Specifics of nursing care for patients with spinal muscular atrophy

This thesis is focused on defining the specifics of nursing care for patients with disease spinal muscular atrophy. SMA is classified as an incurable genetic disease that causes progressive muscle atrophy and deformity of the body but sensory perception and intellect remain preserved. The incidence of the disease in the neonatal and childhood prevails, however the outbreak of the disease in adulthood is not any exception. Suitable nursing care is of great importance in maintaining the health of the patients for the longest possible time and delaying the onset of worsening health. The thesis is divided into theoretical and empirical part. The theoretical part deals with SMA disease, incidence, classification, treatment options and nursing care. In connection with the theme of this thesis, we choosed three targets. 1.: Map out the specifics of nursing care of pediatric patients with spinal muscular atrophy from the perspective of nurses. 2.: Map out the specifics of nursing care of adult patients with spinal muscular atrophy from the perspective of nurses. 3.: Comparison of the specifics of nursing care of pediatric and adult patients with spinal muscular atrophy. We used qualitative research method using a semi-structured interview to achieve our goals. To assess quality goals, we used the following research questions: How nurses specify the nursing care provided to a pediatric patient with spinal muscular atrophy? How nurses specify the nursing care provided to an adult patient with spinal muscular atrophy? With which needs of patients suffering with spinal muscular atrophy have the most experienced nurses experience? The research group consisted of six respondents, three of which respondents had experience with care of pediatric patients and the other three with care of adult patiens. As a processing technique we used open coding, through pencil and paper. The information obtained was divided into categories. The research shows that for nurses caring for pediatric patients the age is important at first occurrence of the disease as that influences the possibility of communication. Prevails ensure physiological needs. For nursing care, throughput of the tracheostomy cannula and status check of the oral cavity is important. In terms of nutrition the nurses prefer serving food through the PEG. They also agreed on the need for positioning. Emptying urine is ensured by the PMK or urinating in diapers. The nurses agreed on serving laxatives and manual defecation. The survey of nurses caring for adult patiens explains that the most common barrier is a state of consciousness, mental status and impaired verbal and nonverbal communication. For the needs of the nurses they state the physiological needs, as well as the fulfillment of psychological needs. In the nursing care of respiratory ways nurses report caring for tracheostomy cannula and a suctioning. In the case of nutrition they prefer per os (orally) and after that they use the help of NGS or NJS. The most common way of serving the diet they indicate the PEG. In case of immobility of the patient they all agree on positioning. For emptying urine they use PMK, one also mentioned epicystostomy. For defecation they agree on serving of laxatives, enemas and manual defecation. The results of our survey highlight the differences in nursing care provided to children and adults. The most relevant in the category of barriers in communication, is the age of the children and adults state of consciousness, the willingness and the ability to communicate. Adult patients desire to fulfill even higher requirements,while with children it is about fulfilling the physiological and social needs. In the nursing care, significant differences are not found. The results obtained in the practical part, I would like to use the knowledge to broaden awareness of nurses who have experience in caring for patients with SMA and to better inform the general public about this rare and often fat