Synapse NeuRx User manual

NeuRxDiaphragmPacingSystem®
Patient/CaregiverInformationand
InstructionManual
ForAmyotrophicLateralSclerosis

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TABLE OF CONTENTS
GLOSSARY OF TERMS .............................................................................................. 3
DEVICE DESCRIPTION .............................................................................................. 5
CONTRAINDICATIONS ............................................................................................... 8
INDICATIONS FOR USE ............................................................................................. 8
INFORMATION ON CONDITION OR DISEASE .......................................................... 8
WARNINGS AND PRECAUTIONS .............................................................................. 9
RISKS AND PROBABLE BENEFITS ......................................................................... 12
ALTERNATIVE PROCEDURES AND TREATMENTS ............................................... 17
BEFORE SURGERY: WHAT TO EXPECT ............................................................... 18
SURGERY: WHAT TO EXPECT ............................................................................... 18
AFTER SURGERY: WHAT TO EXPECT .................................................................. 19
FUNCTIONAL FEATURES ........................................................................................ 20
CONDITIONING SESSION ........................................................................................ 22
CONDITIONING WARNINGS .................................................................................... 25
BATTERY INSTALLATION ........................................................................................ 25
ALARMS .................................................................................................................... 28
CARE OF PATIENT CABLE ...................................................................................... 28
CARE OF THE EXITING ELECTRODE WIRES ........................................................ 29
CARE OF EXIT SITES AND CONNECTOR .............................................................. 29
CARE OF EXIT SITES ............................................................................................... 30
HOW TO SHOWER OR BATHE ................................................................................ 34
CLEANING OF COMPONENTS ................................................................................ 35
TROUBLESHOOTING – NeuRx® EPG ...................................................................... 35
BACKUP INDIFFERENT ELECTRODE INTERCONNECT ........................................ 38
CLINICAL STUDIES ................................................................................................... 41
WARRANTY STATEMENT ........................................................................................ 41
TRAVELING WITH THE NeuRx DPS® ....................................................................... 42
SERVICE ................................................................................................................... 42
REPLACEMENT PARTS ........................................................................................... 42
STORAGE AND DISPOSAL OF PARTS ................................................................... 42
SPECIFICATIONS (DETAILS OF DEVICE OPERATION) ......................................... 43
LABEL SYMBOLS ...................................................................................................... 44
USER ASSISTANCE .................................................................................................. 45

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GLOSSARY OF TERMS
 Alcohol Wipe – individually packaged pad saturated with 70% isopropyl alcohol.
Used to cleanse the skin’s surface. For single use only.
 ALS (amyotrophic lateral sclerosis) – See the section of this manual called
Information on Condition or Disease.
 Arrhythmia – when your heart is beating either very slow, very fast or it feels like it
skips a beat. It is also called an irregular heartbeat.
 Arterial Blood Gas (ABG) – a blood test that measures the amounts of gases in
the blood. The gases measured are oxygen and carbon dioxide.
 Backup Indifferent Electrode Interconnect – can be used instead of the
“indifferent electrode” if that part fails. (“Indifferent Electrode” is explained further
down in this glossary.) The difference is that it goes on the skin instead of under
the skin.
 Capnothorax – a complication caused by air going through the diaphragm and
into your chest during the surgical procedure.
 Chronic Hypoventilation – state in which too little air enters the lungs. This
causes low levels of oxygen and high levels of carbon dioxide in the blood. This
can be due to breathing that is not deep enough or too slow.
 Conditioning – use of the NeuRx DPS® to cause the breathing muscle
(diaphragm) to contract.
 Connector Holder – plastic shell which holds the electrode connector against the
skin. Sticks to the skin with an adhesive like a bandage.
 Covering Bandage – a bandage that covers the electrode wires where they come
out through the skin.
 Diaphragm – the main muscle that is used for breathing.
 Diathermy Treatment – deep tissue heat treatment using energy from an
electrical device.
 DPS – See NeuRx DPS®.
 ECG (electrocardiogram) – test to check the electrical activity of the heart. The
test helps to see if there has been a heart attack, an irregular heartbeat, or other
heart problems.
 Electrode Connector – a part of the NeuRx DPS® which connects the electrode
wires to the patient cable.
 Electrode Wire – See Percutaneous Electrode Wire.
 EPG (NeuRx DPS® External Pulse Generator) (stimulator) – the part of the NeuRx
DPS® System that puts out the electrical signal which stimulates the diaphragm. It

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runs on batteries. It is outside the body. It is connected (through the electrode
connector) to the electrode wires which go into the diaphragm.
 Exit Site (electrode wire exit site) – place where an electrode wire passes
through your skin.
 Forced Vital Capacity (FVC) – the volume of air that one can exhale from the
lungs with force.
 Indifferent Electrode – the one wire which is placed under the skin in the chest
area.
 Laparoscope – a small surgeon’s tool with a camera on the end. The surgeon
puts it into the abdomen through a half inch long incision. It lets the surgeon look
inside the abdomen without the need for a large incision.
 Maximum Inspiratory Pressure (MIP or PImax) – a measure of how well one can
breathe in.
 NeuRx DPS® (Diaphragm Pacing System) – device which sends a small amount of
electricity to the diaphragm muscle. This makes the diaphragm contract. Parts
include the electrode wires, the external pulse generator (“EPG”) box, and the
patient cable.
 Non-Invasive Ventilation (NIV) – breathing help from a small machine that uses a
mask that fits over the nose or nose and mouth.
 Patient Cable – cable which connects the NeuRx® EPG (external pulse generator
box) to the electrode wires. It carries the electrical signal from the NeuRx EPG® to
the wires.
 Percutaneous Electrode Wire – thin wire which is used to send a small amount of
electricity to the diaphragm muscle. One end of the wire is implanted in the
diaphragm. The other end is inserted in an electrode connector outside the body
over the chest area.
 Percutaneous Endoscopic Gastrostomy (PEG) – a tube through which nutrition
is given. It is inserted by a surgeon through the skin and into the stomach.
 Phrenic Nerves – the nerves which carry the signals that tell your diaphragm to
either inhale or exhale.
 Pulmonary Function Test (PFT) – breathing tests that show how well your lungs
are working (exhaling and inhaling). One such test checks the maximum amount
of air you can force out of your lungs (forced vital capacity or FVC). Another test
checks how strong your breathing muscles are when you breathe in (maximum
inspiratory pressure or MIP).
 Stimulator – See EPG (External Pulse Generator).

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DEVICE DESCRIPTION
The name of the device is the NeuRx Diaphragm Pacing System (DPS)®. The NeuRx
DPS® has implantable parts and external parts. The implantable parts consist of five
electrode wires which go through the skin. The tips of four electrode wires are placed
into the diaphragm by your surgeon. The diaphragm is the breathing muscle. The tip
of the fifth electrode wire is placed under the skin in the chest area to complete the
wiring. The other ends of the five electrode wires come out through the skin in the
chest area. The outside ends of the electrode wires will be placed in an electrode
connector. The electrode connector is attached to the skin with the connector holder.
The connector holder has an adhesive patch like a bandage. The electrode connector
has a socket to plug in one end of the patient cable. The other end of the patient
cable is connected to the NeuRx® EPG. The NeuRx® External Pulse Generator (EPG)
is a stimulator box.
The patient kit contains the external parts. These parts include the NeuRx® EPG,
patient cables, batteries and connector holders. The patient cable goes between the
EPG and the electrode connector. The EPG is programmed by your healthcare
provider. The EPG sends electrical signals to the diaphragm. The signal causes the
muscle to contract in order to condition the muscle.
See Figure 1 on page 6 for a picture of the NeuRxTM EPG, patient cable, connector
holder, electrode connector, and electrode wires. See Figure 2 on page 7 for a
drawing showing where the electrode wires are implanted in the body. This drawing
also shows the position of the electrode wires, the patient cable, and the NeuRx EPG.

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Figure 1. NeuRxTM External Pulse Generator (EPG), Patient Cable, Connector
Holder, Electrode Connector, and Electrode Wires.
Electrode
Wires
Electrode
Connecto
r
Patient
Cable
Connector
Holde
r
NeuRx®
External
Pulse
Generator
(EPG)

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Figure 2. Location in the body where the four NeuRxTM DPS electrode wires (A) are
implanted in the diaphragm. A yellow arrow points to each of the four wire implant
locations. The patient cable (B) carries the electrical signal from the EPG (C) to the
electrode wires (A) which stimulate the diaphragm to contract.
A
B
C

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CONTRAINDICATIONS
Are there Any Reasons Why You Should Not Use the NeuRx Diaphragm Pacing
System (Contraindications)? None.
INDICATIONS FOR USE
HUMANITARIAN USE DEVICE
Humanitarian Device. Authorized by Federal law for use in the treatment of chronic
hypoventilation in ALS patients. The effectiveness of this device for this use has not
been demonstrated.
CAUTION: Federal law (USA) restricts this device to sale, distribution and use by or
on the order of a physician.
The NeuRx Diaphragm Pacing System (DPS)® is a device. It is meant for patients
who have both ALS and diagnosed breathing problems. For the NeuRx DPS® to
work, your breathing muscle (diaphragm) must be able to respond to stimulation. This
can be shown before surgery by a diaphragm movement test or by a breathing nerve
test. You must be able to exhale at least 45% of the air that a typical healthy person
could (in an “FVC” test). You must also be 21 years of age or older.
The breathing nerves (phrenic nerves) provide a path from the spinal cord to the
diaphragm. It is over this path that your brain sends messages to inhale. Both the
right and left phrenic nerves must be at least partially working for the NeuRx DPS® to
work. Your phrenic nerves can be tested by your neurologist.
See BEFORE SURGERY: WHAT TO EXPECT for information about these tests.
INFORMATION ON CONDITION OR DISEASE
Amyotrophic lateral sclerosis (ALS) is a disease that affects nerve cells in the brain
and the spinal cord. Certain nerve cells called motor neurons reach from the brain to
the spinal cord and from the spinal cord to the muscles throughout the body. When
the motor neurons die because of ALS, the ability of the brain to start and control
muscle movement is lost. Patients in the later stages of the disease may become
totally paralyzed.
Often ALS affects the motor neurons which reach to the muscles used in breathing. In
this way, ALS can cause the breathing problems described in the Indications for Use
section above.
For more about ALS see the website of the ALS Association: www.alsa.org/about-als

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WARNINGS AND PRECAUTIONS
WARNING: If you use the device full time, then you must always have a back-up
means of breathing help (ventilation) available. If you do not feel that you are
receiving enough breathing help from the NeuRx DPS®, then you should use your
back-up means right away. If you think there is any problem with your NeuRx DPS®,
then you should use your back-up means right away. When you switch to your back-
up means of breathing help, you should turn off the NeuRx DPS®.
WARNING: If this device is not used properly, it may cause injury or electrical hazard.
Follow your healthcare provider’s directions in the use of the NeuRx DPS®.
WARNING: Do NOT use the NeuRx DPS® if you are pregnant. It is unknown if it is
safe to use the NeuRx DPS® if you are pregnant. The clinical trial did not involve
pregnant women.
WARNING: Avoid eating or drinking while conditioning with the NeuRx DPS®. There
is a risk of food or liquid entering your lungs. Do NOT have any food or liquid in
your mouth when you start conditioning. If you use the NeuRx DPS® full time, talk
to your healthcare provider who is helping you with your NeuRx DPS® about the ways
to reduce the risk of food or liquid entering your lungs.
WARNING: NeuRx DPS® could interfere with some medical equipment. Some
medical equipment could interfere with the NeuRx DPS®. Call your healthcare
provider who is helping you with your NeuRx DPS® before having any of the following:
 Implanted cardiac pacemaker or defibrillator. The NeuRx DPS® may
interfere with these devices. There is not enough information to know for sure
whether your NeuRx DPS® can be used safely with these devices. Your
healthcare provider should get an opinion from a specialist. Your healthcare
provider should also test whether your NeuRx DPS® interferes with devices like
these if you need them.
 Surgery. Use of high-frequency surgical equipment may cause burns where
the electrode wires pass through the skin. It might also damage the NeuRx®
EPG if connected.
 Magnetic Resonance Imaging (MRI) test. The NeuRx DPS® has not been
tested with MRI. MRI could cause the electrode wires to move. MRI could also
cause unwanted tissue heating through the electrode wires.
 Diathermy treatment. Diathermy treatment is deep tissue heat treatment. It
should not be done around the implanted electrode wires. Unwanted tissue
heating through the electrode wires could occur.

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 External electrical stimulation such as transcutaneous electrical nerve
stimulation (TENS). Such stimulation should not be done in the chest area
near the electrode wires. Unwanted diaphragm contraction could occur.
 Shortwave or microwave therapy. Operating the NeuRx DPS® close to
(about 3 feet from) such equipment may interfere with your NeuRx DPS®.
WARNING: Do NOT use this device if the skin around the electrode wires gets
swollen, infected, or inflamed. Call your healthcare provider who is helping you with
your NeuRx DPS®.
WARNING — FLAMMABILITY: Do NOT use the NeuRx® EPG in rooms or spaces
where oxygen is being used. This could cause a fire.
CAUTION: Federal law (USA) restricts this device to sale, distribution and use by or
on the order of a physician.
CAUTION: If you think the device is not providing enough stimulation, then call your
healthcare provider who is helping you with your NeuRx DPS®. This could mean that
the NeuRx DPS® may not cause your diaphragm to contract.
CAUTION: Some patients may feel skin irritation or sensitivity because of the NeuRx
DPS®. This may be due to the stimulation. It may also be due to the adhesive on the
skin bandage. It could also be due to the transparent dressing used over the gauze
that covers the electrode wires. (Tegaderm™ and Op-Site™ are examples of
transparent dressings.) Call your healthcare provider who is helping you with your
NeuRx DPS® if this occurs. Irritation can usually be reduced by changing the settings
of the EPG or removing the adhesive.
CAUTION: Avoid touching the patient cable or electrode wires to other metal objects.
Metal objects can conduct electricity. You may accidently get shocked from another
metal surface.
CAUTION: To avoid damage to the device, keep this device out of the reach of
children.
CAUTION: Do NOT attempt to open the NeuRx® External Pulse Generator (EPG).
Doing so can damage the device.
CAUTION: To protect the NeuRx® EPG against damage due to mechanical shock:
 Do NOT drop the NeuRx® EPG. The EPG may break and not be available for
use when needed.
 Use the protective black carrying case provided for transporting the NeuRx®
EPG when not in use.

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CAUTION: To protect the NeuRx® EPG against damage due to moisture, do NOT
allow the NeuRx® EPG to get wet. The EPG may quit working and not be available
for use when needed.
CAUTION: Do NOT get wet while using the NeuRx® EPG. This includes bathing,
showering, swimming, or any other activity in which you could get wet. While the
NeuRx® EPG is designed to keep moisture out, the EPG is not waterproof. If the
location where the electrode wires pass through your skin (the exit sites) gets wet, it
may affect treatment. You may receive less stimulation. You may also feel the
sensation of the stimulation. Take steps to keep the exit sites from getting wet.
Protect the exit sites with a gauze pad and transparent dressing (such as Tegaderm™
or Op-Site™). If the exit site gets wet, clean it with an alcohol wipe. Use an
individually packaged pad saturated with 70% isopropyl alcohol. Allow the alcohol to
air dry before use.
CAUTION: Do NOT have the EPG connected during any type of electrical
diagnostic test such as an electromyogram (EMG) or electrocardiogram (ECG).
The use of the EPG during such tests could interfere with those tests.
CAUTION — ELECTROMAGNETIC INTERFERENCE: Some electrically powered
equipment gives off electromagnetic waves which could interfere with your NeuRx®
EPG.
 When using your NeuRx® EPG around electrical equipment, check the NeuRx®
EPG screen to make sure the EPG is working.
 To reduce the possibility of interference from other electrical equipment, do
NOT use cables or accessories with your NeuRx® EPG other than those
specified.

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RISKS AND PROBABLE BENEFITS
INTRODUCTION
The NeuRx DPS® System was evaluated in a clinical study. The study involved 106
patients at 9 hospitals (8 in the U.S. and 1 in France). From the 106 patients in the
study, 86 would have met the indications for the NeuRx DPS®. The results discussed
here are from those 86 patients. The results show that the benefits of using the
NeuRx DPS® System outweigh the health risks. Risks and probable benefits of the
NeuRx DPS® are described below. The results of the clinical study are also
described.
SURGICAL PROCEDURE RISKS
There are risks of the surgery to implant the electrode wires. These serious adverse
effects were seen in the study:
 Breathing failure occurred in one of the 86 patients in the study after surgery. This
patient also had a feeding tube inserted during the DPS surgery. The feeding tube
moved out of place and caused problems. Those problems led to the patient
needing breathing support, through a tube in their throat, from a breathing
machine.
 Problems caused by the abdominal air getting through the diaphragm and going
into the chest cavity during the surgery (capnothorax). This happened in 15 out of
86 patients in the study (in about 1 out of every 6 patients). Two of these patients
(in about 1 out of every 43 patients) needed special treatment for this problem. In
one case, a small tube was placed in the chest for a short time. In one case, the
patient had to stay in the hospital longer than normal. In the other 13 patients (in
about 1 out of every 7 patients), the problem went away on its own.
 Reaction to anesthesia. One patient had a serious reaction to anesthesia. After
starting anesthesia in the operating room the patient had a low heart rate and low
blood pressure. The surgery was then cancelled before any incision was made.

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Table 1. Serious adverse events from the study which related to the device or the
surgery.
Serious
Adverse Event
Reported
Occurrence
(Likelihood) of
Serious Adverse
Event
Adverse Effect
Reported
Occurrence
(Likelihood) of
Adverse Effect
capnothorax
2 out of 86
patients
(1 out of every 43
patients)
(2.3%)
catheter placement
1 out of 2
patients who
had serious
capnothorax
extended
hospitalization
1 out of 2
patients who
had serious
capnothorax
respiratory
failure
1 out of 86
patients
(1.2%)
tracheostomy,
mechanical ventilation
1 out of 1
patient who had
respiratory
failure related to
the surgery
anesthesia
reaction
1 out of 87*
patients
(1.1%)
low heart rate
(bradycardia), low blood
pressure (hypotension),
and cancellation of
surgery
1 out of 1
patient who had
serious
anesthesia
reaction
*87 patients = the 86 patients who were implanted and the 1 patient who was not
implanted because of anesthesia reaction
There are other surgical risks which were not seen in the clinical study. These include:
 Nerve, tissue or organ damage as a result of the procedure.
 Irregular heartbeat (cardiac arrhythmia) when the electrode wires are implanted
in the chest.

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DEVICE RISKS
Once the surgery is over and you have recovered, there are risks associated with
having the NeuRx DPS® System. The following adverse effects were seen in the
study but none were considered serious:
Table 2. Adverse events related to the device (other than due to surgery). All were
non-serious.
Device
Adverse Event
Reported
Occurrence
(Likelihood) of
Device Adverse
Event
Notes
(About Adverse Effects,
Resolution, etc.)
infection where
electrode wire
passes through
skin (exit site)
8 out of 86
patients
(1 out of every 11
patients)
 7 resolved with antibiotics.
 1 resolved after the wires were adjusted
in the healthcare provider’s office.
 None required removal of the wires.
discomfort from
stimulation
22 out of 86
patients
(1 out of every 4
patients)
 Resolved in 20 patients.
 Unresolved in 2 patients but tolerable
(the patients continued using the NeuRx
DPS®).
wire leading to
diaphragm –
break outside
the body
28 out of 86
patients
(about 1 out of
every 3 patients)
 Affected wire does not deliver therapy
until repaired.
 Requires healthcare provider
appointment to repair.
 No patients had to return to surgery to
fix.
wire under skin
in chest area
(indifferent
electrode) –
break outside
the body
18 out of 86
patients
(about 1 out of
every 5 patients)
 The NeuRx DPS system does not
deliver therapy until repair.
 Requires healthcare provider
appointment to repair.
 No patients had to return to surgery to
fix.

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Device
Adverse Event
Reported
Occurrence
(Likelihood) of
Device Adverse
Event
Notes
(About Adverse Effects,
Resolution, etc.)
wire under skin
in chest area
(indifferent
electrode)
came out of the
body
6 out of 86
patients
(in about 1 out of
every 14 patients)
 No patients had to return to surgery to
fix.
 Fix involves reinserting the wire under
the skin in the healthcare provider’s
office under topical anesthetic.
 Patient may temporarily use an
alternate part (the backup indifferent
electrode interconnect) until an
appointment can be made for the
healthcare provider to reinsert the wire
under the skin.
broken NeuRx®
EPG
(stimulator)
6 out of 86
patients
(in about 1 out of
every 14 patients)
No NeuRx DPS® therapy can occur
without a working NeuRx® EPG. A
replacement NeuRx® EPG can be shipped
to the patient.
broken patient
cables
4 out of 86
patients
(in about 1 out of
every 22 patients)
No NeuRx DPS® therapy can occur
without a working patient cable. Two
cables are provided with each kit. The
patient cables are meant to be disposable.
More replacement cables can be shipped
to patient.
pain where
electrode wire
passes through
skin (exit site)
3 out of 86
patients
(about 1 out of
every 29 patients)
 All 3 cases were “mild” pain.
 One case resolved in 12 days. One
case resolved in 5 days.
 One case did not resolve. The
healthcare provider’s report described
said that pain was “really mild and
occurs rarely”.
skin irritation or
inflammation
5 out of 86
patients
(about 1 out of
every 17 patients)
 All 5 cases were “mild” in severity.
 All cases resolved within a month and a
half (except one case in which the time
to resolve was unknown). The average
time to resolve was 18 days. The
longest known time to resolve was 43
days.

16
There are some device risks which were not seen in the study but are considered
possible. These device risks include:
 Food or liquid entering your lungs while using the device.
 Bleeding where the electrode wire tip is attached to the diaphragm muscle.
 Electrode wire breaks inside the body. This could lead to a loss of therapy or
less therapy. It could require another surgical procedure to correct.
 Allergic reaction to the materials which are used in the electrode wires.
 Use of the NeuRx DPS® causing problems with devices such as heart
pacemakers and defibrillators. (See the warning about this under Warnings
and Precautions in this manual.)
 Problem interactions with some medical equipment:
o High-frequency surgical equipment may cause burns where the
electrode wires pass through the skin. It might also damage the NeuRx®
EPG if connected.
o MRI could cause the electrode wires to move. MRI could also cause
unwanted tissue heating through the electrode wires.
o Diathermy treatment equipment used for deep tissue heat treatment.
Unwanted tissue heating through the electrode wires could occur.
o External electrical stimulation such as transcutaneous electrical nerve
stimulation (TENS). If this is performed in the chest area near the
electrode wires, unwanted diaphragm contraction could occur.
o Shortwave or microwave therapy. Operating the NeuRx DPS® close to
(about 3 feet from) such equipment may interfere with your NeuRx
DPS®.
o Electrical diagnostic test equipment such as an electromyogram (EMG)
or electrocardiogram (ECG). The use of the EPG during such tests could
interfere with those tests.
 Impeding treatment if the location where the electrode wires pass through your
skin (the exit sites) gets wet.
 Electromagnetic interference: Some electrically powered equipment gives off
electromagnetic waves which could interfere with your NeuRx® EPG.
 Accidently getting shocked from another metal surface if the patient cable or
electrode wires touch other metal objects.

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PROBABLE BENEFITS
You may get help from the NeuRx DPS® because in a study:
 The patients who used the NeuRx DPS® plus non-invasive ventilation (NIV)
survived 16 months longer (on average) than patients who just used NIV. This
16 month time frame is from the time of diagnosis. Survival time was measured
until death or the need for a full time ventilator and a tube in the throat
(tracheostomy). The patients who just used NIV were similar patients in a
different study.
 The patients who used the NeuRx DPS® plus NIV survived 9 months longer (on
average) than patients who just used NIV. This is from the time they started
using NIV.
 Some patients had a feeding tube placed with the NeuRx DPS®. All of these
patients survived past 30 days. Normally, 2 to 25 patients out of 100 (up to a
fourth of all patients) would not survive this long.
 Some patients could not get used to or were unable to use NIV but used the
NeuRx DPS®. They survived an average of 16 months after getting the NeuRx
DPS®.
 Some patients had their sleep tested after using the NeuRx DPS® for 4 months.
They tended to have better sleep than before they received NeuRx DPS®.
ADDITIONAL INFORMATION
Another study was done after the Food and Drug Administration approved the NeuRx
DPS®. The “post-approval study” involved 60 patients at 10 hospitals in the U.S. The
study results are in a report called the “Post-Approval Clinical Study Summary”
(document 77-0088) available from your doctor or Synapse Biomedical, Inc. (To
request a copy, call Synapse Biomedical Customer Service at 1-440-774-2488
extension 137 or email [e-mail].)
ALTERNATIVE PROCEDURES AND
TREATMENTS
Other breathing treatments exist for patients with ALS. These include non-invasive
ventilation through a mask or invasive ventilation with a tube through the throat.
Non-invasive ventilation (NIV). The common device used for NIV is a bilevel or
continuous positive airway pressure machine. These supply air to the mouth or nose.
The choice of mask depends on fit, air leaks, looks, and claustrophobic concerns. A
number of current papers show NIV may help patients with ALS. Current treatment

18
advice is that all patients with ALS and respiratory symptoms, or an FVC less than
50%, should be offered NIV. In general, NIV may be used with the NeuRx DPS®.
Mechanical ventilation. At some point, ALS reduces the ability to breathe so much
that invasive ventilation becomes the only choice for survival. Invasive ventilation
uses a tube placed through the throat connected to a ventilator. This can prolong life
for several years.
BEFORE SURGERY: WHAT TO EXPECT
Your healthcare provider will need to do breathing tests to see if your breathing
problems can be treated using the NeuRx DPS®. These tests include checking how
well your lungs are working (breathing out and breathing in). One test checks the
maximum amount of air you can force out of your lungs (forced vital capacity or FVC).
Another test checks how strong your breathing muscles are when you breathe in. A
blood test may be done to see how much oxygen and carbon dioxide are in your
blood. In another test, a small monitor is placed over your finger during sleep. The
monitor measures the level of oxygen in your blood. The results of any one of these
tests can show if you could be a candidate for the NeuRx DPS®.
SURGERY: WHAT TO EXPECT
The surgeon will make a short cut (incision) in the skin of your abdomen. This incision
will give the surgeon access to your diaphragm. The incision will be about half an inch
long. A tube will be placed into the incision. Carbon dioxide gas will be pumped
through the tube to fill your abdomen. A small tool with a camera on the end
(laparoscope) will be inserted into the tube in your abdomen. After the surgeon looks
into your abdomen, 3 more incisions will be made. These incisions will also be about
half an inch long. A tube will be placed in each incision for the surgeon to work
through.
The surgeon will then insert a tool called a probe through one of the tubes. The probe
will help the surgeon find the best location to place the electrode wire tips in the
diaphragm. The surgeon will use the probe to test several locations on the
diaphragm.
Once the surgeon has found the 4 best locations, the surgeon will remove the probe.
The surgeon will then insert the tips of 4 electrode wires in these locations. The
surgeon will put the other ends of the 4 electrode wires on the outside of your body.
These 4 wires will come out through your skin in the same area. A fifth electrode wire
is then placed just beneath the skin in the same area to complete the wiring.

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After surgery, you will be able to see about 1 to 2 inches of each electrode wire
outside your body.
After surgery you may have an x-ray. This is done to check for abdominal air that may
have gone to your chest (capnothorax). If needed, a tube may be placed in your chest
to remove the carbon dioxide. After the surgery, your surgeon will decide how long
you will have to stay in the hospital.
AFTER SURGERY: WHAT TO EXPECT
Your NeuRx DPS® may be implanted as a same-day surgery. Depending on your
condition before and after surgery, you may need to spend the night. If you have a
second procedure at the same time as your DPS implant you may need a longer stay
in the hospital. You should discuss this with your surgeon prior to surgery.
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While you are recovering from surgery, a healthcare provider will adjust the settings
on your NeuRx® EPG. The purpose is to give the right amount of stimulation to your
diaphragm. If the stimulation makes you uncomfortable, tell your healthcare provider.
He or she can change the settings to reduce or eliminate the discomfort.
Before you leave the hospital, the staff will give you detailed instructions on how to
use the NeuRx DPS®. You will also be given this manual. You will use the NeuRx®
DPS to condition your diaphragm. In conditioning, the NeuRx® EPG sends a small
amount of electricity through the wires to your diaphragm. This causes your
diaphragm to contract. When your diaphragm is conditioned, it will do a better job of
helping your breathing. See the sections on Conditioning Sessions and Conditioning
Warnings for information about conditioning your diaphragm.
You will need to keep dry while using the NeuRx® EPG. The NeuRx® EPG is not
waterproof. Refer to the section “HOW TO SHOWER OR BATHE” on page 34 for
instructions on bathing, showering, swimming, or any activity during which you could
get wet.
If your NeuRx® EPG is properly cared for, the device should keep working for years.
However, you will need to change the battery in your NeuRx® EPG about every 500
hours of use. The following sections will walk you through the use, care and
troubleshooting of the NeuRx® EPG.

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FUNCTIONAL FEATURES
To turn the NeuRx® EPG ON: Press and release the two buttons on the front of the
NeuRx® EPG at the same time.
To turn the NeuRx® EPG OFF: Press and release the two buttons on the front of the
NeuRx® EPG at the same time.
The buttons must be pressed at the same time. This is a safety feature to guard
against accidentally turning the EPG on or off (Figure 3).
Figure 3. On/Off Buttons.
The NeuRx® EPG has a screen that shows what the NeuRx® EPG is currently doing
and if it is working properly.
The NeuRx® EPG screen shows the breaths-per-minute (BPM) and when the
individual electrode wires are active. When the EPG sends electricity to the
diaphragm to trigger inhaling, a letter “A”, “B” or “C” is shown below each of the
electrode wire numbers (1, 2, 3, and 4). This shows that the NeuRx® EPG is working
properly. The example in Figure 4 below shows the NeuRx® EPG with “AAAA” on its
screen. This means that it is working properly during inhaling. When any letter “A”,
“B” or “C” appears below the electrode wire numbers 1, 2, 3, and 4, it means that the
NeuRx® EPG is working. Other examples could be “AABB”, “BABA”, “BACC”,
“BBBB”, “BBCC”, “CCCC”, etc., just as long “A”, “B” or “C” appears below each of the
four electrode wire numbers.
Figure 4. “A”, “B” or “C” showing that the NeuRx® EPG is working properly during
inhaling for each electrode wire (number 1, 2, 3, or 4).