Patient age ranged from 27 to 76 years (mean 57.7 years) and the majority (65.3%) was male. All New York Heart Association (NYHA) functional classes were represented. Time since the first known diagnosis of HCM varied (range 4–45 years, mean 16 years) Mean time with an ICD was 6.7 years and ranged from 2.4 to 16.6 years, with the exception of one patient who had an ICD for 1 year before explant due to heart transplant. Both primary (n = 20) and secondary ICD indications (n = 6) were represented. Experiences of at least one appropriate or inappropriate ICD shock were reported in 3 and 8 different patients, respectively. Characteristics of each participant are described in Table 1.
The findings of the study are presented as 10 narrative themes describing the experience of HCM patients living with an ICD. The order follows the narrative thread in the patients’ interviews and serves to depict that some things often happen before other. In the discussion section these narrative themes are further interpreted and reflected upon at a more abstract level in the light of 5 theoretical themes.
HCM symptoms, diagnosis, and medication
Shortness of breath was the predominant HCM symptom, which was especially pronounced at exertion. Other symptoms were unspecific, such as tiredness, lack of stamina, syncope, and palpitations. Notably, no patient suffered from chest pain. ECG signs or a murmur sometimes lead to an echocardiography confirming HCM, but discovery also occurred during family screening or as a result of medical investigations for other reasons, including childbirth, general surgery, or when an infection, stroke, or cardiac arrest were managed.
The HCM diagnosis was often delayed and initially misdiagnosed as something else and the patients occasionally expressed worries about health providers´ actual knowledge about the disease. This trust was particularly damaged when a relative experienced SCD. Patients with a family history of SCD were easily convinced of the value of an ICD, whereas patients with other risk markers, such as non-sustained ventricular tachycardia sometimes questioned the need of an ICD before implant. At the time of interview, the term hypertrophic cardiomyopathy and its abbreviation HCM were unknown to many and they called the disease an enlarged heart, heart trouble, or heart thickness. One young patient said, Then (at the time of implant) they said hypertrophic cardiomyopathy…and I really understood it, while others required their physicians to write the term down for them. The patients reported high compliance with the prescribed HCM related medications (beta-blocker or calcium-channel antagonist) despite lack of short-term symptom relief, but dosage was often lowered due to presumed side effects.
Although HCM is not always diagnosed early in life, women of childbearing age still reported that they wanted to have children despite the risks of passing on the condition to their children. One severely symptomatic older man said that he would have had fewer children if he had known what the disease progression would mean, but otherwise did not have much concern. Parents of young children pondered the consequences of genetic testing for their children. One couple talked openly to their child about HCM to avoid confusion. Even when patients received genetic counseling, they sometimes had only a vague understanding of how the disease can be passed on to their children. Cascade screening was challenging and sometimes impossible due to broken families, estrangements, and dysfunctional family dynamics, such as the young woman who could not be tested for HCM because her parents did not tell her that the disease ran in the family. No patient in the study blamed parents for their HCM.
Implant decision, surgery, and wearing an ICD
Few primary prevention patients had a clear idea about the risk markers that made them eligible for ICD implant and sometimes these patients were not initially motivated to get an ICD. The experience of the implant procedure varied and they often recalled considerable pain. Complications requiring surgery were tolerated but some patients thought that preoperative information was sometimes lacking. Others reported feelings of isolation: When I was waiting for surgery, I felt like a chicken going into the slaughterhouse.
All young patients disliked people staring at the scars from the implant procedure, especially when bathing, but after some years many joked about the scar and claimed the ICD was a part of their body. An elderly woman said she initially avoided certain clothes which exposed the device, but later on, this did not trouble her. Some male patients even let people touch the scar. When ICD patients were playing with children, the device served as a reminder of the disease and made it real. A mother of a 5-year old daughter called it the life-saver and her daughter said she wanted one as well. Descriptions like my heart runs on batteries were common and show awareness and acceptance of living with technology. Gratitude, trust, and security were expressed along with a feeling of privilege because it is such a costly device. However, the device sometimes caused local irritation and required padding when using a seat belt or carrying a backpack; patients sometimes said they needed a cushion when lying in certain positions in bed.
ICD knowledge and worries
A few patients knew that the ICD shock-function could temporarily be inhibited by magnet application; among patients who experienced inappropriate shocks this was common knowledge and some even had a magnet with them. Patients were worried about the lack of ICD-specific knowledge among health personnel. They had encountered this lack of knowledge in emergency care, primary care, and specialized care outside of cardiology units. The ICD card, which is provided to all patients, was considered helpful but there were suggestions for necklace or a bracelet with information, and one patient obtained one from a patient organization. Such easily visible identification could prove invaluable in an emergency situation, in which the patient was unable to communicate.
The difference between a pacemaker and an ICD was generally common knowledge among patients but they did not think this was known to the general public or among health care providers. The experience of the vibration alert function of some ICDs was sometimes confused with an ICD shock; some individuals realized this for the first time during their interview. The ICD device usually contraindicated medical investigations such as magnetic resonance imaging or transcutaneous electrical nerve stimulation which limited full access to health care. Some patients had reflected about deactivation in case of terminal illness and were concerned that health care providers would not recognize the ICD or distinguish it from a pacemaker at life’s end.
ICD provides reassurance
All patients felt secure and grateful to receive an ICD. None regretted the decision to implant the ICD. They often had nicknames for the device, i.e. my life-saver, the fire extinguisher, a friend of mine, and life insurance. The word secure was announced numerous times by different patients. A young man whose father died suddenly at an early age felt overwhelmed and said, Everybody should have one…I am protected but they are not….
Even patients who experienced several inappropriate shocks persevered and accepted the therapy as part of their new life. Some of them came to terms with the shocks within a couple of weeks and one woman said, You know that it [the ICD] actually works. Other expressions were, it was horrible, unpleasant, but I know I won’t die from it and I know how it feels…it is just a dreadful feeling. Another commented, It is damn nasty, really nasty, but there is no pain afterwards and it feels like a strong electrical discharge. Typically they described their first feelings as, scary, nasty, unpleasant, horrible, terrible, dreadful, and ghastly. Metaphors for the shock were being hit by a stone, and being shot by a revolver, and I jumped a foot, and I was like a jumping jack. The unpredictable nature of shock therapy was described as a bolt from out of the blue, but these victims of inappropriate shocks came to terms with the shock and actually felt reassured after a few weeks. One patient who got the opportunity to talk to her device physician the same day a shock occurred, felt immediate relief. Most of the time, repeated ICD shocks caused a witness to summon an ambulance. Close relatives who witnessed a shock might become overly protective or avoid situations like the one that preceded the last shock. Although patients typically coped with the situation at the time it happened, relatives were also influenced by the dramatic event. A 4-year-old daughter avoided physical contact with her father for a short time after he experienced several shocks. One exceptional case involved a disappointed patient who had experienced complications, including device system infection and several inappropriate shocks due to a fractured Sprint Fidelis lead. She had considered (but rejected) device explant in favor of an external defibrillator. She described her situation as, I can never relax … and be a human being again. However, she appreciated the fact that her device had also delivered appropriate therapy. When experiencing an appropriate shock due to ventricular arrhythmia one patient typically fainted, but felt almost normal soon afterwards. It was a dramatic event for the people around him, but not for the patient who did not always seek medical attention after a shock. A survivor of a ventricular arrhythmia described his adrenergic response, It was fantastic…I was sitting in a dark room and everything turned bright white.
Feeling healthy despite disease
Individuals spontaneously described themselves as healthy and did not perceive themselves as victims of disease. Their identity did not change even though they had to undertake several changes in their life. They typically denied being sick because they had adapted to a new lifestyle and accepted their limitations: My husband has energy but I have almost no energy, and I learned to live with it, and I listen more to my body… Upon reflection, they occasionally did realize that they had experienced a life-altering event. These changes may have kept them away from certain activities and was most dramatic after surviving a cardiac arrest. As time passed, patients coped with these events, reoriented themselves, and achieved new goals. When asked about his heart problem, an older patient replied: Maybe I do not need a device. I feel healthy. In patients with systolic heart failure or atrial fibrillation, physical limitations such as shortness of breath and tiredness were pronounced and they considered that it was HCM, not the ICD, that was severely limiting life. HCM affected their professional opportunities and made them dependent on other people’s help. Patients who had secondary prevention ICD felt safer and expressed gratitude that they had a new chance at life. For them, it was obvious that they had a severe disease but this did not make them give up their joy of living. Even though some secondary prevention patients admitted slight cognitive impairment, they still felt eager to continue their old activities. The family members of SCD survivors were often unable to continue ordinary life, expressed worries, and even suffered sleep disturbances according to the patients. In this group, it was common for partners to be overly protective of the ICD patient, especially when there were young children in the family. In general, young patients were more worried and felt more limited than older patients, who remembered the worries they had about their health during adolescence and early adulthood. A woman said, My teenage years were difficult. I sometimes think: Why me? I am so nice, why couldn’t she get it instead? Still, I think everything is crap but I have become wiser and gained more perspective. Shortly after diagnosis, patients usually had a feeling of being different but later accepted and adapted to the lifestyle changes their condition demanded, and did not see themselves as stigmatized.
In general, patients perceived their underlying HCM, rather than the ICD, as constituting the limit on their leisure-time activities. Athletic activities had to be avoided, modified, or restricted, and many patients were unsure about their recommended level of activity. Mountain trekking, badminton, ice hockey, soccer, dancing, swimming, and hunting sometimes had to be restricted, but patients adapted to these restrictions or switched to other activities. Young patients felt more limited by recommendations to restrict their activities than older patients did. In some cases, driving was restricted by the authorities but this restriction was not always communicated to the patient. In other instances, a concerned spouse might advise the patient against driving. Such advice was at times ignored. When the patient had to limit driving, this impacted both his leisure and professional activities. Physical intimacy was not affected by the device and patients did not express fear of shocks during sex. However presence of severe heart failure or comorbidity limited sexual performance. In fact, one patient who had intercourse the next day after ICD implant said, We did it immediately when I came back from the hospital…just because I wanted to test it.
Inability to work was associated with symptoms of HCM or comorbidities, especially atrial fibrillation and progression to heart failure or cognitive impairment in cardiac arrest survivors. Younger patients were more worried about their work and sometimes struggled to reorient themselves professionally. These adaptations included less travelling, avoiding stressful situations, reducing their workload, and accepting being on sick leave now and then. Sometimes colleagues helped with certain tasks such as climbing a ladder or heavy lifting. With age, the concerns about working capacity diminished. Looking back, patients with an early onset of symptoms had military service exemptions but thought they were otherwise free to pursue their own career goals. A welder had to change his line of work specifically because of the ICD and other patients sometimes could not pursue work that involved driving or electromagnetic exposure. If this brought on economic constraints, patients adapted to their modified standard of living and did not report it as being problematic.
Relationship, support, and insurance
Following cardiac arrest, patients found their personal relationships were vastly changed. The survivor expressed gratitude, had a renewed appreciation for life, and modified goals and values. By contrast, the emotional response of family members was ambiguous…The event has made us better connected but also creates problems…these worries can be really tiresome…on the other hand, we have a shared experience that somehow bonds us. While patients were offered support, including referrals to psychology professionals from the health care system, family members were seldom involved. Generally, patients shared their feelings about their condition with the family, but occasionally the patients did not allow relatives to attend their clinical visits because they did not want them to worry, they expected it would result in overprotection and restrictions, or they wanted to make their own decisions independently but would introduce relatives later on. Occasionally (two patients) the disease was considered by the patient as a contributing factor in divorce. In younger patients, identification phenomena were observed, such as the man who became very anxious when he reached the age at which his father had died unexpectedly or when the child of an ICD patient said she wanted to get an ICD like her mother. In some cases when a patient and a family member went out for a walk, the family member would deliberately choose a less strenuous route to accommodate the patient. Having HCM caused emotional distress as well as physical symptoms in teenagers, who had an acute perception of themselves as being different from their peers.
No patient attended patient organization meetings, but a few had joined a Facebook group for ICD patients and one patient had found the American HCM patient association webpage. Older, highly symptomatic patients were less likely to use the internet as source of information than younger patients. Several patients expressed concern that they lacked information about their prognoses, which they considered the responsibility of their physicians to communicate to them. Not all of the information that patients found was considered beneficial. The fact that athletes drop dead…it is not advantageous for me. Furthermore, extensive talk and information about the disease sometimes conflicted with the patients’ self-image of being normal. Another young patient said, The less you know, the less ill you are. Swedish citizens are covered by a national insurance that pays for medical expenses, including the ICD. In addition, private insurance compensated certain patients for disability. Patients reported unexpected problems when renewing coverage or trying to sign up for a new policy.