Cystic Fibrosis Flashcards
what does the upper respiratory tract consists of
nasal cavity and the pharynx.
what does the lower respiratory tract consists of
the larynx, trachea, bronchi and lungs.
describe the nasal cavity
function is to provide a passageway of air when mouth is full, clean and humidy air, olfactory senses and determine the sound of voice.
describe the pharynx
divided into:
nasopharynx which prevents swallowed material from entering the nasal cavity
oropharynx which extends from soft palate to epiglottis providing passageway for air, food and drink
laryngopharynx which extends from epiglottis to oesophagus to provide a passageway for food and drink
describe the larynx
passageway of air between pharynx and trachea containing laryngeal cartilages for maintaining an open airway and countians vocal cords for sound production.
describe the trachea
membranous tube attached to the larynx beginning below the cricoid cartilages, containing c-shaped hyaline cartilage rings to maintain an open airway.
describe the bronchi
maintain open airway to supply air to and remove air from alveoli for efficient gaseous exchange.
describe the lungs
divided into lobes and no major blood vessels so part of it can be removed.
what causes cystic fibrosis
Cystic fibrosis is caused by a faulty CFTR gene.
• In about 70% of cases CF is caused by a defective channel protein failing to reach the plasma membrane from its site of production inside the cell.
• In the remaining cases the channel protein is incorporated into the plasma membrane but does not function normally.
o In some cases, the channel protein fails to bind to ATP.
o In others, ATP binds to the channel protein but the channel doesn’t open.
The failure of these ion channels to function normally causes the affected cells to produce thick, viscous, mucous secretions.
why are people with cystic fibrosis at increased risk of infection and lung damage
Mucus coats breathing tubes so they become so thick and sticky that cilia are unable to sweep the bacteria and other particles up and out of the lungs. The chance of infection is high due to the extra bacteria present. This can lead to conditions such as pneumonia. The bacteria which is trapped in the lungs can lead to frequent, severe and permanent lung damage. The extra white blood cells which destroy the bacteria also destroy some healthy cells causing further lung damage. The debris from the White blood cells also line the lining of the alveoli further, increasing the respiratory issue.
discuss the main symptoms of cystic fibrosis (at birth, resp symptoms, lungs, and infections)
types and severity of symptoms differ widely from person to person.
Symptoms at birth include serious bowel obstruction in 10% of babies with CF, a few will also have jaundice.
Respiratory symptoms include persistent coughing to clear thick mucous from the lungs, coughing fits can also lead to vomiting and urinary incontinence.
Inflammation of the lungs can cause wheezing, shortness of breath and breathing difficulties upon exertion.
Lung bleeding and collapsed lung can also occur.
Recurring chest infections are common caused by a continual build up of mucous in lungs. High risk of cross infection with others who suffer from CF.
CF effects both lungs and digestive system- how does it affect the digestive system
Babies with CF can be born with bowel obstruction (meconium ileus). The bowel will contain meconium which should be passed within 2 days of birth, if not, must be surgically removed.
There is excess mucous along the digestive tract, this blocks the the ducts of the Pancreas so not enough enzymes reach intestines, particularly fat digesting enzymes and insulin. The pancreas is then slowly destroyed by fibrous cysts causing malabsorption of nutrients, in turn, causing malnutrition and struggle to gain weight. This can also effect stools by excess fat in the system, causing smelly stools that are hard to pass.
discuss treatments for CF
Nebuliser – saline to loosen up the mucus and make it easier to cough up.
Medications – anti-inflammatories and antibiotics to help clear and control infections in the lungs. Pancreatic enzymes to aid digestion.
Physiotherapy – to clear the mucus in the chest.
Exercise – to loosen mucus.
Appropriate diet and vitamin supplements.
Lung transplant in extreme cases
