Brent has done a wonderful and thorough job recounting my health problems this past year through his blog. However, I wanted to share my experience from my own perspective, so here it is.
In early June of last year my endocrinologist diagnosed me with diabetes insipidus, in which the body doesn’t hold onto water as it should. Later that month I saw my primary care doctor for a gash on my shin that wasn’t healing. After taking my blood sugar, doing some lab tests, and consulting with my endocrinologist, I was diagnosed with type 1 diabetes. I went through the entire gamete of keeping close track of what I ate, counting my carbs, recognizing the symptoms of hyper- and hypoglycemia, checking by glucose levels, and giving myself insulin shots.
This was not the end of it. I was experiencing an onslaught of symptoms: problems with cognitive function (thought process/memory/concentration), lethargy, fatigue, muscle weakness and atrophy, abdominal fat accumulation, mood swings, anxiety, dizziness, loss of balance and coordination, easy bruising, slow healing wounds, weight loss, and increased blood pressure. Finally a state of severe confusion and disorientation in mid-August prompted my doctor to call an ambulance to take me to the emergency room. Apparently my ACTH (adrenocorticotropic hormone) levels were excessively high, causing high levels of cortisol, a stress hormone. This resulted in a diagnosis of severe cushing’s syndrome.
Four days after I was admitted to the hospital I experienced a fall onto the floor in the middle of the night. My neurologist believed the fall was due to low potassium (which was as low as 2.4 mEq/L in June) and high blood pressure. He suspected a seizure and immediately started me on Keppra, an anti-seizure medication, just in case it happened again. No one could ever find any evidence, nor did they have any reason to believe I actually had a seizure. I was in a dark hospital room with trip hazards including various cords and an IV pole. I was “confused,” and wasn’t being monitored. Yes, of course I fell down.
The neck fracture I sustained crushed my C5 vertebra, knocked it out of alignment, and chipped off a bone fragment. Dr. Florman, my neurosurgeon, removed the C5 vertebra, added a spacer from a cadaver, and screwed in a titanium plate to fuse together the C4 and C6 vertebrae. After the surgery, fluid built up in my lungs, and I had to be put on a ventilator. The experience of facing death was incredibly frightening and surreal at the same time.
Just to add to the list of medical maladies, I was diagnosed with extreme osteoporosis. This explains why I had been losing so much weight, and have been so weak and having so much bone and muscle pain. My endocrinologist, Dr. Rockwell, ordered bone density tests which showed my z-score to be -3.8 standard deviations (another way of saying “off the chart low”), and my L4 lumbar vertebra was -4.3. She started me on estrogen and Fosamax (which is supposed to keep old bone from being broken down by osteoclasts and thus preventing risk of fracture). I soon replaced the Fosamax with a daily injection of Forteo, a bone building medication which stimulates osteoblasts to regenerate new bone, thus increasing bone density and bone strength. My oncologist noted from a PET scan that I have a lot of compression fractures in my spine. Most noticeably one at T-12, and most recently T-8. Dr. Keller from osteopathic manipulative medicine came in during my most recent hospital stay (2 weeks ago) and “manipulated” my spine and muscles/bone around it. It felt wonderful, and I can see the benefits to engaging in this therapy regularly.
Dr. Rockwell, said that at one point I had the highest cortisol levels she’d ever seen at 9,000 mcg/dL (normal being 5-23 in AM and 3-16 in PM). She then said “You are about as metabolically deranged as they come.” A few days later my primary care physician, Dr. Mahoney, told me I was “the sickest, most complicated patient” she’s ever seen. I found this all a little disturbing.
After some tests and scans I learned that I have cancerous lesions on my liver. However, they did not appear to be the source of ACTH production. My doctors determined that the only way to quickly reverse the dangerously high levels of cortisol was to remove my adrenal glands. So at the end of August I endured a bilateral adrenalectomy. Since then they have discovered cancer in my iliac bone and in the lymph nodes in my neck. I had the lymph nodes surgically removed. However, surgery on the iliac and liver is not an option because my body is too weak to sustain major high-risk surgery and because I could have cancer in any number of places which has not yet been identified. I spent four weeks in the hospital and another two weeks in the New England Rehabilitation Center.
The oncologists up here are not very familiar with neuroendocrine cancer, which is what I have. It is in the class of neuroendocrine tumor (NET) cancers because it secretes hormones (ACTH in my case). At PopTech this year Brent had the opportunity to meet and speak with Dr. David Agus, who was Steve Jobs’ doctor. He said that my cancer and situation made me a very rare case. He referred to me as a “zebra,” which is considered the mascot for the NET cancer community. He said Maine hospitals have no experience with zebras, and strongly advised us to go to Boston. We got a referral to see Dr. Matthew H. Kulke, who is the Director of the Program in Neuroendocrine and Carcinoid Tumors at Dana-Farber Cancer Institute and an Associate Professor of Medicine at Harvard Medical School. Dr. Kulke is an expert in NET (Neuro-Endocrine Tumor) cancers. He only sees zebras.
My particular case is especially puzzling even to the experts at Dana-Farber. They believe I have the only known case of metastatic cancer of the pituitary gland. Dr. Kulke said that the ACTH-secreting tumors are very likely to have metastasized from my pituitary tumor which I had treated with multiple surgeries and radiation several years ago. It was thought to be benign, as they typically are. I told Brent that I should no longer be referred to as a zebra, but instead an okapi.
My only option at this point was chemotherapy. I started the Temodar Dr. Kulke prescribed in early February, which is a pill form that I take for five days of a 28-day cycle. The side effects are not as severe as traditional chemotherapy, although the first night or two of each round I’d be nauseous and vomiting constantly for several hours. The pain management recommended by Dr. Kulke has greatly improved my days. However, the dosage was evidently too toxic for me because it brought my platelet count down dangerously low, and then brought down my red blood cell count as well. Once my blood levels were back up to a normal range, Dr. Kulke put me on a lower dosage which seemed to have no toxic effects, and also appeared to have shrunk my tumors according to the PET-CT scan I had done.
After starting the third round of Temodar, I experienced serious hypertensive crisis episodes. I was talking to Brent on the phone about three weeks ago, and feeling perfectly fine; then suddenly I felt an intense rush of pain and pressure in my head which quickly moved to my abdomen and chest. I was sweating, nauseous, and dizzy; I felt my heart pounding; and I was extremely anxious. I suddenly couldn’t talk, and started panicking. This worried Brent, and he called an ambulance to take me to the ER. I felt like I was going to die. Shortly after, I heard a banging on Brent’s office door, then men yelling. Brent later told me that Skye didn’t even know I’d left, and Hayley told him that a man in a black shirt and black pants with a flag on his shirt came and took me away. It had to have been scary for her! My blood pressure in the ambulance was 220/120. I was given IV meds for pain and to reduce my blood pressure, and I was taken for an MRI and X-Ray. Nothing out of the ordinary was seen on the scans, and I continued to have several more of these hypertensive crises during the week I was in the hospital.
Several of my doctors have suspected that the hypertensive crises are due to effects of Temodar (my chemotherapy medication) in which catecholamines are being released from the shrinkage of tumors. Catecholamines are hormones released into blood during times of physical or emotional stress. The physicians also suggested a pheochromocytoma and carcinoid syndrome, both of which were ruled out, as they showed up negative after several tests. Whatever the cause, I have added both daily and emergency blood pressure medications to my lengthy medication regime. I certainly like puzzles and a good mystery; however I don’t like to be one.
I appreciated the many visits I received from friends, and regret that I was not always in a good state to socialize while I was in the hospital. While I was having so many cognitive problems related to my illness and medications, it was difficult to communicate or understand what was going on around me, or even recognize who was in the room. Sometimes I would go from being somewhat talkative and upbeat to spiraling down to a state of disorientation, incoherence, panic, or exhaustion. I realize it may have made some people uncomfortable and even scared.
The girls have all been very strong and so willing and eager to offer their help and companionship. I know they each worry about me and about Brent, especially when I am having an especially rough time with my pain. I hate for them to watch as my health declines, and know that I may not live for very much longer. They have been resilient and positive, although I know it is difficult for them as they have each individually expressed to me their worries, concerns, and feelings. It breaks my heart to see them worried and sad. It also saddens me to think I might not be here for them and see them through the many changes they will go through as they grow up. They have mentioned their desire to spend more time with me, and I absolutely look forward to and enjoy the all moments we have together. I love them each so very much!
Brent is my rock, and has been amazingly strong and supportive throughout this entire ordeal. He has sacrificed so much. He is working hard and trying to manage our finances to make up for my lost income and medical insurance, and paying for increasing medical bills. He’s missing out on the things and activities he enjoys, like flying, snowboarding and photography. He thinks and worries about me every day, constantly. I try to imagine his fear and anguish when I nearly died after surgery in August. Although I am no longer in hospital critical care, it is very hard on him to see me in intense pain on “bad” days and declining health. I can no longer do and will never be able to do many of the things we enjoyed doing or planned on doing together. One day I will be gone, but Brent must continue on, and thinking about the future. Not knowing how soon I will die, or how sick I will be between now and then makes it very difficult to make any plans.
I feel that I have been doing significantly better the past few weeks. My doctors’ recommendations for pain and symptom management have made a huge improvement in the way I feel. Brent and I have taken many trips down to Dana-Farber in Boston, and have many more to come. I am feeling more positive and hopeful. Mostly I want to live well, without being a burden or worry to my family.
This was our summer, autumn, and winter of discontent. Spring is supposed to be a time of new life and regrowth. We will wait and see.