Preview Mode Links will not work in preview mode

I'm Aware That I'm Rare: the phaware® podcast

A new podcast series devoted to raising global pulmonary hypertension awareness with dynamic stories from PH patients, caregivers and medical professionals from around the world. New Episodes every Monday & Thursday!

Use the search bar above to search episodes by topic. Search "phawareMD" to discover podcasts with medical professionals.

Get our custom smartphone app (for iPhone™, Android™, and Amazon™ devices). It's the most convenient way to access the phaware™ podcast.

The views and opinions expressed in the phaware® podcast do not necessarily reflect the official policy or position of phaware global association. Information on phaware.global and phaware social media sites is provided for general information only. It is not intended as legal, medical or other professional advice, and should not be relied upon as a substitute for consultations with qualified professionals who are familiar with your individual needs.

To learn more about PH visit www.phaware.global. phaware® is a 501(c)3 organization. © Copyright 2015. All Rights Reserved.

Generously Supported in part by Actelion Pharmaceuticals US, Inc., Bayer, United Therapeutics Corporation, Acceleron, CVS Health & PhaseBio Pharmaceuticals.

Jane Price - phaware® interview 327

May 19, 2020

Jane Price's daughter, Connie was diagnosed with pulmonary hypertension at age 3. At age 4, she was the 2nd child (the first girl) to ever have the Potts Shunt operation in the UK. In this episode, Jane shares Connie's 2 year post surgery story for everything to become more stable.

Hi, my name's Jane Price. I'm calling from England, Essex. My daughter, Connie, has pulmonary hypertension. She is six. She was diagnosed when she was three, so we've been in this world for about three years now.

She is the middle child. At the time, we only had my older daughter and her, and she was quite late at doing the things that her sister had done in terms of walking, moving around, that kind of thing. I'm an educator, I'm an assistant head teacher, so I'm quite au fait with what children do and their steps and their processes. So, I wasn't overly worried, each child developing at their own pace, that kind of thing. But when she did start walking at around 15, 16 months, she just didn't want to do it. That's the only way I can describe it. We'd be on play dates with her sister and her friends and children that were Connie's age, and they would all be off and running around in the park and she would sit in her buggy and just watch. Or she might get up, do a little bit and then come and sit back down again. No amount of cajoling her to try and get on with it worked.

Then, we'd notice other little things like you could hear her breathing a lot. She always seemed to have a cold, so we put it down to that. Her lips would go blue quite quickly. So if she was in a paddling pool in the summer, her lips would be blue. If she had walked, if we'd managed to get her to walk a distance, I remember being at Center Parks, which is a camping place over here and she'd walked no more than a hundred yards, and she just didn't want to do anymore. When you looked at her face, her face was completely pale except for these really dark blue lips.

I kept saying to my husband, this isn't normal, but my oldest daughter, who's three years or two years older, she was born with brain damage unfortunately, and ended up with cerebral palsy, which is mild, thankfully. But at the time, every time I went back to the doctors for something to do with Connie, I felt like they thought I was an over-protective mum. I kept relating it back to maybe I'm projecting my worries and everything that happened with Lucy, my first daughter, on to Connie. This went on for a year or so.

Then, we were at my daughter's fifth birthday party, my elder daughter's fifth birthday party. It was a very hot summer day. It was before the party had started and the children were all running around in the playground. Connie came into the hall where we were having the party. She was really whingeing and she came over to me and, as she reached me, she collapsed and fainted. She came round as quick as she went down and we gave her an orange juice to bring up her energy levels. The party was literally just about to start. It was Sunday, so of said, look, she's fine. She was fine. She was up and about again. I said, well, I'll take her to the doctor's tomorrow, which I did.

I went with the list. I sat there that night, I remember it very clearly and I wrote a list of everything, blue lips, lack of energy, doesn't want to walk, won't join in, drinks like a fish, she's just trying to get water down her all the time. You couldn't give her enough water. The fact that her hands were always freezing, the breathiness, her heart, you could basically see pounding in her chest, and one of the veins in her neck, you could always see pulsing.  

I went to the GP, the doctors over here. I put this list in front of them, and I just said, you need to put this kid together and work out what it is because it's something. So, they did blood tests, and back with nothing. They eventually agreed to do a heart scan that they classed it as non-urgent. This was September, and when I got the letter through for the appointment, it wasn't until the following April, so quite a way. In February, she started being really sick and her heart was going crackers and she was dehydrated. So, they admitted her to hospital. I remember speaking to the consultant at the time and said, she has a heart scan in a few weeks’ time, can we bring it forward? He said to me, if she had something serious, you'd know about it by now. With that, I thought, okay, I'm being paranoid. This is me projecting here. To the point that when the heart scan eventually came around in April, I told my husband not to come. I said, I'm going to get there. They're going to scan her and they're going to say she's fine. So, that's what we did. I went.

Probably about 45 minutes into the scan, I realized that the scans don't tend to last this long unless they find something. He called the student over that was in the room and started pointing things out. So, I texted my husband and I said, I think they found something. They stopped the scan and said that, at the time, they thought it was something mechanical, but he wasn't an expert and wanted somebody from a hospital in London to come down and see her, which was the following week.

So, we went back and my husband came with me this time. Again, he scanned her, and again, the scan went on. I remember the nurse took Connie out of the room and they sat us down and they said, it's either something mechanical, which is easily fixable. It's a cardiomyopathy of some description or it's PH. He said, we don't want it to be PH and don't Google it.

Three days later, we were admit it to St Thomas' the Evelina, one of our children's hospitals here, and we were there for two days. She had another syncope at which point, she was diagnosed with PH and transferred straight to Great Ormond Street Hospital, and we were there for seven weeks while they tried to stabilize her.

Unfortunately for Connie, the medication doesn't work very well for her. It never did. She was put on triple therapy. Over here, that consists of bosentan, sildenafil and the epoprostenol drug through the Hickman line 24/7. So, they put her on that straight away. Unfortunately, she didn't really respond to it. It stabilized her to an extent, but he said out of a hundred kids, she would be classed in the bottom 25. We would have hoped the drug therapy would have got her over the 50 line. She's still in the bottom 25.

At that point, we began looking at transplant for her. Then, they came up with offering us the Pott shunt surgery, which is where they obviously connect the descending aorta and the pulmonary artery together to create a shunt, like a pop off valve, for the excess blood to go through so the heart doesn't have to work as hard to pump it through. At the time, we were told that there would be a 15% chance that she wouldn't pull through the surgery. And they put it to us in terms of if you have a quadruple heart bypass, which everyone considers to be a really severe and complicated surgery, you've got a 5% chance that you won't pull through. So to sit there and say that my, at the time, three year old daughter would have a 15% chance of not pulling through this surgery, it was utterly terrifying.

Over here, at the time, only one child in the UK had had this surgery done. Even then, the surgeons from Paris had come over to do the surgery at Great Ormond Street. So nobody in the country had performed this surgery. As you can imagine, we were overwhelmed to start with, we'd only been in this world at the time for about four weeks, and terrified. So, we opted not to go forward with the Pott surgery, that we would look to go down the transplant list. We couldn't contemplate what that all meant at the time. It was just too big. And in a way, transplant for us is what you see in the movies. It's child gets new heart, lives long, healthy life, end of story. We didn't know any better.

We found out very quickly that that's not the case, but we didn't know any better at the time. So, we then went into the world of transplant, and Connie had all of the procedures, the pre-procedures, like the vaccinations and all of those things. We had all the counseling sessions with the team at Great Ormond Street, and the explanations blew us away of what it actually entailed. But we were still thinking that's the route we'd go down. She got discharged at seven weeks to manage at home and it just seemed that every week that we were going back up, we were having weekly appointments, we were just getting more and more bad news. That she wasn't responding. That she was sick still, and a decision had to be made.

I remember, quite clearly, I think it was late July, beginning of August of that year, her consultant sat us down and said, it's decision time, you need to decide. They put the Potts surgery back on the table at this point. They were saying to us that Pott surgery will buy her five years and then a transplant could buy her potentially 12 years. So you then, in your head, you're thinking you've got a stacking game that you're playing. You're trying to buy her years. At the time, my husband and I just kept thinking, but if we make the decision to put her on the transplant list now and she gets a transplant tomorrow, if she's that fortunate, have we just taken two years away from her because she could be okay for the next two years? It probably makes no sense to anybody, but in our world, at the time, it made sense. Alternatively, she could go on the list tomorrow and not get a transplant in time and then you should have done the Pott surgery.

Everything was a gamble and it was a gamble that I just couldn't take. I had, with my eldest daughter, being given the decision that ultimately led to her having brain damage, and it just felt like I was being put in that position again. I remember sitting in the room with my husband and the consultant was talking to us about both procedures, and the transplant team had come in, and everybody was in the room, and I couldn't even lift my head up. I said, I can't make this decision. My husband turned around and said, she needs you to tell her what is Connie's best shot.

And he basically said that transplant could take, on average, up to 18 months. Connie doesn't have that long. So for us, ultimately, there was no choice. We had to go with this completely unknown, if you like, procedure in the UK and take a chance that somebody somewhere knew what they were doing enough to get her through it. So initially we were going to go to Paris and have it done. And the surgeons there go on some kind of four weeks of sabbatical or four week holiday in August. This is the time it was. Nobody was coming back to us or they weren't being quick about coming back to us. And it just felt like they weren't going to take care of my child in the way the team at GOSH would. And I didn't feel safe.

So, we asked them to move the surgery back to Great Ormond Street and that we would put our faith and our trust in the surgeon and particularly the anesthetist at GOSH. So, she was booked in to this surgery. It was the most terrifying day of my life. She sailed for it. She absolutely sailed through it and she came home after 10 days. Everybody was a little bit in shock at how well she responded to being in that position. But again, with Connie, she didn't respond well enough to the surgery. The initial thoughts along the Potts, based on the child that had had it done previously, was that within six months, she'd be able to come off her backpack and off her Hickman line and, all being well, would have a good four or five years prior to transplant.

All indications from the other child that had this was that this little boy was now walking up mountains with this school and taking part in adventurous PE and physical education and things like that. So everything was really positive. It just took Connie a lot longer to get there. The two year anniversary just gone at Christmas of having her Pott surgery and she came off of her backpack about four weeks before that. So, coming up to two years was when her heart was responding well enough to come off that medication. So, currently, she's doing really well. Her heart numbers are normal for the first time in her little life. She's just about to switch to ambrisentan, because she's old enough for that drug now, which is a higher strength than the bosentan that she was on. So, that should continue to have improvements.

The only downside that we found out after the Pott surgery, we were under the impression that, like I was saying before, in terms of the stacking game, when her shunt began to stop working, she will be eligible for transplant. But unfortunately, over here, there is not enough data to support that she would be a good candidate for transplant anymore. So, at the moment, although her consultants would certainly put her forward for transplant should the time come, at the moment, they don't feel confident that the transplant team over here would accept her onto the transplant list anymore.

So, we are putting all our eggs in the hope that the Pott shunt buys her between five and 12 years. Evidence is now showing it's almost as long as you'd expect a lung transplant to be, which would be up to about 12 year average. So, there's evidence now to show that the Pott shunt should sustain life for that long as well. Within that time, we're hoping that there is enough evidence to convince the team at Great Ormond Street that transplant from Pott shunt can be done, and can be done successfully. Certainly, we know that that's the case for a couple of PH patients over in America that this has already happened to, all be it adults. So that's where we are at the moment.

We have a support group on Facebook, but obviously being so rare, there is no one in our immediate vicinity that has this kind of understanding. At the moment, she understands that her lungs don't work the way they should and she understands that this does naughty things to her heart. The one thing that we were very conscious of when she came off of her backpack, off of the epoprostenol drug was that she didn't assume, and that her sister didn't assume, that that meant she was better, that she was cured because she's not. So, the language that we use at the moment with her is that her heart is doing better because of the surgery, but her lungs are still naughty.

One of the things that myself and my husband really struggle with is the next bit, especially as my eldest is eight going on nine, not yet in the world of social media, not yet in the world of Google and phones in terms of being on them independently, but is soon obviously going to be. No amount of me trying to prevent her looking this up is going to help, because friends will get there. Her friends, rather, will get there. She'll find out. So, it's about, what do we tell them that doesn't terrify them? So, we have a counseling team, a psychology team at Great Ormond Street that are now working with us on how we talk about what it is in a way that doesn't scare them, and in a way that doesn't create anxiety for them.

My eldest daughter didn't cope well being separated from us from the amount of time that she was when we were initially in hospital, despite my husband and I ensuring that one of us was always at home with her. Home for those seven weeks was at her grandparents' house and we would come back from the hospital and stay there with her while the other one stayed at the hospital with Connie, and this was every day. My husband and I saw each other for an hour a day so that one of us was always with the kids.

She was so young herself at that point. She was five. So, consequently, now she has a lot of counseling herself just to try and deal with the hospital visits that still happen, the emergency services that turn up at our house because we've had to call 999, seeing her sister being blue lighted to hospital or having the community nursing team round checking her oxygen levels when we think that her chest might be infected, that kind of thing. It has a profound impact on a little person. She started obsessing with children dying and just couldn't cope with it. So, she's in counseling and she also attends the local hospice, has a sibling support group for children who are going through something with a family member or close friend that's either life limiting or terminal.

So, she has got a group of friends that, although they don't understand PH, are in a similar position where they are either a young caregiver or are going through having a sibling or a parent who has an illness or who has died. So, we're hoping that that will give her a group of friends over the years that share that understanding with her. Connie seems to accept it all quite easily, but I don't think she remembers life… I know she won't remember life before being in hospital, whereas Lucy does. Then, we had our third daughter, Annabelle, at Christmas last year. So they're both a little bit obsessed with her at the moment. I think that's eclipsing everything else at the moment for them.

My name is Jane Price, and I'm aware that my daughter is rare.

Learn more about pulmonary hypertension trials at www.phaware.global/clinicaltrials. Never miss an episode with the phaware® podcast app. Follow us @phaware on Facebook, Twitter, Instagram, YouTube & Linkedin Engage for a cure: www.phaware.global/donate #phaware #ClinicalTrials