Tag Archives: heart defect

Moms Shouldn’t Have to Do This!

We just finished our nightly medicine routine.  Which right now includes this:

Abby is on Nupogen right now…which is administered by a shot.  Nupogen is a medicine meant to help Abby’s bone marrow increase white blood cells.  We are quickly learning that the post-transplant medicine regimen is a balancing act.   The balancing act is difficult, as each patient reacts differently to different medicines.  What works for one patient, does not necessarily work the same way for another patient.

Killing the white blood cells is a part of life for a transplant patient.  The white blood cells are purposely killed off so that the body will accept the new heart.  Abby is on medicines that are purposely meant to do this.  As a result, she is susceptible to catching the diseases and viruses floating around.  To help combat this, they prescribe medicines that help fight off bacterial, viral, and fungal infections.  One of these is called Valcyte.  Apparently, now and then, the Valcyte reacts with the anti-rejection meds and causes critically low white blood cells.  Abby is one of those patients.  She can not come off the Valcyte until she is off the prednisone (steroids).  The steroids help prevent rejection, so they can’t just take her off.  Balancing act.

Abby’s white blood cell count is critically low.  It is at 200.   Cardiologists try to keep transplant patients around 2000-4000.  200 means that Abby can catch ANYTHING and anything she does catch can be extremely dangerous.  So, luckily, the Nupogen shots should work.  She has already had one set of shots last week.  Instead of working like they were supposed to, her WBC fell from 500 to the 200 we are at now.  So she was put back on the Nupogen shots, at a much higher dose.  We opted for the in-home health company to deliver the shots to us so we didn’t have to drive to the hospital to get her the shots.

So, Justin and I have been giving her the shots.  I know that parents sometimes have to give their children shots.  Diabetes patients, cancer patients, and others give shots on a daily basis, for a much longer time than I have to.  I’m not here to say that these few shots are the end of the world for us.  I’m not here to try to make a bigger deal out of this than it is.  The needle is small.  It just has to be given in a fleshy part of the body.  It really isn’t that bad.

What I’m here to say is that NO parent should have to give their child shots.  The flinching, the bleeding, the “ouch” muttered under her breath…I hate all of it.  I hate KNOWING I am causing her pain.  I am usually the one to comfort.  I am the one to hold her hand while someone else puts needles in her.  My natural instinct is to try to ease her pain, to talk her through the fear and anxiety associated with needles.  I can’t do that if I am rubbing her arm with alcohol, counting to three, and sticking a needle in her arm.  I hate it!  I am so sorry to all you parents that have to do this daily, for a child’s entire growing up years.

I know that shots aren’t the end of the world.  I know that often the anxiety over the stick is worse than the stick itself.  The bottom line is that I just don’t like being the one to cause any of this.

All that being said, sometimes this road requires us to do things that we hate doing.  None of us likes to put our child through pain and fear and anxiety.  But I would do it…again and again…if I had to.  I do it because I love her.  I do it to keep the sacred gift inside her chest beating as well as it has been since she received it.  I do what I have to…just like so many mother’s of children with perfect broken hearts.  And in doing so, I find a strength within myself.  No mom should have to do this…but sometimes, we have to.

Thank You Local Media for Featuring My Daughter

STEVE SMITH/Special to the Standard-Examiner Abby Wardell (third from left, back row) participates with other cast members in "Joseph and the Amazing Technicolor Dreamcoat" at the Terrace Plaza Playhouse

Standard Examiner reporter, Jamie Lampros, featured a wonderful article about Abby and her heart defect.  You can read the article here, or you can read the same article on the Standard Examiner blog here.  We were so pleased with the response we have received so far from this article and were even more thrilled that Abby was able to discuss important issues surrounding heart defects including pushing for mandatory pulse ox testing, detailing some of the frustrations of growing up with a heart defect, and giving hope and encouragement to others in the same situation.  Lampros called Abby “Amazing” and I couldn’t agree more!  While our last surprise media experience turned out to be somewhat discouraging, this story on Abby was wonderful.  I hope that if you have a minute, you drop Lampros a line and let her know how much we appreciate her work for Abby and for all our broken hearted warriors.  You can leave a comment on the story to thank her for her work.  Let’s show her as much support as we gave Mr. Trentelman.

And, speaking of amazing Abby…yesterday’s round of tests and hospital visits proved she deserves this title.  Abby’s heart function has shown an increase!  The biventricular pacing, or CRT (cardiac resynchronization therapy) worked, along with the medicine regimen, at least enough to give Abby’s heart a boost.  Very few people are candidates for biventricular pacing, and even fewer pediatric patients.  The hopes of having Abby’s heart improve with this type of pacemaker therapy was a long shot.  At a great website, CHFpatients.com, the reasoning surrounding this kind of treatment for heart failure is discussed.  Very rarely is the left ventricle paced in conjunction with the right ventricle.  This kind of treatment is somewhat new and is mostly only used in adult populations.  Abby’s team felt it was worth a shot for her as she met all the requirements for CRT.  And that shot worked…at least enough.

Enough that Dr. Everitt felt that she was willing to present Abby to the board next week to see if Abby could have her scoliosis surgery before transplantation.  This scoliosis surgery has really given us a lot of frustration towards how to best treat Abby’s heart failure.  Without the scoliosis surgery looming over our heads, we would be content to let Abby go on like she is now until she showed more severe signs of heart failure whether that was in two weeks, two months, or two years.  Only then would she be listed.  Abby’s curved back, which is now progressing at a rapid pace, was a problem though.  Her back surgery needed to be done within a year or 18 months at the most, otherwise the progression of her spinal curve would be severe enough that treatment would be nearly impossible.  Listing Abby for a heart transplant before her heart had completely given out was the only option we thought we had so Abby could get her back fixed.  Having the back surgery after transplant was not a great option either, as the bodies ability to heal and fight of infection would be greatly diminished due to the transplant medicines.

Now, if the board of surgeons and cardiologists approve it, Abby will be able to have her scoliosis surgery before transplant.  The surgery will be risky…very risky.  A special team of cardiac anesthesiologists, cardiac nurses, and OR staff who have worked with heart patients before will be in the operating room monitoring every breath and every beat and every vital sign.  Of course, an ECMO machine will be on hand in case of serious complications.  We know the risks are high, but we have no good or safe way of proceeding.  A choice has to be made and we, along with her cardiologists, feel this is the best way to move forward.

Abby’s road still leads to transplant, but not before her scoliosis surgery, both of which are HUGE hurdles this little fighter has to make it over.  And to be honest, I feel that she will.  This direction feels right to me.  This way of moving forward has made the most sense to me since learning about Abby’s heart failure in October.  So, our good news includes a major scoliosis surgery most likely taking place in the next six months, but for our perfect broken hearted girl, the looming surgery is hard to gear up for, but one she knows needs to be done, and she has taken the news like she always does, with the grace and optimism of an angel on earth.

Finding A Balance Between Overprotecting and Responsible Parenting

Parenting any child is difficult.  Instinctively, I want to do all in my power to prevent pain, harm, or injury to my children.  I am naturally a mother who leans more towards the overprotective type.  Having overprotective tendencies and a child with a congenital heart defect is a recipe for a world of confusion and frustration, especially when it comes to how much physical activity is good for my child.  I know this discussion, in one form or another, crops up frequently in the heart community.  Just last week I noticed comments or questions in different social media sites geared towards this very question.  A mother of a child with repaired Tetralogy of Fallot was wondering if organized soccer was okay for her adolescent son.  An adult CHD’er living with tricuspid atresia commented that she felt parents made a much bigger deal out of their children’s heart defects than was necessary.  An adult Tetralogy of Fallot survivor with a pre-teen with Tetralogy of Fallot asked why parents limited their child’s physical activity when the cardiologist suggested no physical limitations were necessary.

These questions and others like them are inevitably part of raising a child with a heart defect like Tetralogy of Fallot.  The opinions are varied.  While some parents feel no restrictions are best, just like the doctor ordered, others have a hard time feeling good in giving their heart child free reign to exercise or engage in physical activities.  The fears of how strenuous exercise will affect an already fragile heart weigh heavily on their mind.  As children with heart defects get older, these complications will be multiplied as the child themselves will begin to ask to play sports or be involved in activities that are physical in nature.  I went in search for the answers for myself.  I want to know what is best for my TOF girl.  The answers I found may be of interest to you too.

We have been told by Abby’s cardiologist since she was very young that she “will limit herself” and her body will “know when she needs to rest.”  If Abby wanted to play soccer or swim or run was all determined by her body cues.  If she was tired, she should rest.  If she was out of breath, she should stop exerting.  This sounds nice.  No restrictions is the gist.  But let’s put this to the test.  Abby has been a VERY driven and determined child since birth.  If something  was hard to do, Abby was determined to conquer.  Not only is Abby a go-getter, but her small size has always bothered her.  She is a full head and shoulders smaller than all her friends and has always made up for being small with a larger than life zest for life.  Abby has been known to push the limits from the time she was in school.  Keeping up with peers and living a full life is something that Abby strives for.  If they are playing a game in PE and she feels winded or tired, she will attempt to push through it.  That’s what you learn in PE isn’t it?  Push through the pain.  Just give us ten more.  Don’t stop now.  No pain no gain.  So, in a world where she is taught that to gain anything, you must push through even when you want to stop, coupled with her determination, the cardiologist’s advice that she will know when to stop does us no good. I would have to go elsewhere to find specific recommendations for Abby’s physical exertion.

I found a wealth of information in an article titled “Phsyical Activity Recommendations for Adolescents with Repaired Tetralogy of Fallot: Review of the Literature and Guidelines for Practitioners.”  The article was written by two APRN’s, Rachel Caplan and Patricia Jackson Allen.  Right of the bat the article states that “adolescents living with Tetralogy of Fallot have not been provided clear, understandable information regarding their congenital heart defect (CHD) and its implications on their ability to participate in physical activities” (191).  The article cites many studies in which parents and patients both admit that they are unclear on if they can participate and how much exertion is okay.  It has been found that parents often misinterpret information given by physicians and pass this misinformation on to the CHD patient.  As many as 39% of TOF parents restrict their child’s activity levels unnecessarily due to misinformation.

The article stated that all parents should have a  deep understanding of their child’s heart defect.  Let me repeat, a DEEP UNDERSTANDING.  Not a basic synopsis.  Only 28% of parents could correctly identify their child’s specific heart condition in a diagram.  Why would this be important?  In order for a parent to make an  informed decision as to which physical activities are appropriate for their CHD child, they need to know how the heart is affected and what effects will in turn be placed on the body.  Does your child’s CHD affect the right ventricle or left ventricle?  Are the arteries affected?  Are the pulmonary functions affected?  Is blood flow, blood pressure, or blood volume increased or decreased as a result of your child’s CHD?  Do they have valve problems?  Regurgitation problems?  The answers to all these questions will directly affect which activities and sports are appropriate and encouraged for your child.  Since you don’t always get a clear cut answer as to which physical activities are okay from the doctors, you need to know to act for yourself.  You can’t rely on what other parents have let their child do, what Shaun White is able to accomplish, or what your child wants to do, because every case is different, and there is no one size fits all answer.

The article stated that in 2007 the American College of Cardiology Foundation at the 36th Bethesda Conference came to a consensus that “children with TOF who have had a complete TOF repair who have normal or near-normal right heart pressure, have mild or no right ventricular volume overload, have no evidence of a significant residual shunt, and whose ambulatory electrocardiogram or exercise test shows no atrial or ventricular tachyarrhythmia abnormality can participate in all intensities of static and dynamic sports” (197).  The article highly recommends that ALL heart patients who will be participating in sports or physcial activities have a complete exam which includes exercise tolerance tests, pulmonary function tests, EKG’s, ECHO, X-Ray and physical exam by a cardiologist and/or sports cardiologist before participating, making sure the cardiologist knows that the visit is specifically to determine how much physical exercise is appropriate for the patient.

The physical guidelines given by the foundation suggested 30-60 minutes of daily moderate to vigorous activity for all TOF patients with repaired TOF and NO other complications.  That being said, the article also stresses that “individuals who have significant residual abnormalities after corrective TOF surgery, and who also have a history of syncope and/or arrhythmias, may be at risk for sudden death” (193) when participating in sports.  TOF with pulmonary regurgitation, artery anomalies, or other surgical complications should refrain from strenuous activities and stick to low intensity sports and activities.  It is also noted that TOF patients should be careful when playing contact sports and/or sports in which fainting may cause severe bodily injury.  Tables are included in the article which include guidelines and recommended sports for TOF patients.  The article is a PDF file and will not allow me to cut and paste but I would LOVE to email you the tables if you would like!  It is also important that you have regular follow-up visits with your cardiologist and keep them informed of the CHD child’s activity level.

It is important to understand that NO other parent or CHD patient can decide for you how stringent you should be on physical activity for your child.  Evidence has shown that exercise is valuable for heart patients but every heart patient is different.  The only people who should decide on what activity levels are appropriate for your child are you and your child’s doctor.  Each patient is different and your child may have limitations mine does not just as mine may have circumstances that prevent physical exertion that your child is free from.  This blog post is meant only as information I found interesting and hopefully helpful.  I am not advising one way or the other if your child should participate in sports and/or which sports they should be allowed in.  The article  helped me clarify my understanding of the importance of physical activity for my TOF child, relay some fears of how physical activity might affect her,  as well as the importance of understanding her broken heart and the implications that broken heart has for Abby’s physical well being.

Caplan, Rachel & Patricia Jackson Allen.  “Physical Activity Recommendations for Adolescents with Repaired Tetralogy of Fallot: Review of the Literature and Guidelines for Practitioners.”   Pediatric Nursing, Jul-Aug 2011  37(4): 191-9.  Retrieved from Academic Search Premiere.  21 Mar 2012.  Web.

 

Unexpected

I’ve said before that none of us on the road with congenital heart defects never really knows where this road might take us.  Life has been going on like normal for us.  Abby was just like any other kid…doing the things kids do…but two days ago, we were forcefully reminded that Abby has a heart defect, that Abby is fragile and extremely resilient all at the same time.  We were reminded that Abby has a perfect broken heart indeed. 

We had a routine cardiology check-up Tuesday…although right now that feels like it was ages ago.  We were getting ready for Abby’s scoliosis surgery.  It was scheduled for Monday, October 10th.  Abby’s orthopedic surgeon requested Abby have a heart check-up before he was comfortable operating.  I can’t help but wonder if that surgeon, Dr. John Smith, was somehow inspired to ask us for that check-up before her surgery.  He quite possibly saved her life. We came in for the check-up, with the usual routine-chest Xrays folllowed by an ECHO and then a visit with the Dr. Day.  The radiologist took the first Xray and I immediately saw that Abby’s heart was too big.  It was touching all the way out to her ribs.  Something was definitely not right.  She had her Echo and then we met with her  cardiologist.  He confirmed my small concern about her heart size, and followed with news that Abby’s heart function was concerning.  His line of questioning about us noticing if Abby had decreased energy, swelling in hands or eyelids, fatigue with exercise was definitely NOT usual.  His complete shock that Abby had actually gone swimming over the summer and not felt fatigued triggered that old sense of dread, nausea, and panic.  He then related the news that Abby’s left ventricle was functioning at 15%…

It was like a punch in the stomach.  I’ve been there before though, and it didn’t catch me completely blind sided, but it definitely took us by surprise.  Her heart was basically in heart failure, only Abby was exhibiting none of the outward signs of failure.  Back surgery was off, and Abby was admitted.  We all felt a bit dazed and confused. 

Abby was started on an IV administered drug called Milrinone.  The next morning a repeat ECHO was done, again showing us decreased function in the left ventricle.  She sat all day, NPO (the dreaded no food by mouth) until 2:00 PM when she was taken for a cath.  It was actually the first Cath Abby has ever had.  A part of me was hoping, really hoping, that they would find the problem, and fix it.  Whether that meant a stent or a reworking of the coronary arteries…at least it would be fixed.  We were not so lucky.  About three hours after they started the cath they paged us back to discuss the findings.  Abby’s cardiologist and surgeon explained that Abby’s coronary arteries looked normal.  Function was good, size was good, nothing in the coronary arteries would explain her heart failure.  

So, we received no answers, and moved on to plan B.  Biopsy’s were taken of Abby’s heart and sent off to labs in Salt Lake and in Cincinnatti.  This was most likely the result of a virus.  A virus.  Respiratory bugs, gastrointestinal bugs, those nasty little germs that make most kids sick for a few days and then clear up.  They are most likely to blame.  Abby picked something up and it attacked her heart.  If, and I say if because it could just be that Abby’s heart is plain old wearing out, but if a virus has caused this, we are looking at one of three scenarios.  

1.  Abby’s heart will function poorly for up to six months.  With medicine, her function will be helped along until her heart fully recovers on its own. 

2.  Abby’s heart will continue to function poorly, never fully recovering, but able to be stabilized and helped with daily meds and close check ups.

3.  Abby’s heart is shot.  The meds may help function but most likely will only be a temporary fix, a band aid so to speak. 

So, we sit here in the CICU right now with more questions than answers.  It is 5:00 AM and I’m watching her sleep–praying for answers and praying that the road ahead of us is the easier path, but knowing that Abby’s broken heart will take us where it will.  Today we will have an MRI, hopefully telling us how much of the heart muscle they feel is viable and how much is just dead tissue at this point.  We should also get back some of the results of the biopsy, and we may be able to tell what caused this and if they feel Abby’s heart function may return.

She will be started on a some oral meds today which will hopefully help the heart function better.  These meds are a trial and error for getting the right dosage to help the heart work properly but not cause too much increase in blood pressure. 

As for Abby, she is characteristically amazing to the staff here.  They all expect a “very sick” little girl, only to see Abby, chatting, walking, asking questions, and seemingly unaffected by her weakened heart.  Why she is not sick, is baffling, but hopefully a positive sign that her heart she has now is recoverable and can keep on sustaining her.  

As for me, of course the questions flood my mind.  I wonder…did I miss some signs I should have?  Was she fatigued or exhausted and I didn’t notice?  Could I have prevented the virus that may have caused this?  and most importantly, What now?  Where do we go from here?  How will this affect my broken hearted girl?  Will life change from here on out?  Are we up for what may lay ahead? 

I hope that in the coming days, we can answer some of those questions. Until then, I hope for good news.  I prepare for bad news.  And I love my daughter a little more.  Cherish her wit and charm and spunky Abby”ness” fully. Until then, I love Abby’s brothers more.  I feel gratitude for family and friends who have dropped everything to come be at our side or take care of our kids or sit with Abby when I couldn’t be here. Until then, I lean on my husband and look to him for strength when mine is waning.  Until then, I stand in awe of those taking care of Abby, working to fix this problem.  Until then, I pray a little more and remember that every minute we have with those we love is precious.  Until then, I sit at Abby’s side, holding her hand, counting the beats of her perfect broken heart.

Protecting the Broken Hearted

It seems as a parent of a child born with a broken heart, my field of vision is often confined to her heart defect.  When I look at her, I can’t help but think about her in terms of the heart defect.  I have only experienced two days of her life where I wasn’t aware that something was wrong with her heart.  We celebrate anniversaries of surgery dates.  Time is often measured by when her last cardiology visit or open heart surgery was, and I’m constantly aware of the milestones and accomplishments she achieves because I know many who don’t get those.  Seeing her without thinking about that amazing but broken heart is difficult.

I’ve preached here before to make sure that Tetralogy of Fallot does not define your child–and I stick to that.  She never looks at herself  and thinks, “It’s pretty amazing I got to come to Disneyland and ride this roller coaster considering I have a heart defect” or “It’s been eleven years since my open heart surgery to correct my Tetralogy–it’s amazing what I’ve accomplished in that eleven years.”  Those are my thoughts, reserved to me alone.  Abby is just like any other eleven-year-old, whose thoughts focus on how cute the clothes at Justice are or how much she loves Taylor Swift or how excited she is to see the last Harry Potter movie.  Her heart defect is a fleeting thought, a part of life like visiting the dentist or having a physical–nothing more to her.

At the Dentist

I’ve heard adult CHD’ers claim that parents of heart children make a bigger deal of the heart defect than the actual heart patient themselves.  I completely agree with that statement…after all, when a doctor hands you a brand new, tiny, beautiful baby and tells you their heart is seriously flawed, your world comes crashing down.  The baby however, is blissfully unaware of her flawed heart, working overtime to keep her body alive.  After all, when you walk into an intensive care unit and see your tiny baby covered from head to toe in wires, tubes, and bandages that are keeping her alive, the emotional impact is huge.  That baby will never remember a thing from the traumatic experience.  Parents naturally make a bigger deal out of their children’s heart defects because they KNOW the difference.  They know that life should not be that way–that it isn’t fair!

There is a but to those statements…one that heart parents would do well to heed.  There is a danger in overprotecting the broken hearted.  Studies have shown that adults living with heart defects, which is now at 90% of those born with heart defects, who are overprotected during adolescence, suffer from heart related anxiety as adults.  The study encourages parents to stress greater independence in their heart children which will foster improved psychosocial adjustment.  They will be less likely to need attention due to their heart defect and will not need to define themselves according to their heart defect–in essence, having better self-esteem and social behaviors.

  To be honest, that is easier said than done!  Giving up the overprotective urge is difficult.  But it can be done–within reason.  I will always overprotect Abby to some degree.  I tell her teachers about her heart defect and encourage her to sit out on activities if she feels tired or fatigued to an extreme.  But she is allowed to take gym class (with a doctor’s okay).  I worry about her when we ride roller coasters or swim in the ocean.  Hot days create a broken record effect in which poor Abby hears me ask “have you been drinking enough water?” 100 times a day.  But she is also encouraged to play outside on hot days.  She is allowed to ride the roller coasters (with the okay from her cardiologist).  She is taken to the beach or the mountains or backpacking or camping or swimming…just like all her friends.  She is given every opportunity to live life to the fullest…after all, we didn’t do all in our power to save her life to make her sit it out on the sidelines!

I have to constantly find that fine line between overprotectiveness and being a responsible parent to a heart child.  Abby’s cardiologist and pediatrician help me create those lines.  Every activity is discussed and weighed out but, finding the balance can be done.  I am far from perfect at it, but I try to take off the heart shaped glasses frequently, and remember that Abby is not just an eleven-year-old with a heart defect…but just an eleven-year-old!