Right before the rug was pulled out from beneath us!

Friday, April 29, 2011

Check Up!

Yesterday,  Jake felt well enough to return to school.  We went to the cardiologist to check the incision. It looks good.  He can take a shower now, and no longer needs to keep it bandaged up.  He gave us a script to have Jennifer's MRI done. He suggested Shands.  I will call today to get that appointment set up. We have an appointment to have the ICD read on May 17th and don't have to return to the cardiologist for 6 months. YEAH!  However, if he becomes symptomatic, we need to go in right away and get the ball rolling for the myectomy.  We have appointments with the dentist, psychiatrist and councilor coming up, so we are staying busy. But all is well and we can breathe!  Getting use to the life adjustments of low sodium and no caffeine are a struggle, but he is making awesome choices with little to no nagging.  I can honestly say, I can feel the weight lift off my chest and I can breathe easy, knowing life is going to continue. I am amazed at these wonderful doctors who spend so much time with us explaining everything, making sure we understand what is happening. Seriously yesterday, our doctor spent an hour with us one on one. He is awesome.  I truly appreciate all of your prayers and kind words throughout this challenging time for my family. Thank you, seriously, just doesn't quite do it justice, but it needs to be said... Thank you for all your prayers.

Thursday, April 21, 2011

Discharged Today!

Last night, as my little boy lay in the hospital bed, uncomfortable and unable to sleep he said," Thank you"... I said," For what?" He said," For researching so much and getting me up here, so I can get better." I said," Honey, it's what mom's do."  He replied with , "Not every mom." Yeah, I cried.

Before we left the hospital, they went over EVERYTHING! I'm so glad they were so thorough. The biggest thing I'm concerned with is that they said to WATCH FOR INFECTION. They gave him an antibiotic, he needs to take four times a day. Infection means trouble... So we have to keep it clean....Also before we left the hospital,  the incision had a small little patch of red blood, that the nurse was concerned with. She said it should of looked darker. She said to keep an eye on it.
And then they went over and over all the information to  make sure we got it. As a teacher, I know I can't just say something once and expect a student to get it. I have to teach it in different ways exposing a student to the same information in different ways. That is what we got today. It was like every nurse and doctor we spoke with explained it to us again.... at first I thought it was a little silly...then the teacher side of me kicked in and I thought OMG... They are teaching us how to live with an ICD.

For the next six weeks Jake can not lift more than 5 pounds with his left arm. They stressed the importance of following the activity restrictions because of placement of the leads from the ICD.  NO repetitive over the head arm movement, because they put the lead in a different vein than usual, so it has a bit more of a bend which may cause it to get worn faster.  Jake will need to wear a medic-alert bracelet or necklace. He will also need to carry a card with him to prove he has an ICD. They gave him a temporary card today and the permenent card will come in about 6 to 8 weeks.  He needs to stay away from large electrical motors, strong magnetism and welding equipment.  His ICD will set off alarms in a metal detector, so he'll need to be patted down at the airport, ( which is why he needs the card to prove he has an ICD) and no he is NOT as excited as you'd think he'd be about that... Also, he needs to avoid the hand held wands due to magnets.
  He can use a cell phone on the opposite ear of the ICD, and he can not carry a cell phone in his breast pocket ( The nurses suggested 6 inches away from the incision is a safe distance). The instruction booklet says to avoid holding the following items closer than necessary to the implant: hand held applainces with motors, such as hair drawers and shavers,  light shop equipment such as drills, table saws, etc.  and transmitters for radio-controlled equipment or toys.
Certain tests and procedures can interfere with his ICD so he will not be able to have , electrocautery, Lithotrispy, MRI's,  Radiation therapy, Radiofrequency ablation, and he should not use a TENS unit.
Also they are recommending a healhty heart diet- oh boy this could be the worst part for Jake- low in fat, low in saturated fat and cholesterol, moderate calories and sodium, and high in fiber.
  We will see Dr. Mathews next Thursday, then in six weeks, we will need to see an electrophysiologist who will  moniter the ICD, and check it's battery life and read the info from it... They said something about being able to the send info through a land line phone... but I'll learn more about that after our first visit to the electrophysiologist. Today before we were released from the hospital, we met with an electrophysiologist, he read the ICD and said it is set to do a shock therapy if his heart rate reaches 230 beeps per minute and the battery is projected to last 9 years. He also explained that if there is something wrong with the ICD it will vibrate letting Jake know that something is not right. So he tested it, Jake said it felt like a cell phone on vibrate inside his chest. If it goes off we have to call the electrophysiologist's office.
Dr. Marons office called and said the next available appointment for an MRI is Monday... We fly home Friday... So we will get Jenn's MRI done in Florida.
So how is the little romeo doing?  He's sleeping. A few minutes ago he gasped as he was sleeping, Jenn & I jumped to his side, he looked at us like we were crazy, scratched his chin and went back to sleep. He sleeps until it's time to eat. Then and only then can I get him out of the room, but if you know Jake he LOVES food... so that makes sense.
So the tough little guy, refused pain meds. I am concerned about the flight home tomorrow. What if there is turbulence? I'm paranoid as we walk down the street, trying to stand in front of him .. running block... making sure no one bumps into him. I can't wait to get home to our somewhat normal life.

Wednesday, April 20, 2011

Surgery went well

What a day! We met with the surgeon at 9, Jake commented that he really wanted pictures of the procedure, so the doctor asked a nurse if he had his cell phone handy. I handed the nurse my camera and aked if he could use my camera. The doctor asked, "You trust us with your camera?" Jake said," She trusts you with my heart but not her camera..." Sharp as a tack...and not nervous at all... They took him back around 9:30.... and told us it would be about 2 1/2 hours before we would hear anything.
At 11:19, Dr. Link called and said everything went well, they tested the device and it did what it was suppose to do. So everything was good. And that's when it hit.... the tears wouldn't stop... It was like I could finally breathe.
They wheeled him out about 30 minutes later and he tried to make a joke and then busted out in song, " Just a small town girl, living in a lonely world" Then he proceeded to tell all the nurses he loved them, and tried to get their phone numbers. The nurses were calling him Romeo.
He feels good , his pain is only about a 3 right now. They just came in and  gave him an antibiotic to prevent infection. Right now.. all is good.... Thank you Lord, for answering our prayers.
Jake wanted to look at the incision right away but the nurse suggested he leave it alone until tomorrow. After that it needs to be changed every day. No showers for a week, sponge baths only.  No touching the incision. Only small movement of the arm for a couple days. We will have to see Dr. Mathews in a week , and again after 6 weeks. Then there is another doctor we have to see every 3 months. Then we will have to have the battery changed in the ICD  about every 7 years.  I'm trying to remember eveything.
Dr. Link stopped in after the surgery and checked out the pictures. He thought it was a neat idea and was proud of the photos he took.  Dr. Maron stopped in to check on Jake, and let us know that they are trying to get Jenn in for the MRI up here on Friday, if for some reason they can't fit her in, we will have to have it done in Florida.
I am staying the night here in the hospital with him... 

Tuesday, April 19, 2011

Meeting with the surgeon

It's really hard to believe something so small is going to keep Jake alive. The ICD fits in the palm of my hand. Technology is amazing. We met with the surgeon today, Dr. Mark Link. He passed an ICD around for us to see.  He explained it will be placed below Jake's collar bone in between the layer of fat and muscle. He also said you will be able to feel it under the skin and although we all want to feel it we should wait about four weeks before we actually do....  I can picture Jake walking around saying , " Hey wanna feel my box." He also explained that Jake will be able to be more active than he has been. As a rule of thumb as long as he can talk while he is doing the activity, he can do it.  NO burst of energy, though.

  We were thinking we might go on a tour ride to see the cool historical things around but Jake is just too exhausted. He took a four hour nap after lunch.
  The funny thing is, being a Florida girl, I never had the desire to visit Boston, but now, I'd like to come back, when everyone is healthy and see a show, and check out the historical sites. Jake thinks Boston is cool. He likes that you can walk to everything. I know we aren't up here for pleasure, but I beleive in making the most of the situation. I hate that he is too exhausted to experience this place.
I don't have to be such a meanie anymore, he is quickly adjusting to the lower sodium diet, and making good choices on his own.  Because he can not eat anything after midnight, he wanted a "good" dinner. So we went to a restaurant close to the hotel called RockBottom.  It was very good.
   Everyone keeps asking how I am holding up. Every cloud has a silver lining... It stinks that Jake has to have surgery but honestly I am relieved. I know it sounds morbid. My little boy is having surgery, and I'm relieved. But right now if he does anything strenuous he could  die, after tomorrow that is not going to be a concern. He may get shocked, but he won't die. So, today, I'm relieved . Tomorrow I will be on pins and needles, a nervous wreck. It won't be pretty.  We have to be at the hospital at 9 a.m. where they will prep him for surgery. The surgery will take about 1 1/2 to 2 1/2 hours.

Monday, April 18, 2011

Test Results

We spent the day at the hospital having tests done. First, Jake had an echocardiogram, the doctor briefly stopped in after he looked at the results and decided not to do the stress echocardiography due to the risk and it wouldn't give us much more info anyways. The next test took a little longer because Jake had some kind of reaction to the contrast dye, they had to stop the test take his bp and give him oxygen before finishing the test. Jenn had her echo while Jake was still having the MRI.
So... the results... Jake has 3 out of the 5 indicators for being at risk for sudden death.
 The other two indicators are a test they did not perform on him, and family history which is imcomplete. They strongly recommend the Implantable cardioverter defibrillator. He will have it implanted Wednesday. The defibrillator will help his heart continue to beat, shocking it into the right rhythm, if needed. He has some obstruction but doesn't need the myectomy yet. We have to keep an eye on it.

I can't even describe the feeling I had when we saw the MRI scans showing the actual size of the septum thickness.  I kept trying to picture it using the pictures from the internet, but actually seeing it, slice by slice and see the thickeness completely through the heart, I still can't describe it.  The doctor spent a long time with us explaining everything to us, making sure all our questions were answered.
 Tomorrow,we are meeting with the doctor who will actually implant the defibrillator on Wednesday. Even though he will have the defibrillator he still will have limited activities. If he does strenuous activities, the defribrillator will shock him which is not pleasant.
Jenn's wall thickness was borderline. The doctor would like to do an MRI to get a more detailed picture of the thickness to see if she has HCM or not.  He is going to see if we can have it done while we are up here. She is NOT excited about it, she hates needles.
   The beta blockers Jake is on could be the reason he is sleeping all the time, so we are going to switch that but not until after the defibrillator is implanted. AND they drew blood today for genetic testing , we won't know the results of that for a couple months but hopefully if it pinpoints the mutated gene, it will be easier to test other family members.
 Jacob is doing fine. He likes the doctor and his special hand shake. He doesn't seem anxious about the surgery. He is his normal everyday self, picking on his sister every chance he gets. The only thing that seems to be bothering him is me.... because I keep taking pictures of everything, and I'm enforcing the  2000mg daily sodium intake.

Sunday, April 17, 2011


Jake enjoyed his first experience flying, He looked out the window and pointed out lighthouses on the way. Jenni on the other hand, decided planes are not her friend. We landed safely, Jake and Jenni experienced their first ride in a taxi, and then we ate at a resturant called Jacob Wirth for lunch. Jake's request because of the name. Jake has been sleeping since we checked into the room.  It's funny that he LOVES history. Here we are in Boston, so close to so many historical sites and he isn't well enough to visit them. He is 18. He should be out seeing the sites... not sleeping.    Bill & Jenni have gone for a walk. They are looking for something for dinner.It is a struggle to find take out low in sodium. The Hotel is beautiful however, it does not have a refrigerator. So it looks like we will be eating out a lot.  I did bring Jake some snacks for the plane trip. He was not impressed with my low sodium choices, but he ate them.
I'm trying to stay sane.  Right now, as he sleeps, and Jenni & Bill are out for a walk, I am here with nothing else to do but think of why we are here. I'm scared, anxious, and thankful.  I just want them to put the ICD in so I won't have to worry so much. I jokingly say it is his ICD security blanket. Really I think it is mine. I know that little box is going to keep his heart beating. I can't wait for it to be implanted. However, I don't want to think about the discomfort he will be in after.
I know we are here to start the road to him getting better... so that is what I will focus on. Getting my little boy better.

Saturday, April 16, 2011

Better Than He Was Before

In my head I keep hearing,” Gentlemen, we can rebuild him.  We have the technology. We have the capability to build the world’s first bionic man.  Jacob will be that man.  Better than he was before. Better, stronger, faster.”  Ok maybe not the first and definitely not faster, but his heart will be better and stronger…
We are flying to Boston in the morning. The first appointment is at 9 am Monday morning, Jake will have an Echocardiogram,  an MRI, and a Stress Echocardiography and Jenni is scheduled for a resting Echogradiogram.... all before our visit with the doctor at 12:30. On Tuesday, we have a consult with the doctor implanting the defibrillator.  Wednesday is the day we all get to breathe. It is the day Jake will get his little ICD security blanket. (The ICD will monitor the heart rhythm, identify abnormal heart rhythms and determine the appropriate therapy or shock to return his heartbeat to a normal rhythm.) Hopefully Jake will be released on Thursday and we can fly home Friday. 
It amazes me that this little box with some wires attached are going to be able to keep my little boys heart beating. It's hard to look at him and realize he could be taken from us. He looks so healthy. Even now that I know how sick his heart is, the only clue you'd have is how tired he is. He sleeps a lot. It is overwhelming to think of what could happened. We still have to make it to Wednesday. I have faith in God that everything will be work out. 
 I have to say I am AMAZED at the outpour of love, and support from family and friends. On Thursday, after the doctor had called, I called my sister, the next thing I knew she had  arranged our hotel stay and was able to get our airfare taken care of. I can’t even begin to tell you what a relief that was.  The last three days have been so crazy, and emotional.  That was one less huge thing I had to worry about. The hotel is right across the street from the hospital. 
Yesterday someone said to my husband,” Why Boston?” We have some good Cardiac Hospitals here in Florida.”  And yes we do, but when the CEO & founder of The Hypertrophic Cardiomyopathy  Association says,” You need to go to Boston.” You go to Boston. Period.  Please don't judge us for our choices, the way I see it, You don’t go looking for a discount plan when it’s your son’s heart. You go where the experts tell you to go. If it were your child what would you do?
Jake is in good spirits. He is not enjoying the 2000mg sodium limit on his daily food intake. Some friends and family stop by to wish Jake well in Boston, bringing him Superman under garments for his stay in the hospital. Thank you to all of you who are praying. 

Wednesday, April 13, 2011

Surgery next week!

Sucker punched.  That's how I feel...  I knew the fight was coming, but today I got sucker punched...I thought we had a little more time.  My appointment with Lisa from Hypertrophic Cardiomyopathy Association was at 4 o'clock.  I had already faxed her Jake's test results. She took all the history and information, then I was explaining to her that because the cardiologist had said Jake was severe, I have stopped him from doing pretty much everything. However, this week we have just started going for about a 1/2 mile walk because I was concerned about his sudden weight gain. She told me not to be too concerned when a doctor uses the term severe. She said she'd let me know when she looked at this results...That's when it came... the feeling as if someone had punched me right in the gut...   "SHIT".... then " yeah, he classifies as severe."  or was it HOLY SHIT!  At this point I'm not quite sure anymore...  After she looked at his test results she said  I would not be continuing those walks with Jake. He is at severe risk for SUDDEN DEATH. He needs a ICD (implantable cardioverter defibrillator)now. At 4:35,  She emailed a doctor she highly recommends while I was on still on the phone with her and CC'd it to me....  seriously I copied and pasted two lines from it...
"He needs to be seen asap… and we need to get the ICD in asap.. hopefully on the same visit. 
HELP….  Can you please call mom to get her in at the next available slot!
It was 4:46 p.m. when I hung up the phone with Lisa. I took a chance and called the doctor's office she was recommending. They took my info, and said the lady who schedules everything had stepped out but she would get with me when she came in... Because it was so late, I thought I wouldn't hear anything until tomorrow. At 5:03, I received a call from Murdena, letting me know that it will probably be next week, she would call me in the morning to confirm the exact date as everyone had already left. At 5:04, I received an email from the receptionist with  Hotel information and At 5:13. I received an email from the doctor.  It looks like we are going to Tuft Medical Center in  Boston, next week. I posted the times because it happened so fast. I'm still a little shocked.   If you look at Jake he looks like a healthy kid... 
SO how is my kid handling it... He is excited to be going on an airplane. Also the crazy boy is working on impersonating Chinese voices from movies he has seen because Lisa said he needs to be in China Doll Mode...  sitting on a shelf looking pretty but doing nothing... The only question he asked was.. "Will they have to put me on bypass to do this?"
So tonight... I will find him the answer... 

Saturday, April 9, 2011

The Road Less Traveled ?!?

 My family and I were enjoying life, driving down the road, and over the bridge relishing the view. When suddenly, there was nothing but brush, trees, and weeds.. no road.. no path… Instant reaction was to put it in reverse…. Oh but NO! The bridge had collapsed.  There is no way back… to go on… we MUST go forward.  Being unprepared, I dug out my nail file (cause who carries a machete) and start cutting away at the brush… It’s been a little over a month of cutting away and finally I can see what looks like a path…Yes! Yes it is... a path.. well worn by others whose lives have been afflicted with HCM... I called Hypertrophic Cardiomyopathy Association…  I have an appointment to get more information from them on Wednesday.

Suddenly my family find ourselves on this road less traveled but not by our own choice... I am so thankful there is an association to help guide us through this. It is helpful to hear from others who are living with HCM.  It turns out Jake's psychiatrist also has a personal connection to HCM.  It effects 1 in 500 people.
I found this picture online. I think it pretty much explains it.

Hypertrophic Cardiomyopathy is a disease in which there is a primary hypertrophy ( enlargement or overgrowth) of the cardiac muscle with increased systolic (blood pressure when heart is contracting)  but impaired diastolic ( period of relaxation & dilation) function of the heart.  In non medical terms..the thickening makes it harder for blood to leave the heart, forcing the heart to work harder to pump oxygen rich blood to the rest of the body. 

Nine days until our next appointment with the cardiologist.

Tuesday, April 5, 2011

Too Much of a Good Thing

Education... It's my life, after all I am a teacher.  Many nights I stay up late, researching everything I can so I know what we are up against.... It's my way of fighting the fear. What's the old saying?  Knowledge is power... I wish I could function differently, and believe ignorance is bliss, I'm sure I would of gotten a much better nights sleep if I had.  I have read many, many pages about what a Myectomy is.  I know it's a procedure that will last three to four hours. I understand that they will open Jake's chest and shave off a portion of his heart wall to open more space.  I guess I didn't really think it through because what I DID NOT realize until last night is that to do the surgery, they will have to put Jake on cardiopulmonary bypass...   Umm , Yeah.. I teach Kindergarten...I'm not a doctor.. but I watch one on T.V... what little I know about this I learned from watching Grey's Anatomy... So, a bundle of nerves, I read on to have my fears confirmed... A machine will temporarily take over the function of Jake's heart and lungs during the surgery keeping his blood and oxygen circulating throughout his body.  The thought of my little boy's heart not beating, seriously, made me break out in a cold sweat.  I have faith in God and the doctors, but that's my son's heart. It's scary.  This morning, as I drag my tired booty to work, I wonder if Knowledge really is power... or in this case is it too much of a good thing...

We have a busy week, Today we have an appointment with the Psychiatrist, tomorrow the dentist to get all his dental needs taken care of before the surgery, and Wednesday we have an appointment with the councilor.  His mental health is just as important as his physical health, and this is a lot to deal with.

Sunday, April 3, 2011

March 3rd

 March 3rd,  that was the day the rug was pulled out from under me. Who knew what a difference one day can make... everything is the same.... yet it's so different....  1:15 p.m..... the doctor delivered the blowing news that Jake has Hypertrophic Cardiomyopathy.  One minute I was joking around,  taking pictures of my son having an EKG done, and the next minute I had my head in my hands trying to stop the spinning. The next 15 minutes were spent with the doctor explaining the seriousness of this condition, saying how lucky we are that this was diagnosed, because this condition in children Jake's age is usually diagnosed in an autopsy. Then my crazy little guy asked if he would need surgery, and was excited because he thinks scars are chick magnets...
We were sent to the cardiologist the very next day, where again the doctor spent a lot of time with us one on one making sure we understand the limitations of the condition. No running, no baseball, no basketball, any strenuous activity could result in sudden death. Then, I felt like I was hit with a ton of bricks as he handed me a doctor's note to give to Jake's school requiring them to have a defibrillator on hand in case Jake goes into cardiac arrest.  I'll never forget that night, turning on the T.V. to a news report about Wes Leonard, a 16 year old boy dying after scoring the winning basket at his basketball game. That was the slap in the face to say, seriously we are the lucky ones, we were diagnosed before it was too late.  In the next week, the news covered two other children who lives were cut short from the same thing my little boy was diagnosed with.
In the few weeks that have passed, we have been through many ups and downs. Doctor visits, stress tests, echocardiograms for all the kids and the counselor appointments have been keeping us on the go. We have definitely been on an emotional roller coaster ride.  Jake is trying to cope with his sudden physical restrictions. I'm trying not to go insane when he pushes the limits.  We are incredibly blessed that we have an amazing doctor, who genuinely cares. The doctor is recommending a myectomy and an implantable cardioverter defibrillator.  The hospitals around here do not do these procedures, so we will have to travel.  Our doctor is researching which hospital will best meet Jake's needs. So right now it's a waiting game. Our next appointment is in 15 days.