Beginning our Journey with CPVT

There are times when you can look back and see the hand of God at work and things make sense in hindsight.  Less often, though, for me, I see the hand of God at work in the midst of uncertain times.  Those are times of great encouragement.  That is what has been happening these past few months.  Let me start at the beginning of the story.

Back in July, during the heat wave we were having, AJ, our 8 year-old, and his 9 year-old brother were walking on the road to Grandma's house.  Their house is just a short walk away and the boys wanted to walk over.  I told them explicitly not to run because I didn't want them to overheat.  It really was a hot day.  I followed behind them a few minutes later in the van with the girls and found Pepe standing in the middle of the road, hands in front of him, motioning for me to stop.  I was frustrated with his seeming lack of concern for safety so I slowed down and told him not to do that, it was dangerous.  AJ was walking on the side of the road.  Pepe explains to me that AJ had passed out on the side of the road.  I loaded them both in the van, very concerned about AJ, and took them to Grandma's where I layed AJ down in a cool room and got him something to drink.  I asked Pepe what happened.  He said AJ just passed out but Pepe thought he was joking.  First he stepped on his arm to make him get up then he started tickling him.  Both got no response.  Then AJ woke up and Pepe realized this was not a joke.  AJ was pale and slightly sweaty but seemingly okay.  He laid there for while and then went back to playing.  I returned to working at the house and gave Grandma clear instructions to call should AJ feel any worse.  Though it may seem like I didn't care, you must know that my sister always fainted.  It was chronic and persistent and we learned to live with it.  I thought this was just a reaction to the heat and once he was in a cool room with something to drink he would be fine.  And he was.

On a cool August 25th, AJ was playing outside the house, running around with his brother and friend, when he stopped to catch his breath and promptly passed out.  His friend found him, called his brother, who ran to get his Nana and Papa.  Papa carried him into the house and realized his heart was racing.  We layed him down and tried to cool him off as he was very sweaty but with cold, clammy hands.  He was tired so I took him to the van to lie down and kept checking on him repeatedly.  He was doing fine, liked the quiet time and seemed totally okay.  Remember, fainting in my family is common.

Though it seemed trivial, I mentioned this to our family doctor a few days later on August 29th  and she immediately sent AJ for an EKG and ordered an EEG and a referral to a paediatrician.  The EKG happened later that afternoon while the wait to see the paediatrician was a little longer.  Just over one month later, on October 4,  I received a call that AJ could see a doctor in a town 1 1/2 hours away on November 5.  I declined, saying I would rather have a paediatrician in the main city only 1/2 hour away.  They warned the wait for that could exceed 1 year.  I quickly said a prayer, talked to my hubby, and decided to wait that year.  Later that same afternoon, I received a call from a paediatrician in that city that they wanted to see me the next day, October 5.  I say God had a hand in that, wouldn't you?  The paediatrician AJ now sees is a wonderful young doctor who is insistent on pursuing all tests possible to figure out the cause of AJ passing out.  On that first visit, he asked a bunch of questions then sent AJ for many tests - a breathing test, another EKG and blood work, muscle tone tests and coordination tests.  1 week later, on October 12, AJ went for his EEG to see if the problem stemmed from his brain.  He was a little trooper, having all those wires attached to his head but the technologist working with him was so great.

On November 1, AJ was running around a play structure when he came to me and told me he felt like he was going to pass out.  I made him sit and put his head between his knees and he felt okay after a few minutes.  I tried to keep him from running but he was annoyed with me.  Later that same morning, we did a very unscientific heart rate check.  His resting heart rate was 99 bpm and after running/walking for 5 minutes, I couldn't count it at all.  It was irregular and hard and unreadable.  Then I was a little concerned.

We had to wait a number of weeks to ensure all the test results were back but then we went to see the paediatrician again on November 13, this past Tuesday.  All test results came back normal.  His blood is normal.  His EEG was normal.  His EKGs are normal.  However, the paediatrician was still not convinced there wasn't something more so he referred us to a cardiologist.  He did warn it could take 3 months or more to see a specialist like that.  He also sent AJ for another EKG.  2 days later I had 2 phone calls asking if we could please come to the Variety Children's Heart Clinic at 1:30 on Friday, November 16, only 3 days after the referral.  What a fantastic God, orchestrating this all for us!! 

We arrived at the clinic but had no idea what was about to happen.  AJ had an ultrasound of his heart and it seemed, to me, that the technician spent a considerable amount of time looking at a certain part of his heart, listening to the way it beat.  After that, AJ had to have a stress test.  He was to lie on a bed so they could hook him up to many different wires and then lie there for a bit so they could get a good reading of his heart at rest.  Then he was to get on the treadmill and walk/run for 10 minutes.  Every 3 minutes it would elevate a little more and go a little faster.  His job was to not fall off the treadmill and keep up walking/running for the whole 10 minutes.  He really wanted to stop after 7 but he kept going.  After he was done, they sat him down, wires still attached and gave him a bright red Popsicle.  While he was eating it, more and more people came to see the printout of his heart's activity, just as they had been doing while he was running.  They also continued to ask him is he was okay.  Eventually the doctor we were supposed to see came to me and told me he had a colleague he wanted me to see that was a specialist in the area of what was going on with AJ.  He wasn't sure if the colleague was around or available but he would phone anyway.  Turns out he was around and willing to take AJ as a client, which is another example of God's hand at work.  He might not have been there any other day but he was on Friday.  They looked over AJ's results for a long time, talking with each other, measuring this and that and then sending us to another room for follow-up.  There I was asked a whole pile of questions by a resident about the history of AJ passing out, our family history, if anyone ever died suddenly or unexpectedly, if anyone fainted lots, etc.  Eventually our specialist came in, a Pediatric Cardiologist/Electrophysiologist and told us that the news he was about to tell us was not good.  In fact, it was very scary news.  AJ has a rare and very serious, potentially deadly, heart condition called Catecholaminergic Polymorphic Ventricular Tachycardia (CPVT).  It is a condition in which the heart beats exceedingly fast and therefore fails to pump enough blood through the body, causing you to black out.  AJ's heart reached almost 300 bpm while on the treadmill, about 3 times faster than it should.  Often times people do not find out about this condition until after someone in their family has died suddenly and unexpectedly.  It is a genetic condition and is often present in more than one family member.  It is a non-curable condition but completely treatable.  AJ will be on medication for the rest of his life to control his heart rate.  However, at this point, AJ is restricted from any physical activity which would raise his heart rate until they can confirm the dosage of medication he needs.  We also get to carry around an AED (Automated External Defibrillator) because we never know when his heart will need to be shocked back into rhythm.  AJ has named it "FRED" and it now goes with us everywhere.  AJ will eventually be able to do everything a normal kid does when he can show on a stress test that the medication is keeping his heart from racing.  As I mentioned, it is a genetic condition so the rest of us need to be tested as well.  In about 3 weeks, all the kids will go for a stress test to see if any of them also have this condition.  Jay and I will have to go to another clinic for our testing as the kids obviously go to a pediatric one.  Some of the kids are dreading it, some are super excited!

We don't know the whole breadth of this journey yet.  We do know that God has promised us in Joshua that we can be strong and courageous, we don't need to be afraid or discouraged because God is always with us, never leaving us.  It is the reason I was completely calm throughout the whole 3 hour appointment.  It is why I continue to be strong today.  I am relying on the Lord's strength and He is so faithful to carry me.  I already wondered what he had in store for us when all these appointments came together so well.  You begin to wonder what he is about to do.  But He is so good and loving.  AJ is still very much alive.  God sustained him through each fainting episode.  God sustains him each day.  And God is walking this journey with us.  We are surrounded by people that love us and pray for us and we give God the honor and glory for it all.  If you would like to pray for us, we would love it.  Pray for peace, for strength, that the side effects of the medication would be minimal, that the medication would be effective and that through this whole journey, we would continue to praise our God for the marvelous Sustainer he is and bring glory to his name.  Amen!  :)