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Running head: CASE STUDY PAPER 1 Case Study Paper Bianca Iafrate University of New Hampshire CASE STUDY PAPER 2 Epidemiology/Pathophysiology Acute myeloid leukemia (AML) results from a defect in the hematopoietic stem cell that differentiates into all myeloid cells: monocytes, granulocytes (neutrophils, basophils, eosinophil’s), erythrocytes, and platelets. Specifically, there is an excess of myeloblasts, which would be found through a WBC with a differential. Exceeding 30% of leukemic blasts in a bone marrow aspirate is required for a definitive diagnosis of AML (Lowenberg, 1999). The blast cells have taken over and are producing an excess of immature white blood cells, which prevent the development of healthy WBCs, RBCs, and platelets. There becomes such excess that they spill out into the blood stream, which is how they are found through blood cultures. There is not a specific age group that is mostly affected by this disease, although it is not as common in those younger than age 55. The chances of developing the disease do increase with age and peaks at about 67 years old (National Cancer Institute, 2011). The prognosis tends to become worse with age, in that stem cells of the elderly are slower and slowly produce the white blood cells necessary to fight off infection. This leads to their immune system weakening and further healthy complications follow secondary to the disease. In the United States, the yearly incidence of AML is approximately 2.4 per 100,000 and as mentioned, increases progressively with age. The survival rate among patients who are less than 65 years old is only 40% (Lowenberg, 1999). Symptoms of AML usually occur over a period of weeks and happen suddenly. The causes of the symptoms are due to the lack of normal blood cells being produced. Fever and infection result from a low neutrophil count; weakness and fatigue, dyspnea on exertion, and pallor from anemia; petechiae, ecchymosis, and bleeding tendencies from thrombocytopenia CASE STUDY PAPER 3 (Hinkle & Cheever, 2014). Other symptoms include splenomegaly, hepatomegaly, lymphadenopathy (enlarged organs), hyperplasia of the gums, loss of appetite, night sweats, back and joint pain/tenderness, and bone pain from expansion of marrow (National Cancer Institute, 2012a). Case Study Prompts C.O. is a 43-year-old woman who noted a nonpruritic nodular rash on her neck and chest approximately 6 weeks ago. The rash became generalized, spreading to her head, abdomen, and arms and was accompanied by polyarticular joint pain and back pain. About 2 weeks ago, she experienced three episodes of epistaxis in 1 day. Over the past week, her gums became swollen and tender and she was severely fatigued. Because of the progression of symptoms, she sought medical attention. Lab work was performed, and C.O was directly admitted to the hematology/oncology unit under the care of a hematologist for diagnostic evaluation. Her chest x-ray examination showed normal lung expansion and heart size, without lymphadenopathy. Skin biopsy showed cutaneous leukemic infiltrates, and bone marrow biopsy showed moderately hypercellular marrow collections of monoblasts. Her lumbar puncture specimen was free of blast cells. The final diagnosis was acute myeloblastic leukemia. C.O. is to begin remission induction therapy with cytrabine (Ara-C) 100mg/m2/day as a continuous infusion for 7 days and idarubicin (Idamycin) 12mg/m2/day intravenous (IV) push for 3 days. She is scheduled in angiography for placement of a triple-lumen subclavian Hickman catheter before beginning her therapy. She has multiple symptoms that correlate with the diagnosis of AML including: rash, spontaneous bleeding, joint pain, fatigue, and tender gums. A skin biopsy and CASE STUDY PAPER 4 laboratory work is the best way to confirm this diagnosis. The bone marrow biopsy is done to examine and diagnose cell type and in this case, to confirm malignancy. Her complete blood count (CBC) came back showing very elevated white blood cells and monocytes. Her lymphocytes and neutrophils were decreased, as well as her hemoglobin, hematocrit, and platelets. The most important finding is that her blast cells came back at 17%. In healthy individuals, blasts are not found in the blood stream and for this patient, it shows that there is excess causing them to dump from the bone marrow into the blood stream. Before beginning her chemotherapy, she had a triple lumen subclavian Hickman catheter inserted. After C.O. returns to the unit there are different assessments that the nurse should perform and things to monitor. Potential problems that can occur are erythema, bleeding, irritation, infection, and displacement (Sommer et al., 2013). An Xray is essential to confirm the placement of the catheter and in the event that it is suspected that the catheter is displaced. Phlebitis, infiltration, extravasation, and air embolism should be assessed for. The nurse should monitor for signs of infection (redness, swelling, drainage, tenderness) as well as monitoring the condition of the dressing and changing it per facility protocol. The catheter should always regularly be assessed for good blood return and flushed with 10mL syringes. After placement of the catheter, she is to receive cytrabine and idarubicine. Cytarabine is an antimetabolite, which interferes with the biosynthesis of metabolites and acids necessary for RNA and DNA synthesis, as well as inhibiting DNA replication and repair (Sommer et al., 2013). Idarubicin is an antitumor antibiotic that interferes with DNA synthesis by binding DNA and preventing RNA synthesis (Sommer et al., 2013). CASE STUDY PAPER 5 These chemotherapy agents’ works to stop the replication of the leukemia cells and allows the body to repair and produce healthy blood cells again. There are important adverse effects to note for each of these medications and nursing interventions that follow. Cytarabine’s adverse effects include: liver disease, pulmonary edema, arachnoiditis (indications include nausea, headache, and fever), and it may reduce digoxin levels. The nurse should monitor liver enzymes, indications of jaundice, breath sounds, as well as advising the patient to notify the provider of shortness of breath, nausea, vomiting, headache, or fever. These manifestations may be treated with Decadron. Digoxin levels and EKG should also be monitored. extravasations off vesicants, and alopecia. The patient’s WBC, ANC, platelet count, and Hgb/Hct should be monitored; assess for bruising and bleeding gums, avoid crowds and contact with infectious individuals, administer antiemetics before beginning chemotherapy (Zofran), stop chemotherapeutic medications if extravasation occurs. Advise patients that hair loss will occur 7-10 days after beginning of treatment and will last for a maximum of 2 months after the last administration. Five days into her continuous infusion of cytarabine, her vital signs are reported: 110/54mm Hg, 115, 26, 101.6F. At this point, the nurse should perform neuro assessments; auscultate heart and lungs, and checking her CBC. Tachycardia is a symptom of anemia so monitoring her labs is important. The nurse should look for other signs of infection because although she does have a high temperature, she does have an increased WBC due to her AML so this could just be a contributing factor. A high temperature could also be the result of arachnoiditis; so close monitoring should be done. After notifying the intern of these findings, there are new orders written: blood cultures CASE STUDY PAPER 6 now x2 sites, Acetaminophen (Tylenol) suppository 650 mg q4-6h prn, Imipenem/Cilastatin sodium (Primaxin) 500 mg IVPB q8h, and notify the physician for a temp greater than 100.0F. As mentioned, blood cultures should be done to watch for a decrease in Hgb/Hct for anemia, decrease in platelet count for thrombocytopenia, increase or decrease in WBCs with differential. Acetaminophen will help control C.O.’s rising fever and if she were to experience headaches. Primaxin will work to treat any additional infection, and notifying the physician for a high temperature should be done as well. Catheter care should be continued per facility protocol and monitoring for adverse effects mentioned above. Regular vitals signs and assessing for bleeding and/or bruising, especially when her laboratory results have not been seen. One her last day of continuous chemotherapy, her laboratory findings were reported. Her WBC decreased dramatically to 1,200/mm3, as did her Hgb, Hct (6.8g/d, 21.3%), and platelet count (49,000/mm3 to 17,000/mm3 ) from admission. Her lymphocytes have increased and her monocytes have decreased from admission. Her neutrophil count increased by only 1% (4%-5%), but luckily her blasts have gone from 17% on admission to 5% and she has no evidence of bands in her blood. Looking at these lab findings, her calculated ANC is 60. Neutropenia is diagnosed for an ANC of 1,000 or less, so C.O. has developed neutropenia. Her WBC count and neutrophil count is abnormally low and her immune system has weakened, making it even harder for her body to fight off infection. Her decreased Hgb and Hct indicate anemia and her decreased platelets put her at a high risk for bleeding. Considering this data, she will most likely receive packed RBC’s and platelets. CASE STUDY PAPER 7 On day 14 after completion of her therapy, a bone marrow biopsy shows C.O. is in complete remission. This means that there are no signs of cancer in test, physical exams, and scans. This does not however, mean that you are cured. With continued blood product support and antibiotic coverage, her marrow recovers and she is discharged from the hospital. HLA (human lymphocyte antigen) typing has been performed on all siblings. Her oldest brother is a perfect HLA match and has agreed to donate bone marrow or stem cells. C.O. is to be readmitted to the bone marrow transplant unit within the next few weeks. In Williams’ “Comprehensive Review of Hematopoiesis and Immunology”, he explains that when bone marrow transplants (BMT) was done on patients in chemotherapy-induced remission, the mortality rate decreased, due to a lower relapse rate and a decreased rate of transplant-associated mortality. The risk of relapse is also lower when it is earlier in the course of the disease. Allogeneic Hematopoietic Stem Cell Transplantation (AlloHSCT) is from a donor other than the patient, including possibly a related donor such as a family member. C.O. will have this type of BMT done because her brother is going to donate. During this process, C.O. receives high doses of chemotherapy and possibly total-body irradiation to completely eradicate the bone marrow and any malignant cells and help prevent rejection of the donor stem cells. The collected HSCs are infused IV into the recipients and travel to sites in the body where they produce bone marrow and go through the process of engraftment to place themselves (Hinkle, 2014). Engraftment may take 2-4 weeks (or perhaps longer), and once complete, the new bone marrow becomes functional and begins to produce healthy RBCs, WBCs, and platelets. CASE STUDY PAPER 8 When undergoing a bone marrow transplant, C.O. will be at high risk for infection, sepsis, and bleeding, as well as possible side effects from the high dose chemotherapy (nausea, vomiting, fluid/electrolyte imbalance, etc.). Real complications can also arise from nephrotoxic chemotherapy agents. As the nurse, it is important to monitor vitals signs and blood oxygen saturation, assess for adverse effects such as: fever, chills, shortness of breath, chest pain, nausea, vomiting, hypotension or hypertension, tachycardia, anxiety, and taste changes. Her CBC should be monitored because until the body starts to produce healthy blood cells again, anemia, excessive bleeding, and infection become a higher risk. Skin care and mouth care should also be interventions. In any bone marrow transplant, patients are at a risk for graft-versus-host disease. In this disease, the donor cells view the recipient’s tissues as foreign. The donor lymphocytes initiate an immune response against the recipient’s tissues (skin, GI tract, liver) during the beginning of engraftment (Harris, 2010). Clinical manifestations of acute GVHD: rash progressing to blistering and appearance similar to second-degree burns, mucosal inflammation of the eyes and the entire GI tract with subsequent diarrhea that may exceed 2L per day, and biliary stasis with abdominal pain, hepatomegaly, and elevated liver enzymes progressing to obstructive jaundice. Complications that can occur during the first 100 days are encephalopathy, hemolytic uremia syndrome, hemolytic anemia, and thrombotic thrombocytopenia. Clinical manifestations of chronic GVHD: dry eyes or vision changes, dry mouth, white patches inside the mouth, fatigue, muscle weakness, chronic pain, joint stiffness, skin rash with raised, discolored areas, as well as skin tightening, SOB, vaginal dryness, and weight loss. CASE STUDY PAPER 9 Evidence/Research Based Support A retrospective study of 740 patients treated for grade II-IV aGVHD, 531 patients were treated with steroid and complete or partial responses were achieved in 44% of patients with improvement in skin, liver, and gut disease at 43, 35, and 53% (Jamil & Mineishi, 2015). Table 2 (below) shows the staging and grading of graft-versus-host disease. Steroid and CI remain the standard for initial treatment of the disease. In a peer reviewed journal article, a case study was discussed of a 43-year-old mother of four children diagnosed with AML. Throughout her hospitalization, she complained of severe pain, numbness and tingling, sweats, depression, and overall distress. She worried often and was anxious and had trouble sleeping. She received different medications to help relieve these symptoms. Psychological and emotional care given by the nurses was a main point in this article. Nurses would make sure to give her periods of rest in a quiet, dimly lit room. Emotional distress is common in patients with cancer and has been of interest to healthcare providers for decades (Holland, 1977; Weisman & Worden, 1976). Emotional distress is an established factor in patients’ decisions to reduce or stop treatment (Nerenz, Leventhal, & Love, 1982). Psychoeducational interventions can include education and supportive attention, which are recommended for the reduction of anxiety (Galway et al., 2012). Patients with this CASE STUDY PAPER 10 kind of disease need to be informed about the disease process, their medications, side effects, etc. to helping them understand and to help them get past certain barriers that are making them want to stop treatment or fall into depression. Summary/Conclusion Williams’ “Comprehensive Review of Hematopoiesis and Immunology” helped to give me a better understanding of the transplantation process. The advances of BMT have depended heavily on the availability of blood component support. (Williams, 2004). It also helped me to review the basics of understanding hematopoiesis and the disease process. In 2008 a study was done to test the effects of radiotherapy as a way to control joint pain in patients with AML. This was a peer reviewed journal article that observed an 80-year-old female’s pain before and after radiation therapy. Prior to the therapy, she was completely bedridden and standard pain management was unhelpful. With low doses of radiotherapy, she was able to participate in physiotherapy with minimal to no pain under her death several weeks later (Chakraborty et al., 2008). There are few cases to help support this evidence, but leukemic cells are relatively radiosensitive, hence the low prescribed dosage. The primary objective in treating patients with AML is to induce remission and thereafter prevent relapse (Lowenberg, 1999). Cytarabine is one of the hallmark treatments to induce remission, which was what C.O. was being given. Based off the literature that I found, targeting the malignant cells with therapeutic strategies is essential. After undergoing a BMT, nurses have to watch closely for beginning signs and symptoms of graft-versus-host disease. For those who develop chronic GVHD, steroids are the top treatment. Topical corticosteroids can be used for skin GVHD and an immunosuppressant CASE STUDY PAPER can also be prescribed as the condition worsens. It is critical to recognize and treat chronic GVHD early, before all other organs become affected. 11 CASE STUDY PAPER 12 References Albrecht, T., Walton, A. L., & Bryant, A. L. (2015). The Unique Supportive Care Needs of a Mother With Acute Myeloid Leukemia During Treatment. Clinical Journal Of Oncology Nursing, 19(1), 16-19. Chakraborty, A., Wells, R., Doherty, M., Huynh, C., & Selby, D. (2008). Joint Pain in AML: Successful Pain Control with Radiotherapy. Journal Of Pain & Symptom Management, 35(6), 670-672. Ferrara JL, Yanik G. Acute graft versus host disease: pathophysiology, risk factors, and prevention strategies.Clin Adv Hematol Oncol. 2005 May. 3(5):415-9, 428. Galway, K., Black, A., Cantwell, M., Cardwell, C.R., Mills, M., & Donnelly, M. (2012). Psychosocial interventions to improve quality of life and emotional wellbeing for recently diagnosed cancer patients. Cochrane Database of Systematic Reviews, 11. Harris, D.J. (2010). Transplantation. In J. Eggert (Ed.). Cancer basics. Pittsburgh: Oncology Nursing Society. Hinkle, J.L., & Cheever, K.H. (2014). Brunner & Suddarth’s Textbook of MedicalSurgical Nursing (13th ed.). Philadelphia: Lippincott Williams & Wilkins. CASE STUDY PAPER 13 Holland, J. (1977). Psychological aspects of oncology. Medical Clinics of North America, 61, 737-748. Jamil, M. O., & Mineishi, S. (2015). State-of-the-art acute and chronic GVHD treatment. International Journal Of Hematology, 101(5), 452-466. Lowenberg, B., Downing, J. R., & Burnett, A. (1999). Acute myeloid leukemia. New England Journal of Medicine, 341(14), 1051-1062. National Cancer Institute. (2012a). General information about adult acute myeloid leukemia. Nerenz, D.R., Leventhal, H., & Love, R.R. (1982). Factors contributing to emotional distress during cancer chemotherapy. Cancer, 50, 1020-1027. Sommer, S., Johnson, J., Roberts, K., Redding, S. R., Churchill, L., Ball, B., Henry, N. J., Leehy, P., & Roland, P. (2013). RN Adult Medical Surgical Nursing (9th ed). Weisman, A.D., & Worden, J.W. (1976). The existential plight in cancer: Significance of the first 100 days. International Journal of Psychiatry in Medicine, 7, 1-15. CASE STUDY PAPER 14 Williams, L. “Comprehensive Review of Hematopoiesis and Immunology: Implications for Hematopoietic Stem Cell Transplant Recipients” in Ezzone, S. (2004) Hematopoietic Stem Cell Transplantation: A Manual Nursing Practice Oncology Nursing Society: Pittsburgh, PA for