journey Cystic Fibrosis Victoria Adult News, Edition 12, 2006
Live on, live strong in this issue …
by Wood Fitzpatrick FEATURES
All about Pneumothorax
Living with CF is an all- Hypertonic Saline Solution - Mark Elkins
consuming thing. In fact it is so
involving that when I dream of NEWS
being in someone else’s shoes, Introducing the new Support Team
I still have CF. It’s a kind of
New Respite Program launched
constant ‘companion’, driving
me to live a life that is full and Managing health outside CF Centres
exciting. Hospital visits during the Games
My name is Wood Fitzpatrick, Alfred Parking concessions
formerly Edward but evolution
has reared its beautiful head. I PROFILE
am one of two children and CF Front: Wood Fitzpatrick (CF)
has never been in my family
tree. Born in Central North
Queensland I had the unfortunate surfed most of the breaks.
luck of starting my life in a small I also love snow skiing and I’m
coal mining town where I did not currently planning my first overseas
endure life in the town or school trip to celebrate my 30th birthday
very well. I was able to leave with a ski holiday in New Zealand. A
(thankfully) at the ripe age of 14 few other favourite things I like is live
years. That is really when my life music and theatre.
began. —and decided to settle here. I have been Having CF actually drives me
I always had the desire to see as here seven and a half years and can’t to lead a fuller life. I am fortunate
much of my own country as possible see myself ever leaving. enough to have good lung function
before travelling abroad. I set out on a I have many passions in life and and my active lifestyle keeps me fit and
journey to backpack aound Australia being a fast-paced and loud guy, my moving. CF is a lot of hard work, but
from top to bottom. Not really having passions generally revolve around it has also made me grow up faster to
any particular career in mind I fell extreme sports. I own a dirt motorcycle deal with the massive responsibility of
into hospitality. It suited my social on which I’ve scaled hundreds of looking after my own health. It makes
personality (I’m a Leo) and I really mountains and soared over thousand of me live in the ‘here and now’ instead
loved the lifestyle that came with it. jumps, big and small. There is no cooler of in the past or future, and that’s an
After eight years of travelling down thing than popping a wheelie! attitude towards life that plenty of
the east coast of Australia, I discovered I still surf regularly and miss the people aren’t lucky enough to have.
the best place on earth—Melbourne east coast sea air where I lived and
Your support team is here to help
In December last year Wendy CFV provides many services to
Collins, Community Services Manager, adults with CF including regular
and Sarah Gliddon, Community Support newsletters, free hospital TV service,
Worker joined CFV just in time to adult respite program, CF scholarship
handle the Christmas hamper rush. program, laptop loan service and
Wendy has recently moved from discounted gym memberships.
NZ and brings a wealth of social work Next time you are being admitted to
experience to her role in heading the The Alfred or Monash Medical Centre
CFV support team. Sarah’s experience request a CFV Notification Form which
in disability services and her post- will notify Sarah and Wendy of your
graduate qualifications in genetic admission and let them know how they
counseling also make her a valuable can help you.
addition to the support team. CFV support team Sarah Gliddon and Wendy Collins.
CFV - Enhancing the lives and meeting the changing needs of Victoria’s Cystic Fibrosis Community
What is Pneumothorax?
pneumothorax occurs when injury such as a knife wound or a car may require an immediate chest-tube
air gets into the space accident can result in a pneumothorax. insertion to allow the air to be released
between the lung and the Medical procedures such as and the lung to re-expand.
membrane that covers it when small transbronchial biopsy, thoracentesis, The only known way of preventing
breaks occur in the lung wall. and chest tube insertion can all increase all pneumothorax is to reduce the risk
Pneumothorax is the rupture of the the risk of a pneumothorax. by not smoking.
air-filled sacs (blebs) on the lung which Approximately three out of 1,000 Recurrent cases of pneumothoraces
results in air escaping from the lung individuals are affected by traumatic may require surgery to eliminate the
and entering the chest cavity, causing pneumothorax each year. chance of having future episodes.
the lung to collapse. Depending on Both tension and traumatic Recurrent cases of spontaneous
the severity of the incident, various pneumothoraces can be a life- pneumothorax may become more
symptoms will be experienced within threatening emergency. serious and an x-ray is the only reliable
minutes of the initial collapse. method in determining the severity of
The reason why spontaneous Prognosis
Symptoms include sudden chest pneumothorax occurs is unclear,
pain, shortness of breath, chest although it has been attributed to tall, Unfortunately a pneumothorax
tightness, easy fatigue, rapid heart thin men (between 20 and 40 years has a very high rate of recurrence.
rate, bluish color of the skin caused by of age), smokers, and in some cases, As many as 50% of patients with
lack of oxygen, pain in the arm and a women during their menstrual cycles. a pneumothorax may experience
stabbing sensation in the back. Causes of a tension or traumatic recurrence, (with tension pneumothorax
Pneumothorax is detected using a pneumothorax differ depending on it depends on the treatment.)
stethoscope; a doctor may be able to each situation. A penetrating trauma to Luckily there are no long-term
determine decreased breathing sounds the chest, lung infection, or a recurrent complications once successful therapy
and a possible collapse but tests to prior pneumothorax may all lead to a has been completed.
diagnose the type of pneumothorax are pneumothorax.
usually undertaken with a chest x-ray
and a test on arterial blood gases. What you can do
Treatment and prevention
Spontaneous pneumothorax – call
Types of Pneumothorax The goal in the treatment of your local doctor or hospital.
pneumothorax is to remove the air from Tension or Traumatic pneumothorax
Pneumothorax is classified into the pleural space so that the lung can – Dial 000 Emergency and request an
three main types: spontaneous, tension re-expand. For small pneumothoraces, ambulance immediately.
and traumatic pneumothorax. the body may absorb the excess air
Spontaneous pneumothorax has on its own. In more serious cases such Reference: http://www.pneumothorax.org/
two classifications of its own - Primary as a tension pneumothorax, patients pneumo.nsf/index.html
and Secondary. As with all other types
of pneumothorax, primary spontaneous
pneumothorax occurs without any prior
lung disorders or diseases. Secondary
pneumothorax can occur as a Some facts about Pneumothorax and CF:
complication of underlying lung disease
such as cystic fibrosis. • Spontaneous pneumothorax is a common complication in CF
Tension pneumothorax is a severe
pneumothorax and the lung collapse patients, more likely to occur later in the course of the disease
is usually more than 50%. The cause • The first case of pneumothorax in CF was reported in 1966
of this type of pneumothorax can
be a traumatic event, or a severe • Spontaneous pneumothorax occurs more frequently in older
spontaneous pneumothorax. It differs
from spontaneous pneumothrax in
that the collapse is much more severe • Incidence of spontaneous pneumothorax in CF patients is 6.4%
and can cause many serious medical
problems. • The youngest age of initial pneumothorax was 4 years old, however
As the air escapes from the lung the mean age of occurance is typically in the mid-teens
it enters the pleural space, increasing
pressure in the area. This can cause the • Both men and women are equally at risk of pneumothorax
heart, trachea, and great vessels to be • Pneumothoraces can occur in either lung
pushed towards the unaffected side of
the chest. • Smoking is a known risk factor for primary spontaneous
Traumatic pneumothorax occurs pneumothorax
when a physical injury results in a
puncture wound to the lung, causing
the lung to collapse. Any physical
Aussie surfers give
researchers a clue to
improving CF treatment by Mark Elkins
ustralian researchers observed signs of improvement or any possible Participants’ lungs were also
Aussie surfers with CF had adverse effects. assessed very closely for signs of more
fewer lung exacerbations inflammation, but no evidence was
than those not exposed regularly to salt found. The use of Hypertonic Saline did
water. This inspired US and Australian What did the trial show? not affect which bacteria were found in
researchers to investigate further. Two The main outcome of the trial was a the lungs, nor their concentration in the
studies on the findings of the effect modest rise in lung function after four mucus.
of Hypertonic Saline (salt water) on weeks. This continued until the last trial
patients with CF were published early
this year in the New England Journal of
test at 48 weeks. Subjects had far fewer Is Hypertonic Saline
flare-ups and needed significantly fewer
Medicine. The findings established that
Hypertonic Saline inhalation is a valid
“extra” antibiotics to manage their suitable for any other
treatment which can reduce infection
Among other benefits reported were
and improve the overall quality of life improved attendance at school, work or
for people with CF. There is a lung disease called
other usual activities and feeling that bronchiectasis, which also causes
overall health and quality of life was chronic infection in the lung with acute
About Hypertonic Saline better. flare-ups. One short-term trial has
shown that Hypertonic Saline can be
Hypertonic Saline is simply a
strong, sterile, salt-water solution of Were there any adverse helpful in peope with bronchiectaasis,
but no long-term trial has been
about 7% salt (about twice as salty
as seawater); sometimes weaker or
There is no evidence that Hypertonic
stronger concentrations are used. Sterile Hypertonic Saline can cause the Saline is beneficial for other lung
Hypertonic Saline can be turned into airways to narrow – just like they diseases. Most other lung diseases don’t
a mist by a simple nebuliser for lung can do in asthma. In the National cause infections and many cause airway
inhalation. Hypertonic Saline Trial, all subjects narrowing (such as asthma, chronic
took a drug to open their airways, obstructive pulmonary disease (COPD),
called a bronchodilator just before
How does it help in CF? each Hypertonic Saline dose. This was
and emphysema). In these conditions
Hypertonic Saline would not be helpful
When Hypertonic Saline is inhaled very effective in limiting the amount and could very well be detrimental.
into the lungs, the body tries to dilute of airway narrowing. For this reason,
it is strongly recommended that people
the strong salt concentration. The cells
lining the airways are triggered to with CF take a bronchodilator before I have CF. How can I get
release water. This restores the layer of each dose of Hypertonic Saline, and are
supervised when they inhale their first
some Hypertonic Saline?
moisture lining the airways, thinning
the mucus. This helps the mucus to be dose of Hypertonic Saline. Although there is no commercial
more easily cleared, the same way it Lung function should be measured manufacturer of Hypertonic Saline most
does in healthy lungs. You could think before and after the first dose, to hospitals with large CF Clinics were
of it as the lungs “flushing out” the check that the bronchodilator is strong involved in the National Hypertonic
stagnant, infected mucus. enough to prevent significant airway Saline Trial, and therefore have a
Studies undertaken during the mid narrowing. supply already available.
1990s showed that inhaling nebulised More coughing during and Do not try to mix up your own
Hypertonic Saline temporarily increases immediately after inhalations is normal solution of salt and water. You will
the speed at which mucus is cleared but this should settle after the first few not be able to make a sterile solution
from the lungs. Subsequent short-term doses, or at most, after a few weeks. A and you may not prepare the correct
trials showed that inhaling nebulised lower concentration of saline may be concentration.
Hypertonic Saline on a regular basis more tolerable if coughing persists. For more information or to discuss
(usually twice per day) improves lung Some people in the trial found the your interest in trying Hypertonic
function in people with CF. taste of the saline difficult to tolerate. Saline contact your CF treating Doctor
The recent trial tested 164 stable However, the saline had a bitter who will organise a ‘tolerance test’ for
subjects with CF aged over six years taste-masking agent which was only you to monitor any possible hyper-
twice daily for 48 weeks who inhaled needed for the trial. The hypertonic reactivity to the Hypertonic Saline.
Hypertonic Saline. They continued saline you would receive from your CF The tolerance test will rule out any
all their other standard therapies and centre would taste much better. Not sensitivity to Hypertonic Saline before
received whatever additional treatment surprisingly, some people still find the you can be administered a prescription
they normally would receive. taste quite salty but not that difficult to which will be filled by your treating
Patients were checked regularly for tolerate. hospital pharmacy.
As the Commonwealth Games rapidly
approach we have been thinking about our
members in country Victoria and how we can
help you get around any inconveniences of
any pending travel to clinic visits.
Pictured location: The Church Accommodation, Dayelsford.
As you may be aware a number of hotels,
motels, serviced apartments and hostels in
Melbourne are fully booked over this time.
Those that aren’t are charging premium
prices for accommodation. CFV will make
every attempt to book accommodation for
clinic visits and hospitalisations over this
period. Unfortunately, it may be necessary
CFV has recently launched our new Adult Respite Program. Funded through to book this some distance from the hospital.
the generous support of Trip of a Lifetime, each respite package includes one While it is unlikely, it is possible that
night’s accommodation and breakfast the following morning. accommodation may not be available over
The respite packages allow a short stay at one of a range of locations the period of the games. Also be aware many
around Victoria to help reduce stress levels and take a break from your normal of Melbourne’s streets will be closed during
routine. Locations include Queenscliff, Daylesford, the Grampians, Echuca, St this time.
Kilda and the Dandenong Ranges.
To be eligible, adults with CF must be members of CFV and submit an Neither The Alfred nor Monash Medical
application to Wendy Collins at CFV. Some conditions apply but you will be Centre have plans to change clinic
able to access the respite program at least once a year. Exceptions may be arrangements.
made depending on your individual circumstances.
For further information or to discuss a booking please contact either If you are from out-of-town try to reschedule
Wendy or Sarah on 03 9686 1811. your appointments (if possible) to the weeks
prior or after the Commonwealth Games. If
you do decide that it is necessary to attend
Consult your CF Care Centre first your existing appointments and require
assistance with accommodation please let us
know as soon as possible.
Many people with CF have incidental medical problems and may seek
advice from medical practitioners outside of their usual CF Care Centre.
Examples where this may happen includes abdominal pain, constipation,
impacted wisdom teeth and dizziness in hot weather. Parking at The Alfred
Occasionally, these problems may require investigation by doctors in other
hospitals and result in a surgical referral. Unless doctors in other centres or Patients attending CF clinic at The Alfred as
clinics are aware of patients having cystic fibrosis, decisions may be made either an in-patient or outpatient may need
that would be appropriate for the general community, but could have severe to use the car parking facilities. To ensure
consequences for those with CF. you receive a discounted rate of $10 you can
If you find yourself needing medical assistance you should ask your contact CF Coordinator, Felicity Finlayson
treating doctor to call your CF Care Centre prior to initiating investigations or on 9276 3443 prior to your appointment or
therapy. Qualified and experienced CF staff can then discuss relevant medical speak to Tracey Schutt, the Nurse Manager
problems that patients with CF experience with your doctor prior to treatment. who can complete the relevant paperwork.
Ideally you should contact your usual CF Care Centre in the first instance Some people may be eligible for the Special
before seeking help elsewhere unless in a medical emergency. For further Circumstances rate ($6). This is a social work
information about the policy regarding medical management outside CF care mediated process and patients will need to
centres you should contact your usual medical care team. A clear treatment contact Sharon Sutherland, the CF social
plan with contact details will often prevent unexpected difficulties during worker, to request this rate.
Publication disclaimer: Cystic Fibrosis Victoria Inc.
This newsletter is designed and produced by Cystic Fibrosis Victoria Inc. Material and opinions 80 Dodds Street, Southbank, VIC 3006
expressed in this publication may not necessarily reflect those of Cystic Fibrosis Victoria. Edited by Phone (03) 9686 1811 Fax (03) 9686 3437
Kerryn McIver, Communications Manager, firstname.lastname@example.org Email email@example.com Website www.cfv.org.au