So, how do we manage cystic fibrosis?
Unfortunately, there is no cure, yet.
Treatment is focused on the management
and on preserving your
patient's lung function.
You can manage a lot of
things with your patient.
The first is their respiratory status.
You're going to practice
airway clearance techniques,
and this is where the patient
will be helped with
postural drainage and percussion.
They'll go under chest
physiotherapy and treatments
to help shake up those secretions.
The patient's also going to
be taking inhaled medications
and this is going to help
preserve their lung function.
Your patient's also going to take
antibiotics to prevent infections,
such as pneumonia, that can further
compromise their respiratory status.
And there's new medications.
These are the CFTR modulators.
Regarding the management of your
patient's nutritional status,
they're going to need to take
pancreatic enzyme supplements
and this is because the pancreas
gets clogged with this mucus
and is not able to release these.
The patient's going to need to take
pancreatic enzyme supplementation
with every fat-containing meal and snack,
and these are active for 45-60 minutes.
Your patients with cystic fibrosis
are also going to be put on
a high-calorie, high-fat diet because
they require a lot more energy
just to breathe and fight
their lung infections.
The energy needs of people with
cystic fibrosis are estimated to be
1 ½ to 2 times the needs of
those without cystic fibrosis.
So, a high-calorie diet, high fat,
and about 40% of their total calories
from fat is generally recommended.
The next involves preventing infection.
This involves hand hygiene, also avoiding
secondhand smoke, avoiding sick contacts.
Patients with cystic fibrosis
should get their annual flu shot,
and eventually, the patient
may need a lung transplant.
Lung transplantation is the only
definitive treatment option for patients
with cystic fibrosis with
end-stage lung disease,
but it's still not a cure,
because the defective gene
that causes the disease is found in all
of the cells of the patient's body,
with the exception of their
newly transplanted lung.
Here, on the left, you'll see a diseased lung
being removed in a patient
from cystic fibrosis.
On the right, you'll see the
new fresh donated lung
being transplanted into the recipient.