Cystic Fibrosis

PATIENT CARE PRESENTATION

NICOLE BRUNNE
100613938
PCAR * 4302
MARCH 11TH 2018 Picture: (Fandom, 2016)
Table of contents

1. Cystic Fibrosis Condition Overview

2. Patient Overview
 Past History
 Location of treatment
 Signs/Symptoms
 Stage of CF
 Safety Precautions/or contraindications for treatment
 Current medical management
 Rehab plan
 FITT principle
What is Cystic Fibrosis/CF?
 Most common genetic disease that is life threatening
 affecting Canadian children and young adults
 CF affects the digestive system and lungs
 Causes mucus in body to become thick/sticky
 Mucus builds up and affects organs
 Severity differs from patient to patient
 Infections in the lungs, destruction of lungs and loss of lung function
 Reason for majority of deaths in CF patients
 Complication with CF
 Difficulty digesting fats/proteins
 Inability to absorb nutrients sufficiently
 CF related diabetes
 Sinus infections
(Cystic Fibrosis Canada, 2018)
Causes of CF
 Genetic disease
 Autosomal Recessive disease
 Occurs when a child inherits two abnormal
genes, one from each parent
 The parents are carriers only and do not
have CF or exhibit any symptoms
 When both parents are carriers
 25 % chance the child with be born with CF, 50
% the child will be a carrier, 25 % child will be
born neither
 Females with CF have greater deterioration of Picture: (Cystic Fibrosis Canada, 2018)
pulmonary function with increasing age and a
younger mean age of death

(Cystic Fibrosis Canada, 2018)

Pathophysiology of CF
 Defects in the cystic fibrosis gene
 Codes for a protein transmembrane conductance
regulator(CFTR)
 Functions as a chloride channel and regulated by
cAMP
 Mutations of CFTR results in abnormalities of cAMP-
regulated chloride transport
 Defective CFTR results in:
 Decreased secretion of chloride and increased
reabsorption of Na and H20 across epithelial cells
 Mucus is than more stickier for bacteria to adhere
 Staphylococcus aureus, haemophilus influenzae and
aeruginosa are common bacterial infections (Al-Nemrawi
et al, 2018)
 Secretions become thicker as well and become Picture: (British Columbia Respiratory Therapy, 2015)

difficult to clear from system

(Sharma, 2018)
In the lungs..

 Thicker than normal mucus forms in airways

 Breathing problems
 Mucus traps bacteria and this can lead to
 Frequent lungs infections
 Permanent lung damage
 Trouble “getting air in”
 Obstructive lung disease

Picture: (National Lung, Heart and Blood Institute, 2018)

(Healthwise Staff, 2017)
In the pancreas..

 Mucus blockages can affect normal

digestive processes
 Increased risk for infections
 Harder to absorb nutrients from food
 Lower than normal body weight and
development
 Difficulty gaining weight

Picture: (Healthwise Staff, 2017)

(Healthwise Staff, 2017)

Symptoms of CF
 Multi-symptom disorder
 Persistent cough with thick mucous
 SOB
 Chest infections, may lead to pneumonia
 Bowel disturbances
 Weight loss/hard time gaining weight
 Salty sweat
 Infertility for men
 Decreased fertility for women
Picture: (Mayo Clinic, 2016)

 Pulmonary involvement occurs in 90% of

CF patients
 End stage lung disease is the primary
COD (Cystic Fibrosis Canada, 2018)
Diagnosing CF
 If child is suspected to have CF
 Doctor will perform a “sweat test”
 Test measures the amount of salt content present in the sweat
 Median age of diagnosis is between 6-8 months of age
 Genetic testing, prenatal and newborn screenings can be done to diagnose CF
 If not diagnosed at birth, watch for:
 Diarrhea, not growing/gaining weight, breathing problems that do not go way, wheezing,
clubbing, rectal prolapse, polyps in nose/sinuses
 Imaging that is helpful with for diagnosis
 Radiography, CT of chest, Ultrasonography, Contrast barium edema

(Sharma, 2018)
Prognosis

 Life expectancy for individuals with CF

 Steadily increasing
 On average live to mid 30’s
 New treatments have helped individuals live
into their 40’s or longer
 Individuals with a mild form may live a normal
life expectancy
 Individuals may need a lung transplant
during the progression of the disease

Picture: (Healthwise Staff, 2017)

(Healthwise Staff, 2017)

Treatments/Therapy

 No cure
 Management of CF focuses on treating respiratory and digestive
problems to prevent infections or secondary complications
 Treatment combines medications, home treatments like respiratory therapy,
and nutritional therapy
 Depends on the symptoms the patient presents with
 Different treatments are used to relieve different symptoms
 Lung function tests can be used to monitor progression
 Therapy
 Respiratory therapy
 Digestive therapy
 Postural drainage

(Healthwise Staff, 2017)

Respiratory Therapy

 Any treatment that slows down lung damage

 Improves breathing
 Used to rid of mucus and bacteria in lungs
 Mechanical equipment used:
 High frequency chest compression vest
 Positive expiratory pressure therapy (PEP)
 Techniques used
 Airway clearance techniques
 Postural drainage with chest percussions
Picture: (Cystic Fibrosis Foundation, 2018)
 Deep breathing exercises to build up strength of respiratory muscles
 Coughing
 Aerobic exercise
 Helps to loosen mucus, encourages coughing, improves O2 flow

(Healthwise Staff, 2017)

Digestive Therapy

 Replaces certain digestive enzymes

 Creon or Ultrase
 Helps to ensure nutrients and minerals are digested properly
 Individuals usually change their diet:
 Taking in high caloric foods, high fat foods, nutritional drinks, feeding tube

(Healthwise Staff, 2017)

Interdisciplinary Team

 GP
 Pediatrician
 Respirologist
 Internist
 Gastroenterologist
 RT
 PT
 Social Worker
 Pharmacist
 Psychologist

(Healthwise Staff, 2017)

Exacerbation Warning Signs
 CF individuals loose/are unable to absorb salt effectively
 Careful to always supplement salt intake during:
 Exercise
 On hot days
 Warning Signs
 ↑ cough
 ↑ sputum production
 Fever
 Weight loss
 ↑ RR &/or WOB
 New wheezing/crackles
 ↓ exercise tolerance
 ↓ FEV1 of >10% over 3 months
 ↓ 02 saturation of >10% over 3 months
 New findings on chest x-ray

(Maybury, March 9, 2018)

Patient Overview
 Patient Overview: Lily
 Patient name : Lily Davis
 Age: 7 years old
 Weight: 50lbs
 Gender: Female
 Diagnosed after birth
 CF classified at mild
 Current medical status mild CF to recent exacerbation of CF with increase in mucus and
secretions in airways
 Parents are carriers of CF gene
 Mother is a nurse, Father works for the military
 Only child
 Lives in bungalow with swimming pool and swing set
 Lives 10 minutes from school
 Recently more interested in diagnosis and wants to understand therapy
 Loves to swim, play soccer, use swing set, likes to do yoga with mom
Medical History

 Hospitalized at 4 yo
 feeding tube surgically placed
 Regular visits to SickKids for lung function tests, blood tests
 Previously had lung scan at 2 yo
 Recently diagnosed with mild form of ADHD
Current Medical Management

 Uses PAP medical treatment for lungs 2 times a day

 Morning at 6am and night at 7pm
 Takes Ultrase for digestive therapy
 Takes with every meal (Healthwise Staff, 2017)
 Parents looking to start airway clearance techniques daily
Current Symptoms

 SOB with long periods of physical activity or excitement

 Persistent cough
 Recently increased mucus and secretions, rough cough
 Below average weight for children her age
 Salty sweat
Safety Considerations for Patient

 Monitor for excessive SOB or mucus production throughout session

 Monitor vitals: HR, RR
 Watch for fatigue or exhaustion when first starting rehab program
 Have oxygen near by for emergency
 Infection control critical
 “Cepacia +”
 Hand-washing and appropriate sanitization of equipment is critical to
avoid passing between patients
 Avoid treatment immediately following meals
 Aerosol therapy medications prior to manual chest physiotherapy
 Energy conservation
(Maybury, March 9, 2018)
Rehab Plan

 Therapy visits: Lily to attend 4 sessions at SickKids Hospital with parents to

educate on airway clearance techniques and introducing FITT plan
 Therapy sessions will be planned for 11am which is after lily’s respiratory
therapy in the morning
 Airway clearance techniques
 Taught to parents to do daily
 Postural drainage of upper and lower lobe of R and L lung, with 5 minutes of
percussions (each position), 2 minutes of vibrations (each position), followed
by 2 coughs after each position
 total of 20-25 minutes of therapy (Maybury, February, 12, 2018)
 FITT principle
 Will include warm up, aerobic exercise, cool down, stretching, strengthening
(Maybury, January 29, 2018)
FITT M-S Mon. Tues. Wed. Thurs. Friday Saturday Sunday M-S
Rehab Warm-up Cool-
Plan Down
Frequency 2X/d 2X/d 2X/d 2X/d 2X/d 2X/d 2X/d 2X/d 2X/d
7 days/w 7 days/w 7 days/w 7 days/w 7 days/w 7 days/w 7 days/w 7 days/w 7 days/w

Intensity 106 (220-7)-80 (220-7)-80 X (220-7)-80 X .40 (220-7)-80 X (220-7)-80 X (220-7)-80 X (220-7)-80 X 106 bpm
bpm X .40 + 80= .40 + 80= 133 + 80= 133bpm .40 + 80= .40 + 80= .40 + 80= .40 + 80=
133 bpm 133 133bpm 133bpm 133bpm
bpm bpm

Time 10 min. 60 min/d 60 min/d 60 min/d 60 min/d 60 min/d 60 min/d 60 min/d 10 min.
Split up Split up into Split up into 20 & Split up into Split up into Split up into Split up into
into 20 & 20 & 40 min 40 min 20 & 40 min 20 & 40 min 20 & 40 min 20 & 40 min
40 min

Type Jumping Aerobic Aerobic Aerobic Aerobic Aerobic Strengthenin Strengthenin Hop Scotch
Jacks Walking to Walking to Walking to Walking to Walking to g: g:
school= school= school= school= school= Throwing 1lbs Throwing 1lbs
20min, 20min, 20min, 20min, 20min, ball back & ball back &
After After school After school After school After school forth=20min, forth=20min,
school sports sports group= sports sports group= Swimming=40 Swimming=40
sports group= 40min group= 40min min min
group= 40min (Gymnastics) 40min (swimming)
40min (swimming) (soccer)
(soccer)

Stretching/ Kids Yoga-15 Kids Yoga-

Flexibility minutes 15 Minutes
 References
Sources:
Al-Newrawi, N., Alshraiedeh, N., Zayed, A., and Altaani, B. (2018).
Low molecular weight chitosan-coated PLGA nanoparticles for
pulmonary delivery of tobramycin for cystic fibrosis. Journal of
Pharmaceuticals. 11(1). Retrieved from http://www.mdpi.com/1424-
8247/11/1/28/htm
Cystic Fibrosis Canada. (2018). What is cystic fibrosis. Retrieved from
http://www.cysticfibrosis.ca/about-cf/what-is-cystic-fibrosis
Healthwise Staff. (2017). Cystic Fibrosis. Retrieved from
https://myhealth.alberta.ca/Health/pages/conditions.aspx?hwid=h
w188548
Maybury, L. (2018, January 29). PTOT 4304 Course Lecture.
Maybury, L. (2018, February 12). PTOT 4304 Course Lecture.
Maybury, L. (2018, March 9). PCAR 4302 Course Lecture.
Sharma, G. (2018). Cystic fibrosis. Retrieved from
https://emedicine.medscape.com/article/1001602-overview#a3
Pictures:
British Columbia Respiratory Therapy. (2015). Cystic fibrosis hope as new gene therapy
improves condition. Retrieved from http://bcrt.ca/cystic-fibrosis-hope-as-new-gene-
therapy-improves-condition/
Cystic Fibrosis Canada. (2018). What is cystic fibrosis. Retrieved from
http://www.cysticfibrosis.ca/about-cf/what-is-cystic-fibrosis
Cystic Fibrosis Foundation. (2018). Airway Clearance. Retrieved from
https://www.cff.org/Life-With-CF/Treatments-and-Therapies/Airway-Clearance/
Healthwise Staff. (2017). Cystic Fibrosis. Retrieved from
https://myhealth.alberta.ca/Health/pages/conditions.aspx?hwid=hw188548
Fandom. (2016). Cystic fibrosis lungs. Retrieved from
http://fragile.wikia.com/wiki/File:Cystic-Fibrosis-Lungs-Pictures-3.jpg
National Lung, Heart and Blood Institute. (2018). Cross-sections of a healthy airway
(top) and an airway affected by cystic fibrosis (bottom). Retrieved from
https://www.hopkinsmedicine.org/research/advancements-in-
research/fundamentals/in-depth/piecing-together-cystic-fibrosis
Mayo Clinic. (2016). Cystic fibrosis. Retrieved from
https://www.mayoclinic.org/diseases-conditions/cystic-fibrosis/symptoms-causes/syc-
20353700