John is 5 years old and was diagnosed with cystic fibrosis following intestinal obstruction after
birth. He has frequent infections despite respiratory therapy. Answer the following question
regarding Cystic Fibrosis.
1)John’s parents would like to have another child. What is the probability that the child will have
cystic fibrosis?
2)Describe the basic pathophysiology of cystic fibrosis and the effects in the body.
3)What are the effects on airflow of mucus obstruction in the lungs?
4)Why does John have frequent infections?
5)Why does John have fluid-electrolyte imbalances particularly when experiencing a high fever?
Solution
1) Since Cystic Fibrosis is an autosomal recessive disease, the chances of its occurrence are 1 in
four births. John\'s first child is having Cystic Fibrosis, still, the chance is 25% for the next child
to have the disease. The child may or may not have cystic fibrosis.
2) In cystic fibrosis, there is a mutation in the CFTR gene and different mutation in the gene
cause different protein formation which results in mild or severe disease. The gene is responsible
for the formation of a protein that anchors on the outer walls of the exocrine glands including
lungs, pancreas and sweat glands. The encoded protein creates a channel which is responsible for
the movement of halogens from inside and outside of the body. the nonfunctioning of the protein
results in the blockage of the channels of the affected organs and causes infection and
remodeling of the lungs. There is damage in the digestive system due to the accumulation of
digestive enzymes. Due to this, there are chances that the patient will acquire chronic infection
and proliferation of bacteria in the accumulated mucus in the lungs.
3) The airflow in the lungs gets affected in cystic fibrosis as there is deposition of mucus in the
small airways of the lungs. Since the mucus gets lined is dehydrated and is difficult to clear and
eventually obstructs the air pathway causing the destruction in alveoli and deposition of foreign
particles in the airways. This can lead to frequent chronic infections.
4) The reason behind the frequent infection of John is the mucus deposition in the air pathways
which leads to the proliferation of bacteria in the nutrient-rich mucus. These bacteria forms
biofilms inside which is difficult for the immune cells to penetrate and which makes the
condition severe.
5) This condition of John is due to the improper transfer of ions in the body due to the narrowing
of the channels. This gets severe as the body fights with the soaring body temperature as well as
the defect in the channels of ions transport..
DEMONSTRATION LESSON IN ENGLISH 4 MATATAG CURRICULUM
John is 5 years old and was diagnosed with cystic fibrosis following.pdf
1. John is 5 years old and was diagnosed with cystic fibrosis following intestinal obstruction after
birth. He has frequent infections despite respiratory therapy. Answer the following question
regarding Cystic Fibrosis.
1)John’s parents would like to have another child. What is the probability that the child will have
cystic fibrosis?
2)Describe the basic pathophysiology of cystic fibrosis and the effects in the body.
3)What are the effects on airflow of mucus obstruction in the lungs?
4)Why does John have frequent infections?
5)Why does John have fluid-electrolyte imbalances particularly when experiencing a high fever?
Solution
1) Since Cystic Fibrosis is an autosomal recessive disease, the chances of its occurrence are 1 in
four births. John's first child is having Cystic Fibrosis, still, the chance is 25% for the next child
to have the disease. The child may or may not have cystic fibrosis.
2) In cystic fibrosis, there is a mutation in the CFTR gene and different mutation in the gene
cause different protein formation which results in mild or severe disease. The gene is responsible
for the formation of a protein that anchors on the outer walls of the exocrine glands including
lungs, pancreas and sweat glands. The encoded protein creates a channel which is responsible for
the movement of halogens from inside and outside of the body. the nonfunctioning of the protein
results in the blockage of the channels of the affected organs and causes infection and
remodeling of the lungs. There is damage in the digestive system due to the accumulation of
digestive enzymes. Due to this, there are chances that the patient will acquire chronic infection
and proliferation of bacteria in the accumulated mucus in the lungs.
3) The airflow in the lungs gets affected in cystic fibrosis as there is deposition of mucus in the
small airways of the lungs. Since the mucus gets lined is dehydrated and is difficult to clear and
eventually obstructs the air pathway causing the destruction in alveoli and deposition of foreign
particles in the airways. This can lead to frequent chronic infections.
4) The reason behind the frequent infection of John is the mucus deposition in the air pathways
which leads to the proliferation of bacteria in the nutrient-rich mucus. These bacteria forms
biofilms inside which is difficult for the immune cells to penetrate and which makes the
condition severe.
5) This condition of John is due to the improper transfer of ions in the body due to the narrowing
of the channels. This gets severe as the body fights with the soaring body temperature as well as
the defect in the channels of ions transport.
