Adenocarcinoma with unknown primary - Dave's NET story (part 1)

Adenocarcinoma with unknown primary - Dave's NET story (part 1)

My name is Dave and I am currently 66 years old. I have been married to Cherryl for 43 years and have 2 adult children. – My first diagnosis was in 2008. Prior to diagnosis I was a practicing professional engineer/commercial manager, a graduate of Auckland, Christchurch, New South Wales and Oxford Universities, a member of the Institute of Professional Engineers, and of the Institute of Directors. 

At the time I was on contract in Wellington, but my home was Tauranga.

In December 2008 I suffered severe shoulder and chest pains, after exercise, and was admitted to Wellington Hospital. Initial diagnosis from ultrasound - “multiple lesions in liver”. After numerous tests and a punch biopsy in Tauranga, this was re-classed as AdenoCarcinoma of Unknown Primary”.  The largest lesion was 135mm diameter.  The condition was deemed inoperable and I was given a life expectancy of 3 months. 

I accordingly took leave from work, and started to put my affairs in order, while undertaking tests and treatment.  Having come from a technical background I was (and still am) only interested in science based remedies, and treatments based on logic.  I have been appalled by the number of money making quackery remedies promoted to cancer patients. 

My initial reaction to the diagnoses was disbelief, denial, and worry. For the next 2 years I went through a period of mourning over lost opportunities, missing family. Tears were never far below the surface. 

I was referred to oncology who put me on a chemo routine of 6 rounds of ECX over a 5 month period.  The chemo required a full day of infusion, after which I would feel good for a couple of days (steroids ?) and then would go into a “black hole” for 3 days. This was like a very bad flu, during which I would be incapable of any mental activity. Side effects included loss of body hair and fingernails, nosebleeds, cramps, headaches, hiccups and headaches.  I did not experience predicted nausea.  On my son’s advice my wife provided a high protein diet to overcome the destruction and healing process triggered by the chemo.  At the end of the chemo program, a CT scan showed no change to the liver lesions. 

I was then referred (in the absence of any alternative) to try, at my expense, a new technique, SIRT, which was being carried out on a trial basis by a Wellington surgeon. His procedure required an initial surgery to redirect blood vessels to/from the liver and to implant a ‘Portacath’ for subsequent injection of the SIRT treatment.  During the course of this procedure, the surgeon found the primary, being on the exterior of the duodenum. He was unable to excise this, and conducted a jejunostomy to allow unrestricted flow from the stomach. His biopsy resulted in my diagnosis being changed to neuroendocrine carcinoma.   

Following the SIRT procedure I had an infusion of 5FU for 1 week every 4 weeks, for 12 months. This was fed into the Portacath, from a pressure bottle which I carried in a bag hung from my waist. Six months after this treatment was completed, the Portacath blocked. 

Despite having now survived more than 12 months, my life expectancy was unchanged at 3 months.  It was explained to me that eventually the cancer would overwhelm the liver and death would result from ascites. This could happen in a relatively short time frame.  With the continuous medical treatment, the desire to be close to my family, and the unchanging prognosis, I did not return to work over this period.   We sold our property in Tauranga and relocated to Wellington to be close to family. 

Having beaten the odds to this point, I felt uncomfortable about the primary still being in place.  While my demise would be more likely from liver failure, I felt that the SIRT had slowed the liver lesion growth, and that a major risk was in the primary seeding other metastases.  An exploratory gastroscopy could not pass into the duodenum and it was suspected that the primary had grown to the point where it was causing an obstruction.  After taking conflicting advice from a number of specialists, I decided to have the primary removed. This was done at my expense, in a private hospital. The surgeon conducted a partial gastrectomy, which to date has not resulted in any unmanageable side effects. 

One consequence of the procedure is “dumping syndrome”. With a stomach volume less than half of previous, if I overeat, some 4 hours later I get sweats, nausea and a feeling of general malaise. This has been explained as a type of shock.  Not pleasant and one I have to be careful to avoid.  

On recovery I tried to resume work, but found that my previous position was no longer open. As a further insult, the Institutes of Professional Engineers and of Directors, deemed that having been out of the work force for some 2 years, I no longer satisfied their Continued Professional Development criteria, and thus lost my practicing qualifications. Neither of these professional bodies has a mechanism in place to enable someone who for whatever reason has had to step aside from their career for a period, to re-enter, without going through a development period. At my age, no company would employ an unqualified engineer/manager nor would they employ anyone with a limit on their potential term within the company.  I felt it would be unethical for me not to disclose my condition at interview, nor did I feel that my commitments to treatment would enable me to fulfil the time commitment obligations of a senior position. 

On recovery from surgery, I again went under the treatment of oncology and started a monthly injection of Octreotide.   At this time my life expectancy was reassessed as “conditional survival”, which was explained that I had reached the limits of statistical data, and “the longer I lived the longer I was likely to live”. 

I have now been on octreotide for 4½ years.  During that time I have had CT scans at 3 month intervals which have shown variable outcomes of growth/regression, but all within the bounds of measurement. On a 4 year scale, there does not appear to have been any appreciable development of the cancer.   I have a small part time job, and have relocated back to Tauranga for lifestyle and better climate.  

Throughout this period I have been largely free from incapacitating symptoms, but I do get breathless and tired after exertion, and am subject to peripheral cramps particularly at night. I do get a few symptoms consistent with carcinoid syndrome, but not sure if this is from the cancer or from the octreotide. My weight has steadily declined from close to 90Kg to around 82Kg today. 

My current life expectancy is unknown, but if trends are continued I will live forever! 

Dave wants to highlight:…

• The importance of hope
• Throughout my journey we have been through cycles of
• - despair with the initial diagnosis
• - hope of a successful outcome of chemo
• - despair when the chemo didn't work
• - hope when I was given the opportunity to have SIRT treatment
• - hope of future and better treatments.

Without hope, it is very difficult to face the challenges which the cancer brings. For me it is important for to always have a forward plan, and a "plan B" in case the current treatment is not effective. If the oncologist fails to communicate his treatment plan, or appears to not have one, it is easy to fall into the trap of despair.

Living with cancer.

Now that NET is better understood, I am no longer dying of cancer.  Instead I am living with cancer. I try not to allow the cancer to dominate my life, even if it is the elephant in the corner of the room. I try to continue with normal activities and not allow the cancer to dominate my thoughts and actions. Many of my friends have no idea I have a NET.