Cystic Fibrosis Presentation

Cystic Fibrosis Presentation

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SLIDE 1
Life threatening condition
Inherited disease
Common in Caucasian
• Affects the production of mucus, sweat, digestive juices and infertility

Nurses deal with many different types of illnesses and conditions. There are different kinds of genetic diseases. One of the most common life threatening conditions is cystic fibrosis. According to the US Cystic Fibrosis Foundation, “projected life expectancy for the patients has increased from 31 to 37 years over the past decade” (O’Sullivan & Freedman, 2009, p. 1891). Cystic Fibrosis inherits two faulty genes for the disease from both parents. This condition is most common in white people of Northern European ancestry. It affects the production of mucus, sweat, digestive juices and infertility. The mucus becomes thick and sticky, blocking the airways, which leads to recurrent infections. Cystic fibrosis causes a person’s sweat to become salty, which upsets the balance of minerals in the blood and causes health problems. The thick mucus can also block the ducts in the pancreas. The pancreas makes digestive enzymes which helps break down food. Without the enzymes, the intestines cannot absorb fats and proteins that may cause bulky stools, intestinal gas, a swollen belly from constipation and pain and/or discomfort. Men and women may experience infertility as a result of having cystic fibrosis.
SLIDE 2
• Affects the production of mucus
• Decreases sweat, digestive juices
• Increases infertility
• Cystic fibrosis affects the production of mucus, sweat, digestive juices and infertility. The mucus becomes thick and sticky, blocking the airways, which leads to recurrent infections. Cystic fibrosis causes a person’s sweat to become salty, which upsets the balance of minerals in the blood and causes health problems. The thick mucus can also block the ducts in the pancreas. The pancreas makes digestive enzymes which helps break down food. Without the enzymes, the intestines cannot absorb fats and proteins that may cause bulky stools, intestinal gas, a swollen belly from constipation and pain and/or discomfort. Men and women may experience infertility as a result of having cystic fibrosis.
• SLIDE 3
Impacts on Development
• Self esteem
• Lack of sleep
• Depression
• Anxiety
• Adolescence is a time of many challenges including physical, intellectual and emotional challenges. Developmental issues face all teens but the impact is elevated for those with cystic fibrosis. Puberty is delayed with cystic fibrosis, which can affect a child’s self-esteem and elevate their sense of being different.

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Reproductive infertility is a topic teens have a difficult time talking about it but with cystic fibrosis, it must be addressed. Options for girls on birth control are limited. Certain antibiotics may interfere with the effectiveness of birth control pills in females while male teens are likely to become infertile. Adolescents need over 9 hours of sleep, with cystic fibrosis, the need of sleep is important for optimal health. Chronic coughs and side effects from medication can cause insomnia for patients with cystic fibrosis. Lack of sleep can impact a teenager’s ability to concentrate and cause dramatic mood swings, which can lead to anxiety and depression. Common stressors that can lead to depression in are feeling physically unwell, endless daily treatments and missing major life events such as sports, parties and prom due to frequent hospitalizations. Adolescence is the transition period from pediatric to adult care for patients with cystic fibrosis. Besides feeling nervous about meeting a new care team, teens are thinking about goals beyond high school such as college, and moving away. Moving away means being away from familiar settings including health care support provided by their parents. Even though this stage is exciting, it can also create increased anxiety.

SLIDE 4
• Identity Versus Role Confusion
- Dramatic physiological changes
- Preoccupation with appearance and body image
- Acquiring sense of identity

Erikson’s theory of cognitive development can be used to develop nursing interventions for an adolescent suffering from cystic fibrosis. According to Erikson’s theory an adolescent would be in the identity versus role confusion stage. During this stage adolescents reach sexual maturity. They are also very body conscious and concerned with their appearance more than ever before. At this stage they begin to develop a sense of identity. “Acquiring a sense of identity is essential for making adult decisions such as choice of vocation or marriage partner”
(Potter & Perry, 2005, p. 161).


SLIDE 5
Nursing Interventions – Education
• Education
- Help establish a sense of identity
- Provide adequate information
- Establish confidentiality
Adolescents suffer from embarrassment in silence. They are afraid to ask questions about all the challenges they are going though. Nurses need to present information proactively and speak alone with the teenager to help them feel comfortable with asking questions. In doing so the nurse can utilize Erikson’s theory that adolescents are developing their own sense of identity and making adult decisions. By providing adolescents with adequate information regarding their condition and treatment options they will be able to contribute to their plan of care and make decisions regarding their health. “Throughout their time in care, healthcare professionals should empower young adults and promote their independence using various strategies, for example providing information, considering their opinion” (Al-Yateem & Moyola, 2013, p. 33). The concept of confidentiality needs to be explained to the adolescent. Establishing confidentiality helps develop a sense of trust between the patient and nurse. It allows for a more open and honest discussion and will help in developing a good plan of care.

SLIDE 6
• Nursing Interventions – Encouraging Active Lifestyle
Honest feedback regarding body image
• Physical activity
• Exercise
• Benefits and barriers
• Physical activity and exercise are recommended for management of cystic fibrosis. Adolescents with CF are less active than their peers. This may have a negative effect on their appearance and body image. By utilizing Erikson’s theory we can address the adolescents concerns about body image by being honest regarding what changes to expect during treatment for cystic fibrosis. By being honest with what to expect, adolescents can begin preparing themselves for these changes. Nursing interventions to assist with body image and appearance should include promoting exercise. Promoting physical activity and exercising regularly as part of the management of cystic fibrosis is important. “Regular aerobic exercise enhances cardiovascular fitness, enhances airway clearance, slows declines in lung function, and improves body mass” (Swisher & Erickson, 2008, p. 107). There are benefits and barriers when promoting an active lifestyle to adolescents with CF. The barriers are physical discomfort such as muscle soreness, fatigue, joint pain and increased heart rate. Coughing and shortness of breath are also part of the problem. The important benefits are delayed onset of dyspnea, maintenance of bone density, enhanced cellular immune response, and increased feelings of well-being. The team of clinicians and caregivers needs to create an individual plan for the adolescent with CF. It is important to education and promote physical activity and exercise as a part of lifestyle for maintaining both overall health and for improving lung symptoms and energy levels.

SLIDE 7
Nursing Interventions - Nutrition

• Nutrition
- Long-term survival
- Maintain lung health
- Encourage higher, healthy caloric intake

Dramatic physical changes are seen during adolescence. According to Erikson, adolescents are very concerned with their appearance and when suffering from cystic fibrosis they may have a poor appetite, which will affect their physical growth and development. Informing adolescents that maintaining a healthy diet will help them with their appearance will encourage them to eat more. “The benefits of maintaining good nutrition in regard to long-term survival and lung health cannot be overstated” (O’Sullivan & Freedman, 2009, p. 1899). The mucus from the CF can block the body from absorbing nutrients from food. Explain to the teenager that eating a lot of calories is part of living. Fortunately, most popular foods are also high in calories. The trick is to still eat healthy food with high protein, vegetables, fruits and dairy. Let the teens invite their friends over for snacks or meals. It will make getting calories more fun with friends. Supplements such as pancreatic enzymes are encouraged for those patients with less than optimum growth.

SLIDE 7

Common Treatments
There are many types of treatments for cystic fibrosis. The focus for treatments is to keep the lungs clear and infection free. Antibiotics are often used to help treat and prevent the onset of lung infections. Bronchodilators are used to keep the airway clear by helping the muscles located around the bronchial tubes to relax. Breathing devices are also used to help exercise and strengthen the lungs. Sometimes surgical procedures may be necessary to help clear the airway and improve breathing. Procedures such as an endoscopy and lavage can be performed to suction out excessive mucus build up.

.
SLIDE 7
Evaluation of Nursing Interventions
There are ways to evaluate the nursing intervention when taking care of adolescents with cystic fibrosis. At each physical exam, the height and weight should be measured including calculating their body-mass index (BMI). By doing this regularly, the nurse can access if there is a decrease in BMI or stunting of growth and if necessary provide the adolescent with nutritional counseling. Good nutritional well-being has a positive correlation with good healthy lungs in cystic fibrosis. If the nutritional status is poor, supplements should be strongly considered. Patients with pancreatic insufficiencies need supplements such as pancreatic enzymes. CF patients have difficulty digesting foods due to thick mucus that can clog the ducts of the pancreas creating poor digestion. Pancreatic enzymes helps digest and absorb the food in patients with CF. When CF adolescents get hospitalized, nurses need to find out how much physical activity they participate in or how often they incorporate exercises into their daily routines. Identifying the barriers and communicating with the CF team to find ways to help the individual in promoting an active life style into their daily routines helps decrease hospitalization. Nurses need to identify ineffective communication. In order to improve the ability to communicate, nurses need to educate and increase the knowledge of multiple needs of the adolescents with CF. Providing reassurance enhances communication and understanding regarding their fears and anxieties. Having a team approach allows the adolescent to communicate about specific problems. Members of a multidisciplinary CF team include doctors, physiotherapists, dieticians, social workers, and nurses.


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