CYSTIC FIBROSIS 2023 NEW UPDATE
1. What is cystic fibrosis??: a dysfunction of the exocrine glands that
causes the glands to produce thick, tenacious mucus.
2. What organs does cystic fibrosis affect??: Major organs affected are
the lungs, pancreas, small intestine, and liver. Abnormally thick
mucus leads to me- chanical obstruction of organs, which alters their
functions.
3. What are the risk factors of cystic fibrosis??: Cystic fibrosis is
hereditary and transmitted as an autosomal recessive trait. Thus, both
parents must be carriers of the gene.
4. What are the signs/symptoms of cystic fibrosis??: History of chronic
repira- tory infections...Meconium ileus at birth manifested as
distention of the abdomen, vomiting (may
be bile-stained), and inability to pass stool. Absence of pancreatic
enzymes. Respi- ratory findings: Fatigue, Chronic cough; Thick, yellow-
grey mucus, Positive sputum culture (Pseudomonas aeruginosa,
Haemophilus influenzae), Fever; Shortness of breath, dyspnea, and
wheezing, Cyanosis, Difficulty exhaling air, resulting in hyperinflation
of the lungs, Barrel-shaped chest, Clubbing of the fingers and toes
Gastrointestinal findings: Large, loose, fatty, sticky, foul-smelling
stools, Impaired digestion, Failure to gain weight, Delayed growth
patterns, Distended abdomen, Thin arms and legs, Atrophy of buttocks
and thighs
Integumentary findings:Sweat, tears, and saliva are abnormally salty
Endocrine and reproductive system findings: Delayed puberty,
Viscous cervical mucus, Decreased or absent sperm
5. What are the laboratory test for cystic fibrosis??: A sweat chloride test
should be used to measure the amount of chloride in skin sweat. A
normal chloride concentration of sweat is less than 40 mEq/L. Values
of greater than 40 mEq/L in infants are suggestive of cystic fibrosis,
and values greater than 60 mEq/L in children indicate the presence
1/
, of cystic fibrosis.
A stool analysis can indicate the presence of steatorrhea (undigested
fat) and azotorrhea (foul-smelling from protein).
Sputum culture and sensitivity can detect infection.
6. What are the diagnostic procedures for CF??: Blood glucose-
Hyperglycemia related to insulin resistance or deficiency
Chest x-ray-May indicate diffuse atelectasis and obstructive
emphysema Abdominal x-ray-Detect meconium ileus
7. What should a nurse do to provide care of a patient with CF??: Give
respiratory treatments to include aerosol therapy, chest
physiotherapy, breathing
2/
1. What is cystic fibrosis??: a dysfunction of the exocrine glands that
causes the glands to produce thick, tenacious mucus.
2. What organs does cystic fibrosis affect??: Major organs affected are
the lungs, pancreas, small intestine, and liver. Abnormally thick
mucus leads to me- chanical obstruction of organs, which alters their
functions.
3. What are the risk factors of cystic fibrosis??: Cystic fibrosis is
hereditary and transmitted as an autosomal recessive trait. Thus, both
parents must be carriers of the gene.
4. What are the signs/symptoms of cystic fibrosis??: History of chronic
repira- tory infections...Meconium ileus at birth manifested as
distention of the abdomen, vomiting (may
be bile-stained), and inability to pass stool. Absence of pancreatic
enzymes. Respi- ratory findings: Fatigue, Chronic cough; Thick, yellow-
grey mucus, Positive sputum culture (Pseudomonas aeruginosa,
Haemophilus influenzae), Fever; Shortness of breath, dyspnea, and
wheezing, Cyanosis, Difficulty exhaling air, resulting in hyperinflation
of the lungs, Barrel-shaped chest, Clubbing of the fingers and toes
Gastrointestinal findings: Large, loose, fatty, sticky, foul-smelling
stools, Impaired digestion, Failure to gain weight, Delayed growth
patterns, Distended abdomen, Thin arms and legs, Atrophy of buttocks
and thighs
Integumentary findings:Sweat, tears, and saliva are abnormally salty
Endocrine and reproductive system findings: Delayed puberty,
Viscous cervical mucus, Decreased or absent sperm
5. What are the laboratory test for cystic fibrosis??: A sweat chloride test
should be used to measure the amount of chloride in skin sweat. A
normal chloride concentration of sweat is less than 40 mEq/L. Values
of greater than 40 mEq/L in infants are suggestive of cystic fibrosis,
and values greater than 60 mEq/L in children indicate the presence
1/
, of cystic fibrosis.
A stool analysis can indicate the presence of steatorrhea (undigested
fat) and azotorrhea (foul-smelling from protein).
Sputum culture and sensitivity can detect infection.
6. What are the diagnostic procedures for CF??: Blood glucose-
Hyperglycemia related to insulin resistance or deficiency
Chest x-ray-May indicate diffuse atelectasis and obstructive
emphysema Abdominal x-ray-Detect meconium ileus
7. What should a nurse do to provide care of a patient with CF??: Give
respiratory treatments to include aerosol therapy, chest
physiotherapy, breathing
2/
