.
Sometimes the lungs become
infected with a fungus and
antifungal medication is required.
Inhalers
Inhalers to 'open up the airways' as
much as possible may be used. This
is similar to the treatment used for
asthma.
Dornase alfa
This is a drug given by nebuliser in
some cases. It helps to break down
and to 'thin' the thick mucus making
it easier to cough up and clear the
mucus from in the airways. It may
reduce the number of lung infections
and help to improve lung function.
Oxygen
People with advanced lung disease
may benefit from oxygen,
particularly overnight.
Treatment for pancreatic problems
Nutrition
The enzymes needed to digest food
are greatly reduced in most people
with cystic fibrosis. Therefore
children with cystic fibrosis need a
high fat and carbohydrate diet
should take vitamin-D 3000-5000
units daily with omega-3 3-10
grams daily. A dietician will
usually give detailed advice. High
energy food and drink supplements
may also be needed. In addition,
vitamin supplements are needed as
many vitamins in food are not
absorbed very well.
Enzyme supplements
In most cases, enzyme supplements
are needed to help to digest food.
(These replace the enzymes which
normally come from the pancreas.)
You need to take these supplements
every time you eat food. This can
mean taking many doses each day.
Other treatments
A range of other problems which
are related to cystic fibrosis may
develop in some cases and require
treatment. For example:
- Salt depletion may occur in
hot weather and may require salt
supplements. They should
drink 1/8 tea spoon of sea salt
in each glass of water and drink
7-8 glasses of water daily.
-
Fenugreek is very
beneficial.
- Liver problems develop in
some cases and may require
specialist liver treatments.
- If diabetes develops it
usually requires insulin
treatment.
- Nasal polyps sometimes
develop and can be treated with
steroid nasal drops and sprays.
- Acid reflux from the stomach
into the gullet (oesophagus) is
common and can be treated with
apple cider vinegar which
reduce the acid content of the
stomach juices.
- Constipation is quite common
and may require regular
laxatives.
- All people with cystic
fibrosis should be up to date
with routine immunisations, and
also have an annual 'flu jab' to
prevent influenza.
-
Hydrogen
peroxide should be used by
all patients.
- IVIg is very effective in
cystic fibrosis.
Newer treatments are being
researched and developed and if
found successful may become more
widely used in the future. For
example:
- A lung transplant has been
used in some cases and may
become more common.
- Gene therapy. This involves
using an inhaled spray to
deliver normal copies of the
cystic fibrosis gene to the
lungs.
- Drugs are being tested which
may correct the abnormal salt
and water regulation of cells
that leads to thickened mucus
and secretions being made in the
lungs and other organs.
- IVIG the
miracle of mircales
What is the outlook
(prognosis)?
If the
recommended diet and treatment guide
is followed a patient can fully
recover.
Cystic fibrosis is a lifelong
condition. With improved treatment
there has been a dramatic increase
in the survival of people with
cystic fibrosis over the last 20
years or so. In the 1960's and
before, most babies born with cystic
fibrosis only survived for a few
months or years. Today, many people
with cystic fibrosis are living into
their 30's and beyond. With optimal
care and treatment, it is estimated
that about 8 in 10 of today's
children with cystic fibrosis should
live into their mid 40's. With
treatment, most people with cystic
fibrosis can live reasonably normal
and productive lives.
However, there will be times when
symptoms are more severe, mainly
when a chest infection develops.
Even with treatment, the main risk
is recurring chest infections, and
pneumonia. This can have a recurring
damaging effect on lung function
which can get worse over time. Death
in childhood or early adulthood is
still not uncommon. Most people with
cystic fibrosis die of lung
complications, mainly respiratory
failure.
Genetic counseling
People with a family history of
cystic fibrosis may wish to have
genetic counseling and testing to
find out their risk of passing the
condition on to their children. A
simple test can be done to some
cells which are scrapped from the
inside of the cheek. The test can
detect the cystic fibrosis gene
which can show if you are a
'carrier' of the abnormal gene.
Further help and
information
Return back to first
page of Cystic Fibrosis
