Cystic Fibrosis By Rnpedia.Com

1. Body systems affected by cystic fibrosis include:

Pancreas

Sweat glands

Lungs

All of these

Cystic fibrosis primarily affects the glands that secrete. An abnormal protein is produced making secretions sticky. These thickened secretions are found in the body's sweat, the lungs' mucus, the pancreas' output, liver output, and reproductive organs.

Explanation

Cystic fibrosis primarily affects the glands that secrete. An abnormal protein is produced making secretions sticky. These thickened secretions are found in the body's sweat, the lungs' mucus, the pancreas' output, liver output, and reproductive organs.

2. Cystic fibrosis is treated by:

Airway clearance techniques that aim to move the thick mucus from the lungs

Taking special vitamins

Taking pancreatic enzymes to help digest food

All of these

All of these are treatments for cystic fibrosis. Airway clearance techniques help people with cystic fibrosis move thickened mucus from the lungs. Pancreatic enzymes are taken to help digest the food. Special vitamins are needed to help replace vitamins A, D, and K which may not be absorbed well by the CF patient. There are more treatments, of course, but these are the main ones to help keep the person with cystic fibrosis healthy.

Explanation

All of these are treatments for cystic fibrosis. Airway clearance techniques help people with cystic fibrosis move thickened mucus from the lungs. Pancreatic enzymes are taken to help digest the food. Special vitamins are needed to help replace vitamins A, D, and K which may not be absorbed well by the CF patient. There are more treatments, of course, but these are the main ones to help keep the person with cystic fibrosis healthy.

3. Is there a cure for Cystic Fibrosis?

True

False

There is currently no known cure for Cystic Fibrosis. It is a genetic disorder that affects the lungs and other organs, causing difficulty in breathing and other symptoms. While there are treatments available to manage the symptoms and improve quality of life, there is no definitive cure for the condition.

Explanation

There is currently no known cure for Cystic Fibrosis. It is a genetic disorder that affects the lungs and other organs, causing difficulty in breathing and other symptoms. While there are treatments available to manage the symptoms and improve quality of life, there is no definitive cure for the condition.

4. Cystic fibrosis is diagnosed by:

Echocardiogram

Complete blood panel

Chest X-ray

Sweat test

A small electrode is placed on the skin, which stimulates the sweat glands. Sweat is collected for a period of time and then analyzed. People with cystic fibrosis have an increased amount of chloride in their sweat, due to the abnormal protein manufactured by the cystic fibrosis gene.

Explanation

A small electrode is placed on the skin, which stimulates the sweat glands. Sweat is collected for a period of time and then analyzed. People with cystic fibrosis have an increased amount of chloride in their sweat, due to the abnormal protein manufactured by the cystic fibrosis gene.

5. The nurse in charge is reviewing a patient's prenatal history. Which finding indicates a genetic risk factor?

The patient is 25 years old

The patient has a child with cystic fibrosis

The patient was exposed to rubella at 36 weeks’ gestation

The patient has a history of preterm labor at 32 weeks’ gestation

Cystic fibrosis is a recessive trait; each offspring has a one in four chance of having the trait or the disorder. Maternal age is not a risk factor until age 35, when the incidence of chromosomal defects increases. Maternal exposure to rubella during the first trimester may cause congenital defects. Although a history or preterm labor may place the patient at risk for preterm labor, it does not correlate with genetic defects.

Explanation

Cystic fibrosis is a recessive trait; each offspring has a one in four chance of having the trait or the disorder. Maternal age is not a risk factor until age 35, when the incidence of chromosomal defects increases. Maternal exposure to rubella during the first trimester may cause congenital defects. Although a history or preterm labor may place the patient at risk for preterm labor, it does not correlate with genetic defects.

6. A client with cystic fibrosis is taking pancreatic enzymes. The nurse should administer this medication:

Once per day in the morning

Three times per day with meals

Once per day at bedtime

Four times per day

Pancreatic enzymes should be given with meals for optimal effects. These enzymes assist the body in digesting needed nutrients. Answers A, C, and D are incorrect methods of administering pancreatic enzymes.

Explanation

Pancreatic enzymes should be given with meals for optimal effects. These enzymes assist the body in digesting needed nutrients. Answers A, C, and D are incorrect methods of administering pancreatic enzymes.

7. When administering pancrelipase (Pancreases capsules) to child with cystic fibrosis, nurse Faith knows they should be given:

With meals and snacks

Every three hours while awake

On awakening, following meals, and at bedtime

After each bowel movement and after postural drainage

Pancreases capsules must be taken with food and snacks because it acts on the nutrients and readies them for absorption.

Explanation

Pancreases capsules must be taken with food and snacks because it acts on the nutrients and readies them for absorption.

8. Which of the following clients is at high risk for developmental problem?

A toddler with acute Glomerulonephritis on antihypertensive and antibiotics

A 5-year-old with asthma on cromolyn sodium

A preschooler with tonsillitis

A 2 1/2 –year old boy with cystic fibrosis

It is the developmental task of an 18-month-old toddler to explore and learn about the environment. The respiratory complications associated with cystic fibrosis (which are present in almost all children with cystic fibrosis) could prevent this development task from occurring.

Explanation

It is the developmental task of an 18-month-old toddler to explore and learn about the environment. The respiratory complications associated with cystic fibrosis (which are present in almost all children with cystic fibrosis) could prevent this development task from occurring.

9. Cystic fibrosis is caused by:

A defective gene that causes abnormalities in the brain

It is not known what the cause is

Someone who eats too much salt

A defective gene that leads to the making of an abnormal protein

Usually, the gene involved makes a protein called CFTR (Cystic Fibrosis Conductance Transmembrane Regulator). This protein regulates the movement of sodium and chloride in the body's cells. Disruption of this protein leads to thicker secretions due to the imbalance of salt and water in the cells. Too little salt and water are outside the cells, making everything thicker

Explanation

Usually, the gene involved makes a protein called CFTR (Cystic Fibrosis Conductance Transmembrane Regulator). This protein regulates the movement of sodium and chloride in the body's cells. Disruption of this protein leads to thicker secretions due to the imbalance of salt and water in the cells. Too little salt and water are outside the cells, making everything thicker

10. At what age is this disease diagnosed?

Early years [0-5]

Childhood years [5-12]

Teenage years [12-17]

Adult years [18-50]

50+

This disease is diagnosed in the early years, specifically between the ages of 0 and 5.

Explanation

This disease is diagnosed in the early years, specifically between the ages of 0 and 5.

11. The foul-smelling, frothy characteristic of the stool in cystic fibrosis results from the presence of large amounts of which of the following:

Sodium and chloride

Undigested fat

Semi-digested carbohydrates

Lipase, trypsin and amylase

The client with cystic fibrosis absorbs fat poorly because of the think secretions blocking the pancreatic duct. The lack of natural pancreatic enzyme leads to poor absorption of predominantly fats in the duodenum. Foul-smelling, frothy stool is termed steatorrhea.

Explanation

The client with cystic fibrosis absorbs fat poorly because of the think secretions blocking the pancreatic duct. The lack of natural pancreatic enzyme leads to poor absorption of predominantly fats in the duodenum. Foul-smelling, frothy stool is termed steatorrhea.

12. A 6-year-old with cystic fibrosis has an order for Creon. The nurse knows that the medication will be given:

At bedtime

With meals and snacks

Twice daily

Daily in the morning

Pancreatic enzyme replacement is given with each meal and each snack.

Explanation

Pancreatic enzyme replacement is given with each meal and each snack.

13. One of the most important pulmonary treatments in cystic fibrosis is:

Inhaled beta agonists.

Inhaled corticosteroids.

Chest physiotherapy.

Oral enzymes.

The major pulmonary problem with CF is thick tenacious secretions. CPT moves the secretions from the small airways to the large where they can be coughed out. Options a and b are used but are secondary to option c; the oral enzymes that CF patients take are for digestion, not pulmonary reasons.

Explanation

The major pulmonary problem with CF is thick tenacious secretions. CPT moves the secretions from the small airways to the large where they can be coughed out. Options a and b are used but are secondary to option c; the oral enzymes that CF patients take are for digestion, not pulmonary reasons.

14. A child with cystic fibrosis is being treated with inhalation therapy with Pulmozyme (dornase alfa). A side effect of the medication is:

Weight gain

Hair loss

Sore throat

Brittle nails

Side effects of Pulmozyme include sore throat, hoarseness, and laryngitis. Answers A, B, and C are not associated with Pulmozyme; therefore, they are incorrect.

Explanation

Side effects of Pulmozyme include sore throat, hoarseness, and laryngitis. Answers A, B, and C are not associated with Pulmozyme; therefore, they are incorrect.

15. The nurse is teaching the mother of a child with cystic fibrosis how to do postural drainage. The nurse should tell the mother to:

Use the heel of her hand during percussion

Change the child’s position every 20 minutes

Do percussion after the child eats and at bedtime

Use cupped hands during percussion

The nurse or parent should use a cupped hand when performing chest percussion. Answer A is incorrect because the hand should be cupped. Answer B is incorrect because the child’s position should be changed every 5–10 minutes and the whole session should be limited to 20 minutes. Answer D is incorrect because chest percussion should be done before meals.

Explanation

The nurse or parent should use a cupped hand when performing chest percussion. Answer A is incorrect because the hand should be cupped. Answer B is incorrect because the child’s position should be changed every 5–10 minutes and the whole session should be limited to 20 minutes. Answer D is incorrect because chest percussion should be done before meals.

16. The mother of a child with cystic fibrosis tells the nurse that her child makes "snoring" sounds when breathing. The nurse is aware that many children with cystic fibrosis have:

Choanal atresia

Nasal polyps

Septal deviations

Enlarged adenoids

Children with cystic fibrosis are susceptible to chronic sinusitis and nasal polyps, which might require surgical removal. Answer A is incorrect because it is a congenital condition in which there is a bony obstruction between the nares and the pharynx. Answers C and D are not specific to the child with cystic fibrosis; therefore, they are incorrect.

Explanation

Children with cystic fibrosis are susceptible to chronic sinusitis and nasal polyps, which might require surgical removal. Answer A is incorrect because it is a congenital condition in which there is a bony obstruction between the nares and the pharynx. Answers C and D are not specific to the child with cystic fibrosis; therefore, they are incorrect.

17. 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?

Immediately before a meal

At least 2 hours after a meal

When bronchospasms occur

When secretions have mobilized

The nurse should perform chest physiotherapy at least 2 hours after a meal to reduce the risk of vomiting and aspiration. Performing it immediately before a meal may tire the client and impair the ability to eat. Percussion and vibration, components of chest physiotherapy, may worsen bronchospasms; therefore, the procedure is contraindicated in clients with bronchospasms. Secretions that have mobilized (especially when suction equipment isn’t available) are a contraindication for postural drainage, another component of chest physiotherapy.

Explanation

The nurse should perform chest physiotherapy at least 2 hours after a meal to reduce the risk of vomiting and aspiration. Performing it immediately before a meal may tire the client and impair the ability to eat. Percussion and vibration, components of chest physiotherapy, may worsen bronchospasms; therefore, the procedure is contraindicated in clients with bronchospasms. Secretions that have mobilized (especially when suction equipment isn’t available) are a contraindication for postural drainage, another component of chest physiotherapy.

18. The nurse is providing a health teaching to the mother of an 8-year-old child with cystic fibrosis. Which of the following statement if made by the mother would indicate to the nurse the need for further teaching about the medication regimen of the child?

“My child might need an extra capsule if the meal is high in fat”

“I’ll give the enzyme capsule before every snack”

“I’ll give the enzyme capsule before every meal”

“My child hates to take pills, so I’ll mix the capsule into a cup of hot chocolate

The pancreatic capsules contain pancreatic enzyme that should be administered in a cold, not a hot, medium (example: chilled applesauce versus hot chocolate) to maintain the medication’s integrity.

Explanation

The pancreatic capsules contain pancreatic enzyme that should be administered in a cold, not a hot, medium (example: chilled applesauce versus hot chocolate) to maintain the medication’s integrity.

19. The recommended diet for someone with cystic fibrosis is:

High calorie and high fat diet

High fat diet

Low fat diet

High calorie diet

The current recommendation is 120-150% of the recommended daily allowance of calories for children, and 40% fat. Adults with cystic fibrosis also need to eat high calorie food, with increased fat. This is because a person with cystic fibrosis doesn't absorb nutrients as well from their food, especially fat. A certain amount of fat is needed in your diet to keep you healthy.

Explanation

The current recommendation is 120-150% of the recommended daily allowance of calories for children, and 40% fat. Adults with cystic fibrosis also need to eat high calorie food, with increased fat. This is because a person with cystic fibrosis doesn't absorb nutrients as well from their food, especially fat. A certain amount of fat is needed in your diet to keep you healthy.

20. If one of your parents have the disease does that mean you have a chance of getting the infection?

True

False

Both your parents must have the gene in order for you to get Cystic Fibrosis

Explanation

Both your parents must have the gene in order for you to get Cystic Fibrosis

21. An 8-year-old client with cystic fibrosis is admitted to the hospital and will undergo a chest physiotherapy treatment. The therapy should be properly coordinated by the nurse with the respiratory therapy department so that treatments occur during:

After meals

Between meals

After medication

Around the child’s play schedule

Chest physiotherapy treatments are scheduled between meals to prevent aspiration of stomach contents, because the child is placed in a variety of positions during the treatment process.

Explanation

Chest physiotherapy treatments are scheduled between meals to prevent aspiration of stomach contents, because the child is placed in a variety of positions during the treatment process.

22. A 2-year-old client with cystic fibrosis is confined to bed and is not allowed to go to the playroom. Which of the following is an appropriate toy would the nurse select for the child:

Puzzle

Musical automobile

Arranging stickers in the album

Pounding board and hammer

The autonomous toddler would be frustrated by being confined to be. The pounding board and hammer is developmentally appropriate and an excellent way for the toddler to release frustration.

Explanation

The autonomous toddler would be frustrated by being confined to be. The pounding board and hammer is developmentally appropriate and an excellent way for the toddler to release frustration.

23. A child who has recently been diagnosed with cystic fibrosis is in a pediatric clinic where a nurse is performing an assessment. Which later finding of this disease would the nurse not expect to see at this time?

Positive sweat test

Bulky greasy stools

Moist, productive cough

Meconium ileus

Moist, productive cough Option c is a later sign. Noisy respirations and a dry non-productive cough are commonly the first of the respiratory signs to appear in a newly diagnosed client with cystic fibrosis (CF). The other options are the earliest findings. CF is an inherited (genetic) condition affecting the cells that produce mucus, sweat, saliva and digestive juices. Normally, these secretions are thin and slippery, but in CF, a defective gene causes the secretions to become thick and sticky. Instead of acting as a lubricant, the secretions plug up tubes, ducts and passageways, especially in the pancreas and lungs. Respiratory failure is the most dangerous consequence of CF.

Explanation

Moist, productive cough Option c is a later sign. Noisy respirations and a dry non-productive cough are commonly the first of the respiratory signs to appear in a newly diagnosed client with cystic fibrosis (CF). The other options are the earliest findings. CF is an inherited (genetic) condition affecting the cells that produce mucus, sweat, saliva and digestive juices. Normally, these secretions are thin and slippery, but in CF, a defective gene causes the secretions to become thick and sticky. Instead of acting as a lubricant, the secretions plug up tubes, ducts and passageways, especially in the pancreas and lungs. Respiratory failure is the most dangerous consequence of CF.

24. Cystic fibrosis is also called:

Bronchiectasis

Sticky mucus disease

Mucovicidosis

Irritating cough disease

Cystic fibrosis is often called mucovicidosis, especially in other regions of the world. This refers to the thick, sticky mucus secreted by the exocrine glands of someone affected by cystic fibrosis.
Cystic fibrosis was named that in 1938 by Dr. Dorothy Anderson, a pathologist in New York City. She described changes in the pancreas as cystic, or fluid-filled, and fibrotic, or scarred.

Explanation

Cystic fibrosis is often called mucovicidosis, especially in other regions of the world. This refers to the thick, sticky mucus secreted by the exocrine glands of someone affected by cystic fibrosis.
Cystic fibrosis was named that in 1938 by Dr. Dorothy Anderson, a pathologist in New York City. She described changes in the pancreas as cystic, or fluid-filled, and fibrotic, or scarred.

25. Which of the following is the approximate prevalence ratio for cystic fibrosis?

1: 25,000

1: 5,000

1: 2,000

1: 800

The approximate prevalence ratio for cystic fibrosis is 1 in 2,000. This means that for every 2,000 individuals, one is estimated to have cystic fibrosis.

Explanation

The approximate prevalence ratio for cystic fibrosis is 1 in 2,000. This means that for every 2,000 individuals, one is estimated to have cystic fibrosis.

26. Cystic Fibrosis is a

Cystic Fibrosis is a genetic disease caused by a mutation in the CFTR gene. This gene provides instructions for the production of a protein that regulates the movement of salt and water in and out of cells. When the CFTR gene is mutated, the protein is not formed correctly, leading to the buildup of thick, sticky mucus in the lungs, digestive system, and other organs. This genetic mutation is inherited from both parents and is considered a genetic disorder rather than an infection.

Explanation

Cystic Fibrosis is a genetic disease caused by a mutation in the CFTR gene. This gene provides instructions for the production of a protein that regulates the movement of salt and water in and out of cells. When the CFTR gene is mutated, the protein is not formed correctly, leading to the buildup of thick, sticky mucus in the lungs, digestive system, and other organs. This genetic mutation is inherited from both parents and is considered a genetic disorder rather than an infection.

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