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.
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