Sunday, 17 March 2013


This last day or two I've been busy getting ready to go back in hospital to have an increase in my dose of my intravenous drug Epoprostenol. This hasn't been done for a while and the doctors want to try and keep me stable, there have been one or two signs of deterioration lately and they also want to try and increase my exercise capacity to give me a better quality of life. Although they are quite pleased how my heart is coping, it is still not working like a 'normal' heart with its irregular beat and its extra loud sounding beats and murmur and the fact that it's still dilated on the right side, it is having to work extra hard to keep up with me all the time. I still find it hard when I see this written in black and white and when the consultant says it's not like a normal heart, although I have been living with this knowledge all this time now. I suppose it's another of those things that goes into that little set of compartments at the back of my mind, so I can carry on going forward into the future without thinking too hard about it. So this increase in Epoprostenol is planned to help my heart cope and work better.

Crono pump
Epoprostenol is a prostacyclin drug which relaxes and expands the blood vessels and stops blood cells clumping together. It increases the survival rate for PH and is similar to the natural prostacyclin produced by the cells lining blood vessels. It is only active in the blood for five minutes so has to be taken as a continuous intravenous infusion twenty four hours a day. The delivery system is complex and requires you to learn sterile preparation, operating a pump and caring for the catheter, line and entry site. I change it every twelve hours, to leave it longer would be at risk of causing an adverse reaction, my heart would suddenly have the help it needs to work withdrawn.

All the bits, twice a day! The orange line is every other day
It is stored as a powder and it's a complex procedure involving needles and syringes and dilutant solution to prepare it. Sterile conditions are needed for this preparation as the drug enters the blood system and is directed straight to the blood vessels in the heart and lungs, before it also reaches all the other blood vessels in your body. So infection is also another major risk as well as interruption of the drug.

I have to carry a spare drug kit and pump with me at all times in case of emergency and an interruption to the pump and I have to check the catheter entry site daily for any signs of infection and report any unusual symptoms I may be experiencing in case it's due to infection. An infection getting into the blood stream can cause septicaemia, which is life threatening. So having this drug does bring its challenges, but being well prepared and knowing the signs for infection these risks can be minimised.

All the extra bits: keeping sterile, sharps bin and waste tray
For many people on this drug there are some side effects, amongst them: flushing skin, jaw pain, headache, nausea, diarrhoea, palpitations and rashes, but they usually settle down once you are established on the drug. If you are a regular reader of my blog you will know that the side effects I usually experience are unusually severe, so I am preparing for a rough couple of weeks. It usually takes just a couple of days in hospital to have an increase, but my past experience has been at least a couple of weeks and then a good month or two to recover. I know it is the right thing to do though and can only follow the recommendations of the experts, I also need to demonstrate to the transplant team that I will follow the medical advice given, there will be no 'shall I, shan't I' when it comes to post transplant drugs.

Dressing change time
Obviously I am not really looking forward to all of this and will be going into hospital with some trepidation, but I keep asking myself, 'what is the worst that can happen?' Well at worst it may be several weeks of being ill and a month or two to recover then I will be back on my feet and feeling better with the added bonus of being half a stone lighter and energised for summer or at best I might be pleasantly surprised and be out of hospital in a few days with a new drug routine and a spring in my step! So I have been checking and repacking my hospital bag and trying to keep all the household chores up to date so I'm organised for Tuesday.

It had been crossing my mind whether to ring ahead to Duchess Ward and give them a few weeks warning that I am coming so they can book their holidays while I'm in there. Some people are remembered for all the wonderful things they do in life, but not so me with the ward staff at Papworth. I know I am well remembered as the 'puking patient'! Even the lady who kindly brings the tea  round commented to me at Christmas how well I was looking compared to usual, on that occasion they hadn't increased the medication! Really though I am looking forward to seeing familiar faces more than ever, if things go badly they are warm and compassionate and treat me as family and they will help me get through it and get me home again like they've done more than once before. Because I've been through the minefield of Epoprostenol, the specialist PH nurse at Papworth has asked me a few times to speak to patients who are facing having the treatment, so perhaps I haven't done that badly after all.

Aside to all of this, I was brought back down to earth even more by a letter I received from the Transplant Team, along with all the other patients waiting for lungs. The letter explained about all the various types of lungs I may be offered: smokers lungs; lungs belonging to a patient with a brain tumour; lungs from an older person; lungs from a drug addict or someone with high risk sexual behaviour; organs from a donor following a cardiac death; organs that have been placed on a machine to improve their function before transplantation. Now all this sounds very daunting and also highlights what a chronic shortage of organs there is. This had already been explained to me during my transplant assessment, but it is hard to see it all noted down in one long list, but I have had to agree to have whatever can be offered, the alternative isn't much option. I also trust the Transplant Team fully  that they will check the organs are fully suitable before going ahead with a transplant and I'm not going to dwell on it any further, it's yet another thing that can go into one of those storage compartments I have in my head.

We did have a chuckle though trying to imagine different scenarios of receiving lungs from someone who had the whole range of problems, what would the chances of that be? Well as long as they are good strong lungs, I would be more than happy to accept the wonderful gift and be grateful for evermore!

It's a good job there is a lot of support around at times, support from other patients who are going through the same, there are quite a few of us and support from patients who have PH. On Saturday it was good to meet up with the Papworth PH Matters Support Group in the Papworth village library. This group is for patients and anyone else who has been affected by PH. We had some excellent speakers, the chairman of the PHA Association UK, who just happens to be a PH specialist nurse too and specialist nurse from Papworth informing us about many aspects of PH. We enjoyed a lovely buffet, raffle and a good chat. Everyone is welcome to the meetings, you do not have to be a Papworth patient and next time in June we are going to get a tour of the path lab at Papworth and see what they get up to with all those blood samples they like to take, we will also have a talk about all the different blood tests we have to suffer! We will also be having a talk in September from one of the PH research team from Cambridge University. The help, support and professional information we get from the group is invaluable to us patients, so thank you to all those who voluntarily organise everything for us. The dates for the next meetings are: Saturday June 22nd; Saturday September 7th; Saturday November 30th. The meetings are held in the Papworth Everard Library Complex and start at 12 noon, everyone is made really welcome.  

Well it's time for all guns blazing: dentist, eye testing and a visit from BUPA to service my medical pumps on Monday and warfarin clinic on Tuesday morning and by Tuesday mid day I will be checking in on Duchess ward at Papworth. Mr G is now in charge of updating my blog ...  

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