The meds are working, Praise the Lord!!

We knew this day was coming for several weeks now but it didn't make it any easier to endure.  This was the day we took all 5 kids into the children's heart center for testing.  AJ needed to be retested to see if his meds were working and the rest of the kids needed to be tested to see if they also carry the same heart condition AJ does.  When we arrived at just after 12:45pm, the kids all removed their coats and boots and were immediately weighed.  Then the 2 little girls came with me while they had their ECG.  They have never had any testing like this done so they were mighty apprehensive.  Telling them they were just going to have stickers all over their tummy didn't seem to help but once Ali had her test done, Ari was quick to follow.  After all, she watched Ali and saw she did fine so it was okay for her as well.  While the little girls were having their ECGs, the older 3 were off with Jay for echo cardiograms (ultrasounds of their hearts).  They were great for their tests.  After all, all you do is lie on your side and have some goop smooshed on your chest.  She rubs a wand all over your heart area and you can watch and listen to your heart - quite fascinating, really.  When they were done, it was time for AJ to have his stress test.  Since he has done this before, it was simple, really.  Lie down, hook up all the wire, run on the treadmill, endure the speed and incline changes for 11 minutes and you are done!  Well, we were thrilled to see that he was able to go for twice as long on the treadmill before his heart started showing dangerous and abnormal heart rhythms.  That means his beta-blocker medication is working and we are so thankful for that!  Praise Jesus!  Once he was done, it was Pepe's turn.  He was not so excited.  Stoic.  Serious.  No fun.  Just get it over with.  He ran.  He stopped at just shy of 7 minutes, complaining his legs hurt.  I was a bit disappointed in him quitting but they were able to get the results they needed.  Perfectly normal!  Thank you, Lord!  Then it was RJ's turn.  She was looking forward to this for so long.  She was sooo excited about running on the treadmill.  So, they hooked her up and eventually, after a very long wait, she was able to hop on the treadmill.  She only lasted just over 7 minutes before she asked to stop but the technician said it is hard for little kids (RJ is 6) to run coordinated on an inclined treadmill.  But she did enough to get normal test results so we are super thrilled about that, too!   While the older 3 were on the treadmill, the younger 2 were off for their echo cardiograms.  But Ali did not like it one little bit.  Jay had to lie down right behind her on the bed so the test could be done.  It was but she wasn't happy about it.  Ari was much better, a little more adventurous than Ali.  Then, after everyone was tested, AJ was hooked up to the holter monitor, which is a portable ECG that monitors him for the next 24 hours.  He has a bunch of wires stuck to him with lots of tape holding it all in place so it is rather uncomfortable but he is a trooper.  I promised Lego when he is done!  :)  Then we sat for what seemed like forever to hear all the results.  It was tiring watching one staff member after another leaving at the end of the day and we were still there.  Finally, it was just our family and our doctor left.  And then he told us all the good news.  AJ's meds are working.  Pepe and RJ have normal test results.  Ari and Ali, though, because they are too young for the treadmill, will have to have a holter monitor test at some time in the future to rule out any heart condition.  But their tests were also normal (which, incidentally, doesn't mean much since AJ also has normal ECG results and a normal echo cardiogram.  That is why the holter and/or stress test is so important.).  So, that was all good.  Now on to the next steps.  Jay and I must be tested.  We need to go to our family doctor and explain what is happening with AJ and request testing for ourselves.  AJ will fly to another province to have an MRI of his heart in the new year.  This is another test the doctor is expecting to have normal results.  That is the funny thing about this rare heart condition, called CPVT.  Most resting results are normal.  The heart muscle itself is also normal.  It is just an inability of the heart's electrical system to handle adrenaline so it runs into problems with exercise or emotionally-induced stress.  Once the MRI is completed, AJ will go for genetics testing to see if they can identify the gene causing the CPVT.  In 40%-60% of the cases, they cannot identify the gene but they can some of the time.  If they can, then we know for certain he has CPVT and the rest of the family can also be tested so we would know with certainty who has it and who doesn't.  If the gene cannot be identified, a couple of other invasive tests can be performed to solidify a diagnosis of CPVT.  Since those tests are not mandatory and since they are full of risks, we are inclined to not do them.  It is not that important to us that we have a certain 100% diagnosis of CPVT if it means AJ's life is at risk.  We know that the meds are working and we trust the doctor.  He is one of only 9 specialists in the country and the only one in our province that knows and understands this relatively new and rare condition.  With all the research we have done (which isn't hard to do since there is not that much written about it - remember, new and rare) we know that this is what AJ has.  It fits every description to a tee.  So, for now the AED stays with us until the medication controls it perfectly or until they implant an internal defibrillator.  Whatever the case, we are thankful for today's results and were so glad to leave the clinic 4 1/2 long hours after we arrived.

Owner Building a Home - Week 26 - Flooring Arrives, Prep For Countertops

I know it is already over a week ago that this should have been posted - sorry to be so slow!

This week was a super slow week for me.  I had a doctor appointment with AJ on both Tuesday afternoon and Friday afternoon so that meant I wasn't at the house at all.  I also had company coming over on Wednesday evening so I stayed home that day to clean and prepare some food.  Saturday was the day after learning of AJ's heart diagnosis and all I wanted to do was curl up in bed.  We stayed home.  So, given I was only there 2 days, I will just post some photos of some of the things we saw happen.  Jay continued to work each day but I don't always know what he has done.  However, things continue to progress, even it if it is slowly.

We crushed more glass for the counter top.

The guys continued to form the counter top form.

They installed the ceiling medallion in the dining room.

They also built a mock-up of the coffered ceiling in the living room.  The cardboard was just to get a sense of how deep we wanted the "beams" to be.

Jason went to the US to pick up our Brazilian Pecan flooring that I had ordered from Georgia about a month ago and had shipped to Pembina, ND.  It is a little nerve-wracking to order something this expensive from a company you have never been to.  We hoped the flooring was everything we wanted it to be and . . . so far . . . it is! It is so gorgeous.  Hopefully the installation goes just as smoothly as buying and getting the flooring.

 

While I was in the city with AJ on Tuesday, I went to many stores buying crown moulding, a dresser to be used as a vanity, colored glass to be smashed into itty bitty pieces, groceries, lighting, etc.  The van was super full but it was a good day.  On Friday I only made one stop after the 3-hour long appointment but it was to pick up the charcoal colorant for the counter tops we will be pouring next week.  It was necessary even if I would have rather come straight home.  Sitting in city traffic during rush hour after hearing your son has a serious heart condition is not my idea of a good time.  But God was faithfully with me and I am so thankful for that.

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!  :)