The parents
of children who have a complicated medical diagnosis are often required to
educate themselves about various medical procedures in order to make
potentially life-changing decisions about whether a risk is worth the
reward. For us, one of our first risk v.
reward decisions involved breathing pacers.
When Mighty
Z was born we heard about the "breathing pacemaker" (Avery Bio-medical), a device that can
provide ventilatory support for patients with chronic respiratory insufficiency
whose diaphragm, lungs, and phrenic nerves have residual function. It sounded good, and without knowing the
details, I really wanted Mighty Z to have one put in when she was a baby --
right up until I found out that the procedure the doctors then planned to
employ to install the breathing pacer involved cutting into the phrenic nerve,
and then, I changed my mind.
Let me tell
you about the phrenic nerve. It arises
from the cervical, or neck region, of the spine that supplies movement to the
diaphragm. The body contains a left and
a right phrenic nerve, and they follow different paths, though they both begin
in the neck. The phrenic nerve plays a
crucial role in the respiratory process in that it causes the diaphragm to
contract. Cutting into the phrenic nerve
could damage the nerve, and if that happened Mighty Z could be ventilator
dependent 24/7 for the rest of her life, with no chance of ever getting her
trach out. Allow someone to cut Mighty
Z’s phrenic nerve? Thanks, but no
thanks. The risk was not worth the
reward.
When Mighty
Z was 2, her doctor brought the subject up again. He said that the procedure for performing the
breathing pacers surgery had changed, and that now the surgeons would wrap the
breathing pacers around the phrenic nerve so there would be no damage to it. The new procedure sounded like an
improvement, but I was still hesitant. Mighty Z was doing well with her trach and her
ventilator, so why rock the boat?
I needed to
know more about breathing pacers in order to make a decision about whether they
made sense for Mighty Z, who was surviving without them. I began researching the breathing pacers, and
through my research I learned that a breathing pacemaker consists of surgically
implanted receivers and electrodes, and an external transmitter with antennas
worn directly over the implanted receivers.
The external transmitter and antennas send radiofrequency energy to the
implanted receivers just under the skin, which then convert the radio waves
into stimulating pulses. These pulses
are sent down the electrodes to the phrenic nerves, causing the diaphragms to
contract, and it is this contraction that causes inhalation of air. When the pulses stop, the diaphragms relax
and exhalation occurs. Repetition of
this series of pulses produces a normal breathing pattern.
While a ‘normal
breathing pattern’ sounded like light at the end of the tunnel, the new and
improved procedure for installing the breathing pacers still involved what seemed
to me to be a long and scary tunnel. The
new procedure involved what is unquestionably major thoracic surgery. Specifically, to install the breathing
pacers, the surgeons would need to implant the electrodes an inch and a half
below each nipple, and then dissect all the way up to the collar bone on either
side and wrap the receivers around the phrenic nerve. Yikes!
At that point in time, the surgeon had only performed one implant
surgery on a 13-year old girl, and Mighty Z was almost 3. Was the risk of Mighty Z undergoing major
thoracic surgery (unproven in one so young) worth the potential reward of
freeing Mighty Z from her trach and from her ventilator for up to 12 hours a
day? I wasn’t sure, so again I
hesitated. Some decisions are hard to make quickly (or at all), and I
guess I needed a push.
I got the
‘push’ I needed from Dr. Keens (one of the leading doctors in CCHS). Dr. Keens called me to discuss the
contemplated procedure, and he, too, encouraged me to go forward with the
breathing pacer surgery for Mighty Z. With his voice added to her other doctor’s,
plus all the information I learned through my research, I decided that the risk
was worth the reward.
I allowed
the surgery when Mighty Z was 3.
Afterwards, we had to wait six months to allow the implants to graph to
Mighty Z's body before turning the external receivers on. After the wait period passed, we did just
that. It is now 8 years later, and we
have never looked back. With the
breathing pacers Mighty Z no longer needs her trach.
When Mighty Z is sick and she needs 24-hour ventilator support, she
throws her external receiver in her purse and we are on our way to the
doctor.
I was scared
to make the decision to allow Mighty Z to have the breathing pacer surgery at
age 3, but I’m so glad I did. I had to give Mighty Z the chance to be a typical
kid -- to jump in the pool, to take a bubble bath, to do all the things she
couldn't do with a trach.
For Mighty Z
and our family it was the best choice we ever made. Here is the TV show The Doctors talking about the breathing pacer
http://www.thedoctorstv.com/videolib/init/7035
http://www.thedoctorstv.com/videolib/init/7035
