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Four months without a heart
She lived for months without a heart.
D'Zhana (dZON-ah) is only 15 years old, but she feels lucky to be alive. Last summer, D'Zhana was diagnosed with cardiomyopathy, which is a dangerously enlarged heart. In the U.S., about 30,000 children have a dangerously enlarged heart. More children die from the condition than cancer, but there has been little done to improve the odds. Outcomes are the same today as they were 30 years ago.
While D'Zhana waited for a second heart, Dr. Marco Ricci was on the team at Holtz Children's Hospital that kept her alive with a heart-assisted machine. D'Zhana walked the halls of the hospital with no heart as the machine pumped life through her body. D'Zhana lived with a heart for almost four months until a second heart became available.
The second transplant was a success.
To learn more about the machine that kept D'Zhana alive, please read the doctor's interview below:
Marco Ricci, M.D., Chief of Pediatric Cardiac Surgery at the University of Miami/Holtz Children's Hospital, tells the story of one girl with an enlarged heart who beat the odds by living on a heart assist machine for months.
What condition did your patient, D'Zhana, have?
Dr. Marco Ricci: She had a condition that is called dilated cardiomyopathy. With that, the heart muscle becomes progressively weaker. As that process occurs, the heart starts to dilate and at some point, the loss of function becomes so severe that heart failure appears. Even though there are some medical treatments that can prolong the process, at some point when the heart function becomes very poor the only alternative is a heart transplant.
Is this something seen primarily in children?
Dr. Ricci: This is a disease of the heart muscle that can be seen in both children and adults. It can affect children of any age.
What causes the condition in children?
Dr. Ricci: The cause is unknown in most cases. In some cases, there's evidence to suggest that a viral infection, for example involving the upper airway, can result in dilated cardiomyopathy.
Is it correct that this is not a congenital heart defect?
Dr. Ricci: Right. She was not born with this.
Are most heart defects in children congenital?
Dr. Ricci: Most heart defects are congenital, which essentially means that some children are born with anomalies of the heart that then requires them to be operated on, but in this particular case, the problem was not congenital.
How old is D'Zhana?
Dr. Ricci: She's 15.
How long has she been having problems?
Dr. Ricci: Probably about a year before she came to us. She's originally from South Carolina and she came to us maybe the spring of 2008. Shortly after she came to us, we understood that she had a very severe form of dilated cardiomyopathy. Her heart was just failing and there was no alternative but to puther on a heart transplant list
What was the family's reaction to her diagnosis?
Dr. Ricci: This is, unfortunately, a very common story, especially when this process affects children. Children can actually do very well and cope with even severe heart failure for a very long time, and then at some point something happens and they become very sick. We were very lucky because when she came to us, she already knew what she had and she had been told at that point she needed a heart transplant. We do see other children who come to us, whom the diagnosis hadn't been made and you had to disclose that to the families. It's just heart breaking.
How common is the condition?
Dr. Ricci: It is relatively uncommon population-wise, but it is, for those that are involved with treating children with heart problems, it is not that uncommon.
Was a heart transplant the only option for her?
Dr. Ricci: At that point, her heart failure was so severe that she had no other choice but a heart transplant. Unfortunately, the heart becomes so weak and it is barely able to pump blood to the body, so the only other option is to just replace it with a new heart. Medical therapists can sometimes prolong or extend the periodof time to which one can sort of wait for a transplant, but in the vast majority of cases, don't necessarily reverse the process, so a heart transplant is really the only option available in some cases.
Does she have a custom made total artificial heart?
Dr. Ricci: We initially did the heart transplant in July 2008 because her native heart was so weak that she couldn't be managed with medications anymore. When the heart became available, we did the heart transplant, which essentially means we removed her own heart and replaced a new heart in her chest.
At the time, was this thought to be a permanent fix or was this just to buy her more time?
Dr. Ricci: That was the definitive operation. We would have given her a new heart, but we saw immediately or shortly thereafter that the heart wasn't working right, which is unfortunately, a small risk for having what we call a primary graph lung function, meaning that the new heart just doesn't work. It can happen for a variety of reasons. Sometimes, it just happens without a clear reason. As that occurred, we had to place her on an artificial pump to temporarily sustain the new heart that wasn't working well. We understand that in most cases, this is actually a reversible process. That is why we placed her on a temporary pump. A day or so later, we noted that her heart wasn't coming back, and additionally there was a large blood clot that had formed in one of the heart chambers and it was extending towards one of the great arteries that is called aorta, so at that point, we were left with no option but to go back in and remove the transplanted heart because it was not functioning. At that point, we had to use the machine as a substitute to keep her alive.
How did you keep her alive without a heart, and for how long?
Dr. Ricci: We kept her alive for nearly four months. The way we approached the problem was by using essentially a device that is available on the market and it's normally used as a heart assist – it can assist one or both ventricular chambers when they fail. The only difference was that we used the pump as a heart substitute and not as a heart assist.
Was she awake during this ordeal?
Dr. Ricci: She was completely under anesthesia during the operation and she progressively became more awake as time went on over the next several days. Of the four months that she was on the artificial heart with no heart, for the first month and a half, approximately, she was very ill. She couldn't breathe on her own, so she needed the respiratory machine to breathe. Her kidneys were not working, so she needed dialysis every couple of days. She had a number of other problems. Her gastrointestinal tract was very sick, so she remained in the intensive care unit for the entire time. For the first month and a half, she was very ill. She was so ill that at some point we thought we were going to lose her. She also had a very severe infection, and when patients develop infections on the artificial heart or any artificial device, they tend to be very severe because the body has a reduced ability to fight the infections and also because the presence of the foreign body to assist in circulation is one of the things that can make the individual more vulnerable to die from the infection. Despite all the difficulties, we were able to overcome most of the problems so that after a month and a half or two months after the operation, she actually started to get better. We were able to take her off the respirator. It seems so surreal to have someone walking around without a heart.
Dr. Ricci: It is not that surreal in our world, but it was very surreal to her. She was very emotional when she sort of regained consciousness and awareness of her surroundings, which happened probably when she started to get better a month and half or two months after the operation. She was very emotional and mom and dad, especially mom, they were by her side all along, but it was very surreal for her. She had no heart, and her circulation, her life was being sustained by the machine, so that was very difficult for her to accept. You can imagine a teenager, a 14 year old – she's used to going to school and doing all the things that 14 year olds do – being stuck in a bed in the intensive care unit connected to an artificial heart, not being able to get out and do all the things that other kids of her age would do.
What was being planned for her, medically, while she was being kept alive?
Dr. Ricci: After the first couple of months, when she started to get better, we started making arrangements to proceed with the second transplant. Of course, we knew that this was not a situation that could have been protracted forever, because you can't live on an artificial machine forever and the clock is ticking. Every day that she was on the machine, there was a risk for developing complications – some of them are very severe, such as stroke, blood clots can form in the machine and can go anywhere, including the brain. Thankfully, that did not happen, but we were increasingly aware of the need for doing a second transplant, which was the only definitive solution to her problem. As she got better, we made the arrangements and we, at some point, placed her back on the transplant list and when her condition allowed and when a new donor became available, we went ahead and did a second transplant. Two days later, we also transplanted her kidneys.
Did she get a custom made artificial heart?
Dr. Ricci: Unfortunately, there isn't an artificial heart available on the market that can be used in this patient population, in children. There is an artificial heart that can be used in adults. Even though this technology has been around for many years, only the latest versions of artificial heart are now safer to use. The unfortunate aspect of this, because of the relative rarity of this condition in children especially, is that companies aren't heavily invested in the development of this technology, so there really isn't a substitute that can be used in children to completely replace the heart even though technically, she wasn't a small child, she was a relatively big child. The alternative we had was to use the machine that is currently available on the market but is normally used as a heart assist, which instead we used as a total heart replacement.
Did you have to do anything to that machine differently then how it's normally used?
Dr. Ricci: Yes. In reality, there are two machines, two different pumps. Each pump replaces one ventricle. The heart normally has two ventricles – the right ventricle and the left ventricle. Each pump normally assists one of the ventricles, so we use two pumps to replace both ventricles. The modification that we had to make essentially consisted of creating artificial conduits to connect the machine to the blood vessels without the heart, because normally, the machine is connected to the heart. In her case, the heart was removed, so the machine had to be connected to the blood vessels. That's why we had to be creative in making those connections.
Was this technique developed here, or has it been used elsewhere?
Dr. Ricci: I think it's been used. There was one similar case done in an adult in another institution in the United States. We also did the second case in an adult, but it had never been done in a pediatric patient.
What's hooking up the machine to the body?
Dr. Ricci: Artificial conduits. There were four of them – two for each ventricle.
Without that option, without those modifications, would D'Zhana have been able to be kept alive?
Dr. Ricci: She needed a machine to function as a heart substitute. That was, to us, the most effective, perhaps creative way of accomplishing that goal, because there isn't a machine that can be used as a substitute in a pediatric patient.
When another heart becomes available, does she go into the operating room with this device still on her, and what happens next?
Dr. Ricci: The process for listing somebody for a transplant is actually quite extensive. It involves a number of health care providers. Essentially, the way it works is that when a heart becomes available, after making the arrangement and preparations, we go to the operating room with her. She was very emotional. From an emotional perspective, that must have been very difficult for her. At that point, it was the third surgery and the second transplant, knowing that the first transplant had failed. I remember she told us she had spoken to God and she was fine and she was ready to go. It was a very emotional moment, but when the time came to go to the operating room, she was also very serene. She went under anesthesia and we carried on with the operation and we opened her chest and took all the artificial material out, including all the connections and the machines used to support her circulation. We removed all that and inserted the new heart, which went well.
Did it work right away?
Dr. Ricci: The new heart worked right away. It was a very good organ and she made a very expeditious and uneventful recovery after that.
Did she get a pediatric heart or an adult heart? Does it matter in this case?
Dr. Ricci: We normally go by weight and body size. We also try to match the age, but there is no absolute matching in terms of age.
How long was her recovery?
Dr. Ricci: She was home in probably two and a half weeks.
What's her prognosis?
Dr. Ricci: Her prognosis is good. The new heart is working very well. She still needs to take her medications. Some of them are called immunosuppressant medications. They are used to make sure that the immune system doesn't adversely react to the new heart. The new heart is perceived by the human body as a foreign organ, so the immune system of the recipient tends to attack the new organ and reject it, so that process needs to be controlled. That's why patients have to take immunosuppressant medications until the new heart is there, because otherwise, the risk of rejection becomes significant. The immune suppressant medication can have very significant side effects, but in her case, she is coping with those very well. The life expectancy of a new transplanted heart is not forever. It is approximately 15 years. That means, on average, a patient that has received a heart transplant, after 15 years, will face the issue, potentially needing a second transplant, which in her case will be a third transplant.
What are the chances of her getting the same condition again?
Dr. Ricci: She won't get the same condition again. What normally happens is that after a certain number of years, there's a process called chronic rejection. It goes on irrespective of the efficacy of immune suppressive medications, so even though the immune suppressive medications have been very effective in controlling the acute rejection, they are not as effective in controlling the chronic process of rejection, which occurs over many, many years. At some point, the process becomes very advanced then the transplanted heart starts to lose its function. When that happens, we have no choice but putting a new one in.
How innovative is this technique for adults, as well?
Dr. Ricci: It's a very uncommon occurrence and in adult patients, it's done under occasions that normally, a machine that is intended to be used as a heart replacement, but an artificial heart is used. In pediatric patients, that's not really an option. Even though the same artificial heart has been used in very rare occasions in children that are grown up and have a large body mass and body size, there's really no alternative in a smaller child, so this is the only option if the heart needs to be removed.
Do you think that your success here, with the adult patient as well as D'Zhana, will encourage more cardiac surgeons to try this?
Dr. Ricci: It's not a completely new technique, but this is a strategy that allows physicians to think about having one more tool and one more option available should the need for removing the heart come about, which is indeed quite rare, but it is possible. The emphasis on the children is because there really isn't a technology that has been developed that can be used in children as a complete heart replacement. That's why children are very different from adults. Hopefully, this case will also increase the awareness of how technology needs to be developed also in the field of pediatric patients, not only for adults, in terms of heart replacements, which hasn't really occurred.
Why is it that the same artificial heart can't be used in children?
Dr. Ricci: Unfortunately, the number of children that require this technology as a heart replacement is very rare. In adults, it's also uncommon, but it is not quite as rare. Companies that manufacture these devices have more incentives in manufacturing and developing devices that can be used in adults, but they don't have the same interest for children because the cost of developing these technologies is very significant. The children that could be potential candidates for this technology are only a few, but the costs are so high and the time and the process and whatever needs to be invested and research and development is so significant.
In theory, it could work, but is it not being done because the research hasn't been done?
Dr. Ricci: Right. Some of the currently existing technology for adults could be modified and applied to children. The problem is making the same device smaller, and that doesn't necessarily mean that the same device will be reliable. There's a lot of technology that needs to be modified and size isn't always one consideration.
How amazing is it that D'Zhana is alive today?
Dr. Ricci: I think she certainly is lucky. After the first transplant, the first heart failed. I think that she is alive because of the fact that she was young and sometimes children are very resilient. They are very resistant to a number of offending factors, including infections and other things, but she's also alive because of the team that has cared for her. The team is enormous and it involves nurses, doctors, support staff at Holtz Children's' Hospital that has worked relentlessly, worked tirelessly on her. For example, one of the things that was very difficult to manage from our point of view was the fact that she was extremely emotional and that was actually quite difficult to control. As part of the team, we involved one or two psychologists that came to see her on a daily basis and put her on medications. She was really having a hard time coping with her current status when she was supported by a machine.
I remember one day that she wanted out to see the outside. That was maybe after three months or so that she had been on the machine, so she was feeling better. She could get out of bed with assistance, so we put her in a wheel chair and we went downstairs. We went to the main lobby of the hospital and took her outside and she was protected – she had a mask and gown and monitors with her, and she got out and she kept looking at the outside, looking at the sun. She was a little emotional and it must have been very difficult for her. Then she saw something that looked like a facility not too far from the lobby of the hospital. It's actually a school. She asked whether that was a school, so she was probably thinking of days when she was at school and she could do all the other things that kids normally do, and there she was stuck in a wheel chair connected to an electric machine. That must have been an incredible experience for her.
Physically, can she do now what she did before?
Dr. Ricci: She's basically a normal child. Except for the fact that she has to take some precautions and do certain things, she can actually live near a normal life.
For more information, please contact:
Omar Montejo
University of Miami Office of Communication
(305) 243-5654
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