Cystic Fibrosis Flashcards
is an inherited disease of the secretory glands
Cystic Fibrosis (CF)
The cystic Fibrosis disease is characterized by
thick mucus secretions, particularly in the pancreas and lungs, as well as electrolyte abnormalities in sweat gland secretions.
Cystic Fibrosis is inherited from an
autosomal recessive trait
______________ results in the inability to transport small molecules across cell membranes, leading to dehydration of epithelial cells
Abnormality of the long arm of chromosome 7
For early detection during pregnancy, ________ or _________ can be performed.
Chorionic Villi Sampling or Amniocentesis
Females affected by Cystic Fibrosis may have
thick cervical secretions that limit sperm motility
Affected males may be unable to reproduce secondary to persistent plugging and blocking of the vas deferens by
Tenacious seminal fluid
For cystic Fibrosis situation, patients who desire to become pregnant may have options like
*Alternative insemination
*In Vitro fertilization
the Acinar cells of the pancreas normally produce
*lipase
*trypsin
*amylase
Absence of pancreatic enzymes in the duodenum results in
inability to digest fat, protein, and some sugars.
Bowel movements become large, bulky, and greasy
Steatorrhea
An increase in the intestinal flora and fat results in
an extremely foul odor
increased bulk of feces often leads to
protuberant abdomen
Fat-soluble vitamins, particularly these vitamins cannot be absorbed in the absence of fat absorption resulting in vitamin deficiency
*Vitamin A
*Vitamin D
*Vitamin E
Meconium in newborn is normally
thick and tenacious
organisms most frequently cultured in CF situations
*Staphylococcus aureus
*Pseudomonas aeruginosa
*Haemophilus Influenzae
__________ may occur as a result of absorption of air from alveoli behind blocked bronchioles.
Atelectasis
The level of chloride to sodium in perspiration is increased
two to five time above normal
The level of chloride to sodium in perspiration is increased two to five time above normal resulting in
“salty” perspiration
Cystic Fibrosis may be suspected in a newborn who fails to regain normal birth weight within
7 to 10 days of birth
may occur because of inadequate peripheral tissue perfusion
Clubbed Fingers
Clubbed fingers may occur because of
inadequate peripheral tissue perfusion
Respiratory Infections due to Cystic Fibrosis develop around
4 to 6 months of age
wheezing and rhonchi are often heard on
chest auscultation
detects abnormal salt concentrations
sweat testing
a normal concentration of sodium chloride in sweat is
20 mEq/L
more than _______ of sodium chloride if diagnostic of CF
more than 60 mEq/L
may be used to detect pancreatic enzymes and reveal the extent of pancreatic involvement
Duodenal secretions
the easiest pancreatic enzyme to assay
trypsin content
may be tested to determine the extent of Atelectasis and emphysema
Pulmonary function
is a collaborative process to reduce the involvement of the pancreas, lungs, and sweat glands.
Therapeutic Management
Cystic fibrosis (CF) is a genetic disorder that primarily affects
lungs and digestive enzymes (pancreas)
CFTR
Cystic Fibrosis Transmembrane Conductance Regulator
Individuals who inherit only one faulty gene are called as
“carriers”
most common in what decent?
Caucasians of Northern Europe Descent
digital clubbing is a sign of
hypoxia (low oxygen levels)
refers to the abnormal enlargement and rounding of the fingertips or toes
Digital Clubbing
is a sinonasal manifestation of cystic fibrosis
Nasal Polyps
Signs and Symptoms of Cystic Fibrosis:
*persistent cough
*salty tasting skin
*digital clubbing
*frequent lung infections
*wheezing, or shortness of breath
*nasal polyps
*poor growth or poor weight gain
can be generated using an ultrasonic compressor and administered via a nebulizer mask.
nebulized mist
can be included in the mist
to help thin and loosen secretions
A mucolytic
children may have such thick bronchial secretions that they are unable to cough them up, even after they have been thinned by mist or aerosol therapy, they need
Chest Physiotherapy
How to diagnose Cystic
Fibrosis
*sweat testing
*duodenal analysis
*stool analysis
*pulmonary testing
is a special procedure used to manage pancreatic insufficiency in
individuals with CF
pancreatic enzyme replacement therapy
(PERT)
PERT involves taking
oral pancreatic enzyme supplements
with meals and snacks
elevated blood pressure in the lungs,
pulmonary hypertension.
Bacteria line the airways in diseases that cause permanent widening of the
large airways
bronchiectasis
Nursing Diagnosis for Cystic Fibrosis
*Impaired Gas Exchange related to thick respiratory secretions
* Ineffective Airway Clearance related to copious bronchial secretions as evidenced by nasal polyps
* Acute Pain related to bowel blockage
* Risk For Deficient Fluid Volume related to excessive fluid loss
* Imbalanced Nutrition related to malabsorption
________ of the chest becomes enlarged in CF
The anteroposterior diameter of the chest
