Cystic Fibrosis, Obesity, and Child Abuse Flashcards
What is cystic fibrosis?
is an inherited, recessive, genetic disorder that affects the exocrine glands (those glands that secrete mucus) due to a genetic defect.
How does CF present?
CF usually presents with primary problems in the lungs, sinus cavities, and digestive system
What are the early symptoms of CF?
Early symptoms include a dry cough, and an increased respiratory breathing rate
What are the later symptoms of CF?
Later symptoms include frequent cough and frequent respiratory infections.
What is the incidence of CF?
1/2500 births
___% are diagnosed with CF in the first year of life, ____% by age two and ____% by 10 years of age.
60% are diagnosed in the first year of life
75% by age two
90% by 10 years of age.
Approximately _____ people in the US are diagnosed with CF
30,000
Adults with CF now account for _____%
of CF population
38.7
The key to Cystic Fibrosis is …
Clogging of ducts
With CF, the affected areas of the body are …
the airways, liver, pancreas, intestine, and
reproductive tract.
With CF, Usually, death occurs early; often before age ___-___ (median life expectancy ____ yrs)
30-40
37.5
How are airways affected by CF?
Clogging and infection of bronchial passages impede breathing
Infections destroy the lungs
Lung disease accounts for the most deaths from CF
How is the liver affected by CF?
Plugging of small bile ducts impedes digestion and disrupts liver function in 5% of pts
How is the pancreas affected by CF?
Occlusion of ducts prevents the pancreas from delivering critical digestive enzymes to the bowel in 65% of patients
Diabetes can result as well
How is the small intestine impacted by CF?
Obstruction of the gut by thick stool necessitates surgery in about 10% of newborns
How is the reproductive tract impacted by CF?
Absence of fine ducts, such as the vas deferans, renders 95% of males infertile. Occasionally, women are made infertile by a dense plug of mucus that blocks sperm from entering the uterus.
How is the skin impacted by CF?
malfunctioning of sweat glands causes perspiration to contain excessive salt. Measurement of chloride in sweat is a mainstay of diagnosis.
What are the chances of a baby getting CF?
If both parents are carriers; ● A 25% chance that the child will be born with cystic fibrosis; ● A 50% chance that the child will not have CF, but will be a carrier; ● A 25% chance that the child will not have CF, and will not be a carrier.
Gene responsible for CF identified in1989:
CF Transmembrane Regulator Protein (CFTR).
In normal cells, CFTR allows …
the release of Clˉ from the cell.
When the Clˉ leaves the cell, an imbalance is created which …
draws water out of the cell through osmosis.
Pathophysiology of CF
● Abnormally thick, viscous mucus secretions principally affecting:
◦ BRONCHIAL TUBES
–◦ obstruction, damage to cilia, immobility of mucus blanket
◦ PANCREATIC DUCTS
–◦ digestive enzymes cannot leave the pancreas- malnutrition
◦ SWEAT GLANDS
–◦ salty sweat loss
◦ CARDIOPULMONARY SYSTEM
–◦ nailbed clubbing, airway collapse, etc.
◦ REPRODUCTIVE SYSTEM
–◦ sterility in males, difficulty with conception in females
Which lower airway infection is a death sentence for patients with CF?
p. cepacia
What red flag occurs when a newborn doesn’t poop?
meconium ileus
What are the treatments for CF?
1) Postural drainage⇒ bronchial dilator⇒ gravity drainage, percussive therapy
2) Intermittent positive pressure breathing (IPPB)
3. Self- administered airway clearance techniques (ACTs), Autogenic drainage, active cycle of breathing, ThAIRapy Vest and PEP devices such as the Acapella and the Flutter
How does CF impact exercise response?
● Related to severity of lung disease
● Can be affected by nutritional status
● Can be affected by pathophysiology of skeletal muscle tissue
