JENNY COSTE This is the case of Jenny Coste, 9 years old and was diagnosed with Acute Lymphoblastic Leukemia. She complains pain in her legs and loss of appetite early July of 2013. She plays sports in her school and her family thought it might be the cause until she feels tired all the time and does not want to participate in school activities. Her symptoms become severe and decided to see the doctor. In early September of 2013, they visit their General Practitioner, Dr. Brown and she was observed to be very pale and has rapid respiration.
Diagnostic tests and laboratories was ordered and finally diagnosed to have acute lymphoblastic leukaemia. She undergoes intensive chemotherapy and have been in and out of the hospital for 18 months. She had remission for a fleeting period, but unfortunately a relapse occurred. Her health condition swiftly dropped despite assertive effort of treatment until her family decided to put her in palliative care. Acute Lymphoblastic Leukemia or ALL is one most common blood cancer in children less than 15-year-old of age. Jenny’s diagnosis is acute lymphoblastic leukemia.
Ordinarily, blood cells are emerging from an immature cell or stem cell which can give rise to several different cell types. First phase of hematopoiesis or formation of blood cells is the separation into lymphoid and myeloid cells. Secondly, these cells then form their own blast cells, the antecedent to the final mature cells. Moreover, develop cells of the myeloid lineage produces monocytes/macrophages, erythrocytes, megakaryocytes/ platelets, neutrophils, eosinophils, and basophils. Develop lymphoid cells produces T lymphocytes, B lymphocytes (which additionally separate into plasma cells), and, perhaps, normal iller cells.
As a last point, the cells continue to go under the process of mitosis in which chromosomes of DNA forms normally and mature cell produces. As development of normal hematopoiesis continues, there is the potential for one cell to change and divide rapidly skipping the normal growth signals (Spizer 2010). Huang and Yen (2011) stated that “chromosome instability can result in aneuploidy”. Aneuploidy happens when chromosome does not separate properly between the two cells regardless of whether their chromosomes are properly attached to the spindle or not.
This faulty segregation of chromosomes allows cells to quickly change gene expressions patterns and develops its own mechanism to survive growth conditions. In ALL, malignant transformation of an immature blast cell, specifically originates in lymphoid cells, abnormally multiply quickly. Pathophysiologically, leukemic proliferation in the bone marrow leads to crowding out of normal hematopoeitc cells, leading to bone marrow failure (Spizer 2010). Consequently, normal bone marrow cells may be displaced and replaced.
The growth in the size and amount of marrow and/or this infiltration of leukemic cells expands outside the bone marrow causes bone and joint pain therefore explain Jenny’s symptoms of leg pain. Additionally, red cell precursors and megakaryocytes from which platelets are formed decreased, leading to anaemia, bleeding and bruising (Nair & Pete 2013). Jenny observed to be very pale and increased in respiratory rate. The respiration increases due to compensation of the respiratory system in a reduction of red blood cells which carries oxygen in the blood.
Furthermore, decreased normal white cells makes the patient liable to pick up infections which may be in relation to unexplained fever. In addition, invasion of the central nervous system of leukemic cells can lead to headaches, vomiting, cranial nerve palsies, convulsions and coma. Similarly, weight loss, muscle wasting and fatigue occur when the body cells are deprived of nutrients because of the immense metabolic needs of the proliferating leukemic cells. Jenny has signs and symptoms including of feeling tired, loss of appetite, lethargic and being irritable.
One of the most predisposing factors which a medical expert may suspect an acute lymphocytic leukemia is the abnormality in Jenny’s white cell count coexisting with her signs and symptoms (Nair & Pete 2013). The exact cause of Acute Lymphoblastic Leukemia is unknown, but there are following suspect being involved in the development of this disease such as environmental causes, infectious agents (especially viruses), genetic factors and chromosomal abnormalities (Nair & Pete 2013).
The premedical history of Jenny having varicella when she was three years old may play a role in the development of the disease but still needs further investigation. Various laboratories and diagnostic tests will confirm and detect the prognosis of the patient, such as bone marrow biopsy and lumbar puncture. The essential diagnostic feature of acute leukemia is a hyper-cellular bone marrow with greater than 30% replacement of blast cells according to Nair and Pete (2013). Since ALL progresses rapidly, it needs treatment as soon as the disease is detected.
The treatment will depend on the subtype of ALL, age and general health of the person and the genetic make-up of the leukemic cells (Nair & Pete 2013). Acute Lymphoblastic Leukemia includes an intensive treatment such as radiotherapy and chemotherapy. The time required to eliminate the large leukemic-cell population to undetectable levels is the single most powerful prognostic factor in ALL in children. Jenny’s choice of treatment consists of a common backbone of chemotherapy, including vincristine, anthracyclines-doxorubin and prednisone.
According to RoseIhnman and Kuehl (2014), the effectivity of this combination can target a remission in up to 90% of patients. However, Hunger and Mullighan (2015) believes that a survival rate of 10 to 20% of almost all children with relapsed occurs, commonly in the central nervous system. The vincristine mechanism of action is not precise, but appears to “bind to crystallise critical microtubular proteins of the mitotic spindle, thus preventing their proper polymerization and causing metaphase arrest” therefore blocking the cell division.
On the other hand, doxorubicin acts as an interference to “DNA-dependent RNA synthesis”. It distorts DNA leading to cell apoptosis. The use of dexamethasone versus prednisone varies among groups of ocorticoids has pros and cons. Dexamethasone is associated with greater untoward side effects than prednisolone. Although, dexamethasone exhibits more activity since it can penetrate blood brain barrier, it can also cause greater adverse effect (aba & ui 2010). In studies of Inaba and Pui (2010), there is no difference noted in efficacy of the two drugs.
Traditionally, the goal of treatment was to decrease the blasts cells in the bone marrow of <5% and achieve morphologic remission. Approximately, 80% – 90% of children achieved complete remission. The availability of drug modification has been made apart from induction regimens of vincristine, anthracycline and glucocorticoid (Rabin & Poplack 2011). Adams et al. (2011) made an evaluation tool called QuEST Tool to determine the quality of life in patients receiving steroids particularly in children with acute lymphoblastic leukemia.
The focus of the study involves the determination of adverse effects of corticosteroids in long term or high dose therapy apart from physical side effects such as mood, behavior, cognition, body appearance and image as well as family relationships. This information can help in future drug development towards negative side effects of corticosteroids. Considering the age of Jenny, the degree of side effects of chemotherapy depends on her well-being during treatment. The target of drugs incorporated into her is to kill the leukaemic cells but it can also harm the normal cells resulting to neutropenia, thrombocytopenia and anaemia.
Eventually, the body will get weak and series of drug adverse effect worsen it. Although she achieved a remission after 18 months, it did not last long. A relapse occurred not long enough after the remission which occurs in 15 to 20% of children with ALL. Even more, the prognostic factors in relapse includes the time of onset which believed that a shorter time is associated with a worse prognosis (Hunger & Mullighan 2015). As was previously stated, the prognosis of Jenny is not good based on the onset of relapse. Her health progress continuously declining and her family and healthcare prepared a palliative care team.
Palliative care assists people to live a quality life and supports a comfortable life as possible. It includes treatment of symptoms which may be physical, emotional, spiritual or social. Jenny was discharged from the hospital and her family needs a thorough information regarding care for her. Assistance from health care professionals, community and home care specialist should be available for palliative care to work. For decades, researchers have been looking for answer on how to prevent the risk factors in relation to cancers in children. Several hypotheses remain controversial of the cause of leukaemia in pediatrics.
On the other hand, Ajrouche et al. (2015) further study the role of early stimulation of the immune system to decrease the risk of childhood ALL. It includes factors related to strengthening the immune system such as breastfeeding, normal delivery and exposure to animals in early life. The outcome of their review does not support the theory that cesarean segment is identified with ALL, however, has produced additional proof that breastfeeding, common infections, and customary contacts with animals in infancy, all of which are conditions in boosting the immune system, may have a protective role regarding ALL.
In conclusion, Jenny’s case is among 10-20% of children who have not achieved complete remission. The development of treatment to cure acute lymphoblastic leukaemia in children has been improved throughout the years. There is limitation to the case study of Jenny since it does not provide detailed information of the course and condition during her treatment. Unfortunately, pediatric malignancy, affects also the family psychologically, emotionally and financially. In the end, healthcare, family and community support is essential in achieving better palliative care.