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 versus reward decisions involved breathing pacers.
Pacer from Avery Biomedical
When Mighty Z was born with Congenital Central Hypoventilation Syndrome (CCHS)—also known as Ondine’s Curse—we heard about the “breathing pacemaker” (Avery Biomedical), 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 involved cutting into the phrenic nerve. Then I changed my mind.
The Phrenic Nerve and Pacer Implantation
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 two, her doctor brought the subject up again. He said that the procedure for performing the pacer 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 diaphragm to contract, and it is this contraction that causes inhalation of air. When the pulses stop, the diaphragm relaxes 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. It 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 collarbone 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 three. 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 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.
Making the Decision
I got the push I needed from one of the leading doctors who cares for children with CCHS. This doctor 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 three. Afterwards, we had to wait six months to allow the implants to graft to Mighty Z’s body before turning the external receivers on. After the wait period passed, we did just that. It is now eight 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 three, 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, take a bubble bath, and 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.