Acute Lymphoblastic Leukaemia - Case Study Example

LEUKAEMIA (A Critical Analysis of Patients End-Of-Life Journey Through Paediatric Intensive Care Unit from A Nursing Perspective) Introduction It was a tough challenge for the nurses to maintain competence in carrying out nursing care for a 6-year-old dying child named Therese, in the Paediatric Intensive Care Unit (PICU) having been diagnosed to have an Acute Lymphoblastic Leukaemia (ALL). This recent PICU admission is a relapse of Therese’s ALL initially diagnosed eighteen months ago. During the 18-month cancer treatment, both Therese and Arlene (the mother) struggled with bravery and strong fighting spirit to overcome this big test of faith. Arlene, though scared and weary, exuded strength to face life’s reality about dying. The PICU nurses showed professional competence in carrying out their role of providing holistic care to the patient and family. Eventually, Therese passed away due to severe thrombocytopenia secondary to leukaemia in relapse. Patient’s end-of-life journey will be discussed on this paper with overview of the patient’s illness from the time of diagnosis to the acute and chronic phases but mainly focuses on the terminal phase at the PICU and on how the nurses responded. History of Patient’s Present Illness It was a gloomy afternoon of April 2009 when Arlene, 32 years old and a single mother of a 6-year-old girl named Therese, first noticed two bruises on her daughter body, one on the arm and another on her thigh. Therese had intermittent fever for four days and looked pale and lacks agility according to Arlene. She used to be a very active and playful child but on this date Arlene noticed a big change on the child’s behavior. Therese loss appetite for food, she no longer joined playing around with her playmates, she was complaining of joint pain, and she became weak and always felt sleepy. Arlene decided to seek medical advice and on confinement to the hospital her daughter was medically diagnosed to have Acute Lymphoblastic Leukaemia. Leukaemia Concepts and Identifiable Risk Factors Associated with the Patient’s ALL Leukaemia, the most common cancer in childhood, is a group of cancer diseases of blood-forming tissues such as the bone marrow and lymphatic system. The problem in leukaemia is the production of an excessive number of immature white blood cells (WBCs). Crowding from these excessive immature cells compromises the production of other cells in the bone marrow. The leukaemias are categorized by subtype into two major classifications: acute lymphoblastic leukaemia (ALL), which is the most common type in children, and acute myelogenous leukaemia (AML), most frequent in adults and the most common form of leukemia overall. The primary difference is the type of leukocyte or WBCs involved. In ALL, the immature WBCs are lymphocytes, whilst in AML the WBCs involved are cells from the myeloid line, primarily granulocytes and monocytes (Johnson, 2010, p.155). ALL is considered acute because immature lymphocytes proliferate rapidly and because the disease is fatal without treatment. However, with appropriate medical intervention, survival rates for children with ALL now exceed 80% (Tecklin, 2008, p.536). Possible risk factors associated with ALL in children include environmental (ionizing radiation), genetic, and infectious (Belson, Kingsley and Holmes, 2007). Genetic risk factors are those that are part of our DNA. They are most often inherited from our parents. While some genetic factors increase the risk of childhood leukaemia, some cases of leukaemia are not linked to any known genetic causes (American Cancer Society, 2009). In Therese’s case, traced was a family history of cancer and a 3-month-old first cousin with G6PD deficiency both on paternal side. Glucose-6-phosphate dehydrogenase (G-6-PD) deficiency is a hereditary condition in which red blood cells break down when the body is exposed to certain drugs or the stress of infection (U.S. National Library of Medicine and National Institutes of Health, 2010). ALL and G6PD deficiency are both blood-related diseases. End-of-Life Care Concept for Paediatric Leukaemia Paediatric palliative care is a philosophy of patient care as well as an organized program for delivering care to children with potentially life-limiting illnesses. This care focuses on enhancing quality of life for the child and family, minimizing suffering, optimizing functions, and providing opportunities for personal growth. Paediatric palliative care is achieved by effectively managing pain and other distressing symptoms (such as fatigue, dyspnea, nausea and vomiting, anxiety, constipation, anorexia, depression, confusion), as well as providing psychological and spiritual care from the beginning (at diagnosis) that continues throughout the entire course of a childs life and beyond. Paediatric palliative care neither hastens nor postpones death; it strives to guide and assist the child and family in making decisions that maximize their quality of life (Jennings, 2005). Critical to understanding dying persons concerns is to understand both the patients and the familys needs in a variety of areas. These needs cut across many domains--physical (pain control); psychosocial (maintaining close relationships with others); spiritual (integrating or resolving spiritual beliefs); financial (overcoming the costs of medical or hospice care, having adequate funds to cover other financial obligations unrelated to care); and psychological (knowing about the illness and its course over time, talking over emotional difficulties, knowing that ones family is informed about the illness and that family members will be cared for well). Attending to as many of these needs as one can contribute to both the patients and familys quality of life (Hayslip, 2010). ALL Diagnosis and Treatment Prior to Relapse (Acute to Chronic Phase) An overview of the 18-month cancer treatment for Therese is crucial to better understand the series of events that may have significantly affect her final journey in the PICU. During the initial hospital confinement, CBC (complete blood count) diagnostic test of Therese revealed 171 (x 103/mm3) WBCs. According to American Academy of Paediatrics (2010), normal WBC level for children ages 5-8 years old ranges from 5.0-14.5 (x 103/mm3). Therese’s WBCs was marginally high compared to the normal level. Apart from the very high WBCs of Therese, the physician also noted many immature lymphocytes--indicative of presence of cancer cells. Another diagnostic test underwent by the patient was bone marrow aspiration (BMA). BMA procedure is done by a doctor who specializes in leukaemia by inserting a needle into a large bone, usually the hip, and removing a small amount of liquid bone marrow for examination under the microscope (National Cancer Institute, 2010). Therese’s BMA test resulted to positive leukemia cells in the bone marrow sample. Bone marrow relapse with or without extramedullary involvement usually points to poor clinical outcome (Brown, 2010). Definite with CBC and BMA diagnostic tests, Therese was medically diagnosed to have ALL. Dr. J.C. (the oncologist) told Arlene that as long as the treatment regimen is properly followed Therese has 70% chance of survival. Currently, ALL leukaemia is treated by chemotherapy, radiation therapy, or bone marrow transplantation (BMT) or cord blood transplantation (Science Education Resources at Carleton College, 2009). The aim of treatment for ALL is to destroy the leukaemia cells and enable the bone marrow to work normally again. Chemotherapy is the main treatment for ALL. Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (National Cancer Institute, 2010). Usually, a combination of chemotherapy drugs (and steroid medicines) is given according to a treatment plan often called a protocol or regimen (Macmillan Cancer Support, 2010). There are three phases of chemotherapy treatment for ALL: (1) induction phase (lasts about one month) to bring the disease into remission, (2) consolidation phase (lasts about four to eight months) to reduce the number of diseases cells left in the body, and (3) maintenance phase (if patient stays in remission after induction and consolidation) to destroy any disease cells that remain so that the leukemia is completely gone (LeMaistre, Shaughnessy, and Stein, 2010). With Therese’s ALL, she underwent 19 chemotherapy sessions and was on the maintenance phase of treatment when the relapse occurred. Her chemotherapy drugs include Cytarabine, Cytoxan (cyclophosphamide), Doxorubicin, and Trexall (methotrexate). Diligence, faith, fighting spirit, and optimism of the child and the mother were observed by the nurses all through out the 18-month treatment period. Emotional and spiritual strength were boosted through Arlene’s openness and cooperation to share her feelings to the parents of the other children having leukaemia and treated in the same health care facility, participating in religious activities of the church, and daily meditation. Arlene’s family and friends were also very supportive to her in matters of providing support. Nursing Care for Recurred ALL at the PICU with the Patient on Focus (Terminal Phase) The relapse of Therese’s ALL occurred 18 months after the initial diagnosis. Relapse, or recurrence of leukaemia, can occur anytime during therapy or after completion of treatment. Generally, it is more difficult to cure a child after relapse of the leukaemia; relapse during or soon after the completion of treatment is considered less favorable than relapse a year or several years after treatment (Ped-Onc Resource Center, 2009). Therese’s ALL relapsed because she missed a session of chemotherapy due to intermittent fever that eventually resulted to complications of pneumonia and sepsis, thus cancer cells enormously increased and resulted to multiple organ dysfunctions. BMA test showed 75% blasts of leukaemic cells in her bone marrow and lumbar puncture test also resulted to presence of leukaemic cells in her central nervous system (CNS). According to Kyle (2008), as the bone marrow expands or the leukaemic cells infiltrate the bone, joint and bone pain may occur. The leukaemic cells may permeate the lymph nodes, causing a diffuse lymphadenopathy, or the liver and spleen, resulting in hepatosplenomegaly. With spread to CNS, vomiting, headache seizures, coma, vision alterations, or cranial nerve palsies may occur (Kyle, 2008, p. 992). Patient symptoms during this severe stage upon assessment were pain, drowsiness, increased respiratory rate, loss of appetite, irritability, blood streak in the urine, bleeding gums, and blood clot on both eyes. Children with terminal cancer often experience a great deal of pain particularly when death is imminent. According to Wood (2008), pain has been identified as the fifth vital signs by Australian and New Zealand College of Anaesthetists and the Chronic Pain Coalition in an attempt to facilitate accountability for pain assessment and management. Whether the child has DNR order or the status remains that of “full code”, pain management is central to the nursing care of the child who is dying from cancer (Ricci and Kyle, 2008, p.1646). Ricci and Kyle (2008, p.1646) also cited Hooke et al.’s 2002 study, a position paper of The Association of Paediatric Oncology Nurses entitled “Pain Management for the Child with Cancer in End-of-Life Care” the following points for the nurses to consider: 1. Prevention and alleviation of pain is a primary goal of care in the child dying of cancer. 2. Children and parents are equal partners with members of the health care team in managing the patient’s pain. 3. Children dying of cancer may require aggressive dosing of analgesics. Medications that do not have a dose maximum should be escalated, sometimes rapidly, to achieve adequate pain control or to maintain pain control when tolerance has occurred. 4. The nurse’s role in caring for children who are in pain at the end of life includes assessment, identifying expected outcomes, and planning, performing, and evaluating interventions. All children with cancer will experience pain. In leukaemia, bone marrow expansion due to colony stimulating factor can be the cause of severe back pain (Fisch and Burton, 2007, p.210). Pain from the invasion of lymphoblasts into the periosteum and bleeding into the joints can be excruciating (Hatfield, 2008, p.497). Pain is a subjective symptom relevant to the patient’s feelings and perceptions with considering nonverbal signs of pain on assessment. There are various assessment tools used by clinicians to assess patient’s pain and these include: Visual Analog Scale (VAS), Numeric Pain Intensity Scale, Simple Descriptive Pain Intensity Scale, Graphic Rating Scale, Verbal Rating Scale, Pain Faces Scale, Numeric Pain Intensity and Pain Distress Scale, Brief Pain Inventory, and Memorial Pain Assessment Card (Leavitt, 2009). In the case of Therese, Paediatric Pain Profile (PPP) multi-dimensional pain assessment tool was utilized by the nurses. The PPP showed better intra-rater reliability than inter-rater, which suggests that it is more consistent when used by the same observer over time. As it was designed to be a parent-held document, this is in line with its intended use (Royal College of Nursing, 2009). The nurse’s pain assessments were documented with the last PPP result of 56 points, 60 points being the most painful. Analgesia was administered several times by the nurses per physician’s order, as well as other treatment medications prescribed. Total parenteral nutrition was continuously administered as ordered and close monitoring of vital signs were done. Laboratory blood test for CBC was followed up revealing results of 13 (x 103/mm3) platelet, 16 (x 103/mm3) hemoglobin, 55 (x 103/mm3) hematocrit, and 192 (x 103/mm3) WBCs. Whilst the patient was experiencing general body weakness in the PICU, the nurses paid attention on how to conserve patient’s energy in her activities. Sleep patterns were assessed and changes in thought process and behaviours were noted. The folks were instructed in energy conservation techniques. Activities like sitting on the chair and ambulation were encouraged only when the patient had already rested for a reasonable period of time. Food intake and appropriate nutritional supplement were encouraged. Constant monitoring of vital signs and monitoring of breath sounds to note feelings of panic or air hunger were done. For dependent nursing interventions, supplemental oxygen was always on stand by and being administered as needed, as ordered by the physician. Medications were administered per physician’s order. On the third day (last day) of Therese in the PICU, the patient seldom talk and was very weak and drowsy, so a great deal was given by the PICU nurses on the nonverbal signs of pain and behaviour observed and these were facial grimacing, moaning/groaning, restlessness, and agitation. Eventually, physical assessment revealed bleeding gums, streaks or blood in the patient’s urine and stool, blood clot on both eyes, pallor and some bruises on the skin. A significant sign of the patient’s farewell was noted by the nurse when Therese said to her mother her last statement as… “Momma, I’m sorry… I’m so tired now… I want to sleep”. At 9:00 AM, imminent death was determined by the doctor. All members of the healthcare team were informed about this medical appraisal, whilst the nurses’ ultimate goal was diverted and focused on providing comfort and peaceful journey towards the end-of-life. According to Jennings (2005), Nursing staff pre-bereavement sessions are held prior to the patients death. Education regarding how to talk to and support patients and families through the dying process is provided. Active bereavement sessions are held when a paediatric death occurs to provide the staff with a safe place to express their feelings and get support. The hospice bereavement counselor, music therapist, and chaplain facilitate the group discussions. A DNR order was made and discussed by the physician to the mother of the patient who eventually signed it in spite of great sorrow. By 11:00 AM, the patient’s body temperature rose to 39ºC and eventually resulted to septic shock. According to Tomlinson and Kline (2010, p.395) septic shock is a systematic response to pathogenic microorganisms in the blood (as cited by the authors from Brown, 1994). Children who have a compromised immune system from diseases such as human immunodeficiency virus (HIV), asplenia, or cancer chemotherapy have the greatest risk of developing septic shock. Tomlinson and Kline added (as cited from Bruce and Grove, 1992) that fever is the first symptom of possible sepsis; however, the febrile neutropenic child will not frequently demonstrate clinical symptoms of sepsis until after the initiation of antibiotic therapy. Nursing interventions during septic shock were the administration of mechanical ventilation and volume resuscitation as ordered and patient’s vital signs were assessed. According to Morrell, Micek, and Kollef (2009), the first priority in a patient who has severe sepsis or septic shock involves stabilization of the airway and breathing. Supplemental oxygen should be provided to the patient and institution of mechanical ventilation should be performed if necessary. Second, an assessment of perfusion should be performed. Blood pressure should be assessed by a sphygmomanometer or arterial catheter if blood pressure is labile. The nurses stood by the patient’s side for ongoing monitoring and nursing care. Nursing Care for the Patient’s Significant Others Whilst the patient was cared by the PICU health care team, her mother was also experiencing as much pain with the anticipatory loss of her precious child. The nurses were keen in providing as much comfort to the patient’s folks as possible to cope with the anticipatory grief, letting them feel that were not alone in this experience. The National Cancer Institute (2010) defined anticipatory grief as the normal mourning that occurs when a patient or family is expecting a death. Anticipatory grief includes depression, extreme concern for the dying person, preparing for the death, and adjusting to changes caused by the death. Anticipatory grief gives the family more time to slowly get used to the reality of the loss (National Cancer Institute, 2010). It is important that the nurse should understand the phases of grief [(1) Shock and numbness, (2) Yearning and searching, (3) Disorganization and despair, (4) Reorganization] so that appropriate nursing interventions be applied to the patient and family. In this particular case, anticipatory grief (anxiety) of death was managed by facilitating the development of a close and trusting relationship with the patient and family. Stage of grief currently experienced by the family was assessed properly and they were provided with explanation of the grieving process. An open and nonjudgmental environment was provided acknowledging the feelings of the patient’s significant others and portraying the roles of an active listener and patient advocate. The folks were encouraged by the nurse to verbalize their thoughts and concerns where expressions of emotions were acknowledge and accepted. The nurse maintained honesty and never gave false hope to them. Arlene remarkably elicited strength in coping as she never showed hostility or any other acting-out behaviour. Nevertheless, the nurses elicited strength in their personal awareness about the reality of dying. Upon reaching the reorganization phase of grief through nurses’ assessment of the patient and folks, the dying care nursing intervention was initiated by providing an open environment for discussion with the significant others about their desires or plans pertaining to death. The nurse assisted the significant others to identify their source of strength and upon identifying their need for spiritual strength and blessings for Therese, the nurse informed them that they can invite any spiritual advisor of their choice or they may prefer the health care facility’s in-house spiritual advisor to render spiritual blessings. An arrangement was made thereafter. The nurse offered information to the patient’s family about support group having similar experiences with which they can participate in, as well as bereavement counselors that they can keep contact with to support their grief process. Upheld was the high regard of patient and family’s autonomy as the nurse respected their decisions. Significance and Implications of Analysis and the Interrelationships of These to the Patient The ability of the nursing staffs and the other health care providers in supporting the patient’s significant others through the dying and grief process affects the family’s coping ability in carrying the circumstance of losing their loved one. Provision of supportive presence that conveyed empathy to the patient and family made them feel that they are not alone in experiencing this loss. The strength exuded by the nurse whilst providing comfort and care to the patient also became the source of strength for the patient’s folks. Upholding advocacy in matters that favored the patient and the family by providing them accurate and honest information, in a sensitive manner, enabled them to make the best decisions that met their needs. With proper nursing assessments, correct nursing interventions were given to the patient and to the significant others and their needs as to physical, spiritual, psychosocial, and psychological (emotional) aspects of facing a tragedy in life were answered. Providing good physical care to a dying patient is a nursing responsibility and this few remaining days of the patient’s life was the best time that love and pampering was showed to her the most. Thus, dying was journeyed by the patient gently with as much as no anxiety of pain as possible whilst the family was eventually coping to accept the reality of death. The end-of-life journey of the patient was met with dignity and peace and the family was systematically assisted and supported in their grief and bereavement process. Implications and Recommendations for Future Practice When doctors has nothing left to do about an imminent dying of the patient, the responsibility to take care of the patient’s end-of-life needs and family’s coping are handed on to the nurse. Among all the health care providers, it is the nurses who have direct and constant contact to the patients and families. Handling a patient in the PICU with an acute illness and whose death is imminent is a tough challenge on the part of the nurse because there are so many things to do and think about but with an overall goal of providing utmost patient care all along to the end-of-life journey. In spite of this overwhelming responsibility for the nurse, being one of the very significant persons on the life of the dying patient should be considered as blessing and a realization of self-worth. In PICU setting, technical competence and intellectual initiative in providing skillful and prompt care procedures are prerequisites for nurse quality care. In-depth research and analysis of other scholarly literatures and evidence-based researches are recommended to the nurses for enhancement of knowledge of up-to-date nursing quality care so that overall competence in the area of professional practice shall be achieved. Bibliography American Academy of Paediatrics, 2010. Normal laboratory values for children [online]. Available at: [Accessed 27 November 2010]. American Cancer Society, 2009. Childhood leukaemia [online]. Available at: [Accessed 02 December 2010]. Belson, M., Kingsley, B., & Holmes, A., 2007. Risk factors for acute leukaemia in children: A review: risk factors. WebMD LLC [online]. Available at: [Accessed 28 November 2010]. Brown, V.I., 2010. Acute lymphoblastic leukaemia: Excerpt from the 5-minute paediatric consult [online]. Available at: [Accessed 06 December 2010]. 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