Cystic fibrosis

1

Seventy percent of CF patients are diagnosed by what age?

G-tube

Some children with CF receive supplemental feeding through one of these overnight to increase caloric intake.

A, D, E, K

These are the fat soluble vitamins.

cirrhosis of the liver

This is when scar tissue replaces function liver tissue, associated with blockage of the common bile duct in CF.

double lung transplant

This may be given to a child or young adult, but is usually reserved as a last resort.

total parenteral nutrition

This may be needed to support nutrition if g-tube, increased calories are not enough.

poor absorption (failure to thrive, slowed weight gain)

This may result from an intestinal blockage in CF patients if it impedes absorption.

prolonged neonatal jaundice

This may result from an intestinal blockage not allowing bilirubin waste products to be expelled in newborns in CF patients.

bile duct

A tube that carries bile from the liver and gallbladder to the intestine.

dehydration, heat stroke

Because they can't hang onto Cl, CF patients lose Na, which follows Cl, putting them at increased risk of thew two problems.

pancreatic duct obstruction

Bulky, foul-smelling, frothy stools, 2-3 times the normal amount, and excessive flatus, are associated with what complication of CF?

bacterial

CF causes recurrent _____ infections and colonization in the lungs.

A, D, E, K

CF patients are given water soluble forms of these vitamins, because they have trouble processing fat.

high protein, high calorie, low fat

CF patients diets should include these three things.

fluid, nutrients

CF patients need to get adquate _____ and _____ intake to promote mucus removal and fight off weight loss.

crowds

CF patients should avoid these and exposure to people with respiratory infections because they are prone to colonization.

3, 3

CF patients, usually have __ meals and __ snacks per day.

autogenic drainage

Controlled breathing to remove secretions, breath shallow and slow to start for a few minutes, then more deeply with larger exhale, then even deeper.

caucasian

Cystic fibrosis is more common in this ethnic group.

infertility

This occurs due to mucus build-up in the male and female reproductive tracts, including absence of a vas deferens in men and mucus covering the cervix in females.

irregular ovulation

This occurs in female CF patients due to chronic illness and poor nutrition, as well as genital tract obstruction.

lipase, trypsin, amylase

If CF causes a blockage of the pancreactic duct in the pancreas, what three enzymes won't reach the duodenum?

60

If Cl in sweat reaches __ mEq/l or more, CF is confirmed.

pilocarpine test (sweat test)

If an infant has an elevated trypsinogen (IRT) gene testing is undertaken. If that finds one mutation, this test is done to confirm the diagnosis.

1/4

If both parents are heterozygous, what is the chance their children will have CF? (Recall they need 1 defective gene from each parent to get the disease.)

0

If only one partner is heterozygous, what is the chance they'll have a CF child?

insulin

If pancreatic ducts are blocked, what hormone won't be released resulting in CF associated diabetes?

digestion

If pancreatic enzymes don't reach the intestines due to CF, _____ will be impeded, especially of fats, proteins, and fat soluble vitamins.

fats, proteins, vitamins

If pancreatic enzymes don't reach the intestines due to CF, digestion will be impeded, especially of _____, _____ and fat soluble _____.

1/2

If two heterozygous parents have a child, what are the odds that their offspring will at least carry the trait of CF?

1/4

If two heterozygous parents have a child, what are the odds that their offspring will be unaffected and not carry the trait.

S. aureus, H. influenzae

Impaired mucus clearance in the lungs sets the stage for bacterial colonization. Most patients have these two bacteria colonized in early childhood.

Pseudmonas aeruginosa

Impaired mucus clearance in the lungs sets the stage for bacterial colonization. Most patients have this bacteria later in life. It is associated with increased loss of lung function and decreased survival.

distal intestinal obstructive syndrome

In CF, mucus builds up along the intestinal tract, slowing down the emptying of food. This the build-up of stool behind the mucus-filled area, causing a blockage high up in the intestines.

salt

Increased _____ content of the sweat is a sign of CF.

NSAIDs

Long term use of this drug is recommended for 6-17 year olds with CF to slow the loss of lung function.

meconium ileus

Mucus in CF patients can block the intestines, causing this problem when newborns fail to pass stool for 24-48 hours after birth.

40

Normal Cl level in a healthy persons sweat is __ mEq/L.

lengthened expiration

On a pulmonary function test, signs of CF are consistent with obstructive disease, so look for this result.

trypsinogen

Test for this pancreatic enzyme are accomplished through a heal prick at birth and rapid assay analysis.

plugging, bronchiectasis

The pathololgy of CF includes mucus _____, resulting in inflammation and eventual _____ that starts in the upper lobes and progresses down.

bile duct obstruction

The prevalence of problems associated with complication of CF increases with age.

mucolytics

These are agents that destroy or dissolve mucus.

steatorrhea

These are fatty, floating stools associated with pancreatic enzymes not reaching the intestines and impairing digestion and fat absorption.

antibiotics

These are first used to prevent colonization with pseudomonas aeruginosa, then as maintenance after colonization in CF patients.

salt supplements

These are given to CF patients in hot weather, because they can't hang onto Cl.

pancreatic enzyme supplements

These are given with every meal to aid in digestion, because because the bile duct gets blocked by mucus resulting in pancreatitis.

sputum culture

These are taken routinely to see if a patient is colonized with bacteria.

pancreatic enzymes

These are taken with meals and with snacks to aid in digestion.

pancreatic enzymes

These can be taken whole or sprinkled on food, but cannot be chewed or crushed as they are enteric coated enzymes that must get to the duodenum to be effective. (I'm not sure how that makes sense, but it's in the notes.)

antiinflammatory, bronchodilator

These drugs are used in CF patients to open the airways so that they can better expel secretions.

CFTR modulators

These drugs improve the function of the defective genes in CF, but they do not cure the disease.

heterozygous

These people do not have CF, but they are carriers and can pass it to their children.

hemozyotes

These people have all or substantially all of the manifestations of CF.

mucolytics, antibiotics

These two drugs are nebulized in the lungs of CF patients to clear mucous and fight infection.

Na, Cl

These two electrolytes are lost in excess amounts through perspiration in people with CF, especially in summer and during exercise when sweat increases.

daily weight, energy expenditure

These two nutritional assessments should be monitored in CF patients.

chronic sinusitis, nasal polyps

These two signs are consistent with CF because all of their mucous membranes develop thick secretions due to the impaired transport of Cl.

infertility

Thickened cervical and vaginal secretions in CF patients causes this problem.

Focal biliary cirrhosis

This can cause cirrhosis of the liver, associated with blockage of the common bile duct in CF patients.

distal intestinal obstructive syndrome

This complication of CF can be caused by frequently missed pancreatic enzymes and not enough fluid intake, especially when exercising in warmer weather.

distal intestinal obstructive syndrome

This complication of CF can cause painful cramping and stomach aches, fewer bowel movements, vomiting, and a feeling of fullness accompanied by diarrhea.

weight loss

This complication of CF occurs because of mucus build-up in the intestines impairing absorption, as well as pancreatic and liver changes that impair digestion.

barrel chest

This deformity occurs because of air trapping in CF, making it difficult to breath.

clubbed fingers/toes

This deformity occurs with hypoxemia in CF.

Ensure

This drink can increase caloric intake in CF patients.

trypsinogen

This for babies is for screening purposes because heterozygous carriers can also have increased levels.

chest physiotherapy

This includes manual postural drainage, high frequency chest wall oscilation, autogenic drainage, and positive experatory pressure masks.

chest physiotherapy

This is typically done in the a.m. upon waking, mid morning, mid afternoon, and nightly. Try to avoid meal times.

rectal prolapse

This is when part of the intestines protrudes outside the anus due to coughing and frequent hard to pass stools in CF patients.

azoospermia

This is a complete lack of sperm that occurs in 98 percent of male CF patients due to genital tract abstruction that causes a lack of vas deferens and abnormal development of vas deferens.

Hypoprothrombinemia

This is a deficiency of clotting factors, caused by damage to the liver associated with blockage of the common bile duct in CF patients.

cystic fibrosis

This is a gene defect that impairs chloride transport across epithelial cell membranes, resulting in thick, viscous secretions.

Trypsinogen

This is a pancreatic enzyme precursor that is elevated in the blood of most CF patients at birth due to partial blockage of the pancreatic ducts.

inguinal hernia

This is a protrusion of a small loop of bowel through a weak place in the lower abdominal wall or groin, caused by genital tract obstruction, increased coughing, and frequent hard to pass stool in CF patients.

hydrocele

This is a sac of clear fluid in the scrotum, caused by genital tract obstruction, increased coughing, and frequent hard to pass stool in CF patients.

CPT vest system

This is a vibrating vest used to loosen secretions before chest physiotherapy.

portal hypertension

This is an elevation of BP in the arteries feeding the liver, associated with blockage of the common bile duct.

double lung transplant

This is an option for some CF patients to extend the lifespan and improve lung function, but it does not cure the disease.

puberty

This is delayed in both male and female CF patients due to decreased nutritional status and lower body fat composition.

chest physiotherapy

This is done BID by parents when a child is healthy and QID by a respiratory therapist when the child is hospitalized to help clear secretions from the lungs.

chronic wheezing

This is indicative of CF because mucus plugging results in inflammation of the lungs.

chronic inflammation

This is indicative of CF because patients cough all the time, which is hard on the lungs.

hyperinflation

This is indicative of CF on an x-ray because mucus plugging traps air in the lungs.

productive cough

This is indiciative of CF because they have thick, viscous secretions in their lungs.

pilocarpine test (sweat test)

This is more likely to be accurate in infants and is generally not attempted under patients weighing less than 5 kg.

azotorrhea

This is nitrogen in the feces in urine, associated with impaired protein digestion caused by pancreatic duct blockage.

pilocarpine test (sweat test)

This is the gold standard for CF diagnosis. It measures abnormal Cl content of sweat.

heterozygous

This refers to people who have one defective gene and one normal gene.

exercise

This should be encouraged because is stimulates mucus removal, enhances pulmonary function and can be as effective as CPT if done on a regular basis.

genetic testing

This should be offered to people with a family history of CF or partners of a CF patient if they plan to become pregnant.

sweat test

This test looks for increase NaCl in sweat, which is a sign of CF.

DNA test

This test looks to see if the patient has two defective CF genes.

gene testing

This uses a panel that looks for the most common CF genes, but there are more than 2,000, so some are missed.

hyponatremia

What electrolyte imbalance would you expect to see in a person with CF, causing dizziness, lethargy, malaise, or thirst.

33

What is the median life expectancy for someone with CF.

meninges

Which of the following is not affected by CF? - lungs - meninges - liver - pancreas - intestines - reproductive tract

all are correct

Which of the following is not affected by CF? - lungs - pancreas - intestines - bile ducts - genital tracts

pancreatitis

inflammation of the pancreas

postural drainage

use of body positioning to assist in removal of secretions from specific lobes of the lung, bronchi, or lung cavities