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CHAPTER I INTRODUCTION

Health is a complex phenomenon. The World Health Organization (WHO) has defined health as a state of complete physical, mental and social well-being and not merely the absence of disease. Despite this broad definition, health is traditionally assessed by observing mortality (death) and morbidity (illness) rates. Therefore the balance and the presence of disease becomes the prime indicator of health. (Hockenberry and Wilson, 2007) Beliefs about health and illness are a major feature of every known culture. Health is often viewed as a continuum on which optimal wellness, at one end, is the highest level of function, and illness, at the other end, results in death. Every person is somewhere on the continuum. A change in an individual health state can also change his or her location on the continuum. (Ignatavius, 2006) Illness is the stage during which specific symptoms are evident. Most illnesses have local symptoms related to the body organ affected and also systemic symptoms that affect the entire body, such as fever, increased white blood cell count, or headache. Many childhood infections have an accompanying rash on the skin (exanthema) or mucous membrane (enanthem). (Pillitteri, 2007) Pediatrics is defined as the branch of medicine that deals with the childs development and care and the diseases of childhood and their treatment. Family-centered care recognizes the strength and integrity of the family as the core of planning and implementing health care. The family as caregivers and decision makers are an integral part of both obstetric and pediatric nursing. (Leifer, 2007) Pediatric nursing or child

health nursing is the specialty nursing care of babies, children and adolescents. A nurse who specializes in this area is usually referred to as a pediatric nurse. The spelling paediatric nursing is more common in English-speaking countries outside the United States. The breadth of this field of practice may be considered as similar to that of adult nursing, with an equivalent sub-specialty for most adult illnesses and some unique areas of practice. For example, pediatric cardiology and oncology are both established subspecialties in most major pediatric facilities. Pediatric nurses play an important role in making children better. A pediatric nurse is the one responsible for rendering care and building rapport with a pediatric client. They can make a scary experience for children and their families much more bearable. (Upson, Margo 2012) Pediatric clients are within the age group of neonate, infant, child, and adolescent. A neonate is considered to be full term when it is born between 38-42 weeks. An Infant ranges from 29 days to 1 year of age. Child/children ranges from 1 to 12 years of age. A child is considered as an adolescent when his/her age is within 13 to 18 years of age. (W.A., 2012) There are many diseases and disorders seen in a pediatric practice. These are the ten usual illnesses that pediatric clients have: Asthma, Eczema, Gastroenteritis, Impetigo, Otitis media, Pinworms, Scabies, Strep throat, Urinary tract infection, and Dermatitis. (Pediatrics.com,2012) Minor illnesses, such as colds and intestinal disturbances, are common, especially in the early years: children from age 0 to 3 years experience six to nine illnesses per year. From age 4 to 10 years, children develop, on average, four to six illnesses per year. (MedicineNet, 2012)

Infants developmental task is to form a sense of trust versus mistrust. Child learns to love and be loved. Toddlers developmental task is to form a sense of autonomy versus shame. Child learns to be independent and make decisions for self. Preschoolers developmental task is to form sense of initiative versus guilt. Child learns how to do things and that doing things is desirable. School-age childs developmental task is to form a sense of industry versus inferiority. Child learns how to do things well. Adolescents developmental task is to form a sense of identity versus role confusion. Adolescents learn who they are and what kind of person they will be by adjusting to a new body image, seeking emancipation from parents, choosing a vocation, and determining a value system. (Pillitteri, 2007) Hirschsprungs disease is a congenital anomaly that results in mechanical obstruction from inadequate motility of part of the intestine. It accounts for about one fourth of all cases of neonatal intestinal obstruction. The pathology of the Hirschsprungs disease relates to the absence of ganglionic cells in the affected area of the intestine, resulting in a loss of the rectosphincteric reflex and an abnormal microenvironment of the cells of the affected intestine. (Hockenberry, 2007) Hirschsprung disease occurs at an approximate rate of 1 case per 5400-7200 newborns. The exact worldwide frequency is unknown, although international studies have reported rates ranging from approximately 1 case per 1500 newborns to 1 case per 7000 newborns. Both So et al and Leon et al have studied the mutational spectrum of the disease in China. Approximately 20% of infants will have one or more associated abnormality involving the neurological, cardiovascular, urological, or gastrointestinal system. (Lee, 2012)

Clinical manifestations vary according to the age when symptoms are recognized, the length of the affected bowel, and the occurrence of complications such as enterocolitis. In the newborn period, abdominal distension, vomiting, constipation, and failure to pass meconium within the first 48hours of life are likely to occur. Neonates may also have signs of acute intestinal obstruction, including abdominal distention and bilious vomiting. Some infants have abdominal distention that is relieved by rectal simulation or enemas. (Hockenberry, 2007) The majority of the children with Hirschsrpung disease require surgery rather than medical therapy with frequent enemas. Once the child is stabilized with fluid and electrolyte replacement, if needed, surgery is performed, with a high rate of success. Surgical management consists primary of the removal of the aganglionic portion of the bowel to relieve obstruction, restore normal motility, and preserve the function of the external anal sphincter. In most cases this accomplished in two stages. First, a temporary ostomy is created proximal to the aganglionic segment, to relieve obstruction and allow the normally innervated dilated bowel to return to normal size. (Hockenberry, 2007)

PURPOSE AND OBJECTIVES This case study aims to present the nursing care of a male pediatric neonate client diagnosed with Hirschsprungs disease. Specifically, this case seeks to achieve the following objectives: 1. Identify the factors that lead to the development of the problem which is Hirschsprungs disease.

2. Analyze the interrelationship of factors that leads to the development of Hirschsprungs disease. 3. Explain the interrelationship that leads to the patients problem 4. Discuss the relevant interventions that were utilized to resolve the problem. 5. Describe evaluation of care through patients responses

SIGNIFICANCE OF THE STUDY The results of this case study are intended to give benefit to the following people concerned: To the Client. Being the primary source of quality nursing care, the outcome of this case study will initiate an alternative therapeutic approach in rendering care with his present condition. To the Family With Similar Case. This case study will help in improving their awareness as each in the family constituents. It also aims to help them identify and understand the causes of such illness and somehow intends to initiate points to consider as to looking after their sick child with Hirshsprungs disease. This case acknowledges each intervention utilized and/or shall be utilized for the patient. The family would have a better background of what the patient will be experiencing and whatever care needed for the patient. It also would somehow allow significant others to accept whatever event or condition the family will confront in the near future, allowing them to cope in a more challenging and therapeutic manner. To the Health Care Providers. this study aims to provide additional information regarding the disease with the same case with similar condition and will somehow

provide updated trends and issues related to giving atraumatic form of management that may eliminate or at least minimize the psychologic physical distress experienced by the family in the health care system while handling a case with Hirshsprungs Disease. To the Student Nurses. this study may serve to provide learning experience and relevant information regarding effective nursing care and management as well as the variation in the strategy and techniques of handling clients with similar case. This may also serve as a basis of information regarding an actual learning condition allowing and inspiring the student nurses to embrace the challenges of clinical experience in a pediatric unit. To the Future Researchers. this study will serve as a reference for clinical condition with Hirshsprungs Disease and this may serve as a basic guide in meeting a better care plan and may give a better baseline data of Hirshsprungs Disease in an actual application

SCOPE AND LIMITATION This is a case study which focuses on the care of a male neonate client with Hirschsprungs Disease. The patient receiving the focus of care is J.K.A., a 21 day old male. The researchers utilized the retrospective A type of approach for this study because we obtained data through assessment of the client and interview with the clients relatives. Data was gathered from the medical records daily interactions, charts, and medical records wherein actual and potential problems were identified. The clinical focus is the care of clients across the lifespan with oxygenation, fluid and electrolytes and metabolism disorders.

The scope of this study was focused in meeting the needs of a male neonate client with Hirschsprungs Disease. Assessment was done to determine the possible nursing diagnoses that would fit to the clients condition. The researchers were able to plan possible nursing interventions that could help to maximize level of functioning of the client. The study was done through a face to face contact and assessment of the client in between the time of 0600H-1400H from July 09, 2012 until July 11, 2012 and 1400H2200H from July 16, 2012 until July 18, 2012 at the 6th floor (front) of a tertiary hospital in Makati. Data was gathered through the clients medical record, interview using forms, observations and interaction of the researcher with its family. The data gathered from the patient was handled with confidentiality. The limitations of the study was that the client was only handled for 6 days, thus further assessment before the contact of the researchers were only from medical records like the patients chart. The researchers only had an opportunity to interview the mother of the client. The researchers were not able to trace genetic disposition of Hirschsprung's disease and trisomy 21 for paternal and maternal side. During the interview the mother denied any family history of Hirschsprung's disease and/or Trisomy 21 and the researchers were not able to interview the father. Hence verbalization of the mother and family was very much important because it can add up to our studies about the history and other factors on how the client developed the condition. The researchers were not able to retrieve the results of karyotyping because it was done on other hospital. The results will help the researchers to determine the genetic variants that could possibly lead to the patient's condition. The researchers weren't able to retrieve the statistical data of cases of Hirschsprung's disease in the Philippines because the DOH werent able to

present a data regarding Hirschsprungs disease statistical. The evaluation of the nursing interventions performed was focused only on the time of actual handling of the client. BACKGROUND OF THE STUDY The study was conducted in a private tertiary hospital in Makati City. The said hospital is well-known because of its high standards of giving care and high standard facilities. It is equipped with up-to-date equipments that go with the modern technology to cater medical needs of its patient and meet their expectations. The institution also has highly- qualified staffs and capable licensed health care provider to offer outstanding services to its clients. The hospital conceptualized the total management for the clients satisfaction. The institution aims to be internationally acclaimed when it comes to standards and services. The gathering of data was done at the Nursery in the 6th rectangular of the said hospital. It is subdivided into 6th front and 6th rear. The 6th front was composed of small private rooms. There are designated nurses station for each 6th rear and front. This area is composed of an organization which includes consultants, resident physicians, interns, unit manager Ms. H.P., charge nurses, staff nurses, ward clerks, nursing aides or orderlies, technicians and janitors. It has five rooms. Four of the rooms are subdivided into five rooms; the two of them can be occupied by two patients at the same time. The remaining room is subdivided into two rooms. In the medication room, they have drug cart, thermo scans, and an emergency cart which is composed of different medications and tools. Racks for holding patients charts are present in the nurses station so they can easily refer to the chart whenever there is a new doctors order. They also provide wheel chairs and push carts for patients who are to be discharge. There is also a pantry in the

middle of the rectangular and has an area where soiled linens are placed. The hospital's department of pediatrics is committed to providing exceptional health care to pediatric patients. The researchers are on duty during Mondays to Wednesdays for three weeks. They are assigned in the morning shift from 0600H to 1400H. The researchers offer their services to the fullest for their patients satisfaction. As part of the morning care rendered to the patients, student nurses together with the nursing aides are required to change the linen in every patients room. They are also to take part on bed bath and oral care of the patient. The other procedures that the student nurse should perform during their shift includes interview, focus assessment in a cephalocaudal method, documentation, Physical examination, completion of

vital signs monitoring, intake and output

monitoring, assisting in administration of medications, observation in administration of intravenous fluids and monitoring IVF. These interventions encompass the holistic care of the clients in terms of promotion, prevention, curative and rehabilitative care. In one of the small private rooms, located at 6th floor front of the hospital, is where the study was conducted. It has a warmer where the patient will be lying while receiving his/ her treatment, a crib, a television set, a chair and a bedside table. The researchers chose the client, a male neonate pediatric client diagnosed with Hirschsprungs disease with Trisomy 21 to be studied because they believed that this case is an opportunity to learn not only merely the detailed medical surgical case it gives about Little People but also there are characteristic health problems peculiar to each nature of development. Also, it gives the researchers an opportunity to formulate nursing problems recognizing both the vital role that families play in childs life, growth and development.

It also allows the researchers to formulate management to other pediatric clients with similar health condition and to determine appropriate nursing interventions to be rendered. The researchers also believe that they will be gaining new experiences as they learn and explore more about the case.