Respiratory Support in Sheffield
provides information about respiratory assessment,
breathing muscle weakness, Non-Invasive Ventilation (NIV) and
of Breathing Muscles
clinic visits you will be invited to perform one or more
simple breathing tests (see SVC, SNIP, PCF) which help
us to monitor the strength of your breathing muscles. We
will also ask you some questions about your breathing,
some of which may not seem directly connected to your
changes in these can be the result of a change in the
strength of your breathing muscles. Assessing your
breathing regularly allows us to detect changes early
and thus offer treatment as soon as it might be useful
treatment options are available?
Non-Invasive Ventilation (NIV)
of the respiratory muscles can occur just as it does in
other muscle groups (limbs, trunk, throat). Whilst we
cannot stop this weakness we can offer mechanical
support which improves your breathing and quality of
life. This support is usually known as NIV.
NIV is a
way of helping get a little more air in to your lungs as
you breathe to ensure that you fill your lungs every
time you breathe in (inhale) and thus are able to
breathe out more effectively (exhale). This ensures that
you move air in and out of your lungs (ventilate) as
efficiently as possible and achieve appropriate levels
of oxygen and carbon dioxide in your blood. Improved
ventilation should result in an improvement in the
symptoms associated with respiratory weakness.
provided by a small machine which acts like a small
compressor, delivering air under some positive pressure
to you every time you breathe. The air is delivered via
a tube and a mask. The machine is portable (wt 4kg).
to visit our web-based resource myNIV for more
information about NIV (non-invasive ventilation)
would I use the NIV?
Initially we would encourage people to use their NIV
when they sleep. Your breathing is normally reduced when
you sleep so, if your breathing is less strong, your
breathing during sleep will be more affected.
Maintaining effective breathing during sleep with some
mechanical support (NIV) helps improve the quality of
your sleep, is least intrusive and improves how you feel
during the day.
respiratory strength continues to decline some people
will chose to use the NIV during the day as well as at
night time but this varies from person to person. There
is no reason why NIV cannot be used for 24 hours a day
but not everyone wants to do this and we will support
whatever decision you make. Many of the symptoms of
breathlessness can be helped with medication and advice.
How will NIV be introduced if I chose to try it?
would be introduced if overnight sleep studies or your
symptoms suggested a trial of NIV would be beneficial to
you. It can be started in hospital or at home depending
on your circumstances, safety and preferences.
come into hospital you will be admitted to the Neurology
ward where the nursing staff have a lot of experience of
helping people start to use NIV and a Respiratory
Physiologist who will explain how the machine works and
then try masks and settings with you. You will try the
NIV overnight, usually for 1-2 nights before taking it
home to continue using the system. The Respiratory
Physiologist will see you in hospital whilst you are
using it there and then at home. Once established on NIV
the Respiratory Physiologist will visit you at home
regularly and see you in the Outpatient clinic whenever
you attend. A telephone and email helpline is available
for use at any time.
are set up at home the Respiratory Physiologist will do
exactly the same, introducing the machine, masks and
setting s to you at home and then follow you up in the
Is it difficult to use and look after the NIV system?
the NIV machine is very simple once the Respiratory
Physiologist has established the appropriate settings
for you. Usually all you, or a carer, have to do is
switch the machine on and off and be able to put the
mask on and take it off. The settings on the machine are
locked so that you cannot alter them by mistake,
something a lot of people worry about.
Sometimes it is difficult for people to put a mask on
and remove it by themselves if their hand and/or arm
function is reduced. This is taken into consideration
before we decide to introduce NIV and we have a number
of masks to try to find the most appropriate one for
When would the best time to start using NIV be?
varies from person to person as we try to tailor
treatment to the individual.
Experience and evidence from published research suggests
that the best time to introduce NIV is when people are
experiencing the symptoms associated with weakening
breathing muscles and have measurable changes in the
strength of their breathing muscles. This is why we
test your breathing regularly and ask you questions
about your breathing.
Sometimes, however, our tests may show a change in your
breathing but you are unaware of any symptoms. Initially
using NIV is not easy so it is understandable that the
people who may do best initially are those who perceive
a significant improvement in their symptoms following
the introduction of NIV. However many people are not
aware of how much their sleep quality, for example, has
been affected and we would encourage people with
objective measures of respiratory weakness to try NIV
and see if they detect an improvement in how they feel
during the day. Many are pleasantly surprised.
decision to use NIV remains that of the person with MND.
If they really struggle initially we will always offer
respiratory support again at a later date. We also
understand that using NIV takes time so we have no
minimum hours of use requirement. We will support
everyone using NIV with the objective of enabling them
to get the most out of the available ventilatory support
as they can manage.
What are the benefits of NIV?
Research has shown that people with MND and weak
breathing muscles who use NIV regularly have an
improvement in survival and quality of life.
people with MND find that coughing and clearing thick
secretions from their chest becomes difficult if their
respiratory muscles get weaker. If this is becoming a
problem for you we can advise on how best to help thin
these secretions to make it easier for you (of which
there are a number of management options – some which
are mentioned below):
ensuring you drink plenty to keep well hydrated
trying pineapple or other exotic fruit juice (they
contain an enzyme which helps thin secretions)
trying a medication which thins mucous (Mucodyne)
also advise about the use of devices (breath stacking,
mechanical insufflators-exsufflators) which help
stimulate or simulate a cough and, if appropriate,
supply the equipment and train you to use it.
Management of other problems with secretions should be
discussed with the team who can advise you in relation
to your particular problem.
(Slow Vital Capacity): a measure of how much air you can
get in to your lungs. You will be asked to take a big
breath in and then to blow it all out steadily into a
machine via a mask. You will be asked to repeat the test
3 times but with a short rest in between each blow.
(Sniff Nasal Inspiratory Pressure): a way of measuring
how strong your inspiratory (Breathing in muscles)
muscles are. A small probe will be placed in one of
your nostrils and you will be asked to sniff hard. Again
this test will be repeated 5-10 times but you will have
a rest in between each sniff.
(Peak Cough Flow): a measure of how effective your cough
is. You will be asked to take a big breath in and then
cough as hard as you can into a peak flow meter via a
mask. This test will be repeated 3 times with a rest in
between each test.