Cystic fibrosis Flashcards
What are the symptoms of cystic fibrosis with respect to the digestive system
o Pain
o Cramping
o Gas
o Constipation
o Blockages
o diarrhea that does not go away
o foul-smelling stools
o greasy stools
o frequent urinating
How does cystic fibrosis affect the functioning of the digestive system
- Thick and sticky mucus blocks the ducts in the pancreas and prevents digestive juices containing enzymes from reaching the small intestine to digest food.
- The build-up of mucus in the ducts of the liver prevents bile from being secreted out to emulsify and digest fats.
- The mucus will get very thick and flow slowly causing stones in the gall bladder to form which sometimes need to get removed.
- The pancreas does not make enough bicarbonate to neutralise the stomach acid which causes various symptoms.
What diet modifications are required for cystic fibrosis
A special diet low in fat and high in carbohydrates and proteins. The diet is supplemented with pancreatic extract and large doses of Vitamin A, D, and K.
o Dairy
o Fruits and vegetables
o whole grains
How does cystic fibrosis affect the regulation and protection of the body
The CFTR protein enables chloride ions to pass into and out of cells. Chloride ions attract water to cause the mucus lining the cells to be at a healthy consistency. People with CF however lack these CFTR proteins meaning insufficient amounts of chloride ions to be passed into and out of cells causing the mucus to be secreted to be very thick and sticky. This thick mucus lining the lungs will make it more difficult for the little hairs to move and carry mucus out of the body causing bacteria to build up. The build-up of mucus in the pancreatic and gall bladder ducts causes pancreatic lipase to not digest fats and pancreatic amylase to not digest carbohydrates. If carbohydrates cannot be broken down into glucose, they experience a lack of energy.
Inheritance patterns
Cystic fibrosis is an autosomal recessive disorder. This means both copies of the gene in each cell have mutations. Both parents carry a single copy of this recessive genotype, however, do not phenotypically display it.
The genetic cause
It is caused by mutations in the gene that produces the cystic fibrosis transmembrane conductance regulator (CFTR) protein. In people with CF, mutations in the CFTR gene can disrupt the normal production of the CFTR protein found in the cells of the lungs and other parts of the body
Occurrence of the allele in the Australian population
1 in 25 Australians is carrier of the disorder.
Rate of occurrence of CF in newborns in Australia
1 in 3700 babies are born with CF in Australia.
Survival rates past and present
In the 1980’s the life expectancy of individuals with cystic fibrous was 14 years. Now the life expectancy is around 38 years old.
Find the treatments applied to cystic fibrosis
- Synthetic enzyme tablets
- Diabetic therapy
- Chest wall oscillation
- antibiotics
- Vaccines
what do synthetic enzyme tablets do
this supplement will mimic what the pancreas will usually make. It replaces the enzymes which cannot be excreted by the pancreas due to the clocked bile ducts.
what does diabetic therapy do
In some CF patients, there is a lack of insulin due to the blocked pancreas. Because of this patient can be diagnosed with CF related diabetes as they require insulin to maintain their blood sugar level.
what does chest wall association do
Is a device that will rhythmically press on the chest to clear mucus from the lungs.
what do vaccines do
hepatitis A and B can reduce liver problems.
Search for community support groups that help families with members with CF
Peer support cystic fibrosis Australia Provides support via –
o phone calls
o Email
o Skype
