weight gain of more than 2% of baseline body weight because of fluid retention and the development of hepatomegaly or liver tenderness(Pulla et al., 1998). Therefore the physical assessment of the patient with hepatic veno occlusive disease should focus in evaluating weight gain and liver size. Ursodeoxycholic acid Ursodeoxycholic acid, a hydrophilic acid is used as a prophylactic medication in the management of liver problems. It acid is often administered to aid in the prevention of transplant-related hepatic complications in post stem cell transplantation. It stabilizes and protects hepatocytes by altering the lipid composition and decreases elevated levels of liver enzymes by facilitating bile flow through the liver (Ruutu et al., 2002). Defibrotide In the stem cell transplantation setting, defibrotide is used as the first line drug therapy. Defibrotide is a single-stranded polydeoxyribonucleotide that has anti-thrombotic, anti-ischaemic, anti-inflammatory and thrombolytic properties without causing significant systemic anticoagulation. It is administered intravenously, infused over 2 hours in four divided doses per day. Dosage is calculated on a 15mg/kg basis (Chopra et al., 2000). Management of Fluid and Electrolyte Balance The principal mode of management in hepatic veno occlusive disease is supportive consisting strict monitoring and careful management of fluid and electrolyte balance. This involves maintaining the intravascular volume to optimize renal blood flow by restricting sodium and using diuretics to decrease extravascular fluid accumulation (Pulla et al., 1998). Patients should be assessed for fluid retention. In children, liver congestion is one of the first signs of fluid overload. Liver size can be approximated by palpating over the lower edge of the liver and noting its location relative to the costal margin. Hepatomegaly is felt as a hard, nodular, left lobe liver during an abdominal exam (Poliquin, 1990). Patient’s weight should be monitored at least twice daily. Weighing patients daily can help the nurse assess and evaluate fluid balance. The patient’s weight may be abnormally elevated due to presence of edema or ascites (Poliquin, 1990). Management of Ascites and Fluid Retention Patients should be assessed for fluid retention. In children, liver congestion is one of the first signs of fluid overload. Liver size can be approximated by palpating over the lower edge of the liver and noting its location relative to the costal margin. Hepatomegaly is felt as a hard, nodular, left lobe liver during an abdominal exam (Filipovich, 2005). Patient’s weight should be monitored at least twice daily. Weighing patients daily can help the nurse assess and evaluate fluid balance. The patient’s weight may be abnormally elevated due to presence of edema or ascites. In addition to rapid gains of weight, fluid retention in the extravascular fluid can also manifested by ascites. Ascites is the accumulation of fluid in the peritoneal cavity. The ascetic fluid characteristically produces abdominal distention. A dull sound of the abdomen when percussed reveals presence of ascites. Measuring the abdominal girth and comparing it with the baseline measurement at least twice daily can help the nurse assess and detect development of ascites (Ford, 2001). The presence of ascitic fluid causes an obstruction of the blood flow through the liver sinusoids to the hepatic venules and vena cava, resulting to increased hydrostatic pressure in the portal venous system that eventually leads to hepatocytes damage. Because of the hepatocellular damage, the liver is not capable of inactivating aldosterone, which in turn, stimulates the kidney to retain sodium and water. Thus the more water is retained, the more the volume of ascitic fluid grows (Kist-van Holthe et al., 1998). If the client is found to have ascites, fluids and high sodium must be restricted in the diet and the nurse should see to it that restrictions are strictly followed. Majority of patients can survive hepatic veno occlusive disease with conscientious fluid management. Fluid and electrolyte balance is corrected by improving renal sodium excretion and restricting sodium and water intake (Pulla et al., 1998) Extravascular fluid is also manifested by a discrepancy between intake and output. Output should not be less than the intake, and this can be checked by strict monitoring of the intake and output every shift (Ford, 2001). In addition, evidence of clinical manifestations of fluid overload such as presence of ascites, and edema requires potassium-sparing diuretics. Diuretics are prescribed to promote excretion of the extra fluid and guarantee clinical improvement due to fluid mobilization (Kist-van Holthe et al., 1998). Proper Positioning The presence of ascites leads to many problems, Ascites compresses the liver and thus interfering with its function. It may also cause ineffective breathing pattern due to the increased intra-abdominal pressure on the diagphragm. It may also cause shallow breathing and impaired gas exchange resulting to respiratory compromise (Wujcik et al., 1994). The nurse should carefully check for clinical indicators of pulmonary edema, including dypsnea and orthopnea due to the increasing volume of ascitic fluid. If the patient is any respiratory involvement, the nurse should see to it that pulmonary and respiratory measures are implemented to improve respiration. One measure to promote effective breathing pattern is to position client to a semi-Fowler or high-Fowler to facilitate breathing (Ford, 2001). Hemodynamic Status Post stem cell transplantation, recipients should be closely observed for bleeding and assessed for hemodynamic status. Inevitably, post-transplant patients are at risk for bleeding during the period before the platelet count recovers. Patients who develop severe hepatic veno occlusive disease may have marked bleeding due to coagulation deficiencies from liver cell malfunction (Filipovich, 2005). Bleeding can either be nasal, oropharyngeal, intestinal or urinary. If there is evidence of bleeding, defibrotide should be discontinued (Chopra et al., 2000). In addition to this, if bleeding is accompanied by a drastic fall in hematocrit count, it may require red blood cell transfusion support. In general, red blood cell transfusion support is given when the hematocrit falls below 20% or hemoglobin was less than 8 g/dl. Platelet transfusion is also given when platelet counts fall below 20 x 109/L. Platelet transfusion requirements in patients with clinical evidence of hepatic veno occlusive disease are twofold greater than patients without liver disease . Regular post-transplant blood counts will help determine if new white blood cells are starting to be produced indicating that the transplant has been successful (Park et al., 1997). Pain and Anxiety Management As a rule, pain should be relieved and stress should be decreased for all pediatric patients, whether it is a component of a disease process, or a result of an injury, or a result of therapeutic or diagnostic procedure Pain should be assessed routinely, along with vital signs. Failure to properly assess pain can result to the under-treatment of pain (Green, 1990). Children are specific population who may have difficulty communicating their pain and discomforts because they may lack the vocabulary to express and describe symptoms. To assess the child’s pain, the Wong-Baker Faces Pain Rating Scale can be used (Jackson, 2005) Pain should be monitored and intervention modified as the clinical situation demands. Pain relief is a vital component in the care of anxious, uncomfortable post stem cell transplant pediatric patients. The use of adequate analgesia makes children more comfortable and the physical examination and diagnostic procedures easier (Pederson et al., 2000). In addition, regular post-transplant blood counts can create a lot of stress and fears to a pediatric patient. Assessment of the child’s knowledge and level of anxiety concerning a technique must be done before initiating a procedure to increase the child’s cooperation. Specific measures to keep discomfort at the minimum level during a painful experience are very crucial (Kane and Primomo, 2001). Psychological Support The post-transplant period is physically and psychologically exhausting. Each episode of the stem cell transplant crisis is very debilitating to the child. Patients may encounter problems with self-concept and self-esteem, anxiety, depression, social isolation and decreased participation in activities of daily living. It is the role of the nurse therefore to provide a great deal of emotional support to the patient and family members throughout the process (Gould et al., 2000). Conclusion The relevance of a thorough nursing assessment in pediatric patients with hepatic veno occlusive disease post stem cell transplantation is to detect treatable problems early. Careful assessment of the liver function also help determine if defibrotide needs dose adjustment and modification if there is a slow or no response, or discontinued if significant toxicity was encountered during assessment, or reduced when symptoms begin to be resolved (Richardson et al., 2002) This exercise has made me realize that in spite of the generally poor forecast for pediatric patients after stem cell transplantation, an intensive nursing care is critical and vital in the care of these patients because the patient can experience many clinical problems. Bibliography BARON, F., DEPREZ, M. & BEGUIN, Y. 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