It doesn’t have to be this way

There is a new report being presented at the American College of Cardiology meeting in Orlando today. You could conclude that this report is disturbing, but I want to dispute it. No, I am not saying that the report is incorrect, but that you don’t have to be “trapped” by the results.

This report contends that even though Congenital Heart Surgery techniques save a child’s life, their quality of life may not improve. The full text of the press release is here. Since the full report is not available at the moment, let’s look at a few points in the press release:

“Advanced treatments often result in unintended complications, particularly when combined with the hemodynamic impact of the heart defect itself,” says Bradley Marino, MD, a cardiologist from the Heart Institute at Cincinnati Children’s Hospital Medical Center and the study’s lead author. “The child’s neurodevelopmental, psychosocial, and physical functioning are all impacted by these complications, and they may adversely affect the child’s quality of life. If we know the predictors, we can put interventions in place to change the outcome for them.”

I actually agree with and support what they are trying to do. As we all know, even though two children may have the same heart defect, the physical effects can be vastly different based on the severity of the defect. Two people, for example, can have a Ventricular Septal Defect (VSD), yet one can be affected by it much more than the other due to how big the VSD is and where in the heart wall it is located.

To measure how much of an effect these factors have on children, Dr. Marino and his team developed the Pediatric Cardiac Quality of Life Inventory (PCQLI), a self-administered questionnaire that quantitatively assesses health-related quality of life in children and adolescents age 8-18 years, and their parents. The questions are designed so that anyone with a third grade reading level can understand and answer them in fewer than 10 minutes.

Dr. Marino and his colleagues recruited 759 pairs of patients and their parents. The patients were between the ages of 8 and 18 and had at least one prior cardiac surgical procedure. The researchers evaluated the effect of income, socioeconomic status, recent hospitalization, Fontan palliation (a surgical approach to treating hearts with one ventricle), and a greater number of doctor visits in the past year.

The Quality of Life Inventory seems to be a list of simple questions that 759 patients and their families answered. “Fontan palliation” means that the patient only has one ventricle, so this would put patients with Hypoplastic Left Heart Syndrome (HLHS) and defects that affect the right ventricle – like Tricuspid Atresia – on the study list. So I would be considered for this study. Also, it seems that there are very few factors being studied that the patient can affect. Number of doctor visits could be affected by both income and socioeconomic status, as a family with fewer resources could be more likely to skip a doctor’s appointment.

Modifiable factors associated with lower quality of life scores among patients after surgery included recent hospitalizations and greater number of doctor visits. Non-modifiable factors included Fontan palliation and both lower income and socioeconomic status.

The study takes this into account – you can get more or fewer “points” based on hospitalizations and doctors visits. But a lower income, a lower socioeconomic status, AND having only one operational ventricle are automatic deductions.

So I get points off for only having one working ventricle. The study is right, this IS a limiting factor. But the study is also wrong… I have met a lot of Tricuspids and HLHS survivors who are full of life. Two women I met at the Adult Congenital Heart Association (ACHA) convention in Philadelphia both have Tricuspid Atresia, both of them are full of love for life and have a full schedule. One of them is engaged! I love them both like sisters – I’ve said before that my cousin is the sister I never had, but now I have THREE sisters!

I also met Rose, a young lady with HLHS who was looking into an uncertain future, because she is among the oldest HLHS survivors alive and no one can predict what might happen to her in the future. So Rose had brought her heart-healthy fiance to the convention: If he was going to put a ring on her finger, he needed to know what the score was! (You go, Rose!)

Do not get me wrong – living with a heart defect, especially one that leaves the patient with one ventricle, can be difficult. You have to adapt an attitude that you are going to take care of yourself, and be disciplined enough to know that there are things you can’t do and things you shouldn’t eat, and stay away from them. It is difficult to find a child with this attitude – that kind of self control only comes with age and experience. Even now, I “misbehave” occasionally.

So don’t let this study turn you into a “woe is me!” person, or make you into an overprotective parent. Your quality of life is determined by what you do, and how much effort you put into it.


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2 Responses to “It doesn’t have to be this way”

  1. Pages tagged "incorrect" Says:

    […] bookmarks tagged incorrect It doesn’t have to be this way saved by 14 others     YondaimeThe4 bookmarked on 03/30/09 | […]

  2. Amanda (Liam's Mom) Says:

    Thank you! Our son Liam has a great attitude after 13 heart surgeries (only 5 open-heart) the rest were caths and on weird wire removal thing. Liam is only five (six in a few weeks!) but even though he has tricuspid atresia + TGA which makes him surgically something in between HLHS and HRHS, we go hiking in the mountains here in CO and make him walk to school. We go slow, but we go. We try not to let his heart condition limit his exposure to new ideas and experiences or to let him become what one very wise and experienced pioneer heart mom calls a cardiac cripple.

    I don’t know, I guess that all you heart kids and heart grown ups have enough stacked against you to have any of the joy or newness of life diminished by our parental fears or hovering. It’s my fear, not Liam’s. I know someday he’ll have to face his own fears as he starts to “get it” – and he’s already starting to get it, but why everything all at once? I want to help him deal with whatever comes AS it comes and not spend every day waiting for shoes to drop when we can be enjoying the days between the really hard times behind us and what may happen one day.

    I think that attitude makes up for a LOT of disadvantages. There are so many people on this planet who. on paper, have it WAY better that my son or than you do, yet they are far more miserable and difficult and don’t have the excuse of a broken heart. I just don’t want Liam to ever make his heart and excuse to not live his life fully or to think it’s ok to do that. Yes, it is unbelievably unfair that he has these strikes against him, but it’s up to him and his grace to determine how he will handle the life he does have even when it becomes more challenging as he ages.

    Thank you for your blog, it shines like a beacon for those of us on the road behind you.


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