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⚄ 2.1.2.1.1 Breathing issues in neuromuscular diseases
⚄ 2.1.2.1.2 Breathing issues in IBM
⚄ 2.1.2.1.4 Overview of respiration
⚃ 2.1.2.2. Respiratory testing.
⚃ 2.1.2.3. Supplementing breathing.
⚄ 2.1.2.3.1 Techniques to increase lung volume
⚄ 2.1.2.3.2 Manual Lung Volume Recruitment (LVR)
⚄ 2.1.2.3.3 CPAP and BiPAP (Nighttime)
⚄ 2.1.2.3.4 Portable ventilation (Daytime)
⚃ 2.1.2.5. Pneumonia vaccination.
⚃ 2.1.2.6. Specific IBM research.
In many cases, IBM causes weakness in the major muscle involved
in breathing, the diaphragm.
≻ In likely 20% of cases, this weakness will be severe enough to
interfere with normal breathing and require medical intervention.
≻ This complication can usually be well-managed if it is
identified.
⚃ 2.1.2.1. Key Points.
⚄ Research indicates that weakness of the diaphragm or other respiratory problems are more common in IBM cases than once appreciated.
⚄ Research has found a high frequency of obstructive sleep apnea (OSA) in patients with inflammatory myopathies.
⚄ Recent research found a higher than expected proportion of diaphragm abnormalities in patients with inflammatory myopathies even though they did not display gross lung involvement or have obvious respiratory complaints.
⚄ Although only a few cases have been documented in the
literature,
≻ I have recently been contacted by several people diagnosed with
IBM who are currently using Bipap machines to address respiratory
insufficiency.
⚄ It seems prudent for IBM patients to have respiratory testing to establish a baseline of function and to disclose any current issues.
⚄ Early management of respiratory issues is important, by the time symptoms become noticeable, major impairment may have already occurred.
⚄ The routine use of manual lung volume recruitment (see 3a) would appear to have many benefits for the average IBM case and especially for those people in wheelchairs whose respiration may also be affected by postural issues.
⚄ Respiratory infections and pneumonia appear to be a major concern for IBM patients, regular use of lung volume recruitment is a prophylactic and may reduce the impact and severity of respiratory infections.
⚄ IBM patients should consider vaccination for pneumonia (see 4 below).
⚄ In cases where there is diaphragmatic weakness, the use of a mechanical breathing supplementation may be necessary (see 3b).
⚄ Paradoxical breathing is a symptom of the weak diaphragm.
≻ Here is a
web article discussing paradoxical breathing
(pdf).
⚄ 2.1.2.1.1 Breathing issues and neuromuscular diseases.
⚅ The most common cause of death in neuromuscular diseases
is chronic respiratory failure.
≻ The weakness of the diaphragm, intercostal muscles, and
accessory respiratory muscles cause respiratory insufficiency and
diminishes ventilation.
≻ This type of insufficiency is known as type 2 respiratory
failure or failure of the respiratory pump.
≻ It differs from type 1 hypoxic respiratory failure caused by
various lung diseases.
⚅ Neuromuscular weakness causing respiratory failure is usually nonspecific, chronic, and insidious in presentation.
⚅ Reduced ventilation and ineffective cough from the weakness of respiratory, pharyngeal, and laryngeal muscles compromises the airway clearance and predisposes these patients to recurrent pneumonia and premature death.
⚅ Noninvasive ventilation with airway clearance therapy is
considered standard practice for patients with neuromuscular respiratory
failure (see 3.2 below).
≻ The major goal of noninvasive ventilation is to ensure adequate
ventilation.
⚅ Some patients need full-time ventilation and mouthpiece
ventilation when awake (see 3.3 below).
≻ An angled mouth-piece or straw-type mouthpiece is placed near
the mouth so that patients can trigger a breath by creating a small
negative pressure (sip ventilation).
≻ Mounting the ventilator on a wheelchair and suspending the
mouthpiece on a gooseneck stand allows the patient to remain mobile and
still have access to positive pressure as needed.
⚅ Although the patient's neuromuscular disease might not
present with respiratory failure in the initial encounter, they are at
high risk of developing one.
≻ Hence, prompt and timely screening is mandatory to identify
patients that will benefit from NIV, as supportive respiratory therapies
have proven to improve the quality of life when initiated promptly.
≻ Follow-up rate must be determined based on the established rate
of progression of respiratory failure.
⚅ Reference: Ang, J. F. & Digala, L. P. (2020). Neuromuscular respiratory weakness. In N. Arora et al. (Eds.), Neuromuscular Urgencies and Emergencies , pp. 15-21. Springer.
⚄ 2.1.2.1.2 Breathing issues and IBM.
⚅
Incidence:
Diaphragmatic involvement and respiratory issues have not been an issue
highlighted in IBM research.
≻ However, recent research has drawn into question the incidence
of respiratory related weakness in the inflammatory myopathies (PM, DM,
IBM).
≻ Oldfors and Lindberg (2005) cite Teixeira et al, (2005) and
report "weakness of the diaphragm is probably an underdiagnosed
manifestation of inflammatory myopathies, including s-IBM."
≻ Subacute respiratory failure was reported in one patient with
s-IBM who required mechanical ventilation (Voermans, et al, 2004).[See
relevant research presented below]
⚅ In the Shelley (2021) study, diaphragm weakness was diagnosed in 16% of IBM patients, developing some five years after symptom onset.
⚅ Anecdotal evidence supports the finding that some IBM patients present with severe respiratory problems.
⚅ There are several things that could contribute to poor
respiration and IBM cases, primarily and of most concern, direct
weakness of the diaphragm or the chest muscles reducing the volume of
air moved in and out of the lungs.
≻ In addition, poor posture both sitting in wheelchairs and in
bed, laying in one position all-night, can constrict the chest and
lungs.
≻ Finally, there could be an impact from some of the medications
that some IBM patients take.
⚄ 2.1.2.1.3 Neuromuscular disorders and sleep:
⚅ Quotations taken from article: Diaphragm muscle weakness
becomes specifically manifest during REM sleep, when the diaphragm is,
under normal circumstances, the only effective muscle pump. Patients who
have diaphragm paralysis cannot breathe while supine, even in the awake
state. Lesser forms of diaphragm muscle weakness become apparent during
REM sleep, particularly if patients lie supine. Individuals who have
neuromuscular disease and diaphragm involvement exhibit the greatest
oxygen desaturations in REM sleep, so that this stage becomes a test of
diaphragm muscle function. … Patients who have neuromuscular
disease may have restrictive lung disorder as a consequence of chest
wall muscle weakness, scoliosis, and pulmonary microatelectases that
result from chronic hypoventilation, repeated episodes of aspiration,
and retained secretions. … Sedentarism in patients who have
altered muscular function promotes obesity, another factor that burdens
ventilatory efficiency during sleep. Obese patients suffer mechanical
reduction of intercostal muscle function, and subjects who have
abdominal obesity exhibit marked diaphragm dysfunction that is
particularly evident during REM sleep.… Patients who have
neuromuscular disorders are at high risk for the development of
sleep-related respiratory disorders and respiratory failure. A variety
of concurring abnormalities converge in patients who have neuromuscular
disorder that explain their vulnerable status. Diaphragm weakness and
failure is the most important determinant of sleep-related respiratory
insufficiency. … Noninvasive positive airway ventilation has
revolutionized the treatment of most nocturnal respiratory abnormalities
found in patients who have neuromuscular disorder. Positive pressure
breathing corrects obstructive sleep apnea, improves hypoventilation,
and assists diaphragm failure.
≻ From: Culebras, Antonio (2005) Sleep and Neuromuscular
Disorders. Neurol Clin 23,1209 1223.
⚄ 2.1.2.1.4 General overview of respiration.
⚅ Air is breathed in through the nose and mouth.
≻ The air travels down through the trachea then through large and
small tubes in your lungs called bronchial tubes.
≻ The airways (various tubes or passages) to your lungs look
something like an upside-down tree with many branches.
≻ At the ends of the small bronchial tubes, there are groups of
tiny air sacs called alveoli.
≻ The alveoli have very thin walls, and small blood vessels called
capillaries run in the walls.
≻ If stretched out flat, the surface area of the lung would equal
that of a tennis court.
≻ Oxygen passes from the alveoli into the blood in these small
blood vessels.
≻ At the same time, carbon dioxide passes from the blood into the
alveoli. Carbon dioxide, a normal byproduct of the body's metabolism,
must be removed from the blood.
⚅ Breathing is an automatic function controlled by the
brain.
≻ A complex feedback mechanism tells the brain how to breathe and
how fast, for example, after exercise, we breathe faster and deeper.
≻ This control is accomplished through the levels of gases in the
blood including the amount of oxygen and carbon dioxide.
≻ Abnormal levels can disrupt brain control of breathing leading
to problems.
⚅ Thus, the two critical aspects of lung function are
getting enough air in and out of the lung and having a proper and
effective exchange of oxygen and carbon dioxide in the alveoli of the
lung itself.
≻ IBM could affect the amount of air going in and out.
⚅ Oxygen and carbon dioxide play important but different
rules in respiration.
≻ Although the body requires oxygen for metabolism, low oxygen
levels do not stimulate breathing.
≻ Instead, breathing is stimulated by higher carbon dioxide
levels.
≻ Sensory organs in the brain, aorta and carotid arteries monitor
the blood and sense oxygen and carbon dioxide levels.
≻ Normally, an increased concentration of carbon dioxide is the
strongest stimulus to breathe more deeply and more frequently.
≻ Conversely, when the carbon dioxide concentration in the blood
is low, the brain decreases the frequency and depth of breaths.
≻ A person's exhaled breath is approximately 4.5% carbon dioxide.
≻ A person's breathing rate influences the level of CO2 in their
blood.
≻ When respiration decreases, CO2 levels in the blood increase;
likewise, excess levels of CO2 in the blood stimulate breathing to
increase in frequency and become deeper.
≻ Breathing that is too slow, or shallow can cause a serious
symptom called
respiratory acidosis,
while breathing that is too rapid may lead to hyperventilation,
which may cause the reverse problem, respiratory alkalosis.
≻ Respiratory acidosis is acidosis (abnormal acidity of the blood)
due to decreased ventilation of the pulmonary alveoli, leading to
elevated arterial carbon dioxide concentration (PaCO2).
≻ Initially, acidosis can be experienced as drowsiness or a
headache, but it is a serious symptom leading to coma.
≻ Chronic respiratory acidosis may be caused by neuromuscular
disorders that reduce airflow volume.
⚅ Above based on: http://lungdiseases.about.com/od/termsdefinitions/f/howlungswork.htm (now defunct)
⚅ When the diaphragm contracts, it moves down, the chest
cavity enlarges, reducing the pressure outside the lungs.
≻ To equalize the pressure, air enters the lungs. When the
diaphragm relaxes and moves back up, the elasticity of the lungs and
chest wall pushes air out of the lungs.
≻ When laying on one's back, the normal respiratory function of
the diaphragm can easily be observed; as the diaphragm moves down, it
puts pressure on the abdomen, making the abdomen rise.
≻ Thus, as air enters the lungs, the chest and abdomen rise
together;
≻ If the diaphragm is not functioning normally, the chest and
stomach fall together on an exhalation paradoxical breathing (see below)
can be observed.
≻ As the chest rises on inhalation, the stomach falls as the
diaphragm is not expanding down into the abdominal cavity. From:
http://www.merck.com/mmhe/sec04/ch038/ch038e.html
⚄ 2.1.2.1.5 There are two main categories of lung problems as follows:
⚅ 1. Obstructive Lung Disorders:
≻ Essentially, airflow is obstructed from smoothly going in and
out of the lungs.
≻ Characterized by a limitation of expiratory airflow, airways
cannot empty as rapidly as normal (such as through narrowed airways from
bronchospasm, inflammation, etc.).
≻ Examples: Asthma, Emphysema, Cystic Fibrosis.
⚅ 2. Restrictive Lung Disorders:
≻ Essentially, there is nothing in the way, but for some reason,
the normal volume of air is restricted and does not reach the lungs
–
chronic respiratory insufficiency.
≻ Characterized by reduced lung volumes/decreased lung compliance
(this is where IBM would fall).
≻ Examples: Interstitial Fibrosis, Scoliosis, Obesity, Lung
Resection,
Neuromuscular diseases,
Cystic Fibrosis
⚅
Airflow:
Air flows to and from the alveoli as lungs inflate and deflate during
each respiratory cycle.
≻ Lung inflation is accomplished by contracting respiratory,
diaphragmatic, and external intercostal muscles, whereas deflation is
passive.
≻ So, on inhalation, the diaphragm contracts (it is a muscle) and
moves downward, creating negative pressure (a vacuum) in the chest
cavity and allowing the lungs to inflate (sucking air in); this can be
easily seen as the chest and abdomen expand outwards together as the air
goes in.
≻ On exhalation, the diaphragm relaxes and moves up as the air
leaves the lungs, the chest and stomach fall together.
⚅ FRC is the volume of air in the lungs when the respiratory
muscles are fully relaxed, and no airflow is present.
≻ The volume of FRC is determined by the balance of the lungs'
inward elastic recoil and the chest wall's outward elastic recoil.
≻ Restrictive lung diseases are characterized by a reduction in
FRC and other lung volumes.
⚅
Reduced air volume:
The main feature of restrictive disorders is reduced breathing volume.
≻ This means less air coming into and going out of your lungs.
There can be two effects, not enough oxygen received and not enough
carbon dioxide expelled out.
≻ As mentioned above, this type of problem often occurs at night
and often disturbs your sleep.
≻ It can cause insomnia or excessive daytime sleepiness.
≻ It can also cause you to have a headache when you wake up.
≻ Low oxygen levels at night can contribute to heart problems.
≻ These problems include heart failure and high blood pressure.
≻ Low oxygen levels are more common during the REM stage of sleep.
⚅ Neuromuscular disorders affect an integral part of the
respiratory system; a vital pump made up of the chest wall, pleura, and
respiratory muscles.
≻ The respiratory pump can be impaired at several levels,
including problems in the central nervous system, spinal cord,
peripheral nervous system, neuromuscular junction, or respiratory
muscle.
≻ The pattern of ventilatory impairment is highly dependent on the
specific neuromuscular disease.
⚅ In the case of IBM, it is the possible effect on the
diaphragm or the intercostal muscles that are of most concern.
≻ A weak diaphragm is similar to the picture in amyotrophic
lateral sclerosis in practical, clinical terms can be seen as
paradoxical breathing, explained below.
≻ Sleeping on one's that back tends to worsen these symptoms.
⚅
Paradoxical breathing:
In healthy breathing, the chest and abdomen expand out together on
inhalation.
≻ When people have paradoxical breathing, they physically reverse
the normal breathing process.
≻ With the person laying down, this is easily seen as the weak
diaphragm is sucked up on the in-breath, causing a noticeable lowering
of the abdomen on inhalation – the chest expands while the abdomen
falls.
≻ Paradoxical breathing is common in patients with sleep apnea and
is a sign of diaphragmatic weakening.
⚅
Common symptoms of restrictive disorders:
Initially, symptoms may be relatively minor especially in the case of a
slowly developing weakness.
≻ Symptoms are usually noticed initially at night, often by
disturbed sleep and shallow breathing.
≻ Headaches upon awakening may be a sign of increased carbon
dioxide levels.
≻ Daytime tiredness or falling asleep easily during the day are
also indicators of possible nighttime respiratory problems.
⚃ 2.1.2.2. Respiratory testing.
⚄
Baseline breathing measurements:
In my opinion, anyone with a diagnosis of IBM should ask their physician
for a referral to a respirologist for a breathing analysis.
≻ This usually entails taking a sample of arterial blood to
analyze and a computer to take measurements as the patient blows in and
out of a tube.
≻ This measures a number of different aspects of breathing and can
easily determine if there are problems with the volume of air going in
and out.
⚄ Baseline measurements can be helpful if breathing problems develop later on, as subsequent tests can be compared to the initial baseline to gauge how much of a decline has taken place.
⚄ The actual numbers derived from the tests are a general
indicator and may be related to symptoms or not – some people with
low numbers have few symptoms, some people with better numbers may have
a lot of symptoms.
≻ Again, these tests are important in helping the doctors
determine how best to manage your particular situation.
⚄ The spirometry test is performed using a device called a
spirometer.
≻ Most spirometers measure the volume and speed of airflow and
display the following graphs that summarize respiratory function.
⚄ These tests are very simple to take and take about 20 minutes to administer.
⚄ If more information is needed, a patient may have to go to
a sleep lab for further diagnosis.
≻ For example, a
polysomnogram,
a complicated test that measures 16-24 variables recorded during an
overnight stay, may be administered to help diagnose respiratory
problems during sleep.
≻ An expert technician monitors the sleeping patient during the
procedure.
⚃ 2.1.2.3. Supplementing breathing.
⚄ 2.1.2.3.1 Techniques to increase lung volume.
⚅ There have been several different techniques used to try to
increase lung volume.
≻ In the old days,
negative pressure
was used to create a vacuum in the lungs to assist inhalation by making
it easier to breathe in.
≻ Most people will remember the image of an
iron lung.
≻ These techniques using negative pressure are seldom used today.
⚅ Techniques using positive pressure to breath air into the lung have a long history;
⚅⚀ And the Lord God formed man of the dust of the
ground
and breathed into his nostrils the breathe of life,
and man became a living soul.
- Genesis
⚄ 2.1.2.3.2 Manual Lung Volume Recruitment (LVR):
⚅ This procedure is done with a fairly simple piece of
equipment called a bag valve resuscitator.
≻ The patient puts the end of the hose in his or her mouth, draws
a full breath and then the bag is squeezed, either by the patient or by
an assistant, introducing more air into the lungs.
≻ Three compressions usually constitute one treatment, three or
four treatments are done three or four times a day.
≻ The patient may feel some minor discomfort during the inflation.
≻ This procedure forces the lungs to deeply inflate.
≻ This is especially important as it is common for areas of the
lung to remain uninflated if breathing is shallow.
≻ This often causes a condition called atelectasis
[AT-lect-toe-sees], essentially part of the lung is collapsed.
≻ If this condition goes on for some time, the air sacs of the
lung may lose their elasticity and become fibrous preventing future
inflation.
≻ Also, these areas are prone to accumulate mucus and material
that may contribute to infection and pneumonia in the future.
⚅ The apparatus used is illustrated below.
⚅ The valve circled is a special one-way valve to allow "breath stacking" that is, to allow multiple inflations. From: Hess, D. (2006). Noninvasive Ventilation in Neuromuscular Disease: Equipment and Application. Respiratory Care , 51, 896-912.
⚄ 2.1.2.3.3 CPAP and BiPAP (Nighttime issues):
⚅ Overview: In some cases, IBM can cause weakness in the
diaphragm – the main muscle that is used in breathing.
≻ The presentation of a weak diaphragm is essentially the same as
sleep apnea.
≻ The diaphragm cannot provide enough air volume during the night,
and the person wakes up gasping for air.
≻ Another symptom is extreme fatigue during the day.
≻ The treatment is similar to sleep apnea; however, CPAP is
generally used for sleep apnea.
≻
As I understand it, a BiPAP machine is usually chosen/preferred for
IBM-related diaphragm weakness.
≻ It's quite expensive, and I have seen cases where they supply a
CPAP because it is cheaper.
⚅ Some patients may progress to the point where breathing
during the day is difficult.
≻ In these cases, a respirator can be used attaches to a
wheelchair.
≻ This will be illustrated below.
≻ These respirators usually have a hose that comes around in front
of the person's face, and they can suck on the end, and a breath of air
is provided.
≻ These machines are battery-operated and last about six hours.
≻ When at home, the machines can be plugged in.
⚅
CPAP:
Continuous Positive Airway Pressure.
≻ In this technique, a machine delivers a constant air volume to
the face throughout the breathing cycle: it can only be set to one
pressure level.
≻ Thus, when exhaling, you are exhaling against the flow of
pressure; this has been compared to putting your head out of the window
of a moving car and breathing, feeling the pressure of the air coming at
the face throughout the inhalation /exhalation cycle.
≻ CPAP is usually used in situations of obstructive sleep apnea or
where alveoli need to be recruited and expanded, such as in congestive
heart failure and pneumonia.
⚅
BiPAP:
Bi meaning two, PAP meaning Positive Airway Pressure.
≻ A BiPAP is a small machine used to provide a source of air that
can be delivered through a hose and mask to a patient's lungs.
≻ A BiPAP (or BPAP) uses two different pressures; one higher
pressure is used on inhalation; then the pressure drops to a lower
pressure on exhalation.
≻ The pressurized airflow to the airway and lungs helps you inhale
more deeply and improves your body's oxygen.
≻ A BiPAP machine is about the size of a shoebox with a hose and
carbon dioxide exchange connected to a mask.
≻ To use the BiPAP, you have to wear a small mask over your nose
when you sleep at night. Some people wear a mask that covers the nose
and mouth.
≻ When early respiratory problems disrupt sleep, these machines
are usually used at night.
≻ The air passes through a humidifier, and in some cases, it can
also be heated as required.
⚅ This positive pressure wave during inspirations unloads
the diaphragm, decreasing the work of breathing; therefore, this form of
ventilation has been used extensively in patients with chronic
respiratory failure due to neuromuscular problems or chest wall
abnormalities.
≻ In patients with respiratory failure, a common technique is
begin with the expiratory level at 5 and the inspiratory level at 15.
≻ The levels are adjusted based on patient comfort, tidal volume
achieved and blood gases.
⚅ BiPAP is a registered trademark of Respironics, Inc. Other manufacturers make VPAP and Bilevel machines that provide this same basic features.
⚅ Here are a couple of illustrations of typical machines.
⚄ 2.1.2.3.4 Mechanical Supplementation – Portable ventilation (Daytime issues):
⚅ Many years ago the only treatment for this kind of issue
would be an iron lung and many people who had respiratory issues related
to polio ended up in an iron lung.
≻ Today, compact battery-operated ventilators are used to supply
air on demand to assist patients who require breathing support during
the day.
≻ This is also called
mouthpiece ventilation
because the air is usually delivered through a mouthpiece.
≻ Generally speaking these patients will also be mobility impaired
and will be in a wheelchair.
≻ The most common scenario is that a respirator is attached to the
back of your wheelchair with a tube that goes around in front of your
face.
≻ You can trigger a breath of air by gently sucking on the tube.
≻ Then, the volume corresponding to an average breath will be
delivered by the machine.
≻ These systems are called non-invasive because there is nothing
that enters the body.
≻ In some cases, usually involving spinal cord injuries,
ventilation must be delivered by a tracheostomy tube that is inserted
directly into the throat.
⚅ A common misconception is that these machines are
providing oxygen.
≻ Generally speaking, supplemental oxygen is not required; it is
the volume of regular room air that is the problem and when the person
receives enough volume, generally speaking, they will be receiving
adequate oxygen in that air.
≻ Supplemental oxygen is usually required in cases where the lung
itself is compromised for example in COPD.
⚃ 2.1.2.4 Cough Asssist.
⚄ Coughing is an important normal behaviour that clears mucus from the lungs and helps protect them from infection.
⚄ The ability to cough depends on two factors; having sufficient air in the lungs and having enough muscle force in the diaphragm and rib muscles.
⚄ Muscular disorders, including IBM, can affect the muscles of the diaphragm affecting both air intake and muscle force.
⚄ A cough assist machine blows air into the lungs and pulls it out quickly to clear mucus.
⚅ There are two phases, intake where the machine pushes air into the lungs and expulsion, where the machine actively sucks the air back out and pulling mucus with it.
⚅ Sometimes a supplemental suction machine is also used in conjunction with the cough assist.
⚄ If you feel that you have excess phlegm or if you think that you cannot effectively cough, you should discuss this with your physician.
⚄ Here is an illustration of a common cough assist machine.
⚃ 2.1.2.5 Pneumonia Vaccination.
⚄ Pneumonia is an inflammation of the lung caused by
infection with bacteria, viruses, and other organisms.
≻ The pneumonia vaccine (Pneumococcal Vaccination) is a shot that
can protect you from an infection called pneumococcal (pronounced
"NEW-moe-KOK-al") disease.
≻ The germ responsible for this disease can attack different parts
of the body, including the lungs, causing pneumonia and blood stream
(bacteremia).
≻ If it reaches the brain, it can cause meningitis. All of these
are serious infections.
⚄ There are more than 80 different types of pneumococcus
bacteria, currently 23 of these are covered by the vaccination.
≻ The pneumococcal polysaccharide vaccine (PPV) is injected to
stimulate an immune response to produce antibodies directed against
pneumococcus bacteria giving the patient protection.
≻ Usually, one injection protects for life although in some cases
a booster may be required.
≻ Sometimes the injection is called a Pneumo-Vac.
⚄ This vaccination is recommended for the elderly and for people with chronic conditions that make them prone to developing chest infections.
⚄ Note 1: This is not the same as a flu vaccination which is
generally given in the fall of the year.
≻ A different vaccination is prepared each year in anticipation
for the upcoming flu season.
≻ There is some controversy whether IBM patients should be getting
a flu vaccination or not, some doctors advocate for, some against.
⚄ Note 2: Pneumonia can also have other causes, a common cause in muscle disease is the aspiration of food into the lungs.
⚃ 2.1.2.6. Specific IBM Related Research.
(chronological order)
Harlaar, L., Ciet, P., van Tulder, G., Brusse, E., Timmermans, R. G. M., Janssen, W. G. M., de Bruijne, M., van der Ploeg, A. T., Tiddens, H. A. W. M., van Doorn, P. A., & van der Beek, N. A. M. E. (2022). Diaphragmatic dysfunction in neuromuscular disease, an MRI study. Neuromuscular Disorders, 32 (1), 15-24. https://doi.org/10.1016/j.nmd.2021.11.001
Shelly, S., Mielke, M. M., Mandrekar, J., Milone, M., Ernste, F. C., Naddaf, E., & Liewluck, T. (2021). Epidemiology and Natural History of Inclusion Body Myositis: A 40-Year Population-Based Study. Neurology , https://doi.org/10.1212/WNL.0000000000012004
Lelièvre, M., Hudson, M., Botez, S. A., & Dubé, B. (2021). Determinants and functional impacts of diaphragmatic involvement in patients with inclusion body myositis. Muscle & Nerve, 63 (4), 497-505. https://doi.org/10.1002/mus.27170
Goyal, N. A., Cash, T. M., Alam, U., Enam, S., Tierney, P., Araujo, N.,
… Mozaffar, T. (2016). Seropositivity for NT5c1A antibody in
sporadic inclusion body myositis predicts more severe motor, bulbar and
respiratory involvement.
Journal of Neurology, Neurosurgery, and Psychiatry, 87
(4), 373-8. http://doi.org/10.1136/jnnp-2014-310008
Available here:
pdf
Rodríguez Cruz, P. M., Needham, M., Hollingsworth, P., Mastaglia, F. L., & Hillman, D. R. (2014). Sleep disordered breathing and subclinical impairment of respiratory function are common in sporadic inclusion body myositis. Neuromuscular Disorders, 24 (12), 1036-1041. https://doi.org/10.1016/j.nmd.2014.08.003
Cruz PMR, Needham M, Hollingsworth P, Mastaglia FL, Hillman DR. Sleep
disordered breathing and subclinical impairment of respiratory function
are common in sporadic inclusion body myositis. Neuromuscul Disord
[Internet]. 2014 Aug 20 [cited 2014 Sep 18]; Available from:
http://www.ncbi.nlm.nih.gov/pubmed/25227894
Available here:
pdf
Della Marca G, Sancricca C, Losurdo A, Di Blasi C, De Fino C, Morosetti
R, et al. Sleep disordered breathing in a cohort of patients with
sporadic inclusion body myositis. Clin Neurophysiol [Internet].
International Federation of Clinical Neurophysiology; 2013 Apr 10 [cited
2013 Apr 23];124(8):1615-21. Available from:
http://www.ncbi.nlm.nih.gov/pubmed/23583020
Available here:
10.1016/j.clinph.2013.03.002
Muscle Nerve. 2009 Feb;39(2):144-9.
Obstructive sleep apnea in patients with inflammatory myopathies. Selva-O'Callaghan A, Sampol G, Romero O, Lloberes P, Trallero-Araguás E, Vilardell-Tarrés M.
Curr Opin Neurol. 2005 Oct;18(5):497-503.
Diagnosis, pathogenesis, and treatment of inclusion body myositis.
Oldfors A, Lindberg C.
Goteborg Neuromuscular Center, Department of Pathology, Sahlgrenska
University Hospital, Goteborg, Sweden Goteborg Neuromuscular Center,
Department of Neurology, Sahlgrenska University Hospital, Goteborg,
Sweden.
PURPOSE OF REVIEW: We provide an update on progress gained from research
into sporadic inclusion body myositis (s-IBM). RECENT FINDINGS: Most
research on s-IBM has focused on the inflammatory reaction or the
accumulation of pathological proteins in vacuolated muscle fibres. The
inflammatory reaction is characterized by clonal expansions of
lymphocytes, predominantly CD8 cytotoxic T cells, which invade and
destroy muscle fibres. The costimulatory molecules identified
demonstrate that muscle fibers can act as antigen-presenting cells, and
the expression of various chemokines in muscle indicates their
importance in the immunopathogenesis of s-IBM. The region of interest
for a susceptibility gene in the major histocompatibility complex has
been narrowed, and for the first time, it has been demonstrated that a
chronic viral infection can trigger the inflammatory process leading to
s-IBM. The nature of the accumulated material associated with the
vacuoles has been extensively investigated over the past few years.
Amyloid-b and phosphorylated tau protein in intracellular inclusions are
a characteristic finding in s-IBM, which may lead to calcium
dyshomeostasis and endoplasmic reticulum stress. The proteasomal system
is upregulated, including immunoproteasomes. 'Molecular misreading'
leading to ubiquitin mRNA mutations and accumulation of pathological
ubiquitin in muscle fibres may be associated with proteasomal
dysfunction. There is still no efficient treatment for s-IBM, but the
effects of new, more specific immunotherapies have begun to be explored.
SUMMARY: Recent findings indicate that both inflammatory reaction and
abnormal protein accumulation are important for the pathogenesis in
s-IBM. The link between them continues to await elucidation.
Neuromuscul Disord. 2005 Jan;15(1):32-9.
Diaphragmatic dysfunction in patients with idiopathic inflammatory
myopathies.
Teixeira A, Cherin P, Demoule A, Levy-Soussan M, Straus C, Verin E,
Zelter M, Derenne JP, Herson S, Similowski T.
Polymyositis, dermatopolymyositis, and inclusion body myositis imply
chronic inflammation of skeletal muscles. Pulmonary complications
include aspiration pneumonia, interstitial pneumonitis, or respiratory
muscle myositis. This study aims at better describing their impact on
respiratory muscles. Twenty-three consecutive patients (12 PM, 5 DM, 6
IBM) were studied (static inspiratory and expiratory. pressures;
diaphragm function in terms of the mouth and transdiaphragmatic pressure
responses to bilateral phrenic stimulation). Pulmonary parenchymatous
abnormalities were mild (6 cases) or absent. The mouth pressure produced
by phrenic stimulation was 6.83+/-3.01 cm H2O, with 18 patients (78%)
diagnosed with diaphragm weakness (<10 cm H2O) and lower values in DM
(4.35+/-1.48 cm H2O) than in IBM and in PM (P<0.05). Diaphragm
weakness is frequent and probably overlooked in inflammatory myopathies.
Further studies are needed to delineate the clinical relevance of these
results.
Quotes from article:
Polymyositis (PM), dermatopolymyositis (DM), and inclusion body myositis
(IBM) are auto immune diseases defined by an idiopathic chronic
inflammation of skeletal muscles. Their main clinical expression is limb
muscle weakness. Cardiac, gastrointestinal and respiratory complications
can occur. The latter are major sources of morbidity and mortality [1]
and include aspiration pneumonia and instertitial pneumonitis (up to 40%
of PM and DM patients) [2]). In addition, respiratory muscle can
obviously be involved by the inflammatory process but prevalence data
are scarce. This is however potentially clinically relevant. Indeed,
besides dyspnoea, diaphragm dysfunction reduces the ability of the
respiratory system to face sudden increases in ventilatory demand. In
the context of inflammatory myopathies, this could explain some of the
episodes of acute respiratory failure that sometimes punctuate the
course of the disease. Diaphragm dysfunction also causes hypoventilation
during sleep [3] and disrupts sleep architecture [4], leading to daytime
sleepiness, impaired cognition, and a poor quality of life. This forms
the logical basis of treatment by nocturnal positive pressure
ventilation. Amyotrophic lateral sclerosis provides a good illustration
of the clinical relevance of diaphragmatic dysfunction in neuromuscular
diseases. In this setting, diaphragm dysfunction correlates with
dyspnoea [5], disorganized sleep [4], and a reduced survival [4]. This
explains how positive pressure ventilation can prolong survival [6] and
improve quality of life [7]. Of note, the consequences of sleep-related
hypoventilation on oxygenation will be more severe in the presence of
preexisting gas exchanges abnormalities. Such abnormalities are not
uncommon during inflammatory myopathies, because of the frequency of
aspiration pneumonia or interstitial lung disease. Therefore, diagnosing
diaphragm dysfunction could be germane to the clinical management of
patients with inflammatory myopathies.
The salient finding of this study, conducted in a relatively large cohort of unselected patients with inflammatory myositis, is a prevalence of diaphragm dysfunction (defined according to modern non-volitional diaphragm testing techniques) in excess of 75%. This suggests that the involvement of the respiratory muscles by the myositic process is more frequent than generally thought and implies that the diagnosis is probably overlooked.
Neurology. 2004 Dec 14;63(11):2191-2.
Primary respiratory failure in inclusion body myositis.
Voermans NC, Vaneker M, Hengstman GJ, ter Laak HJ, Zimmerman C,
Schelhaas HJ, Zwarts MJ.
Neuromuscular Centre Nijmegen, Department of Neurology, University
Medical Centre Nijmegen, The Netherlands.
Case report. A 58-year-old woman sought treatment for slowly progressive
muscle weakness, dysphagia, and weight loss. Her medical history was
unremarkable, and she did not use any myotoxic drugs. Physical
examination revealed normal speech, mild facial weakness, dysphagia
without aspiration, and generalized muscle weakness (Medical Research
Council [MRC] score, 4) with asymmetric weakness of the forearm muscles
(right, MRC 4; left, MRC 3). Muscle atrophy was most pronounced in the
quadriceps muscles. Creatine kinase was mildly increased (285 U/L;
normal, =180 U/L). Nerve conduction studies were normal. EMG
demonstrated spontaneous activity and a mixed pattern of shortduration
low-amplitude and long-duration high-amplitude motor unit potentials at
submaximal voluntary contractions. By EMG, forearm flexor muscles were
more involved than forearm extensor muscles. Muscle biopsy from the
anterior tibial muscle revealed a small number of muscle fibers
surrounded by collagen, fat cells, and inflammatory infiltrates. IBM was
diagnosed according to established criteria, and the patient was
referred to a rehabilitation clinic.
Eight months after initial presentation, she gradually developed
shortness of breath on exertion. She subsequently experienced morning
headaches, excessive daytime sleepiness, and confusion. One day she was
found unconscious as a result of severe hypercapnia and hypoxemia and
required intubation and mechanical ventilation. Arterial blood gas
analysis after intubation showed elevated PCO2 (7.9 kPa = 806 mm H2O)
and bicarbonate (33.7 mmol/L), reflecting chronic hypoventilation.
Ancillary investigations showed no signs of cardiac disease or
respiratory infection. Initially, nighttime apneas occurred lasting up
to 45 seconds with hypercapnia (PCO2 8.2 kPa = 836 mm H2O) and elevated
bicarbonate (34.8 mmol/L), after which mechanical ventilation was
adjusted. Apparently, the respiratory drive in our patient was depressed
during sleep. This probably resulted from bicarbonate retention and
sleep deprivation caused by frequent arousals during REM sleep because
of hypercapnia.7 Her general condition improved, and the need for
daytime ventilatory support decreased. She underwent tracheotomy and
thereafter required volume-regulated ventilation for 2 hours in the
afternoon and at night. Two months after admission, she was discharged
home on continued mechanical ventilation.
The presence of severe respiratory failure made us reconsider her
diagnosis. Empirical treatment with prednisone (50 mg daily for 4 weeks)
resulted in minimal increase in muscle strength. Nerve conduction
studies of the phrenic nerve demonstrated low amplitudes of the compound
motor action potential of the diaphragm bilaterally. EMG revealed poor
recruitment without spontaneous activity of the diaphragm and
intercostal muscles. Muscle biopsy from the tibialis anterior showed
myopathic changes with invasion of non-necrotic muscle fibers by
mononuclear cellular infiltrates, basophilic rimmed vacuoles, and
sarcolemmal HLAABC positivity of all muscle fibers, consistent with the
diagnosis of IBM6 (figure). Pulmonary function testing revealed mildly
impaired vital capacity (1.87 L; normal, 2.60 L) and decreased mouth
pressures (maximal expiratory pressure, 3.01 kPa = 307 mm H2O; normal,
8.90 kPa; maximal inspiratory pressure, 1.90 kPa = 193 mm H2O; normal,
6.90 kPa), reflecting an extraparenchymal restrictive pattern. It was
concluded that the patient had IBM6 with respiratory muscle weakness,
which had resulted in primary respiratory failure.
Discussion. Respiratory failure in patients with IBM is generally
believed to occur only secondary to aspiration pneumonia or coincidental
pulmonary or cardiac disease. Primary respiratory failure has, to the
best of our knowledge, been reported only twice in literature. One of
these reported patients had a concomitant human T-cell lymphotropic
virus type infection. In our patient, respiratory failure resulted from
hypoventilation caused by weakness of the diaphragm and intercostal
muscles. Our limited awareness of this manifestation of IBM delayed its
recognition and resulted in life-threatening respiratory failure. Only
then the ventilatory support was started, and the vicious circle of
respiratory muscle weakness, hypoventilation, hypercapnia, sleeping
vproblems, deterioration of the general condition, weight loss, and
severe respiratory failure was broken. Moreover, we started highdose
corticosteroid treatment, which involves a risk of serious adverse
effects. In conclusion, primary respiratory failure can occur in
patients with IBM, and awareness of this potentially lifethreatening
complication is warranted, especially for patients with signs and
symptoms suggestive of hypoventilation.
J Neurol Neurosurg Psychiatry. 2002 May;72(5):650-2.
Human T cell leukaemia virus type I associated neuromuscular disease
causing respiratory failure.
Littleton ET, Man WD, Holton JL, Landon DN, Hanna MG, Polkey MI,
Taylor GP.
Polymyositis and inclusion body myositis have rarely been described in
association with human T cell leukaemia virus type I (HTLV-I) infection.
Most of such patients have coexisting HTLV-I associated myelopathy
(HAM). Two patients with HTLV-I infection, myopathy, and respiratory
failure are described. The muscle biopsy specimen of the first patient
bore the histological features of inclusion body myositis and there was
no evidence of concurrent myelopathy. The second patient had HAM, and
her muscle biopsy showed non-specific myopathic and neuropathic changes.
Both patients developed respiratory muscle weakness over eight years
after diagnosis of myopathy, leading to hypercapnic respiratory failure
requiring mechanical ventilatory support. Respiratory failure as a
complication of HTLV-I associated myopathy has not previously been
described.
from the discussion: The myopathogenic mechanism underlying the
association with HTLV-I infection is not proven, but has previously been
supported by the detection of the virus within inflammatory cells
invading muscle tissue in patients with polymyositis and inclusion body
myositis. Our first patient is only the third reported case of HTLV-I
associated inclusion body myositis. Furthermore, our two patients are
the first reported cases to suggest that HTLV-I infection can cause
disease of the proximal and respiratory muscles of such severity as to
cause ventilatory failure.
Medicina (B Aires). 2002;62(1):37-40.
[Inclusion body myositis. Report of 4 cases]
[Article in Spanish]
Basquiera AL, Caeiro F, Palacio S, Theaux R, Casale A, Lucero C,
Alvarellos A.
Inclusion body myositis (IBM) is a primary inflammatory myopathy
characterized by an older age at presentation. We describe four IBM
cases fulfilling Mendell's diagnostic criteria. All patients were older
than 60 years at diagnosis and the mean length of time from onset to
diagnosis was 5.7 years. Two of them complained of leg weakness with
unsteady gait and the other two, of upper limb weakness. Three patients
had dysphagia, one of them had diaphragmatic paralysis and another had
bilateral blepharoptosis. Histological sections of the muscle biopsy
showed mononuclear cell invasion of nonnecrotic muscle fibers, rimmed
vacuoles, intracellular amyloid deposits and 16-21 nm tubulofilaments by
electron microscopy. Mitochondrial anomalies were found in two cases.
Only one patient had transient response to steroid therapy. Our serie
shows that clinical presentation of inclusion body myositis includes a
broader spectrum than the classical description.
Intern Med 2001; 40:940-944.
Mori S, Hamada H, Yokoyama A, et al.
Severe inclusion body myositis with interstitial pneumonia.
We report a patient with a severe inclusion body myositis (IBM). His
illness was unusual in terms of a rapid progression, high creatine
kinase levels, and complication with interstitial pneumonia. He
responded well to immunosuppressive agents such as corticosteroids,
cyclosporin A, cyclophosphamide, and immunoglobulin. The present patient
indicates the wide range of the disease, and that immunosuppressive
agents may be useful for treatment of IBM.
Chest. 1993 Sep;104(3):975-7.
Inclusion body myositis as a cause of respiratory failure.
Cohen R, Lipper S, Dantzker DR.
Inclusion body myositis (IBM) is a slowly progressive myopathy that has
not been reported to affect respiratory muscles. It is often refractory
to treatment and a muscle biopsy specimen is necessary for the
diagnosis. This is a report of a patient with IBM who quickly progressed
to respiratory muscle failure requiring intubation.
CASE REPORT
A 69-year-old woman was admitted to the ICU with respiratory failure.
She had a 10-day history of progressive shortness of breath, cough,
lethargy, and weakness. There was no history of fever, sweats, or
chills. The patient was hospitalized in 1987 with a similar
presentation. At that time, she had complained of a 2-week history of
progressive dyspnea and slowly worsening limb weakness for the
preceeding five years. The electromyogram (EMG) at that time was
compatible with a myopathy; however, the creatine kinase (CK) and liver
function test results were normal. She was found to have an elevated
partial thromboplastin time (PTT) that was shown to be due to a lupus
anticoagulant. Her antinuclear antibody (ANA) titer was 1:320. Her
platelet count was normal as were the results of her thyroid function
tests. A Tensilon test was negative. The chest radiograph was normal.
She required mechanical ventilation and was treated as having mixed
connective tissue disease and received large doses of intravenous
methylprednisolone sodium succinate (Solu-Medrol). Her hospital course
was complicated by multiple infections that were, in part, attributed to
the large doses of steroids. As there was no clinical improvement, the
steroid therapy was discontinued. The patient underwent a muscle biopsy
from the left quadricep that showed isolated myofiber degeneration,
atrophic fibers, and no inflammatory infiltrates. She was discharged
from the hospital 1 year later with a diagnosis of idiopathic myopathy.
She remained well until the present hospital admission without worsening
of the myopathy or further respiratory problems.
The physical examination showed the patient to be in moderate
respiratory distress. She had petechiae on both calves and poor air
entry in the lungs, which were otherwise clear. The neurologic
examination showed her to be alert and oriented, with distal muscle
atrophy and strength that was rated as 1/5. There was also proximal
muscle weakness rated as 3/5. Findings from the sensory examination were
normal. Deep tendon reflexes were rated as 1/3 in the lower extremities
and 2/3 in the upper. The plantar reflexes were downgoing. Laboratory
tests showed a platelet count of 19,000/[mu]l and a hemoglobin of 12
g/dl. The white blood cell count and differential cell count were
normal; she had a PTT of 51.7 s with a normal prothrombin time. The ANA
titer was 1:640, anti dsDNA was normal. The arterial blood gases
revealed a pH of 7.40, a [PCO.sub.2] of 49 mm Hg, and a [PO.sub.2] of 55
mm Hg on room air. The CK, lactate dehydrogenase, and liver function
test results were normal. The chest radiograph was normal. She refused
to perform a tidal volume maneuver. The negative inspiratory force was
-- 12 cm [H.sub.2]O. The EMG was again consistent with myopathy.
Her respiratory status quickly deteriorated requiring intubation. The
low platelet count was thought to be secondary to immune
thrombocytopenia and responded to intravenous immunoglobulin therapy
with normalization of the count. A muscle biopsy specimen revealed
occasional fibers containing small round or angular vacuoles situated
centrally or peripherally. Vacuoles showed granular rimming with the
modified Gomori stain (Fig 1). Electron microscopy showed the vacuoles
to contain membranous whorls and rare tubulofilaments (Fig 2). The
features were those of IBM. The patient's course worsened after she
developed an upper gastrointestinal tract bleed and sepsis; she died due
to complications of the sepsis.
DISCUSSION
Inclusion body myositis is an inflammatory muscle disorder that was
initially thought to be due to a viral etiology although that remains
unproven.[1] There is a 2:1 male preponderance, and the onset is usually
after age 50 years. The average duration from onset of symptoms to
diagnosis has ranged from 5 to 19 years.[2] This disease is usually
characterized by a slow but progressive course, distal muscle weakness,
and resistance to immunosuppressive therapy. The weakness and atrophy
can be asymmetric, with selective involvement of the quadriceps,
iliopsoas, triceps, and biceps muscles. Early loss of the patellar
reflex can occur due to the quadricep weakness and a neurogenic disease
is often suspected.[2,3] This disorder can clinically mimic idiopathic
polymyositis and in one report accounted for almost one third of the
adults with "polymyositis unresponsive to therapy."[3] The
occurrence of respiratory failure due to IBM is distinctly unusual.
While dysphagia has been noted to occur in 40 percent of those affected,
our patient did not complain of any swallowing difficulties and there
was no clinical or radiologic evidence of aspiration. Lotz et al[2]
reported a 55 percent incidence of cardiovascular signs and symptoms
such as hypertension, ECG changes compatible with ischemia, and rhythm
disturbances in their 40 patients. Other diseases such as malignancy and
diabetes have been associated with IBM,[2] but to our knowledge,
respiratory muscle weakness had not been reported. The positive ANA,
immune thrombocytopenia, and antiphospholipid antibody present in our
patient give evidence of disturbed immune regulation. Both elevated ANA
titers and thrombocytopenia were reported in patients with IBM;[2,3] we
have found no reported association with lupus anticoagulant. Serum CK
levels can be up to 10-fold elevated or normal.[3] Electromyographic
findings are those of a myopathy, although there is occasionally
evidence of a neuropathy.[1,2,4] A muscle biopsy specimen is important
in excluding primary neuropathic disease and in diagnosing IBM and
distinguishing it from other myopathies that may cause respiratory
failure such as polymyositis and systemic lupus erythematosus. Not only
is the natural history of IBM different, but since it is refractory to
treatment with corticosteroids and cytotoxic agents, the correct
diagnosis can spare the patient the side effects associated with these
medications. It is important to obtain the biopsy specimen from muscles
where the inflammation is active and the biopsy site should be
correlated with activity on the EMG.[4,5] The inflammation may be
distributed in a patchy fashion as likely was the case in our patient
and occasionally multiple or repeated biopsy specimens are needed to
establish the diagnosis. Review of first biopsy specimen did not show
any evidence of IBM. It is likely that the patient had IBM when she was
first admitted to the hospital in 1987. However, for the above-mentioned
reasons, the diagnosis was not made on the initial muscle biopsy
specimen. A low index of suspicion, as IBM is thought to evolve slowly,
and the lack of reported respiratory muscle involvement contributed to
the lack of earlier recognition in the present case.
Brain. 1989 Feb;112 ( Pt 1):65-83.
Distal myopathy with rimmed vacuole formation. A follow-up study.
Sunohara N, Nonaka I, Kamei N, Satoyoshi E.
A long-term follow-up study of patients with familial distal myopathy
with rimmed vacuole formation and a review of the literature indicates
that the prognosis of the disorder was extremely poor as to daily life.
Although the initial symptom appearing in early adulthood was muscular
wasting and weakness in the legs, especially the distal muscles, severe
generalized skeletal muscle involvement with sparing of the facial,
extraocular, bulbar, intercostal and diaphragm muscles was recognized in
the advanced stage. The disease is probably inherited as an autosomal
recessive trait, while there is a considerable female preponderance, the
female-to-male ratio being 2:1. The disorder is distinguishable from
various types of distal myopathy on the basis of clinical and
pathological findings, and other myopathies with rimmed vacuole
formation, including inclusion body myositis, from a prognostic
viewpoint.
⚃ 2.1.2.7. Glossary:
⚄ Alveoli / Alveolus: [Ave-VOLE-ee] The alveoli are the final branchings of the respiratory tree and perform gas exchange for the lung. The thin gas-blood barrier between the alveolar space and the pulmonary capillaries allows rapid gas exchange. Oxygen and carbon dioxide diffuse through the alveolar epithelium and the capillary endothelium.
⚄ Arterial blood gas: Arterial blood gas is the concentration
of oxygen and carbon dioxide carried in arterial blood. Arterial blood
gas analysis is a test in which blood is taken from an artery in your
wrist to evaluate how effective your lungs bring oxygen to the blood and
remove carbon dioxide from it.
The following 4 "rules" of ABG interpretations help in
evaluation:
1) For every acute rise of 10 in PCO2 the bicarbonate will rise by 1
2) For every chronic rise of 10 in PCO2 the bicarbonate will rise by 4
3) For every fall by 10 in PCO2 the bicarbonate will fall by 2
4) For every acute rise of 10 in PCO2 the PH will fall by 0.1
⚄ Assisted Cough Manoeuvre: A manually Assisted Cough Manoeuvre involves the application of an abdominal thrust or costal lateral compression using various hand placements after an adequate spontaneous inspiration or maximal insufflation.
⚄ Atelectasis [AT-lect-ta-sees or A-Tell-lect-ta-sis]is a partial or total collapse of the lung. It occurs when all the gas in the alveoli is absorbed and not replaced.
⚄ Diaphragm: [DIE-a-fram] The diaphragm is the muscular partition located between the chest cavity and the abdominal cavity. It plays a major role in respiration. The diaphragm descends during inspiration and the inspiratory muscles contract. During expiration, the diaphragm rises, the inspiratory and the rib cage rise , muscles relax, and the rib cage descends.
⚄ Dyspnea [DIS-me-ah] is a term used to describe difficulty in breathing. Dyspnea is a common symptom of many lung diseases. Shortness of breath, short of breath, difficulty breathing, breathing difficulty
⚄ FEF Max: The maximum Forced Expiratory Flow rate (FEF) measured during a Force Vital Capacity (FVC) manoeuvre.
⚄ FRC is the volume of air in the lungs when the respiratory muscles are fully relaxed and no airflow is present.
⚄ GPB: Glossopharyngeal [GLOSS-off-fur-IN-gee-ILL] Breathing (GPB) is a "frog-like" method of breathing, which consists of stroke-like action of the tongue along with constricting action of the pharynx pumping air through the larynx into the lungs.
⚄ Hypoxia: a shortage of oxygen in the body.
⚄ Hypopnea is a medical term for abnormally shallow breathing or slow respiratory rate. Among the causes of hypopnea are neuromuscular disease or any condition that entails weakened respiratory muscles. For people with neuromuscular disorders the most common treatment is the use of BIPAP or other non-invasive ventilation.
⚄ Intercostal muscles: The intercostal muscles are located between the ribs. There are two kinds of intercotal muscles: internal and external. The intercostal muscles are controlled by the brain (medulla). The internal intercostal muscles are located on the inside of the ribs and extend from the front of the ribs, around back and past the bend in the ribs. The external intercostal muscles are located on the outside of the ribs, and wraparound the back of the ribs.
⚄ LVR: Lung Volume Recruitment (LVR) refers to breath stacking, techniques allowing for maximum insufflation capacity.
⚄ MIC: The Maximum Insufflation Capacity (MIC) measurement (litres) is the maximum volume of air stacked within the patient's lungs beyond spontaneous vital capacity. MIC is attained when the patient takes a deep breath, holds his breath and then breath stacking is applied using a LVR resuscitation bag, a volume ventilator or glossopharyngeal breathing (GPB). When measuring a MIC, the therapist should assist the patient with his/her optimal insufflation technique, introduce the spirometer in the post mode and instruct the patient to completely exhale the MIC volume through the spirometer. The documented volume must be clearly identified as a MIC and not a post bronchodilator study.
⚄ Peak expiratory flow rate: The peak expiratory flow rate measures how fast a person can breath out (exhale) air. It is one of many tests that measure how well your airways work.
⚄ Obstructive sleep apnea (OSA). A common type of sleep apnea caused by obstruction of the airway. It is characterized by pauses in breathing during sleep called apneas (literally, "without breath"), each last long enough that one or more breaths are missed, and occurring repeatedly throughout sleep. In obstructive sleep apnea, breathing is interrupted by a physical block to airflow, despite the effort to breath.
⚄ PCF: Peak Cough Flow (PCF) is measured by using a peak flow
meter.
The PCF is the velocity of air being expelled from the lungs after a
cough manoeuvre. This measurement can be expressed in L/min or L/sec
(L/min divided by 60).
It is useful to measure:
- spontaneous PCF (PCF sp)
-PCF from MIC (PCF bag, PCF vent, or PCF gpb)
-PCF from MIC with an assisted cough timed with the cough (PCF bag &
assist, PCF vent & assist or PCF gpb & assist)
⚄ Pneumonia is a common lung infection that affects millions of people each year. Pneumonia is characterized by an inflammation of the lungs caused by a bacteria, virus, or fungus. Pneumonia can be mild, moderate, severe, or even fatal. Since the symptoms of a cold are similar to the symptoms of pneumonia, it's difficult to tell which illness you might have.
⚄ Polysomnogram: An overnight sleep test used to diagnose sleep-related problems.
⚄ Respiratory acidosis is acidosis (abnormal acidity of the blood) due to decreased ventilation of the pulmonary alveoli, leading to elevated arterial carbon dioxide concentration (PaCO2). Chronic respiratory acidosis may also be caused by neuromuscular disorders that reduce airflow volume.
⚄ Sleep apnea, is a sleep disorder characterized by pauses in breathing during sleep. These episodes, called apneas, each last long enough, so one or more breaths are missed repeatedly throughout sleep. Breathing is interrupted by the lack of effort in central sleep apnea; in obstructive sleep apnea, breathing is interrupted by a physical block to airflow despite the effort. In mixed sleep apnea, there is a transition from central to obstructive features during the events themselves.
⚄ Spirometry is a pulmonary function test that measures the air volume and flow rate within the lungs. It is used to diagnose lung diseases and determine treatment.