Lung Transplant and Antireflux Surgery Saves Man with Rare Disease

Podcast Transcript

Kyle: Hey, everybody welcome to The Comeback. I'm your host Kyle Michael Miller. During this episode we're talking with 40-year-old Ajit Tolani who underwent a lifesaving lung transplant and anti reflex surgery after doctors in India could no longer help him. We're also joined by Dr. Atul Mehta from Cleveland Clinic's Department of Pulmonary Medicine. But first, here’s Ajit in his own words.

Ajit: Hi, my name is Ajit Tolani. I’m 40 years old and now live in Beachwood, Ohio. When I was 15 years old and living in India, I remember I went for a shower and my hands started turning blue. Doctors confirmed I had scleroderma. It’s a rare auto-immune disease that can be life threatening over time. I felt healthy overall, until 20 years later and I started getting fevers and pneumonia. In 2016, doctors in India told me I only had a few months to live. I flew from India to Cleveland Clinic. I almost died waiting for a lung transplant, but doctors found a donor for me. Cleveland Clinic also had to do an innovative anti-reflux surgery and it worked. I’m loving time with my family. I have been given a second chance at life.

Kyle: Welcome to the show you guys.

Ajit: Thank you.

 Dr. Mehta: Thank you.

Kyle: Ajit, you’ve been through quite a remarkable medical journey that started when you were younger, when you were a teenager, and it’s taken you from India to here in the United States today. How are you feeling?

Ajit: Believe it or not I feel much better than ever before because now the focus is more on health. And, I'm under the supervision of the right experts here. I’m feeling great.

Kyle: You’ve been living with this disease for quite some time but it really wasn’t until about 2014 that it started causing problems. How did it impact your life?

Ajit: I remember it was one fine afternoon. I was sitting in my office and I started feeling that I had a temperature. When I went and checked it was a hundred and four Fahrenheit. That was kind of a wakeup call. We went to the doctor and the doctor said it was simple pneumonia and they treated me with antibiotics. After that, the pneumonia keep flaring up again and again. I started observing much more shortness of breath. I used to exercise regularly in those days but my whole strength for doing exercises and everything started going down.

Kyle: Dr. Mehta, help us understand this disease a little bit more, what is scleroderma?

Dr. Mehta: Scleroderma is a disease of unknown cause. Nobody knows why one develops scleroderma. There is no etiology. There is no causation for it. The condition involves what we call soft tissues of the entire body. Predominantly it involves the blood vessels and causes narrowing of the blood vessels, the different parts of the body. Second thing is it also involves the esophagus, your food pipe, and impairs its action. The contractions of the esophagus are significantly impaired as a result of all the changes taking place in the soft tissues of the body. It also causes high blood pressure in some patients. It also causes similarly pressures increased in the lungs as well what we call pulmonary hypertension. In some individuals the soft tissues of the lungs can also get involved with scleroderma which is referred to as pulmonary fibrosis in medical term, basically the scarring of the lung.

Kyle: Some of the symptoms that Ajit mentioned, are those typical for a patient dealing with this condition?

Dr. Mehta: Yes. They are kind of typical. What Ajit described was when his disease had progressed significantly. In the beginning most patients would have a very common term, it's a medical term, but it's a very common term called Raynaud’s phenomenon. Meaning that when any patient with scleroderma is exposed to cold temperatures, especially the fingers and sometimes the toes, they would become red, white and blue. It is a significant pain in the fingers and the toes of these individuals. Another very interesting thing with scleroderma is that there’s tightening of the skin as well. There is a loss of wrinkling because of the soft tissues involved. What happens is some patients are not able to open their mouth properly because of the tightening of the skin around the mouth. These are some of the very early features. Some of these features Ajit did have and fortunately did not have all the symptoms of scleroderma.

Kyle: Ajit, throughout the next few years you continued to see doctors in India, but instead of your condition getting better it got worse. Then in September of 2016, doctors delivered you pretty bad news. What did they say?

Ajit: I had those pneumonia flare-ups and they did my scans and then they said, oh you have ILD, interstitial lung disease. So we went to seek opinion from more senior doctors there and they said it looks good. Let's give you some medication and you'll be fine. It looks like you'll probably be fine. This was just that July. A month after that we did a scan again the scan showed significant deterioration within a month. So we went back, we scheduled an appointment, and when he saw the scan just after a couple of months he said, I'm sorry my friend your game is over. Me and my entire family were shocked. We had a situation where the doctor had told us you'll be fine. In fact, at that time we brought up the topic that you think transplant is a thing which we should be thinking? He said, no, no, no, you don't need a transplant you'll be fine. Yeah, it was really shocking for all of us. The worst is that we lost time in the process.

Kyle: What was your family's reaction when you told them the news?

Ajit: My father didn't speak for 24 hours. My wife, she was heartbroken. My sister was with me, she was shocked. Yeah, so it was not a happy situation.

Kyle: When you were seeking medical treatment in India you also had reached out to Dr. Mehta for some second opinions. Dr. Mehta, what was your first reaction when you saw Ajit’s scans and when you first started consulting him about the case?

Dr. Mehta: I did my medical school in India and I have lots of friends and family members still in India. That's how I met Ajit, through my family members. They said, Atul is a lung specialist from the Cleveland Clinic, why don't you go and seek his opinion? The first time that I met Ajit I knew the severity of his illness. It was quite evident to me looking at his CAT scans and his pulmonary functions. I had a very candid discussion with him that his condition had significantly progressed and soon he should consider lung transplantation. One of the other things was that looking at his CAT scan it was quite evident that his esophagus, his food pipe, was not working properly. He was aspirating some of his stomach contents, some of his food, into his lungs causing aspiration pneumonia which might have been the reason for the fevers he was experiencing once in a while. My initial reaction was that he had a severe lung involvement with his scleroderma, possible aspiration, lack of function of his esophagus, and that he should be considered for lung transplantation.

Kyle: But you couldn't help him when he was in India. He had to come to the United States to get care.

Dr. Mehta: I knew that no institution even in the United States would do a lung transplantation on him because many institutions do not want to take the risks of this type of procedure. It was only the Cleveland Clinic because I knew the cardiothoracic surgeon and I knew the thoracic surgeon. I have confidence in my entire medical team of lung physicians like myself. We have everything it takes for a successful lung transplantation. Hence, I told him that this thing can only be done in the U.S. and it could only be done at the Cleveland Clinic.

Kyle: Ajit, did you know you were in good hands when you first started talking to Dr. Mehta?

Ajit: It was so difficult to even talk to doctors in India. Here whenever we needed him Dr. Mehta was always there. It was unbelievable for us that whenever we need him, he’s there. Either by phone or e-mail or in person. We had that great confidence and comfort through that.  And not only Dr. Mehta, once we came here we saw the entire team was like that.

Kyle: Dr. Mehta, scleroderma can cause a domino effect on some patients and that was the case for Ajit. Would you sort of walk us through the different medical treatments that he went through here?

Dr. Mehta: It was the most essential thing that we take care of his oxygenation. Lung transplantation is not something you can do electively. It always happens as an emergency procedure because we have to have a donor. We have to have the donor of the size of Ajit’s chest as well as donor of Ajit’s blood group. So we had to wait. Ajit was placed on the transplant list. Even my gastroenterologists had not seen a patient with such a severe esophageal involvement with scleroderma. We had to plan out surgery after surgery to take care of his esophagus so that he did not develop aspiration after the lung transplantation was done.  Ajit can tell you for how many months he was not even allowed to eat. I know he was cheating once in a while.

Ajit: Never! That one I can swear. I once by mistake gobbled down but then I informed him.  

Dr. Mehta: So he was listed but then his condition deteriorated. The first thing we had to do was to put him on the ventilator just to maintain the oxygenation. But we could not keep him on the ventilator for a very long period of time and expect the lung transplant to be successful. We had to switch him to something we call ECMO. It's basically an artificial lung to oxygenate his blood outside his body and then circulate it through his lungs and his brains and his heart. Meanwhile we had to perform a tracheostomy on him so that we could also ventilate his lungs and keep them expanded as well.

At one time Ajit deteriorated while everything was going on. He was waiting on the transplant list but he developed fever. He probably does not remember some of these events because he was kept sedated all throughout this time and because he was spiking fever. We were afraid he had infection going on in his body and we cannot do transplant when he is infected. Believe it or not I had to face the family to tell them that I'm taking him off the transplant list. Their only plea was that if his fever goes away please give us a chance. For 72 hours if he gets better please relist him. A miracle did happen and his fever disappeared and we reenlisted him. We reactivated him on the list. Luckily for all of us he got the donor and you see Ajit is sitting next to us.

Kyle: He's got a big smile on his face too. It’s an incredible story.

Dr. Mehta: It is an incredible story. I've been doing lung transplants for over 30 years but every case is unique. You want to do the best for your patient and it takes a whole village. We have a fabulous thoracic surgery team. They did a special procedure which is called fundoplication. They take the top portion of the stomach and wrap it around his food pipe so that he would not aspirate.

Kyle: Ajit you’re a complicated patient!

Aji: I would actually like to say one more thing. It's my understanding that the fundoplication they gave me was probably first in the world involving this level of severity along with a lung transplant. I don't think there is any known cases of that.

Kyle: Dr. Mehta, have you seen any other patients who have been as sick as Ajit was?

Dr. Mehta: Yes and no. Every patient is different. As I mentioned, every patient is unique. Having been at the clinic for 38 years, that's before you were born…

Kyle: Yes.

Dr. Mehta: I have seen a few things, but every patient is a unique experience and the patients are the best teachers. But of course yes, Ajit’s case was also unique in the sense that he came all the way from India and everything went as we wanted it to. That's the very unique thing about it.

Kyle: Ajit, how's your quality of life today?

Ajit: Oh excellent. I do gymnastics now if that kind of explains the situation. I go to my office everyday eight hours. I happily live with my family and do everything, play games.

Kyle: Would you say that your life is better today after all of the medical treatment than it was even before you started?

Ajit: Oh yeah, way better I would say. The quality of life here, which we got after the procedures, particularly if you follow all the advices from your doctor can actually be better than before. Think about it, earlier you were on your own, right? Now, I'm not alone. I have at least a team of 50 doctors behind me who are continuously watching me. So my life is much better and I'm much more watched.

Kyle: And you didn't move back to India, you stayed in Cleveland. Which we like that you're here. You’re family now.

Ajit: We love it here. This is the city who gave life to me and my family. I have an 11-year-old daughter and she feels very attached to the Cleveland Clinic too. She says I don't want to leave the area, I want to study here and be here now. She wants to be a doctor too.

Kyle: So Ajit, we're gonna play a quick game called Go Fish. We have a fish bowl in studio if you want to take the lid off of the bowl.

Ajit: Okay.

Kyle: There’s three questions in there. Pull the question out, read it, and then you can give us your best answer.

Ajit: The question is what is one life lesson learned from this medical journey? I think it's not even worth it to have a single moment of regret or unhappiness. Live it to your hundred percent. Live every moment. You never know when your life changes. In three months, from doctors in India promising me that I’ll be fine then they declared me almost dead. So yes, it's not worth it. Just live. Enjoy. Do your best. It’s okay to go through these treatments. But you will be able to do best of the best only when you live every moment after these treatments. Do everything.

Kyle: It's good advice. Two more questions. Question Two.

Ajit: What advice do you have for someone trying to stay positive during a health scare? Communicate and talk to your healthcare advisers because they are the ones who exactly know where you are and what you are. In all fairness, they may not tell you everything because that will make you more anxious. Have a trust in them. They are thinking way beyond what you think they are thinking about you.

Kyle: Last one.

Ajit: How important is never giving up? I'm right in front of you an example of that. There was time even after transplant when I was giving up because the sickness goes to your mind. But this time the doctors came and talked to me. I was kind of depressed but the psychologist came and doctors came they would talk or nurses would talk. Then they said, no, don't give up. My life, my family, my wife they said, this is your second life respect it. Respect the donor who had actually given up a life to give you this life. So don't give up. And that’s when, believe it or not, I had necrosis of ten fingers and toes. It was extremely painful but I wouldn't give up exercising. I wouldn't give up keeping myself fit. There we are. Don't give up. Just follow a principle that if you're getting a second life honor it. Respect it.

Kyle: I like that. Dr. Mehta do you have any final thoughts before we finish up today?

Dr. Mehta: No I think I'm very happy, very grateful that Ajit is doing so well. This is the most gratifying thing for a physician when their patients do very well.

Kyle: Ajit, how about you? Any final thoughts?

Ajit: I want to say one more thing definitely and it comes out of me now that I am fit. A lot of people come to me and talk to me about how to get fit. Especially from India because I'm one of those very few Indians who have been able to take this disease and come here. I'm counseling a person and the doctor advised him that it is better to die on oxygen than go for transplant. No, it's not like that. Life after transplant can also be as great as I'm having, right?

Kyle: We're so glad you're doing so well today. You look good.

Ajit: Thank you.

Kyle: You have a great family and a great quality of life.

Ajit: Thank you.

Kyle: We're glad to hear it.

Ajit: Dr. Mehta still gets scared when I do gymnastics.

Kyle: Just be careful!

Dr. Mehta: I don't want you to break any bones.

Kyle: Thank you both so much for taking the time to chat with us today. Thank you everybody for listening. You can find additional podcast episodes on our website https://my.clevelandclinic.org/podcasts, iTunes, SoundCloud, Stitcher and Google Play.