Living with a rare skin disease

David Kendall shares his story

David Kendall has Extramammary Paget’s disease (EMPD), a rare cancer-related condition which is characterised externally by a chronic eczema-style rash of the skin in the anogenital regions or as is the case with David, the groin area.

Doctors described David’s EMPD case as “one in a million” due to its rarity, with the result being that he has found it difficult to access definitive information about it online. Upon speaking with David, the British Skin Foundation has added some information on EMPD on our website here.

The 58 year old father of two and former EDF Energy worker from East Kilbride has decided to chat with the British Skin Foundation to share his thoughts on living with such a rare condition. 

How did you feel when you found out the condition you had was so rare?

It took a while to sink in; I have a scientific background so I have a natural feeling of needing to understand everything which was very challenging in this case. Having something so rare means that it can prove difficult to find others in a similar situation. Very recently I was invited to join a closed group of people worldwide who have various forms of the illness and are sharing their experiences, treatments & progress - this has really helped but is late in the day for me. One positive is that they only found me through my BSF fundraising efforts.

Who did you turn to for support when you realised there were no patient support groups for this condition?

I collated as much information as I could from the internet to get as detailed a knowledge of the condition as possible. I then worked with my various consultants to help me understand what was going on. My family doctor and the nursing sister at work were also excellent in doing all they could to help and reassure me. However, it would be fair to say that few people I encountered on my journey had come across the illness before.

How long did it take before you suspected that the rash you had was something more than just a rash?

Many months as I went through a number of treatments first, none of which improved the situation. At a works medical they suggested that I really needed to follow this up as it wasn't getting better & had started to spread. My GP referred me to a dermatologist & after an initial consultation a biopsy was carried out which determined that it was EMPD.

Do you find you are now able to deal with the disease now you know what it is?

I think I have dealt pretty well with it and have tried to live as normal a life as possible. I have been fortunate so far as it has been manageable to date. However, I now have awareness of the course the disease can take and know of others where it has developed into a very serious and in some cases life threatening situation. Research into the disease is limited as are the treatment options (which mainly revolve around surgery as there is limited experience of the successful use of chemotherapy). Whilst the external signs of EMPD are visible it is hard to determine if the disease has been removed without detailed postoperative analysis of the tissue removed – the first two operations failed to remove all the disease and the third resulted in a very small margin. I am now believed to be clear but I also know the recurrence rate is quite high so I remain in a monitoring situation.

What has been the hardest thing about having EMPD?

Probably the long periods of uncertainty as I went through a wide range of tests before the initial intervention was started. As I have a history of inflammatory disease, for example I have Crones disease, a number of checks of the extent of the EMPD were required before progressing to surgery which resulted in initial delays in treatment. Talking to people can also be a challenge. Getting biopsy results after surgery that showed it hadn't all been removed was also a difficult time. 

What advice do you have for someone who has just found out that they are suffering from a rare disease?

Try to stay as calm as possible and try not to overthink the situation. Become as knowledgeable about your condition as you can so that you are able to talk things through with your consultants. Try not to overreact to what you read - I have seen such a wide range of reports on individual or a few grouped cases but there isn't much of a definitive nature to reach a conclusion from. Remember that each case is different - you have to place your trust in others whilst having enough understanding to steer your course when you need to make an input. I have also found it helpful to live in the moment and try not to dwell on the situation even at the most difficult times - my family have been brilliant in helping me through all of this and kept me positive throughout.

Last year you took part in the Glasgow Men’s 10k for the British Skin Foundation, raising over £5,000 for skin disease research*. Do you think taking part in the challenge helped you to cope with EMPD?

The 10k really helped as it kept me focused during my illness. I have significantly improved my fitness over the last 5-6 years and getting ready for the 10k really helped get me through it. I have had feedback that the high resilience levels I have built up over the last few years have contributed to my recovery. Following each of my 3 surgical procedures I had to take a break from strenuous exercise but was back at the gym starting to slowly build back up after 3-4 weeks. BSF has been an ideal partner to me as they have really helped with the awareness raising and I am very grateful for their support.

Thanks for chatting to us David.