ACC: An "Orphan" Disease

In an earlier post, I attempted to give you a medical lesson on adrenal cortical carcinoma. I mentioned that it was rare. Actually it is termed, ultra rare. As an ultra rare cancer, this disease receives little to none (more none than little) government funding for research. Unfortunately, rare and ultra rare cancers fall behind in resources and funding, so finding a cure for these types of cancers will not happen without the help of government authorities such as the Institute of Cancer Research. Treatment for patients with these rare cancers (aka orphan diseases) fall far behind others due to the limited number of individuals diagnosed each year.


As mentioned earlier, Al's treatment of choice was chemotherapy. Since his Cushing Syndrome was so out of control, chemotherapy is not an option for him, at least for now. His plan B was the chemo drug, Mitotane. I have since found out that this drug is a derivative of DDT which is a well-known pesticide. It is also marketed as an "orphan drug" due to the small number of patients in need of it. Also, Mitotane is a 40+ year old drug that was used to treat breast cancer patients that many years ago. As breast cancer treatment has wonderfully progressed, ACC's treatment is still the 40 year old drug. To me, it is insane that my husband's only choice of treatment is a 40 year old pesticide!


While I can't fund the cure for ACC, I can help in some small way. While doing research on ACC, I went to Lance Armstrong's Foundation, Livestrong. Adrenal Cancer is not even listed as one of the diseases in their library. I hope one day to contribute to that site and lend some "expert" knowledge on ACC. Until then, I have made a donation to do my part to find a cure. Our family is wearing the yellow bracelet tagged Livestrong in honor of Al. Won't you make a small donation and do the same?

Quick Update #4

I'm sorry it's been a week since I posted anything of significance, but by the time I get home at night I just want to sleep! Next week I am going to make an effort to write while I am at rehab with Al and hopefully this blog will be more timely.

As you know, Al is back at Holmgreen for rehab and this time he is up for the challenge. As we now know, his last attempt at rehab was too soon. Apparently, the Cushing Syndrome was not stable enough for him to focus on regaining some of his strength. He was still overwhelmed with fatigue, weakness and loss of appetite. This time around it's another story! The Cushing is stable, so he is ready to put the effort forward to get stronger. He is focused and ready for physical therapy. He has already had two sessions and it is completely different between this visit and the last one. Earlier today he complained that he was not getting PT because it was Saturday. He thought that was ridiculous and griped about it for an hour. As we both were dozing off...PT knocked on the door and told him it was time for his therapy. If he could have jumped out of bed, he would have to beat them to the gym. As for me, I stayed in the room and continued that nap!

In summary, he is doing great. He is focused, stronger and hungrier. Continue praying for his therapy sessions as we both want him to come home soon.

Roller Coasters Scare Me

I am not a fan of roller coasters. I guess I was when I was younger, but as I matured, the need for speed also matured. It is amazing how these young kids will stand in line for hours just to get on the latest and greatest of these rides. I think the last time I got on a real roller coaster was when the whole family took a trip to Disneyworld in 2004. Al and I rode Space Mountain, which in today's terms is a relatively mild ride. Even though it looks like I was having a blast, I really was secretly counting the seconds until the ride was over.

I have been on a roller coaster for the last 12 weeks. It is up and then down. Currently, we are up, but even I know that most roller coasters have a sudden drop. I'm tired of the drops. As Dr. S says, "we need to catch a break". But as I say, "we need to catch a miracle".

One Step Forward, Two Steps Back

Al was so excited to be discharged from the hospital last Friday. As my last post indicates, he checked into Holgreen for some rehab. He adjusted really well there especially when we got to his new room and he saw the full-sized bed with air mattress. No more twin size for this 6'3" and "large" sized man.

Rehab began really well. The first day he sat up in a wheelchair for about an hour which was more than he had done in the last two weeks. They also took him to their gym where he attempted to stand up. It didn't happen the first day but it did the second day. He was pumped. Also, he began other therapy to strengthen his leg muscles.

Friday after physical therapy he took a nap, but I could tell he was restless. He woke up not feeling very well. He felt that he was struggling for each breath. By early evening his pulse oxygen level was down to 89 and then 84. That is a serious drop. Holgreen called the ambulance and we were back in the ER. We were in the ER for four hours and he was very sick the entire time. He was struggling for each breath, sweating and he had a 101 fever. Diagnosis: fluid in the lungs/pneumonia. This time we were sent to ICU, or as I call it, the "Expensive Care Unit". He got excellent care there and after two days he said he felt the best he had in weeks. After two days, he was moved to a regular room and we are optimistic that he will continue to improve.

Obviously, there is a pattern here. He is on the granddaddy of all antibiotics, and it can only be given through an IV. Within a few days of coming off the antibiotic he gets sick. This time, his doctor will run the usual course of treatment through the IV and then he will keep him in the hospital for another 48 hours to see how he reacts.

Al is disappointed because he feels he was making progress and was anxious to begin extensive physical therapy. On a  positive note, he also feels that he is able to concentrate and focus much better than in the previous weeks. Look out managers...I'm sure he will begin calling each one of you to check on your departments.

Sleeping Alone

I don't know about other wives, but I stay with my husband when he is in the hospital. It is not the most pleasant experience, but at least we are close by. When he was in the hospital in October and November I slept every night in a recliner that pulled out into a "bed". The "bed" consisted of a big lump between cushions that settled on my lower back. If you wanted to roll over, chances are that you would hit an elbow or arm. In other words, it was the worst sleep possible.

This stay in the hospital, we were admitted on Christmas Day. I think the charge nurse felt sorry for us, so she put us in a semi-private room with no roommate. It was early in the day and we stayed on our side of the room but kept one eye on the door to see if we would be sharing facilities with some other poor soul that had to spend the holiday in the hospital. By early evening no one appeared, so my eye roamed toward the empty bed. I sheepishly asked the charge nurse if I could sleep in the bed. She said yes and I jumped up and down with delight. I was going to sleep in a bed in the hospital! Lord, thank you for showing me favor.

The next night, I asked her again and she gave me the same answer. I brought my own blanket, laptop, iPad, Kindle and house shoes. This was luxury to me. We stayed together in that room in separate beds for 8 out of the 10 nights he was recovering from pneumonia. A friend and Brian pinch hit for me the other two nights. Don't get me wrong, I had my moments of frustration and exhaustion. You cannot sleep in a hospital, especially when nurses and aids and technicians are coming in the room every 20 minutes. I griped, but my husband wanted me there and of course I wanted to be there so we made the best of the situation.

We escaped the hospital tonight and Al moved into a skilled nursing facility ALONE. It is a very nice place, much nicer than the hospital, but they discourage overnight visitors. He is there to regain strength and they encourage him to do things on his own. If he needs water, he needs to reach for it himself without his wife jumping up and getting it for him. I must confess that earlier in the day all I could think of was sleeping in my own bed and how wonderful it would be. I changed my mind after I had to say goodnight to my husband. When I left there, I cried for him and for me and for the space cancer has put between us.

New Year's Resolution

This time of year everyone is vocalizing their New Year's resolutions. Every year I have the same ones: Lose weight, eat healthier, exercise more and give up Sonic Dr. Peppers (regular, not diet). This year, I think I will have only one: To try not to worry.

My super optimistic husband always says: "Don't worry until it's time to worry". Well, I think that women are programmed differently than men when it comes to worrying. Who usually stays up and waits for the kids when they are out at night? Usually the Moms. Who is the first to call the doctor when their child is sick? Moms.

With my husband's illness, I worry about everything. Was today's chest X-ray better than yesterdays? Was his potassium level up or down? Are his legs too swollen or not swollen enough? And the list goes on...

When you love someone it is easy to worry about their health and comfort. I have come to realize that you can only do so much. The rest is up to God. Put it in his hands and stand back and marvel at the outcome. I tend to do this better when I remember one of my favorite scriptures: "For God hath not given us the spirit of fear; but of power, and of love, and of a sound mind." 2 Timothy 1:7.

Happy New Year and praying that 2011 will be a year of miracles!