Gene Stutter

legalities etc

2010-08-12T23:24:00.001+01:00

Erm, not sure how to say this but it would appear that we have explored all orthodox medical avenues and they have turned into cul de sacs....

Sutent is obviously not working so perhaps it's time to explore other avenues.... which of course may also turn into cul de sacs....

Would I do this if it was me with phaeochromocytoma?
Would I be googling and exploring, looking for a solution, a prolonging of life?

Somehow I think not.... but it's not me... it's the man I love and I don't want to see him suffer.

So check out Pam's story ( http://lifewithpheo.blogspot.com ) and tell me what you think... Am I grasping at straws ? Could marijuana lower Ian's hormone levels?

Is medicinal weed available in the US the same as the weed we could buy (illegally) in the UK from someone who grows it in his basement/shed etc...?

There are ways of "smoking" weed without the tobacco - vapourisers for instance - which are easily available, but getting hold of the weed itself is a different matter. How can I be sure of a quality product with the appropriate levels of THC? What if it has been sprayed with chemicals or something else?

So many questions, so little time...

The priority now is maintaining hormone levels within acceptable limits. Adrenaline levels of 40000+ is not acceptable. My main fear is CVA (a Cerebral Vascular Accident or stroke) I don't want the man I love to be crippled, reduced to a vegetable, immobile, incontinent and unable to express his needs. Of course this may happen anyway but it's a Hobson's Choice when you are faced with watching your loved one either deteriorate from cancer, have a heart attack or suffer a major stroke...and medicine can keep you alive for a long time in a debilitated state these days...

I've always had a "living will" and a "death plan" my nearest & dearest are aware of so my life is not prolonged unnecessarily. I don't want any heroics should I personally suffer a major event but the big lad doesn't have this in writing and legally I could be in big trouble if he became debilitated and chose to end his suffering, despite knowing he really wouldn't want to endure the humiliation and despair severe & prolonged illness can cause...

So, what to do?

Decisions, decisions, decisions...

Comments welcomed......

Love, Life & Complications...

2010-08-10T00:44:00.000+01:00

Over the last few months Ian has been feeling well. He has gained weight and the drop in his adrenalin levels have vastly improved his general wellbeing & mood. With funding for Sutent (Sunitinib) now being provided by Pfizer the worry over funding/money/battling the unfairness of the NHS has ended. It was a huge relief and enabled us to regain our lives. We found each other again and remembered why we had fallen in love all those years ago.
I've learned to live in the present, not worry about the future and enjoy each day. Ian has got his life back and is no longer dominated by cancer.
Unfortunately recent tests have shown an increase in adrenalin levels again. Back up to 42,000: higher than they were pre treatment with Sutent. Although he is feeling OK and we are maintaining a "Que Sara Sara" attitude there are many nights I don't sleep. Despite the reassurances from the consultants that the tests aren't always accurate and the fact that Ian is feeling well I can't help wondering if Sutent has ceased to be effective, or another "phaeo" is happily proliferating in a hidden corner of his body somewhere.
So we are spending time with friends, enjoying the often bizarre local summer festivals and Derbyshire customs. Ian is applying for tickets to every game at Old Trafford (the only place I am allowed to sing in public!) and we are hoping to see the comedian Ross Noble and possibly John Simm in Hamlet in October.
Whatever happens now my job is done. I have saved Ian's life many times now: through a combination of luck, recognising symptoms, providing appropriate care and juggling his medication. Debra, Claire, Elaine, Gill & Robyn amongst others saved me too and got me through the most despairing times.
I'm not a great believer in fate, nor do I have any faith in deities but I wonder if my early experiences in life gave me the resilience and tenacity to fight the system, manipulate the media and give Ian the best chance at life.
It's been a year now since Ian was literally dying. We've worked hard to strengthen our relationship and I'm happy we have had these 15 years of laughter & passion with all the ups and downs and have got back to where we were; the first night we met when I knew he was "the one". Many people go through this life without ever finding the love we have shared. We've been lucky. So I raise a glass to Doris Day "Que Sara Sara" (Whatever will be will be)...

And so it all began...

2010-08-10T00:12:00.000+01:00

With a stutter in a gene...somewhere remote probably, a fluke occurrence somewhere between interphase & mitosis at a guess. Maybe it occurred pre birth, maybe later something somewhere in the complexity of human development went haywire. The how or why isn't really important. What is important is dealing with the here and now... and so our "cancer journey" begins

Update: November 2009

2010-06-21T22:43:00.004+01:00

Ian's hair has gone white and after 5 days of his 3rd treatment with "Sutent" the side effects have kicked in: fatigue, pain, erratic blood pressure & temperature. I have been busy knitting and hope to put up more photos soon - the photos etc. take longer to do than the knitting! Over £100 has been raised for the Pamela Northcott Fund. Many thanks to everyone for their support. Watch this space for new funky wonky chunky hats !!!!

Sutent works! December 2009

2010-06-21T22:42:00.005+01:00

After a devastating 2 yrs we have finally had some good news. Many of Ian's tumours have reduced and many have disappeared. Many thanks to everyone for their support, we couldn't have achieved this without you. However, this is only one of many battles before we hopefully win the war. Wonky Knits has been busy knitting for family members but aims to open an Ebay shop or similar in the new year.... watch this space....

update: December 2009

2010-06-21T22:41:00.002+01:00

Ian is now taking his second course of Sutent (sunitinib) for the treatment of phaeochromocytoma and the initial test results have been extremely promising. His life threatening symptoms have more or less disappeared but the side effects from the drug have been quite severe. A scan in December will hopefully show a reduction in tumour size and/or tumour numbers. As far as we are aware Ian is the first person in the UK to use the drug Sutent for this particular type of rare cancer. It is used successfully in the NHS for kidney cancer patients but because Ian's condition is so rare the NHS refused to fund it. The drug costs £3610 for 28 tablets and because we are paying privately for it we also have to pay for consultations and associated tests (£440 each).

Thanks to everyone who helped with the fund-raising for Ian's Appeal Fund which has now ceased.

Proceeds from the sale of "Wonky Knits" will continue to be donated to the Pamela Northcott Organisation who help people like Ian who have to fund their own cancer treatment in the UK.

Pheo update: April 2010

2010-06-21T22:38:00.000+01:00

As Ian's website is now closed (thanks to David for providing & managing it for 8 months) I thought I should post on here where we are up to and why Wonky Knits is raising money to help people like Ian who have to fund their own treatment in the UK...

Ian has now been taking Sutent (Sunitinib) for his non-genetic "malignant phaeochromocytoma" since September 2009. He takes a 50mg tablet a day for 4 weeks then has a 2 week break. So far the CT scans have shown a reduction in numbers and size of the tumours in his lungs.

The side effects are tolerable; different but no worse than the side effects the other drugs he is taking to control his hormone levels (the tumours produce excessive amounts of adrenalin & various other hormones), blood pressure and pain.

We are hoping to get the application for funding in by the end of April 2010 and also hoping it will be agreed this time. We have evidence now that Sutent is working for Ian so we think Derbyshire County Primary Care Trust should fund it. It is expensive: £3610.00 every 6 weeks at his current dose but if he continues to improve the dose may be reduced so the drug will be cheaper or may be discontinued completely, hopefully along with all the other drugs he is taking. So we await the PCT's decision on how much a man's life is worth......

Its just a ride...and a rant

2010-06-21T21:38:00.000+01:00

On the 27th May 2010 the NHS meeting was held that decided whether Ian lives or dies. Of course no-one within the organisation had the decency or compassion to inform us of the outcome, we had to phone the following day, it was 9 working days before the paperwork arrived and again required a phone call to request it. Ian had been vomiting for 2 days, which he rationalised as "a bit of dodgy fish" but I think it was stress. The atmosphere crackled with tension. Our son sensibly departed to his girlfriend's house, perhaps sensing any slight misdemeanour on his part would cause WW3.

I consider myself a liberal pacifist but at that time I could understand what drives people to snap: to jump off a bridge or drive their car at 100 miles an hour into a wall; or lash out at someone who dares to cut in front and nonchalantly steals "my" parking space. If this was America I could empathise with the kids that take their parents' guns and let loose in their school. The tension is unbearable.

This is what no-one in authority, or anyone who has never had to put their lives literally in bureaucrats hands comprehends. Not only do we live with cancer and the very real threat of loss on a daily basis but we have also to cope with inequitable, unjust and infuriating systems that measure our lives in terms of cost analysis, algorithms and acronyms. QALY = quality adjusted life years - will this expensive drug give us value for money? Will the patient (an anonymous, faceless entity) live for more than a few months with the drug than without it and is a few months worth the investment. In many cases "no". The patient regardless of age, current health and well being is not valued as an individual, a human being, but in terms of cost effectiveness. He/She will die anyway, everyone does, so why prolong the agony? Why give him or her the opportunity to have some normality, to take a holiday, to say goodbye, to make his/her peace with the world?

"We have to make some very difficult decisions" the bureaucrats state, I doubt "public health consultants" or "chief executives" ever lose sleep over the fact they have sentenced a human being to an early, agonising and untimely death because they have ensured that the money saved will provide lots more 360 degree analyses for the managers to evaluate their performance and pat themselves on the back. 3000 hours paid work for managers at a conference centre (expenses paid), consultancy fees for the organisation that ran this event to produce a pretty folder for each attendee that will end up collecting dust in a drawer. (RANT, RANT, RANT)

Ian is watching inane TV, unsure how to cope with my impotent rage at the injustice of the situation we find ourselves in. So I rage quietly in writing and listen to Bill Hicks on the headphones, immersing myself in righteous anger and pouring another glass of wine. As Bill says, "It's just a ride" but right now it's just making me nauseous.....

NHS says NO! 28th May 2010

2010-06-21T21:26:00.001+01:00

Despite substantial evidence of clinical effectiveness NHS Derbyshire County have refused to fund Sutent for Ian on the grounds it is not "cost effective". There is nothing further to say.

Sutent supplied at last...21st June 2010

2010-06-21T21:25:00.002+01:00

Pfizer have agreed to supply Sutent for Ian indefinately. Thanks have to go to all the teams at the Hospital who worked so hard to achieve this. I imagine it's been an administrative nightmare as well as taking up a great deal of the Consultants' time.

As much as I would like to appeal the NHS denial of funding and humiliate the PCT for putting profit before patient care I simply don't have the energy. We'll probably still have to pay for the tests, consultations etc. but £400 is much easier to find than £4000 every 6 weeks.
It is a huge relief and I now need to recuperate and regain my own health and sanity. Its been a massive learning curve and an immense battle.

Many thanks to Kate Spall at The Pamela Northcott Fund for being an invaluable resource and a real inspiration and support in the darkest of times. Also to Rose & Julia at the Kidney Cancer Support Network and all the other "pheo" survivors throughout the world who I have relied so much upon for advice. Friends and family - you know who you are - xx

Scans, graphs etc

2010-06-21T21:24:00.000+01:00

Ian's graph & scans showing significant reductions in catecholamines (the hormones that his tumours produce) since starting Sutent. The scans also show reductions in tumour numbers and sizes.
Hoping to get back to knitting soon !