Ten years of PH: Alex’s story

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This interview was recorded in September 2021

Alex, it’s good to meet you. I know you quite well but for the sake of people listening, could you please introduce yourself.

My name is Alex, I’m 36, 37 next month. I have been diagnosed with pulmonary hypertension for 10 years now. I live in Leicester and work part time, fortunately for me, from home. I’ve got a 15-year-old son that takes up a lot of time. That’s me!

Alex, I know that you’re involved with the PHA Facebook page, and you’ve seen new people being diagnosed with PH over recent times as well as in the past. I think one of the values of this conversation is to let you explain how you manage what some people are going to start to experience or are already experiencing. To give some context, could you tell us what the path to your diagnosis was and how it happened? The good, the bad and the ugly.

Finley [Alex’s son] was born in 2006 and at it was the start of 2007 when I experienced my first black out. From then on, it gradually got worse in terms of my breathing, and I was struggling because, at the time, I lived in a two up, two down so to getting up the stairs was difficult. Also, from having a new born, I put everything down to being exhausted from that. I kept going to the doctors and trying to explain my symptoms which was shortness of breath, I couldn’t keep my eyes open because I was so tired all the time and I was starting to swell up as well. At the time, the doctor put my swelling down to a medication that I was on which was a contraceptive pill. The doctor also put the fact that I was so exhausted and struggling with my breathing down to panic attacks and anxiety.

You raised a really important point there. A lot of people have been labelled with panic attacks and anxiety, particularly, young females and young mums. How did you handle that?

My partner at the time had quite a bad accident which resulted in a broken back and various other injuries. He was in hospital for eight weeks. By the time he came home and learnt how to walk again, it made sense to me that perhaps I was having panic attacks, anxiety and felt exhausted because I had an 18-month-old and had to experience that situation. To me, it made sense. I thought to myself, yeah of course I have, who wouldn’t be with what I had on my plate back then? To be honest, I think I just accepted it.

What was the prompt to get the diagnosis? What was the crucial stage in all of this?

All of my symptoms and going back and forth to the doctors continued until 2011. At that time, I was with my, now husband, Ben. My whole body was swollen, and I couldn’t even walk, I couldn’t get from one room to another. My son was supposed to be going to a birthday party and I was trying my hardest to get him ready and Ben just said to me: “It’s no good.” [because I couldn’t do it]. He said: “We’re going to the Leicester A&E and we’re not going to come home until it’s sorted.” Off we went and it when on from there. I didn’t come home then for six weeks.

It was a long build up to that point wasn’t it. If you had that time again, would you have done anything sooner? Do you think something else would have prompted sooner?

Yes definitely. We look back now, and everyone says why did we think ‘that’s just Alex’. It sounds awful to say but I think everyone thought that I was lazy, and I didn’t want to do anything. So now we all look back and think why nobody said, ‘this is not right Alex’, just get to the doctors. I think I am one of those people who tries to get on with it no matter what. Like I mentioned earlier, I think if the doctor told me that it was anxiety and panic attacks, back then, that’s what I believed because of the situation that I was in. I definitely would have gone to the doctors sooner. I did go to the doctors, but I would have maybe pushed for more tests or asked to be referred. Obviously, what was going on at home was not normal, every time I walked up the stairs, I passed out and I couldn’t bend down to put clothes in the washing machine without thinking that I was going to collapse. When I went into hospital, they drained 12 litres of fluid from me so that’s how swollen I was. It’s ridiculous really.

Can you remember the time when you and Ben were told that you had PH? What was it like?

Vaguely, it’s all a bit of a blur to be honest. I think part of me was relieved that I knew what was finally wrong with me and that there was a plan in place to, hopefully, relieve my symptoms. Obviously, there’s the other part of you that’s petrified because Finley was just turning five. When you’re having all of that information given to you, it’s hard to absorb it when all you are thinking of is your son and everything else.

Can you remember what the initial questions you had in your head were? You’ve got this PH, which is a very rare condition, was it about the future? What sort of questions did you want to be answered fairly quickly?

My future was all that I was bothered about at the time because Finley was so young. We had planned to have a bigger family, but we were then told that wasn’t really the path we could go down. I knew then that I just wanted to know exactly how my future would be for Finley’s sake.

Were your conversations with Ben easy to have? Obviously in a couple, there is a lot of support there. How did you approach those conversations together?

I think Ben is a very positive person anyway and he will always look for the positive in things. To be honest, our conversations were quite easy because we knew that we would get through it together. I knew he wasn’t going anywhere and that we were going to get through it.

What about the rest of your family, like your parents, who don’t expect their daughter to be unwell? What was it like approaching conversations like that?

To be honest because I had been transferred from Glenfield to Sheffield, with us living in Leicester, my family did spend a lot of time going backwards and forwards. When we did have the big chat, they were all there and wanted to be there for us and find out exactly what was going on. A lot of the information came from Dr Elliot who explained it to all of us.

As time has gone on, what about the conversations with your son about this? I know parents sometimes struggle to know how to have these conversations with their sons and daughters. How have you approached that and how do you continue to approach that?

I haven’t sat down and told Finley the whole story. Sometimes, I do think to myself – should I? But from his eyes, because he was so young when I got diagnosed, this is how I have always been to him anyway. He’s never really known me any different. He knows I’m not well and knows about the situation and the disease. In terms of sitting him down and giving him the full works, we haven’t actually done that.

You probably don’t need to. He’s lived quite openly within that. Listening to people through my day job as a nurse consultant or through the PHA, some people find themselves tied up in knots – with the best intentions – trying to protect their sons, daughters and grandchildren about PH. They act like everything’s ok and there is nothing wrong but, fortunately, we live in the world with the Internet. I have heard of a number of parents who firstly said that they tried to protect their children from good or bad news but then the children find out about it online. It sounds encouraging that you have been open and it’s about living with it and getting on with it in your family life.

That’s it. We are a very open family, and he knows that he can talk to us about anything. He’s never known any different, this is just how I’ve always been. He doesn’t actually have that many questions anyway.

If you were to meet a newly diagnosed individual with PH, from your experience, what are the nuggets that you would want to share with them about how to get on with life?

First of all, I think the main thing is to take the time to absorb everything you’ve been told. There is no rush to get your head round it. Everybody deals with things differently. If you do have any questions, it’s a good thing to speak to someone. I know that you guys at the PHA are always at the end of phone but also the Facebook group is really helpful. I think it is important to speak to patients so that you have got real life experiences rather than going to Google. You can put anything into Google, and it will tell you the worst news. There is always a positive in any situation. Just go with how you are feeling, there is no time span in order for you to realise how things are going to be.

You raised a really good point there; it’s the peer support. I know, from a clinical perspective, we can give advice but we’re not living with it. You’re the experts at this, you’ve had ten years of living with it 24/7. The peer support should not be underestimated at all. Like you said, there is a lot of good within places like the Facebook group. You can go and watch and read things and you don’t even need to take part in it to be reassured about how to do things. For example, having conversations with parents and children, finding out what others did and didn’t do, the same with working and not working. When you were right at the beginning of your diagnosis, what was it that you needed to know to get on with things? What were the things that you wanted to ask the questions about the most?

It sounds quite morbid, but it really was all about my future. I went into panic mode about how long I thought I was going to be here because I wanted answers for that. No one can tell you answers for that because every person is different. To be honest, I got the diagnosis and within a couple of weeks I went back to work. I think I was almost in denial. I got told all of this information, started my medication which made me feel so much better and almost felt back to my old self that I thought ‘let’s go’. I went straight back to work; Finley went back to school, and I think I just threw myself into everything. Like I mentioned earlier, I think you do need to take the time because its only now, ten years on, that I think to myself that I was a bit nuts for being the way I was back then. I should have slowed myself down.

You raised a point there about it almost sounding a bit morbid, but I don’t think it is. It is really important and healthy question to explore so we can have those conversations. As you said, there is often no exact answers to that, but you responded very quickly to treatments and that is really positive. How are you now overall? How impactful is PH on you?

Not very. I keep myself extremely busy, I like to be active. I think, looking now where I am, I have learnt over the years that now I can have a normal life with PH. I knew back then that it was never going to stop me. Finley, Ben and my family were my priorities, nothing was going to stop me.

Is it a case of working out how to do normal things but in a different way?

Yeah.

I think that’s why the ability to think these things through is important. You have to think about different things in a different way. You spoke at beginning about things you had planned with your family for the future, how impactful was that for yourself and Ben?

To be honest, it was one of the second or third things we were told after my diagnosis. To start off with, we put it to the side for a while. Over time, because I responded to my treatment very quickly and very well, it came back to the point that we thought we should look into it. It was one of those things. At the beginning when you get told, it didn’t seem as important as it first seemed because I had to deal with my diagnosis first. It wasn’t until we decided to revisit it and we got quite far along the path that we came to the point that we knew that it was not going to be for us. It’s a hard one. Finley is not Ben’s child, so I think, consciously, for me I automatically thought that we’ve got Finley so we’re ok. But then that puts Ben in the situation where he hasn’t got any children and it was tough. We made sure that we remained on the same page, like when I got diagnosed and we knew we were going to get through it together.

It’s encouraging to hear that because in serious illnesses, couples can really struggle sometimes. One of the things that is really clear from what you are saying is that having an open and, sometime difficult, conversations with your partner. The thing is to not not talk about it. Even if it is hard, you still need to talk about it, otherwise, these things can be a complete wedge.

Definitely, I think one of the most important things is to speak about it. You can make yourself ill by having all of these thoughts in your head until you say it out loud which can be a relief. Some conversations can be extremely hard and upsetting for days. Once you have spoken about it and everything is out in the open, you can deal with it and carry on.

Sometimes these things become little secrets that no one talks about. One person quite powerfully told me how it became their ‘dirty little secret’ because it was so difficult to talk about that they never talked about it. It is upsetting but we can work things through with that.

It is ten years since your diagnosis, happy anniversary. Are you going to do anything to celebrate the anniversary?

I did plan to but then with Covid, even though I would be outside at this time of year, it swayed me off a bit. I had a little get together with my family. I was hoping to do a big thing with my friends and family at home but unfortunately have not been able to.

What’s Covid been like for you? You’ve got PH so you fit into the shielding group. How has Covid been for you?

To be honest, in the beginning, I was absolutely petrified. Over time, I’m only just now starting to go out to places, and I still don’t feel comfortable. I think it has had quite a big impact on me mentally and feeling anxious about going out. Unfortunately, it has had an impact on me but, as always, I know I’ll get back to being my normal self, it’s just going to take a little longer.

I think that is very sound advice because a lot of people are in a very similar position to you in feeling anxious about getting back out and about. I think the very wise words are taking your time and learning how to do it. Yesterday, for the first time, I went to the supermarket and realised that I wasn’t wearing a mask and I normally do. It makes you question what you should be doing. I know it’s just a small example of getting back to normality, which might take time, and being respectful and aware that not everyone feels as comfortable with it.

That’s it, you have to do what you feel comfortable with. If you feel safer going into the supermarket with a mask on, which I do, then I will wear my mask. To make myself feel safe and happy, I do what makes me feel comfortable which is wearing a mask.

Thinking of the future, I know the treatments that you are on is oral medicine but has the possibility of transplant been raised with you?

I’ve been to two appointments over the ten years at the transplant clinic and I have got another one coming up in December. The first appoint was right at the beginning so it was extremely scary. I know it is a possibility in the future but, for me, I take things day by day. At the moment, with how I feel, and I feel great, I tend to shelf a lot of things. I don’t know if that’s a good thing or a bad thing but to put it to one side and not think about it works better for me to live my life. If that situation does come along, I am going to have to deal with it then. We will see, hopefully I am too well.

The transplant is always part of the PH pathway if your age is right. I think one of the other things is that it is a potential safety net for some people, rather than where you’re heading to. With ten years of experience from yourself, it’s about how to deal with it. You haven’t put it under the carpet, it’s just on the shelf and it’s there if you need it. Hopefully things will continue as you are, but the safety net is there.

Alex, I just want to thank you for sharing this time with us. It will be really good in the future for you and Ben to have a conversation with me again. I think it will be really good to have that conversation because we can do magical things in managing people with PH and clever treatments, but it doesn’t make you to get on with life, it just enables those things to happen. You have really demonstrated that, and we are really keen to build up this library of interviews of positive and honest experiences because it’s not easy. Thanks again Alex, we will speak to you again soon.