Nursing Diagnoses

Nursing Diagnosis 1:

Ineffective airway clearance r/t inability to clear mucus AEB productive cough, wheezing, SOB.

Goal: Patient will maintain clear airway for remainder of hospital stay.

Interventions:
1. Encourage activity to promote complete expansion of bronchioles.
2. Use mucolytic agents and do not administer cough suppressants (cough is needed to clear secretions).
3. Provide aerosol therapy 3-4 times a day or as prescribed.
4. Provide moistened oxygen to correct hypoxia and acidosis.


Nursing Diagnosis 2:

Altered nutrition less than body requirements R/T inability to digest fat AEB poor weight gain, steatorrhea.

Goal: Patient will not maintain current weight/gain weight during remainder of hospital stay.

Interventions:
1. Provide a high calorie, high protein, moderate fat diet.
2. Supplement vitamins ADEK as water-miscible forms.
3. Make sure the room temperature is always below 72 degrees F to prevent excessive perspiration (salt loss).
4. Supplement with synthetic pancreatic enzymes before each snack and meal.


Nursing Diagnosis 3:

Risk for altered skin integrity r/t acid stools AEB meconium.

Goal: Patient will maintain skin that is clean, dry, and intact for the remainder of the hospital stay.

Interventions:
1. In infants: change diaper immediately after stool is passed.
2. Check the rectum after bowel movement to see if it has prolapsed.
3. If prolapse is present, replace it gently with a gloved lubricated finger.
4. After replacement, tape the buttocks together to maintain gentle pressure.


Nursing Diagnosis 4:

High risk for ineffective family coping r/t chronic illness of child AEB distress, anger, crying, etc.

Goal: Family will demonstrate effective coping strategies by the end of the shift.

Interventions:
1. Teach proper home care of the CF patient. 
2. Schedule regular follow-up visits with the patient and family.
3. Arrange for home schooling or tutor as needed.
4. Encourage all family members to participate in care to reduce caregiver strain.


Nursing Diagnosis 5:

Impaired gas exchange r/t airway obstruction by nasal obstruction AEB productive cough/sputum.

Goal: Patient will achieve oxygen saturation of 95 or higher by the end of shift. 

Interventions:
1. Encourage exercise appropriate to the physical ability of the patient.
2. Maintain a patent airway.
3. Monitor vital signs, ABGs, and pulse oximetry to detect/prevent hypoxemia.
4. Provide supplemental oxygen according to the provisions/requirements. 



References:

http://nanda-nursing-care-plan.blogspot.com/2014/05/3-nursing-diagnosis-and-interventions.html

http://www.nursing-help.com/2011/08/cystic-fibrosis-nursing-diagnosis-and-care-plan.html  

Support Groups

http://cystic-fibrosis.supportgroups.com/

This is an online support group forum where people can come and post their struggles, questions, or victories related to their disease, as well as read and respond to other people's posts. This is a great option for people to see that people all around the world are experiencing the same thing they are - not just people in their own backyard. It is also a great option for people who have limited mobility to connect with others. When a person is very sick with CF, it can be hard to get the energy to come to meetings that meet weekly, biweekly, etc. The advantage of this online support group is that they don't even have to leave the house!



https://www.cff.org/oregon/

This is the Oregon chapter of the official Cystic Fibrosis Foundation. It is centered in Portland which is ideal for CF patients in the Newberg area. This support group is excellent in their outreach programs with numerous fundraisers including Bob Gilder Platt's Golf Challenge, Portland Firefighter Starclimb, and Brewing up a Cure. Not only is this effective in raising funds towards research of the disease, but it also establishes a sense of community support towards those with the disease. The downfalls is that it is less focused on emotional support.

Ideally, a support system includes both emotional and community support. For a person with CF to gain the most benefits, I would recommend involvement in both types of support groups. I would also recommend for family members to get involved in support groups because they too are living with the disease, in a way.

Care Considerations of CF Patient

Care for a patient with Cystic Fibrosis is multidimensional. Here, I will walk though each of the aspects that should be considered regarding care of the hospitalized Cystic Fibrosis patient.

Symptoms:
                  GI distress, hemoptysis, change in sputum character, chest pain, anorexia, vomiting
Signs:
                  Labored breathing, fever, weight loss, changes on pulmonary exam
Data: 
                  Hypoxemia, decreased lung volume, abnormal CXR, leukocytosis, presence of new microbes
Therapies:
                  Oxygen, antibiotics, airway clearance therapies, nutrition, bowel program

* Keep an eye on:
       Weight - rapid changes (usually drops)
       Pulse oximetry - dropping below 90 is a sign of hypoxemia
       Abnormal lab values - particularly sodium which is often lost through sweat
       Culture data - may identify microbes which would signify an infection
       Blood sugars - CF patients sometimes need to be put on insulin therapies due to hyperglycemia
       Imaging - CXR - can identify pneumothorax, other structural lung problems
       Immunizations - prevent infectious diseases
       Preventative care - vital due to weakened immune systems. Be vigilant about hygiene/PPE.



References:

http://medicine.missouri.edu/jahm/review-article-care-hospitalized-patient-cystic-fibrosis-summary-current-practice-guidelines-recommendations-hospitalist-part-1-pulmonary-exacerbation/ 

Dietary Treatments

 People with cystic fibrosis tend to be malnourished and underweight because their mucus keeps intestines from absorbing fat and vitamins. As a result, they require a diet high in fat and calories along with nutritious items and dietary supplements. It is recommended that teens with cystic fibrosis eat 2,900-4,500 calories every day. The problem is that thick mucous in the lungs may impair breathing and make it harder for CF patients to eat. 
                --> Thus, they should focus on foods that are fat and calorie dense in order to meet their unique nutritional needs. 

 
 Fish, nuts, beans, cheese, and eggs are all great sources of calorie-dense foods that are rich in healthy fats.

CF patients also require a greater amount of protein, iron, salt, zinc, and calcium than a healthy person would. 
             Protein: 15-20% of diet
                                               Sources: meat, eggs, soy foods, fish, nuts, beans
             Iron: fights infection, carries oxygen to tissues through the blood.
                                               Sources: fortified cereals, meats, dried fruits, dark green vegetables
             Salt: CF patients lose a lot through sweat.
                                               Sources: add salt to food, eat salty snacks, sports drinks after exercise
             Zinc: promotes growth, healing, and fighting infection
                                               Sources: meat, liver, eggs, seafood
             Calcium: CF increases risk of osteoporosis
                                               Sources: dairy products - full fat dairy products are also good source of  fat and calories



References:

http://kidshealth.org/teen/food_fitness/nutrition/cf_diet.html# 

Treatments & Therapies: How to Take Charge of CF!

In recent decades, cystic fibrosis treatment has come a long way - and as a result, the prognosis of the disease has increased from childhood to where it stands today.  Unfortunately, the disease cannot truly be cured because it is a genetic disease. The faulty CTFR gene that causes Cystic Fibrosis is part of the make up in every cell of the affected's body. However, while there are no cures, there are treatments that can make the disease more manageable, improve quality of life, and even extend the lifespan.

 In the world of pharmaceuticals, the use of antibiotics, mucus thinning drugs, and oral pancreatic enzymes can reduce the complications of Cystic Fibrosis. Antibiotics can lessen the chance of the build-up of bacteria leading to lung infections and subsequent lung damage. Meanwhile, mucus thinning drugs will prevent mucus from becoming so thick as to obstruct airway passages. Oral pancreatic enzymes are frequently given to CF patients to replace the enzymes that are lacking in the body from disease-related pancreatic damage. This allows the digestive tract to be able to absorb the proper nutrients. 

Other treatment options that may be prescribed involve finding ways to improve the structural deficiencies of the lungs and chest. Chest physical therapy in the form of the chest clapper, inflatable vest, or other breathing devices is used to loosen thick mucus in the lungs to make it easier to cough up. 

 

 Inflatable vest

Consists of two parts:
Air-pulse generator: creates rapid bursts of air that make the vest inflate and deflate against the chest wall. The vibration of the vest against the chest wall helps to loosen mucus.
Inflatable vest: creates an airflow that helps to move mucus from the airway walls.

In more extreme cases a doctors might also suggest oxygen therapy, lung transplants, feeding tube or bowel surgeries to better lung and digestive function and improve overall well-being.

References:

http://cfcareli.com/livingwithcf_acts_vest.php 

http://www.mayoclinic.org/diseases-conditions/cystic-fibrosis/basics/definition/con-20013731

https://www.cff.org/

Symptom Progression

Generally speaking, cystic fibrosis has defined identifiable symptoms. Since cystic fibrosis involved thickening of respiratory mucus, many of these symptoms are respiratory-related. These include a persistent coughing that sometimes includes phlegm and wheezing or shortness of breath. This occurs as the lungs struggle to push air out of airways that are obstructed by thick mucus. Some studies indicate that the degree of tissue hypoxia is proportionate to the degree of CTFR mutation.

 Apart from respiratory symptoms, a CF individual may also notice a salty quality to their skin as a result of the increased presence of salt in the sweat. A more commonly known symptom of CF is poor growth or weight gain despite a hearty appetite, as well as experiencing frequent greasy, bulky stools, diarrhea, or difficulty with bowel movements as a result of the decreased production of insulin and digestive enzymes that make it harder for the body to process food. This is why individuals with CF tend to have a small, petite stature.

Symptoms can be further broken down into mild to moderate (stage 2), severe (stage 3), and late stage. Each stage has defining characteristics as the disease progresses. In the mild to moderate phase, cells are hypoxic, the immune system is suppressed, and problems develop with protein metabolism and the synthesis of hormones and neurotransmitters. Mucus also begins to thicken and become stickier. In the the severe stage, the clinical picture quickly deteriorates. Respiratory problems worsen to the next stage - for example, bronchitis and bronchiolitis become bronchiectasis. The downhill slide of the respiratory system often manifests as severe dyspnea, strong chest pain and difficulty breathing. The last stage is where the individual is really fighting with death. Severe alveolar hyperventilation leads to critically low CO2 levels which can often develop into cor pulmonale (high blood pressure in the pulmonary arteries and right heart overload). This is the point where individuals often die. 

References:

http://www.normalbreathing.com/cystic-fibrosis-symptoms.php 

Diagnosis Techniques Aplenty

In the United States, every newborn is screened for cystic fibrosis. This is a practice that is upheld in all 50 states. Typically, newborn screening is accomplished through either a genetic test or a blood test. The genetic test will show whether or not the baby has the CTFR gene while the blood test will show if the pancreas is working correctly.
If a genetic test indicates CF, the results can be confirmed using a sweat test. This test measures the amount of salt in sweat. Doctors will trigger sweat on a small patch of skin - usually on the arm or leg. They will then use an electrode to produce an electrical current, then take the sweat strip in for analysis. High levels produced two or more times confirm positive CF results.
Following this, the doctor may recommend further tests including genetic testing to determine what type of CTFR is causing CF; chest X ray to view any inflammation or scarring of the heart, lungs, and blood vessels; sinus X ray to detect sinusitis; lung function tests to determine how much air can be breathed in and out; and a sputum culture to detect the presence of any bacteria that may be causing an infection (since CF patients are at a higher risk for infection due to thicker mucus).
 Progression of the disease can be summed up as follows:

References:

http://www.nhlbi.nih.gov/health/health-topics/topics/cf/diagnosis