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

Progression & Diagnosis

Cystic fibrosis causes mucus to build up in the lungs and digestive tract, and as a result, it is known to cause progressive damage to the respiratory system and chronic digestive system problems. Mucus – the slippery lubricant produced in your body – is needed to protect the lining of the airways, digestive system, reproductive system, and other organs and tissues. As it moves through the body, mucus can collect bacteria and foreign microorganisms to be swept from the body (for example, the cilia in the nose can sweep mucus out of the nasal passages to remove foreign particles). In individuals with cystic fibrosis, the mucus that the body produces is more thick and sticky than normal, and as a result, it builds up in the airways to clog the passage to the lungs. Not only does this mucus obstruct the airway to make breathing more difficult, but it also allows bacteria to build up in the lungs and cause permanent lung damage. Chronic mucus buildup and infections can lead to the formation of scar tissue and cysts over time. Cystic fibrosis can cause problems of the digestive system as mucus builds up within the pancreas – an organ that produces insulin and enzymes for the body to digest food – and prevents the enzymes from reaching the intestine, thus inhibiting digestion. This obstruction is what causes symptoms of cystic fibrosis such as diarrhea, malnutrition, poor growth, and unexplained weight loss. 

Early diagnosis of cystic fibrosis is extremely beneficial in creating the appropriate treatment plan for the patient. Generally speaking, cystic fibrosis is tested for using one of three methods: the sweat test, newborn screening, or genetic carrier testing. A sweat test is a sample of sweat that is tested for high levels of salt. High salt levels indicate the deficiency of the CTFR gene that would transport the salt ions to other areas of the body. Newborn screening is a series of diagnostic tests performed on newborn babies in every U.S. state to determine illnesses. It is not a definitive way of determining CF, but it can lead to tests that can confirm or rule out a CF diagnosis. Genetic carrier testing is a blood test taken from the parents of an individual who is showing symptoms of cystic fibrosis. This test can reveal whether or not the parents are symptom-free carriers of the defective gene, keeping in mind that one must inherit the defective gene from both parents to show symptoms of cystic fibrosis.  

References:
https://www.cff.org/

 

Who will get Cystic Fibrosis?

Cystic fibrosis has been known for over 200 years. Reports from the Cystic Fibrosis Foundation indicate that there are 30,000 Americans, 3,000 Canadians, and 20,000 Europeans with this disease. Every year, about 2,500 babies  in the United States continue to be born with this disease. These babies can only look ahead to a brief 30 year life expectancy. The majority of children are screened for CF at birth and will be diagnosed by age 2, but there are still some that are diagnosed as adults. Apart from those who actually inherit the disease, 1 in 20 Americans contain the abnormal CF gene. This translates to a whopping 12 million people that are carriers, and since carriers have no symptoms of the disease, they usually carry it unwittingly. 

A clinical study in Saudi Arabia sought to gain information on the morbidity and mortality of this disease. They conducted their research at a hospital over 9 years on confirmed CF patients. They found that over the 9 year period, 190 patients were diagnosed with CF. Of these, 164 (86%) patients lived and 26 (14%) died. Fifty-two percent were male and forty-eight percent were female, indicating a relatively even divide in incidence between genders. Factors that related to early mortality were calculated weight/height, low albumin level, high hematocrit, low mean corpuscular volume, and low mean corpuscular hemoglobin concentration.

Since CF is a genetic disease, it cannot be prevented. Babies with two mutated CF genes will have the disease, and at this time gene therapy can only be used to help prevent some of the complications. The hope for the future is that genetic counseling will be able to detect CF carriers, and possibly persuade them to not have children. Every time two carriers of the gene conceive, there is a 25 percent chance that they will pass cystic fibrosis on to their child. However, the problem here is that the tests to determine if one has the defective gene are only 80-85 percent accurate. Parents should always be reassured that it was not their fault that their child has the disease; they could not have prevented it.

Sources used:

http://www.cdc.gov/excite/ScienceAmbassador/ambassador_pgm/lessonplans/high_school/Am%20I%20a%20Carrier%20for%20Cystic%20Fibrosis/Cystic_Fibrosis_Fact_Sheet.pdf

http://www.ncbi.nlm.nih.gov/pubmed/12883603

Search terms:

Cystic Fibrosis epidemiology, Cystic Fibrosis morbidity and mortality, Cystic Fibrosis prevalence

What is Cystic Fibrosis?

Cystic fibrosis is an inherited genetic disease caused by a mutation in the CTFR protein. 

The CTFR protein is a gene containing the instructions for creating a channel to transport chloride ions in and out of cells. This basic exchange is how sweat and mucous are produced.

When the gene is mutated, it disrupts the epithelium of cells that make up sweat glands in the skin and lining the lungs, liver, pancreas, and digestive and reproductive systems. The epithelium can no longer regulate how chloride passes along the cell membrane, thus mucous becomes thicker. One of the problems with this is that it inhibits mucous' normal function - which is trapping germs and then getting cleared out of the lungs. Cystic fibrosis mucous is so thick that it traps germs but is unable to exit lungs. Germs stay put which can cause infections.

Cystic fibrosis is a genetic disease meaning it is passed on from parents to children through DNA. To have the disease, one must have inherited the faulty, mutated CTFR gene from both the mother AND the father.

The parents are merely carriers of the disease having one faulty gene each and typically do not have signs or symptoms of the disease.