Nursing Diagnoses

When a nurse is taking care of a patient with cystic fibrosis, there are several nursing diagnoses that she should keep in mind. Below are 5 nursing diagnoses that can commonly be applied to a patient with CF:

1) Impaired gas exchange related to increased mucus production and thick mucus secretions.

• This nursing diagnosis is especially important because it involves the oxygenation status of the patient. It is important that the nurse recognize the importance of this diagnosis and quickly intervenes to improve gas exchange and oxygenation. This diagnosis is summarized in further detail in the table below. 

2) Risk for infection related to increased mucus production.

• Nurses should be aware of this diagnosis and watch for signs of infection in the patient. Any signs of infection should be documented and communicated to the provider, so that appropriate therapy (i.e. antibiotics) can be started. 

3) Risk for imbalanced nutrition: less than body requirements related to reduced absorption of nutrients.

• The nurse should recognize this diagnosis and make sure that the child is consuming enough food. Intakes and outputs should be monitored closely and parenteral nutrition/vitamin supplements should be administered correctly and in a timely manner. 

4) Anxiety related to respiratory distress and length of stay in hospital.

• Addressing the anxiety of the patient with CF can improve the therapeutic relationship between the nurse and the patient. It is important to acknowledge that all of the medical treatment they are undergoing can be very stressful on them and measures to reduce their stress/anxiety should be put in place. 

5) Caregiver anxiety related to lack of knowledge about child's diagnosis and uncertainty about the child's prognosis.

• Most times, the patient with CF that a nurse is taking care of will be a child. Therefore, it is important to acknowledge the caregivers in the room and address any anxiety that they may be having over this whole situation. 

In this post, the focus will be on diagnosis #1. There are several different interventions that a nurse can employ to help a patient with CF improve their gas exchange and help with mucus clearance. The table below summarizes the interventions and goals for a child with this diagnosis.

Actual/Potential	Related To	Plan/Outcome	Interventions
Impaired gas exchange	Increased mucus production/thick mucus secretions	Plan is for the child to have increased mobilization of mucus secretions as evidenced by decreased respiratory distress, cyanosis, and coughing.	1) Perform chest physiotherapy frequently 2) Administer O2 3) Assist the child with deep-breathing techniques 4) Administer bronchodilators 5) Assist the child with percussion and/or vibration techniques to clear mucus

References: 

Speer, K. (n.d.). Pediatric Care Planning: Cystic Fibrosis. Retrieved February 28, 2015, from http://www.wolterskluwerhealth.com/pages/welcome.aspx

Silvestri, L. (2011). Pediatric Respiratory Disorders. In Saunders comprehensive review for the NCLEX-RN examination (5th ed., pp. 481-483). St. Louis, Missouri: Elsevier/Saunders.

Page, S. (2013, November 18). Nursing Care Plan & Diagnosis for Cystic Fibrosis with Nursing Interventions & Goals. Retrieved February 28, 2015, from http://www.registerednursern.com/nursing-care-plan-diagnosis-for-cystic-fibrosis-with-nursing-interventions-goals/

Nursing Care for Cystic Fibrosis

As a nurse caring for a patient with cystic fibrosis, there are several different interventions that you should have in place. As a patient with cystic fibrosis, it is important that you recognize your needs and communicate them to your nurse and other members of your patient care team to ensure that you receive the care that you deserve.

One of the primary goals of treatment is to prevent and treat pulmonary infections. This can be accomplished by improving aeration/oxygenation, removing the thick secretions, and providing the patient with antibiotic medications. A primary concern for the nurse is the patient's airway. It is important that the patient's respiratory status is monitored and the airway is clear from secretions so that the patient can breathe as normally as possible. Lung sounds should be monitored and various ways of expelling mucus should be employed (including chest physiotherapy, flutter mucus clearance devices, and hand-held percussors/special percussor vests. It will be important to have oxygen available in the patient's room in case of a bad episode of decreased breathing and oxygenation.

The second area of focus for the nurse will be on the gastrointestinal system. The nurse should ensure that the child's nutritional needs are being met with a high-calorie, high-protein diet that is rich in many vitamins. The nurse should be monitoring daily weights and heights, since many children with CF tend to not grow as quickly. The nurse should also pay attention to any bowel movements that the patient has and listen to the bowel tones in the intestines to make sure there is no intestinal obstruction. The stool should be observed to determine how sticky and difficult to pass it may be. The nurse should teach the child to sit upright after eating to prevent the development of gastroesophageal acid reflux.

Some other areas the nurse should focus on include: checking blood glucose levels, providing emotional support to the parents/child, and educating the child/parents about the care involved. It is important that the nurse supports the patient as they undergo the different stages of childhood development. The nurse plays a very important role in preparing the parents and child for home care with cystic fibrosis. This includes education on different pieces of equipment used to manage the disease and education on signs and symptoms to watch for that may indicate a serious complication and the need for hospital care.

The table below summarizes what areas of focus the nurse should maintain during their care of the patient with cystic fibrosis.

System of Focus	Nursing Interventions
Respiratory System	-prevent and treat pulmonary infections -improve oxygenation -remove thick mucus secretions (chest physiotherapy, flutter device, percussion vests/hand percussion) -administer antibiotics -listen to lung sounds -have oxygen available in room
Gastrointestinal System	-ensure adequate nutrition of the patient -diet should be high-calorie, high-protein, and high in vitamins -monitor daily weight/height -watch for signs of constipation/intestinal obstructions -listen to bowel tones -observe stool for stickiness -prevent GERD by placing child upright after eating
Other	-monitor blood glucose levels -monitor bone growth -provide emotional support to parents and child -provide support to child as they undergo stages of childhood development -provide patient and parent education -prepare patient and parents for home-care

There are many helpful websites with information on the nursing care of the patient with cystic fibrosis. These websites are valuable resources for both nurse and the patient. It is important that the patient understand their diagnosis and can verbalize to the nurse what their needs are. Look at the websites below for more information. They all provide information on what areas of focus should be in nursing care, the importance of interdisciplinary collaboration to care for the patient with CF, and some information on CF centers that specialize in the care of cystic fibrosis patients. 

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

http://nursing.advanceweb.com/Continuing-Education/CE-Articles/Cystic-Fibrosis.aspx

http://www.elsevier.com/__data/promis_misc/2005.pdf

References: 

Silvestri, L. (2014). Pediatric Respiratory Disorders. In Saunders comprehensive review for the NCLEX-RN examination (6th ed., pp. 482-483). St. Louis, Mo.: Elsevier/Saunders

Cystic Fibrosis Treatment Options

Many individuals struggle with the fact that cystic fibrosis has no cure. However, treatment and management for the disease has progressed over the years and it is becoming possible to live a longer, fuller life. Seeing a doctor who specializes in CF or going to a cystic fibrosis care center can help the individual receive the personalized care they will need.

When treating cystic fibrosis there are several goals that the healthcare team will have in mind. This includes preventing and managing lung infections, clearing sticky mucus out of the lungs, preventing and managing blockages in the intestines, making sure the individual receives adequate nutrition, and preventing dehydration in the individual.

Lung problems are one of the most frequently experienced symptoms for those with cystic fibrosis. Treatment of lung problems can include physical therapy, exercise, and medication. Chest physical therapy involves pounding the chest and back repeatedly (either with hands or a machine) to help clear out the thick, sticky mucus in the lungs. Many people find it easiest if they can use some type of electronic device for chest physical therapy. There are several options for this including mechanical percussors, inflatable therapy vests, and masks. Chest physical therapy is often combined with some sort of breathing exercise to best clear out the mucus from the lungs. An example of an inflatable vest and a mask is pictured below. There is also a video that shows what a typical treatment process can look like to manage common lung symptoms.

Regular aerobic exercise can be very beneficial for those with cystic fibrosis. It is possible that with enough aerobic exercise, the amount of chest physical therapy needed can decrease. When a person is engaged in aerobic exercise, their breathing increases and becomes harder, which helps loosen up the mucus. However, people with CF tend to lose a lot of salt in their sweat. Therefore, it may be important to be on some sort of modified high-salt diet if aerobic exercise is done frequently. 

A variety of medications can be used to help treat some of the common lung symptoms with CF. Antibiotics are frequently used to treat recurring lung infections. Anti-inflammatory medications may also be prescribed to help reduce swelling in the airways. Bronchodilators can be used to help open up the airways and are often taken before chest physical therapy to help improve the amount of mucus that can be coughed out. A provider may also prescribe special medications that can reduce the stickiness of the mucus. It is important to know that if lung symptoms persist and become very severe, oxygen therapy may be needed. A lung transplant is the most drastic option and is only used when the disease has progressed to a point where other treatment options are no longer effective. 

The second most common symptoms experienced involved the intestines and digestive system. Nutritional therapy is typically the first-line of treatment for these symptoms. Getting adequate nutrition is very important as it allows for the child to grow and develop at a normal rate and have enough strength to fight off the frequent lung infections. The diet should be rich in calories, fats, and proteins. Some of the common components of nutritional therapy include: enzymes taken orally to help with fat and protein digestion, vitamins A, D, E, and K supplements, high-calorie shakes, and a high-salt diet combined with aerobic exercise. If the child is still unable to get enough nutrients, they may need to have a feeding tube at night that supplies them with additional nutrients at they sleep. In severe situations, surgery may be needed to remove intestinal blockages. 

Overall, treatment of cystic fibrosis has improved drastically in recent years. Providers now know exactly what steps should be taken to improve the health of the patient and the life expectancy of patients is increasing. These steps include: 

• prompt early diagnosis 
• nutritional support 
• improving mucus drainage 
• antibiotics and anti-inflammatory medications 
• good hygiene practices to reduce the number of infections 

When this basic outline is followed (with additional treatment options individualized to the needs of the patient) cystic fibrosis can be managed to a point where the individual will be able to enjoy their life with their symptoms under control.

References:

How is Cystic Fibrosis Treated? (2013, December 26). Retrieved February 14, 2015, from http://www.nhlbi.nih.gov/health/health-topics/topics/cf/signs

Cohen-Cymberknoh, M., Shoseyov, D., & Kerem, E. (2011). Managing Cystic Fibrosis: Strategies That Increase Life Expectancy and Improve Quality of Life. American Journal of Respiratory and Critical Care Medicine, 183(11), 1463-1471.

Common Signs and Symptoms of CF

According to the Cystic Fibrosis Foundation, the most commonly seen signs in an individual with CF include:

• skin that tastes salty 
• persistent cough (that can include mucus production) 
• frequent lung infections 
• wheezing/shortness of breath 
• poor growth/slow weight gain 
• greasy, large stools or constipation

The signs that are usually first detected when the individual is a baby include salty tasting skin and constipation. Parents typically notice the salty taste of the skin when kissing their newborn. If this is paired with the baby's inability to pass a stool in the first days of life, the baby's provider should be notified.

It is important to know that CF can affect many different organs within the body and that not all of the outward signs can capture this full picture. The organs that can be affected are summarized in the image below.

Respiratory System: The thick and sticky build up of mucus experienced by those with CF makes the lungs prone to the accumulation of bacteria and infections. This will cause frequent coughing (usually with mucus production). Besides lung infections, the individual is also at risk for sinus infections, bronchitis, and pneumonia. Some of these infections can become more serious and cause permanent lung damage. If the lungs are not able to provide oxygen to the rest of the body, clubbing in the fingers and toes may be seen. Clubbing is when the tips of the fingers and toes become wider and more round. Dr. Michael Marcus explains some common CF signs/symptoms

Digestive System: The overproduction of mucus in CF can block tubes in the pancreas, which ultimately presents needed nutrients from reaching the intestines. This leaves the intestines unable to absorb fat and proteins which causes the greasy, black stools. If the blockage is too much in the intestines, constipation can result (this is the most commonly seen in newborns). The inability to absorb necessary nutrients is also responsible for the poor weight gain and growth seen in children with CF. A variety of more serious problems including gallstones, diabetes, and infection of the pancreas can also occur if digestive symptoms become too severe. 

Reproductive System: Men with CF are born without a vas deferens (the tube that transport sperm from the testes to the penis) which leaves them infertile. Women with CF may have a great deal of mucus blocking the cervix, which can make it difficult to get pregnant. 

Skin: Because of an imbalance in minerals, CF causes the salt to be very sweaty. When too much sweat is lost in the salt, it can quickly lead to severe dehydration. Individuals who are very dehydrated may have a fast heart rate, be very tired, have a decreased blood pressure, and can even experience heat stroke. 

Below, Dr. Michael Marcus summarizes some of the common signs/symptoms seen in cystic fibrosis.  

References: 

Diseases and conditions: Cystic fibrosis - Symptoms. (2012, June 13). Retrieved February 8, 2015, from http://www.mayoclinic.org/diseases-conditions/cystic-fibrosis/basics/symptoms/con-20013731

What Are the Signs and Symptoms of Cystic Fibrosis? (2013, December 26). Retrieved February 8, 2015, from http://www.nhlbi.nih.gov/health/health-topics/topics/cf/signs

About CF: Causes, Signs & Symptoms of Cystic Fibrosis | CF Foundation. (2014, May 1). Retrieved February 8, 2015, from http://www.cff.org/AboutCF/

Getting the Diagnosis

Now that cystic fibrosis has become much more understood in the medical world, the importance of testing for this disease at a very young age has also been recognized. All states in the US, therefore, screen all newborns for CF. Typically, this is done with a genetic test and/or a blood test. The genetic test is able to determine if the newborn's CFTR genes are working properly and the blood test is able to check the function of the pancreas.

If either one of these tests suggest that the newborn may have cystic fibrosis, your healthcare provider will then want to confirm this by using a sweat test. This is one of the most accurate ways to get a confirmation of a cystic fibrosis diagnosis. The provider will try and get the newborn to sweat in a small patch on either their arm or leg by rubbing a sweat-producing chemical on an area of skin. Once the newborn has begun sweating, they will apply an electrode which will cause a mild electrical current to run through the skin, which helps get the medication into the skin and initiate sweating. The provider will then collect and analyze a small sample of the sweat. Specifically, they will be measuring the amount of salt in the sweat. If high levels of salt are observed, this can confirm the diagnosis for cystic fibrosis.

At this point, the provider may want to order a series of other tests. There may be more genetic tests to determine the exact type of CFTR gene mutation, which can help the patient and caregivers understand what the progression of their type of CF may look like. A chest X-ray can also be beneficial to look at the inflammation level in the lungs (since this is the most common problem with CF). A sinus X-ray may also be ordered to check the level of sinus infections, another common CF complication. Lung function tests can help your provider see how well the lungs are providing oxygen to the blood. Finally, a sputum culture (where the provider will take a sample of spit/mucus) will help determine whether or not there is bacteria present in the lungs (especially Pseudomonas aeruginosa). If this bacteria is present, it indicates that the cystic fibrosis has advanced and that a more aggressive treatment will be needed.

Cystic fibrosis can also be detected during pregnancy through prenatal testing. This will include amniocentesis and chorionic villus sampling. In amniocentesis, a small hollow tube will be inserted into the uterus through the abdomen. They will then take a sample of some of the amniotic fluid surrounding the baby to test for CFTR gene mutations. With chorionic villus sampling, a small tube is inserted up through the vagina and past the cervix into the placenta. A small piece of placenta will be taken and tested for CFTR gene mutations.

Finally, if you are worried that you may be a carrier for cystic fibrosis, you can be tested by a genetics counselor. They will take either a blood or saliva sample and can determine if you carry the malfunctioning CFTR gene.

References:
How Is Cystic Fibrosis Diagnosed? (2013, December 26). Retrieved January 31, 2015, from http://www.nhlbi.nih.gov/health/health-topics/topics/cf/diagnosis

Cystic fibrosis. (2012, June 13). Retrieved January 31, 2015, from http://www.mayoclinic.org/diseases-conditions/cystic-fibrosis/basics/tests-diagnosis/con-20013731

What's Happening Inside the Body

Cystic fibrosis can be a difficult disease to understand; it is not uncommon to be completely overwhelmed by all of the medical jargon and have a difficult time understanding exactly what is happening to you or a loved one. However, all of the problems that occur with CF come down a mutation in a single gene: the cystic fibrosis gene. This gene is responsible for the protein transmembrane conductance regulator (CFTR) gene. When the CF gene can't function properly, neither can the CFTR gene. Most of the problems seen with CF arise from this CFTR gene not working correctly. However, just because it can be identified that somebody has a defect with their CF gene and their CFTR gene does not mean we can tell what their disease will look like. All CFTR gene mutations look very different, which is one of the reasons why no two cystic fibrosis cases are exactly alike. So far, there have been 1,893 different CFTR mutations identified.

When the CFTR gene is not working like it should, there will be problems with chloride-transport across the surface of cells (specifically mucosal cells). This decreased secretion of chloride leads to an increased reabsorption of sodium and water, which leaves the mucus much more sticky than normal. This sticky mucus can attract bacteria which makes the person prone to infection and inflammation. This stickiness of the mucus is also what makes it so difficult to clear from the body.

The two most common complications experienced by cystic fibrosis patients are lung disease and intestinal disease. Typically, it is the severity of these two diseases that determine how long an individual can live with CF. Typically, with lung disease it will progress from bronchitis, to bronchiolitis, to bronchiectasis. This will eventually lead to cor pulmonale (abnormal enlargement of the right side of the heart) and end-stage lung disease. The cause of death is usually respiratory failure or cor pulmonale in an individual with cystic fibrosis.

References: 

Sharma, G. (2014). Cystic Fibrosis. Medscape. 

Cystic Fibrosis in the United States

According to the Cystic Fibrosis Foundation, about 1,000 new cases of CF are diagnosed each year (around 1 in every 3,500 births). Additionally, 10 million people (about 1 in every 31 Americans) are symptomless carriers for CF. More than 75% of these diagnoses are in patients under the age of 2 years. Currently in the US, about 40% of the CF population is age 18 or older. Right now, it is estimated that 30,000 people in the United States suffer from cystic fibrosis. This statistic makes it the most common lethal genetic disorder among the Caucasian population. The median age of predicted survival of those with CF is in the early 40s.  Half of the population with CF no liver longer than 28 years.

The bacteria most commonly involved in respiratory tract infections in those with CF is Pseudomonas aeruginosa. 52.5% of patients with CF contained P. aeurginosa in their sputum.  The group of patients infected with P. auerginosa includes those age 1 and younger and those between the ages of 2 and 5. P auerginosa is also one of the most deadly strains of bacteria that those with CF can become infected with. Initial signs of infection can be intermittent and multiple other strains may be involved. However, the P. auerginosa will dominate and chronic infection will occur. Once this happens, the complete eradication of the pulmonary infection is usually impossible.

References:
Cystic Fibrosis Foundation - Home. (n.d.). Retrieved January 18, 2015, from http://www.cff.org/

Genes and human disease. (n.d.). Retrieved January 18, 2015, from http://www.who.int/genomics/public/geneticdiseases/en/index2.html#CF

Lipuma, J. (2010). The Changing Microbial Epidemiology in Cystic Fibrosis. Clinical Microbiology Reviews, 23, 299-323. Retrieved January 18, 2015.

Folkesson, A., Jelsbak, L., Yang, L., Johansen, H., Ciofu, O., Høiby, N., & Molin, S. (2012). Adaptation of Pseudomonas aeruginosa to the cystic fibrosis airway: An evolutionary perspective. Nature Reviews Microbiology,841-851. Retrieved January 24, 2015.