You have to have it in your head that you can do it
When Karen Jones was diagnosed with PH after years of living with a connective tissue disorder, she prepared herself to juggle the many challenges the two conditions would bring. But with a penchant for finding ways to adapt, Karen is keeping control of both her physical and mental health. She shares her unique story with Emphasis.
“I was diagnosed with the connective tissue disorder systemic sclerosis in 1999, and balanced bringing up two young children with hospital visits and regular inpatient care.
The condition, an autoimmune disease that causes abnormal growth of connective tissue, caused painful ulcers on my fingers and toes and led to partial amputations of my fingertips. These days my hands are very sore and often bandaged, which along with my missing fingertips drastically affects what I am able to do.
Vascular manifestations of systemic sclerosis include pulmonary arterial hypertension, so as someone at risk, I was already known to the PH service at the Hallamshire hospital when I started to become breathless in 2002.
My symptoms were monitored, but it wasn’t really until 2012 when they started getting worse, and a right heart catheterisation showed the pressures had gone right up. I was officially diagnosed with PH.
I was absolutely devastated to be diagnosed with another serious condition, but at the same time it was a relief that it explained a lot, such as why I couldn’t walk very far, or get dressed very quickly.
As time went on, more medication was introduced. I was already taking iloprost for my sclerosis, and I now also take it for my PH – so it has become my friend over the years.
I’ve recently had to start taking it via a nebuliser, which I’ve found very fiddly because of the limitations with my hands. I had to stay in hospital for a few days to learn how to use it and the medical professionals were concerned I wouldn’t be able to because of my fingers. For me though, I saw a nebuliser as a much better alternative to a Hickman line, so I was determined to find a solution.
One of the things I found difficult was breaking the ampoules (the capsules containing the iloprost), but I used Google to research what could help, and found that you could get little cutters, which made a big difference. The internet is great in terms of helping me find handy solutions and ways to adapt.
It’s not always easy to do things independently, but it’s important for me to try. A nurse even once jokingly called me ‘Miss Do-It-Yourself’.
I believe though that the best person to manage your condition is yourself and for me, it’s about self-preservation as much as anything else. It’s about recognising that something will probably be difficult, but trying to do it anyway.
When it comes to overcoming hurdles, you have to have it in your head that you can do it. I try and concentrate on what I can do, not what I can’t.
One of the challenges for me is when plodding along on an even keel I can think things are under control, but then something will change with one of the conditions and throw new challenges up. So, I may have finished dealing with one thing, but I then have to find new coping strategies for another.
There are positives though. There is so much patient information available for both of my conditions and I’ve found the internet to be very useful, as well as various helplines. It’s good to know there is help there when you need it.
I also feel lucky that the healthcare professionals involved in each of my conditions are all so committed and helpful, and I receive great care wherever I am.
I live near Wigan with my two sons, Thomas, 24, and James, 19 and they are a big support to me. I was divorced in 2007 so it’s been me and my boys together for a long time.
My youngest is currently at university, but he chose a local one so he could stay at home, and my eldest son is a musician, so although he is often out at night, there is always one of them around.
I use the nebuliser seven times a day and it needs washing and cleaning too, but the boys assist me with it and help me keep on top of medication deliveries. They are happy to push me in my wheelchair too.
It has all definitely had an impact on their lives though. Over the years, I’ve missed birthdays and Christmases, and always had to rely on other parents to give them lifts. There is a lot of guilt that comes with these illnesses.
In October, I started having morning carers coming in to help and that was difficult to deal with at first, but I’ve accepted now that I need the help.
I can’t walk upstairs, I can’t get dressed easily, and I can’t decide to simply go out for a stroll. I’ve lost freedom of movement and that has been particularly challenging psychologically. Disease progression is tough to deal with too.
But there are little things I do that help me cope, such as planning. I write things down a lot and when my fingers hurt too much to write or type, I use voice technology to dictate what I want to say.
I find it especially useful to write down when I take my medication and I would encourage others to do the same. As well as helping me keep track it also helps me feel in control, and I write to-do lists for the same reason.
I like routine, as it helps when the rest of life feels chaotic with hospital visits and changing medications.
I’ve visited the transplant unit at Wythenshawe Hospital twice but because of my sclerosis, they don’t think I will do well. Although I’ve not had a direct ‘no’, I don’t think transplant is an option, so it’s important to me that I continue with my current PH treatments with a positive mindset.
I’ve had some clinical counselling sessions which have helped, but ultimately, I’ve realised it’s down to me, as the manager of my own mental health.
Even when faced with something horrendous, I believe we all have it in us to be positive in the end.”