||Finding a Doctor
Unfortunately, finding someone who is "cross trained" to understand
both the effects of neuromuscular disease on respirations and the respiratory
assistance machines available can be hard. The neurologist knows neuromuscular
disease but diddly about the machines. The pulmonologist (physician specializing
in respiratory problems) knows the machines but not neuromuscular disease.
In our screwed up health care delivery system someone might require that
you see a sleep specialist. The sleep guys are used to sleep apneaics who
have good lung power and is very unlikely to be familiar with the problems
of the neuromuscular patient.
Start with your neurologist. A neurologist who works with ALS patients
should certainly be familiar with BiPAP. He may handle your respiratory care
himself, but may prefer to refer his patients to a pulmonologist for respiratory
care at that point. I would be reasonably comfortable with either of them,
but if I were sent to a sleep specialist I would most definitely ask "How
many ALS patients on BiPAP have you cared for?" and if he could not demonstrate
a very good understanding of ALS, I would be looking elsewhere.
The doctor who handles your respiratory care is going to be a very
important person in your care. Patients with ALS who do not want to go on
a ventilator most often die of respiratory failure so this doctor will be
the one who sees you through to the end or sees you through many years on
a ventilator. You may still see other doctors, but odds are that this is
the one who will be there directing most of your care. You need someone who
is comfortable not only in deciding what pressure settings you need and dealing
with any respiratory infection, but also in listening, explaining and helping
you make decisions about things like tube feedings, code status, and whether
or not to switch to full ventilation when BiPAP is no longer sufficient and
perhaps when to discontinue the ventilator. At some point he or she will
likely be the physician you depend on to make the end as easy and dignified
There are several tests doctors can do to monitor how much ALS is affecting breathing:
||A respiratory function test
involves huffing and puffing into a machine to see how
much lung capacity you have. The FVC (Forced Vital
Capacity) which is basically how deep a breath you can take is one of the
most important findings of the test . At about 70% of normal you begin to
notice problems, less than 60% is considered moderate, less than 40% is severe.
People with neuromuscular diseases should have the test done while they are
lying down as that is when their breathing is the most impaired. A respiratory
function test/FVC does not show whether you have sleep apnea or if hypopnea
is even worse when you fall asleep, however.
||Another helpful test is a simple test of blood oxygen level. This is reported
as Oxygen Saturation (O2 Sat) percentage. An O2 Sat of 96% means that 96% of your red
bloods cells are carrying a full load of oxygen (are saturated) like they
are supposed to be. Normal is 90% or better (usually high 90's). O2 Sat
monitoring can be done with a simple little device you clip or tape on your
finger tip. No needles! For us, the important thing is to wear the device
while lying down and sleeping. A spot check in the middle of the day will
no doubt show excellent levels when breathing problems first begin because
our breathing is at its worst when lying down and/or sleeping.
To accurately test you, your doctor can arrange for you to use an O2 Sat
monitor at home overnight. You just put the device on your finger at bedtime
and wear it all night. It monitors oxygen saturation and heart rate, and
records it. The next day you return it, the results are retrieved from its
memory, and reported to your doctor. If you are breathing too shallowly (hypopnea)
your O2 Sat should drop. (CO2 levels would be more accurate in spotting hypopnea
but as discussed below, they are not as easily done accurately.) If you have
apnea, your O2 Sat will drop briefly but dramatically at repeated intervals.
These two tests along with a diagnosis of a neuromuscular disease such
as ALS and a description of the problems you are having should be sufficient
to get a prescription for BiPAP and insurance reimbursement for it. Unfortunately
sometimes it gets more complicated. Physicians and/or insurance companies
may want more diagnostic evidence and want other tests done:
||ABG's (Arterial Blood Gases)
Doctors tend to be irritatingly fond of these lab tests. ABG's require drawing blood from an artery (usually
in the wrist - an "uncomfortable" procedure). ABG's can
tell a doctor a lot more about your respiratory status than just oxygen levels.
For example, they tell the CO2 levels, can help determine if your breathing
problems are due to lung problems or other problems such as liver or kidney
disease and even how well your body is able to compensate for the problem.
But ABG's are NOT always necessary.
Although ABG's are a perfect way of checking CO2 levels, they are a total
waste of time for the early stages of breathing problems in ALS if they are
done when you are wide awake and breathing normally! When breathing problems
become severe CO2 levels will be high even when you are awake, but not early
on when you first could begin benefitting from BiPAP.
DO NOT allow them to draw
ABG's during a daytime appointment (unless you have some other lung disease
too) as a first step in determining if you need BiPAP. You have to be in
pretty bad shape to have a high CO2 while you are wide awake and being stuck
in an artery with a big needle! INSIST that the doctor explain
what he needs to know from the ABG's that he can't figure out simply by knowing
that you have ALS and overnight O2 Sat monitoring. Your doctor may believe
that the insurance company will reject a claim for BiPAP without this bit
of supportive evidence but I would ask him to file with all the other info
he has on you first and would not agree to the test unless the insurance
company rejected my claim.
Some physicians and/or insurance companies will want you to have a sleep
study done to verify the need for BiPAP. This
expensive test involves spending a night in a sleep lab trying to sleep while
you are all wired up with monitoring devices. The information will tell the
doctor whether you have sleep apnea and help determine what kind of pressure
settings your machine will need. A sleep study is necessary to get
a diagnosis of Sleep Apnea, but for an ALS patient that should not be
necessary. The use of BiPAP for the breathing problems accompanying ALS is
well documented and standard care. Simply having a diagnosis of ALS and evidence
of the onset of breathing problems should be sufficient to justify ordering
Bi-PAP. A secondary diagnosis of sleep apnea or hypopnea by a sleep lab should
not be needed. But, depending on your insurer, you may have to jump through
their hoops. Again, your doctor may believe that the insurance company will
reject a claim for BiPAP without this bit of supportive evidence but I would
ask him to file with all the other info he has on you first and would not
agree to the test unless the insurance company insisted on it.
If you are required to have a sleep study, be aware that ABG's are commonly
done with them. Again it is important that the blood be drawn while you are
asleep -- or as soon after awakening as possible. (No one is going to sleep
through an arterial blood draw!) Ideally the blood should be drawn first
thing upon awakening. I would refuse to have the test done if it was not
done at least before I was out of bed. I have heard of instances where it
was done after the patient was up to the bathroom, dressed, and ready to
go home! No wonder the ABG's did not support her need for BiPAP! You always
have a right to refuse a test or medication, and in this case not only is
the test useless but it will work against you in getting reimbursement for BiPAP!!!