Nursing Diagnosis for Cystic Fibrosis patients

For Cystic Fibrosis patients, there are 4 main nursing diagnoses that helps to direct nursing interventions and patient care.

 Nursing Diagnosis 1: Ineffective airway clearance R/T inability to clear mucus.

Interventions:

– Provide moistened oxygen (30-40%) to correct hypoxia and acidosis.

– Use mucolytic agents and do not give cough suppressants.

– Aerosol therapy 3-4 times a day.

– Postural drainage

– Encourage activity

– Provide respiratory hygiene

– Frequent mouth washes

– Frequent observation of the child

 Nursing Diagnosis 2: Altered nutrition less than body requirement R/T inability to digest fat.

Interventions

– Provide a high calorie, high protein, moderate fat diet.

– Supplement vit. ADE and K as water miscible forms

– Add extra salt in diet, especially during summer.

– Supplement with synthetic pancreatic enzymes before each snack and meal.

– In small infants open the capsule and mix the contents to 1 tsp. of food or warm water and give. It should not be added to bottle or formula.

– Make sure the room temperature is always below 720F to prevent excessive perspiration

 Nursing Diagnosis 3: Risk for altered skin integrity R/T acid stools

Intervention

– For infants change diapers immediately when the stool is passed.

– After a bowel movement check the child’s rectum for prolapse.

– If prolapse is present with a gloved lubricated finger replace it gently back.

– After replacement tape the buttocks together to maintain gentle pressure on the anus.

Nursing Diagnosis 4: High risk for ineffective family coping R/T chronic illness of patient.

Interventions:

– Teach the home care

– Regular follow up visits

– Arrange for schooling or a home tutor

– Encourage all family members in giving care

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

Support groups for Cystic Fibrosis

For any patient in the hospital, the need for support from others is important to the patient’s emotional health as well as their overall well-being. Support groups are organizations of people who have a common disorder and meet to discuss their experiences, share ideas, and provide emotional support for one another. In my research for Cystic Fibrosis, I found a primary website that lists several secondary websites for support groups.

The primary website is by Cystic Fibrosis Research Inc. (CFRI)- their mission is to continue Cystic Fibrosis research, provide educational and personal support, and spread awareness of cystic fibrosis. They want to inform, engage, and empower the Cystic Fibrosis community to reach the highest possible quality of life.

There are several secondary websites offered from the primary website. The first is a support group from the Cystic Fibrosis Research Inc. (CFRI). This support group is facilitated by a social worker who is knowledgeable in issues facing CF caregivers. This support group is open to those able to participate in person, as well as to participants nationwide by telephone. They offer a range of events and programs that provide information, support and vital resources, so as to help people navigate the challenges associated with cystic fibrosis. The second is Heroes of Hope support group. This website seeks to recognize and honor unique individual patients with Cystic Fibrosis who strive to live full, productive lives and serve as role models to  others, all while managing their own healthcare needs. This support group allows people to nominate their hero. The third is the support group by the Living Breath Foundation. This foundation is a non-profit, volunteer-led organization helping patients living with Cystic Fibrosis. They provide localized help to individuals and families in California and Arizona with financial assistance and educational scholarships. Their goal is to alleviate some of the stressors that that can along with Cystic Fibrosis. They give any body the opportunity to receive scholarships as well as donate money to their cause. 

http://www.cfri.org/cfsupport.shtml

http://www.cfliving.com/

http://www.cfri.org/support.shtml

http://www.dailystrength.org/c/Cystic-Fibrosis/support-group

http://www.heroesofhope.com//

Nursing Care for patients with Cystic Fibrosis

Nursing Implications\Care

In providing care for a patient diagnosed with Cystic Fibrosis, the goal of care is to:

§  Maintain lung function as close to normal as possible by controlling respiratory infection and clearing airways of mucus.

§  Administering nutritional therapy such as enzyme, mineral and vitamin supplements to maintain adequate growth

§  Educate patient and family members in counseling, care instruction, airway clearance techniques and use of equipment

In the assessment of my patient I would assess lung sounds, respiration rate, depth, pulse, temperature, capillary refill, blood pressure, as well as other vitals. Specifically in patients with Cystic Fibrosis, I would focus my assessment on lung function, oxygen saturation, weight and height and sputum or cough production. I would be responsible for nutritional needs, nebulizer therapy, care of indwelling venous devices, enteral feeding, oxygen therapy (including inceptive therapy), and psychosocial\emotional support. As by protocol, I would practice proper hand hygiene but also if my patient was at risk for developing an infection I would pay special attention to cross-contamination by friends or family with the patient and make sure to education them on prevention of spreading infection. In my communication with the patient, I would provide verbal instructions for treatment and care, demonstration of motor skills, and printed instructions for at-home review. I would ensure efficacy of teaching by asking the patient to explain instructions and demonstrate motor skills. I would refer patient to the appropriate specialists and support groups. 

The website I would recommend is the website by the Cystic Fibrosis Foundation at https://www.cff.org/. I would recommend to anybody who is interested in being educated or is diagnosed Cystic Fibrosis because it provides reliable, up-to-date information available for most issues on Cystic Fibrosis. The website is accurate, current and readable. Navigating around the website was very easy, they was lots of bold texts and pictures for the different links. They had recent news on the Cystic Fibrosis Foundation and their mission, how to live with Cystic Fibrosis, caregiver help pages, and resources for more information. They also provided contact information such as a toll free number and a search box for locating care centers near patients.

www.cysticfibrosis.org.uk/media/82046/nursing-management-standards-may01.pdf

https://www.cff.org/

Cystic Fibrosis Diet

The viscous, sticky mucus that is produced in the lungs is also produced in the digestive tract and pancreas. This inhibits digestive enzymes from breaking down and absorbing nutrients from the patient’s diet. This is why a patient with Cystic Fibrosis follows a diet high in calories and high in fat. Maintaining healthy weight and possibly increasing it, is a successful strategy in fighting infection and keeping your body strong. The diet consists of special nutrients such as minerals, vitamins and enzymes.

Minerals: help with normal growth, function and maintenance of good health

Calcium

Essential element (body does not make it on its own) that contributes to building strong bones and teeth as well as maintaining nerve and muscle health. Sources of calcium include dairy products and green vegetables. Calcium supplements can also be used in patient’s that are lactose intolerant and can’t have dairy sources.

Iron

Essential element (body does not make it on its own) that contributes to carrying oxygen in red blood cells from the lungs to the body. Sources of iron include meat sources such as beef, lamb, and plant sources such as nuts, leafy greens and whole grains.

Sodium Chloride

A salt, that contributes in the maintenance of fluid balance in the body. Patients with Cystic Fibrosis lose more salt in their sweat, which means they much eat more salty foods, especially during hot weather where they will sweat more frequent. Sources of sodium chloride include salted meats, nuts, margarine.

Zinc

Contributes to the immune system, cell v=division, cell growth, wound healing, and breakdown of carbohydrates. Sources of Zinc include oysters, poultry, beans, dairy, and whole grains.

Vitamins- help your body grow, function and fight off infection

Vitamin A

Contributes to bone and tooth formation, vision health, cell function and immunity. Sources of vitamin A include liver\fish oils, dairy, eggs, and leafy green vegetables.

Vitamin D

Contributes to building and maintain strong bones and teeth. Without enough vitamin D, bones can become thin and brittle. Sources of vitamin D include Cod liver oil, dairy, egg yolks, and cheese.

Vitamin E

Is an antioxidant, protecting compounds in the body from combining with oxygen; when compounds become oxidized they become harmful to the body. Also helps keep red blood cells healthy, fights infection and maintains intestine health. Sources of vitamin E include vegetable oils, nuts, seeds, and green leafy vegetables.

Vitamin K

Contributes to blood clotting and synthesis of proteins found in plasma, bone and kidneys. Sources of vitamin K include spinach, kale, cabbage, fruits, meats, dairy and eggs.

Enzymes- help speed up the breakdown (hydrolysis) of food molecules

Taken by mouth, they function in your intestines to allow digestion of food and absorption of nutrients to feed your body. Pancreatic enzyme supplements come in capsule form. Inside each capsule are small beads that contain the digestive enzymes. Each of the beads are enteric coated, which allows them to be dissolved in the small intestine where the digestive enzymes are released and help digest food.

https://www.cff.org/Living-with-CF/Treatments-and-Therapies/Nutrition/

http://sciencelearn.org.nz/Contexts/Digestion-Chemistry/Looking-Closer/Digestive-enzymes

Medical Treatment of Cystic Fibrosis

Cystic Fibrosis treatment aims at helping patients to maintain their health so they can live a long, quality life. Each treatment plan will is individualized and tailored to the needs of every Cystic Fibrosis patient. The main structure of treatment encompasses airway clearance techniques, nutritional therapies, oral\inhaled\nebulized medications, and a fitness plan.

Airway clearance Techniques

Airway clearance techniques (ATC’s) helps to loosen the viscous, sticky mucus so it can be cleared from the patient’s lungs by coughing or deep breathing. The process of clearing the airways helps to decrease the risk of developing a lung infection and improves overall lung function.

ACT’s can be coupled with other treatments such as inhaled bronchodilators, mucus thinning medications, and antibiotics. Bronchodilators help to widen the airways by relaxing the lining in the airway walls; they should be inhaled before starting ACT’s. Mucus thinners help thin and mobilize the mucus out of the airways so it is easier for the patient to expel the mucus by coughing; they should be taken by nebulizer during ACT”s. Antibiotics help to terminate any infectious pathogens; they should be inhaled after ACT’s are finished because the lungs will be the most clear of mucus at that point allowing the antibiotics to travel deeper into the lungs.

Airway Clearance Techniques include:

Coughing

Reflex that helps clear mucus due to high-speed airflow. Sometimes even after long periods of coughing, mucus will not be loosened, this will cause shortness of breath.  

Huffing
Technique performed by taking a breath in a forcefully exhaling afterwards. You progressively start at softer huffing and work up to a more deep and forceful huff. Huffing is not as forceful as coughing, but can work better for longer periods of time without causing shortness of breath or fatigue.

Active Cycle of Breathing (ACBT)
This cycle of breathing asks the patient to take normal, gentle breathing in the lower while relaxing the upper chest and shoulders. Then patient then begins deep breathing exercises- holding the breath for 3 seconds to allow more air into the lungs and loosen the mucus. This may also be done with chest clapping or vibrating, following by more normal breaths. The cycle finishes with a force expiration technique where huffs vary in length of duration.

Autogenic Drainage (Self Drainage) This drainage uses various airflow levels to move mucus. It uses very high airflows in different parts of the lung. The patient inhales to different levels and then adjusts how they breath out to maximize airflow and expel mucus. Breathing out may best be done by huffing in a certain pattern tailored to the patient’s ability to breath.

High Frequency Chest-Wall Oscillation (The Vest)
A specialized vest is attached to a machine that vibrates the vest at high frequency. A small amount of pressure is applied to the chest through the vibration of the chest. The best vibrates and moves mucus into bigger airways so the patient can expel them by coughing. The patient can turn the machine whenever needed so they may huff or cough.

Nutritional therapies

Good nutrition is an important part of maintaining health with Cystic Fibrosis. Maintaining a balanced, high-calorie diet will help supply your body with nutrients it needs to stay healthy and fight off infection. 

Oral\inhaled\nebulized medications

These medications go directly into to source of inflammation and mucus build-up in the lungs. This makes then highly effective. Two mucus thinning medications are Dornase Alfa (Pulmozyme) and Hypertonic Saline solution.

Dornase Alfta

A mucus thinning medication that helps a patient in making it easier to expel mucus by coughing or huffing. Getting this mucus out of the lungs helps to reduce the risk of lung infections or slow down damage from chronic lung infections. It’s mechanism of action is “cutting” into the mucus like scissors. It cuts up the DNA strands outside of the cell that makes Cystic Fibrosis mucus thick and sticky.

Adverse effects are usually mild and are temporary. They include changes in or loss of voice, discomfort in the throat, red watery eyes, rash, dizziness, fever or runny nose.

Hypertonic Saline Solution

Solution that is composed of water and high amounts of sodium; this makes the water sterile and prevents germs from getting into it. Because patients with Cystic Fibrosis have very dry airways, the hypertonic saline solution mist acts as a mucus thinner by helping clear the thick mucus from the lungs and reducing the risk of lung infections.

Adverse effects include increasing coughing, sore throat and chest tightness (saline can sometimes irritate the airways.  

Fitness plans

Maintaining regular physical activity provide several benefits for patients with Cystic Fibrosis. These benefits include improved lung function, increased bone strength, more energy, management of diabetes and heart disease and better overall health. Just adding 21 minutes of physical activity (walking, running, lifting, biking) can help improve your health. Participating in physical activity can also be planning in a pattern during the weak- an example would be Monday, Wednesday, Friday are running days. Tuesday and Thursday are lifting and core exercising days. During physical activity, the patient is allowed to perform in as much as they feel comfortable with.

http://www.dailymail.co.uk/health/article-3085695/New-treatment-brings-hope-cystic-fibrosis.html

https://www.cff.org/Living-with-CF/Treatments-and-Therapies/

https://libbyasbestos.org/education/maintenance/airway.cfm

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

Symptoms and Signs and Cystic Fibrosis

The severity of symptoms\signs of Cystic Fibrosis are individualized, where the disease process may improve or worsen over time. The age of onset is also variable, sometimes appearing at birth, or later developing during adolescence and adulthood.

5 most frequent symptoms of Cystic Fibrosis Patients

Symptoms	Occurrence in patients (Percentage)
Persistent cough that produces viscous mucus	98%
Salty-tasting skin	98%
Foul-smelling, greasy stools	94%
Weight loss and poor growth\development	94%
Infertility	98%

Symptoms in newborns may include:

Salty-tasting skin, no bowel movements within the first 24-48 hours of life, delayed growth, and failure to gain weight

Symptoms that occur later in adolescence and adulthood:

Clubbed fingers due to low oxygen perfusion,pancreatitis, weakness, increased heart rate, low bone density leading to osteoporosis, continued respiratory symptoms including pneumonia, shortness of breath, excessive mucus production, and coughing. Infertility most commonly occurs in men due to the absence of being born with a vas deferens. The vas deferens is a tube that delivers sperm from the testes to the penis. Infertility also occurs in women; viscous mucus may seal off the cervix making it difficult to become pregnant.

Symptoms related to bowel function include:

Nausea and loss of appetite, severe constipation, increased gas and bloating, distended belly, and weight loss. Excessive mucus produced in patients with CF blocks digestive enzymes produced by the pancreas; without these enzymes, the intestines can’t fully absorb nutrients from food eaten. This results in pale\clay colored stools that contain mucus or have a foul smell. Frequent straining while passing stool can cause parts of the rectum so protrude outside the anus. This is called rectal prolapse. This condition can be fixed by general surgery

Symptoms related to respiratory function include:

Fatigue, persistent coughing, wheezing, increased mucus production, recurring cases of pneumonia, shortness of breath, activity intolerance, loss of appetite, inflamed nasal passages, nasal congestion from nasal polyps

https://www.cff.org/What-is-CF/About-Cystic-Fibrosis/

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

http://www.medicinenet.com/cystic_fibrosis/page4.htm#what_are_the_signs_and_symptoms_of_cystic_fibrosis

http://www.healthline.com/health/cystic-fibrosis#Symptoms4

http://www.nytimes.com/health/guides/disease/cystic-fibrosis/overview.html

Diagnosis of Cystic Fibrosis

Doctors diagnose Cystic Fibrosis based upon results from several tests

Sweat Testing

This test is the standard for confirming a Cystic Fibrosis diagnosis because of its sensitivity and specificity, simplicity, and availability. A sweat test measures the amount of salt in sweat.For this test, the doctor triggers sweating on a small patch of skin on an arm or leg. He or she rubs the skin with a sweat-producing chemical and then uses an electrode to provide a mild electrical current. This may cause a tingling or warm feeling.

Sweat is collected on a pad or paper and then analyzed. The sweat test usually is done twice. High salt levels confirm a diagnosis of CF.

Sweat Cl Concentration Ranges

Age	Normal (mmol/L)	Intermediate (mmol/L)	Abnormal (mmol/L)
≤ 6 mo	≤ 29	30–59	≥ 60
> 6 mo	≤ 39	40–59	≥ 60

Pancreatic Function Test

Pancreatic function should be assessed at the time of diagnosis, usually by measuring fecal fat excretion in stool within a period of 72 hours.

Pulmonary Chest X-ray

Chest x-rays are done at times of increasing pulmonary symptoms (difficulty breathing) by routinely checking every 1 to 2 years. X-rays define the extent of lung damage, airway abnormalities, hyperinflation (alveoli become less elastic in lungs) and bronchial wall thickening. 

On the left is a healthy lung x-ray, on the right is a x-ray with the diagnosis of Cystic Fibrosis

Pulmonary Function test

This is the best indicator of patient status and should be done routinely 4 times a year. Pulmonary function can now be evaluated in infants by using a raised volume rapid thoracoabdominal compression technique. Pulmonary function tests indicate hypoxemia with a expiratory flow between 25% and 75%. Fifty percent of patients experience improvement in pulmonary function after aerosol administration of a bronchodilator.

Also, screening oropharyngeal or sputum cultures should be done 4 times a year.

Newborn Screening Test

This is performed through a genetic test or a blood test. The genetic test shows whether a newborn has abnormal\mutated CFTR genes. The blood test shows whether a newborn's pancreas is working properly. Universal newborn screening for Cystic Fibrosis is now standard in the US; > 90% of cases are first identified by newborn screening, but about 10% are not diagnosed until adolescence or early adulthood. Despite advances in genetic testing, the sweat Cl test remains the standard for confirming a CF diagnosis in most cases because of its sensitivity and specificity, simplicity, and availability.

Carrier Screening Test

CF carrier screening is recommended for couples who are planning a pregnancy. If both parents carry a CFTR mutation, prenatal screening of the fetus can be done by amniocentesis (procedure where a small amount of amniotic fluid is withdrawn and tested).

http://felixonline.co.uk/science/2949/tackling-cystic-fibrosis/

http://www.merckmanuals.com/professional/pediatrics/cystic-fibrosis-cf/cystic-fibrosis