VIDEO: My life on IV medication for PH

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I’d like to welcome people to this infocast today. I’m going to be chatting to Rinku to get her experiences of what we call ‘transitioning’ onto, and living with, intravenous (IV) medication to treat pulmonary hypertension. I want to learn from your perspective Rinku, about what it’s actually like and the challenges, and what helps you get to that point of going onto IV.

Just as a way of introduction, can you give us some background and what you’d like us to know about you?

My name’s Rinku, and I’m a mum, and a qualified occupational therapist at my local hospital in Coventry. I’m married, with two young children who I adopted after going to IV epoprostenol so my journey for that is quite different, but it started once I was diagnosed with PH.

I was told having children was a risk factor so we took that on board and between us as a couple we came to the decision that adoption would be best for us. As my journey and condition progressed, we decided to go onto the IV medication.

How long have you been diagnosed with pulmonary hypertension?

I’ve been diagnosed for ten years now.

When you were diagnosed, were you acutely unwell? Or did you become unwell over a long period of time?

My journey started at university. I was doing my last year at university and all of a sudden, I started noticing I was very breathless, and they couldn’t work out what it was. I’d been on holiday to India, and they thought I had TB, so they sent me for some scans at that point – but when they came back it wasn’t any of those medical conditions.

Because my heart came back enlarged in one of the scans, that’s when they thought something was going on and that’s where the PH journey started about ten years ago. The only symptom I ever had was shortness of breath, but nothing else.

So, a fairly classic picture really. Maybe in the future we can come back and talk about that pathway and some of your experiences but we’re here to talk about intravenous drugs. So, how long have you been on intravenous drugs?

I’ve been on intravenous since 2016, so four years.

Four or five years then, yes. How did you get to that point? Obviously, your condition was deteriorating, but how did you get to that point where you said “that’s the treatment route I’m happy now to go down”? Or would you not use the word ‘happy’?

For many, many years I was stable on bosentan and ambrisentan. But my liver starting failing after five years on them so I decided ok, that’s fine, and went onto the epoprostenol inhaler.

But the pressures were still not coming down enough and the consultants thought that my heart was struggling at that point.

Were you struggling as well? Was it that your symptoms were getting worse, or just your measurements getting worse?

It was more my measurements; I didn’t feel it myself. I said “no I don’t want this [IV], I don’t feel unwell enough”. But one doctor said this would be the best thing, that I just needed to go onto it, and I was a bit resistant to that. But then we spoke to another consultant and they said “ok, let’s have a look into it”, so they made me do a bike stress test.

They showed us all the evidence from it, each graph and chart, to show us what my heart was doing at each stage. And my oxygen exchanges, and what they were doing too.

The said “this is where your heart is getting stressed, and why we think you need to go on it [IV]” so that really informed our decision.

I couldn’t feel it, but when I was doing certain activities or stressing myself / doing certain exercises, that’s where it was going to impact.

That’s really helpful. From my experience and listening to lots of people over the years with PH, sometimes it’s that these more ‘advanced treatments’ (like nebulisers, or IV) are more demanding, so people need to feel more poorly or unwell to accept them. In a way, that wasn’t quite the case with you. You had to work through the process.

It’s also interesting Rinku that you made a joint decision with your husband when you were working this through.

Yeah, we both asked the questions and my husband read a lot around the IV medication – what it does and how it works, and how it would help me. So, he helped me to make an informed decision, as well as the evidence presented to us by the doctors at the hospital, telling us this is where it’s happening and this is where you can see the change.

So that really, really helped. I also did a lot of research by talking to people who were on IV at that point. I asked the hospital if they could put me in contact with anyone I could talk to about IV medication and my fears and looking at that sort of stuff.

I even asked the hospital to show me how to make the medication before I went on it and the pump.

This might sound a really daft question, but what was it that your husband brought to this decision? Was he asking the technical stuff, was he reassuring? What did you find really helpful?

He is very technical, so he was looking at the evidence base that was out there for this drug. He was looking at the analytical literature and graphs and stuff that I wouldn’t probably think about. I’m more emotional and would look at it at face value – deciding it would either work for me or not. He helped me look at the pros and cons, and that if I went on this my life expectancy would expand and things like that.  So, it was more of an informed decision.

He really helped me grasp that if I went onto this, it would be a challenge, but we could work through it together. It’s about knowing you have that support as well.

This might be the wrong word, but I’ll use it – what were your main worries or fears about going onto IV? What were the bits that, rather than saying “ok let’s just go for it”, were the barriers or the understanding that you needed to get to?

It was basic things in life. How do you shower with this? How do you carry the pump around with you all day, every day? How do you sleep with it? How do you travel with it? It was those anxieties for me.

I’m completely independent; I go to work, I work full time on a busy ward – how would it impact my life?

It was all of those questions, and just the fear of going onto it. It was scary but doing all the research and talking to people really helped that. The first bit was making sure I had all that information that I’d gathered.

Obviously, I got over all those barriers, because I’ve learned how to live with it now after four years and I can tell people about it.

But it was things like, where do I put the pump? Can the pump get wet? Is it going to limit me? Can I go and exercise with this? It was all about how it might impact my life, but four years onwards now, I just live with it.

I think that’s why I’m so grateful to you for sharing your story. I’m a nurse consultant in Sheffield and as a doctor you can tell people what it’s like, but there is nothing like hearing from someone who has lived it and done it.

Going in the shower, going on holiday, things like that… Hearing someone’s testimony saying they’ve had the same worries, but you can do it.

Did you have any concerns about what we call ‘body image’ – because you have a line there? Was that part of your worries?

It was, with my line.  Obviously being at work as well but at home it was like, oh I can’t wear this, because I don’t want to expose my line. The other complication I’ve got is that I can’t have dressings on my line, so that’s one big factor.

Is that because you react to dressings?

Yes, it’s because I react to dressings. So, any clothing or garments I choose have to be quite specific to me.

I used to wear a lot of dresses, or things that were low cut or strappy, so I had to change and adapt all of those things.

But it hasn’t changed the way I am; it’s just doing something different. And I suppose it’s knowing that change is probably going to be good for me.

It’s scary when you tell someone they’ve got to change something. They put a defense up – I thought ‘I’m not going to be able to change that’, or ‘I’m not going to be able to do that’. So many thoughts are going round your head, like how am I going to do this, or sort this out?

I was working in a hospital, so that dramatically changed.

Do you think that, as someone who works, that working in a hospital was helpful? Or is it that you had more worries because you knew more?

My husband I are both health professionals, so we know a bit too much. We have to try and take a step back and think ‘we’re the patient this time’. We have to stop ourselves overthinking about it; this is informed by our team.

I thought I knew enough, but I don’t think I did at that time. With work for example, I worked on a busy ward and they had to change that because I can’t go with IV onto the ward in case someone pulled [the line], so that changed quite a bit for me.

As a health professional I don’t think you can still be as informed. You’ll know where to look out for things and what to do, where to seek help and how to approach people with the line and things like that.

So, if I went to the hospital for example, and the first thing I tell them is I have an IV pump and you can’t stop it, you can’t stop my Hickman line – to get that across to a junior doctor is quite challenging for anyone. Being a health professional, I’ve managed to do that and that has really helped.

Asking the question, saying ‘no you can’t do that’ and ‘this is what I need’… those type of things have helped.

What sort of things, from your perspective (and I’m still focusing on your decision to go on IV) –  what sort of questions did you ask healthcare professionals? If you were talking to someone going through this process, what were the top three questions you found really helpful?  

The questions we asked were: How is this going to change my pressures? How is this going to change this side of my condition and what do they envisage around life expectancy, and how the medication will help me?

That’s the biggest question I think, that really informed us, and was our main thing. “This is how the medication is going to prolong your life, or give you that energy”.

The other thing I would ask is: Would they recommend this as a final option, or would they want to trial something else? It’s important to ask, and keep open-minded, because there is always research and things going on. So that was the other question I asked.

The other thing is the pump. Obviously, they want you to stay on it as long as possible, but I asked about the cassettes (which are different medication strengths, I’m on quite a low dose). How much are they expecting you to have, and how will that impact you as well?

That was one of my main questions, because of how many vials I’m currently on now and how many I was on when I first started (which was one). So that changed.

So, in essence, you were looking to hear from health professionals about ‘how is this going to benefit me’? I think that’s really important. You need to know that. Once you’ve been on a treatment for so long, it’s almost like goal setting. In three months, or 12 months, how will I feel?

In that initial period of time, when you went into hospital to start IV, how long were you in for? How long did it take? Talk us through the practical things…

I was informed of the side effects it can have first, and that I could potentially have a long stay in hospital. But luckily, I was only in and out for about a month – so I didn’t have to stay there for the whole journey.

I went in for the first two weeks and had a PICC line put in. I got used to making the medication, doing all that with the nurses, and getting confident looking after the PICC line. Every day I would practice how to make the IV epoprostenol; practicing with the nurses and watching them do it.

You can have the PICC line for three months at a time, so I was told, but I took the decision to say ‘can I just have my Hickman line now?’

I brought it forward for myself because I just wanted it to be there and done because that was the plan in my head.

I think it’s important for people listening to this to point out that different people will have different ways of administering it. I know you’re talking about a PICC line; some centres go straight to a Hickman line, it’s a variable protocol. It’s helpful to understand that.

So, you’ve gone through that process of learning it in the hospital setting, where it’s quite different in the safety of a hospital. Then you go home, and you’re on your own now…

Yeah, I was allowed to come out of hospital for four days (on ‘weekend leave’) but I had the number for the nurses. Every night I had an issue with my pump so that was quite scary.

Making up the meds, we kept getting air bubbles, so it was a bit like ‘oh, this is not what I expected or what I thought it would be’. That every night I would be ringing the hospital saying this is happening, the line’s saying this on it, and I’m there going ‘oh, why is it saying high pressure when I haven’t got anything there?’

It was a lot of to-ing and fro-ing with the hospital and problem-solving.

What I’d done initially is set myself up with a trolley and organise completely where I was going to put my meds, what time I needed to make my meds and how long it was going to take. So, it was planning all that and setting the time aside to do all this and have it all labelled. I was quite organised and made sure everything was in containers so that if I needed any help, and my husband needed to do it, he knew where to go as well. He was trained up at the same time.

It sounds like giving yourself patience and time and being kind to yourself. It is very different coming home, sometimes to disorganised situations. It’s very different at home. Sometimes everyone else is at home as well, family and husband and things like that.

So that’s really helpful, and maybe in the future we can come back and look at your trolleys and what works for you, as it’s different for different people.

I took advice from the nurses on that; what do you think I should use, what surfaces should I use, how do I keep everything sterile, and clean? They did guide us, and I kept showing them what I had ordered and how I was going to organise myself.

There were other patients on the ward as well, so I was able to bounce ideas off them.

Thinking about a new person coming down this route, what have been your main challenges with the pump? Again, your top two or three challenges?

I think the challenges are wearing the pump – how you’re going to wear it, and how it’s going to be comfortable for you. You’ve got to work that out.

Some people like to have it over their shoulder. I like to wear mine in a pump bag, and I just have it round me so it’s tucked away, out the way, and I can get on with things.

When going out and about, I have an extra ‘emergency’ bag. I leave it in the car with everything set up, so I don’t have to worry about it each time. We can go out and do whatever we want; shopping, days out and things like that.

The other things are showering and sleeping with the pump. It’s not a ‘challenge’ but you have to work out what’s best for you. So, for example, some people put it in the bed with them, but I put it on the side.

When I’m showering, I put it on the hook and shower quickly. It’s just working out those things.

You need to have a look at your environment, have a look at how things are set up at home and how you’re going to manage those.

Because you’ve got to wear this 24/7 you’ve got to work out what’s best – like pump bags, or side bags.

It sounds like one of the techniques is that you just have to plan that bit more than you did before, but I presume it then becomes much more of a reassurance and a routine?

Yeah, I can go anywhere now. I just look at the environment. Last year we went on holiday to Canada, and I thought about where I was going to make my medication (so it was a clean and sterile environment), where I was going to shower, and how I would make sure no-one touches the pump or pulls the line or anything.

So, it was working out those things, thinking about your environment and thinking about what you’re going to do and how you’re going to manage those.

What’s it like with the children? It’s obviously a joy having the kids, and you adopted them, but in the context of yourself, what have the discussions with the children been like?

My boy was very little so we’ve always introduced the pump to him. We’ve given it a name actually, it’s called Bubbles. My little boy likes to call it Bubbles. Everyone else, my nieces and nephews, when I first got it, they named it for me. They said ’oh yeah, it’s called Bubbles’, so that’s what it’s called.

He knows I’ve got this ‘poorly’ on me, and he’s not allowed to touch it. He’s also aware that sometimes mummy needs extra time, or that she can’t run around and do things.

I was naughty and gave him some syringes and things to play with, so he was familiar with all the technical bits. He never touches anything or does anything with it. He’s tugged on my line by accident because of messing around and playing with him, which I still do, but he knows mummy’s pump’s there, and he can’t step on it or touch it.

With both of my children, we’ve always said ‘look at the pump, have a feel of it, have a play with it’ -so we’ve never kept it from them.

I suppose it’s about making it not something to be fearful of, but also it’s not a toy, so it’s getting that balance?

Yeah, it’s about balance. He’s four, he’ll ask about it, he’ll say ‘how’s Bubbles doing mummy, is Bubbles ok’? So instead of asking me how I am, he asks how Bubbles is doing.

That’s how he addresses it and that’s what all the kids do now. They’ll say, ‘how is Bubbles, is he ok’? So, it’s just about introducing them to it early and saying ‘this is the pump, this is why mummy can’t run around too much with you, but she is going to do sit-down activities’.

Just one final question for this chapter then (I would like to come back and do chapter two as there are lots of things that you’ve raised) – for someone on the verge of going onto IV, and thinking about it, from your perspective, what has being on IV done for you? What are the biggest benefits, for yourself?

The fact that I haven’t deteriorated, and I’ve got a lot of energy – I’ve got two kids, I work full time, so it’s given me that energy. It’s given me the confidence to go out and do things more and not worry about being too breathless, so it’s managing everyday life.

Even though I probably don’t feel it, I know it’s helping my heart and lungs. All my walk tests have improved and I’m able to do a lot more and have a lot more energy – like I said, I work full time and have both children. If I hadn’t gone onto the pump, I think I would have struggled.

It is good to hear such a positive response to treatment. Lots of people will have lots of different responses – and not everyone will have such a positive response as you; being able to work full time with children. But you talked there about how it reassures you, that even if you don’t feel that much better, it’s that certainty that the drug is working. So, it’s reassuring?

It definitely is. I know that I’ve got loads of energy and the pump is keeping me here. I’m here, four years down the line – it could have been a different story.

From your husband’s point of view then, four years down the line, does he continue to support you with this and help with questions / answers when you’re not well?

If I’m not feeling well, he supports it completely, he knows IV is really important. If I need to do something with the IV medication, or go into hospital because of it, he just takes over the kids – so I know that support network for the kids is there. I don’t need to worry; I can just go and get my treatment and come back.

So, he’s very supportive. He’s always asking if I’ve made my meds on time, or if he needs to help me make them. And the changing of the pump, and remembering, as sometimes I forget (I don’t know how, I just forget!) He prompts me and asks if I need anything, and if there’s a medication delivery, he’ll take that in.

He’s very, very supportive about the pump and he knows it works well for me. I think he’s seen a change in me in terms of not being as breathless. I don’t feel it, but he tells me. If I am breathless, he’ll ask if I’m ok and check in on me.

It’s interesting that you say you don’t know how you forget [to change the pump], but I think one of the things you’re describing there is how it becomes part of your life. So, I think it’s quite reassuring for people to hear that this can become a ‘normal part of life’.

Thank you Rinku, I’m really appreciative of what you’ve said and as I say, we’d love to come back and speak more – and maybe even get your husband on and chat. We’ll send him a PHA hippopotamus if he agrees, tell him!

You’ve given some very personal experiences of it which we’re really grateful for, but obviously everyone is different so people should use their specialist centres – the nurse specialist team in the first instance – to ask questions. Would you agree with that?

I definitely would. Speak to your PH centre. They are the experts – ask any questions, mention any fears that you have regarding this. Make an informed decision, but at the end of the day you’ve got to remember you’ve only got one life and you want to carry on. For your family and yourself really, it’s about making an informed decision about the IV. It’s not a bad thing; I’ve done everything I wanted to do and probably would have done even if I didn’t have the IV, but with it I’ve been able to do more probably.

I think that’s a really positive note to finish on, “with the IV I can probably do more”. Thank you very much Rinku.