Paula

Age at interview: 46

Brief Outline: Paula was born with congenital heart disease. After an operation aged 3 her heart was healthy until her 30s. She has had a new heart valve and a pacemaker. She is constantly tired and breathless on exertion. She regularly travels 400 miles for check-ups with her original cardiologist whom she trusts completely.

Background: Paula is married with three adult children and five grandchildren. She worked as a funeral director before retiring on health grounds. Ethnic background: White British.

More about me...

Paula was born with congenital heart disease and had open heart surgery at three years old. She was expected to live only a few years but her heart was fine until her early thirties when she needed a replacement pulmonary valve. Five years later she was given a pacemaker, the battery for which ran out recently, so she is now on her second device. 

When she was young, Paula was advised by her doctors not to have children, but she went ahead and had two healthy sons; she now has a stepson and five grandchildren. She was also told she would never be able to work, but she has done, although she later retired on medical grounds. Paula believes in taking control of her own life and making informed decisions about risk taking and will deal with any consequences. She has smoked since age 15 and nowadays also smokes cannabis recreationally.

Over the years Paula has had problems with blood clots causing deep vein thromboses (DVTs), pulmonary embolisms, strokes and transient ischaemic attacks (TIAs). She has taken warfarin since her first DVT, apart from when she was pregnant, when she had to inject herself with heparin instead. After her heart valve replacement she was put on other medicines including bisoprolol, lisinopril, lansoprazole and atorvastatin. Eighteen months ago spirinolactone was added to the mix. She also takes thyroxine and hormone replacement therapy, having had an early and difficult menopause. 

Paula also has ‘drop attacks’, for which her doctors can find no explanation. She has always suffered with bone pain as a result of the open heart surgery, particularly when bending. Her tricuspid valve is regurgitating. She is constantly tired and gets breathless on exertion, which is worse in cold weather, but she tries not to let it affect her life and tries to do everything she can. Because she was born with a heart condition she doesn’t consider these things as symptoms but just how she is normally. She has achieved more in her life than she ever imagined she would, so takes each day as it comes. Her limitations have, however, caused her to feel sorry for herself at times; this was particularly the case when her children were young and she couldn’t join in with physical games, but with the help of therapy she has managed to come to terms with this.

Paula has continued under the care of the consultant who she saw in her 30s despite her having moved 400 miles away ten years ago. She and the doctor are of a similar age, have a good relationship and she has 100% trust in him and doesn’t want to see anyone else. She can phone his secretary if and when she has any concerns. Her local GP also has a good relationship with the consultant and they discuss any changes to Paula’s care. Earlier in her life Paula wanted to know everything about her condition and its treatment, but nowadays her faith in her consultant is such that she no longer asks questions, she just agrees to whatever he says is needed. Any decisions are taken through discussion with her and her husband. They see the cardiologist every six months, or more often if they have concerns. It takes them two days to drive each way. She has a thorough check-up and any extra tests that are needed are done there and then so she doesn’t have to return another day. She also sees the pacing team while she is there for a check on her pacemaker.

Because Paula lives in a very remote location she has been given a device to monitor her pacemaker from home. At a specific given time, she places the device over her pacemaker to download the data, which is then sent by satellite to the hospital. If there are any problems they will be dealt with by her local hospital where possible, otherwise she will be asked to see her usual team 400 miles away. Because she takes warfarin Paula’s blood clotting time has to be checked regularly and the dose altered accordingly. Paula’s mother bought her a home testing machine for her clotting time for her 40th birthday, which she uses once a week with finger-prick lances and test strips supplied on prescription. This has enabled her to keep her clotting time more stable than before because she can do it more frequently, no longer has to wait two days for the result, and with the blessing of her doctors, alters her warfarin dose herself. She also uses a home blood pressure machine occasionally if she feels as though her blood pressure has dropped below normal. She also weighs herself regularly and tries to keep her weight stable after having dieted to lose weight a few years ago.

Paula had surgery aged 3 for a congenital heart condition and was then well until needing a valve replacement and then a pacemaker in her 30s.

So can you tell me a bit about your heart condition?

I was born with congenital heart disease, Tetralogy of Fallot. It’s basically four problems wrong with the heart, which have been, were rectified when I was three. That was when I had my first open heart surgery. I had regular checks as far as I’m aware up until I was about 16 when I left home, got married, started my own family. Never had any intervention really from any cardiologist until I was in my early thirties. I started to feel unwell, passing out. Went to the doctor’s and said I needed a new valve replacement, which I was absolutely shocked ‘cos I really believed that the operation I had when I was three was like going for your tonsils out. Once that was done you were fit and healthy. I had a normal life right through school, had two beautiful healthy boys.

When I was in my early thirties I had a valve replacement done, which at the time I believed I would be in hospital, valve replacement done, out of hospital and I’d be back to being my early twenties, which didn’t work. I had quite a lot of psychological problems dealing with what had happened, which has all been sorted out and I went through therapy for. And I had my pulmonary valve replaced in 2001, it’s all been very successful, I have regular checks. I normally have my heart checked at least every six months. And then in 2006 I had a pacemaker fitted. And about 8 weeks ago I’ve just had another pacemaker fitted as that was running, that had run out of battery life, so they put a new one in, a bit of an upgrade apparently.

Paula found it difficult to come to terms with being on a transplant list but although now off the list she would be prepared to have one if offered.

Two years ago I did get word that maybe I would be going on a transplant list, that would be the next step forward. Thankfully my health has picked up and I am not on a transplant list, but there is always that, it is a possibility further on in life, which at the time I couldn’t deal with. I don’t want anybody else’s heart, I’m happy with mine. My heart’s got me to 46 and I’m proud of that. I don’t want another one, I’m quite happy with that. So I did go through another emotional time; I had fantastic family who supported me, talked me through it all. Two years on, even though I'm not on a list, and it doesn’t look like I would, I don’t need a heart transplant in the near future, but I have got my head round it, and if that’s the case, yeah, bring it on, let’s go for it.

Oh okay so now you would be prepared to have…

I would now, yes, yeah, yeah. I didn’t agree with it, I’ve always been very, we are born individually with what we have and we should all be thankful of what we have. And I did find it very very difficult to comprehend having some, somebody else’s heart to keep me going, because as far as I was aware, and as far as I was concerned, my heart’s done me proud, and I would feel like I was letting it down. It probably sounds ridiculous but everybody has their own emotional ways of dealing with things. The family sat down, my three boys, my husband, we all sat down, had a discussion about it. They all put their points of view across and, to be fair, they were all right because I chose to get married, I chose to have a family, and as a mother and a wife, and now a grandmother, it’s my responsibility to keep me here as long as possible to look after them. And if that means I need a transplant, I need more operations, then so be it. So yeah I’ve, that’s all sorted and thankfully, like I say at the moment, I’m not needing a transplant and I’m not going on any list, so it’s just keep ticking over and keep looking after myself as best as I can and live my life to the full.

Paula lives 400 miles away from her consultant but travels to see him twice a year because he treats her with respect and she trusts him to give her the best quality care.

I mean the medical profession have been absolutely fantastic with me, I can’t fault them in any aspect. But on saying that, they are not always right and they don’t always know everything, so it, it’s got to be, I think you need a really good relationship with whoever you’re with, that they understand what you, you know, how you know yourself to how they know you. I do think that helps.

I travel four hundred miles to see my consultant. I won’t see anybody else unless my consultant says, you know, he’s away or whatever, you, somebody else is going to be seeing you, and that’s happy. The pacing team are exactly the same, I see the same consultant and they’re aware that, you know, I do travel that distance for a reason, because in my opinion that’s the place I get the best care, the best quality, I, my husband is informed with everything that’s going on, I’m treated like a human being, you know, it’s not a Mrs So-and-So, it’s first name terms. I know his wife’s name, I know his children’s names, I know what his kids do, what they want to do when they’re grown up. It’s a very very personalised relationship. 

Paula has been weighing herself regularly since starting a weight loss diet 3 years ago; she is no longer dieting but keeps an eye on her weight.

I weigh myself at least once a month. That’s not due to my heart condition or, well yeah, no it is, it is, it is due to my heart condition. I have always been advised that I should stay round about 9 stone in weight, because of obviously the more weight you put on the more pressure and more stress you’re putting on your heart. I totally relate to that. I’m very small framed anyway and a couple of years ago, it would be about three years ago, I did get up to about ten and a half stone, which I wasn’t comfortable with in my own self, so we decided, my husband and I both decided that we would go onto a diet. So we went on a diet and we weighed ourselves every week. Again, on a Monday, routine, we weighed ourselves every week for 18 months and monitored our weight. We then didn’t come off the diet but gradually introduced things we shouldn’t be having: sweets, takeaways, the usual that people over indulge in every now and again, still keeping on the diet mainly. So I would say we regularly weigh ourselves at least once a month. 

Paula manages her blood clotting time (INR) using a home testing kit and adjusts her warfarin dose according to her measurements and what she eats and drinks.

Right I have a, a small machine which I can show you. It’s basically a finger prick, very similar to the people that have diabetes. You have a pen, a finger prick, you have a little strip, you put that, your blood on a strip, you put it in your machine and it’ll come up with your INR. 

The actual test strips I get on prescription, and the lances that go into the pen I get on prescription. So although the machine I had to pay for initially, I do get the lances and the test strips on prescription.

I’ve found that where, because we live in such a remote area, when I was getting my INR tested at the doctor’s, by the time it got to the hospital and the lab, it was all delayed, so by the time the, the specialist would ring me with my dosage it would be two days later. So I’d either stop my INR in between the test, the blood test and the results, or carry on taking it and then by the time I’d done the next test it was all completely up in the air. 

So I found that if I did it myself, I do it every week, like I say, on a Monday, and my INR has been in range ever since I’ve been doing it, apart from, obviously, when I’ve had minor ops obviously you come off your warfarin and, you know, if I’ve been ill or if I do, which I don’t drink, very very rare, but if we do have a family occasion, Christmas, weddings, funerals, christenings, and I do have a drink, if, I know what compensates and how to dose my INR, I know what foods alter my INR personally to me, so if we go out and have a curry, but we’ve had a curry maybe two or three nights on the run if we’re on holiday, I know that affects my INR, so I’ll just for those two nights I’ll maybe miss a dose, or instead of taking five milligrams I’ll maybe only take three, so that I can keep it. But I’ve been doing it for six years and I think it’s habit and you know your own body, nobody knows your own body like yourself. And it’s the best thing that I’ve ever done. 

Paula downloads the data from her pacemaker at a mutually agreed time with the hospital.

I generally get an appointment and what I do is I’ll download, if I get an appointment say 20th July, 9 o’clock, I’ll download my pacemaker, which is a very simple procedure. You just put a device on top of your pacemaker and it will record. The information will then get sent down to my hospital. If they pick anything up that needs, or they feel is not quite right or needs changing, depending on how severe it is, if it’s something that just needs a little tweak they will ask the local hospital to do it, which they’re quite happy to do, they do work in conjunction with each other. If it’s something a little bit more serious that, you know, that the specialist wants to see, then I will get an appointment to go down and, and have a check done there. If everything’s fine I don’t hear anything, as my Mum always used to say to me, “No news is good news”. So I don’t, I don’t worry about it, I just, if the appointment comes through I do it, send it down, it’s all done via satellite. If I feel that maybe my heart’s not in its correct rhythm or I’m feeling breathless or something’s not quite right, I can then ring the, the pacing team, explain to them the situation, and they’ll say, “Right, well if you do a pacing check within the next half an hour, we can check it and we’ll get back to you”. So it’s all done harmlessly, effortlessly, great. 

When Paula moved 400 miles away from her trusted consultant she tried seeing a local one but didn’t like him, so went back to the old one despite the distance.

What if your doctor four hundred miles away said, “It’s silly you coming all the way down here”, you know, “You should, we should transfer all your care to your local hospital”. How would you feel about that?

I would say, “Fine if that’s what you want to do, you don’t want me as a patient, that’s fine, but don’t expect me to see anybody else, I’ll deal with this on my own”. I wouldn’t see anybody else. It’s as simple as that. And the subject has been approached.  He did say to me, you know. Well, to be fair, when I moved from where I used to live up here to where I am now, there is a four hundred mile difference between my consultant, I did go to a consultant nearer by, which seemed like a good idea at the time. I had one consultation with this other consultant whom I was told that I was basically a hypochondriac, I’m not qualified to test my own INR and monitor my own health issues, blood pressure that kind of thing, so there’s absolutely nothing wrong with me and I need to get a grip of my life. So I then got up, said, “Thank you very much”, and walked out. I came home and I phoned my previous consultant who I was under originally,  spoke to his secretary and I said, “There is no way I’m seeing anybody else; I want to come back and see my normal consultant”, who I’d been under, who’d gone through the, the  pulmonary, the valve replacement with me and, and everything. And she said, “Oh there is proper channels we need to do, so we’ll go through the proper channels and we’ll see how it goes”. And then within two months I got an appointment to see my original consultant, and I said to him then, “Don’t ever, ever ask me to see anybody else because it won’t happen”. So that would have been ten years ago, nine years ago. I’ve lived here for ten years, so it would have been about nine, eight, nine years ago I went back to him.