Cystic Fibrosis

Question 1

a genetic disorder that causes the EXOCRINE glands to work incorrectly. This leads to some major complications that can affect the respiratory (both upper/lower), digestive (pancreas, liver, intestines), integumentary, and reproductive systems.

Answer 1

Cystic Fibrosis - thick, sticky mucus starts blocking the airways. It will block oxygenation and puts the patient more at risk for infections which in the long run these chronic infections damage the lungs and affect the sinuses

Infertility issues

The mucus blocks the pancreatic duct that allows the pancreatic enzymes to flow down over the duodenum. The pancreas can be damaged enough over time that diabetes can develop

Question 2

Quick facts about Cystic Fibrosis

Answer 2

most common in Caucasians and males
today median life expectancy is age 37
usually detected during the 1st year of life
severity depends
Its an autosomal recessive genetic disorder

Question 3

How is Cystic Fibrosis diagnosed ?

Answer 3

The Sweat Test - it is a painless test that measures the amount of salt in the sweat

39 mmol/L or less - NEGATIVE
40-59 mmol/L (needs further testing)
60 mmol/L or more (positive)

Question 4

In cystic fibrosis, there is something wrong with the ______________ glands

Answer 4

exocrine

Question 5

What are exocrine glands? Do they secrete directly into the blood ?

Answer 5

hese are glands that produce and transfer it’s secretions (ex: mucous, tears, sweat, digestive enzymes) via DUCTS to its intended area of the body rather than directly into the bloodstream like endocrine glands.

Question 6

In cystic fibrosis, a gene that makes up the exocrine glands is _________ and this prevents the exocrine glands from working properly.

Answer 6

mutated; The gene that is mutated is called the CFTR gene

Question 7

What is the significance of the CTFR gene?

Answer 7

The CFTR gene, which stands for cystic fibrosis transmembrane regulator, is a protein that controls the channels of sodium and chloride. In other words, it controls the sodium and water transport in and out of the cell.
Therefore, these channels that are within the membrane of exocrine cells that makes our sweat, mucous, tears, and digestive enzymes don’t work properly.
So, the substances produced by the exocrine glands don’t look and work like they are supposed to.

For example: How is mucous supposed to be? Thin and slippery which helps lubricate the structures. However, this is the problem in patients with cystic fibrosis.

In patients with CF the mucous is THICK and STICKY (rather than thin and slippery)

Question 8

What is the deal with mucous in patients with Cystic Fibrosis ?

Answer 8

It is THICK and STICKY, instead of being thin and slippery

Question 9

How does Cystic Fibrosis affect the lungs ?

Are they susceptible to lung infections?

Answer 9

There is a blockage of airways due to the thick and sticky mucous. Overtime this leads to obstructive pulmonary diseases like emphysema …..air trapping can occur which causes hyperinflation

Or bleds can develop and rupture leading to a pneumothorax

Pulmonary hypertension which can lead to heart failure in the long term

Clubbing of the nails due to the lack of oxygen in the blood
Hemoptysis: coughing up blood either due to infection or severe damage to lung tissue (mainly in older patients rather than children)

upper sinuses and formation of polyps (leading to snoring, nasal stuffiness) - a lot of patients end up having sinus surgery and develop nasal polyps that can cause stuffiness and snoring

Very susceptible to lung infections that can create permanent damage to the lungs
Thick, sticky mucous creates perfect conditions for bacteria to thrive and survive! These patients are at risk for pseudomonas aeruginosa, staphylococcus aureus etc.
There is a risk of the development of antibiotic resistance or allergies to antibiotics, which can make treating the infection very hard.

Question 10

How does Cystic Fibrosis affect the gastrointestinal system , specifically the pancreas?

Answer 10

Through the blockage of passage ways within the body. Pancreatic enzymes are not able to get out of the pancreas and help with digestion. Instead they cause fibrosis of the pancreas.

Pancreas (this structure makes pancreatic enzymes):
PAL: protease, amylase, lipase: These substances are secreted via the pancreatic duct into the duodenum and this helps digest proteins, fats, and vitamins.

However, these enzymes are not accessible for proper usage because of the mucous blocking the pancreatic duct. Hence, they will stay in the pancreas and this inflames the structure and can cause fibrosis of the pancreas. In addition, the patient can develop cystic fibrosis related diabetes. Therefore, when a patient is not receiving pancreatic enzymes they will experience:
weight loss, malabsorption, nutrition major issue, delayed puberty, GREASY, ODOROUS STOOL, BLOATNG, ABODOMINAL PAIN

Many patients will need a feeding tube to help with nutrition, PANCREATIC ENZYMES SUPPLEMENTS, fat-soluble vitamins like D E A K

Question 11

How does cystic fibrosis affect the Liver?

Answer 11

mucous blocks biliary ducts which causes the bile to become thick and gallstones can develop along with gallbladder inflammation.
Distal Intestinal Obstruction Syndrome (DIOS): blockage in the intestine….intestines can also make thick mucus like the lungs and pancreas and when thick mucus and stool get together it can lead to a blockage in the gut.
Meconium ileus (infants): sticky meconium becomes stuck in the ileum of the intestine

Question 12

How does Cystic Fibrosis affect fertility ?

Answer 12

in males and females: most people who have cystic fibrosis are unable to have children because of the thickness of mucous. For example, women have troubling conceiving because the cervical mucous is not friendly towards sperm, and males tend to be sterile because there are issues with the vas deferens duct that helps sperm go for the testicle to the urethra (thick mucus blocking it or the duct is not formed correctly).

Question 13

How does Cystic Fibrosis affect the integumentary system ?

Answer 13

sweat glands produce too much salt. Therefore, the sweat/tears are very salty. The patient is at risk for electrolyte complications and dehydration. However, the salty sweat is helpful with diagnosing CF (sweat chloride test).

Question 14

Nursing Interventions for Cystic Fibrosis- what should we focus on ?

Answer 14

MUCOUS

INFECTION
(most patients will die due to a lung infection or issue of some type),

NUTRITION

preventing blockages in GI system

Question 15

How can we help patient with Cystic Fibrosis clear airways?

Answer 15

MUCOUS: Huge part of the everyday routine treatment and this includes: Chest PT, postural drainage, using PEP devices and nebulizers along with huff coughing

Question 16

When is it best to perform chest physiotherapy with Cystic Fibrosis patients ?

Answer 16

Perform when the stomach is empty to prevent reflux of food and vomiting……1-2 hours after meals (hence in between meals). NOT after (reason: regurgitation) or before (reason: risk of decreasing the appetite because coughing up thick sticky mucous can taste bad which can decrease a person’s appetite).

Question 17

How often does a patient with chest PT perform Chest physiotherapy ?

What else should I remember about chest physiotherapy?

Answer 17

**Patient usually performs this 2-4 times per day or more depending on if they are fighting a lung infection. Sessions vary: 20 minutes to an hour.

Chest PT: percussion (cupping hands and percussing of the over the lung fields and take special care when percussing over spine, stomach, breast bone, organs to avoid injury), some patients use vibration with a special vest and then huff coughing is performed

Question 18

How do airway clearance devices help with clearing the airway?

Answer 18

Airway Clearance devices: PEP (positive expiratory pressure): Helps with clearing the airway

PEP devices: assists with moving mucous from the lungs…..person breathes in and out of device (resistance when breathing out and this helps even more to move mucous)….creates a vibration (performs several times) and then huff cough to expel mucous. Device names: Flutter valve or Acapella

Question 19

How can we help a patient with Cystic Fibrosis prevent infection ?

Answer 19

wearing a mask due flu times or when sick, hand hygiene, avoid sick people, and stay up-to-date with vaccinations, lung transplant possible if lung problems severe

Question 20

Exercise & Cystic Fibrosis

Answer 20

regular aerobic exercise to help keep lungs healthy and clear secretions. Educate to monitor sweating because of the risk of losing too much salt (may need salt supplements and increase caloric needs when exercising)

Question 21

Nutrition interventions for patient with cystic fibrosis ?

Answer 21

pancreatic enzymes, fat-soluble vitamins (ADEK) , high calories, high protein and high fat diet

Some patients have feeding tubes because their caloric intake is very high, especially during illness or during the late stages of the disease.

Question 22

Pancreatic Enzymes and Cystic Fibrosis -

what should we educate patient about taking their pancreatic enzymes ? When should patient take them?

How do you know the patient is receiving an adequate amount of enzymes?

Answer 22

Swallow if possible. Don’t chew or crush them!

If person can’t swallow the capsule, you can open it and put it in an ACIDIC FOOD like apple sauce. Do not mix in an alkaline-based food like yogurt, pudding or ice cream because this will inactive the enzyme.

TAKE BEFORE ALL MEALS AND SNACKS

The patient’s stool is NOT greasy or odorous and they are free from abdominal bloating or pain.

Question 23

Cystic Fibrosis - Obstruction & Constipation Prevention

Answer 23

Drink a lot of water to keep mucous in gut thin, use Miralax Polyethylene Glycol (common) daily as prescribed by MD to prevent constipation
Monitor for bowel obstruction: signs: no bowel movement, pain, can’t pass gas etc.

Question 24

Cystic Fibrosis & Diabetes Mellitus

Answer 24

pancreas is damaged….monitor blood sugar (can drop or be high)…may need insulin

Question 25

Patients who have CF are on a wide variety of medications like the following:

Answer 25

stool softeners
pancreatic enzymes
nasal sprays
vitamins
antibiotics
anti-inflammatories (inhaled or oral)
mucolytics (oral or nebulizer)
bronchodilators (oral or inhaled)

Question 26

You’re assisting a patient with performing chest physiotherapy. It is very important you have the patient ___________ during the therapy sessions

Answer 26

Huff Cough

This is a special type of coughing that will help the patient cough up the mucous during the CPT. It is very important the patient performs this during their CPT sessions.

Question 27

The physician gives an order for a patient with cystic fibrosis to use a positive expiratory pressure (PEP) device to help with airway clearance. As the nurse you will order which device from supply:

Answer 27

Flutter valve

This device assists with moving mucous from the lungs to the airway so it can be expelled. Another device that does this is called an Acapella.