Morton A. Hirschberg

Symptoms

Introduction

Acromegalics undergo physical and psychological changes from the onset of their disease. An Acromegalic may experience a few or many of the symptoms listed. During the course of the disease additional symptoms, some of a more severe nature, may develop. Finally, this document is written from the perspective of a mature Acromegalic.

Physical Changes

Before puberty excessive growth to extreme height. After puberty, inappropriate growth. Growth can be of the fingers, hands, feet, jaw, eye ridges, and head. Soft tissue growth can be both external, such as the pads of the fingers or swelling of the feet, or internal with virtually every internal organ being enlarged. Fingers can become fat so rings do not fit, the feet large and wide so that shoes do not fit, and the head larger so that hats do not fit. Growth of the jaw may lead to separations or big gaps between the teeth. The tongue may grow and become very wide.

Due to the slow development of physical changes, this insidious disease may take many years before a correct diagnosis is made. Complications among those having the disease for any length of time arise from cardiovascular problems (i.e. enlarged heart and attending blood supply system) or colon cancer. These two are the leading causes of death among Acromegalics.

The following list of physical symptoms is meant to be as complete as possible.

• Arthritis and/or related symptoms (pain in the joints).
• Black and blue marks of the sole and/or instep of the feet.
• Bone spurs or bony growths on the spine.
• Carpal tunnel syndrome. Caused by growth of hands without corresponding connective tissue growth (the tendons do not grow).
• Deepening of the voice.
• Loss (weakening) of cognitive abilities and/or memory lapses.
• Colon polyps (the colon may also get longer).
• Drooling while either awake or asleep (see also sleep apnea).
• Eye ridges thicken and eyes look deep set (look like a cave man).
• Feet grow and/or swell (may not be bilateral).
• Fatigue (unusual tiredness or loss of energy).
• Headaches (from mild to severe).
• Hirsutism or hair growth on arms, legs, shoulders, chest and back.
• Kidney stones.
• Lower lip grows.
• Lower testosterone level in males.
• Abnormal menstrual cycle in women.
• Nose grows.
• Numbness and/or weakness of the hands.
• Osteoporosis.
• Reduced sexual drive and possible impotence.
• Saliva gland enlargement.
• Skin discoloration or darkening.
• Skin odor.
• Skin tags or other growths.
• Sleep apnea. Irregular breathing and breathing stoppages while asleep.
• Snoring (see also sleep apnea).
• Sudden and excessive sweating and hot flashes.
• Teeth separation or other facial deformities.
• Thickening of head or jaw (may not be bilateral or uniform).
• Thickening and toughening of the skin.
• Tingling, burning sensations or pain in the extremities (worse when tired - that old crazy bone feeling, only continuous).
• Thyroid gland swelling and/or other thyroid problems.
• Vision impairment and/or blindness.

Psychological Changes

Psychological changes are not well studied among Acromegalics or well reported. This may be especially true of prepubescent Acromegalics. Nonetheless, this is an important aspect of the disease, and the following symptoms or personality changes may occur.

• Loss (weakening) of cognitive abilities and/or memory lapses.
• Mania (but no depression. All that growth hormone gives one a constant high).
• Megalomania (feeling of great power and invincibility).
• Rage (fits of almost uncontrollable temper - with remorse afterward ...oh that wonderful growth hormone!)

Tests

Introduction

A growth hormone blood test can pretty much determine whether or not one is an Acromegalic. X-rays, Magnetic Resonance Imagery (MRI), and colonoscopy testing may follow. No mention is made of an angiogram and unless there is a surgical error this test is not normally administered. Angiograms are not discussed here except to say that they are not risk free. Complications arise in 3 - 5% of the patients tested.

Blood testing. Risks: really none, but an Acromegalic may be poked several times before a vein is penetrated due to tough, thick skin and veins. Glucose blood testing may also be performed where blood is drawn every half hour after a short (12 hours or so) fasting period. Risks: really none, but the blood may clot during glucose testing requiring multiple pokes. A good phlebotomist is a great asset. Telling some phlebotomists you are an Acromegalic and have tough skin may make them nervous. Don’t let yourself be poked more than three times by anyone.

Colonoscopy. Acromegalics develop colon polyps and an order of magnitude greater frequency than the normal population. Five percent of the normal population develops polyps while 55% of Acromegalics do (based on one study). A rectal examination itself cannot determine the presence of polyps. Risks: bleeding, pain, and perforation of the colon. The latter does occur with a 1 - 3% frequency while bleeding and pain may be more frequent. There is also the associated risk of the general anesthetic. The alternatives to a colonoscopy, while perhaps less risky, present far less diagnostic value. These are: (1) Ingesting barium and having x-rays. Risks: virtually none, but may not indicate the absence of polyps, especially if there are cloudy areas on the x-ray, and, (2) Partial scoping. Risks: as with full scoping, however, no anesthetic is administered. This is not the most fun in the world. Results may not be definitive and if polyps are found a complete scoping will be required anyway. Tell the doctor performing the colonoscopy you are Acromegalic. Tell him your colon may be long. That will help him. Bottom line (no pun intended), a full scoping seems to be the best option from any viewpoint (again, no pun intended).

Magnetic Resonance Imagery (MRI). Brain scans with and without contrast (Gadolinium) to completely confirm the diagnosis and determine the size and position of the tumor. Arterial blood flow can also be obtained when the contrast material (Gadolinium) is injected. Risks: without the contrast, none. With it, allergic reactions are possible but rare. If you are claustrophobic, take a walk around the machine. You will see it is hollow and you can see through it. It is not like a cigar wrapper. There are also machines which are completely open but the results are better with the more confining machine. Have them play music for you. I like the ear plugs and no music. Just like riding in a helicopter.

X-ray. Even before an MRI, x-rays of the hands and feet can show bone growth as well as soft tissue growth. Risks: those usually associated with x-ray; in general, virtually none.

Remedies

Introduction

There are three basic remedies for treatment of Acromegaly: drug therapy, radiation, and surgery. Complete normalization, generously speaking, occurs in between 55-80% of acromegalics. This means that various combinations of remedies, even all three, may be required. Each is now discussed separately.

Drug Therapy

There are two major drugs used to treat Acromegaly: bromocriptine (Parlodel) and octreotide acetate (Sandostatin). Neither of these drugs will kill the tumor, although there might be some reduction in size. They are used strictly to control the disease by reducing the growth hormone until it is in the normal range. If they can be tolerated and are effective, a life long regimen may be in order. Drug therapy is perhaps the least intrusive remedy for Acromegaly.

Bromocriptine (Parlodel) is taken orally. Side effects include stomach problems such as nausea. Dosage is increased incrementally to establish tolerance and effectiveness. The drug is usually taken at meal times to reduce unwanted gastronomic side effects.

Octreotide acetate (Sandostatin) is taken by injection. There is a wide range of possible side effects including stomach problems. Nausea, loose stools and gas (flatulence) are among them. Asymptomatic gall stones is perhaps the most serious of the possible side effects and occurs in 30-50% of those taking the drug. Dosage is increased incrementally to both establish tolerance and determine effectiveness. Daily injections of 1-3 shots may be required. A time spacing of the drug is suggested to keep the growth hormone level uniform throughout the day. More about octreotide may be found on the PNA web site or in the Pituitary Patient Resource Guide. Octreotide is a relatively expensive drug.

Radiation

There are two forms of radiation therapy: conventional radiation and Gamma knife (stereotactic radiosurgery).

Conventional Radiation

Daily treatments over a four to six week period (20-30 treatment sessions). Conventional radiation is not as well focused as is the Gamma knife. Side effects include: hair loss, burning, temporary and permanent memory loss, and a litany of glandular problems brought on by pituitary damage. Side effects may occur long after treatment and could result in requiring life long drug therapies as a corrective measure. Successful results may take effect from several months to 10 years after treatment. This remedy, then, would most likely be used in conjunction with surgery and/or drug therapies.

Gamma Knife (Stereotactic Radiosurgery)

Multiple emanations of cobalt 60 are administered in one treatment sitting. While the beams are focused and are low level, there are Risks: Side effects are as above. Not everyone is a suitable candidate for Gamma knife. This remedy is not effective if the tumor is too large. Successful results may take effect from several months to several years after treatment. This remedy then would most likely be used in conjunction with surgery and/or drug therapies.

Of the two, Gamma knife would be my first choice.

Surgery

There are two forms of surgery available: frontal craniotomy and transsphenoidal.

Frontal craniotomy

This form of surgery, which involves opening the skull and moving the brain to expose the pituitary area, is rarely done anymore. Risks: high. Side effects run a huge gamut including stroke.

Transsphenoidal

This has become the surgical procedure of choice. Risk: usually less than 1% exhibit complications. Of course, there is a risk with any general anesthetic. Effects can be that many symptoms such as headaches still persist. In addition, it may not be possible to excise the entire tumor, leaving growth hormone levels high. This procedure is performed by going through incisions made in the nose and behind the upper lip. No visible scars show and pain is just about nil. If surgery is repeated, risks jump to a 5% complication rate. It may still be necessary to have drug or radiation therapy after a successful surgery.

All things considered, transsphenoidal surgery has to be considered the best remedial alternative for continued health and longevity, especially with the prospect that drug and radiation therapies will not be required. Anything over 50% seems like great odds!

Epilog

Finally, once an Acromegalic, always an Acromegalic. Testing and monitoring must be performed on a regular basis.