Thursday, June 21, 2012

Mighty Z's Breathing Pacemaker

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

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Sunday, June 17, 2012

Not Quite Rock~Star Status

You might be viewed as a Rock Star for being able to handle a child who, in many ways, is like my Mighty Z -- whatever your child's diagnoses or even if you have no formal diagnosis. People who have typical kids look at you in awe and they should, because you are a Rock Star.



However these people cannot see the real feelings swimming around in your head, or hear the not–so-good-thoughts.  The thoughts of "I can't do this anymore," or “I am so terrified of this syndrome.” Even the REALLY bad thoughts of wanting to run away. I remember driving home from the hospital when Mighty Z was just a baby and the thought ‘just keep driving and never look back’ popped into my head. I am not proud of that, but it did flash through my mind. I quickly banished the thought, and felt guilty for thinking it.



Sometimes I thought it would have been better if I hadn’t gotten pregnant, or maybe, it would have been for the best if my baby hadn't made it. Thoughts like these are really what have gone through my head at different times in Mighty Z’s life. I think to myself if anyone could look inside and see or hear the thoughts that have just popped into my head, they would know I am not a Rock Star.



I don’t think like that now, but when I was first introduced to this journey I balked at it and said I can’t, I can’t do this. While everyone around me kept saying, ”Wow, you are so strong, you are so great, I could never do this, and you are such a Rock Star.” In many ways I wanted to scream that I am not great, strong, or a Rock Star! I am doing everything that I can just to survive the day!


I have made horrible decisions, and sometimes forgotten how to do simple stuff or just forgot to turn on a pulse ox machine. I have allowed doctors to do stuff to Mighty Z that I should never have allowed. I still think of the times I have allowed doctors to let her sleep off the ventilator and yes, in my heart of hearts, was hoping that she really was fine.


These are the thoughts and feelings that I have felt. They are not very nice thoughts and defiantly not Rock Star status thoughts and feelings, but there you have it. I guess I am just human at times.



 Why I am I telling you this? Because we all can’t be Rock Stars all the time.  Sure we can resuscitate our children, change a trach with the skill of a PICU nurse, tube feed our babies, rig up a device to let our paralyzed babies have some sort of movement, change a colostomy bag, and strap on AFO’s without blinking an eye.


All we can do is realize we are not super human and can only do the best we can at the time. Love yourself, be kind to yourself and know you are a Rock Star.


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