I underwent an abbreviated session of IVIG. I was referred to a HematologistÂ and underwent my first bone marrow biopsy. I did not know what to expect, so I am assuming I was a bit more relaxed for the procedure. It went well and yielded negative for lymphoma. I consulted with a RheumatologistÂ who explicitly told me he felt that I had medically unexplainable physical symptoms, told me how he was a Harvard graduate, and how he was usually right 99% of the time, and I emphasize, “99% of the time.” Â When I left the examination room to use the lady’s room, he went on to tell my husband that he felt I was suffering from severe depression and wanted to refer me to a Neuropsychiatrist. Despite the fact he had an excellent reputation in his specialty area, I noticed he was taking notes on plain white sheets of paper with a thick black magic marker. In all my travels thus far, I do not believe any doctor recorded notes using an offensive smelling magic marker.
I went back to the Neurologist for a follow-up visit. I do not think he had seen me in a few months. My husband went to the appointment with me. We were called into his office. He turned to me and said, â€œLook, it is the incredible shrinking woman!â€ What was he thinking, or was he really even thinking at all? This angered Barry immensely and caused him to really question his integrity and professionalism.
My condition did not seem to improve much despite new treatments. I was determined to find an answer and an appropriate medical treatment. I began consulting with many NYC specialists including Rheumatologists, Hematologists, Cardiologists, Endocrinologists, OtolaryngologistsÂ and Neurologists. Some doctors dismissed my case, others cited me as suffering from severe depression, and others were completely perplexed, as I was not black and white, did not fit neatly into a box and was certainly not a textbook case. However, it did not stop my determination or diligence. I consulted with a wonderful Rheumatologist who knew I was gravely ill, who wanted to try to help me but was quite baffled by my case.
I began experiencing tremors and shakes. I was referred to an Endocrinologist in NYC for a consultation. He continued to yawn and was seemingly nodding off during the consultation but amazingly heard every word I said. He tested me for blood sugar issues and diagnosed me with reactive hypoglycemia. I now had to adjust my eating habits and begin eating a low carb diet consisting of small, frequent meals. This was a huge adjustment for me, as most of my favorite foods were rich in carbohydrates. The pounds started to dramatically shed between the low carb diet and the disease process itself.
A thymic mass was detected in a scan. It may have been a tremendous clue which was perhaps not looked into carefully enough for connections to my health issues.
I began consulting with Pulmonologists and Thoracic Surgeons. Opinions were mixed regarding how to proceed. I was carefully monitored over the course of the next few months.
I was referred to another Hematologist/Oncologist who also saw the urgency of getting expert medical care. It was decided that I should be sent out to the Mayo Clinic for evaluation.
My girlfriend KE had become instrumental in my daily struggles. She provided me with a sense of optimism, care, concern and extreme levels of comfort. As a Hebrew School Teacher, she had a close connection to one of the Rabbis in the community. I asked her to arrange a private prayer session in my home. That afternoon was filled with laughter and wonderful conversation amidst weeping and fear. At one point, the three of us formed a small circle, clutched each others hands, and the Rabbi chanted Hebrew prayers as the tears strolled down my face. I was trying to grasp anything I could. It was a very memorable day.
I planned to head out to the Mayo Clinic. I was extremely anxious about the trip, flying out there, leaving my children for an unspecified amount of time and the whole unknown. My husband stayed behind with the kids and my mother-in-law. My mom flew out with me from NY, and my sister met up with us a day later from Florida. The trip going out there was fine. I was prescribed a very low dose of Valium just to calm my nerves and make it through the flight. I made sure I was prepared with food to constantly snack on, as blood sugar level fluctuations were an issue since being diagnosed with reactive hypoglycemia. The flight was long, we had to take connecting flights, but nevertheless, things went smoothly. On the second leg of the plane, we actually flew at a pretty low altitude. It was amazing to see all the grassy plains and the beautiful wide open spaces. There were also many doctors on board headed out for a conference, so I felt especially comfortable. We stayed at the MarriotÂ which was connected directly to the main facility of the Mayo Clinic. The building was spectacular, and one staff member was kinder than the next. I had registered and was then given my itinerary for week one. I was assisted to my first appointment by transporters in a wheelchair. As I traveled through the building, I was told the most heartwarming, hopeful stories of complex health crisisâ€™s that were ultimately solved by Mayo Clinic doctors. I really felt as though I was in very good hands. I was registered under RheumatologyÂ so the RheumatologistÂ would ultimately be my case manager. I was scheduled to also be examined by dermatology, neurology, and oncology. I spent the first week undergoing what was termed to be a million dollar work-up. Some of the tests were quite unusual, my favorite being the sweat test. The nurses allowed you to pick your favorite CD, and the sound quality inside the testing chambers was awesome. After almost 10 days of intense evaluation, I returned back to the RheumatologistÂ who summarized all departmental findings and designed a treatment plan. I was diagnosed with mixed connective tissue disease, alopecia universalis, and Raynaud’sÂ Phenomenon.Â The treatment would be a gradual building up of a low dose of methotrexate(an oral form of chemotherapy) to try to bring the disease process under control. The plan was forwarded to my NY doctors, and I was scheduled to begin my regimen as soon as I returned. It was time to leave Minnesota and head back to NY. I ate an early breakfast, took my vitamins and took a Valium in preparation for the return flight. Despite the fact that I was gravely ill, security gave me a difficult time about my bottle of Benadryl which I needed to carry for allergic reactions and a bottle of water. We finally made it through security in this tiny airport and awaited boarding in the gate area. My stomach began to feel sick; I became extremely nauseous and started vomiting. I ripped off my wig and got as comfortable as possible, replacing it with a cotton covering. A nurse approached my mom and sister to provide some comfort and let us know she would be on board with us. The plane was extremely small and could maybe accommodate 50 passengers. It was also blazingly hot on board. Sometime into the flight, I began to feel really sick and felt as though I was going to pass out. I let my mom and sister know I was possibly in need of oxygen. The stewardess went to the back of the plane and asked K for assistance. K was the nurse on board who frequently traveled to the Mayo Clinic with her young daughter who was being treated for a very rare disease. K laid me in the aisle at the front of the plane, took my pulse and requested an oxygen tank from the flight crew. Unfortunately, the first tank was completely empty, but the second tank was luckily in working order. K lightly administered the oxygen holding the mask slightly away from my face. She stated my pulse was threadyÂ and made me also sip very slowly on some apple juice. K contemplated having the pilot make an emergency landing before our scheduled destination, but I began to stabilize. We touched down in Chicago and made it through the first leg of the flight. The paramedics came on board to deplane me and check me out before giving me the okay to proceed on the next flight. My pulse and blood pressure were stable, and I ate a light meal before boarding onto the next flight. I felt much better. We boarded the next flight in preparation to go to NY. We departed from the gate area only to find we would need to sit on the runway for an hour due to a delay and the engines were shut down. The cabin quickly became hot and stuffy, and I was extremely sensitive to temperature changes. I asked the stewardess for some ice and some paper towels. For whatever reason, she only had a wet cloth that she had run under cold water to give me. That did not really help my condition. I was feeling sicker by the minute. The captain made an announcement that we would be returning to the gate due to the fact that there was an ill passenger on board. We got back to the gate, and I deplaned with my mother. Paramedics were again called, and I felt better after getting some fresh air and a snack. The management team was also called to the gate area, and the pilot refused to let me back on the plane stating that once they returned to the gate for a passenger, that passenger could not reboard and would have to be placed on another flight. Luckily, there were two empty seats on a flight that left shortly after the original flight, and the staff ensured I was placed in bulkhead seating with plenty of leg room. The cabin was almost frigid, and I was made to feel as comfortable as possible. I was so relieved when we landed in New York as was my husband, my children and my father who were our official welcoming and greeting committee at the airport.
I quickly built up to 20 mg.Â of methotrexate with no serious side effects. It was too early to really tell if the medication was working, as it could sometimes take 3-4 months to build up in your system. In the meantime, I started developing intense pressure in my chest and begin wondering if the thymus was continuing to grow. I was scanned again, and the size seemed to have remained the same, but the majority opinion was to have a radical thymectomy to remove my entire thymus. I had researched and researched, because there were just so many unknowns about the thymus. The doctors felt it was benign, but one doctor stated that benign could always turn to malignant. That statement, in addition to the discomfort, was the deciding factor for me. The surgery could be performed in one of two manners. There was a very invasive method whereby your ribs would have to be cracked or a newer cutting edge manner in which they cut three holes on the sides of each breast and went into your chest through the incisions. Of course, I opted for the newer technique and found a really fantastic and very compassionate surgeon at New York Presbyterian Hospital. Besides, heÂ was one of the surgeons who performed surgeryÂ on Bill Clinton, and I thought, if he was good enough for the former president, he was certainly good enough for me.
The upcoming surgery was consuming me, but not having a will was consuming me even more. I felt a strong need to have all of my personal affairs in order before going under the knife. My girlfriendÂ LR’s brother is an attorney, so I contacted him. My husband had very mixed emotions about making a will, but I was doing so even if it meant I was doing it solely. It did not take much convincing for him, and before you knew it we were drafting our will. Most of our decisions came rather easily. The most difficult decision of course involved the kids. Although you hoped and prayed it would never happen, the what ifs were certainly a reality for us. What if we were both deceased when the children were young? Who would be granted custody? We spoke to our family members to make sure of certain things and made what we felt was the best decision, although we knew in our hearts, we wanted to instill our own philosophies and values into our children. I also drafted a wish list which I told my girlfriend about. The list was rather short and my wishes rather simple and easily attainable, but nevertheless, it was my wish list. I personally owned it and wanted these very simple requests carried out.
I was the first one scheduled for surgery on the morning of March 17th. Coincidentally, or maybe â€œaccidentally on purposeâ€, this was also the date that I met my husband back in 1989, so it just had to be a lucky day. I had to stop the methotexateÂ two weeks prior to surgery which would disrupt the whole cycle, but it had to be done. My father and husband drove me in for the surgery. They began to prep me. By this time, I had traded in my bandanas for the much more comfortable and appealing chemotherapy caps. I could not go into surgery with my cap, and that was somewhat traumatic for me. They kind of became what a security blanket is to a baby. The disease process had just taken so much of a part of me, and the way I looked was sometimes more bothersome than others. The disbelief never really subsided, even though I saw myself in the mirror with each and every passing day and night. The doctor placed a sheer blue surgical cap on my bald head, and I kissed everyone goodbye. Once in the OR, the team explained everything they were going to do. The room was frigid. They marked my body with marker markings, strapped me down, administered the infamous sedation medication and before I knew it, I was completely out. I woke up in recovery and became violently sick. I began throwing up even the slightest sip of ginger ale. Once more stable, I was transferred to the Critical Care Unit where there was one nurse assigned to 2 patients continuously. By the next day, I was feeling a little better. My surgeon made his rounds to let me know things went well, he saw some lung nodules that were suspicious looking that he also removed, and now, we would just have to await the biopsy reports. I was connected to some machine with tubes inserted into the incisions to help with my breathing. All of a sudden, I found it very difficult to breathe and saw blood and fluids leaking down the side of my body. My family was there at the time and quickly got the nurse who made everyone leave the area. It turned out that one of the tubes came out of my chest. I found it amazing at how calm the nurse and doctors remained while I was basically having a nervous breakdown. During the second night in the hospital, I had a really bad allergic reaction. One of the doctors was called in to evaluate me, and I was given Benadryl to treat an acute allergy attack. If only someone would have made a connection there as well. With every new doctor visit, my husband and I always mentioned these severe allergic reactions that would cause hives, facial swelling, shortness of breath, vomiting and diarrhea. Were there any doctors that could have made the connection or in actuality should they have? The surgery was over, and I was recovering from it. The scars were fresh in my skin. It was though little by little I was being branded for my own death sentence.
The biopsy results were in. Thankfully, the thymus and lung nodules were all benign, but there was a prevalence of b cells in the thymus. Was this something to be concerned about? I went back on the methotrexate with relative ease.
Written by Tara Notrica
I was really inspired by your story in the MDS Foundation Newsletter. I was diagnosed with MDS RARS when I was 39 and have watched my numbers decline over the past seven years. Unfortunately, my brother and sister are not a good match for a bone marrow transplant.
So, Iâ€™m launching a podcast called Living Withâ€¦intimate conversations about hope and inspiration. Its an interview style podcast focusing on inspirational people fighting cancer and other illnesses. I would love to interview you for the podcast! If you are up for it, I would love to connect with you over Skype to talk about your journey with Mast Cell Disease.
All the best,