Instincts

This particular post has been very hard to muster up.  The fear I feel each and every day regarding Jarod and what has been going on has me beyond distracted so this is to recap the last two weeks.

Instinct: How can such a small word, described as "something you know without learning it or thinking about it" (Merriam-Webster) bring so much turmoil and emotional distress? That answer is easy...when it comes to someone you love with all your heart, especially one of your children, a child who struggles each and every day to push forward despite feeling his absolute worse, you will do whatever it takes.  I don't hate much, but I do HATE CHD's! I must say that it has been years, over 14 years, when I last felt this gut wrenching fear related to Jarod's CHD, Hypoplastic Left Heart Syndrome. 

It all started at about 11 PM on Saturday night, 5/12/13. I could tell earlier in the day that Jarod wasn't feeling quite himself. Later that night, he actually asked for a nebulizer treatment because he couldn't breathe. We did what we always do and started the nebulizer treatments for his asthma. He was able to rest that night but the next day he just wasn't himself. I noticed that his belly was big and this concerned me somewhat so I actually called our local urgent care to ask if there was a doctor that would be comfortable seeing him. The very nice receptionist checked for me but advised me to take him to the ER because of his cardiac history. I had decided to employ "watch and wait" as long as he didn't get worse. He rested throughout the day on Sunday and he seemed okay so we rode it out. The next morning he was feeling bad, though he would never admit it, so I called in to work so that I could be with him and take him to the pediatrician. I must say that I work with an AMAZING group of people who would drop anything, anytime of day, to help me out. 


We were seen by the pediatrician, not his regular one, but one who knows him well. She too felt this was his asthma and started the standard, dreaded 60 mg of prednisone a day for 5 days. This high dose is usually the only thing that helps, especially since last Fall when we found out the stent placed in July in his pulmonary artery is compressing on his left bronchus. We already had a regular medication follow up for Thursday regarding his anxiety of upcoming surgery with our regular pediatrician, so the doc asked that we come back in for re-evaluation. As the days went by, Jarod continued with his normal course but I did notice he was more short of breath this time, more fatigued and that his oxygen level was normal which is not the norm when it comes to his asthma. His belly continued to get bigger, actually so much bigger that he couldn't wear his regular pants, and had to wear shorts with an elastic waistband.

We followed up on Thursday and saw his regular pediatrician. He is always very up front about his concerns. He listened to Jarod's heart and lungs for an extra long time and also noted that he had a more than 4 pound weight gain just since Monday. He was concerned enough about congestive heart failure that he sent us over for a STAT chest x-ray and labs. He called me later that evening and said that the labs didn't suggest congestive heart failure and to continue the nebs and follow up with cardiology as already scheduled on 5/30.


Friday came along and Jarod seemed a little worse so, just for my peace of mind, I called cardiology. As I said earlier, it has been years since I called with concerns about Jarod's heart. In the near 15 years since he was born, this is only the second time I felt the need to call cardiology. The down side is that we are once again starting with a new cardiologist who doesn't know us. I spoke to the nurse and she felt that it was urgent that we come in for a clinic visit, a repeat chest x-ray and echo so we went to UVA that afternoon. We saw the on-call cardiologist who was concerned about his increase in weight and the swelling in the abdomen. He started Jarod on Lasix 20 mg twice daily in hopes of getting some of the fluid off and asked me to follow up by phone on Monday. 

When Monday came around, there was little improvement and Jarod was even having to sleep sitting upright over the weekend. I relayed this info to the cardiologist and he had us double the Lasix to 40 mg twice daily and to follow up by phone Wednesday because, if this did not work, the plan would be to admit Jarod for IV Lasix. During this time I have kept a close eye on his weight and his breathing. Watching closely, when I checked on him Tuesday night, I noticed that he was sweating profusely. I am talking clothes and bedding soaked with the AC on sweating which shouldn't come from the Lasix. By Wednesday morning, I was relieved that the swelling in his belly had gone down, not completely but noticeably less, and he was losing weight. 


Deep down, I worry that this might be Jarod's liver, this is what my gut instinct is telling me. Because Jarod has a Fontan circulation, there is always the potential for liver problems as well as lung and GI problems. We had seen the cardiologist at CHOP who feels that he already has fibrosis of the liver so this is always in the back of my mind. There have been many days these past two weeks where I am actually afraid to wake up in the morning to check on Jarod, fearing the worst! 

On Wednesday morning, I had to call the cardiology nurse to report how Jarod was doing. I did mention to her about the sweating and it was brushed off, she said "it was hot last night" even though I told her the AC was on and no one else was uncomfortable. When I mentioned my concern about his liver, and requested she ask the doctor about liver imaging, she advised me that imaging is not necessary and that the concern they have with post-Fontan kids is heart failure or protein losing enteropathy (PLE) and that I need to keep the 5/30 appointment because they want to monitor him closely since surgery is getting close. By this time, I am feeling overwhelmed and defeated. I am talking to a cardiac nurse who may be a great cardiac nurse, but who has absolutely no idea who I am or who my child is because we are starting with yet another new cardiologist. 


On Wednesday night, Jarod had another night of sweating profusely. By this time, I am really concerned and trying to figure out what is going on. I look up the medications he has been prescribed recently and there is a "moderate interaction" for prednisone and Risperdal and then Lasix and Risperdal so why didn't the pharmacy point this out??...not really sure but I did my own research and read some scary stuff so on Thursday, I had decided I had to have some peace of mind so I called Jarod's pediatrician to discuss the sweating and the cardiology visit. He was very patient and as always, wanting to help in any way he could. We talked things out and he really wasn't sure what to make of it but certainly didn't want to cause any worsening heart failure. Yes, I hear from the pediatrician that they feel it is heart failure, not cardiology. Not really knowing what to make of this, he did admit that it was a "shot in the dark" but that maybe Jarod's thyroid stimulating hormone (TSH) was high so he had me hold his Synthroid that evening. Low and behold, there wasn't any sweating! He asked me to call and let him know since he was just grasping at straws so when I called on Friday to report this, once again he wanted STAT labs. Fortunately, I was able to reach JW and he picked Jarod up from school to take him to the lab. The pediatrician called later on to let me know that yes, his TSH was a lot higher so he decreased the Synthroid dose but also that his liver profile was higher than it was just a week ago bringing me back to the instinct that this is his liver. 

As the days go by, my gut tells me something is very wrong. Because he is in no distress, I will wait until that 5/30 appointment with cardiology, the new transplant cardiologist that we were specifically assigned to when Jarod's last cardiologist left.  I will have many questions for him and I am going to insist on a liver ultrasound. Jarod is scheduled for a very risky aortic valve/root replacement on 6/27 at CHOP and I don't want them to miss anything.  One question I will absolutely be asking is, "if this was your child, what would you want?".

Thanks for taking the time to read and I will update when I know more.  

Keeping The Emotions In Check

Where oh where do I start? This waiting game is proving to be a very tough time for us. Jarod continues to act out and have an attitude towards many things these days, especially when it comes to school. Teachers are calling and e-mailing about his behavior and downright defiance when it comes to day to day school activities which we are seeing as well with home activities. While some teachers have been very patient and phenomenal with Jarod, some get mad and just don't get the impact and fear of what an upcoming open heart surgery entails for a 14 year old with cerebral palsy. I can tell you if it were me, I would be a little on edge too!

We have always been as honest as we can with Jarod in terms that he understands but this time is all new territory for us. Although Jarod has been in the hospital at least yearly, every single year of his life but one, this upcoming surgery is big. His last open heart surgery was nearly 13 years ago at the age of 2 years old and he just doesn't remember it. While some parents choose a different approach, this is the course we have chose and one I prefer for our family. I want Jarod to be able to advocate for himself and understand as much as possible when it comes to his heart and his overall health and to understand that all of this is done to help him feel better. 

Now that we actually have a date for surgery, things have escalated to say the least. Back in March, I asked if we could try some medication to see if this might help his anxiety. As much as I didn't want to add yet another med, I felt this was important for Jarod's well being. He was started on a low dose of Zoloft by one of the pediatricians familiar with him, also at the recommendation of the developmental pediatrician at Kluge. We had to follow up a month later to see how he was doing. We followed up a month later and when telling the pediatrician the Zoloft wasn't really helping much, he did say that normally he would not treat for something like this but he sees just how much the fear and anxiety is impacting his life so he agreed to continue and he increased the dose. That first night was like a light had been switched on in Jarod and not in a good way either. Behavior was getting much worse and Jarod was doing things at school that were so out of character for him. While I won't go into detail about his actions, he actually got in enough trouble to get suspended on a Friday. At this point, I called the pediatrician's office and the nurse took down my concerns but the doctor would not be in until Monday. The following Monday, the doctor called and I explained what was going on and asked for a week or two off from school to see if this would help. I must say, we have some of the best pediatricians around who are so supportive and know that we as parents actually know what is best for our kids. He explained that sometimes kids can have a "paradoxical effect" to the Zoloft and we should stop it right away. We immediately stopped the Zoloft and had an appointment the following morning to get in with his counselor.

Jarod went to see his counselor but had not seen her in awhile.  She had been helping him work through some bullying issues that were happening at school and has not needed to talk until now. As soon as she saw him, she knew this was not the same Jarod and, along with the pediatricians recommendation, we were scheduled with a psychiatrist that Thursday in hopes of some help and answers.

Thursday arrived and we saw the psychiatrist. He spent a good amount of time getting to know Jarod and explained that sometimes Zoloft and other similar SSRI's will uncover an "underlying mania" so now the work up begins to see if Jarod is bipolar. At this point, my heart is broken, broken because I just want Jarod to catch a break.  I mean really, how many more diagnoses and specialists do we need to add to the list??? While I know this is no one's fault, the guilt I feel for requesting the Zoloft is literally eating away at me night and day. On the other hand, I feel fortunate to have found out this information now when Jarod weighs only 75 pounds and not later when he is at 200 pounds and could physically hurt me or anyone else. As mentioned before, behavior was out of control and he was doing things he never would have done like picking up knives, grabbing medication bottles, etc. so this was some scary stuff.

As of now, the plan is to get Jarod's mood stabilized so Risperdal was added and then increased at the second visit. The psychiatrist will monitor him closely and the hope is to get his mood stabilized and then add medication for his ADHD.   He has had the diagnosis of ADHD since 2009 but after trying two medications that both caused chest pain and palpitations, we chose to put this on the back burner. Now that he is older, it is time to re-address this and the psychiatrist will try Jarod on Strattera, which is a non-stimulant, in the next few visits. One thing the psychiatrist was really interested in was when exactly the anxiety started. We really noticed a difference after our visit to CHOP in November. Jarod is old enough to be a part of the discussions with all of his doctors and asks his own questions. It was after that visit, we started to notice more anxiety. Later, after talking with Jarod about his fears and concerns, we found out that he was interpreting CHOP as "hi ya", like a karate chop. One of the first concerns from the psychiatrist was using the word "CHOP" with kids. While Jarod is old enough to understand a lot, he did not understand that CHOP was just an abbreviation so we are very careful of this now and if we say CHOP, we remind him just what it stands for.

As I write this, Jarod does seem to be getting back to himself.  He doesn't look and seem as "lost" as he was though he is extremely tired and will fall asleep at the  drop of a hat.  A lot of this is his heart but add to that his medication and this just makes him exhausted.  I hope as the days and weeks go by, we will have our sweet Jarod back to his normal self once again.

Weekly Medication Reminder

It's Sunday evening, and Jarod reminds me "it's time to get my medicine ready".  To me, this is bittersweet.  A boy his age should not have this on his mind but yet every Sunday evening, this is what he thinks about.  I am so proud that he has taken a proactive part in his health and well being.  Since birth, Jarod has been on medications for his heart.  In the beginning, it was very easy to just slip them through his feeding tube.  After the feeding tube, we resorted to crushing them in applesauce or pudding which worked well for years.  Despite Jarod's cerebral palsy, he is one smart cookie.  There was one particular hospital admission when the nurse was going over all of his medications with me.  Out of nowhere, Jarod blurts out "I don't take any medicines!".  It was then that I got the look, the look from the nurse no mom wants, that look where you want to turn around to see who she is actually looking at but you realize she is looking at you, waiting on an explanation.

From that moment on, Jarod has learned what each pill is for, what it looks like, why he takes it and when he takes it.  This list now includes meds for his heart, asthma, thyroid and anxiety plus inhalers so this is quite the feat for him but he knows his stuff! There have even been times in the hospital where he has asked me if it was okay to take because it looked different.  This is another important lesson that we learned early on.  During one admission, he was only 6 months old, and I did not recognize the pill.  I was told it was a different brand of aspirin but I made her check.  It turns out, it was a 325 mg aspirin and Jarod was to only get 1/4 of a baby aspirin.  It NEVER hurts to check because while our nurses are great, they are human too.  Learn as much as you can and never be afraid to question anyone who is taking care of your child.

Just for reference, Jarod takes: lisinopril 10 mg, 81 mg aspirin, levothyroxine 112 mcg, Singulair 10 mg, sertraline 25 mg, multivitamin, Flovent inhaler daily and uses his Proventil inhaler and DuoNeb nebulizer treatments as needed.  Because of his thyroid, he is due to have blood drawn too.

Our Personal Introduction To Hypoplastic Left Heart Syndrome

When we first found out we were going to have a second child, we were thrilled. Our oldest son, JW, was 3 years old and we just knew the timing was right. It wasn't long though before our lives would be changed forever. At a routine ultrasound, my OB was spending a really long time on a particular area and then went out to get another doctor to get his opinion.  They took a look together and their silence spoke volumes. I of course was a nervous wreck while my OB tried to assure me that it was just precaution to be referred to UVA for a fetal echo. It was on that spring day in March of 1998 that has lead us down the path we have been living for the past 15 years. The pediatric cardiologist confirmed the hunch my OB had which was yes, our unborn son had Hypoplastic Left Heart Syndrome (HLHS), a complex congenital heart defect (CHD), fatal if left untreated.


At this time we met with a surgeon, met with a second OB who offered to terminate the pregnancy even though we were at 22 weeks (never an option for "us") and we talked more with the pediatric cardiologist. We were given the options of 1) Transplant but newborn hearts are hard to come by; 2) The Norwood Procedure, the first part of the 3 stage surgical repair; or 3) comfort care which is to take him home to pass away at home. We took in as much information on the defect as we could and went home to research more on the internet. I don't think I stopped crying for a week but vividly remember JW holding my hand and telling me "don't worry mommy, the doctor's will fix his heart".

As the months went by, and after much research and a second opinion at MCV, we decided that MCV would be the best place for Jarod to have his surgery. We had close, weekly follow up visits with my local OB until Memorial Day weekend of 1998 when we made the drive to Richmond to be admitted for induction. It was a long labor and finally, at 3:30 in the morning on 6/2/98, Jarod arrived into the world very quiet.  He was whisked away just as fast as he came into the world.  We barely got a glimpse of him before he was taken to the NICU, it all happened so fast that they didn't even take time to get an arm band on him.

As the hours went by, we had a lot of doctors and nurses in and out of the room with updates, concerns and even thinking that surgery couldn't be performed because he only weighed 5 pounds, 4 ounces. After the surgeon evaluated Jarod, he felt he could proceed and surgery was scheduled for 2 days later. Jarod sailed through the surgery and was home in less than 2 weeks but did have to come home with a feeding tube which lasted about 3 months.

We settled into our routine, and were now used to the multiple doctor appointments and the dreaded feeding tube and all the stares and whispers that come with that.  It was not long before we learned that the surgeon was moving to Texas and we knew the second stage of the procedure was done around 6 months old. Near that time, Jarod started having more symptoms and was working really hard to breathe. Not sure what to do, our cardiology team at MCV had trained with Dr. Norwood when he was at CHOP so they called him for a consult and on 12/27/98 we were at A.I DuPont Hospital for Children for Dr. Norwood to perform the hemi-Fontan. Jarod sailed through that surgery and was home in 5 days! Unfortunately, soon after getting home, Jarod came down with a respiratory infection which landed him in our local hospital for a week, testing positive for RSV, which lead to his asthma.

Once again, we settle back into a routine but in this time we notice that Jarod isn't hitting those milestones that he should be so our pediatrician ordered a brain MRI which showed that he had cerebral palsy.  With this, I was absolutely crushed.  To me, this was more devastating than the heart defect because at least they were working to "fix" the heart but the CP was something that would never get better. Once again we were faced with another obstacle and more specialist appointments and just didn't know what the future was going to hold for Jarod. There were some doctors that said he would never walk or talk but sometimes the doctors just need to step back and realize that they are talking to a parent who will go to the end of the earth and back for their child. We ended up going to Kluge Rehab Center for Jarod to be fitted for a wheelchair and as I watched him, it broke my heart. I just couldn't commit to a wheelchair because I felt that if I put him in the wheelchair, that would be where he stayed. It wasn't long before he pushed his walker aside and started walking. Though his speech still needs a lot of work, he is talking and goes to regular school.

Ahhh, things are starting to seem really normal with the doctor appointments becoming fewer and fewer and all continues to go well. We had a year without any procedures but at a routine follow up and meeting with the new surgeon at MCV, he told me that "I think I can fix this if we wait a year". My medical and mom radar was going wild and I insisted the results be sent back to Dr. Norwood so he could complete the repair which we did in 6/00. Once again, Jarod sailed through the surgery but he ended up developing an infection in his incision that went into his bloodstream, landing us almost 6 weeks in the hospital for IV antibiotics.  I think this was the hardest part, having to be so far from home, missing JW's kindergarten orientation, his first lost tooth and so much more.

Now, that the repair has been completed, we sit back and look on in amazement at how Jarod continues to inspire so many people. He has this severe heart defect that required 3 major surgeries before 2 years old, asthma, cerebral palsy, probable Celiac disease, thyroid problems and ADHD but he always wakes up every morning with a big smile and a go get em attitude! I only wish I had half his courage and bravery.

Although Jarod's repair is complete, during a cath last summer, it was found that he has a rare complication of both a severe leaking and enlarged aortic valve which now needs to be replaced. Jarod is scheduled for an aortic valve/root replacement on 6/20/13 at Children's Hospital of Philadelphia by Dr. Spray. At that time, it will be decided if they will remove the stent that was placed in his left pulmonary artery but now compressing on his left bronchus.  We will be using this blog to update how Jarod is doing during and after surgery in Philadelphia.

Thanks for taking the time to learn about Jarod's Heart. Please feel free to e-mail me with any questions you may have at heartkdzrck@comcast.net.  

The photo below is a "normal" EKG for Jarod :)