Archive for the ‘Tetralogy of Fallot’ Category

24 hours at Johns Hopkins

November 28, 2010

“Because your whole world can change in 24 hours.” – The Paper (1994)

Tuesday, November 28, 1944: Sometime during the evening of the 28th, Dr. Alfred Blalock places a telephone call to the Surgical Laboratory at Johns Hopkins Hospital. His Surgical Assistant, Vivien Thomas, has recently developed a surgical correction for the heart defect known as Tetralogy of Fallot, also known as Blue Baby Syndrome. The two men have planned for Thomas to teach Blalock the steps needed to successfully complete the surgery during an operation on a dog. Blalock has done the operation only once and many more teaching sessions are needed.

Blalock is calling with grim news: Earlier he had asked Thomas about the possibility of operating on 19 month old infant Eileen Saxon. Weighing only nine pounds and often cyanotic, the dusky blue color that gives this malady its name, she is deteriorating rapidly. At the moment she is so cyanotic that she is purple and is struggling for every breath. Dr. Blalock tells Thomas to meet with Elizabeth Sherwood, the operating room supervisor, first thing in the morning. Thomas has invented several surgical tools specifically for this operation and he is to make sure that they are available.

Thomas is stunned and reminds Blalock that he doesn’t know the operation very well. “But if you don’t get ahead of yourself, break it down into smaller and smaller steps as you work, it can be done.” It is one of the familiar sayings Thomas uses when he is teaching proper surgical procedure and for a moment, Blalock feels as if he is the assistant.

After Blalock hangs up, Dr. Helen Taussig orders him home. Blalock protests, but she reminds him that he plans to operate in the morning – an operation that could very well be emergency surgery. The hospital has his telephone number should he be needed during the night. At roughly the same time, Thomas and Blalock leave for their respective homes. Segregation is still prevalent at the time and Thomas leaves by a back entrance; neither man knows the other one has left.

Dr. Taussig spends the night on the ward; Eileen’s parents are also there. Although they don’t know it, this is an ominous sign: in the 1940’s, visiting hours rules were strictly enforced unless a patient was seriously ill.

Wednesday, November 29, 1944: Too nervous to drive, Blalock asks his wife Mary to take him to the hospital. She lets him out of the car in front of the towering Johns Hopkins dome. Dr. Blalock enters the building, walks through the rotunda (rubbing the toe of the Statue of Christ for luck, an old Hopkins tradition) and turns left. From here he exits the building through a side door, walks approximately 50 yards, and into the Harriet Lane Home for Invalid Children. Vivien Thomas enters the Hopkins complex from a side entrance and goes immediately to Elizabeth Sherwood’s office. Miss Sherwood knows nothing about Dr. Blalock’s plan to operate but immediately shows Thomas the selection of items that will be available to Dr. Blalock. Thomas adds custom-made clamps and needles to the collection. These needles are no more than 1/2 inch long. Thomas insists that the clamps and needles not become part of the general operating room supplies – they have been custom made for this operation only.

Blalock and Taussig examine the child and confer. Eileen has not improved during the night, and Taussig concedes that there is nothing else that she can do. She leaves the meeting as Thomas arrives, perhaps to return to Eileen’s bedside or for a quick trip to the Cafeteria. Blalock and Thomas discuss the upcoming operation. They go over some of the more critical steps, and also discuss “routine” points such as where the incision should be made. Thomas informs him that Miss Sherwood has promised that the large operative theatre will be available but needs to know when the operation will begin. Blalock decides that the operation will take place after the morning rounds, unless events dictate otherwise. He leaves to confer with Eileen’s parents and to conduct Rounds. Thomas did not normally participate in Rounds so he would have gone to the Surgical Lab, although he may have gone to his office. He calls Miss Sherwood and informs her of Blalock’s decision.

The operative team convenes in the Scrub Room annex connected to Room 706. Although first chosen at random, the majority of Hopkins’ early heart surgeries will take place here and the room will come to be known as “The Heart Room.” Dr. William Longmire and Dr. Denton Cooley will assist. An unknown person sets up a movie camera pointed at the operating room table; this film still exists in the Johns Hopkins Hospital Archives.

Blalock continues to discuss the upcoming operation with Thomas as he prepares for surgery. Thomas is not scrubbed in and has no intention to – he is not allowed on the Operating Room floor. He will be seated in the raised seats of the theatre, however. Helen Taussig will be in the Operating Room, even though she is not a surgeon. She’ll spend most of her time at the head of the table, monitoring the patient.

A few minutes before Eileen arrives, Blalock quietly asks his scrub nurse to find Thomas and help him get scrubbed in. As expected, Thomas is seated in the bleachers above the OR. Blalock also orders a milk crate and has it placed behind him. Thomas stands on the crate, peering over Blalock’s shoulder at the operative field.

The operation begins with a curving incision near the 4th rib on the child’s left side. With Thomas guiding him, Blalock gently works past the lung and cuts a path to the heart. The heart is small, dark, and obviously struggling. William Longmire later said “I remember watching him open the patient and just thinking it was impossible.”

Blalock works patiently, finding the Left Subclavian Artery and the left branch of the Pulmonary Artery. He places a clamp on the Subclavian to cut off blood flow – using one of the clamps designed by Vivien Thomas for this procedure – and cuts it. He then places two similar clamps on the left branch of the Pulmonary Artery. Making a small opening in the Pulmonary Artery, Blalock uses the tiny needles Thomas has prepared to sew the Subclavian Artery into the Pulmonary Artery. After double checking his work, Blalock removes the clamps. He is unable to feel blood flowing through the new connection.

Legend has it that Helen Taussig said “Al, the child’s lips are a lovely pink color!” The Johns Hopkins online exhibit about the operation states that the anesthesiologist said “The boy’s a lovely color now!” at a later date, during the third operation. Blalock’s operative notes comment that the circulation in the nail beds of Eileen’s left hand “appeared to be fairly good.”

The difficult segments are complete but the operation is far from over. Sulfanilamide (an antibiotic) is introduced into the incision and Blalock begins to close. He sews the soft tissue closed with silk sutures and is finally done. The operation has taken about ninety minutes. (CLICK HERE to perform the Blalock-Taussig Procedure yourself. Read Blalock’s operative notes here:  PAGE 1 PAGE 2)

Eileen is moved to the Recovery Room, where Dr. Henry Bahnson is responsible for her care. As one might expect, Blalock and Taussig look in on her often.  Bahnson’s opinion is that the little girl is still very blue but improves over time. Eileen’s mother comments “When I saw Eileen for the first time, it was like a miracle… I was beside myself with happiness.” Very little is known of Thomas’ movements after the operation. He is seen in Recovery and also in his Lab.

As the sun sets on the city of Baltimore, Eileen remains in critical condition but she is stable. The operation is a success, but in a few months it will fail and she will need another Blalock-Taussig Procedure, this time on her right side. She will die just before her 3rd  birthday.  The doctors determine that the surgery is more suited to an older child whose blood vessels have had a chance to grow. In early 1945 Blalock and Taussig co-authored a medical journal article about the first three procedures. Hundreds of patients would flock to Johns Hopkins Hospital to receive the life-saving surgery, even though the odds were long: an article in the February 17, 1947 issue of The American Weekly noted that 14 of the first 70 patients had died.

But parents noted that 56 of them had lived and were growing up, something that had never happened before. The era of Congenital Cardiac Surgery has begun.

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Celebrate Red and Blue Day

November 19, 2010

“What sort of day was it? A day like all days, filled with those events that alter and illuminate our times…” – You Are There, 1953

November 29, 1944: Dr. Alfred Blalock took one final look into the incision. It looked right… he had been operating for years, surgery shouldn’t make him nervous anymore. But this operation did. He had completed this same surgery on a dog only once, and no one had ever tried it on a human before. Let this work…

“Watch for bleeding,” his assistant reminded him as he started to remove the clamp. Blalock nodded, ready to drop the clamp back into place if the new connection leaked. But not too hard, too much pressure and you crush the Pulmonary Artery; do that and you kill the patient.

His partner, Dr. Helen Taussig, stood near the head of the table. Heart surgery had been her idea, she had just as much riding on this operation as he did. Probably more – she had assured both him and the child’s parents that the theory behind this operation was sound. The little girl’s heart defect caused Cyanosis – she was literally suffocating from lack of oxygen. Taussig’s theory was to reroute a blood vessel to the lung and increase the amount of oxygenated blood available. Blalock’s assistant, Vivien Thomas, had designed the operation and tested it. All three of them had their reputations on the line.

And the irony of it all was if things went bad, he’d probably be the one to suffer least. Blalock was the Chief of Surgery, after all. Taussig was an almost deaf female doctor (who ever heard of such a thing?) and Thomas was a Black man who official job description wasn’t supposed to bring him anywhere near a scalpel, much less doing experimental surgery. If things went wrong, they would be the ones hung out to dry.

So let’s not allow things to go wrong, Blalock thought as he inspected his work again. “I’m removing the clamps,” he finally said.

Reaching into the open wound, he gently touched the new connection. “I can’t feel any flow,” Blalock said, disappointed. After a long pause, Taussig spoke.

“Al, the baby’s lips are a glorious pink color.” Stunned, the surgeon watched as the child’s blue lips slowly turned pink.

Before that day in 1944 heart defects were almost always fatal, usually during the first year of life. Occasionally a child was lucky enough to survive to late childhood or the early teens, but that was only under the best of circumstances. And that “lucky” child had no strength, no energy, and very little Quality of Life. Even after that first surgery (the Blalock-Taussig Shunt)  there was still only one operation, designed to relieve the effects of one heart defect. The odds weren’t good, but CHDers now had a chance. And sometimes one chance is all you need.

CHD Survivors, our families, and our friends celebrate November 29 as Red and Blue Day. Participating in Red and Blue Day is simple – just dress in red and blue clothing. You don’t have to donate any money (though if you choose to, your favorite CHD Support Group would be an excellent choice!) and you don’t have to volunteer to do anything. Simple as can be. If anyone compliments you on your good taste or your color scheme, just be prepared to explain why you chose those colors.

A Heart Defect is an Invisible Disability… many of us don’t even look like we have a health problem. Some of us are Cyanotic, but you have to look really close (and know what you are looking for) to see it. But November 29 is OUR DAY, so wear Red and Blue… and let’s stand out!

Second Hand Heroes

September 24, 2010

We’re not your classic heroes. We’re the other guys. – Mystery Men (1999)

The other day I discovered a Facebook based Heart Defect group engaged in a pointless mental exercise: They were listing celebrities that are connected to the CHD Community in some way that they feel should become a “Congenital Heart Defect representative”. Once that happened, “awareness” would be raised, money for research would come pouring in, and we’d all be saved.

Oh good grief.

I’ve seen this happen before; the group concentrates its efforts on recruiting that one big megastar and ignores the people who are actually doing the job. Because true advocacy for an issue is difficult work. You don’t just stage a few events, look good for the camera, and recite the proper sound bites. It takes time, and the job is detail oriented grunt work. The i has to be dotted and the t crossed correctly and that is your job. Long hours, very little sleep, and the “pay” is a pittance, if anything. You do this because you believe.

And by the way – if all you are doing is “raising awareness”, then you are not helping. That young mother doesn’t know Heart Defects is the most common birth defect… and she doesn’t care. What she does know is that her child is very sick and the doctors are throwing words at her she doesn’t understand.  She wants someone who can explain this to her in simple English and give her some hint of the future. And even if the future is dire, she wants to talk to someone who will tell her the truth, and gently let her know that sometimes its OK to say goodbye.

I’m honored to know people like this and count them as friends; I’ve also shared some of their stories on Funky Heart! over the past two years. You probably haven’t heard of these people anywhere else. They don’t mind; because they aren’t seeking personal glory.

The most “famous”  Heart Warrior I know might be Heather, a CHDer who saw an ad in a magazine for a TV show seeking women with heart disease. From the wording of the ad Heather realized they were looking for people with Acquired Heart Disease and ignoring CHDers. Heather got in contact with the show producers and gave them a piece of her mind – and as a result, appeared on the TV show How to Look Good Naked!

Then there is George, a retired doctor with Tetralogy of Fallot who spoke at Lobby Day 2010. I always thought George was kind of reserved, but I found out he just waits until the proper moment. When he got through speaking we were ready to tear the walls down! “One day, Congenital Heart Defect survivors are going to live to be eighty, ninety, and even one hundred years old,” he said. George is also doing his part to keep Heart Warriors going – During Lobby Day he took a young adult/older teenager under his wing. Near the end of the day George told him “Remember what we’ve done here. One day this will be your job.”

Karen T. Chavez and Kim Rooks serve as Co-Directors of the Broken Hearts of the Big Bend. Heart Defect Awareness isn’t always the answer, there are times when what is needed is Education and Support. If you have a Cardiac Kid in the Tallahassee (Florida) area, Broken Hearts members can walk you through what may come next – and be right there with you, lending a helping hand when you need it. Why? Because we’ve all been there.

Colorado’s Amanda Adams has a Cardiac Kid; when he was born she could find very little information about his defect and almost no support. Frustrated, she decided that no mother should ever be in such a situation… and began the support group Hypoplastic Right Hearts.

Amy Basken is into everything – right now, she’s on the Advocacy Staff of both the Adult Congenital Heart Association and the Children’s Heart Foundation; serving as the National Advocacy Coordinator for Mended Little Hearts; and serves as Chairperson of the National Congenital Heart Coalition. She does all this “because I am a mom of a kid with a heart defect.”

A former teacher, Amy Verstappen‘s health deteriorated after the birth of her daughter. Unfortunately she had some doctors we were not familiar with Congenital Heart Defects in Adults and they almost sent Amy for a heart transplant! Thankfully Amy found Cardiologist Dr. Carol Warnes. Dr. Warnes saw the X-ray, knew what she was looking at, and got Amy what she really needed – a valve replacement. After the surgery this same Cardiologist challenged Amy to do something about the adults with CHD who were not getting good care. Her journey has taken Amy out of the classroom to serve as President of the Adult Congenital Heart Association.

And not to be forgotten is the late Jim Wong, a PhD who lived and worked on the West Coast. Jim’s training was in Chemistry, but when it came to learning about heart defects he just wanted to know as much as possible… and he never hesitated to share what he had learned.  He often traveled to Washington to serve on a patient advisory committee for the National Institutes of Health, and was at every ACHA event I attended until he was too ill to attend.

Fifty-four years old, Jim was one of the older CHD survivors, and grew up in the days when CHDers were X-rayed, scanned, and fluoroscoped as much as possible. A lot of older survivors pass away after a bout with cancer, not because of their heart. There is currently no medical evidence to prove this, but one theory contends that absorbing all that radiation while they were young leaves older Heart Warriors more susceptible to cancer.

Jim Wong died of cancer earlier this year, perhaps as a result of all those x-rays. I like to think he died the way he lived – helping others learn about defective hearts.

George

May 16, 2010

My friend George spoke at the recent Lobby Day event in Washington DC. I wish all of you had been there to hear him.

Like many of us there, George has a Congenital Heart Defect (CHD). His defect is Tetralogy of Fallot (ToF) and he is a retired Pathologist from out West. That’s right – a retired Pathologist. I’m not going to insult him by listing his true age, but George worked a full career with a major heart defect.

He’s quiet and unassuming, probably the result of his years in the medical field. Pathology is a very scientific branch of Medicine; attention to detail is a must and you do not jump to conclusions. So when George stood up to speak, I wasn’t expecting what came next.

Long story short: George tore the house down! He stated that he had recently celebrated a birthday and then he said;

“I firmly believe that one day those of us with heart defects will routinely live to be eighty, ninety, and even one hundred years old.”

That did it for me right there. I’m 43 years old, but I’m 42 years, 7 months past my “expected” life expectancy. And here’s a respected member of the CHD community, a retired scientist, saying that he thinks that one day we’ll live a good long time? And might even hit the century mark? And all we have to do to start down this path is obtain funding for the Congenital Heart Futures Act? Right then, I was ready to walk down to the Capitol (Three blocks away), climb the dome, and rip that statue right off the roof!

George also has a very dry wit; he can tell a joke with such a deadpan expression that you don’t even realize he’s joking. But his humor can bite, as he proved when he recalled an exchange from his younger days.

“`When I attended medical school they taught me that people like you didn’t make it,’ a doctor told me. I didn’t know what to make of that so I didn’t say anything.”

People like you? Wait a minute… that includes me!

“A few moments later the doctor asked me `Have you thought about which medical school you would like to attend?’ I answered, `Not the one that you attended.'”

You tell ’em, George!

What good is a deaf cardiologist?

November 30, 2008

Not much.

I’ve written about Vivien Thomas and Alfred Blaock; so it is time to write a few lines about the third person involved in the Blalock-Taussig Shunt: Pediatric Cardiologist Helen Brooke Taussig.

For those of you who may not know, Taussig’s my personal hero. When I was a patient at Johns Hopkins in 1967, Taussig herself examined me. From what my parents tell me, she was officially retired, but still lived in the Baltimore area and “stopped by the office” occasionally. I was five months old at the time, so I don’t remember it.

Her father was Frank Taussig, a prominent economist who taught at Harvard. Imagine his frustration when his daughter tried her best in school but still seemed to just not get it. I think every parent has shared that frustration at one time or another. It wasn’t her fault – it turns out that Helen Taussig had Dyslexia. Finally after a lot of patient work with her father everything just “clicked” and she was able to graduate. She went to the University of California at Berkley and then applied to Harvard Medical School.

The problem was, Harvard didn’t accept women into its medical program, so Helen tried Johns Hopkins Medical School instead. After she graduated, she took the job as head of the Cardiac Clinic at Hopkins’ children’s hospital, the Harriet Lane Home for Invalid Children.

Ironically, after becoming a doctor, Helen Taussig began to lose her hearing. And what good is a doctor – especially a cardiologist – who loses their hearing?

Thankfully, it wasn’t a sudden event. Helen Taussig lost her hearing slowly, and was able to compensate. Obviously a hearing aid would help, and she took full advantage of them. The movie Something the Lord Made is inaccurate in one respect: Mary Stuart Masterson (the actress who played the role of Helen Taussig) chose to use a more advanced hearing aid than was available at the time. The earpiece available in the early 1940’s was the size of an earmuff, and Masterson thought that such a large, bulky prop would distract from the character. (Look at this 1940’s era photo of Dr. Taussig examining a child in a wheelchair. The black box balanced on the edge of the chair is not Taussig’s medical bag, it is the amplifier for her hearing aid.)

As her hearing faded, she lost the ability to hear some of the chest sounds that a Cardiologist needs to hear to make a diagnosis. So she taught herself how to feel heartbeats by placing her fingertips lightly on the patient’s chest, and for years her hands were her “second stethoscope.” (Click here for a close-up photo of Taussig’s fingertip examination.)

Known mainly for her work in Pediatric Cardiology, few remember that Helen Taussig also played a critical role in averting the Thalidomide crisis in the United States. While Frances Kelsey receives credit (deservedly so) for not authorizing the drug for sale here, Taussig got involved when one of her German students commented that there had been a marked increase in the occurrence of Phocomelia in her home country. Taussig began to study the problem, and before long had determined that the morning sickness drug Thalidomide was causing birth defects. Because of Kelsey’s and Taussig’s actions, only 17 American children were affected by the drug.

So, what good is a deaf cardiologist? It all depends on who that deaf cardiologist is!

Documents

October 19, 2008

Holding a copy of Helen Taussig’s book Congenital Malformations of the Heart is almost like holding a copy of scripture. This is where it all began: before publication of this book in 1947, there was almost no understanding of Congenital Heart Defects (CHD). No way to diagnose them. No field of Pediatric Cardiology, barely any heart surgery to speak of. Very few survivors. No groups like The Congenital Heart Information Network (TCHIN), Mended Hearts or the Adult Congenital Heart Association (ACHA). Hardly anything. And certainly no Adventures of a Funky Heart.

I have heard stories of doctors from the late 1940’s and early 1950’s examining their patients with a stethoscope in one hand and Taussig’s book in the other. And finally, instead of telling the worried parents that the situation was hopeless, being able to offer at least a small chance: “They’re making amazing progress with surgical solutions at Johns Hopkins, maybe if we contact them…”

She was years ahead of her time. Taussig wrote an entire chapter concerning a defect she described as “Atresia or marked hypoplasia of the aortic orifice prevents the expulsion of blood from the left ventricle in the normal manner. In such abnormalities the development of the left ventricle is also usually defective.” This defect is almost certainly what we know today as Hypoplastic Left Heart Syndrome (HLHS).

Other records exist that actually predate Congenital Malformations. Alfred Blalock’s surgical notes from the first Congenital Heart Surgery (later known as the Blalock-Taussig Shunt) have been saved and are online. You can read them here. (Page 1 Page 2) And here is a photo of the clamp that Vivien Thomas designed for the surgery.

The first operation occurred on November 29, 1944. Blalock and Taussig wrote a paper about the shunt that was published in the Journal of the American Medical Association on May 19, 1945, that brought the news to the world. (Here’s a drawing from that article). Normally works in medical journals are not noted in the public press, but a medical reporter who was actually a doctor (and not a well informed amateur, as many of them are) read the article. He wrote a short blurb about it that was printed in his paper and picked up by the wire services.

And the people came. At first they went to their local doctors, to find out all they could about this new operation. And then they came to Johns Hopkins Hospital…on the train, in cars, and some of them actually walked, bringing their sick children to the one place that might could help them. Blalock and his team actually performed an estimated 300 heart operations in a single year. The American Weekly printed an article about Taussig, Blalock, and the B-T Shunt in its February 17, 1947 issue and still more people came.

And in late 1947 Congenital Malformations of the Heart was published. The life changing surgery was only designed for Tetralogy of Fallot (ToF) patients, but now there  was a way to figure out exactly what was going on in a defective heart, and with knowledge came a chance.

There is a small section titled “The Plan of the Book” in which Taussig notes how her book is organized. In the second paragraph is this chilling sentence, which was true in 1947: “Although occasionally a patient with one of these malformations may live to adult life, in most instances death occurs before eighteen months of age.*” At the bottom of the page, the footnote reads “*The recent advances in vascular surgery may alter the prognosis in this group of malformations.”

I think Dr Taussig would be thrilled with the results of recent advances in vascular surgery.