Cystic Fibrosis

Question 1

What is Cystic Fibrosis?

Answer 1

It’s 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.

Question 2

What are exocrine glands?

Answer 2

What are exocrine glands? These 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 3

CF,describe charastics of the mucous

Answer 3

THICK and STICKY (rather than thin and slippery)

Question 4

CF affects which structures:

Answer 4

Lungs (upper and lower),Gastrointestinal (blockage of passage ways within the body),Pancreas ,Liver,Distal Intestinal Obstruction Syndrome (DIOS),Infertility,Integumentary

Question 5

EFFECTS Lungs (upper and lower):

Answer 5

upper sinuses and formation of polyps (leading to snoring, nasal stuffiness)
blockage of the airways: overtime 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)

Question 6

LUNGS are very succeptable to..

Answer 6

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 7

Gastrointestinal effects:

Answer 7

blockage of passage ways within the body

Question 8

Pancreas (this structure makes pancreatic enzymes):what are they called?

Answer 8

PAL: Protease, Amylase, Lipase

Question 9

PAL: Protease, Amylase, Lipase

Answer 9

These substances are secreted via the pancreatic duct into the duodenum and this helps digest proteins, fats, and vitamins.

Question 10

why doesnt the Enzymes provide proper usage?

Answer 10

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.

Question 11

when a patient’s body is not receiving pancreatic enzymes they will experience:

Answer 11

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 12

the LIVER MUCOUS blocks what?

Answer 12

biliary ducts which causes the bile to become thick and gallstones can develop along with gallbladder inflammation

Question 13

Distal Intestinal Obstruction Syndrome (DIOS):

Answer 13

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 14

Infertility in males and females:

Answer 14

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 15

EFFECTS of the Integumentary

Answer 15

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 16

which race is CF is more common?

Answer 16

Caucasians and males, but can affect any race or gender.

Question 17

NIH (National Institute of Health) statistics:

Answer 17

life expectancy in 1960 was age 10. Today the median life expectancy is age 37 (“NIH Fact Sheets – Cystic Fibrosis”, n.d.).

Question 18

when is This is most commonly detected

Answer 18

during the 1st year of life…child will have GI and respiratory issues (ex: infant may have a meconium ileus).

Question 19

Severity depends…

Answer 19

(not all patients are 100% alike in their severity)

Question 20

how Is it an autosomal recessive disorder?

Answer 20

the child had to receive one mutated gene from both parents. In most cases, the parents are carriers of the disease but don’t have symptoms of CF.

Question 21

a picture of Inheritance of Cystic Fibrosis

Answer 21

https://www.registerednursern.com/wp-content/uploads/2018/07/cystic-fibrosis-nclex-nursing.png

Question 22

Diagnostics

Answer 22

Sweat Test: painless test that measures the amount of salt in the sweat

Question 23

whats the process of the sweat test.

Answer 23

Pilocarpine and electric current is applied to the skin to help it sweat (usually on the arm). A gauze is used to collect the sweat which is sent to a lab to measure the salt in the sweat

Question 24

Results of sweat test

Answer 24

Results:
39 mmol/L or less negative for cystic fibrosis
40 to 59 mmol/L needs further testing, not conclusive
60 mmol/L or more POSITIVE for cystic fibrosis

Question 25

Nursing Interventions and Treatments for Cystic Fibrosis

Answer 25

the nurse focuses on: Mucous, Nutrition, Treatment for infection (most patients will die due to a lung infection or issue of some type), preventing blockages in GI system

Question 26

CF has wide variety of medications, which are?

Answer 26

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

Question 27

how to treat the Mucous:

Answer 27

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

Question 28

mucous tx: what is CPT

Answer 28

Chest physiotherapy (Chest PT): helps drain the airways of that thick sticky mucous
It is done with postural drainage along with percussion and vibration to facilitate the draining of mucous from the lung lobes followed by huff coughing (THIS IS A HUGE PART OF treatment for CF patients!!!!)

Question 29

how to Chest percussion:

Answer 29

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 30

how long do you do chest percussion?

Answer 30

**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.

Question 31

When should you perform chest PT on a patient?

Answer 31

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 32

what is Airway Clearance devices:

Answer 32

PEP (positive expiratory pressure):

Question 33

what does PEP do?

Answer 33

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 34

Preventing infection:

Answer 34

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 35

Exercise tx:

Answer 35

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 36

GI tx:

Answer 36

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

Question 37

Pancreatic enzymes tx:

Answer 37

Swallow them! 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.

Question 38

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

Answer 38

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

Question 39

When would you administer these enzymes?

Answer 39

Administer before all meals and snacks. Example of these enzymes are: Pancrelipase, Pancreatin

Question 40

Diet is important because

Answer 40

Some patients have feeding tubes because their caloric intake is very high, especially during illness or during the late stages of the disease. High protein and high calorie and fat diet with vitamin supplements like the fat-soluble: DEKA

Question 41

Obstruction and Constipation Prevention

Answer 41

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 42

Cystic Fibrosis Diabetes Mellitus:

Answer 42

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