“I don’t remember a time in my family where there wasn’t the weight of heart disease,” says Salberg, 51. Her great-grandmother succumbed to dropsy (an old term for edema, which is linked to heart failure). Her grandfather, who had a heart murmur, died from sudden cardiac arrest at the age of 43. A massive stroke took her paternal aunt at age 52. At the age of 48, her uncle passed away from sudden cardiac arrest. Her father, who was put on Valium after having been misdiagnosed with anxiety, found out he had HCM at age 59. He had surgery to fix his heart, but he was also weakened by kidney and liver failure, and died at age 73. In 1995, her eldest sister, Lori Anne Flanigan-Munson, passed away at age 36 from complications related to her HCM and mismanagement of her illness. Flanigan-Munson left behind two children ages 10 and 13, who have since been raised by Salberg and her family. “My sister’s death left me shattered,” says Salberg. “It shouldn’t have happened. I was a new mother and also had responsibility for her two children. Frankly, I was scared that I too would die too soon and leave them behind with no support.”
Taking Her Health Into Her Own Hands
Salberg wanted to do more to take control of her condition, and hopefully help others with HCM. So, she decided to start a group that would provide information, support, and advocacy. With the internet just taking off in the early 1990s, Salberg realized that establishing an online presence would enable the organization to reach hundreds of thousands of people. Shortly following her sister’s death, Salberg founded the Hypertrophic Cardiomyopathy Association (HCMA), which is dedicated to providing support, education, and care to people with HCM. Today, the HCMA has an active membership of more than 5,700 families and reaches an estimated 200,000 people per year through its website. The organization has helped thousands of families improve their lives and learn to live well with HCM, which is the most common of all genetic heart conditions, affecting over 1 in 500 people in the general population and up to 725,000 people in the United States, according to the HCMA. When I met Salberg at the American Heart Association Scientific Sessions in the fall of 2019, she introduced me to the cause of all her health troubles — her diseased heart (preserved through a process called plastination) was on display in a glass box. Her years of multiple operations and trying different treatments were over. Since receiving a heart transplant in 2017, Salberg says she has been cured. Getting to this point, however, was a difficult journey, marked by a series of seemingly endless struggles and heartache — both physical and emotional.
The First Signs of Heart Trouble
At age 12, Salberg discovered that she would not escape her family’s history of heart disease. She would occasionally experience chest pain, shortness of breath, and dizziness, but they didn’t happen all that often and they were often minor episodes. “These symptoms can be rare and for some minor — they are often ignored or minimized by both patients and healthcare providers,” says Salberg. In seventh grade, a doctor at Salberg’s school was giving all the students a checkup, which included listening to their hearts. Salberg recalls that as he listened to hers, a look of horror passed over his face. Her physical revealed that she had a heart murmur. After a visit to a cardiologist, Salberg was diagnosed with HCM. “I was concerned, but I was also accused of ‘using the diagnosis’ for attention by teenage ‘friends,’” says Salberg. “I hid my symptoms rather than discussing them, fearing the ignorance of others. I coped by withdrawing from many typical high school activities, but I was happy in the life that I had created for myself. I had a close circle of friends who understood me and to this day they remain my friends.” By this time, Salberg’s sister Lori had already been diagnosed with HCM. (Her brother was screened later, at age 23, and found to be HCM-negative, and her middle sister was screened at age 20 and found to have the familial genetic abnormality but not be troubled by any symptoms.) Salberg’s parents consulted her doctor and discussed putting her on medication, but because her symptoms weren’t too bad they decided against it. Besides, her mother didn’t want her to become a so-called “cardiac cripple” — a person who receives various heart medications and is advised to avoid physical activity. By age 18, Salberg was experiencing increased migraine attacks, light-headedness, chest pain, palpitations, near syncope (fainting), and shortness of breath. Doctors put her on a common beta-blocker, Inderal (propranolol), which is a first-line class of medication for the treatment of HCM as symptoms worsen. But drugs like this didn’t work forever. For Salberg, this was the beginning of an ongoing search for the best therapies to ease her symptoms. “I was 21 so I thought it couldn’t be anything serious,” says Salberg. Then she woke up one morning with her left side feeling completely numb. “I could not move my hand and I had terrible balance and the worst headache of my life,” she says. Her doctor directed her to get to an MRI center where they identified that she had had a stroke. From blood tests, doctors discovered her stroke was related to subacute bacterial endocarditis, an infection of the lining of the heart. Salberg was treated with IV (intravenous) antibiotics for eight weeks. She gradually recuperated, but the stroke left her with partial blindness in her left eye and weakness in her left side. “It was frightening to have a stroke at that age,” Salberg says. “I was 21 with my life in front of me and now I was a stroke survivor — that was a great deal to process. But I returned to work and life and tried to put it behind me.” Stretches of her life would go on without incident when she had her medication just right to manage her symptoms. But trouble always returned. Salberg was in and out of heart failure 15 different times, with episodes of fluid retention and rising brain natriuretic peptide (BNP) levels. (When someone experiences heart failure, BNP protein levels increase.) “During these episodes I was very tired and had a hard time getting out to work, but I did it,” she says. “There were three events of heart failure that left me unable to work for weeks at a time. The rest I just managed with added medications, including diuretics.”
A Tragedy Leads to a New Life Course
Through all the challenges, Salberg was able to build a career as human resource (HR) manager. That path, however, would change after 1995. In the spring of that year, her sister began to experience congestive heart failure, which was not recognized or treated. In June, she suffered cardiac arrest and died at age 36. “My sister died from complications from mismanaged HCM,” says Salberg. “She was put on medication that was contraindicated at the doses she was using. She was put on double diuretics when she was experiencing heart failure, but no one was monitoring things like her potassium level, which dropped and caused her to have cardiac arrest.” Pregnant with her daughter at the time of her sister’s death, Salberg worried about whether her baby would have HCM, and if she too would die and not be there for her child. For two months after her daughter’s birth, Salberg battled with pneumonia and pleurisy, a condition where the lungs become inflamed. Doctors again tied this to another episode of heart failure. As she feared, her daughter did have the familial genetic abnormality causing HCM. Her niece and nephew wound up having the condition too, as well as several cousins. With her sister’s death and ongoing health problems of her own, Salberg was determined to get better answers about her family’s condition and help others find treatments that work. These events led to her forming the HCMA.
After Worsening Heart Troubles, a ‘Cure’ Arrives
Salberg knew that she was at risk of sudden death from cardiac arrest and that she could die suddenly like her sister. That’s why she received her first ICD (implantable cardiac defibrillator) in 1997 to monitor her heartbeat and deliver an electrical shock as needed to restore a normal rhythm. “It’s like having an emergency room in your chest 24/7,” she says. She ended up having five such units implanted in her body, replacing previous units because of either faulty operation or the availability of new, improved electronics. Although Salberg was learning more and more about HCM, the disease was still taking its toll on her family. In June 2008, her father, Larry Flanigan, passed away after a lifelong battle with misdiagnosis and finally proper identification of his HCM. Around 2010, Salberg began seeing signs that she was declining, despite doing all she could to treat her disease, including undergoing multiple operations. Her ejection fraction (EF), a vital measure of how well the heart is pumping, had dropped. “I knew my heart muscle was getting compromised, that it was getting stiffer,” she says. “I knew that [the average time span] from the onset of a reduced ejection fraction to transplant or death was seven years. Admittedly, I was in a bit of denial because I was in my forties and I didn’t want to deal with that.” Her heart’s deterioration began to take its toll. Salberg did not want to get up in the morning. She struggled to get out of the shower. She struggled to get to work, and when she stood up at her desk, she would be overcome with dizziness. “The palpitations were endless,” she says. “I no longer knew what a normal heartbeat felt like, and I was slowly getting forgetful and foggy in my thinking.” She cut back on her walking, and as a result she lost muscle tone. Her failing heart affected her brain as well — she often strained to put a sentence together. Usually a “workaholic,” Salberg began to cut back her hours. Her normal schedule of 8:30 a.m. to 7 p.m. shrank to 10 a.m. to 4 p.m. “I would get home, take a nap, wake up for dinner, and go back to sleep,” she says. “That was my life for a long period of time.” In September of 2016, Salberg became acutely ill and was admitted to the hospital. With her heart not producing enough oxygen for her body, doctors put her on milrinone, a medication that causes blood to flow more easily through the veins and arteries. The only long-term solution to keep Salberg alive would be a heart transplant, so she was put on the list to receive one. On Groundhog Day of 2017, at 12:30 a.m., she got the call telling her that a heart had been found for her. Before Salberg went to the operating room, she told surgeons to save her old heart. She knew she would want to have it as an example of what an HCM heart looks like to show others. Four days after her successful transplant, her surgeon presented her with her frozen former heart (which she later plastinated to use as a teaching tool). Salberg was surprised at how heavy and dense it felt. “The first thing I said when the doctor handed it to me was, ‘So you are [what] caused all this trouble,’ followed by, ‘Thank you for getting me this far,’” says Salberg. After 12 days in the hospital, she went home on Valentine’s Day. “Later I would find out my new heart came from a 32-year-old woman named Brandy — my angel and my hero.”
Back to Living a Normal Life — Almost
With a new heart, Salberg was cured of HCM. However, her immune system is weakened because of medication she takes to prevent her body from rejecting her new heart, so Salberg has to be extra careful, especially with viruses like SARS-CoV-2 — the one that causes COVID-19 — going around. “I am also at a higher risk of cancer than others because my body can’t fight those little bad cells as well when they start popping up,” she says. “I have to be more aggressive with my overall health management, which is not fun, but the fact is, if I didn’t have my transplant, I’d be dead now.” To protect her new heart, she takes tacrolimus (an immunosuppressive drug), Lipitor (a cholesterol medication), aspirin, and vitamins. Today, Salberg is back to her “workaholic” schedule, leaving the house early in the morning and often not getting home until 10 at night, dedicating most of her time to the HCMA. “It’s now been just over three years since my operation and I’m doing great,” she says, “and I want to help others with HCM do just as well as I am.”
