Cardiac Crisis

After Mighty Z had her loop recorder placed on her heart I felt relieved, relieved to know that if Mighty Z’s heart was pausing, now we would catch it. The first month that we downloaded her loop recorder through the telephone lines, I received a wonderful call from the EP (electrophysiology) nurse saying Mighty Z’s heart showed no signs of pausing (whew). The same with the next month when I downloaded Mighty Z’s loop recorderand I felt like we were on a roll, until Mighty Z came and told me she had had another episode.

 

 I know what you’re thinking. Why wasn’t I there when this episode happened and what is  an “episode?.”Sometimes, not all the time, Mighty Z will faint -- it is very random and you have no idea when it is going to happen or what triggers it. Since Mighty Z is twelve, she hates when I follow her around like a lost puppy and, let’s face it, it gets annoying for both Mighty Z and myself.  So, I let her walk around without me tagging along after each step. Mighty Z told me this “episode” was not a bad one, it was just a little episode. However, being who I am, I quickly downloaded Mighty Z’s loop recorder and waited once again for the EP nurse to call. Waves of dread came over me because I knew Mighty Z’s heart had paused, and I knew that it meant she would have to have a cardiac pacer implanted. This was confirmed when the EP nurse called and said that Mighty Z’s heart had paused for 4 seconds and then again for 5 seconds. Knowing what I know, that was almost double the legal limit for a heart to pause, so I waited to have confirmation from her cardiologist. Within minutes Mighty Z’s cardiologist called and told me the heart-breaking news, that yes indeed, Mighty Z would need a cardiac pacemaker implanted.

 

 

 I always knew that it wasn’t IF Mighty Z would get the cardiac pacemaker, but that it was WHEN. When would Mighty Z’s heart stop? And would I be able to catch it in time to save her life? I will confess, I put on a brave face, called Mighty Z’s daddy, and both grandma’s and calmly told them that Mighty Z’s heart was pausing and that she would need to have a cardiac pacer implanted. My voice was strong with no waver to it as I called each person to relay the news. It wasn’t until that night when the tears started coming and the fear and anxiety started to wash over me. I first got mad at myself, because you see, I knew Mighty Z’s heart was pausing and I kept pushing the cardiologist to listen to me. As I lay in bed and cried, I felt like I had gone on a witch hunt looking for Mighty Z’s heart to pause and I finally found a witch. Yes, I know completely irrational, however Mighty Z was going to have to endure so much more and I wanted to spare her this, even though I knew this was so important.

 

The cardiac pacemaker surgery was set for the next Monday and I was ok until the Saturday and Sunday before. I had to breathe through those waves of fear and anxiety as they attacked me over and over again.

 

When Monday morning came, I bravely packed Mighty Z and my bags and headed up to Children’s Medical Center of Dallas Texas to be admitted. Once through admitting, we were brought back for pre-op, and Mighty Z was given what I always call a cocktail (a mixture of 2 vials of Versed and 4 vials of Lortab, in what looks like a shot glass). With Mighty Z already on her breathing pacemakers, I felt confident that she would be fine, however once she fell asleep, she needed to be bagged (an ambu bag is a hand-held device frequently used by trained professionals as an essential part of resuscitation to provide positive pressure oxygen to a patient whose breathing is insufficient, or has ceased completely. The act of using the device is frequently referred to as bagging. The ambu bag has a long tube that connects directly to an oxygen outlet or tank to force 100% oxygen into a patient’s lungs). For a couple of breathes the EP Nurse and I worked on Mighty Z and then Mighty Z was able to breathe with the breathing pacers , with just a bit of  oxygen to help her. Even though that was a bit scary, I still felt comfortable with the cardiac surgery. The surgery lasted for about three hours and the EP surgeon came out and told us that Mighty Z did great and that we could see her in recovery. When her daddy and I got to recovery, Mighty Z was responsive, yet groggy. Her oxygen levels were great without the need to supply extra oxygen.  The only issue was that Mighty Z’s blood pressure was going crazy -- at some points it was reaching 160/102 and then dropping to 74/50 (normal blood pressure is below 120/80). Mighty Z’s heart rate was spiking to 180 and then dropping to 50 and each time it dropped to 50 we could see on the monitor Mighty Z’s shiny new cardiac pacemaker go off. The cardiac pacemaker went off a total of nine times just in the first hour after her surgery.

 

 

 

Then things started unraveling quickly…Mighty Z started needing oxygen -- not just a little bit -- we are talking 5 liters of oxygen and then Mighty Z’s daddy noticed that her breathing pacers were not breathing for her like they were designed. Quickly her daddy and I began to work on Mighty Z. We changed the antennas to her breathing pacers, then we repositioned them, and then we realized Mighty Z was not breathing at all. I called the PACU (post anesthesia care unit) nurse and told her Mighty Z was not breathing.  Quick as a flash, the PACU nurse started bagging Mighty Z as I hyper extended Mighty Z’s neck and held the mask securely to her face. The PACU nurse then called the attending in the PACU, saying that Mighty Z was unresponsive and not breathing at all. The attending came over and started bagging Mighty Z and had the PACU nurse call the CICU (Cardiac Intensive Care Unit) doctor and have him come down STAT!! The CICU doctor came down and bagged Mighty Z for 30 minutes and then magically her breathing pacers started breathing once again for Mighty Z, however Mighty Z was still unresponsive. The CICU doctor gave her six shots of Narcan (a narcotic reversal) as the CICU nurses pinched and prodded Mighty Z’s little body to get some sort of reaction out of her. It wasn’t until the 6^th shot of Narcan that Mighty Z finally opened one eye.  We tried to keep her responsive without much luck until several hours later. Finally at 9:30 pm, Mighty Z was able to wake up and talk, although it took tons of stimulation from her daddy and I, and unfortunately that lasted for only thirty minutes until she once again feel into a deep sleep. She was still requiring five liters of oxygen for the rest of the night until she woke up the next day, still very groggy and blessedly with no idea of the trauma her little body endured (I praise God for that).

Three days later, Mighty Z was back to her old self and the doctors felt comfortable with Mighty Z coming home and of me taking over her care. Since the anesthesia during surgery, Mighty Z has not had a drop of pain medication, simply because we are all afraid that she would once again go in to a drug induced coma.

 

 

 So to answer the obvious question, “What happened to Mighty Z and why did her breathing pacers stop working, then all of a sudden start working again?”  The answer is simple, the anesthesiologist gave Mighty Z Fentynal on top of the Versed and the Lortab  -- it was too much for her little body with all those drugs racing through her system. It made Mighty Z unresponsive and her CO2 levels go through the roof and made her body go into  respiratory acidosis, which basically paralyzed her muscles since the breathing pacers make the diaphragm (and the diaphragm is a muscle) expand and contract.  The acidosis was not allowing her diaphragm to move even though the breathing pacers were telling the diaphragm to move -- the more the doctor bagged Mighty Z and lowered her CO2 levels, the more the breathing pacers were able to make the diaphragm expand and contract. The Narcan reversed the overdose of anesthesia and made Mighty Z responsive again.

 

I am sure you are overwhelmed with this blog post as I was living it.   If it weren’t for Mighty Z’s daddy, the PACU nurse, the attending PACU doctor, the CICU doctors, nurses and I…well, this blog post would have been completely different or probably not written at all. Many times I forget how fragile Mighty Z truly is and what a Mighty Miracle she is to all of us.

edited by Linda Kruger

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to the brokenhearted

“The Lord is near to the brokenhearted and saves those who are crushed in spirit.”

 Psalm 34:18 (NASB)

 

When the doctors first took Mighty Z to the NICU and I was forced to face that there was something seriously wrong with my baby, this scripture spoke volumes to me.

 

I was both brokenhearted and crushed in spirit in many ways.  I felt God’s presence during those dark first months of my journey into the world of special needs.  The world of Special Needs is ‘special’; it’s just special in a different way.  It is a slower paced world, a world where faith is something you cling to daily.  It makes you stop and smell the roses; it makes you thankful for inch stones and you don’t worry about those mile stones. 

 

 

 It’s a world that shakes your normal world to the core, but it brings a new normal to your life.  Some days you feel HIS presence and some days you feel lost and brokenhearted. That is where Psalms 34:18 has given me comfort.  I go back to that scripture more than anyone knows.

 

 

As I looked at my baby during the times that don’t just try the soul but also try the doctors, I knew that even when I feel shattered, brokenhearted, and crushed in spirit, the Lord is near and He has come to save me.  Although I did not want to walk down this path, I know that I am not walking this alone. My Savior is walking right next to me, holding on, not just to me, but to my baby as well.

edited by Emily Minich

01 02 12

The CCHS Fairy

As you all know, Mighty Z has CCHS (Congenital CentralHypoventilation Syndrome), and since CCHS is a neurological disorder, it affects every aspect of Mighty Z’s body. Simply put, many of the neurons in Mighty Z have repeated, haven’t matured like they are supposed too, and are not going to do the tasks that they are meant to do.

 

Because of that, sometimes Mighty Z’s neurons spark and do the task that they supposed to do and sometimes they don’t. It is almost like faulty wiring in a house.  Sometimes the lights flicker, sometimes they don’t turn on at all, and sometimes every light in the house works. It all depends on the day.

 

The CCHS fairy likes to fly around and strike at any moment.  Just last week we had a beautiful five year old with CCHS running around playing and then all of the sudden her heart stopped and she just dropped like a rock and died. CCHS is a very unforgiving disease.  It comes like a thief in the night with no warning, slinking in just when you think all is well and whacking you when you least expect it. 

 

Mighty Z started passing out in September. First I thought it was some vitamins I was giving her, then I thought it was a medication she was on, and then I realized it was something much more serious.  Mighty Z had been passing out, but she was on her breathing pacers, so at least I knew something was breathing for her. I wasn’t too freaked out until she passed out at school off her breathing pacers. Of course I thought her heart was pausing, a common issue with CCHS.  All I needed to do was have a cardiac pacer implanted and then all would have been fine. However, it wasn’t Mighty Z’s heart: it was something much more sneaky than that.  It was discovered that Mighty Z also has Hirschsprung's Disease.

 

Hirschsprung's is most often diagnosed within the first week after birth.  However, some individuals are diagnosed later in childhood or as adults, though they have had chronic problems their whole lives.  Symptoms of Hirschsprung's include constipation, abdominal distension, vomiting, decreased appetite, cramping, and failure to thrive.

 

How did we miss this? I have no idea; all I can conclude is that I have always known that Mighty Z had sluggish bowels, and her doctors have known this too, but never felt it was an issue. I think we were all so focused on the fact that Mighty Z didn’t breathe on her own at night that her bowels seemed insignificant.

 

If you don’t treat Hirschsprung's disease, stool can fill up the large intestine. This can cause serious problems like infection, bursting of the colon, and ultimately death.  So what do we do for Mighty Z?  What are our options? We are hoping for a pull-through which involves taking out the part of the intestine that doesn't work and connecting the leftover healthy part to the anus. After a pull-through surgery, Mighty Z would have a working intestine.

 

However, we are preparing ourselves for a colostomy or ileostomy. A colostomy surgery is where the doctor leaves part of the large intestine and connects that to a stoma (open hole). Mighty Z would have a colostomy bag. An ileostomy surgery is when the doctor removes the entire large intestine and connects the small intestine to a stoma (open hole).  Mighty Z would have an ostomy bag.

 

I am heartbroken. I know that this is just a bump in the CCHS road; however, I was hoping that the Hirschsprung's  cup would pass my lips.

 

We meet with surgeons next week to discuss our options; we are asking for prayers for peace and knowledge to make the right yet difficult decision on how to keep Mighty Z alive and healthy.

                                      Sweet Mighty Z waiting for another specialist to come in

 Edited by Emily Minich

01 02 12

Fear Factor

Fear is something that is hard to navigate around when dealing with this ‘new normal world of special needs,’ especially when doctors are telling you that your little miracle has little to no chance of living.

 

I know that when Mighty Z was born, the doctors had given her less than a year to live at the most, and they, at times,  are still shocked to see her alive, twelve years later. One thing I experienced many times was that doctors do not know what to make out of these very rare diseases. There is literally no handbook or “how to,” so the doctors go through trial and error and speak on the side of  worst case scenario when  talking to the parents of special needs children.

 

However even though Mighty Z has made it to twelve doesn’t mean that fear doesn’t come slinking around or that death doesn’t try to knock on my door. I have, in fact, been dancing with both of these evil fellows for twelve years and still I sometimes wake in the middle of the night in a cold sweat because fear has managed to slip into my dreams.

 

So what do you do when fear starts lurking around? Well, I will tell you what I do, I jump out of bed and go check on Mighty Z and then I breathe.  I breathe through that fear and I quote Isaiah 41:10 (ESV) “Do not fear, for I am with you; Do not anxiously look about you, for I am your God. I will strengthen you.” And I take another breath and let it out, slowly.

 

 

 Sometimes that helps, and sometimes I just stare at her monitors for the rest of the night and pray over her. Is that a healthy way to handle fear? I don't know but it is they only way I can cope . Sometimes that old bedfellow, fear, tries to stay with me and linger on me like smoke. It is hard to shake fear once  it sinks its talons in you…it is even harder to push it away.

 

While sometimes my weeping may last through the night, joy usually comes in the morning. The joy in the morning when Mighty Z opens those big beautiful eyes never gets old. To make it through another night is a mighty miracle in and of itself.

 

I spend the next daypraising Him for my abundant blessings, and for the joy both of my girls bring me -- regardless of what this world throws my way. Sometimes, during those nights when fear slips in and death starts knocking, and I feel that I am just in the middle of a little storm,  I find that I am still praising Him. 

 

 edited by Linda Kruger

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