Cystic Fibrosis Care as a model for chronic disease care in the clinic. A presentation made on April 29, 2017 to the Wisconsin Chapter of the American Association of Medical Assistants at Lacrosse, WI.

1. Cystic Fibrosis as a Chronic
Medical Condition
Vijay Aswani, MD, PhD, FAAP, FACP
Department of Internal Medicine & Pediatrics
Marshfield Clinic
April 2017

2. Details
• Presentation: 45-50 minutes
• Questions: 10-15 minutes
• Date: April 29, 2017
• Location: La Crosse Center Ballroom, La Crosse
Center 300 Harborview Plaza, La Crosse, WI
54601
• Time:
• Audience: American Association of Medical
Assistants Wisconsin Chapter

3. Learning Objectives
1. Define and describe a chronic medical condition
2. Define and describe Cystic Fibrosis as a chronic
medical condition
3. Identify and recognize key clinical features of
Cystic Fibrosis
4. Describe team-based medical care as a model
for managing chronic medical conditions
5. Describe the role of the medical assistant as a
healthcare provider in delivering care in chronic
medical conditions using Cystic Fibrosis as an
example

4. Learning Objectives
1. Define and describe a chronic medical condition
2. Define and describe Cystic Fibrosis as a chronic
medical condition
3. Identify and recognize key clinical features of
Cystic Fibrosis
4. Describe team-based medical care as a model
for managing chronic medical conditions
5. Describe the role of the medical assistant as a
healthcare provider in delivering care in chronic
medical conditions using Cystic Fibrosis as an
example

5. What is a Chronic Medical Condition?
Case 1: You are in your 1st week as a CMA at Goodhealth
Clinic in Goodplace, WI. Your doctor asks you to select
patients for a special Chronic Disease Clinic that you both
will manage once a month. Which of the following
diseases would NOT fall in the category of chronic
disease?
A. Diabetes
B. Hypertension
C. Pneumonia
D. Cystic fibrosis
E. Asthma

6. What is a Chronic Medical Condition?
A health condition is a departure from a state of physical or
mental well-being. In the National Health Interview Survey,
each condition reported as a cause of an individual’s activity
limitation has been classified as chronic, not chronic, or
unknown if chronic, based on the nature and duration of the
condition. Conditions that are not cured once acquired (such
as heart disease, diabetes, and birth defects in the original
response categories, and amputee and old age in the ad hoc
categories) are considered chronic, whereas conditions
related to pregnancy are not considered chronic. Other
conditions must have been present for 3 months or longer to
be considered chronic. An exception is made for children aged
less than 1 year who have had a condition since birth: such
conditions are always considered chronic.
US Department of Health and Human Services. Health, United States, 2010

7. What is a Chronic Medical Condition?
Case 1: You are in your 1st week as a CMA at Goodhealth
Clinic in Goodplace, WI. Your doctor asks you to select
patients for a special Chronic Disease Clinic that you both
will manage once a month. Which of the following
diseases would NOT fall in the category of chronic
disease?
A. Diabetes
B. Hypertension
C. Pneumonia
D. Cystic fibrosis
E. Asthma

8. 65 Roses

9. 65 Roses
https://www.cff.org/About-Us/About-the-Cystic-Fibrosis-Foundation/The-65-Roses-Story/

10. Learning Objectives
1. Define and describe a chronic medical condition
2. Define and describe Cystic Fibrosis as a chronic
medical condition
3. Identify and recognize key clinical features of
Cystic Fibrosis
4. Describe team-based medical care as a model
for managing chronic medical conditions
5. Describe the role of the medical assistant as a
healthcare provider in delivering care in chronic
medical conditions using Cystic Fibrosis as an
example

11. Cystic Fibrosis as a Chronic Medical
Condition
Case 2: You are in your 1st week as a CMA at
Goodhealth Clinic in Goodplace, WI. Mrs. Kneedy
calls you in a panic. Her 5-year old son, Clue has just
started kindergarten. There is a boy in his class,
Cedric, with cystic fibrosis. She has some questions
for you and Clue’s doctor. Answer YES or NO:
A. “Can Clue catch cystic fibrosis from Cedric?”
B. “Should I keep Clue out of school till Cedric’s CF
gets better?”
C. “Is there a vaccine or cure for cystic fibrosis?”
D. “Will Cedric have a normal life span?”

12. Cystic Fibrosis as a Chronic Medical
Condition
• Cystic fibrosis is a progressive, genetic disease
that causes persistent lung infections and
limits the ability to breathe over time.
• It is a genetic disease (you are born with it)
• It is lifelong
• There is currently no cure
• Lifelong care and treatment
• It shortens life expectancy

13. History of CF?
“woe is the child who tastes salty from a
kiss on the brow, for he is cursed, and
soon must die”
-- European folklore
• In 1938, the American Pathologist Dr. Dorothy Andersen provided the first
description of the disorder in the medical literature, calling the disease
“cystic fibrosis of the pancreas” based on her autopsy findings of children
that died of malnutrition.
• During the heat wave of 1949, Paul di Sant’Agnese was the first to
recognize CF babies were at special risk for heat prostration. He thus
discovered that sweat was abnormal in CF.[1] This led to the development
of the sweat test, which is a major way cystic fibrosis can be diagnosed. He
also founded the CFF

14. 2015: Median age at death: 30.1 years; range with the oldest being 87 years old

15. What Causes Cystic Fibrosis?
• Cystic fibrosis is caused by mutations in the gene that
produces the cystic fibrosis transmembrane conductance
regulator (CFTR) protein. This protein is responsible for
regulating the flow of salt and fluids in and out of the cells
in different parts of the body.
• Mutations in the CF gene cause the CFTR protein to
malfunction, leading to a buildup of thick mucus, which in
turn leads to persistent lung infections, destruction of the
pancreas in most individuals with CF and complications in
other organs.
• The CF gene was discovered in 1989 on chromosome pair 7

19. What’s Up with Sal? Medkidz Explain Cystic Fibrosis, Medikitz Limited 2015

20. Cystic Fibrosis Mutations
• more than 1,700 mutations in the CF gene.
• While some CF mutations are common, others
are rare and found in only a few people.
• Carrier rate in Caucasians is 1:30
• Incidence of CF in live births is about 1:3200
• 90% of carriers are Caucasians or European
Jews
• Most common mutation is delta F508 (86.4%)

22. Diagnosing CF
• Newborn Screening
– 59.6% detected by newborn screening
– 75% diagnosed by age 2 (failure to thrive, rectal
prolapse, acute or persistent respiratory
abnormalities
– In all 50 states, since 2009
• Confirmed by a sweat chloride test

23. Sweat Chloride Test

24. How is it done?
• The sweat glands are made to produce sweat with a
mild chemical called pilocarpine and a little electricity
(pilocarpine iontophoresis).
• A gauze pad or piece of filter paper is put on the skin to
collect the sweat.
• Then, the area is wrapped in plastic for 30 minutes.
• A small plastic strap that looks like a wristwatch also
may be used to collect the sweat. It collects the sweat
into a small tube. If the person has CF, the sweat will be
high in salt (sodium and chloride).

25. Learning Objectives
1. Define and describe a chronic medical condition
2. Define and describe Cystic Fibrosis as a chronic
medical condition
3. Identify and recognize key clinical features of
Cystic Fibrosis
4. Describe team-based medical care as a model
for managing chronic medical conditions
5. Describe the role of the medical assistant as a
healthcare provider in delivering care in chronic
medical conditions using Cystic Fibrosis as an
example

26. Key Clinical Features of CF
Case 3: You are in your 1st week as a CMA at Goodhealth Clinic in Goodplace,
WI. Your doctor and you are really busy. As you room patients and get a
history, which of the following should make you think of CF as a possible
diagnosis?
A. Persistent coughing, at times with phlegm
B. Frequent lung infections, such as pneumonia or bronchitis
C. Wheezing or shortness of breath
D. Poor growth or poor weight gain in spite of a good appetite
E. Frequent greasy, bulky stools or difficulty in bowel movements
F. Small, fleshy growths in the nose, called nasal polyps
G. Chronic sinus infections
H. Clubbing or enlargement of the fingertips and toes
I. Rectal prolapse (when the rectum sticks out through the anus)
J. Male infertility
K. All of these

28. Treating CF
• Airway Clearance Therapy (ACT)
• Inhaled Medicines
– Hypertonic Saline
– Albuterol
– Dornase or Pulmozyme
– Tobramycin or inhaled Aztreonam
• Pancreatic enzyme replacement therapy
(PERT)
• CFTR Modulators

29. What?
• Airway clearance to help loosen and get rid of the thick mucus that
can build up in the lungs. Some airway clearance techniques require
help from family members, friends or respiratory therapists. Many
people with CF use an inflatable vest that vibrates the chest at a
high frequency to help loosen and thin mucus.
• Inhaled medicines to open the airways or thin the mucus. These are
liquid medicines that are made into a mist or aerosol and then
inhaled through a nebulizer. These medicines include antibiotics to
fight lung infections and therapies to help keep the airways clear.
• Pancreatic enzyme supplement capsules to improve the absorption
of vital nutrients. These supplements are taken with every meal and
most snacks. People with CF also usually take multivitamins.

34. Learning Objectives
1. Define and describe a chronic medical condition
2. Define and describe Cystic Fibrosis as a chronic
medical condition
3. Identify and recognize key clinical features of
Cystic Fibrosis
4. Describe team-based medical care as a model
for managing chronic medical conditions
5. Describe the role of the medical assistant as a
healthcare provider in delivering care in chronic
medical conditions using Cystic Fibrosis as an
example

35. Team-based Medical Care
Case 4: You are in your 3rd week as a CMA at Goodhealth Clinic in
Goodplace, WI. Cecil Fredrick, a 25 year old CF patient calls in on
a Monday morning to say he is not feeling well. He has not been
doing his treatments. He wants to be admitted for a CF
exacerbation. Your schedule is full! What do you do?
A. Send him to Urgent Care or the ER
B. Give him the first available appointment – Wednesday
next week
C. Give him an antibiotic prescription over the phone
D. Make it happen: get him in and admitted

39. Learning Objectives
1. Define and describe a chronic medical condition
2. Define and describe Cystic Fibrosis as a chronic
medical condition
3. Identify and recognize key clinical features of
Cystic Fibrosis
4. Describe team-based medical care as a model
for managing chronic medical conditions
5. Describe the role of the medical assistant as a
healthcare provider in delivering care in chronic
medical conditions using Cystic Fibrosis as an
example

40. Role of the CMA
Case 5: You are in your 2nd year as a CMA at Goodhealth Clinic in
Goodplace, WI. You and your doctor are a team. You take pride
in providing the best care to the patients in your practice. Your
doctor and you sit down to discuss how you both can improve
the CF clinic. You suggest:
A. Have team huddles at a regular intervals
B. Assess where your patients are at with respect to the
goals of care and set goals to improve
C. Cultivate relationships with the nurse, pharmacist,
manager and other CMAs in your work area to facilitate
care
D. Be part of setting up PDSA cycles to improve care
E. All of the above 

41. Summary
• Cystic fibrosis (CF) is the most common lethal genetic disease in the
United States.
– 1 in 31 Americans carry the defective gene for CF.
– There are currently about 30,000 people with CF in the United States
and more than half are over the age of 18.
• Caring for individuals with Cystic Fibrosis involves managing
pulmonary exacerbations, nutrition, infections and multiple
complex psychosocial issues.
• We will discuss
– CF as a chronic medical condition – a disease that patients and their
families must live with from birth to death.
– how the diagnosis is made, signs and symptoms of the disease and
challenges in management.
– team-based medical care in chronic medical diseases and the key role
a medical assistant can play.