There are a lot of complications
in patients with cystic fibrosis.
In the respiratory realm, this involves
infective exacerbations of bronchiectasis,
and this is a widening of
the airways in the lungs
caused by chronic inflammation
and obstruction of the airways.
The patients may also get a spontaneous
pneumothorax, or a collapsed lung.
The patients may have hemoptysis
and may cough up blood because,
over time, cystic fibrosis can cause
thinning of the airway walls.
As a result, teenagers and adults with
cystic fibrosis may cough up blood.
And this can also be caused
by an infection in the lungs
that's going to irritate the small
blood vessels causing them to bleed.
The blood is going to
mix in with the sputum
and then this will be
expectorated or coughed out.
This can be quite a frightening
symptom for patients,
but usually, the blood loss is minimal.
Patients may also have polyps
and this is going to further
obstruct their nasal passages
and increase their risk for sinus infection.
The patient may go into respiratory failure.
They may develop cor pulmonale, and
this is right-sided heart failure
that develops due to complications
from the high blood pressure
in the pulmonary arteries, also
known as pulmonary hypertension.
Their right ventricle will slowly
enlarge and become less effective,
and then ultimately, it will fail.
The patient may also have lobar
lung collapse due to secretions.
IV antibiotics, physiotherapy, and
the use of mucokinetic agents
to get that mucus moving can
allow that lobe to re-expand.
The patient will also have
complications in their GI tract.
This includes malabsorption and
steatorrhea due to the mucus
blocking the effective use
of their digestive enzymes.
The body is going to
have a really hard time
absorbing protein, fats,
and fat soluble vitamins.
Steatorrhea refers to the fatty stools
and it's the result of
the impaired digestion
and the absorption of the dietary fats.
The patient can also develop a distal
intestinal obstruction syndrome,
and this involves blockage of the
intestines by thickened stool
where the small intestine
meets the large intestine.
Now this differs from constipation,
and that the backup of stool
is higher in the intestines
and it can be the result of not taking
enough of their digestive enzymes.
The patient can also
develop biliary cirrhosis
and portal hypertension and gallstones
because the duct that carries the bile
from the liver in the gallbladder
to your small intestine will also
become blocked and inflamed,
leading to liver problems and gallstones.
There are other complications in
patients with cystic fibrosis.
Patients can develop diabetes
and this happens in about 25% of
adult patients with cystic fibrosis.
It's called cystic
fibrosis-related diabetes or CFRD,
and these share some clinical features
with both types 1 and types 2.
The thick mucus is going to
cause scarring in the pancreas
and the pancreas can
still make some insulin,
but not enough to be totally effective.
Patients will also experience
Male patients with cystic fibrosis
may experience infertility
because the vas deferens,
which is the tube that connects
the testicles to the prostate,
can become blocked with mucus
or congenitally absent.
With the use of certain fertility
treatments and procedures,
it's possible for some men with cystic
fibrosis to become biological fathers.
The patients may experience
due to their forced repeated coughing.
Patients with cystic fibrosis can also
develop psychological problems.
Remember, this is a chronic illness
and there's an emotional burden
associated with this disease.
Cystic fibrosis involves a
stressful routine of treatments,
and anxiety and depression are not uncommon.
The patient may also develop osteoporosis
and this is decreased bone density,
and this happens for a few reasons
in patients with cystic fibrosis.
The first is malabsorption of the nutrients
necessary to keep your bones strong.
The second is recurrent lung infections.
This is going to cause inflammation
and that can interfere with the
body's natural process of forming
new bones to replace old, damaged bones.
The third is the decreased weight-bearing
exercises due to exercise intolerance.
And the fourth, certain medications
taken to manage the condition
and its complications can affect
the patient's bone health.