I wish we had a Tree of Hearts. Healthy new hearts would hang on the high branches and devices on the lower branches – low hanging fruit! Established guidelines about who, what, in what circumstances, and where, accompanied by a simple set of instructions for physicians all over the world to understand, adapt, and provide appropriate care for these technologies. A handbook for parents and their physicians with a simple statement – The Heart is a Muscle Too – Duchenne Hearts Matter.

 

There would be no need for donors. Parents would never have to sit at the bedside of their sick child, without options or limited expertise. They would not be asked to make impossible choices. There would be no waiting for a device or a new heart. No parent would carry the burden that a new heart for one means another life ends.    

 

My Tree of Hearts would come with guarantees, an assurance of more time, even if it felt like borrowed time.

My Tree of Hearts would have generic hearts, beating in rhythm, ready and available for anyone in need. The transplant team would call the Tree of Hearts nursery to make the request. Within seconds, the new, healthy heart would be on its way to the center. The patient would be prepared for the surgical procedure, chest opened and ready as the surgeon, heart in hand, would remove the broken heart and gently lay this new, healthy heart in its place. Gingerly, the surgeon would attach all of the vessels and in a single instant, the heart would take on the genetic characteristics of the recipient and beat strong and steady. No immune suppression required. Lub dub, lub dub. Lub dub. Music to the heart and to the ears of the family waiting in the wings. The surgical procedure would be easy, no complications. Joyful parents, tears in their eyes, would see their smiling son, the cloud of his broken heart a forgotten memory. Each day of recovery would be better than the last until the patient would leave the hospital, eager and excited about this new life.

 

The ICDs and VADs would come a list of instructions, the ‘how to’s” and essential steps for use. Standards established, proactively intervening. No waiting. None at all. The set of instructions for use would ensure optimal care and instill confidence where the device feels like new, unfamiliar territory. 

 

Some weeks back you may have watched PPMD’s Webinar on Ventricular Assist Devices or VADS for short. Cardiac surgeons from different institutions discussed VADS and the first US experience, a 26-year-old man with Duchenne performed at Cincinnati Children’s Hospital. The discussion was interesting, frightening, exciting, and humbling.  Another possibility. An opportunity for Duchenne – destination therapy for individuals in severe heart failure, now a potential for our sons. We listened, we learned, and parked the information away in the ‘just in case’ section of our brain.

 

And then one day, we woke up, signed on to our Facebook account and were heartbroken to learn that one of our own was in severe failure. All of us pushed the emergency button in our brain. How could this happen. So young. So unfair. So heartbreaking. So wrong. And we reached out to others, to anyone willing to listen, calling and begging for help.
One more time, the community raised their voices in protest. NO! NO HOPE AND NO HELP – words we have heard in the course of our journey and thrown out as impossible, intolerable, unacceptable, and unethical. The word rolled off of our tongues. NO!

 

In the last weeks we have learned more about VADS than we ever wanted to know. 

The idea of an artificial heart and assist devices started long ago. In 1982, Barney Clark received the first artificial heart, called the Jarvick. In 1982, (the dark ages of cardiac surgery) Barney Clark did not expect to live more than a few days, but hoped that Dr. Jarvick and future doctors might learn to save the lives of others someday. Thirty years later, the technology has improved and evolved, and currently there are several FDA approved devices.

 

A VAD is an implantable mechanical pump that helps pump blood from the lower chambers of the heart (the ventricles) to the rest of your body. VADs are used in people who have weakened hearts or heart failure. Although VADs can be placed in the left, right, or both ventricles of your heart, they are most frequently used in the left ventricle. When placed in the left ventricle they are called left ventricular assist devices (LVADs). The procedure to implant a VAD requires open-heart surgery and has serious risks. However, a VAD can be lifesaving if a patient is in severe heart failure. 

 

Here’s the hard part. Not every center has a pediatric VAD program. Not every insurance will allow patients to go outside their state in order to receive a VAD. And there are several types of these devices, each one is a bit different, requiring specific expertise, experience, tools, and management. Physicians are typically trained on one particular type. They become familiar, develop experience, and a certain comfort level with a certain device. Like all of us, we go with what we know, what we can rely on, what is familiar. Physicians at other institutions may prefer a different device. There is no ‘one size fits all’ in terms of devices and physicians are not ‘jack of all trades’, able to adapt and feel confident in procedures and devices that are unfamiliar.   

 

Management of the VAD has serious challenges. ERs have to be alerted in case of an emergency. They have to have tools and knowledge in the event of an emergency. As you might imagine, the local physicians – all of them: cardiac surgeon, cardiologist, neurologist, pulmonologist, nurse practitioner, ER physicians – have to know what to do when, have to have familiar tools, significant expertise and ability, and more than a little experience if the patient is to survive. 

 

At the end of the day, VADs will be useful in Duchenne at certain times and in certain circumstances.
Here are our marching orders:

  1. Convene interested centers/surgeons to begin to develop guidelines and standards
  2. Publish and ensure the community has access to the standards (transparency)
  3. Educational information for physicians and families
  4. Contact information – questions, information and soon, a helpline

 

While my Tree of Hearts sounds like wishful thinking at the moment, we will convene leaders in the field to begin to develop standards for the use of devices such as ICD and VADs, and expand our understanding of the policies and practice of heart transplantation.   

 

For Matthew.

For Mitchell.

For all of our sons.


Pat Furlong, Founding President, CEO
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