CF Flashcards
What is cystic fibrosis
An inherited life-threatening disorder that damages the lungs and digestive system.
Cystic fibrosis affects the cells that produce mucus, sweat and digestive juices. It causes these fluids to become thick and sticky. They then plug up tubes, ducts and passageways
What are the symptoms of cystic fibrosis with respect to the digestive system
People with cystic fibrosis often experience digestive symptoms such as:
• greasy and bulky stools
• frequent and/or difficult bowel movements
• constipation
• nausea
• swollen abdomen
• loss of appetite
How do the conditions affect the functioning of the digestive system
In digestion in people with CF, the small tubes that transport these enzymes out of the pancreas become blocked with mucus. The enzymes build up in the pancreas instead of reaching the digestive system (specifically, the lumen of the gut), causing the pancreas to become inflamed
The pancreas also makes insulin, a hormone that regulates the amount of sugar in the blood. The scarring caused by the build-up of enzymes in people with CF can mean that the pancreas does not produce insulin as effectively, resulting in CF-related diabetes (CFRD), a condition that affects more than 30% of adults with cystic fibrosis.
What diet modifications are required for cystic fibrosis
You are given a special diet low in fat and high in carbohydrates and protein.
The diet is supplemented with pancreatic extract and large doses of vitamins A, D, and K.
How does cystic fibrosis affect regulation and protection of the body
People with CF lose 3-4 times more salt through their sweat than those without CF.
the CF sweat gland is unable to absorb salt back into the blood. This leaves large amounts of salt in their sweat. Because the level of salt in the blood does not rise, the body has no recognition of thirst.
Inheritance patterns of CF
this condition is Inherited in an autosomal recessive pattern, which means both copies of the gene in each cell have mutations. The parents of an individual with an autosomal recessive condition each carry one copy of the mutated gene, but they typically do not show signs and symptoms of the condition.
The genetic cause of each condition
Cystic fibrosis 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 or functioning of the CFTR protein found in the cells of the lungs and other parts of the body.
CF affects chromosome 7
Occurrence of the allele in the Australian population
In Australia, 1 in 3700 people have cystic fibrosis and 1 in 25 are carriers.
Rate of occurrence of condition in newborns in Australia
In Australia, one in 2,500 babies are born with CF, that’s one every four days
Survival rates past and present of CF
Today the average life expectancy for Australians with CF is 38 (still less than half that of the average Australian) and the survival rate for children with CF has increased to 50%. Over the last three decades survival rate of those affected by CF has increased but the mortality rate has remained the same.
Find the treatment applied for CF
antibiotics to prevent and treat chest infections.
medicines to make the mucus in the lungs thinner and easier to cough up.
medicines to widen the airways and reduce inflammation.
special techniques and devices to help clear mucus from the lungs
Community support groups that help families with members with CF
their peer support program provides opportunities for people with CF, their family, and carers to have a chat with a peer volunteer. Their peer volunteers are members of the CF community including parents, siblings, grandparents and individuals with CF. Peer volunteers are available to have a chat, share information, experiences and provide a listening ear.
Facebook groups for women with CF, some of these include:
The Australian CF Camp, Sister5Roses, CF Talk
What test can be done to identify sufferers and carriers of CF
People who are concerned that they may be carriers of CF can be tested via a laboratory test done on a sample of blood or salvia.
A blood sample is usually taken from the baby’s heel within two to three days after birth.
definition of CF
a disorder controlled by a recessive allele, it results in chest infections, a lack of digestive enzymes and increased salt loss.
where are epithelial cells found
including the sweat glands, the lungs, and the pancreas
