Cystic Fibrosis: Personal Perspectives Flashcards
Brief overview of CF Experience of living with CF, a chronic and life-limiting condition Health and emotional impact
Cystic fibrosis is a multi-organ/system disease, affecting the:
Respiratory
Gastrointestinal
Reproductive systems
CF is the most common _____ disorder
Inherited
The serverity of CF is ____
Variable
Severe forms have poor prognosis - no cure at present
Improvements in treatment have resulted in___
Increased lifespans
Death in CF patients is usually due to:
Respiratory failure
Organs are affected by production of:
Excessively thick, dehydrated, secretions of mucus around/in organs
Thick, dehydrated mucus is due to:
Failure of salt (specifically chloride ions) and subsequently water transport by epithelial cells lining ducts
Clinical features: respiratory
Symptoms
> Frrequent coughing
Chronic infections
Lung damage
> > Progressive loss of lung function
Clinical features: Respiratory
Progressive loss of lung function
Due to:
>obstruction of bronchioles by mucus
>colonisation by bacteria, especially antibiotic resistant strains
>damage caused by inflammatory responses
Clinical Features: Gastrointestinal
> Poor growth (failure to thrive)
Chronic malabsorption
Due to:
> Blockage of pancreatic ducts by mucus
>pancreatic enzymes don’t get to food, so digestion is poor (bile blockage)
> Poor digestion of fats (mainly) and proteins
Clinical Features: Gastrointestinal
Other features include:
> Intestinal obstruction in newborns (‘meconium ileus; 15% of neonates with CF)
> Pancreatic insufficiency (85% PTs)
Pancreatitis
CF diabetes, 40% adults
Liver disease
Clinical Features: Reproductive
> Infertility in males
Sub-fertility in females
Due to:
> blockage of vas deferens in males, leading to fibrosis or usually atrophy
congenital bilateral absence of the vas deferens (CBAVD)
> Cervical mucus in females acting as barrier to passage of sperm
> Females may be anovulatory
