by Robert Knutzen, MBA, CEO Pituitary Network Assn.
As you can imagine we read a great deal of medical information here: studies, articles, conclusions, opinions, patient's perspectives, physician's perspectives, etc. What is puzzling, however, is the absence of clear definitions regarding numbers of patients, diagnosis, time of onset, seriousness of complications, etc.
We travel the world for information on treatment and options and perspectives. Sadly; while there is so much progress and sharing of good information on some fronts, it is woefully inadequate and antiquated in others. Clearly there is not one view of acromegalics; we are perceived, not as we really are, but by some as something vaguely remembered from medical school or an old medical article that no one has bothered to update or change in many years.
Allow me to present some facts and translate them into easily understandable language to all of us. Here is the link to the study we sponsored. It is actually a meta-analysis reviewing all pertinent data and studies on pituitary tumors, world-wide over the past 60 plus years. Read it for yourself by clicking here: http://www3.interscience.wiley.com/cgi-bin/abstract/109085480/ABSTRACT.
Though we did our homework it is apparently difficult for many to shed the beliefs of years past, and they have a problem translating actual numbers into usable data. So, here is my try at it. (This corresponds to the population of any country in the world.) But let me begin with the U.S. translation.
In a population of 300 million, we know that 16.7% - by a very conservative estimate - are known to harbor a pituitary tumor. (There are lesions, cysts, and diseases of other kinds, but we'll stay with pituitary tumors for this purpose.) Of this number, 2.8% harbor growth hormone-secreting tumors. (This is also verified in many studies and laboratories.) So: 16.7% of 300 million = 50,100,000 (fifty million) total pituitary tumor patients. 2.8% of that number (yes, we are few by comparison) = 1,402,000 (one point four million) GH-secreting tumor patients, (acromegalics). Divided by 300 (million) that equals a prevalence of approximately 4,676 acromegalics per million. Allowing us an average lifespan of 40 years after diagnosis, we find the annual incidence to be approximately 116.9 patients per million in the population. It is reasonably safe to say this is the percentage worldwide. This is a very far cry from the current mantra that acromegaly is very, very rare and only accounts for 3 per million of population. To arrive at that conclusion you have to believe the following: All acromegalics are diagnosed at the same time, with the same symptoms, and looking just the same.
Now, we know better than that!! I personally know many acromegalics with barely discernible signs of acromegaly and some with no signs at all. I also know those who went undiagnosed for so long, they are sadly deformed and have faces that clearly brand them as acromegalics. However, the medical facts are that acromegaly is an insidious and very invasive disease, taking years to fester and grow, presenting many varied and troubling symptoms and disorders to patients many years before a diagnosis. Who knows how many bilateral carpal tunnel syndrome patients are operated on before a diagnosis of acromegaly is made? Often heart problems, or even excessive tongue size or sleep apnea are treated long before the primary culprit (excessive GH) is identified and the offending tumor is treated or removed.
In country after country, the U.S. included, endocrinologists and surgeons open their presentations on acromegaly to other medical professionals or students with pictures or illustrations of highly disfigured patients who were not diagnosed in a timely fashion. They really are a testament to medical failure, not to medical excellence. My daughter, at six years old, could have identified a late-diagnosed acromegalic at that stage. No medical school needed! I have met many of these patients. They are often educated people: lawyers, university professors, etc. (meaning - they regularly see doctors for check-ups, etc) and still they are paraded about by their treating doctors and surgeons as though they were the find of the century!
Let us pretend for a moment that these patients are like pregnant women. They all go through many stages, in every week, month and trimester. Like a baby-to-be, the pituitary tumor starts off invisible to the naked eye - and grows! Fast or slow, it makes no difference. The tumor is born, GH secretion manifests itself and an acromegalic is born. As a woman is pregnant in her first week, so someone growing a tumor is an acromegalic the first week of GH excess! There is no such thing as being a little bit pregnant. You are or you are not! So it is with acromegaly, you either have it or you don't! The stage you are in may be important, but it is not the determining factor in whether or not you have a GH-secreting tumor. Only time will tell how fast it grows, only you know how it affects you.