March 2012- Aug 2013

So after my appointment in Redwood City, my boyfriend at the time suggested we drive over to Half Moon Bay to hang out at the beach for a while and avoid the evening commute back home. That night he proposed to me on the beach while the sun was going down. It was the best night of my life. We decided to have the wedding soon because I knew the radiation would start  making me sick within about six months and I did not want to be too sick to enjoy our wedding. I started planning right away while recovering from brain surgery and radiation We had an amazing engagement party, thanks to my incredible parents.

In September 2012 I went back to work. Keep in mind, I’m still recovering from my brain surgery, still recovering from radiation, starting work again, all while continuing to plan the wedding. And most people think just planning a wedding is stressful! Whoa, I was a busy lady.  Within a few weeks of going back to work I got scheduled to have a sinus surgery. Each time I have a brain surgery, they go through my nose to get to my brain. During the surgery, they break my nose then cut open a section of skull behind my sinuses and get to my brain that way. It is way better than cutting my forehead open from ear to ear but it has its own downside. Because I have my nose broken each time, and there are all sorts of instruments they use during surgery that go through my nose, my sinuses get pretty damaged. I had sinus surgery in October 2012 and a month later we had our wedding. By the time the wedding was over, Thanksgiving was over and we were home from our honeymoon, I started to feel sick again. I called my neurosurgeon and told him how I was feeling. He said this was great news and it probably meant that the radiation was working and that I would get much more sick before I got better. Although that seems backwards, it was true. As different functions of the gland stopped working it would make me more sick, but then I could take medication to regulate things and feel better again. I thought ok, I can deal with that. I knew it wouldn’t be easy but this was just one more hurdle I would get through.

At this point the term hypopituitarism was thrown around. Hypopituitarism is when more that one hormone in your pituitary gland does not work right. I was there. My ACTH and thyroid levels were not working and then I found out that the hormones that made it so that I could get pregnant stopped working as well. So after all this, surgeries, radiation, my body not working right, I lose the ability to have children on my own. Damn I hate this disease. All I have ever wanted was to be a mom. My major in college was basically how to be a housewife, (not really but it was all about family). I have always wanted to meet the man of my dreams, get married, have children and have a happy life. Now not only have I lost the last 5 years to this stupid disease, I can’t have kids on my own even if I wanted to. There was a small hope that fertility treatments would work, but it was small and expensive. Right about this time I started noticing that everyone around me was getting pregnant, or having a baby or having their second or third baby. Although I was happy for these people, it hurt so so bad.

I try pretty hard to stay positive about everything I have been through. I strongly believe things happen for a reason. I know that there is a purpose to what I am going through. I haven’t quite figured it out yet but I know there is a bigger plan. I have seen so many times that something that devastates me or something that doesn’t go exactly how I wanted it to, always ends up turning out better than I could have thought, and always ends up teaching me something. God’s timing is pretty amazing. I know he sees the bigger picture in all of this but sometimes I have bad days. Sometimes I wonder why. I don’t often ask why me, but there are times when I just really don’t understand why I have to be going through so much pain while other people my age are not even aware of how good they have things. How lucky they are to not be sick.

I have lost so many friends since I first became sick. People who I was very close to stopped talking to me. They stopped being friends. I learned quickly that if someone didn’t know what to say or how to respond to what I was going through, they just said nothing or did nothing. I don’t need people to feel sorry for me, or to feel bad, I just need people here for me. I need a text or a card or a phone call to let me know they are thinking of me. I need a hug, or a visit to say hi. I don’t always have the energy to be the most exciting friend but just because I am sick doesn’t mean I can’t be a good friend. Just because I’m not going out all the time doesn’t mean we still can’t hang out. Besides losing a lot of friends, I have also been blessed by learning who my best friends are. People who I barely knew when I got sick, that completely stepped up and did everything they could to help me and be supportive.  People who I can always count on to be a friend even when I’m not being the best friend I could be. They understand that I’m not always feeling good enough to go out or even get dressed some days. They understand that I have a hard time planning for fun things because I never know how I am going to be feeling from one day to the next. They have been the best people. When I am having a rough time, and they haven’t heard from me lately, they make contact with me and it lets me know they aren’t forgetting about me. Thank you to those few people who have stuck by me even when I’m not super fun to be around. You have no idea how much you have meant to me.

So as April 2013 started getting closer, a year from when I had radiation, I was still feeling really sick. There was no sign of me feeling better. I knew in my gut something wasn’t right but the doctors had told me I should expect to be sick so I just went with that being the reason I wasn’t feeling good. I took a leave from work in April for what I thought would be two months. As I was off work, I just kept getting worse. Finally, I contacted my doctors and had them run my labs again to see if there was any improvement with anything. Much to all of our surprise, not only were things not improving, but my levels were actually getting worse. My endocrinologist and neurosurgeon were both really confused. They knew I would be feeling more sick, but it was not normal for my actual labs to be getting worse. They consulted again with a ton of doctors. Tons! They contacted the radiation oncologist who had done my radiation and asked for his opinion. He said that although the radiation might take 5 years to fully kill everything, there should be some improvement a year after treatment. Because I was getting worse it was determined that the radiation had failed to kill the tumor. At this point the doctors started wondering if I might have ectopic Cushing’s disease, which basically means there is a tumor somewhere else in my body, or in a part of my brain they had not already cut out or radiated, that could be secreting the excess ACTH.

Never have I said this disease was easy to understand. Sometimes after all the research I have done, and living with the disease for so long I feel like I am more of an expert on the disease that most doctors I come in contact with. I have even had some doctors tell me that I probably know more about it than they do.  I’m not sure if I have mentioned this or not but Cushing’s is a very rare disease. Roughly 5-10 people out of every million people have been diagnosed with Cushing’s. At this point, I have had multiple doctors tell me that I am probably 1 of 10 people in the world…the entire world….that have a case so rare. That makes thing’s very hard for a number of reasons. First because it is a rare disease, there is not much information for doctors to rely on when it comes to the best treatments for this disease. Second, it is really hard to have a support system of people who have been though what I have. If you think of people who have had cancer or a stroke or a heart attack, there are tons and tons and tons of support groups for those people. There are events everywhere raising money for research and for a cure for those diseases and health issues. And while all of those diseases are extremely serious and scary and fatal, this disease is too and yet I bet more than likely, anyone who is reading this has never even heard of it. A little piece of me wishes I had the strength to organize a community run to raise awareness about this disease and make money to support research. They have no idea what causes this disease (other than in some cases people who take too many steroids can have similar side effects). I had never taken steroids before I was diagnosed. There are hardly any answers for someone who is diagnosed. At the point I am at with this disease, there are no answers. The doctors are learning as they go along. There are differences of opinions among different specialists. No one has the right answer. When I do my own research now, there is not much that I can find anymore about a situation just like mine, not because I am not looking in the wrong places, but because it’s not there…there are not that many people like me any more.

So after learning that the radiation failed, and that I may have a tumor somewhere else in my body, or that it may still be in my brain, my doctors all got together again to try to discuss what the next option would be. The options at this point were a bilateral adrenalectomy, or there were a few medications that some patients tolerated but they didn’t work forever. My endocrinologist referred my to a general surgeon to consult with him about having both adrenal glands removed. This has been what I have been dealing with all summer. Most people think about concerts, and lakes, and rivers and boating and bikinis and fun summer activities during this time… me, well I get to decide if I should try some pretty crazy medications or have a life altering surgery to cure a fatal disease. So after my surgery consult, we decided that I would try a drug that is pretty well known for helping people reduce the amount of cortisol in their body. So I tried this drug knowing that it would not be a long term solution but that it might help for now. I took the drugs for three days and after the first night of taking them I slept for almost 18 hours. Then, the next day the same thing happened. Then the day after that, and the day after that. After the third day of the drugs I stopped taking them but for three weeks I slept between 16 and 18 hours a day. To me, that is not living. Even if I was awake, I was too tired to get out of bed and if I did make it out of bed I would lay on the couch and fall asleep again. I was so exhausted and weak it was horrible. My doctors decided that drug would not be a good option so they started me on another drug that was experimental. This drug was so strong that you only took it twice a week because it stayed in your system for so long. After two doses of this drug I started having issues with my heart and had extremely bad pain in my left arm and it would go numb all the time. Then my hands and feet would fall asleep all the time. When I would wake up at night to roll over or to get up to go to the bathroom( which because of this disease I have to go about 6-8 times a night..real fun) I would immediately start sweating and get a horrible headache. My doctor decided that drug would probably not be a good solution either. After both of these drugs, the last option was to have my adrenal glands removed. There are no other options at this point that the doctors have to help cure this disease.

A  bilateral adrenalectomy is a very rare surgery. First off, your adrenal glands sit on top of your kidneys. A lot of people have one gland or the other gland removed but typically not both. I was told this surgery is performed a few times a year across the entire United States. You can’t live without at least one adrenal gland. This surgery is what they call an end organ surgery because the problem is not actually in my adrenal glands. The problem is the tumor, but because they can’t find the tumor and it is still sending messages to make cortisol, we have to get rid of the source of the cortisol. Removing healthy organs is not normally something doctors like to do, but in my case this is the last option. I always research everything that my doctors tell me. When trying to research this surgery there is not a lot that refers to having both adrenal glands removed. It’s rare. What I have learned from the doctors and from the research is that your adrenal glands make Cortisol. By having the surgery my body will no longer make Cortisol. I had mentioned earlier that you need cortisol to survive. Now I have too much and after surgery I will have none. So I will have to take medication to have cortisol in my body. This medication will keep me alive and I will have to take it multiple times a day for the rest of my life. The tricky part is that I can’t take too much and I can’t take too little (because you get very sick) but that depending on if I have extra stress in my life (physical, mental or emotional) I will have to adjust my dose. There is not a way to measure how much I have on a constant basis so it will all be up to how I am feeling. Now, most everyone takes some kind of medication and some people are on medicine that they will have to take indefinitely. The difference between this medicine and those medications is that if you miss a dose or two you may feel a little sick but typically it is not cause for an emergency. Usually you can miss a few doses and although it is not ideal it won’t kill you. With this medication, if I miss a dose or two, or don’t take enough when I have more stress, I will most likely end up in an emergency room and it can become a life or death situation. It is similar to a diabetic in that way. If a diabetic misses their medication it can turn into a serious situation quickly. The difference with diabetic medication and the medication I will need is for one, most diabetics can test throughout the day to know how much insulin they should be taking and secondly, if something does happen to them, it is a well known disease and most doctors/ hospitals have medicine easily available. I will have no way to test my levels, and the medication I need is not always a medication that is easily available. So to say the least this has not been an easy decision to make. The decision to end a disease that will eventually kill be by having a surgery that will literally keep me dependent on drugs multiple times a day for the rest of my life. Pretty big decision.

By not having adrenal glands I will have a new disease called Addison’s disease. This is a fairly rare disease as well, although some people have it because their adrenal glands stop working, so it is a little less rare than Cushing’s. I am scheduled to have both of my adrenal glands removed on September 10,2013. I will most likely have 8-10 incisions, 4-5 on each side of my ribs to remove each gland. If all goes the way it should, I will be cured from Cushing’s disease that day. The strange part, is that I will still have a brain tumor and will have to continue to watch that. It is possible that it could grow and I will have to have another brain surgery in the future to try to remove it again.

I have left out of lot of little details, especially about my wonderful support system and the numbers of appointments, procedures, tests, complications and details about my day to day well being. I’m sure I’ll have comments on all of that stuff in the future. I did just check this morning and I have had 36 doctors appointments just since Jan. 2013 and that is just clinic visits, not including er visits, getting prescriptions, MRI’s, or lab work. I have a permanent spot on my arm from all the hundreds of labs I have had. If you have any questions I actually love to talk about everything I have been through. I would rather someone ask me a million questions and be well educated on my disease than to assume they know something and have judgments.