Friday, February 9, 2007

Acromegaly, Andre the Giant, and me!

Why I'm Talking Now
I have a very rare disease called acromegaly. The reason I'm posting about this is there's very little information regarding personal experiences with acromegaly. I feel like I have to put myself out there to offer information on the experiences I've had. And I would like to know more about how this will affect me in future.
What is Acromegaly?
Acromegaly is from Greek akros "extreme" or "extremities" + megas "large" = extremities enlargement. It's not considered a genetic disease. I acquired it somehow.
Statistics of Acromegaly
In the United States:
  • 40-60 people out of every million have acromegaly
  • 3 new cases per 1 million in the U.S. each year
  • approximately 4,676 cases per million
  • 116 new cases per million per year
My Experience
I was having some severe headaches last summer around July 2006, to the point where I felt non-functioning, which is unusual for me. I'd never even had a migraine that I knew of. I had an MRI of my brain and they found a benign pituitary macroadenoma 2.3 X 2 X 2.2cm (or a large pituitary tumor, greater than 20mm).
Small pituitary tumors are actually pretty common. Based on autopsies in the U.S., 25% of people have them. Most are benign, and they don't seem to cause significant medical issues.
My Symptoms:
  • 2.3cm pituitary macroadenoma (large sellar/suprasellar mass, left carotid artery slightly displaced, slight mass effect on optic chiasm)
  • severe headaches (mainly left posterior)
  • severe fatigue
  • enlarged spade-like hands and feet
  • achy joints, enlarged knuckles
  • depression (12 years before acromegaly diagnosis)
  • some confusion/memory and speech issues
  • irregular periods, amenorrhea
  • prolonged lactation
  • soft tissue growth
  • extra abdominal fat?
  • acne
  • developed barrel-like chest
  • enlarged nose and tongue
  • some frontal bossing of the forehead, sunken eyes
  • skin tags
  • snoring
  • high blood pressure
  • arthritis?
  • excessive sweating
  • teeth spacing
  • less libido
  • skin darkening
  • irregular heartbeats?
The Endocrinologist
I went to Stanford and saw an endocrinologist (hormone specialist), Dr. Katznelson. He visibly diagnosed me as acromegalic immediately. I was totally in denial that I could have this "disease". He referred especially to how my hands were so large and spade-like in shape, I had a lot of "soft tissue" growth he said. My ring sizes had grown (I didn't know at the time, but by 3 sizes on some fingers), my shoe size had grown (by 1 1/2 sizes), and I had other visible symptoms of acromegaly like the widening nose, enlarged tongue, and skin tags, along with my non-visible symptoms.

My tumor was behind my nose, beneath the brain in an area of dead air space. However, it was the size of a walnut, and it was squishing my normally pea-sized pituitary gland like a pancake. The pituitary gland regulates the hormones of the entire body. There was the possibility that my pituitary gland might have to be removed, in which case I'd need to be on HRT's (hormone replacement therapy) the rest of my life.

Surgery 12/2006 @ UCSF (San Francisco, CA)
I ended up having surgery at UCSF (San Francisco, CA) with Dr. Kunwar on December 12, 2006 to remove the tumor transphenoidally (through the nose). My surgery began around 8am and only lasted about 3 hours. The surgeon cut through the sphenoid bone, pulled the tumor out and packed the hole with a fat graft from my abdomen. I also had a lumbar drain inserted into my lower spine to reduce any pressure from fluids around my brain. I was a little freaked out by blood dripping from my nose for the next few hours following surgery, especially when I walked around. I had no nasal packing, but eventually it stopped.
I left the hospital by 11am the following morning. It was very similar to a sinus surgery, it seemed almost cosmetic to me. It only took me 4-6 weeks to recover. I had no headaches and basically felt like I had a bad head cold. However I am still very fatigued, which is pretty much normal I think. As far as I know at this point, the tumor was removed 99%, and my pituitary gland is intact.

Based on the statistics, if I didn't have surgery I could have gone blind because the tumor was beginning to press on my optic nerve. And my life expectancy would have been half that of a normal person because of all of the effects of the tumor and the acromegaly.

I had so many symptoms that came on gradually over the last few years, I didn't realize they were related at all. In fact, as each new symptom appeared it was easy for me to explain them away without checking into them because they didn't seem related.
Acromegaly in a Nutshell
Basically, in a nutshell, the tumor caused my pituitary gland to produce too much growth hormone (GH), which caused the acromegaly. The GH caused production of insulin-like growth factor (IGF-1) in my liver. The IGF-1 causes bone and tissue growth. The good news is even though my tumor will possibly recur, it can be controlled. And I can live a normal life with most likely a normal life expectancy thanks to my surgery and continuing treatment. Most likely I'll need to have shots every month to control my growth hormone and IGF-1.
The Famous Acromegalic
The reason I mentioned Andre the Giant (the WWF wrestler, also known as the giant in the movie 'The Princess Bride') is that he had acromegaly also. It's just that his form of acromegaly is called 'gigantism.' Gigantism is a type of acromegaly that begins in pre-pubescent children whose long bone cartilage hasn't fused together yet, leading to excessive bone growth. This in effect leads to excessive height. Acromegaly in adults causes many of the same excessive growth issues (but not excessive height). For example, my organs can grow too large which can cause me many problems like diabetes and congestive heart failure in the long run.
Links: - satellite site of the Pituitary Network Association

Endocrine & Metabolic Diseases Information Service

Acromegaly - Wikipedia

Pituitary Tumors

A personal acromegaly blog

Acromegaly forum

A little about me

I'm 30 years old. Just turned unfortunately :-) I'm married with 3 kids. I'm a big family person, love my family. I'm a student at CSU Stanislaus, although taking this semester off. I'm majoring in Computer Information Systems, although I don't know if that's what I want to do now. I also have a medical condition called acromegaly and I'm looking for others with personal experiences to share.