(above) Dr. Keith Mankowitz at the Washington University Heart Care Institute in St. Louis giving a patient a checkupby Stu Durando
The disease that became the focus of Dr. Keith Mankowitz's cardiology practice was a medical enigma 11 years ago when he opened a center dedicated to its research and treatment.
The condition known as hypertrophic cardiomyopathy has been so misunderstood that it has gone by 75 names and been misdiagnosed in a multitude of ways.
It makes sporadic headlines only when seemingly healthy athletes such as Hank Gathers, Thomas Herrion and Jason Collier die unexpectedly from its effects. Yet the true impact comes more routinely.
"It's becoming more and more recognized that this is a significant condition," Mankowitz said. "It's much more common than we thought it was."
Mankowitz, a native of Johannesburg, South Africa, started the Washington University Hypertrophic Cardiomyopathy Center in 1997. With the aid of a grant, he is increasing exposure for the center at a time when research shows sudden deaths among athletes are five to 10 times more prevalent than previously thought.
Additionally, Mankowitz is working with Biomedical Systems, based in Maryland Heights, to start a pilot screening program this fall for high school athletes with the goal of taking the program statewide.
Last year, Adam Litteken, 16, a student at Francis Howell Central High School, died while playing hockey. It was determined that he suffered from undetected HCM.
At least three other school-aged kids in the St. Louis area have died without warning in the past two years. Damien Nash, who grew up in St. Louis and played for the Denver Broncos, died last year after playing in a charity basketball game.
A 25-year study by the Minneapolis Heart Institute Foundation showed that a young athlete dies unexpectedly every three days in the United States.
"It's only in the last few years that people realize it's really specialized, and you need people very familiar with the condition to take care of HCM," Mankowitz said. "Most cardiologists don't have enough training. Plus, it's an evolving field where there are advances all the time."
Sudden death among athletes, most frequently basketball and football players, has focused attention on HCM. But anyone can be susceptible.
HCM is a genetic disease that causes the heart to thicken abnormally and is estimated to occur in one in 500 people. Diagnosis can be difficult; patients often are told they suffer from other disorders.
Yet clinics like the one run by Mankowitz are uncommon. His is one of 11 in the United States and 14 worldwide that are recognized by the Hypertrophic Cardiomyopathy Association as centers of excellence in treating the condition.
He focuses on patients 16 and older, but children can be tested and diagnosed at younger ages, especially if a family member dies from HCM or is known to have the disease. The best test is an echocardiogram, which is an ultrasound of the heart. It can cost around $1,000.
The cause of Nash's death was not determined, as several other underlying heart conditions can cause sudden death. But HCM is the culprit in 36 percent of sudden deaths among athletes 35 and younger. A standard physical usually is not enough to uncover the condition.
Litteken's mother, Ellen, has known for years that she is living with a heart condition known as mitral valve prolapse, which does not impact her daily life. But it gave the family pediatrician reason to take a harder look at Litteken's two children. Still, no hints of HCM were found before Adam's death.
The rest of the Litteken family has since been tested for HCM, with all results coming back negative.
"We had echocardiograms done, and it's something in hindsight I would have liked for Adam to have done," Ellen Litteken said. "We would have found it. He had no symptoms whatsoever."
Symptoms that might hint at HCM include shortness of breath, lightheadedness, fainting, chest pain, palpitations and fatigue. But sometimes symptoms are not evident.
Dr. Joel Hardin, who lived in St. Louis for 30 years, has been involved in a program in New Jersey that provides free screenings, similar to the program Mankowitz hopes to begin. Lisa Salberg, founder of the HCM Association, said those screenings can be misleading because they are usually too limited to detect HCM. They generally involve an electrocardiogram, not the more revealing echocardiogram.
Hardin countered that any chance to find the disease is worthwhile.
"You start with the realization you're just scratching the surface," he said. "It gives you a clue. When an EKG is abnormal, there's an obligation to explain why it's abnormal."
Mankowitz hopes to start screenings at two high schools in the fall. Pat Barrett of Biomedical Systems said he is prepared to provide electrocardiogram machines to every high school in Missouri next year if school districts approve.
They won't diagnose the disease but can help reveal a potential problem.
Over the years, misdiagnosis of HCM has been common, Salberg said. Her organization surveyed more than 1,100 HCM sufferers and found a host of misleading diagnoses.
People were told they had asthma (19 percent), anxiety attacks (17.3 percent), depression (17.4 percent) and innocent heart murmurs (50.4 percent) along with other ailments, including acid reflux.
"What spurred my interest was parents who had lost children," said Salberg, who was diagnosed with HCM after her sister died of the disease. "I started noticing trends. I'd say, 'Did your child have any diagnosis?' They would say, 'Just a little asthma.' If I heard it once, I heard it 10 times. Some people were diagnosed with more than one thing."
Mankowitz hopes to educate more physicians so the condition can be detected before it's too late.
He led a conference in St. Louis in April with speakers from Washington and New Jersey. More recently he spoke at a conference with an audience of pediatricians. He makes himself available for speaking engagements without charging and has distributed literature to doctors.
"We try to make other physicians in the Midwest more aware and direct them to send us patients," he said. "Reading an echocardiogram is not straightforward and the diagnosis isn't. You have to put a puzzle together and know how to recognize some features. It really is an art."
People who are found to have HCM are told not to take part in many sports because burst exertion can trigger sudden death. Undetected or misdiagnosed, the first sign of a problem can be death.
Shelley Rosenmiller of St. Charles was diagnosed with HCM and had a surgical procedure in November. She realizes she may have been fortunate.
"I tried to do my aerobics and all of that, and I shudder to think that I could have pushed myself too far," she said. "One thing I could never do was distance run. I would get winded. I've often wondered if it could have been that I had this existing condition."
Meanwhile, Ellen Litteken said her family plans to set up a foundation in Adam's name. She is unsure where the money would be donated but is interested in talking to Mankowitz about increasing awareness.
She said, "If we can help one other kid that would be awesome."
The condition known as hypertrophic cardiomyopathy has been so misunderstood that it has gone by 75 names and been misdiagnosed in a multitude of ways.
It makes sporadic headlines only when seemingly healthy athletes such as Hank Gathers, Thomas Herrion and Jason Collier die unexpectedly from its effects. Yet the true impact comes more routinely.
"It's becoming more and more recognized that this is a significant condition," Mankowitz said. "It's much more common than we thought it was."
Mankowitz, a native of Johannesburg, South Africa, started the Washington University Hypertrophic Cardiomyopathy Center in 1997. With the aid of a grant, he is increasing exposure for the center at a time when research shows sudden deaths among athletes are five to 10 times more prevalent than previously thought.
Additionally, Mankowitz is working with Biomedical Systems, based in Maryland Heights, to start a pilot screening program this fall for high school athletes with the goal of taking the program statewide.
Last year, Adam Litteken, 16, a student at Francis Howell Central High School, died while playing hockey. It was determined that he suffered from undetected HCM.
At least three other school-aged kids in the St. Louis area have died without warning in the past two years. Damien Nash, who grew up in St. Louis and played for the Denver Broncos, died last year after playing in a charity basketball game.
A 25-year study by the Minneapolis Heart Institute Foundation showed that a young athlete dies unexpectedly every three days in the United States.
"It's only in the last few years that people realize it's really specialized, and you need people very familiar with the condition to take care of HCM," Mankowitz said. "Most cardiologists don't have enough training. Plus, it's an evolving field where there are advances all the time."
Sudden death among athletes, most frequently basketball and football players, has focused attention on HCM. But anyone can be susceptible.
HCM is a genetic disease that causes the heart to thicken abnormally and is estimated to occur in one in 500 people. Diagnosis can be difficult; patients often are told they suffer from other disorders.
Yet clinics like the one run by Mankowitz are uncommon. His is one of 11 in the United States and 14 worldwide that are recognized by the Hypertrophic Cardiomyopathy Association as centers of excellence in treating the condition.
He focuses on patients 16 and older, but children can be tested and diagnosed at younger ages, especially if a family member dies from HCM or is known to have the disease. The best test is an echocardiogram, which is an ultrasound of the heart. It can cost around $1,000.
The cause of Nash's death was not determined, as several other underlying heart conditions can cause sudden death. But HCM is the culprit in 36 percent of sudden deaths among athletes 35 and younger. A standard physical usually is not enough to uncover the condition.
Litteken's mother, Ellen, has known for years that she is living with a heart condition known as mitral valve prolapse, which does not impact her daily life. But it gave the family pediatrician reason to take a harder look at Litteken's two children. Still, no hints of HCM were found before Adam's death.
The rest of the Litteken family has since been tested for HCM, with all results coming back negative.
"We had echocardiograms done, and it's something in hindsight I would have liked for Adam to have done," Ellen Litteken said. "We would have found it. He had no symptoms whatsoever."
Symptoms that might hint at HCM include shortness of breath, lightheadedness, fainting, chest pain, palpitations and fatigue. But sometimes symptoms are not evident.
Dr. Joel Hardin, who lived in St. Louis for 30 years, has been involved in a program in New Jersey that provides free screenings, similar to the program Mankowitz hopes to begin. Lisa Salberg, founder of the HCM Association, said those screenings can be misleading because they are usually too limited to detect HCM. They generally involve an electrocardiogram, not the more revealing echocardiogram.
Hardin countered that any chance to find the disease is worthwhile.
"You start with the realization you're just scratching the surface," he said. "It gives you a clue. When an EKG is abnormal, there's an obligation to explain why it's abnormal."
Mankowitz hopes to start screenings at two high schools in the fall. Pat Barrett of Biomedical Systems said he is prepared to provide electrocardiogram machines to every high school in Missouri next year if school districts approve.
They won't diagnose the disease but can help reveal a potential problem.
Over the years, misdiagnosis of HCM has been common, Salberg said. Her organization surveyed more than 1,100 HCM sufferers and found a host of misleading diagnoses.
People were told they had asthma (19 percent), anxiety attacks (17.3 percent), depression (17.4 percent) and innocent heart murmurs (50.4 percent) along with other ailments, including acid reflux.
"What spurred my interest was parents who had lost children," said Salberg, who was diagnosed with HCM after her sister died of the disease. "I started noticing trends. I'd say, 'Did your child have any diagnosis?' They would say, 'Just a little asthma.' If I heard it once, I heard it 10 times. Some people were diagnosed with more than one thing."
Mankowitz hopes to educate more physicians so the condition can be detected before it's too late.
He led a conference in St. Louis in April with speakers from Washington and New Jersey. More recently he spoke at a conference with an audience of pediatricians. He makes himself available for speaking engagements without charging and has distributed literature to doctors.
"We try to make other physicians in the Midwest more aware and direct them to send us patients," he said. "Reading an echocardiogram is not straightforward and the diagnosis isn't. You have to put a puzzle together and know how to recognize some features. It really is an art."
People who are found to have HCM are told not to take part in many sports because burst exertion can trigger sudden death. Undetected or misdiagnosed, the first sign of a problem can be death.
Shelley Rosenmiller of St. Charles was diagnosed with HCM and had a surgical procedure in November. She realizes she may have been fortunate.
"I tried to do my aerobics and all of that, and I shudder to think that I could have pushed myself too far," she said. "One thing I could never do was distance run. I would get winded. I've often wondered if it could have been that I had this existing condition."
Meanwhile, Ellen Litteken said her family plans to set up a foundation in Adam's name. She is unsure where the money would be donated but is interested in talking to Mankowitz about increasing awareness.
She said, "If we can help one other kid that would be awesome."
5 comments:
I read this article with interest my son Kenyon died of HMC. Kenyon was 21 yrs. old, 2 mos. from his 22nd birthday. Kenyon was home from college for the summer he would have been a senior the fall semester. Kenyon was a chemistry major and was on the varsity basketball team. Kenyon had worked all day and went to his high school gym to play in a pickup basketball game to stay in shape. At about 6:00 p.m. I received a phone call telling me Kenyon was in the floor unconscious. At the hospital they tried without success to revive him. After an autopsy we would find out he had HMC. I still find it unreal after 16 yrs. that my tall, healthy looking son could die from something I'd never heard of. I have tried unsuccessfully to have changes made in the sports physicals in my area(such as limited echos)to be preformed by one of the best heart hospitals in Louisville Ky. It's like banging your head against a wall. It's always thank you Ms. Lacy we'll look into this and see what we can do. These hospitals in the metro areas should give back to their communities who support them and one way would be to help with heart testing for athletes. After Kenyon passed I called all around the country to find out about HMC so many colleges do not include the echo in their physicals. Such as I found out University of Louisville does use the echo because of the death of an athlete who had died at University of Ky but the University of Ky does not use the echo in their physicals. I talked to Dr.Barry Marron and another doctor in California who I can't recall his name at the moment these two doctors study HMC and also keep stats on deaths due to this condition. I truly believe these stats are not at all true stats! I have had some names they didn't have come to find out they get their info from people who read newspapers for them and report the deaths. If these athelets are not in a scheduled game the deaths most likely will not make the newspapers. So many of these deaths are not in a scheduled game just like my son.
The midwest, we live in Indiana where sports are so popular and so enjoyed, these deaths need to be noted so parents and families would understand this condition kills your child if unknown.
The fatigue is felt at the last and my son did complain of being fatigued in the last few weeks but he had a virus a few months earlier and he thought he hadn't gotten his strength back that was not the case. Well so much for our story, I just hope and pray someone will read this and try to help these athelets have better sports physicals. Thanks
Susan Lacy mother of Kenyon Lacy
slacy@yahoo.com
I am a patient of Dr Mankowitz and living with HCM obstructed, My son is 12 and we have had him tested only finding the condition in him last year, His middle school in St Peters has brought an AED in to his school for safety and we have done two interviews with local news stations to help spread the word about this condition, I hope that more can be done to educate the Physical Education department in our schools, and the coaches that are caring for our children at times, pay attention, your body is trying to tell you something. I had rare symptoms until just years before they found mine.
Symtoms can include, light headedness, dizzy spells, passing out, shortness of breath, chest tightness, palpatations. If you have anything like these, first stop what you are doing, see a dr and follow through with investigating anything you are told, If you dont feel write about a diagnoses, dont settle. I highly recommend Dr Mankowitz if you need to seek a professional in the area of Hypertrophic Cardiomyopathy.
My son passed away unexpectedly last month from HCM. He was a beautiful, healthy, strapping boy who died while on vacation in California last month. He had texted his girlfriend that he had been so tired, but just did not know why. He would have turned 25 on 10/10/09. We are still in a state of disbelief, but are very concerned about our other son who is 27. How can I make sure that this doesn't happen again? Thanks.
According to the article, "The best test is an echocardiogram, which is an ultrasound of the heart. It can cost around $1,000."
I would hope that the test is covered under medical insurance and that you find a reputable hospital in the area to perform the test.
Best wishes to everyone who has been touched by this misunderstood disease.
I am a 35 year old female overweight type 2 diabetic with hypothyroid, just diagnosed with this HCM. I had been telling my Doctors for 5 years that I have been having signs of what felt like someone squeezing my heart so tight and then just letting go allowing blood to rush through my chest area and throwing up stomach acid no matter if I ate or drank. it wasnt until June of 2015 when I finally got to see a heart doc, was given an EKG and was told my readings was that of a 70 year old man, and rushed to the hospital to get a heart cat done, with an echo, stress test and all that fun stuff but not a MRI. nothing was found but a murmur on the left side of my heart, and high blood pressure. months later still complaining, so my heart doc sets me up with a right heart cat. nothing. I then get sent to a lung doc, and a GI. lung doc tested me for everything he could. nothing. GI tests me for stomach issues. I have a hernia in my food pipe. (sorry its early and I cant think proper to spell and grammar)and acid ref nothing else, so she sets me up with a shirk thinking I'm just stressed. no not the problem. at one of my appointments with my lung doc and GI, I tell them both as no one can figure out whats wrong with me, that ive had every test and blood work done that you can think of, but I have not had a MRI on my brain nor on my heart. the next thing I know I'm getting my heart MRI. wow if only someone would of thought of this 5 years ago!!! 2 hours after the MRI, my heart doc calls says there is some issues but I cant get you in here until 3 days from now. can you come in? I go in an am told I have a rare gen heart disease, HCM. but I'm not heathly and I don't exercise that much. so how is this possible?
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