Story about Addison Disease .

When Secondary is Primary

Dec 30, 2015

By: Cheryl


   

Slumping into a chair I lamented to my father, ‘I feel like my life is hanging on a thin strand that could break at any moment.’ It was late October, 1980.

How, in a matter of months, did a formerly healthy, animated career woman of thirty-three, a technology manager at a national aerospace company, come to feel like she was about to draw her last breath? This story actually begins in 1963 when I was only sixteen.

I had a normal, healthy childhood and adolescent development in every way. At sixteen, my menstrual periods became irregular.  The family physician said this was typical of teenage girls, and there was no reason to worry. Four years later my menstrual periods ceased altogether. Again the family physician was not worried. My parents were happy that I was otherwise a healthy, thriving college student.  Like many people at the time, they did not have health insurance.  They trusted the physician and decided against further diagnostics. I was actually relieved not to suffer the monthly discomforts of my sorority sisters, and I had no intention of starting a family any time soon – so why worry?

Two years later I completed college, began a career as a systems programmer in upstate New York, and finally had health insurance. Because I saw marriage and children as a possibility in the future, I went to see a gynecologist to get this problem evaluated and solved. Hospitalization for a procedure to scrape the uterine wall followed, but my condition did not change, and blood tests provided no further clues to this mystery.

In 1971, I changed jobs and moved to Philadelphia, a city with five medical schools including then Women’s Medical College of Pennsylvania and the world-renowned University of Pennsylvania (Penn). Surely the experts here would be able to diagnose my problem. What followed was six years of gynecologist visits: poking, prodding, blood tests, urine tests (a 24 hour collection), X-rays, drugs and hospitalization for a laparoscopic procedure to examine my ovaries. What did not follow was a diagnosis.

Meanwhile, I continued to feel perfectly healthy and energetic, and my career was soaring. In 1975, at age twenty-eight, I became the youngest manager and one of the few women managers at a major aerospace company. Programmers and systems engineers who were many years my senior reported to me. I felt great physically, mentally and emotionally. Likewise on a spiritual level, I had asked Jesus Christ into my life, and I believed that God had a plan for me.

I had endured eight years of testing, and I was discouraged and frustrated. There was no way for me to know that the tools to make a diagnosis simply did not exist yet. Two key developments in medical science were about to catch up with me. First, Robert Ledley was granted a patent on November 25th in 1975 for a ‘diagnostic X-ray system’ also known the CAT-Scan (now called CT scan). Second, the capability was developed to measure the prolactin hormone from a blood test. (Elevated prolactin levels, required for post pregnancy lactation, prevent a woman’s body from menstruating. Prolactin testing is now commonly ordered when a woman is experiencing infertility or irregular menses.)

In 1977, the first test to measure prolactin in my blood was performed. The prolactin test results revealed a level more than ten times the normal range. The results were so high that the lab performed the test multiple times as they were fearful that their equipment was defective.  CT scans were not widely used for diagnosis until the 80s, but in 1977 I had my first CT scan at Penn. While previous head x-rays failed to show any problem, the CT scan clearly revealed that I had a tumor on my pituitary gland. I was horrified by the prospect of a tumor in front of my brain, but relieved that at last I had a diagnosis—a prolactin-secreting tumor.

As a technical expert working to develop creative solutions in the aerospace industry every day, I thought that now they had identified the problem, they would fix it. After years of grueling medical investigations, I was astounded when the specialists wanted to wait and see! Because I felt great and had no other symptoms except lack of menses, they decided simply to monitor the tumor. A year later, when the CT scan revealed that the tumor was growing, my gynecologist prescribed bromocriptine, a relatively new drug, to shrink the tumor. Although previously I had felt perfectly healthy, I began experiencing side effects of severe nausea and headaches, so I could not continue taking the drug.

By 1980, the physicians decided that leaving a growing pituitary tumor unchecked was dangerous. The optic nerve travels under the pituitary gland on its way to the brain. My vision was fine, but a growing tumor could put pressure on, or even destroy, the optic nerve and permanently impact my vision. The surgery was scheduled for late June. On one level I was happy that we were finally going to fix this thing; it would be a temporary interruption, and I could get on with my busy life. But at a deeper level I was terrified. I understood that the pituitary gland was located in the head between the temples. During surgery, the neurosurgeon would cut through my nasal passage and sinuses – virtually lifting off my face.

A week of vacation at the beach just prior to the surgery helped to calm me, but the fear remained until God brought to mind the words of an old hymn by Edward Mote that I had learned as a girl, the chorus of which is:

_On Christ the solid Rock I stand, _

_all other ground is sinking sand; _

_all other ground is sinking sand._

 

As I walked along the sandy beach, I knew that Christ was my solid rock for the future. I experienced a peace that defied understanding. I had no idea then how important that blessed assurance would be. I was about to live a medical nightmare.

Waking up from the anesthetic, I was told that the surgery had been a success. The tumor was encapsulated, and the pituitary gland was left untouched. I had a terrible and continuous headache, but this was to be expected post-surgery because my sinuses were temporarily packed with gauze. I was a healthy young woman who would recover quickly. I was prepared to patiently wait the four to six weeks recommended for recovery – enjoying a mid-summer respite from my busy career.

This break was the perfect time to make plans to get ahead in my career. I decided that I should go to graduate school part-time to earn a master’s degree in business administration. I used the time off to prepare and take the Graduate Management Aptitude Test. This surgery was just a bump in the road. Soon I would be back on the fast track.

My head had never stopped hurting from the day of surgery, but I returned to work after four weeks. Now the pain worsened each day, and I was beginning to feel weak. I contacted the neurosurgeon, who insisted the headaches could not possibly be related to the surgery. He surmised that I had a tension headache from my job and prescribed a vacation. The facts that I had rarely experienced headaches before the surgery and had never had a tension headache were unimportant to him.

By early September I was in agonizing pain and my internist was willing to see me. He theorized that the pain was from an allergic reaction to the suture material used for the surgery. He prescribed prednisone which is commonly used to treat severe allergic reactions. Within a day, the headache pain was much improved, but not gone. I continued to take prednisone and I returned to work. My head ached day and night, and I was so weak that walking up a flight of stairs was a major effort. I set up an appointment with my gynecologist.  Finding nothing wrong with me, he prevailed upon the neurosurgeon, and set up an appointment for me to see him.

The neurosurgeon, whom I credit with saving not only my sight but possibly my life, only granted me a ‘hearing’. He talked to me from behind his large mahogany desk, never examining me or even taking my blood pressure. He maintained that his surgery had been a success, and that the symptoms were ‘all in my head’. When I told him how the prednisone was helping a little bit, he lectured me, saying, ‘Prednisone [a cortisone derivative] is prescribed for tension headaches. But you better get it through your head that you cannot take THAT for the rest of your life.’

I stumbled through the days and weeks ahead. Many mornings I had such severe pain and weakness that I could not drag myself out of bed to go to work. I prayed, but I did not know where to turn for medical help. In October 1980, I truly believed that my life was hanging by a thin strand that could break at any moment.

I then remembered an endocrinologist whom I had met briefly at Penn three years earlier when the tumor was originally diagnosed. He had been brought in on my case by the gynecologist when the pituitary blood work, including the prolactin testing, was done. I telephoned his office, and he returned my call the same day. Amazingly, he remembered who I was and the facts of my case. When he learned that no post-surgical endocrine testing had been performed, he had me admitted to the Penn Clinical Research Center within days.

I was taken off the prednisone, and over the next several days I was given a complete battery of endocrine tests. I was living a crash course in endocrinology and about to learn a new vocabulary. The tests showed that my pituitary gland was not functioning at all – a condition known as panhypopituitarism. My ‘master gland’ was on strike, and had stopped sending messages to the ovaries (causing sterility), the thyroid (causing hypothyroidism), the hypothalamus (causing diabetes insipidus) and the adrenal glands (causing secondary Addison’s disease). I felt a rush of relief that a cause for my symptoms was diagnosed at last. The cause was literally ‘all in my head’ as the neurosurgeon had said. It was in my pituitary gland. Plus, it could be treated, so I wasn’t going to die!

 I was given my first doses of supplemental hormones to replace all the missing ones. The headache remained to a lesser degree, but my energy level began to be restored. I was sure that now I was on the road to recovery, and that once home, life would soon return to normal.

The endocrinologist insisted that I promise him I would order a medical alert bracelet as soon as I returned home. A medical alert bracelet – that was for really sick people! It would be the first time I heard the explanation of Addison’s disease (AD). In a normal person, the adrenal cortex makes cortisol. When you lack cortisol, you can experience disorientation, weakness, tiredness, muscle aches, nausea, dizziness, low blood sugar, low blood pressure and eventual blood vessel collapse; you can even go into shock and die.

The medical team admitted that, after they took me off the prednisone prior to the recent testing, and due to an oversight, my blood pressure had dropped to life-threatening levels before it was caught, and intravenous hydrocortisone was administered. My mind wrapped around this fact. I realized that the prednisone which my internist had prescribed for a suspected allergic reaction (and which was derided by the neurosurgeon as a quick fix for what he wanted me to think was a psychosomatic problem) was the only thing keeping me alive for the past two months. God had allowed a misdiagnosis to ensure I received this life-saving medication!

In a normal person, the body produces larger amounts of cortisol when subject to any kind of stress: physical, medical or emotional. Since I was going to be taking the same dose of hydrocortisone daily, I was told that I would need to take extra hydrocortisone whenever I was experiencing stress. However, I was given no clues as to how to determine that amount except, ‘If your temperature goes over 99 degrees, double your dose.’

The challenge, at that time, was for the endocrinologist to figure out the optimal doses of hydrocortisone and all the other hormones. These were never measured, so there was no record of my pre-surgical levels. I left the hospital in early November with hope, but I had no clue what a rough road still lay ahead. The endocrinologist ordered multiple blood tests, and the medications were adjusted about every six weeks.

My head continued to ache constantly. Some days were better and some days were worse. Adjustments to the medications made no difference in the headache pain. The endocrinologist told me he had never seen a patient with headaches like this. He believed that I had a neurological problem. I went to a neurologist who performed many tests. He was sure that it had to be an endocrine problem. All I knew was that, in the first three months of 1981, I had missed work one third of the time due to pain and exhaustion. Once again, I felt like I was losing my life. Now I feared I would lose my job, too.

These physicians had given up on me. My friends and family prayed for my recovery because they believed that God had not given up on me. At a friend’s suggestion, I contacted the elders of my church to have a special private prayer time, and for them to anoint me with oil. Following our time together, an elder talked with me privately and gave me the name of a neurologist, a member of our church, who had been helpful in the care of his son. Contacting this physician, I discovered that he was a pediatric neurologist who, ‘Didn’t do big people diseases.’ But he listened attentively and offered to arrange for me to see a world-renowned neurologist in New York City.

Soon, I was at New York’s Columbia Presbyterian Hospital having a CT scan and being examined by the neurologist. He, too, could find no clear neurological problem, but his experience led him to prescribe a medication, propranolol, from a class of medications called beta blockers that are normally prescribed to lower blood pressure. In his experience, he had found that this drug was effective in altering brain chemistry, and he thought it just might help me. Within twenty-four hours of taking the medicine the headaches began to decline. After eleven months of endless pain, the headaches ceased altogether a few days later. Many prayers were answered.

Because of my AD, I already had low normal blood pressure. Taking propranolol made it even lower and I experienced the associated symptoms of lightheadedness and dizziness. While a big improvement over a headache, I could not stay on this drug for long. After a few months the neurologist had me stop the medication. Miraculously the headaches didn’t return. I no longer needed that drug. I phoned the pediatric neurologist who had arranged for me to meet this wonderful specialist, so that I could tell him the good news and thank him for his involvement in my recovery. Unfortunately, the phone call came too late. I was told that he had died of cancer a few weeks earlier. But God’s timing to use this man in my life had been perfect.

Now that I was free of constant pain and my endocrine medications were well-controlled, life began to return to normal. I felt healthy for most of that summer of 1981 except for recurring sinus problems. By that fall, I had become a regular at the office of an ear, nose and throat (ENT) specialist who prescribed one antibiotic after another for me. Because of AD, each infection lasted twice as long as it would have for a person without AD. At this early stage of AD, I had not yet learned to listen to my body and adjust the hydrocortisone dosage to help my body deal with infections. In fact, I was fearful of taking too much. In early December, feeling weak and feverish, I went to my internist, who diagnosed me with spinal meningitis and immediately admitted me to the hospital.

The physicians determined that I had a Staphylococcus (staph) infection in the sphenoid sinus, which was causing the meningitis. I was too sick to ask for extra hydrocortisone which I needed because of AD, but God was protecting my life once again. The intravenous antibiotic prescribed for the staph infection produced a burning sensation in my blood vessels. The physicians thought I was having an allergic response to the drug, so they administered prednisone with each intravenous dose. I remained in the hospital for several weeks and eventually recovered at home.

As 1982 began, I faced sinus surgery. The ENT theorized that the staph infection and resultant meningitis was caused by the failure of my sinus to properly drain. The silicone substance purposefully left in the sphenoid sinus during my pituitary surgery was preventing the sinus cavity from draining. A surgery to remove the silicone was uneventful, and I finally began to feel like I just might be healthy again some day.

Each day I took the various medications, and each month my health improved. No more headaches. No more sinus infections. I knew that my panhypopituitarism meant I could never bear children, but I could still have a successful career. I began to dream again about going to get a master’s in business administration (MBA). Spending so much time at Penn for my healthcare only contributed to my desire to go there for graduate school. However, Penn’s executive MBA program required that a student work full-time in management and simultaneously take a full-time load of three graduate courses for six continuous semesters over twenty-four months.

Could I do this? Was I healthy enough to be both a full-time aerospace technology manager and a full-time graduate student at one of the top ranked MBA programs at one of the best universities in the nation? I decided that, if God would allow me to be accepted, He would give me the strength to complete the program. I dug out the results of the Graduate Management Aptitude Test taken in the summer of 1980 and sent in the application. I was accepted, and in June of 1983 I started graduate school. Two years later, I graduated with my class. For me this was proof that I had my life back and could really start living again.

As time passed, I realized how important it was for me to listen to my body and adjust the hydrocortisone dosage before I got sick. As a result, AD no longer interfered with my career in information technology (IT) management. By the time I graduated with an MBA in 1985, I was a director managing sixty people on an aerospace systems integration program. I went on to complete a thirty-six year career in information technology as a partner and program manager in an international consulting company. None of my endocrine problems, including AD, has prevented me from traveling domestically and internationally for both business and pleasure. AD has never limited my full and busy life that has included serving on a non-profit board of directors, volunteering in various capacities at my church, being a member of the executive committee of my local political party, managing the care of a seriously ill family member, and training and taking my dog on regular visits to patients at a veteran’s hospital.

Through those decades, my health has been far from perfect. I’ve had multiple foot surgeries, back procedures and pancreatitis. I also have had both of my hips replaced. With my first outpatient surgery (for a bunion on my foot), I learned how very little most physicians know about AD. Prior to the surgery, I told the orthopedic surgeon that I needed intravenous hydrocortisone to be administered during the surgery. I also gave him the name of my endocrinologist, who was located in the same hospital, should the surgeon have any questions. However, the orthopedic surgeon and anesthesiologist decided not to give me any additional hydrocortisone before, during, or after the surgery. I was released from the hospital late that day, but by the time my friend got me home, I was unable to stand up. I had to crawl from the car into the house. Fortunately, my friend was able to reach an endocrinologist, who had her give me three times my normal dose of hydrocortisone. My energy level returned quickly, and I had a normal recovery.

After this life-threatening experience, I stopped trusting that physicians will understand the seriousness of AD. I learned to get a letter of specific protocol for the administration of hydrocortisone from my endocrinologist in advance of any medical procedure. I then share copies of his letter with all medical professionals (nurses and physicians) who are caring for me. Prior to any procedure that requires anesthesia, I refuse to take off my medical alert bracelet until an active hydrocortisone intravenous line has been put into a vein. In the event emergency care is required, I keep a card with a summary of my medical history and medications in my handbag, as well as in my car. I also have given copies of this card to my closest friend and my siblings.

Contrary to that original neurosurgeon’s pronouncement, I have taken and will continue to take hydrocortisone for the rest of my life. At sixty-five, I am able to live a very active life so long as I take all my medications each day. I’ve learned to listen to my body and make adjustments to my medications when I experience any physical, medical or emotional stress. I believe that God was at hand throughout my journey.  Those who treated me are human, subject to inadvertent errors, and dependent on medical advances that don’t always come soon enough, but God has a plan for my life.  Perhaps one reason my path was so tortuous was so that I could impart to fellow sufferers, and especially the newly diagnosed, lessons that may be helpful and bring them hope.

Most people who know me have no idea that I have such a serious medical condition. They are usually surprised when they find out. I just say, ‘I am either the sickest healthy person you know or the healthiest sick person you know.’

 

_CHERYL_ lives near Philadelphia. She developed Secondary Addison’s disease following surgery for a pituitary tumor in 1980. She is retired from a thirty-seven-year information technology career where she specialized in the aerospace and pharmaceutical industries.  Busy in retirement Cheryl serves on her church’s Deacon Board and as a volunteer with her therapy dog at a veteran’s hospital and a school for severely autistic children.  She is coordinator for her community’s annual Eco Garden Tour and is on the leadership team of a genecology club.  She extends special thanks to Barb, Edie, Ed, Janice, Jessica, and Robin for their editorial assistance with her chapter.

 

 

 

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You are such an inspiration! You should write a book. May God forever keep you in his care.

Commented 7 years ago Susan 90

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