Editor’s Note: In the order section of this issue you will see an original recording by Pierre Jacomet, an award-winning Chilean concert pianist and philosopher who has VHL. After several surgeries for multiple pheochromocytomas, he is no longer able to play the piano. Nothing daunted, however, he has learned to express his classical piano music with the aid of a synthesizer. While the notes are made by the synthesizer, the programming, expression, and musicology are entirely those of this talented man. He is expected to speak to the VHL Symposium in Paris this fall. These recordings were donated by the artist to the VHL Family Alliance, to raise money for a cure. -JWG

I was born in Valparaiso, Chile, on March 24 1933, of French parents, and hold double citizenship. At the age of nine months I was kidnapped but immediately recovered. Kidnapping was a fashionable crime in Chile in those days, after the kidnapping of the Lindbergh baby. My late father told me once that my character changed completely after this episode, of which I still keep a memory, a sort of photo without any sensation attached.

I started feeling bad — that is, different — beginning about the age of seven. I had pavor nocturnus [fear of the dark] and other unexplainable fears. My mother was an excellent pianist, painter and a superb sculptor with gold medals and all that. My piano lessons started with her. Then we moved to Argentina where I pursued my studies at the Beethoven Conservatory, and then with Maestro Scaramuzza.

When I was 15 my parents divorced. Father remained in Buenos Aires and I came back to Chile with my mother. I finished high school and went to College were I started studying Law and Economy. These studies were interrupted because I got married at 21. As children started coming I had to earn a living. The piano was not sufficient to support the family, although I already had finished my studies at the Conservatory and had also studied composition with Maestro Gustavo Becerra-Schmidt who is now in Germany.

I went into business on my own. At the same time I started what was going to prove a long and futile search to determine that was wrong with me. After several wrong diagnoses I was labeled a neurotic and spent 3000 useless hours of psychonalysis. I of course studied psychoanalysis myself during that time, studies that brought me to philosophy and theology.

When I was 33 I opened an office in Buenos Aires where I settled with my wife and 7 children at the age of 38. I became a successful businessman but my marriage went to pieces and in 1972 I divorced my wife who had never gotten used to Argentina.

In 1975 one of my children was abducted. It was horrible. We got him back after six sinister days, paying the ransom. He has never completely recovered from this experience. Then came the military coup d’Etat in Argentina. My business was flourishing at that time and I had offices in New York, Paris and Rotterdam. In November 1978 there was a quasi war between Argentina and Chile. As you may know Argentica was, during that time, under the grip of a brutal military dictatorship. Those current authorities called me to their headquarters and forced me to surrender all my businesses at gunpoint. They subjected me to a long and insufferable psychological torture (following me with unidentified cars, threatening my family, abducting my brother in law, raping one of my nieces, and so forth).

In 1980 the French ambassador got me out of Argentina but all my family stayed there. I went to New York where I lived with Susan (who is now my wife) until 1982. I studied languages and literatures (Spanish, Italian, German, French and Portuguese, which were the languages I spoke). I abandoned business and dedicated myself to studying (ancient Greek, Latin, etc.). We moved to France where we stayed until 1984. I decided to start a new family.

We came back to Chile in February 1984. By then we had one daughter. I started again with the piano and recovered most of my technique. I gave a couple of concerts and was preparing Ravel’s Gaspard de La Nuit and Stravinsky’s Petrushka plus Beethoven’s Opus 101. But since the military episode I had continuous crises. My blood pressure went down to 70/35, with abundant sweating and tachycardia several times a day. This had started in 1979 and was getting worse and worse. Again everything was attributed to neurosis, which made me feel quite an idiot. Besides, all psychotherapies were ineffectual.

Finally, in 1989 I was diagnosed as having Multiple Endocrine Neoplasia (MEN) and two ugly pheochromocytomas* were extracted from my neck. The first surgery was badly performed in Chile and some neck nerves were severed, which left me with a clumsy right hand (I can shake hands, but not play the piano anymore). The second surgery was done in New York City with a partial removal of my larynx and no secondary effects.

Only this year Dr. Richard Haber, from Mount Sinai Hospital in New York, diagnosed VHL. Studying my family group he found kidney cancers, blindness, brain tumors and pheochromocytomas, which are possible results of VHL. He did not find any medullary thyroid cancer, which is characteristic of MEN. In retrospect I have lived with symptoms of VHL from age 7 to age 65, without a diagnosis. My family has had VHL for at least four generations without a diagnosis. The childhood fears, the "neurosis", the cardiac symptoms — all had a physical cause: those pheochromocytomas. Once they were removed the symptoms disappeared.

Right now I feel fine. I do have an angioma in my cerebellum but it’s not causing any symptoms. With help from Altheada Johnson on the VHLFA hotline I sent my scans to Dr. Oldfield at the U.S. National Institutes of Health in Bethesda.

Apparently the MRI was done without contrast and he was not able to see the angioma clearly. He advised me to repeat the MRI, which I intend to do in New York at the end of May.

I continue to record my music with the aid of a synthesizer, and dedicate myself to study, translating philosophic and scientific books. My recording of Bach’s Goldberg Variations was very well received in Germany. My newest field of interest is Molecular Biology. I live very quietly in a small town on the Chilean coast, near Viña del Mar. In all I have nine children, seven grand children. Life has gone by very fast and has been an incredible adventure. It still is. And wonderful too.

*Footnote: Pheochromocytomas (called pheos [FEE-ohs] for short, most commonly occur in the adrenal glands, but may occur outside them, along the Zuckerkandl bodies of the endocrine system, roughly along a line from the groin to the collar bone on both sides. Pheos can cause excessive sweating, cardiac symptoms, and untreated pheos can cause damage to the heart and vascular system. Pheos in the neck in particular can cause neurotic or even psychotic episodes.

As printed in the VHL Family Forum 6:2, June 1998. For permission to reprint, please contact VHL Family Alliance, info@vhl.org.