April 25, 2004

The only description for this past month is miserable. The pain is relentless, the fatigue is debilitating, and the weight loss is bizarre.

Even though my body felt like I had been run over by a truck, I accompanied my daughter, son in law and grandson to the city at 10:00 AM on April 2nd.   Since it was Grandson’s birthday weekend, he took a friend with him. I had made arrangements with the hotel to check in early so the boys would have the afternoon to play at the pool.

My husband had planned to join us at the hotel after work.   He left home before 5:00 PM. We waited and waited for him and I tried to call him on his cell a number of times.  I finally got through at 7:00 PM and the poor man was fit to be tied. He had been in the city since 5:00 PM but couldn’t remember what hotel we were at. He finally arrived at the hotel at 7:30 PM; in time to join us for dinner.

We had a birthday cake in our hotel room that evening and Grandson opened his gifts. The new skateboard and X Box with three games were a hit. We all left the hotel at noon on Saturday and met at A&W for lunch before heading back  home. (A&W was the kids’ choice).

We got home at 2:00 PM on Saturday and by 3:30 PM two of the moms from the Ukrainian Dance Ensemble were in my kitchen helping me cut up fruit for the fruit platters. My grandson’s annual spring recital was scheduled for Sunday, April 4th. All the people attending are invited to join the dancers at a reception following the recital. As soon as the moms left I went back to printing programs. I couldn’t finalize the recital programs until Friday morning so I ended up printing programs until 2:30 AM Sunday morning.

Grandson and I were at the theatre by 10:30 AM on Sunday morning. The kids did several run throughs of their dances and the moms set up the displays and the lunch tables. The recital started at 2:00 PM. We were so proud of Grandson; he didn’t miss a dance step even though he woke up with a fever of 102 on Sunday morning. My husband and I didn’t get home until 5:00 PM since we stayed to help with the clean up.

When I finally got home I crashed. I was in so much pain I could not find a comfortable position in bed. My darling daughter made dinner for us so we went over there at 6:30 PM. After supper we watched the movie “Oh Brother Where Art Thou” with them. As soon as the movie ended we went back home and I went straight to bed.

Monday I did a few things around the house and went out to pick up Grandson’s birthday cake. Since April 5th was Grandson’s actual birthday I had invited family over to my house for birthday cake and coffee at 7:00 PM. Had a lovely evening but I was beyond exhausted.

April 6th was a very disappointed morning. I had placed several calls to the hypertension specialist office trying to book the appointment I was to have six weeks from my first appointment on March 1. I also inquired about my lab results from the tests he had ordered and I had done on March 1. When I called on April 6th, I was told my appointment would be on Sept 1 and I would have to wait for my test results until that time. I told the receptionist that this was unacceptable so she said she would have someone call me this afternoon. What is their agenda? Are they waiting for me to croak so I will no longer bother them?

The Old Hippie finally called me back on April 15th. He told me that the few tests he took were normal and that he would see me again on September 1. He also told me he had no idea why I had so much pain and that I had normal blood pressure readings in his office. Of course I did! The man doesn’t seem understand the concept of intermittent spiking blood pressure. No Name had described my problem of spiking blood pressure in a letter he wrote to the Old Hippie.

The bottom line is that here I sit waiting for nothing. No Name is on an extended vacation and I will not see him again until May 7th. Even though I am on two blood pressure medications I continue to have blood pressure spikes as high as 220/120. The local hospital refuses to do a plasma free metanephrine. The lab director insists that it is too complicated. Even the Old Hippie has no clue about a plasma free metanephrine test which according to the information below has been available since November 1999.

We are told by our government that we have a wonderful medical system. Everyone gets equal rights to medical care. They constantly stress that we do not have a two tier system. This is a blatant lie. We pay through the nose for our medical care. We pay a very high personal income tax; one of the highest in the world. The two tier system does exist in this country. The rich go to the US for their medical care. The others die. The perfect example of this is a friend of my husband’s whom I shall call Joe. About six weeks ago Joe was diagnosed with cancer. He was sent to the “city” for surgery. The surgeon opened him up; promptly closed him up again and sent him home to get his affairs in order. Well, Joe happens to be rich so he and a friend rented a plane and pilot and flew to the Mayo Clinic in Rochester, Minnesota, USA. After 5 days of tests at the Mayo Clinic he was told that there was hope and his situation was not terminal. He came home to regain his strength and will go back to the Mayo Clinic for his surgery. If Joe was a car salesman in Canada, he would be busy planning his funeral and saying his goodbyes to family and friends. If this isn’t a two tier system, what is?

Our hospitals apparently have the right to refuse to do a test that a doctor has ordered. They can get away with using a lame excuse such as “it is too complicated; we run an assembly line here.” We import doctors from South Africa and allow them to practice medicine for 5 years without a valid Canadian medical license. Our Canadian medical schools limit enrollment so our country has a shortage of qualified Canadian doctors. The few good doctors that Canada does produce head to the US as soon as the opportunity presents itself. Instead of working on keeping Canadian doctors in Canada we import them from countries overseas.

To make matters even worse, there is the stigma attached to women over fifty. As far as I am concerned, the medical profession considers women over 50 as “throw aways”. If we are thin we suffer from anorexia, menopause and depression. If we are overweight we suffer from over eating, menopause and depression. If we have low potassium levels we suffer from induced vomiting, menopause and depression. When No Name wrote to a well known kidney specialist on my behalf and cited my low potassium levels, the specialist’s assumption without ever seeing me was that the low levels were probably caused by self-induced vomiting and of course menopause and depression. If we suffer from bone pain it is from lack of exercise, menopause and depression. If we suffer from extreme fatigue and exhaustion we are lazy, menopausal and depressed. If we suffer from high blood pressure we are suffering from self-induced stress, menopause and depression. If we have sleep problems we suffer from sleep apnea, self-induced stress, menopause and depression. It really does not matter what the test results show. If an over 50 year old woman attends a sleep clinic and there is no sleep apnea, the doctor conveniently ignores the result. If her test result comes back lower than normal it is considered a fluke. If her test result comes back higher than normal it is most certainly something she ate to confuse the test result.

Who gives the doctors the right to presume that women over 50 are not interested in a decent quality of life? Who gives them the right to ignore symptoms for years? The doctors in this country have set themselves on a pedestal and consider themselves immune from criticism and accountability. The trouble is they are immune and they have the convenience of burying their mistakes. If my car mechanic messes up my car he is held accountable. If a physician messes up my diagnosis, he just goes on his merry way.

This is the information I received via email this week. According to the website below: Clinical testing for plasma metanephrines with a 2-day turn-around is available from the Mayo Clinic by calling 1-800-533-1510 or +1 (507) 266-5700. The published fee as of November 8th, 1999 was $122.50. CPT code 82491. Please ask for information on blood drawing, shipping instructions, and patient instructions.

Estimated shipping cost from Canada about $100 – $150. (The blood has to be shipped on dry ice.)

Finding Elusive Pheochromocytomas

Review of studies by Graeme Eisenhofer, Ph.D.

A newly developed blood test to detect potentially deadly tumors that form in the adrenal glands has been shown to be significantly more sensitive than traditional diagnostic tests. The new test provides earlier and more accurate diagnoses of these tumors in patients with an inherited predisposition to develop them, possibly preventing complications or death. The study, led by researchers at the U.S. National Institute of Neurological Disorders and Stroke (NINDS) and the National Cancer Institute (NCI), appeared in the June 17, 1999, issue of The New England Journal of Medicine.

“Although rare, these tumors are clinically important because they must be excluded as a surgically curable cause of hypertension in many of the people who develop high blood pressure,” says Graeme Eisenhofer, Ph.D., a researcher in the Clinical Neurocardiology Section of the NINDS, and lead author of the study. “If the tumors are not diagnosed and removed, they can have potentially catastrophic consequences for the patient.”

The mainly benign tumors, called pheochromocytomas (pheos), are sometimes found in patients with von Hippel-Lindau (VHL), a familial cancer syndrome with neurologic complications in which affected individuals inherit a predisposition to develop tumors in a number of organs, including the adrenal glands that sit atop the kidneys.

“Pheochromocytoma can occur in VHL patients as young as 4 years of age. Deaths from unsuspected adrenal gland tumors have been reported in young children in these families and it is important to make the diagnosis early so that surgical intervention can be performed,” said W. Marston Linehan, M.D., NCI’s Chief of Urologic Surgery.

A patient who develops pheos for any reason is at risk for dangerous and unpredictable surges in blood levels of certain adrenal gland hormones that regulate blood pressure and which are responsible for the so-called “fight or flight” responses to stress. The surges in hormones and resulting spikes in blood pressure put the patient at risk for heart attack, stroke, hemorrhage, or sudden death.

Currently, the most reliable tests for pheos use imaging technologies, such as CT (computed tomoraphy) or MRI (magnetic resonance imaging), which can be time-consuming and expensive and which do not necessarily identify a tumor as a pheo. Confirmatory biochemical tests are required for accurate diagnosis. Several biochemical tests are available which measure blood and urine levels of the adrenal gland hormones. But in many cases these tests are not accurate, because some pheos do not release the adrenal hormones regularly or in significant amounts. These tests depend upon catching the pheo during an active episode.

Drs. Eisenhofer, Linehan, and their colleagues studied enzymes important in catecholamine production that may have altered activity in pheos in contrast to normal adrenal tissue. Metanephrines are produced mostly by pheos, not normal tissue. Dr. Eisenhofer and colleagues found that measurements of blood levels of these chemicals makes it easier to differentiate secretion of normal tissue versus pheo. Thus these measurements give a more accurate diagnosis of pheos. A person with a normal plasma concentration of metanephrine and normetanephrine can be fairly confident of not having a pheo. Because of the high reliability of this tests, additional tests are not needed to rule out a pheo, significantly reducing costs.

Altogether, they measured the amounts of normetanephrine and metanephrine in 26 patients with VHL and 9 patients with multiple endocrine neoplasia type 2 (MEN2), another rare genetic disease characterized by pheochromocytomas. Use of the new test detected 97 percent of the tumors, whereas the other tests detected only 47 to 74 percent of tumors. Although particularly useful in diagnosis of tumors in VHL and MEN2, the test also shows promise for improved diagnosis of pheos in the much larger population of patients with high blood pressure where the tumor needs to be excluded.

Symptoms. Pheochromocytomas are usually benign. They may occur in or near the adrenal glands, or anywhere along the sympathetic nervous system roughly from the base of the skull to the bladder. The most apparent symptom, caused by the increased secretion of epinephrine and norepinephrine, is hypertension, or high blood pressure. This hypertension may be constant or intermittent. Attacks may occur every few months or several times daily, and typically last less than five minutes. Physical and emotional stresses can initiate an attack. During severe attacks, patients may experience headache, sweating, apprehension, palpation, tremor, pallor or flushing of the face, nausea and vomiting, pain in the chest and abdomen. There may be a tingling, burning, or crawling sensation on the skin of arms or legs or urinary difficulties.

Testing options. The most commonly used test for a pheo is a 24-hour urine collection. All the urine is collected for a 24-hour period, kept refrigerated, and then analyzed for levels of catecholamines and epinephrine. Patients are asked to avoid caffeine, bananas, vanilla, chocolate and a lengthy list of other foods for two days before the test. Many foods can cause false positives, but caffeine is the most frequent cause of false negative results. The test is somewhat inconvenient, as you have to keep a jug of urine in the refrigerator, and you have to remember to save all urine for this period, even if you wake in the middle of the night. This test is even more difficult to perform reliably with a small child.

Pheos that occur in the adrenal glands themselves are usually the easiest to find. They usually appear quite clearly on a CT or MRI, even when they are quite small. CT and MRI are equally good at showing them. The hardest ones to find are those which occur outside the adrenal glands, in the tissue of the sympathetic nervous system, anywhere from the base of the skull to the bladder.

Dr. Eisenhofer and his colleagues compared the normetanephrine and metanephrine levels in the blood against the blood levels of catecholamines (epinephrine and norepinephrine) and the levels of these and other chemicals in the urine. They found that the blood test was 97% accurate in detecting the presence of pheo tumors, while the other biochemical tests were only 47% to 74% accurate. All patients with MEN-2 had high blood concentrations of metanephrine, while the patients with VHL had almost exclusively high blood plasma concentrations of only normetanephrine. One person with VHL had a normal plasma level of normetanephrine. This patient had a very small adrenal tumor (less than 1 cm.) The higher the sensitivity of measurements of plasma normetanephrine and metanephrine, the more accurate the test in finding pheos.

The study recommends use of HPLC measurements of plasma free normetanephrine and metanephrine as the initial biochemical test of choice. To avoid false-positive results, a list of any drugs the patient may be taking should also be considered, and the patient must be cautioned not to take acetaminophen in any form (e.g. Tylenol, Excedrin, or as an ingredient in cold medications) for at least five days before the sample is drawn. It is best if the sample is obtained in the morning after an overnight fast (water and non-caffeinated soft drinks are permissible). Caffeinated or even decaffeinated coffee should be avoided for at least 24 hours before the test, and the doctorshould be told if these have been taken, as they can cause higher levels of dihydrocaffeic acid in the bloodstream and reduce the accuracy of the test.

If plasma free metanephrines have been run, and they are well within the normal range, then it is highly unlikely that the patient has a pheo and there is little need for further tests. On the other hand, blood or urine catecholamines, even when performed in combination, may yield normal results when there is in fact a pheo present.

Locating the tumor. In most cases of positive biochemical results, CT or MRI scan of the entire abdomen will usually locate the tumor. However, in many cases it is also appropriate to follow up with MIBG scintigraphy — preferably using the 123-iodine labeled compound rather than the 131-iodine labeled compound — to establish more reliably that a located mass is a pheochromocytoma, or to locate an extra-adrenal pheo.123-iodine is ten times as sensitive as 131-iodine. It tends to be less available because it has a much shorter half-life and therefore has to be used within 24 hours of preparation. It is most available near large university centers where they are able to do the “labeling” process in their own research facilities. MIBG-131 finds only 60% of pheos in VHL; MIBG-123 finds in the range of 95%.

Treatment. The treatment of choice whenever possible is laparoscopic adrenal sparing surgery. Since VHL patients often have bilateral pheos in the course of their lifetime, it is important to retain as much adrenal function as possible even when dealing with a single pheo.

1. Eisenhofer, G.; Lenders, J.W.M.; Linehan, W.M.; Walther, M.M.; Goldstein, D.S.; Keiser, H.R. “Plasma normetanephrine and metanephrine for detecting pheochromocytoma in Von Hippel-Lindau disease and multiple endocrine neoplasia type 2.” N.E.J.M. 340:24 (1999) 1872-1879.

2. Eisenhofer, G; Walther, W.M., et al, “Plasma Metanephrines: Novel and Cost Effective Test for Pheochromocytoma,” Proceedings of the 1st International Meeting on Adrenal Diseases, Brazilian Journal of Medical and Biological Research, September 7, 1999.

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