Jacqui’s Story

I was 16 years old, a junior in High School in 1979 when I started to notice something was wrong. I would get very sleepy around 10 AM in English class; I started to eat lifesavers to get me through until lunch at 11 AM. Sometimes my girl friends would say I looked like a ghost, very white, or that I had very dark circles under my eyes.  A few days later, I was feeling so bad I talked a friend into driving me home. I laid down on the couch and everything went blank, the next thing I remember was my younger sister trying to wake me up for dinner. Then there was the time walking home from school that I lost control of my legs, they seemed to want to walk like a high stepping horse, and then I fell to the ground and started crying.  I made my sister promise not to tell my mother, popped another candy and in a few minutes was able to walk the rest of the way home.  All of the before mentioned episodes happened within a two week period just before the day that I could not wake up  and started to go into convulsions. I was taken to the hospital where they thought I had overdosed; I had never even taken an aspirin and was into natural healing at this time of my life.  I was admitted to the hospital and within a week was diagnosed with Hypoglycemia, put on a strict diet and was told if I ate anything with sugar I would die.

I was able to modify my diet and was doing well for about a year. Then the episodes started again. This time I could not wake up and had to be given orange juice to get my blood sugars where they needed to be. I was adamant on eating something before bed and even getting up at 2 AM to eat something again, all this was to no avail. I was once again admitted to the hospital to undergo many tests. I had a great doctor that was new to the area, and even though the tests never showed anything to be wrong with my endocrine system diagnosed me with MEN1.  He was sure that I had tumors in my pancreas and my calcium level was very high as well. I was transferred to Cleveland Clinic to undergo exploratory surgery where they in fact found 5 islet cell tumors and removed my spleen and appendix.  The surgery took place on my 18^th birthday. I was told at the time that I was the first patient to manifest the syndrome with the pancreas tumors as well as being so young, and not having any family members diagnosed in generations before me.

A year later in 1981, I went back into the hospital for parathyroid surgery, also at Cleveland Clinic. At the same time, my Grandmother on my Father’s side of the family had her parathyroid surgery.  They removed the right side of my Thyroid and 3 ¾ of my Parathyroids. My calcium levels dropped some but were still in the high normal range. I was able to go to college and start a good job everything seemed to be going well. Until in 1989, my personality started to change. I became a very mean person, was extremely tired, and moody, I started to put on weight in my torso and face but not in my extremities, (symptoms of Cushing Disease). I had been seeing an endocrinologist every year since I was diagnosed with MEN1 so when tests started to show a chemical imbalance some scans were done to show a slight growth in my pituitary. I was prescribed parlodel to try to shrink the mass.  Things were going well until 1992 when the headaches and depression started. By this time, I had moved from Missouri to Virginia and had another doctor, She had taken me on as a patient and was learning along with me the nuances of the MEN1 condition. She also had contacts at NIH and was able to get me into a protocol for the MEN1 syndrome. I stayed a week at NIH and went through many tests to see what was happening with my endocrine system. I passed a 72 hour fast with no problems occurring – pancreas was working fine. My Cortisol, prolactin and PTH levels were a bit high however. Over the next year, I did many urine collections and blood draws and visits to NIH for further testing. ACTH was another chemical that was being watched. All the tests and further scans showed that the pituitary was going to have to be taken care of first. On top of this, I was also showing two large tumors in my liver, one of these was wrapping around my spine.  In May of 1993 I had the pituitary surgery, all went well and I became a nice lady again. Nevertheless, in November, the pain in my upper torso became difficult to live with, and my red count was down to 9. With further scans, the tumors on the liver had grown and would need to be removed. There were some complications with my intestines twisting after the surgery, so I was put on IV fluids while a tube was down my throat making sure the digestive tract would be able to rest. After two weeks, the intestines straightened out and I was able to eat again. Of course, I lost weight during this procedure and was down to 85 pounds but doing better.

Life was going well for the next few years until my calcium levels started to rise again. I was still going to NIH every other year for a workup of endocrine tests, mainly Bone Density, CTs, MRIs, and Octreotide scans of the areas where known tumors are residing. Along with blood work associated with the condition. In 1999 I had another surgery to remove the remainder of my thyroid and parathyroid. At this time I also had some lymph nodes on the left side of my neck removed (they were suspicious of cancer), and part of the parathyroid transplanted into my left arm. Two days after the surgery, my calcium levels never dropped so they tried to remove the transplant and thought they had it all. Over the years we have done many scans to look for the cause of my high calcium, but nothing is showing up to say I have more parathyroid tissue in my body. Until last year when they did an ultra sound on my left arm, it seems to have some of the transplanted tissue growing and producing PTH. I am to go back to NIH in April 2011 to have the left arm operated on in hopes that the calcium level will go back to the normal range. These past few months have been difficult with bone pain throughout my body and being extremely tired all the time, and having sever mood swings. I am hopeful that I will be able to function better once this surgery has taken place.

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