Furthermore, the respiratory, digestive, reproductive and sweat glands are most affected by cystic fibrosis. There are many types of treatments that can aid these systems. The nervous system and the brain are least affected, making a child’s ability to learn not adjusted by cystic fibrosis.
While the brain is typically not affected by those with cystic fibrosis, the lives of these individuals can become drastically different from those of healthy people. A high maintenance lifestyle typically becomes more prevalent in a child with cystic fibrosis as they can require feeding, treatments, and procedures like blood tests and more. When the disease becomes more severe, there can be behavioral challenges and other psychological difficulties involved. As they become teenagers, frustration may arise as they are in the hospital often to care for their treatments. Body image is often a problem for patients growing up with this disease as puberty and menstruation is typically delayed a few years.
It is common for children with this disease to attain feelings of depression and anxiety due to the lack of total control of their health. Patricia Silva, PhD, is the author of ‘Cystic Fibrosis and Emotional Wellness’. She finds that anxiety is “one of the most common emotional issues faced by these patients”. “This is related to daily treatments, remembering to take medications, missing out on things they want to do, and being hospitalized for periods of time”. Along with this, patients of CF are very likely to suffer from depression and feelings of hopelessness.
Despite all of this, one can seek emotional wellbeing by recognizing the signs and realizing that they are not alone. Patricia Silva states that “patients and loved ones can seek psychological help with specialists or with support groups, which are common not only in associations and organizations like the Cystic Fibrosis Foundation, but also among specialized medical centers”. A child life specialist may be able to recommend patients and their families to these foundations as a way for them to gain support.
Currently, there is no direct cur for cystic fibrosis. There are many treatments for a CF patient. These treatments are to prevent infections and while trying to keep the airways clear in order to live as comfortably as possible. Moreover, maintaining adequate nutrition is also important for a CF patient as they do commonly develop CF related diabetes. These treatments can affect their everyday lifestyle as they can be in the hospital more frequently. CF patients and their families work with many healthcare professionals in order to make their treatment processes easier and more effective.
A child life specialist may intervene with a cystic fibrosis patient in many ways. First of all, there are many books that can be given to the patient, according to their developmental level. There is ‘Cystic Fibrosis and You’, by Emilie Sasso. According to the author, this book was written to encourage children with CF to maintain a positive life and accomplish their dreams. For older CF patients, there is ‘Cystic Fibrosis: The Ultimate Team Guide’, by Melanie Ann Apel. This book is a little more serious by explaining procedures while interviewing other teens with CF on how they live their life to the fullest. The overall goal for a child life specialist is to offer opportunities to play and interact with others in an emotionally safe environment.
Usually, infants will visit the CF clinic everyone to three months during the first year of life and begin more frequent visits around the age of two. Kirsten M. Black, a graduate student in child life administration at the University of La Verne, wrote ‘Child Life in the Cystic Fibrosis Center’, a handbook on how to handle a patient with CF and their family. She goes on to clarify how CF can be defined to a preschool age sibling by explaining it in an age appropriate manner. She states that “parents need to be educated on the importance of enzyme replacement therapy” and much more. Pancreatic enzyme replacement therapy (PERT) can be defined as the utilization of medications that contain enzymes to replace what the pancreas is no longer releasing in order to maintain proper digestion. According to Pancreatic Enzyme Replacement Therapy use in infants with cystic fibrosis diagnosed by newborn screening, a study conducted by pediatricians, “improved growth early in life is associated with better clinical outcomes later in life” determining PERT as an “important therapeutic recommendation”. At the stage of PERT (infancy), the parent is more involved than the patient. The child life specialist may intervene in this situation by providing the parents with informational booklets about PERT. In addition, the child life specialist can demonstrate to the parents how to open the capsules and feed it to their baby.
As children reach preschool age, they begin pulmonary function tests (PFT’s) and start to learn to swallow pills (enzymes and vitamins). This is when the child life specialist can start to teach on how to master swallowing pills. The child life specialist can engage in pretend play with the patient and their dolls and pretend to feed them fake pills. This familiarizes the child the with aspect of swallowing pills. Another way to familiarize the patient with pill swallowing is by playing “hungry hungry hippo”, according to Black. The author goes on to state an example of this as referring to the balls as “enzymes” and asking the child if “they think they can swallow as many enzymes as the hippos in the game”. The parents can also play a role in this learning by reinforcing pill swallowing at home.
As a CF patient approaches the school age, psychosocial difficulties may be developed sa they are adjusting to new routines while entering school. The child may gain more responsibilities of self-care as they are maturing and able to comprehend their disease and the purpose of their treatments a little more. Respiratory therapy, often known as airway cleaning, becomes more prevalent in their life at this age. A child life specialist can plan for intervention in order to inform the school ager on airway cleaning in many different ways. One way is that they can utilize a straw painting exercise. The child life specialist will utilize a straw to blow bubbles into paint while touching paper to the bubbles in order to make prints on it. The purpose of this activity is to introduce respiratory clearance techniques in a fun way. The child life specialist then continues to explain how breathing into a straw against thick paint is “creating a small backflow of air that pushes back into your lungs and helps to open airways” making it easier for mucus to be cleared. Furthermore, this type of education on a school ager allows the child life specialist to incorporate play while teaching them about necessary techniques in an age appropriate manner.
Along with adolescence comes the topics of independence, sexuality and body image. In addition, they learn to take charge of their own airway clearance and the management of enzymes and digestion at this stage. Adolescence also often brings along the challenge of CF-Related Diabetes (CFRD). A lot of patients at this age experience their first hospitalization during this period while having their first experience with intravenous antibiotic therapy. The child life specialist can work with the patient in the Child Life Center to discuss upcoming hospitalization and the need for antibiotic therapy and its implementation. Once the line becomes in place, the child life specialist can engage the patient in movement by playing an active Wii or Xbox game while discussing perceived and real restrictions of having this line in place.
Family centered care can be defined as the establishment of relationships with patients and their families in order to recognize the importance of family roles in health care as an effective influence on the patient’s treatments. In the research known as, ‘Patient- and Family-Centered Care: It’s Not Just for Pediatrics Anymore’, Aaron Clay, RN and Bridge Parsh, RN, MSN, EdD, emphasize that hospitals where family centered care is part of the organizational culture find “not only that patient, family, and staff satisfaction ratings significantly increase, but also that patients’ health outcomes improve”. ‘Practice applications of research. Perceived needs of parents of critically ill infants in a neonatal intensive care unit (NICU)’ is another research on the importance of family centered care done by Kylie Ward. The author found that “answering parents' questions honestly, actively listening to their fears and expectations, assuring parents that their infant is receiving the best care possible” and “demonstrating a genuine concern for the whole family” decreased the anxiety of parents. Family centered care is a large portion of the job of a child life specialist as their aim is to decrease trauma while maintaining development in the hospital.