ICDs in the Young: A Challenge for Physicians, and an Even Greater Challenge for Patients and Families

Ian Law, MD, Jean Gingerich, RN, BSN and Nicholas Von Bergen, MD University of Iowa Children’s Hospital
Ian Law, MD, Jean Gingerich, RN, BSN and Nicholas Von Bergen, MD University of Iowa Children’s Hospital
   For those who have had the privilege of caring for children and young adults with life-threatening heart disease requiring ICD implantation, you are well aware that much of the emotional impact of the ICD occurs after device placement.    Pediatric ICD implants account for less than 1% of devices; how- ever, with advancements in device technology, increasing genetic testing availability (potentially increasing the number of primary prevention implants) and increased longevity of young adults with congenital heart disease, the number of pediatric and young adult patients with ICDs is growing substantially.    ICD placement in pediatric patients results in unique psychosocial issues not typically seen in adults, including: living with a life-threatening illness, adjusting to a scar and “bulging” device (which may serve as a constant reminder of their heart disease and can have a dramatic impact on body image), and at times dealing with the loss of a family member. This occurs at the time when they are trying to develop social independence and a sense of self. For many, ICD placement leads to fear of peer rejection, altered body image, and concern of inappropriate or appropriate shocks. This fear may limit exercise, other activities, or result in fear of being separated from their “safety net” such that they may not return to school or go out with their friends. As they enter into early adulthood, their concerns turn to fear of intimacy, marriage, child-bearing and in general, fear of the unknown. Recently, one of our patients described her concerns about her disease and ICD placement, exemplifying many of the issues addressed above:     “So as far as psycho-social issues, there have been a few things over the last nine-plus years. Early on, it was getting over the hurdle of the self-esteem issues. Being a college freshman with community bathrooms meant anytime I walked through the bathroom with a towel I would get a few looks, or I couldn’t get ready for bed in just a tank top without those same ‘Ooh that looks really gross’ looks, and that was coming from other females. Granted, there was that time I had the same look and even those words from someone I had been dating. It took many summers to get over the fact that I no longer had beautiful shoulders and collarbones to show off.    As an adult, facing dating and hopefully eventually marriage, I continued to have a downhearted feeling that the ICD was going to find its way into my life. Three weeks into dating my husband, I had my first shock while holding his hand. I saw a flash of bright light blitz through my eyes, heard a boom explode in my ear, felt my chest and upper body rise and fall from my driver’s seat, and felt a crazy explosion blow through my foot, causing it to fall off the brake and my car rolled forward toward the garage. I felt that I was asking my boyfriend at that time, ‘Can you really handle this for the rest of our lives?’ If holding my boyfriend’s hand causes a shock, what will sex do? Yet now, even with the support of a husband, concerns and fears still linger. What will we face in the future? Will there be a high-risk pregnancy? Will our children need one of these someday? How about medical bills, surgeries and all the longer term concerns in addition just to the daily annoyances such as not being able to curl up in certain ways?”    In large part, adjusting to ICD placement in children varies from adults due to the level of maturity and breadth of life experiences; pediatric patients will wonder “Why has this happened to me?”, whereas adults will often view the device as something that has the potential to save their life. If you can think back to that time when your most overwhelming concerns were a new set of glasses, a pimple on prom night, or dropping a tray of food in the cafeteria, these types of concerns become magnified following ICD placement: “What if I get a shock in front of people?” or “I heard someone passed out and wet their pants, what if that happens to me?”    These unique concerns have lead us to proactively address the psychosocial and physical impact of ICD implant, including spending a significant amount of time discussing the disease process and ICD implant, answering questions, giving the patients and families educational packets, and often setting up an appointment with a psychologist who is knowledgeable about ICDs and their impact on the patient. This program is modeled after work done by one of the leaders in this area, Dr. Sam Sears. As a result of his research and extensive experience, Dr. Sears has written many enlightening articles on this subject, many of which we include in our informational packets: “How to Respond to an Implantable Cardioverter-Defibrillator Shock” and “Coping with My Partner’s ICD and Cardiac Disease.”    The diagnosis of life-threatening cardiac disease and placement of an ICD affects not only the patient, but the patient’s family and other loved ones. In many instances, the first indication that a patient (or family) has a life-threatening cardiac disease is following an aborted sudden cardiac arrest in their child. The ICD becomes a sign of poor prognosis, significantly altering the patient’s and families’ outlook on life. While challenging at times, our goal is to switch the focus from the fatality of the disease process to empowerment. This process is seldom if ever accomplished in one setting, and is filled with breakthroughs and set-backs. We have found one of the best methods for the affected individuals to gain acceptance and develop lifelong strategies to cope with their disease and ICD placement is to interact with others who are sharing similar experiences. This is especially important to children and young adults, who feel so incredibly isolated. To promote peer-peer interaction, we have identified a network of young ICD recipients who are willing to be contacted, or willing to contact patients and families who are undergoing ICD placement.    In addition to education, we have found that minimizing the physical appearance of the ICD placement can greatly accelerate the acceptance process. Showing a child an ICD generator can be instructional but very frightening, whereas showing a child a photo of another patient who has undergone ICD placement who is smiling or playing with their friends may alleviate many of their fears. Another seemingly minor step that leads to device acceptance is relocating the incision site for generator implant from the typical pectoral area (sub-clavian) to a more lateral site. We preferably make the incision for ICD generator placement along the anterior axillary line, and in thin individuals, place the generator under the pectoral muscle (Figure 1). Showing photos of patients with an axillary scar wearing a bathing suit or tank top can lead to a tremendous sense of relief in a patient who is scheduled to undergo ICD placement. Currently we are in the process of designing a study with Dr. Kathryn Collins (Denver Children’s Hospital) investigating the psychosocial impact of scar location in children and teenagers using quality of life surveys. We look forward to the results of this study as the data is gathered.    To meet the needs of our patient population, and the ICD population as a whole, we have organized an annual conference, “Young Hearts with ICDs” (now in its 7th season), that centers on patient education, support groups, and peer-peer interaction. Prior to the first event, our patients developed a mission statement for the ICD conference:     “We are a patient advocate group offering support and information related to the heart conditions and medical devices that affect our lives and the lives of our families. We exist to address the unique needs these conditions create and support the commitment it takes to maintain the devices that sustain our health. We work in partnership with our medical providers, relying on their collective specialized knowledge to support our mission. We encourage new ICD recipients and their families to participate in the groups at whatever level is appropriate and comfortable for their situation.”    This ICD conference has continued to gain attendance, and we have found that this conference has had a substantial impact on our patients and their families. There are few other places that family members can talk about the fear a parent has when their child receives a lifesaving shock, or the camaraderie that patients will feel when discussing their stories and experiences of being the only child with an ICD in their school. As one of our patients said, “These conferences aren’t just about an ICD; we are people living full lives, going to college, traveling, having children. We can share the difficulties and humorous moments that having an ICD can add to a normal life.” It goes without saying that the healthcare providers that have the opportunity to attend these conferences walk away better clinicians and human beings.    In summary, if we were to pass along one pearl to “take to heart” when caring for young patients with ICDs, it would be to extend your care beyond the four walls of a clinic room, or the confines of a hospital; move beyond medications and device implantation and address the essential and overwhelmingly important psychosocial needs of your patients, which will allow them to live more fulfilling, productive lives. To see more of the ongoing Pediatric EP blogs, please visit: www.eplabdigest.com