Case Study: Cystic Fibrosis Sample
James is nine years old and has cystic fibrosis. James was admitted to the hospital for cystic fibrosis treatment on November 19th at 21:00 p.m. He is currently on Isolation Precautions. James is the only child of mother Yvonne and father Sam. Yvonne is a stay at home mom who spends majority of her time focusing on James cystic fibrosis treatment. James’ father Sam works outside the home, and he often has a long commute to and from his work, but he tries to spend with his son as much time as he can.Case Study: Cystic Fibrosis Sample
Cystic Fibrosis (or CF) is a genetic disease that causes persistent lung infections and limits the ability to breathe over time. CF affects the secretory glands, which are responsible for making mucus and sweat in the body. The CF gene defect causes a thick, sticky buildup of mucus in mainly the lungs and pancreas. In the lungs, the thick mucus buildup clogs the airways and traps bacteria. This effect leads to lung infections, lung damage, and eventually, this will lead to respiratory failure. In the pancreas, the mucus prevents the release of digestive enzymes that allow the body to properly break down food and absorb nutrients. People with CF might experience bulky stools, intestinal gas, or severe constipation. CF also causes the body to become very salty. Thus, when a person with CF becomes sweaty, one loses a large amount of salt. To explain, this can disrupt the balance of the body’s minerals in the blood flow, which can cause an increase in the heart rate, fatigue, weakness, decreased blood pressure, and a heart attack. According to Cystic Fibrosis Foundation (2018), individuals affected with CF may have the following symptoms: “very salty-tasting skin, persistent coughing, frequent lung infections including pneumonia or bronchitis, wheezing or shortness of breath, poor growth or weight gain, frequent greasy, or difficulty with bowel movements, infertility issues, and CF diabetes”.Case Study: Cystic Fibrosis Sample
The cause of CF is a defect in the CTFR (cystic fibrosis trans membrane regulator) gene. This means that CF is a disease caused by a defect in one gene that controls the movement of salts in the body. Each person who inherits the CFTR gene has to receive this gene from both parents. Individuals with only one copy of the defective CF gene are carries but they do not have the diseases themselves. If both parents are CF carriers, the chances of having a child who will have CF are 25% (1 in 4).
According to Cystic Fibrosis Foundation (2018), cystic fibrosis affects more than 70,000 people worldwide, and more than 30,000 living in the Unites States. Moreover, Cystic Fibrosis Foundation (2018) also stated that there are 1,000 new cases of CF diagnoses each year. This disease is usually diagnosed at the age of two. In order to diagnose CF, newborns receive a genetic test or a blood test. A genetic test shows whether a newborn has the CTRF defect genes. A blood test shows whether pancreas is functioning properly. To confirm whether an individual has CF, the physician need to conduct a sweat test to measure the amount of salt in the sweat. Chest Foundation (2018) mentioned “children with CF have high levels of chloride in their sweat because a lack of CFTR prevents the salt on the skin from being reabsorbed back into the sweat glands”.
People who live with CF need to be ready to frequent hospital visits due to the high risk of getting lung infections because of the thick, sticky mucus buildup in the lungs allowing germs to thrive. In the pancreas, the mucus can also stop the absorption of food nutrients to the body. With advancements in modern medicine, there are several ways of treatment to improve the quality of life for an individual with CF.Case Study: Cystic Fibrosis Sample
To date, there is no known cure for CF, but there are various treatment methods, which may help to significantly improve the quality of life for a person with CF. These procedures should be performed daily inside and outside the hospital. Treating the respiratory tract is important to slow down the long-term damage caused to the lungs due to CF. People with CF need to use “airway clearance therapy” (ACT). According to Chest Foundation (2018), it is possible to use a manual chest physiotherapy device called a “VEST” for implementation of this treatment. The Vest is worn like a jacket that shakes and vibrates when it is turned on. The Vest is used to shake mucus for the purpose to help clear the airways, enabling the individual to cough up the mucus. The Vest is a portable device that an individual can use in the hospital and at home.
Another ACT device includes a portable electric chest clapper that is known as a mechanical precursor, which may help to clear the airways of mucus. Moreover, various breathing techniques also may help to clear airways and dislodge mucus. The techniques include a couple of deep breaths followed by relaxed breathing. In the form of a nebulizer or inhaler, mucus-thinning medication is a part of daily treatment for people with CF. It helps to relax the airways and clear out mucus buildup, as well as thin the mucus so that the individual can cough it up more easily, and to help restore moisture and salt to the airway.Case Study: Cystic Fibrosis Sample
The next method of treatment includes the pancreatic enzyme replacement therapy. This medication helps the body to absorb the necessary nutrients from food. People with CF are recommended to eat a high fat, protein, salt, and calorie diet to keep up with the function of the lungs. Pancreatic enzyme medication is taken before every meal and snack to help digest food. American Lung Association (2018) stated that antibiotics are frequently used to prevent and treat bacterial infections in the lungs. It is possible to use antibiotics orally or by inhalation. If the individual is very ill then it is possible to use antibiotics intravenously (IV).
In addition, aerobic exercises are also important to improve the quality of life for a person with CF. Different exercises help to dislodge the mucus and clear the airways so the individual can cough it up. Moreover, exercises may help to improve the overall health condition, mood, and quality of life.
In the most severe cases of CF, lung transplantation is an option. John Hopkins Cystic Fibrosis Center (2018) mentioned that lung transplantation is not a cure for CF or lung disease. Hartert et al (2014) stated that “the average five-year survival after lung transplant is about 60%, and additional medical problems may lower life expectancy further”. However, CF treatment procedures can help to prolong the individual’s life span. Currently, the average life expectancy for an individual with CF is 37 years.
In general, children affected with cystic fibrosis live normal daily life with the challenge of incorporating daily treatments, medications, and airway clearance techniques. Children who have CF go to school, have friends, have hobbies, participate in after school activities, and play sports. Many individuals with CF grow up and attend college, as well as create their own families. Case Study: Cystic Fibrosis Sample
It is always important to remember that CF treatment can take a lot of time from both the child and the whole family. The inclusion of CF treatment methods into a daily life helps to cope with this disease, allowing the family to establish a routine. Allowing siblings to participate in various physical exercises with their sibling who has CF can bring the family unit together. It is very important for the child and family to find a balance between CF and healthy childhood development. Children with CF are still kids, and they need to play with other children, as well as be active in these games. However, the parents need to be mindful and always remember that a child who is diagnosed with CF has specific needs. The mission of parents also includes a necessity to create good relationships with any school or extracurricular staff members because these people may help to manage daily CF treatment of their child. As a result, such an approach may allow the child to participate in normal daily activities, while ensuring compliance with all safety and health recommendations.
James is nine years old, and according to Erik Erikson, the boy is at the industry vs. inferiority psychosocial stage of development. The peer group gains a great significance for school-aged children, as well as this is a major source of self-esteem for them. This period of psychosocial development is characterized by the necessity to explore own abilities and achieve some specific goals. Considering these facts, it can be said that while James is hospitalized on isolation precautions, this might affect his confidence and self-esteem. It means that James’ hospitalization takes the boy away from his normal peer groups. James is missing school activities; he does not participate in hobbies, and is removed from his home environment. In such a way, a child life specialist should analyze these facts and to consider them, when coming up with effective interventions for James to participate in different activities.Case Study: Cystic Fibrosis Sample
Child life specialists play a huge role in the process of child development because they provide psychosocial support for children and their families in a hospital setting. The child life specialists are able to provide medical teachings to the patients and their families in order to help the family to cope with the disease more effectively. Naturally, having a child with CF can be a very scary and overwhelming feeling for both the family and the individual. As it was previously mentioned, typically, a diagnosis of cystic fibrosis is made when a child is approximately two years old. According to Cystic Fibrosis Foundation (2018), infants with CF need to visit the CF clinic every one to three months during the first year of their life, and begin quarterly visits at the age of two.
Parents need to be educated on the importance of various CF treatments, as well as they need to be aware of different information how these treatments are a lifelong commitment for both the child and the caregiver. Moreover, parents also need to be educated on the importance of enzyme replacements before all meals and snacks in order to reach a normal developing body mass. Breathing treatments like the Vest machine is introduced to toddlers. Using some recommendations, it can be noted that a child life specialist can lead the family through the various methods of treatment, explaining their positive influence on the child’s health and psychosocial development. The family members need to know that their child has all chances to live a relatively normal life in a case of receiving the opportunity for self-fulfillment with the use of CF treatment, as well as having a positive feedback from the parents’ side. Moreover, a child life specialist should let the parents know that their child can perform normal daily activities right now, and when the child will become older, different obstacles may occur. Nevertheless, the hospital staff will be always ready to help the parents to cope with new life obstacles and to lead them through any new methods of treatment.Case Study: Cystic Fibrosis Sample
The research has shown that many individuals received their diagnosis of CF, when they were infants or toddlers. It is a very important for the child to become familiar with the Vest and oxygen masks timely. Familiarizing the child through sight and feel with the medical equipment should be similar to game. It means that it is important to let the child to touch this equipment and to play with the objects before using them. This game approach will make the object less scary for the child. For instance, putting the Vest and Oxygen Mask on a teddy bear allows the child to exam how the machine operates before putting it on the child’s own body. It is also important to involve the child into fun breathing interventions, which is possible to conduct at home and in the hospital. These interventions include blowing up a balloon, cotton ball air hockey, or balloon toss, etc. The aim of these interventions is to help the child’s body to move actively, so he/she can breathe easier.
James is nine years old, and this means that he is a school-aged child now. As children start to reach school age, daily school activities and adjustment to new routines can create many psychosocial issues revolving around self-care at school. According to Piaget, James is in the concrete operational stage, and he is far more logical in his thinking. This stage is characterized by the child’s ability to have more rational and organized thinking. In such a way, James is able to understand some complex issues about his disease and to realize the importance of his CF treatment. As a child life specialist, it is important to educate the patient on the ways the body works, the effects of CF, and the purpose of the future treatment. At this age, the child can also begin to do a greater portion of self-care at home and at school regarding the respiratory therapy, such as a nebulizer breathing treatment and airway clearance.
To educate James on the nebulizer breathing treatment, the child life specialist will work with the child using medical play with a teddy bear or medical doll. The child life specialist will ask the patient to show the teddy bear/ doll how to do the nebulizer treatments. As the child goes through the routine, the child life specialist can interject to correct information/ ask questions/ and expand on knowledge of body systems, functions, purpose of treatment, and why they need to properly clean their nebulizer. In such a way, after educating the child, the child life specialist needs to control the patient for the purpose to be sure that all questions are met properly.
Child life specialists will need to educate the child on airway clearance techniques. Case Study: Cystic Fibrosis Sample The child life specialist and respiratory therapist work with the patient to teach airway clearance techniques. The first activity will be a straw maze exercise. James will use the straw to blow the cotton ball through the maze made out of tape to get into the goal at the end of the maze. In this case, the child life specialist will explain the child how breathing into a straw will allow a small backflow of air that pushes back into the lungs and helps to open airway, making it easier for the mucus to be cleared. In the next step, the child life specialist will go on to explain how coughing only removes a little bit of mucus out of the lungs and huffing allows to clear more mucus because a child is gently working using own lungs from top to bottom. The child life specialists can explain this concept utilizing a tube of toothpaste, explaining the child that when he coughs, he is only squeezing the toothpaste at the top of the tube. Therefore, only a small amount of toothpaste comes out verses when squeezing down and below, and paves the way to the top of the toothpaste (huffing). The child life specialist will then show James how the boy, along with his parents, can make his own straw maze at home.
To continue, bronchoscopy is an example of a surgery, which a patient with CF might receive. In this case, a child life specialist needs to educate James about this procedure, helping the boy to understand its specific features and importance. The aim of the child life specialists is to prepare their patients to this procedure, explaining everything gradually, step by step. This means that it is important to show James not only the oxygen mask, but also the room, where the procedure will take its place in future. The child life specialist will show the patient an image of lungs and the airways throughout the lungs. Showing James how the airways in the lungs can become blocked and how the camera can detect exactly how well or not his lungs are doing, it is possible to calm the boy before the procedure. It is important to explain to James that the doctor will take a very small camera that will go into his lungs to check airways blockage, while he will be sleeping. In such a way, it is necessary to be sure that James understands his future condition and that he will not feel anything during the procedure, but he may feel tired. The child life specialist need to put an emphasis on the fact that his parents will be there, when he will wake up and that he will be able to go home later. Making sure that the patient understands why they have to conduct a medical procedure, explaining that his doctor is finding treatment he needs to do at home so that he can return to what he likes, the child life specialist performs a supporting function. Moreover, an additional aim is to reassure the boy that he will be safe after this procedure, and there might appear only a feeling of easy fatigue, while in a few days he will be back to his normal lifestyle. The next step, after explaining to the patient the main nuances of the bronchoscopy, it is good to allow the patient to do a bronchoscopy on a teddy bear. This allows the child life specialists to observe how the medical patient perceives what the procedure will be like and interject with any answers, as well as elaborate on any questions the patient might have.Case Study: Cystic Fibrosis Sample
Child life specialists can ease the child’s fear and anxiety with therapeutic and recreational play activities, and coping stress skills training. Diversion tools are used to temporary distract the patient from any anxiety or pain he might be experiencing. Different items, as small as I-Spy books, play dough, coloring sheets, and kaleidoscopes, can ease the patient’s anxiety and bring familiarity into the hospital setting. Other medical play activities, such as “Make Mucus”, “Bronchial Binoculars”, marshmallow shooters, straw soccer games, straw painting, decorating Vests with stickers, CF word games, and “All About Me” posters, allow to comfort and provide meaningful interventions for the patient coping with inconveniences during hospitalization.
Exploring psychological state of the boy during his hospitalization and some short-term effects that James may be experiencing, it is necessity to mention the boy’s feelings of isolation, anxiety about missing his peers, missing school activities, and anger about being hospitalized. Some of the long term effects of hospitalization for a patient with CF may include the following: the necessity to catch up with school work due to several absences; to be able to be independent from caregivers; to be able to travel or stay overnight away from home while keeping up with CF treatments; to maintain a healthy diet to keep weight at a healthy level; to cope with the psychological effects of multiple hospitalizations and doctors visits, and the lack of understanding from peers.Case Study: Cystic Fibrosis Sample
For children with CF, multiple hospital visits for checkups will always be a priority throughout the course of their lives. As the child life specialists, we can provide support and constant help for both the patients and their families. Child life specialists can offer a parent/ caregiver education on childhood growth and development as well as the effects of CF and hospitalization. The child life specialists can also provide therapeutic and medical play to understand the child’s perspective on future procedures, diagnosis, and treatments. The child life team helps to facilitate patient education and supports group meetings for children with terminal illnesses like CF, as well as advocates for the needs of the patients and their families.
In conclusion, this case study has helped me to learn many important details about cystic fibrosis, and the way in which this disease affects the patients and their families. Moreover, this case study also has demonstrated the fact that for the purpose to live a long and healthy life, a patient with CF needs to go through the life long battle. Most importantly, I have learned the role that the child life specialists play in supporting families and patients with CF. Child life specialists can help ease the anxiety that surrounds the process of CF treatment. Thus, learning about this illness has better prepared me to help CF patients and their families to cope with psychological and physical discomfort, possible stress and other negative life situations, which this illness can bring.Case Study: Cystic Fibrosis Sample