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Cystic Fibrosis Case Study

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Teresa Vasquez

on 15 June 2015

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Transcript of Cystic Fibrosis Case Study

Administer with all meals and snacks
• Within 30 minutes

Can swallow or sprinkle on food
• Prolonged contact with the enzymes can cause mucosal ulcers; if sprinkled on food, rinse mouth afterward

PERT doses should be limited to 2500 lipase units/kg body weight per meal to avoid fibrosing colonopathy.

Monitor stools for adequate dosing
• Loose, fatty - increase dose
• Constipation - decrease dose

Often given with fat-soluble vitamins (A, D, E, & K)

Prednisone
• Corticosteroid

• Decreases inflammation

• Side effects - Glucose intolerance, cushingoid effects (moon face, weight gain), immunosuppression, increased appetite, cataract formation, multiple bone fractures secondary to osteoporosis or osteopenia

• Report any signs of infection (fever, sore throat, cough)

Albuterol
• Short-acting adrenergic β2 agonist

• Bronchodilator

• Side Effects: tachycardia, tremors, angina, anxiety, restlessness, insomnia, headache, dizziness, sweating, palpitations, sweating, hypokalemia, dry mouth

• After shaking metered dose inhaler, exhale, place mouthpiece in mouth, inhale slowly, while depressing inhaler, hold breath, remove, exhale slowly. Give doses at least 1 minute apart

• Use immediately prior to chest physiotherapy and exercise, nebulizer treatments, and as a rescue medication

Pancreatic Enzymes
Pathophysiology
The CFTR (cystic fibrosis transmembrane conductance regulator) gene mutates and becomes defective.
Mucus in lungs becomes thick, builds up and allows bacteria to grow causing infections
Mucus blocks the ducts in the pancreas. Digestive enzymes cannot reach the small intestine which leads to malnutrition and malabsorption.
Excess salt released in sweat can upset electrolyte balance, cause dehydration, and decrease blood pressure.
CF patients are at a higher risk for diabetes, osteoporosis and infertility.


What is CF?
A hereditary disorder

Affects the exocrine glands

It causes the production of abnormally thick mucus, leading to the blockage of the pancreatic ducts, intestines, and bronchi and often results in respiratory infection.

Medications
Variances of CF



Cystic Fibrosis Case Study


Ken Smith is a 3 year old Caucasian
male just being discharged from the
hospital today after an exacerbation of his
Cystic Fibrosis (CF). He is being started on
new medications and vitamins.


References
Quality Indicators

Standards of Care
Patient Education


6 months later
Unfortunately Ken has not been feeling
well for the past few days and his parents
have noticed a loss of appetite due to his
increased worsening cough and
sputum production.
His parents take him to the
hospital where he is re-admitted for another exacerbation
CF affects primarily whites (1 in 3500 in North America and Europe)

1 in 29 whites in the US is a carrier (autosomal recessive disease)

Do not generally see older adults with CF due to disease life expectancy for CF being approximately 37 years

Median age at diagnosis is 6 months, when dx later it is preceded by other vague diagnoses like failure to thrive

Comorbidities
Growth Failure – Due to malabsorption, increased energy needs, and reduced appetite

Diabetes – Due to insulin insufficiency

Bone deterioration, lung scarring, heart dysrhythmias

How is CF diagnosed?
Newborn genetic testing or blood test (faulty CFTR gene)

Sweat Test (amount of salt present)

Prenatal amniocentesis or chorionic villus

CF Carrier Testing

People with one normal CFTR and one faulty CFTR are CF carriers

Other tests: chest x-ray, sinus x-ray, lung function test, sputum culture

Important Labs
Fecal fat
CMP
CBC
Liver Function
Glucose
A1C
Amylase
Lipase

How do we treat CF?
CPT or pulmonary rehab programs

Pancreatic Enzymes, vitamin supplementation (fat soluble), nutrition therapy

O2 Therapy, lung transplant

Antibiotics, anti-inflammatories, bronchodilators

Treat comorbidities

Treatment Goals
Prevent and control lung infections

Loosen mucus from the lungs

Prevent and treat intestinal blockages

Provide adequate nutrition

Prevent dehydration

Ackley, B., & Ladwig, G. (2012). Nursing diagnosis handbook: An evidence-based guide to planning care

(Tenth ed.). Maryland Heights: Mosby Elsevier.


Anderson, R. R. (2009). Religious Traditions and Prenatal Genetic
Counseling.American Journal

Of Medical Genetics. Part C, Seminars in Medical Genetics,151C(1), 52–61. doi:10.1002/ajmg.c.30203


Baker, R.D., Baker, S.S., Katkin, J.P. (2015). Cystic fibrosis: Assessment and management of

pancreatic insufficiency. Uptodate. Retrieved from http://www.uptodate.com/home/index.html


Cystic Fibrosis Center. (n.d.). Retrieved June 6, 2015, from http://www.cincinnatichildrens.org/service/c/cystic-fibrosis/quality/


George, C., & Hazle, L. (n.d.). Cystic Fibrosis. Retrieved June 6, 2015, from http://nursing.advanceweb.com/Continuing-Education/CE-

Articles/Cystic-Fibrosis.aspx


Hockenberry, M. J., Wilson, D., & Wong, D. L. (2012).
Wong's Essentials of Pediatric

Nursing9:Wong's Essentials of Pediatric Nursing.
Elsevier Health Sciences.


How Is Cystic Fibrosis Diagnosed? (2013). Retrieved May 30, 2015, from

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

Huether, S. E., & McCance, K. L. (2013).
Understanding pathophysiology 5th edition
. Elsevier Health Sciences.


Lewis, S. L., Heitkemper, M. M., Dirkson, S. R., O’Brien, P. G., & Bucher, L. (2014). Medical-

surgical nursing: Assessment and management of clinical problems (9th ed.). St. Louis: Mosby-

Elsevier.

Moran, A. (2010). Clinical Care Guidelines for Cystic Fibrosis–Related Diabetes. Retrieved May

30, 2015, from http://care.diabetesjournals.org/content/33/12/2697.full

NCLEX Practice Exam for Safety and Infection Control - RNpedia. (n.d.). Retrieved June 6, 2015, from http://www.rnpedia.com/practice-

exams/nclex-exam/nclex-practice-exam-safety-infection-control/


Psychol, J.P. (1992). Parental response to cystic fibrosis: a contextual analysis of the

diagnosis phase. Web. Retrieved 6/7/2015 from http://www.ncbi.nlm.nih.gov/pubmed/1484333


Respiratory NCLEX Practice Test Part 1. (2010). Retrieved May 30, 2015, from

http://nclexreviewers.com/nclex-review/respiratory-system/respiratory-nclex-practice-test-

part-1.html

Saiman, L., & Siegel, J. (n.d.). Infection Control in Cystic Fibrosis. Retrieved June 6, 2015, from http://cmr.asm.org/content/17/1/57.full


Skidmore-Roth, L. (2011). Mosby’s nursing drug reference 24th ed. St. Louis MI: Mosby Elsevier.

What is cystic fibrosis? Animation. (2011). Retrieved May 30, 2015, from https://www.youtube.com/

watch?v=FMAOEOmLoUE


By:
Rachel Hinker
Ashley Jewell
Heather Sager
Katie Schuchard
Katie Svoboda
Teresa Vasquez
Introduction
A. Opening and maintaining a patent airway
ABC's/ Patient Safety
A male adult client with cystic fibrosis is admitted to an acute care facility with an acute respiratory infection. Prescribed respiratory treatment includes chest physiotherapy. When should the nurse perform this procedure?
A. Immediately before a meal

B. At least 2 hours after a meal

C. When bronchospasms occur

D When secretions have mobilized

You are assessing a 5-year-old who has been admitted with an acute respiratory infection. You review the chart and see the child has cystic fibrosis. What is the priority for assessment?
A. Opening and maintaining a patent airway

B. Modifying the child's diet

C. Checking levels of pancreatic enzymes

D. Giving antibiotics to the child

NCLEX Questions
B. At least 2 hours after a meal
To prevent aspiration and vomiting
The client has begun medication therapy with pancrelipase (Pancrease MT). The nurse evaluates that the medication is having the optimal intended benefit if which effect is observed?
A. Weight loss

B. Relief of heartburn

C. Reduction of steatorrhea

D. Absence of abdominal pain

A client is prescribed long-term use of oral prednisone for treatment of chronic asthma. The nurse should instruct the client to watch for which of the following?
A. Weight gain and fluid retention

B. Nervousness and insomnia

C. Chest pain and tachycardia

D. Dry mouth and constipation

A nurse is providing instructions to the parent of an adolescent client who has a new prescription for albuterol (Proventil) PO. Which of the following instructions should the nurse include?
A. “You can take this medication to abort an acute asthma attack.”

B. “Tremors are an adverse effect of this medication.”

C. “Prolonged use of this medication can cause hyperglycemia.”

D. “This medication can slow skeletal growth rate.”

NCLEX Questions
During client teaching, the nurse stresses the importance of taking prednisone exactly as prescribed and cautions against discontinuing the drug abruptly. A client who discontinues prednisone abruptly may experience:
A. Hyperglycemia and glycosuria.

B. Acute adrenocortical insufficiency.

C. GI bleeding.

D. Restlessness and seizures.

B. “Tremors are an adverse effect of this medication.”
A. Weight gain and fluid retention
C. Reduction of steatorrhea
B. Acute adrenocortical insufficiency
Teach s/s of common complication of CF & when to seek care
Recognize and treat infections as early as possible (characteristic signs like fever, tachypnea, and chest pain may be absent)

Look for weight loss, decreased activity, and anorexia instead

Hemoptysis often accompanies pulmonary infection. If greater than 250 ml/24 hours seek help immediately

CF kids should be kept 6 feet or more a part due to cross infection

While lung infections in people with CF pose no danger to the public, they do pose a significant danger to others with CF.

Droplet particulate containing microbes from a cough or sneeze can travel up to 6 feet

Notify day care, preschools, or other organized group activities to keep CF kids apart.

Pulmonary Complications
Bronchiectasis, atelectasis, & hyperinflation and bacterial infections from stagnate mucus in airways

Teach signs and symptoms of pneumothorax (overtime blebs and bullae my rupture)

An early s/s to watch for are subtle drop in oxygen saturation especially when accompanied by tachycardia, dyspnea, pallor & cyanosis

Use oxygen like a drug, as prescribed because too much oxygen can be harmful like in COPD patients it alters their drive to breath

Metabolic
Greater likelihood for cystic fibrosis-related diabetes [CFRD] from changes in pancreatic architecture and diminished blood supply over time. Watch for S/S

Most likely complication of CF

By age 30 50% of patients will develop diabetes

Has characteristics of both diabetes type 1 and type 2

Reproductive
95% of male children are sterile caused by blockage of the vas deferens
Growth & Development
May be restricted from decreased absorption of nutrients, vitamins, and fats
Gastrointestinal
Greater risk of prolapse of the rectum and bowel obstruction d/t large bulky stools, Malnutrition, and increased intra-abdominal pressure second to paroxysmal cough
Other Education
Need for daily exercise / activity to stimulate mucus excretion, increase vital capacity, as well as increased sense of well -being, and increased self-esteem
Resources & Support Groups
Cystic Fibrosis Foundation http://www.cff.org/

American Lung Association http://www.lung.org/lung-disease/cystic-fibrosis/?referrer=https://www.google.com/

Links to support groups http://www.cfliving.com/resources/resources-links.jsp

Cystic Fibrosis support groups on facebook https://www.facebook.com/CFFColorado

Pediatric dietitian consult for recommended CF Diet → high-fat, high-calorie needed

Physical therapy for CPT (chest physiotherapy) and or ACT (airway clearance therapies)

Percussion and or postural drainage (twice daily) to promote airway clearance
Teaching of flutter mucus clearance device (handheld pipe that facilitates removal or mucus)
Teaching of Huffing breathing exercises
Positive expiratory pressure mask

Psychologist / therapy for child and entire family to address
Patient anxiety, depression, and disturbed self-image related to this chronic illness
Family care giver strain, copping mechanisms
Address family feelings of guilt and self-recrimination

Parents with children who have CF report elevations in both situation-specific and global parenting stress, and a greater number of depressive symptoms than a norm group.
Have helpers
Seek respite care

Genetic Counseling
1 in 4 chances of having another child with CF
Recommendations
&
Resources
http://www.bbc.co.uk/schools/gcsebitesize/science/21c/genes/genetic_diseasesrev2.shtml
Recommendations
Cultural Values & Beliefs
Gene therapies
There are no religious bars to mainstream medical care for healthy or sick newborns, although some potential therapies currently being researched would be unacceptable, e.g., embryonic stem cell transfer or genetic enhancement.
General Association of Regular Baptist Churches

Genetic counseling –potential positive screenings have moral and religious implications (Gods Will)
Brings up questions concerning beginning of human life
Soul or spirit arrival
Personhood
Some denominations discourage prenatal diagnosis while others only discourage invasive therapies prior to birth

Organ Transplantation in advanced CF patients
There are no religious bars to most elements of mainstream medical care for healthy or sick newborns; however, the issue of organ transplantation, especially of the major organs, is unsettled.
Antiochean Orthodox Christian Archdiocese of North America
Invasive treatments:
Unless there is direct benefit to a living person, physical examination, medical testing, medical imaging and photography are discouraged. When such an evaluation is necessary, noninvasive procedures (physical examination, imaging, photography) are more acceptable than invasive procedures (tissue sampling, incisions). In essence, a proportionately weighty reason must be present to allow progressively more invasive procedures. Autopsy is rarely allowed. If required, instructions are available from the local Orthodox rabbi or the Chevra Kaddisha.
Orthodox Judaism

There are two key outcomes that are considered in a patient with CF:

1. Lung function (FEV1) - this indicator allows medical professionals to see what the patients’ capacity is for normal breathing. Healthy people range from 85-115 percent, and children with the diagnosis of cystic fibrosis lose an average of between 2-4 percent each year. By monitoring lung function in these children outcomes can be improved.

2. Body mass index (BMI) - this indicator allows medical professionals to monitor growth and development of children with the disease. These patients have a difficult time digesting and absorbing food, this makes a high fat and high calorie diet extremely important for management of the disease.

Nutrition and lung health are closely linked making these two indicators important for overall cystic fibrosis care.

The most important safety consideration for this population is infection control.

In order to decrease the transmission of patient to patient infection as well as healthcare worker to patient transmission the following should be adhered to:

Standard precautions
- hand hygiene, disinfecting equipment, barrier precautions including the use of a mask, gloves and gown based on the anticipated exposure of the client.

Transmission based precautions
- this applies to patient who have highly contagious infections and include, contact, dropout and airborne precautions.

Hand hygiene
- healthcare workers must wash their hands before and after any contact with the patient or their respiratory equipment.

Care of respiratory therapy equipment
- reusable respiratory equipment must be clean with soap and hot water then disinfected then rinsed with sterile water because tap water and distiller water may contain pathogens.

Segregation
- it is important to keep CF patients apart because it has been shown that they are able to infect one another more easiy this occurs via direct contact with infected secretions. Pathogens like B. cenocepacia harbored in some patients will actually replace less deadly pathogens in other patients. This results in increased morbidity and mortality.

Standards of Care Cont.
Cystic Fibrosis guidelines suggest that patients have four clinic visits a year for their disease.

It is recommended that they receive lung function test frequently as well as sputum cultures and evaluations of nutritional health.

Clinical management after the diagnosis of CF consists of slowing the progress of this lifelong disease. Management includes airway clearance therapies these include postural drainage and percussion to loosen mucus and inhaled medications to thin the mucus. These therapies facilitate lung clearance and in turn increase respiratory function.

Infection control is very important in the management of the disease. The risk of pulmonary exacerbation for these patients is much higher but by controlling infections it is possible for these people to maintain their health.

Patients should receive immunizations in accordance with CDC recommendations, including an annual inactivated influenza vaccine.

Children with CF should keep a safe 6 foot distance from those with a cold or the flu, don't share straws or Chap stick and wash their hands before eating or touching their face.

Nutrition growth can be supervised with weight maintenance and electrolyte balance, it is crucial for this population especially when they are ill or are having a pulmonary exacerbation. Children with this diagnosis should grow at or above the 50th percentile in regards to the body mass index.

Physiological
Ineffective airway clearance r/t increased production of thick mucus AEB hospital admission for respiratory support.

• Auscultate breath sounds every 1 to 4 hours.

• Administer oxygen as ordered to maintain required oxygen saturation level.

• Position client to increase respiration.

• Encourage client to cough and deep breath using the incentive spirometer.

• Teach the client the huff cough.

• Teach and administer medications to help manage disease exacerbation.

Risk for infection r/t immunocompromised state from chronic disease.

• Teach and monitor for signs of infection including fever, redness, or discharge.

• Monitor temperature regularly to catch a fever outbreak early.

• Teach and practice good hand hygiene.

• Teach and help provide proper nutrition to prevent illness.

• Teach and encourage standard precautions.

Psychosocial
Caregiver role strain r/t dependent child with chronic illness AEB parents verbally stating they are overwhelmed with the treatment and management plan.

• Provide caregiver support with stress management and supportive resources.

• Monitor for signs of depression, anxiety, burden, or deteriorating health to intervene early.

• Encourage caregivers to take personal time for themselves to meet their own needs.

• Encourage caregiver to talk about what they are feeling and not being afraid to reach out for help.

• Provide contacts for help including support groups for the parents or home health as a support.

• Assess financial resources to understand what kind and level of support they need.

Impaired home maintenance r/t extensive daily treatments and medications necessary for health AEB noncompliance with the treatment plan and need for hospital admission.

• Assess the concerns of the family and primary care giver about long term home care.

• Provided resources for home health care and other community services to assist in the care they need long term at home.

• Provide adaptive equipment and treatment supplies to increase compliance with the treatment and medication plan.

• Ask the family what support people and systems they have so we are able to assess how much help and support they need.

• Encourage the parents and caregivers to take private time for themselves to take care of their needs.

Cognitive
Stress overload r/t intense stress associated with a child with chronic illness AEB difficulty adapting to complicated treatment regimen outside of the hospital setting.

• Actively listen to understand what is exactly causing the higher stress level.

• Categorize stressors as modifiable and nonmodifiable and help provide resources to change what is in their power to change.

• Help client reframe his or her perceptions of some of the stressors.

• Help client identify stress overload situations and life events and teach them how to deal with them.

• Provide resources such as contacts of who to contact when they do need help or have any questions.

• Provide support groups or counseling for the parents so help provide emotional support.

Fear r/t difficulty breathing AEB signs of respiratory distress including tachypnea, labored breathing, wide eyed and sweating.

• Explore coping skills to deal with difficulty breathing when it occurs.

• Teach parents what to do in the case of an exacerbation affecting breathing so they know what to do and can stay calm for the child.

• Teach the child how to improve their ability to breath when they are in distress so they know what to do.

• Teach the child and family about the treatment regimen so they know what they can do to prevent this fearful event from happening.

• Teach the parents when they should seek medical assistance

Nursing Diagnosis
A school-age child with CF asks the nurse what sports he can be involved in as he becomes older. Which of the following activities would be most appropriate for the nurse to suggest?
A. Swimming

B. Track

C. Baseball

D. Javelin throwing

What treatments in cystic fibrosis would the nurse expect to implement in the management plan of patients with cystic fibrosis (select all that apply)?
A. Sperm banking

B. IV corticosteroids on a chronic basis

C. Airway clearance techniques (e.g., Acapella)

D. GoLYTELY given PRN for severe constipation

E. Inhaled tobramycin to combat pseudomonas infection


NCLEX Questions
A. Swimming
swimming would be the most appropriate suggestion because it coordinates breathing and movement of all muscle groups and can be done on an individual basis or as a team sport.
A. Sperm banking

C. Airway clearance techniques (e.g., Acapella)

D. GoLYTELY given PRN for severe constipation

E. Inhaled tobramycin to combat pseudomonas infection
1 Year
Ken is doing awesome! He and his parents have been able to keep is CF under control and they are happy that he has not had to return to the hospital. Ken is excited that he will be starting preschool soon.
9 Month Home Visit
Since the management of CF is very complex, a home health nurse coordinates a routine care visit. We need to make sure Ken is in a healthy state and check-in with his parent in case they have any questions or concerns.
B. Contact Isolation
Contact Isolation involves the use of barrier protection (e.g. gloves, mask, gown, or protective eyewear as appropriate) whenever direct contact with any body fluid is expected.
When teaching the parents of an older infant with CF about the type of diet the child should consume, which of the following would be most appropriate?
A. Low protein diet

B. High fat diet

C. Low carbohydrate diet

D. High calorie diet

A patient is diagnosed with methicillin resistant staphylococcus aureus pneumonia. What isolation is MOST appropriate for this client?
A.Standard Precaution

B. Contact Isolation

C. Reverse Isolation

D. Respiratory Isolation


NCLEX Questions
D. High calorie diet
CF affects the exocrine glands. Mucus is thick and tenacious, sticking to the walls of the pancreatic and bile ducts and eventually causing obstruction, a moderate fat, high calorie diet is indicated

Three months after Ken's exacerbation, he seems to be feeling much better. Mr. & Mrs. Smith are talking about having another child since Ken is an only child. They decide they need more information about CF and what the possibility of having another child with CF is before they make their final decision.
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