Kenneth Smith's Story

Being given the diagnosis of pheochromocytoma is not something anyone wants to hear. As I look back to June of 2008 when I received this diagnosis, I had no idea how this tumor was affecting my life. The following is a synopsis of my dealing with neurofibromatosis and later on a pheochromocytoma.

Scoliosis

I vaguely remember being told that I had neurofibromatosis (NF1) around the age of five to six. I recall the café-au-lait spots being counted. Combined with the scoliosis that I had, it was a confirmation for NF1. When I was five years old, I had to undergo my first surgery. Surgery had to be done to correct the way my spine was growing. Without correction my heart and lungs would not have had room to grow. The first surgery I had my first Harrington rod put in my back to start the process of correcting the curvature of my spine. Following surgery I also had to wear a Boston back brace twenty-three hours a day. I wore this from kindergarten to eighth grade. I had to wear a white undershirt underneath the brace and a shirt over it. The brace became a part of everyday life for me. I would feel weird not wearing it.

When diagnosed with scoliosis many options were considered. One hospital wanted to put me in a body cast, which was definitely not an option my parents wanted. The way things worked out was amazing. A doctor in Atlanta came to hunt every year to the small delta town of Tchula, where my grandparents lived. He came twice a year to visit my relatives and to hunt. He just happened to be a pediatric orthopedic surgeon who had performed hundreds of surgeries on children like me. The doctor was Raymond Morrissy and he was placed in my life for a reason. He performed all the surgeries on my spine as I grew up.

I traveled to Atlanta over the years for surgeries and measurements for new braces as I grew. The surgery at age thirteen was a spinal fusion and thoracotomy that lasted eleven hours. The last surgery involved removing part of the right rib, collapsing a lung and finally fusing the spine with two permanent rods.

Spinal surgeries can be dangerous, of course, like most surgeries. I’ve had a great deal of pain from these surgeries. Each time I had to learn how to walk and balance myself with the new instrument in my back. I didn’t complain but continued forth even though I had to learn to walk again after each new adjustment. The final surgery had a longer recovery period, and I missed thirty days of school. When I went back to school, I went to school half days for a few weeks. It took about a year to feel well adjusted to the new rods after the spinal fusion. I still remember what happened the day of my final surgery on Oct 3, 1995, when OJ Simpson was found not guilty. The first thing I asked my mother when I was I brought into the ICU was “What’s the verdict? Is he guilty or not guilty? It was on everyone’s mind that day, it was the trial of the century. I am glad that I don’t have to undergo any more back surgeries, and truly appreciative that I can walk and live a normal life.

Complications of Neurofibromatosis

I also have brain tumors on the optic nerve and brain stem. The brain tumors that I have behave differently with neurofibromatosis, as they are slow growing and apparently haven’t changed in size since the doctors first found them around age six. They put me on a wait-and-see approach to these tumors. I’ve had around nine MRIs on my brain and countless x-rays on my back. I actually forget about the brain tumors because I have lived with them so long without their symptoms. So, in essence, all of this has prepared me to face trials in my life because I have been safely brought through all of this by God’s grace.

Pheochromocytoma

To tell my story about pheochromocytoma, I will bring you back in time to three years before initial diagnosis at the end of 2005. I was told by a new doctor that I had high blood pressure. He put me on a beta-blocker called Inderal LA 80mg (otherwise known as propranolol) and told me to come back at the end of December for a follow up. This happened to be a day or two before I was taken off my father’s insurance. I remember my blood pressure still being high, but he chalked it up to blood pressure being higher at the doctor’s office. I did return a few more times for a various blood tests because he couldn’t explain my high blood pressure. I was working at the time and the medicine slowed me down, but I continued taking it.

I will jump ahead to February of 2007. I was having some massive headaches and thought it might be the brain tumors causing this. I went back to this same doctor, and I still remember him not believing me when I said I had a brainstem glioma. I believe his exact words were “There is no way you could have one and still be alive.” I was, of course, frustrated that he didn’t believe me, so I did the next best thing. I called up the pediatrician’s office and had all my records faxed to him. I still remember his nurse and his face after they read through all the medical records. They finally believed me and were concerned. He set up an MRI and, of course, the same results showed up. He was then very concerned. He said the tumor was in the pontomedullary junction (brainstem) and right thalamus and arranged for me to go to an oncologist at UMC. In April he increased the Inderal to 120 mg, which I asked specifically if this was going to slow me down more because the affects of it currently, and he said “no”, that it wasn’t going to have that effect. Well, one week later I was in a car accident, and I know my reaction time was slower than normal and my anxiety level was extremely high.

I went to the neurooncologist appointment the next week and went through the standard evaluation tests, they do to test reflexes, coordination etc., when you are suspected to have a brain tumor. I of course, was completely normal, and all they had was results from a radiologist, since apparently they never received the MRI from St. Dominic’s. I told the neurologist and oncologist I was having these rapid headaches that come for a quick few seconds and then stop, which didn’t happen all the time. Well, little did I know that these headaches were not caused by my brain tumors; it was most certainly the pheochromocytoma.

I was in my second car accident on August 1, 2007, when a hit-and-run driver crashed into the front end of my vehicle. I decided on this day to stop taking the beta-blocker without informing my doctor. The medicine was making me feel terrible and after reading many articles about it I realized it was used for PTSD and could effect memory. I also decided to eliminate caffeine in my diet and drink only water. I know stopping this medicine was the best decision I could have made because if you have a pheochromocytoma you are not supposed to start a beta-blocker before an alpha-blocker. The consequences can be dangerous and I’m glad I stopped when I did. I dismissed the high blood pressure and went on with life.

On April 13 of 2008, little over a year later, I was in my third car accident. I was rear ended and pushed into another vehicle. I went to the emergency room the next week on May 1 because I was experiencing a lot of lower to upper back pain. I had to get an XRAY done to check on the rods in my back to see if they shifted or were causing other issues. I noticed my blood pressure being checked frequently. I had blood pressure of 180/120, and the nurse practitioner there gave me a 0.1mg catapres pill (apparently she was doing the clonidine suppression test). They continued taking my pressure about every fifteen minutes for the next two hours. When they checked it for the last time it had lowered to only 160/120, barely putting a dent in the high numbers. I was told I had high blood pressure, and I told the nurse, “I know, I stopped taking my medicine last August.” She, of course, did not like that response and scolded me for it. She prescribed a 30-day prescription, and I started taking it again. After the visit, I knew I had to get my blood pressure checked out.

I was able to get an appointment on May 7 with Dr. Andy Brown, who was the doctor treating my father’s high blood pressure. He told me to use a blood pressure monitor and keep a log for the follow up appointment the next week and to stop taking the beta-blocker. The next day I went to a follow-up appointment at the rural health clinic for the emergency room. I decided to take the medication one more time because I wasn’t feeling well and thought that might help. I was waiting forever it seemed at the clinic, and I pulled out the blood pressure cuff and took a reading. The result was 218/137. Dangerously high! I informed someone at the desk that I needed to see someone right away. I went back to a room, and the nurse took a reading, and it was still high at 218/135. The doctor and nurse didn’t seem as concerned as the nurse in the emergency room had been.

I went to the appointment on May 13 to see Dr. Andy and gave him the recordings I had jotted down. I was given a jug to start a twenty-four urine test to measure levels of catecholamines and metanephrines. I was called to come in for a follow up appointment on June 11. They also called my father so he could be there at the appointment with me. I knew something was up and looked up what a positive result would be for the twenty-four hour test. The results all said pheochromocytoma, and I knew that was what I most likely had and why my father needed to be there. I don’t think it bothered me reading that result because I thought to myself What is worse than a brain tumor?

The day rolled around and the doctor said “You have a pheo-” and I finished the word and said “pheochromocytoma.” The metanephrine levels were 4526H (with normal levels at 44-300 mcg/24hr) and the epinephrine was 438H (0-25 mcg/24hr). Andy was asking me questions about symptoms I might be experiencing: sweating more than normal, headaches, and flushing. I now know why he was asking those questions. I asked to get a CT scan after the appointment to try to find this tumor. He scheduled a CT without contrast, and I waited about twenty minutes for the results. The results showed a 6cm mass

on my right adrenal gland, which confirmed where the pheochromocytoma was hiding. He gave two options: to have surgery at the hospital or go to the National Institutes of Health, otherwise known as NIH. I heard from an oncologist friend of my aunt who said that the NIH was one of the best places to have it done.

He referred me to an endocrinologist on June 25 to see Dr. Christian Koch. I went to see Dr. Koch the next day and he looked over the results. Again I heard, “You have a pheochromocytoma.” I informed Dr. Koch that I wanted to go to the NIH. He agreed and said he would call up Dr. Pacak and I would hear about it the next week. I agreed to be in this protocol because it would help others who have these rare tumors, and I wanted to do my part. I received my schedule for scans for the end of July, the following week. The week was long. I had to undergo certain scans for the research protocol, including four MRIs, three PETs, a CT, and MIBG scans. All of this eventually did take a toll on me. I had a room in the Safra lodge to myself because I wanted to be alone and not have a TV on or anyone talking to me. I just wanted peace and quiet. I made it through all the scans easily, but I just wasn’t used to this many in a short span of five days. The key to scans is lying perfectly still even if you have to for two hours. It is really not that bad, just something I knew I had to do.

I had my surgery date set for August 26 at the NIH. I also took preoperative medications (Phenoxybenzamine, Demser, and Atenolol) two weeks prior to prepare for the surgery. The reason for this is because if medication is not taken and the tumor is touched without the proper blockade it could send your catecholamine (blood pressure) levels through the roof and you could die on the operating table. I had to undergo an open surgery as opposed to the normal laparoscopic approach, primarily because of the tumor size and, most likely, the two rods in my back. I was of course nervous for the upcoming surgery. I tend not to think about it, and suppress outward feelings. I usually become more visibly nervous the night before my surgeries. I am used to the no eating or drinking after midnight rule, which adds to the nervousness. I know not to be afraid of surgeries since I’ve had them from a young age, it is something that is easy for me to do, almost routine. I came out of surgery after four hours, went to the ICU for about three days, and finally moved into a regular room. The tumor ended up being 8.5 x 6 x 5 cm when it was removed, bigger than originally thought. I did experience pain and discomfort after the surgery, but at least I had pain medicine. I was in the hospital for five days and left Bethesda, MD, on September 2 to fly back to Jackson, Mississippi, to recover.

As I finish writing this, it has been four years since the removal of the pheochromocytoma along with my right adrenal gland and life definitely seems like it should be, normal. I have no idea how I missed the symptoms the tumor was causing, but with it gone, I feel better. I know it wasn’t the Mississippi humidity that was causing me to sweat it was the tumor. Also, it is nice to see my blood pressure and pulse drop to normal levels, without any blood pressure medication. I know the trials that I faced are small in comparison to others out there, but what I have faced has only made me stronger. These health issues have defined who I am and without them, I don’t know where I would be today.

I am still grateful for the doctors in my life. I am especially thankful for Dr. Andy Brown and his team for diagnosing this tumor so fast, as others I know have taken much longer to get a diagnosis. Another thing I found out a year later that my endocrinologist, Dr. Koch, was part of the protocol at the NIH when it

initially started. I know he was incremental in getting me to the NIH because of the connections he has there. The whole experience at the NIH has been great, which I would like to thank Dr.Pacak, Karen Adams, my surgeon Dr. Mary Beth Hughes and the rest of the team there. I know I received the best possible care there for the removal of this tumor. I only did what anyone would do and sought out the best treatment they could receive, especially for a tumor this rare.

I want a cure for this disease and hate to see anyone suffer from the effects of these tumors and hope one day there is a cure for it. I know there is a purpose for my life because I am still here despite odds not in my favor, but I won’t let those slow me down. I will continue to spread awareness wherever and whenever I can so that one day no one else will have to go years without a diagnosis.