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==        ----------- ALS Interest Group -----------         ==
==            ALS Digest #808  (16 February 2001)            ==
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CONTENTS OF THIS ISSUE:
1 .. re: BiPAP (meaning of acronym)
2 .. re: BiPAP (meaning of acronym)
3 .. News from ALSA - North Texas Chapter
4 .. on line books
5 .. Pulmonary Protocol : Ed White

(1)
=====
re: BiPAP (meaning of acronym)
==========
Date   : Fri, 16 Feb 2001 11:09:09 -0800
Subject: Re:

Dear Vincent Celestina,

BiPAP was developed by Dr. Mark Sanders, I believe, and became the trade
mark noninvasive portable home ventilator of Respironics. Dr. Colin
Sullivan in Sydney, Australia published one of the first papers on nasal
noninvasive portable home ventilation in 1987. There are now many other
manufacturers of noninvasive portable home ventilators.

The BiPAP portable ventilator developed from the CPAP equipment used for
sleep apnea. CPAP (continuous positive airway pressure) provides
continuous airflow to keep the upper airway open, in the back of the
throat just above the vocal cords. The BiPAP noninvasive ventilator
provides a second higher level of pressure during the breathing-in
(inspiratory) period to force air down into the lungs to inflate the
lungs, assist ventilation. Thus there are two positive pressure levels
used, one is the lower level expiratory positive airway pressure (EPAP),
and the other intermittently cycles to give inspiratory positive airway
pressure (IPAP). Therefore the trade name "BiPAP". The general name for
this type of assistive device is a "bilevel ventilator".

HCFA calls these "respiratory assist devices" (RAD) when they are used
6-8 hours daily, part time, particularly night time. The HCFA policy and
requirements for reimbur{*filter*}t for RAD equipment is not intended to apply
to people who must use a nasal noninvasive portable home ventilator 16 to
24 hours a day for life-support.

Currently in development are a new generation of "smart" BiPAP (bilevel)
equipment that will automatically adjust the IPAP and the EPAP to the
varying needs of the user. When available these will be more effective
and more comfortable to use.

Edward Anthony Oppenheimer, MD, FCCP
Pulmonary Medicine, Los Angeles

(2)
=====
re: BiPAP (meaning of acronym)
==========
Date   : Fri, 16 Feb 2001 08:47:11 -0500
Subject: Meaning of the "Bi-PAP"

In response to the person who asked about the BiPAP:

BiPAP stands for Bi-level Positive Airway Pressure. The bi-level ventilator
delivers two preset levels of pressure:  one level of air pressure is for
inspiration and the other for exhalation.  Thus, the "Bi" stands for the
two levels of pressure and the "PAP" for positive airway pressure.  The
bi-level ventilator was first introduced in 1990 by Respironics, Inc.
(Pittsburgh, PA), and the manufacturer named their ventilator, the "BiPAP."

Pamela A. Cazzolli, R.N.
ALS Clinical Nurse Consultant
Canton, Ohio

(3)
=====
News from ALSA - North Texas Chapter
==========
Date   : Fri, 16 Feb 2001 14:36:42 -0600
Subject: for digest

News from the ALS Association / North Texas Chapter:

Important Meeting:  Walk to D'Feet ALS

March 1st, 6:30 pm
Junior League of Lubbock Headquarters
4205 84th, Lubbock, TX (behind Maloufs)

Please bring:

Anyone who would like to join us in raising money and awareness to fight
ALS by working on the Walk to D'Feet ALS Team.  (friends, family, PALS,
CALS, co-workers, church members, service organization members, etc.)

any information that would be helpful to share with our group to make this
a successful event.

and most importantly - enthusiasm and great ideas!

Please contact Gina Six or Linda Gibson for more information:

-- Help us "Strike Out Lou Gehrig's Disease" as we Walk To D'Feet ALS on
   October 7, 2001 Lone Star Park, Grand Prairie Texas

If you know of a business that would like to become a Corporate Sponsor
for this walk or if you would like more information yourself call,
817-226-4257 and leave a message for Gina or Linda Gibson or e-mail us

ALSA North Texas Chapter

(4)
=====
on line books
==========
Date   : Thu, 15 Feb 2001 20:51:55 -0500
Subject: on line books

Here are a couple of sources for on line reading for PALS who can't
turn pages.

University of {*filter*}ia

http://xroads.{*filter*}ia.edu/~HYPER/hypertex.html

Project Guttenberg

http://www.***.com/

take care,  Wayne

(5)
=====
Pulmonary Protocol : Ed White
==========
Date   : Fri, 16 Feb 101 10:31:25 PST
Subject: Pulmonary Protocol

Pulmonary Protocol: ED WHITE

I last updated my pulmonary protocol about 2 years ago,
& I thought it would be timely & useful to review this
subject again. I have not made any major changes in my
protocol, but there have been some modifications since my
last review. As I have mentioned before, the timely
introduction of pulmonary care for the ALS patient is
currently the best "drug" to which we have access. The ALS
drug developmental pipeline seems to have about a 2-4 year
vacuum in it, & this makes pulmonary care even more
important. It is our best option currently available for
surviving ALS on a longer term basis.

In my opinion, it is important to introduce ventilation
equipment & all of the variations that go along with this,
including masks, etc. when one's forced vital capacity
(FVC) approaches 75%. This provides enough time for one to
become familiar with all the idiosyncracies involved with
respiratory care. For instance, in my particular situation
it took me about 90 days to get lined out on the equipment
involved & to find the masks that fit my needs. During this
time my FVC dropped precipitously, & in retrospect, I had
waited too long to begin this process. This is certainly
one area where the patient must stay ahead of the curve, &
it is much better to be early than late. The road from
neurology to pulmonology can be very difficult to navigate
&, unfortunately, I have lost many ALS friends in the
process. Attention to detail is a prerequisite to success
in respiratory management.

I currently am using nasal ventilation & I utilize the
volume ventilator primarily during the day & the BiPap at
night. During the day I utilize a system where I have
combined the volume ventilator & the BiPap in a single
integrated system utilizing 1-way valves & modifications to
all the masks such that I can take advantage of the
ventilator's exhaust system for both pieces of equipment.
This allows for easily switching from the ventilator to the
BiPap with the flick of 2 switches. I believe that the
ventilator & the BiPap each have unique advantages &
certain synergistic effects occur when both are used during
a 24 hour period. The ventilator, for me, provides a better
respiratory support system & lung expansion than I can get
from the BiPap. Therefore, I utilize the ventilator
primarily during the day with intermittant use of the
BiPap. However, I find the BiPap more comfortable at night
& more sensitive as my breathing becomes more shallow. In
my opinion, the combination of the two is the best of both
worlds.

The following summarizes the specific equipment &
procedures that make up my pulmonary protocol:
1. During the day, I use a Respironics PLV 100 ventilator.
Although each patient is different, my current settings are
a tidal volume of 1.25 liters & inspiratory pressure of
20-22 cm of H2O. In my case, I seem to like a lot of
pressure. I utilize the Respironics Monarch mask & I have
used this mask for about 5 years. If I switch to the BiPap
during the day I continue to use the Monarch mask. I really
have developed a love affair with the Monarch mask, and
once one gets use to it, it is hard to beat.
2. At night time I use the Respironics STD-30 BiPap with
the diagnostic panal. Again, my inspiratory pressure is
about 20 cm H2O with expiratory pressure of 3-4 cm H2O & a
tidal volume of about 1.2 liters. Breaths per minute is set
at about 10. At night with the BiPap I primarily utilize
the Puritan Bennett nasal pillows. The combination of the
STD-30 with the nasal pillows gives me the sensitivity I
need at night as my breathing becomes more shallow. I also
utilize an in-line humidifier at night as this is very
important to prevent excessive dryness. I recently obtained
a Respironics Synchrony which has more variables and an
alarm system vs. the STD-30. This will eventually take the
place of the STD-30.
3. The pulse oximeter is used to measure O2 saturation
levels & pulse rate. My O2 saturation levels are currently
running 96-99%.
4. I utilize a capnometer to spot check CO2 levels. At
times a {*filter*} gas analysis might be needed for more
accurate & definitive information. With either analysis I
find I am probably somewhat over ventilated & experience
some hypocapnea as my CO2 levels are almost always low or
in the range of 30-32%.
5. For breathing without any equipment & to assist in
coughing, I currently utilize an ambu bag reconfigured with
an 18 inch tube & a mouth piece. Compressions on the ambu
bag result in "air stacking" which allows the lungs to
expand & this is a valuable tool as an aid for coughing.
Also, it is a tool that allows me to be disconnected from
the ventilation equipment for an extended period of time.
It also forces air to the bottom of the lungs allowing for
better expansion & a better exchange of O2 & CO2 in the
alveolae.
6. Occasionally I will need more assistance in coughing &
when this need arises I use the Respironics In-exsufflator.
This equipment is used to assist in coughing & eliminating
secretions. It takes some time to become familiar with this
equipment, but it can be invaluable.
7. For travel I am currently using the Respironics Duet
BiPap. This equipment is very light & compact, but it
provides the inspiratory pressure I require & can easily be
adapted to a vehicle's power source.
8. There are 2 other important points worth mentioning.
First, I have never considered using oxygen & never will.
O2 is not the proper solution for hypoventilation problems,
& if prescribed, one should seek out a second opinion,
preferably from a pulmonologist who understands ALS &
neuromuscular diseases in general. Secondly, I have not had
any respiratory infections for over 5 years. I attribute
this to my pulmonary program, attention to detail, & an
aggressive program of supplements which have greatly
improved my immune system. It is somewhat oxymoronic, but I
have never been healthier in many ways than with ALS.
9. I have installed a backup generator in my home. This
equipment is not used very often, but it is invaluable when
there is a power failure and gives one peace of mind. My
generator runs off of natural gas.

To summarize, the purpose of my pulmonary protocol is
the following:
   1. It is obviously a life support system, & I hope to
maintain my current status on nasal ventilation utilizing
the volume ventilator & the BiPap for as long as possible.
I am now in my 5th year of nasal ventilation.
   2. Having a protocol allows one to monitor his or her
respiratory condition such that we have as clear a picture
as possible concerning that condition & any trends therein.
   3. Having a protocol & starting early allows the patient
to considerably improve the quality of his/her life. Also,
early intervention here helps the patient to evaluate
future respiratory options, such as a tracheostomy. It is
so important to stay ahead of the curve & avoid a crisis
situation. This allows for preplanning &, in my case, I
would feel much more comfortable moving to a trach after
experiencing & educating myself to proper pulmonary care.

Nasal ventilation is a valuable tool for extending
lives, possibly indefinitely. It is the best "drug" we have
at our disposal at this time for extending our life.
However, it does require pre-planning & early intervention
since the transition from neurological care to pulmonary
care can be complicated & difficult. Unfortunately, I found
this out the hard way & fell behind the curve. It is
certainly a very critical area, & it is better to be early
in one's pulmonary care vs late. ALS, in my opinion, is not
invariably fatal & nasal ventilation is an important tool
in supporting this conclusion. New & better ventilation
equipment is being introduced each month & we will be the
beneficiaries of this new equipment. I am now in my 9th
year with ALS with 1/2 of that time being spent on nasal
ventilation, & I feel like I have stabalized & expect to
continue on this protocol for some time with improvements &
modifications along the way. It has been stated that in
life, success depends on 10% of what happens to us & 90% on
how we react to it. This certainly holds true for
respiratory problems associated with ALS. Planning for &
reacting properly to ALS respiratory challenges is a life
or death situation.

Finally, a few other points are worth mentioning. These are
as follows:
1. It's a sad commentary, but ALS research has not been
productive over the last 10 years at the patient level.
Respiratory management is our best bet now for extending
lives. Unfortunately, there are real challenges in making
the conversion from neurology to pulmonology. For instance,
the turnover of respiratory professionals is incredible.
I've had rotating pulmonologists, frequent RT's, (none
lasting more than a year), and various medical suppliers.
There is very little glue in the system, and continuity of
care is hard to find. Many pulmonologists and RT's do not
understand or want to take the time with respiratory
problems resulting from neuromuscular diseases. I could go
on and on about my experiences and those of others, but
suffice to say, the patient must take charge and dictate
his own repiratory program and the timing thereof. After
all, it is the patient and his family that must operate
this equipment and program on an hour to hour and day to
day basis in their home. Complete dependency on someone for
this can be catastrophic. ALSA and MDA clinics can help,
and should be utilized if available to the patient.
However, it still comes down to the patient and his family
to aggressively manage this program if longterm survival is
expected.
2. I will remain on nasal ventilation as long as possible.
I have gotten use to the obstructive nature of the mask
long ago and will not convert to a trach until I have to.
I would not take a chance on losing my voice, which is as
good as the day I was diagnosed, by converting to a trach.
3. For some reason Medicare seems to have a bias against
nasal ventilation, and this is unfortunate. For instance,
Christopher Reeve can remain off his ventilator for about
90 minutes. I'm good for about 4-5 minutes max. Yet, for
some reason, Medicare treats nasal ventilation as an
optional procedure. The only thing optional about it is
whether I want to live or die. Also, nasal ventilation
prevents infections which are more possible with the trach,
and therefore should be favorable to Medicare. I have never
been in the hospital with ALS, and I attribute this to
nasal ventilation.
4. Finally, I have mentioned Respironics equipment a number
of times only because I started with their equipment and I
have had good success. However, there are other good
suppliers of respiratory equipment. For instance,
Pulmonetic Systems has a series of ventilators that provide
both volume ventilation and BiPap capabilities in one piece
of equipment. The equipment is very compact & very mobile.
In the last 10 years we have made wonderful progress in the
area of respiratory equipment, masks, etc. This is one
bright spot in ALS, and the patient should take advantage
of this, as it is the best option we now have for staying
alive. With 80 million baby boomers approaching the age of
ALS, we are about to see an explosion in ALS cases. It is
simply demographics. This will make our 30,000 number look
small, and the incidence and prevalence of ALS, including
all the variations of ALS, will skyrocket. With no
effective longterm {*filter*}, this dictates that we must rely
on respiratory management for the indefinite future as the
best bet we have for surviving this disease.

Ed White
Fredericksburg, TEXAS

=== end of alsd 808 ===