"Gerry, something's not right. We need to go home."
It was Friday, January 2, 2004. It usually takes a lot to pull Sharon from her video poker games and live music at the piano bar at the Orleans Casino, but if she said it was time to go home, we went. The next day we were supposed to leave for San Diego so she could be with our daughter Amy and help her out in the final days of her pregnancy.
Sharon has always been one to get hit with colds and flu bugs worse then anyone in our family, but this time it seemed even more so. I asked some people at work if they could recommend a good doctor (we had tried others since we moved to Las Vegas and Sharon was not impressed by them) and Marsha, in the accounting department, recommended her doctor. I called him and was able to get her in the next day. We figured we would just get some antibiotics and be done with it. Sharon was impressed, however, because he asked quite a few questions and wrote everything down. He gave us a prescription and ordered a chest x-ray because of his concern with her shortness of breath. The diagnostics center was just down the street, so we went there after leaving the doctor's office, stopped off to pick up the antibiotics, and went home so she could get back in bed. Early that same morning Amy gave birth to a healthy grandson, JD.
For years, we knew something was wrong with Sharon. In my mind, it seemed to start back in the summer of 1988 when she was working planting flowerbeds in Minnesota and suffered a heat stroke that landed her in the hospital. Shortly thereafter was carpel tunnel surgery, along with the Raynaud's syndrome in her hands. She also started seeing a rheumatologist. He mentioned something about having a connective tissue disorder, but he did not mention any particular names, or if he did, she did not write it down. She was taking steroids for the arthritis and procardia for the blood circulation to her hands and feet. Then the heartburn started. While we lived in Minnesota, she was scoped and stretched once, and told of the dangers of Barrett's Esophagus. More drugs were prescribed for the acid reflux.
For years we had talked about moving south to get away from the cold. I was just getting sick of the winters, but for Sharon, we believe it was survival. Our youngest, Amy, graduated from high school in 2001, so the three of us made the move, leaving family and friends behind. It was about a year after we moved that Sharon started losing weight, once again having problems with swallowing, along with irritable bowel syndrome. Another scope and stretch, and she was eating again. I guess both Sharon and I knew something was wrong. It seemed like every time we turned around something else was going wrong with her body. She would often comment how she wished they could figure out what was wrong, give her the pill to fix it, and she would feel fine again.
In late fall of last year, we went to the dentist because the nerves of some of Sharon's teeth started to become exposed. The dentist sent us to a periodontist, who in turn said before he could do anything, she would have to see a doctor about getting a physical. He was concerned for her dry mouth that had pretty much destroyed her taste buds and was causing the receding gum line. He thought it could be diabetes, but he admitted it was only a guess. So Sharon told her doctor, along with all her other ailments she had had over the years, and he took notes. The next day, he called Sharon and told her about his findings. Sharon was still pretty groggy when he called, but she was able to tell me that the doctor had found in his medical book something called CREST syndrome, and some other word that she did not write down. He also said that he had seen her chest x-ray and had some concerns. He also wanted blood tests, breathing tests, an EKG, and an echocardiogram.
After dinner, I went to the computer and searched the internet for 'CREST syndrome'. I could not believe what I was reading. It was as if they were talking about Sharon on each web site I went to. I asked Sharon, who was still feeling pretty sick and in bed, if the doctor had mentioned "Systemic Sclerosis". She said, "No, it did not sound familiar," so I let her get back to sleep. I did, however, run off a copy of some of the web sites and left them on the table for her. The next day when I came home from work, Sharon was in tears. She said that the word her doctor used was scleroderma, which was also all over the papers I had run off the night before. She also pointed out to me the parts on lung involvement, which indicated the latter stages of the disease. We had finally found the cause for all her ailments over all these years, only to find that there is no pill to make it better.
That Friday, I took her in for the blood tests. Sharon said that she had never had so much blood taken for testing. The next week was the trip to the heart specialist. When he brought me in with Sharon to talk about the ultrasound pictures, he said everything looked fine, that was, until we mentioned scleroderma, at which point he made sure to get an appointment set up for February 18. He talked about setting up a base line for future visits. We were then suppose to go in for her lung capacity tests at the end of January, but Sharon ended up having a 'flare-up', so they put off the tests until February 19. But since we were there, I asked the main question, "Did the blood tests tell us anything? Is she officially diagnosed with scleroderma?" He showed us the blood report which stated "consistent with scleroderma." It was now official, although Sharon and I knew it already.
Well, yesterday was February 18th, Sharon's forty-fifth birthday, and the second visit to the heart specialist. After the tests, Sharon was told that the right side of her heart was enlarged which is "consistent with someone with her condition." Today we went in for her lung capacity test. Sharon said she should have practiced for it, that she could have done better. She just about passed out emptying her lungs into that machine, especially trying not to cough. Then came the medication, followed by another capacity check. The nurse made it sound like everything went well, but, of course, she would have Sharon's primary doctor come in to talk to us. We could tell right away he was not comfortable. He told us, "The tests indicate massive blockage, and even with the medication we applied, the results did not change.' I got the impression he was ready for us to start pleading with him for some sort of relief for her, but both Sharon and I had been reading the literature, and knew what he was talking about.
This Saturday will be our first support group meeting. We are both wondering what to expect, but at least we will be able to talk to people who know what is going on with us. And next month we will be seeing a rheumatologist who is suppose to be able to handle scleroderma patients. So we will see if she has anything to help Sharon's constant coughing and the other pains associated with this disease. Of course, we pray she will make it to the appointment. This thing seems to be progressing rapidly.
~ Update 4-14-04 ~
Things have been pretty eventful since I last wrote. Within a week of Sharon's birthday, her breathing became very labored, and she ended up in the hospital for a week with pneumonia. Her heart specialist softly scolded her, saying that she is a sick girl and if she ends up with a fever and difficult breathing again to get in right away. The way he put it, her lungs should be taking in four liters of air, but her disease has her limited to two liters, and she could take another half a liter off due to the pneumonia. Her newly assigned pulmonologist was also pushing for oxygen therapy after her stay, but she somehow managed to get out without it.
The next month included an angiogram, a sectional CT scan, a twenty-four-hour urine test, and numerous blood tests. She has about a half dozen different prescriptions that she is taking for circulation, high blood pressure, and digestion, along with using nitroglycerin paste to help dilate the blood vessels in her hands.
Then came the first visit to the pulmonologist since she was in the hospital. Like everyone else, he asked her how long she had been smoking, which she answered with her usual, "Never have." He looked at the sectional CT scan and then checked her oxygen level with a finger monitor. He showed us on the x-rays what pulmonary fibrosis looked like, and how far it had advanced. He started to talk about taking biopsies, but I think we talked him out of that idea. What was it going to tell us, that yes, she had fibrosis? He gave us the "We will wait and see." But Sharon was not happy at all when he prescribed full time oxygen therapy, and scheduled it to be set up in our home that afternoon.
She has been on oxygen for about a month now, and she does admit it has helped her energy level. She has gotten over the stares of people who saw her with the tank dolly. Just last week she was tested for using a pulse regulator, which allows her to use a smaller tank, one that she can carry. It is still a bother, but at least she does not have to use the tank dolly.
Sharon also has been having problems with swelling in her legs and feet. Her heart doctor prescribed a double dose of diuretics, along with potassium, and it seems to help, but she still has her bad days.
We have another support group meeting this weekend. We missed the one last month due to our moving Amy and JD into the house. Our son-in-law is in the Navy and will be deployed out to sea for six to eight months, and the timing worked well. It is nice having someone at home with Sharon, and, of course, Amy does want to be with her mom and JD is a real cutey!
Sharon still likes to hang out up at the Orleans, though we do not stay out as long as we used too. All of her friends have been very concerned and supportive, and if anyone asks, she tells them the oxygen is a temporary thing. But she does hide a lot of pain, which she tells me about but keeps from the outside world.
~ Update 09-20-04 ~
Since I last updated, things have not gotten any better.
In May, Sharon ended up in the hospital when her local lung doctor accidentally punctured her lung while trying to take a biopsy. When we went to see him in a follow-up visit, he came out and asked her if she was on oxygen at home, and HE is the one who prescribed it. We have since changed our lung doctor.
We started going to see the specialists at UCLA. We saw Dr. Furst, one of the country's top scleroderma doctors, along with doctors in the pulmonology department. They ordered an echocardiogram with bubble study be done of her heart, which we were able to have done here in Las Vegas. When we went in to have it done, Sharon ended up with a TIA (slight stroke), and ended up in the hospital overnight. A CAT scan showed that she had previously had a stroke, and the bubble study indicated a PFO (hole) between the halves of her heart. Sharon's younger sister, Becky, had a TIA about two years ago, and ended up having a PFO closure device installed intravenously. This is also what the UCLA doctors suggested.
At the beginning of August, we found ourselves in the hospital again with pneumonia. We had just seen her cardiologist earlier that day and she appeared fine. We told him about the flu shot she had just received, and he warned us that the shot would only protect her from certain strains, and that we still needed to be careful. That same evening, her temperature soared, and we drove her to the hospital. You should have seen the look on the doctor's face when he visited her in the hospital the next day! We all knew he did not have anything to do with her getting sick, but he felt awful for bringing up the possibility. He did point out how quickly things can change with her illness, and that we were right to get her to the hospital as soon as possible.
We also discussed the PFO closure with Dr. Leo, who immediately thought it to be a bad idea. With her pulmonary hypertension, he told us that the PFO is something of a pressure relief valve, and that to close it in her could be devastating. We told him that before anything was done, they would need his approval, which they received after some phone calls and reassurances.
The day after Labor Day, we left for Los Angeles. The PFO closure was set for Thursday, but we had to have it done a day before so they could insert a catheter to get her pulmonary blood pressure and try to lower it by the use of drugs, we believe the result of conversations with Dr. Leo. They seemed pretty confident, but it was not to be. Because of her fibrosis, the pulmonary blood pressure remained high no matter what drugs they used. Now her stay in the hospital changed from installing a PFO closure to checking her out to see if she would qualify for a lung transplant.
At this time, we are waiting for official notice from the UCLA transplant program, though, from the conversations we had with the doctors, we do not have our hopes up. Her stay in the hospital did take its toll, leaving her drained. While there, she had some morning fevers alarmed the doctors, and she had another TIA. She ended up staying two days longer than we planned, and it took its toll.
She is starting to get her energy back, though slowly. We saw her rheumatologist the day after we returned, and she went over Sharon's new drugs with her, along with writing a prescription for a wheelchair and tub transfer bench.
While in the hospital waiting room with her second bout of pneumonia, Sharon laid the big one on me. She said, "If anything happens, do not let them bring me back." Talk about a hit in the stomach. But, with all that she has been going through and the pain that she is in, can I really blame her for the request?
I have one of two wishes, the first being that this terrible disease somehow reverses itself. The second would be that she suffers no pain if my first wish is not granted.
In Loving Memory of Sharon, by Gerald
I am sitting here at my sister Jean's computer in Minnesota. Earlier, she and our sister-in-law Mary were discussing how Sharon's diagnosis first came about a year ago, and I asked her if she had read mine and Sharon's story at www.sclero.org. She must have missed the link I sent with the email last year. But the subject reminded me that I owe you all an update, and while I wait for my laundry to dry, I could be typing.
First off, let me start with a happy story. While we were at UCLA last September (see above), I walked by the comedian Louie Anderson more then once in the lobby of the hospital, but I was to scared to go up and say anything. I ended up having to drive home to Vegas on Sunday evening leaving Sharon in the hospital, figuring I would drive back when they were done with their testing. I ended up driving back to UCLA Tuesday morning, and along the way I made the decision to talk with Louie Anderson if I ran into him again, deep down figuring, "What are the chances?". As it turned out, they were pretty good. Sure enough, there he was again. I figured, "What is the worst that could happen?"
I went up to Louie and said, "Hey Louie, I am a fellow Minnesotan who just wants to say "Hi." He was totally friendly, asking me what I was doing there. I told him that my wife was in the cardiac care unit having problems with scleroderma. He told me about how he had done some fund raisers for scleroderma with his friend Bob Saget, who's sister had died from the disease. I asked him why he was there and he told me he had a dear friend in a coma and he was standing vigil (The friend ended up being Rodney Dangerfield, who past about a week later). Then I asked him if he could stop in and see my wife Sharon, to which he said, "Of course." I did give him an out, saying that I would understand if he did not have the time, but he insisted that it was no bother.
I went to Sharon's room, only to find her upset with the doctors, nurses, and the roommate. I listened to her complaints, while insisting she clean herself up. I got her a washcloth and her comb, and she looked at me like I was crazy, but I insisted she clean up.
When I went to the doorway, she looked even more puzzled. But when Louie walked in, she let out a big "LOUIE!!" He gave her a big hug, and she had tears of joy running down her cheeks. He talked with her about Minnesota, about scleroderma, and about our seeing his New Year's show a few years back. She always bragged afterward how Louie gave her TWO big hugs (I did not tell her how I got one downstairs!). Louie Anderson was always a favorite of mine and Sharon's, but this made him a favorite of all our family and friends.
Back to Sharon's story. We went back to Vegas, where now a wheelchair was a part of our going out. In addition, our daughter Amy's husband was soon due back in San Diego after his six month deployment, and her and JD really needed to be with him for awhile. Of course Sharon thought she could take care of herself, but I still sent out an SOS to our friends back in Minnesota, along with a call to the Red Cross to see if our son Garret could be sent home for family hardship leave.
Garret was home within two days, and within five days they had him temporarily assigned to a local recruiting office. Nice to have him at home, but no help during the day while I was at work. Sharon's sister Becky, made another two week visit, which she extended to three, and our daughter Gina came out for two weeks. Gina also ended up taking a six week leave of absence so she could be here at Christmas. Sharon's brother and sister-in law came out, along with my sister-in-law Mary and our friend Margie. And for those days when I did not have someone to watch Sharon, my boss made clear that a day off was not going to hurt anything, and made sure that I knew it would not be counted as vacation.
In the meantime, Sharon's doctors were trying their best to try and control her swollen legs and feet, a result of congestive heart failure, and she was taking bosentan, which our insurance company paid for under our major medical program (thank goodness!). But, Sharon ended up in the hospital again with breathing problems a week before Christmas. They got it under control once again, but this time, we had a meeting with a hospice councilor. It took three such meetings before Sharon finally agreed that it was time for their services.
After New Year's, again we had family and friends come out to both visit and help out. Amy and grandson JD came up frequently to both help and to visit. My parents were here for two weeks. Gina came out again. We all knew Sharon was in a lot of pain, and if she needed pain killers, she got them.
By now, Sharon had gotten to be skin and bones from her rib cage up, but the swelling in her abdomen was getting worse by the week. At the beginning of February, I asked the hospice nurse (away from Sharon, of course) how much longer did Sharon have. She responded with three to four weeks.
It was also about that time that I came home from work to find Sharon in a deep sleep. I did get scared when at first she did not respond to my shaking her, but she finally opened her teary eyes. She could not move right away, and it took a while for her to even talk. Finally, she was able to tell me about her dream about the bright light, and how she had to fight off the angels from taking her into the light. I wonder now what would have happened if I had not awakened her.
For the last two weeks in February, Sharon's sister Becky and niece Nichole came to town once again. Sharon's hospice nurse could not believe how it raised Sharon's spirits. We went out to eat, shopping, and even did a little gambling up at The Orleana, Sharon's favorite.
A day after Becky arrived, we got news from home that Sharon and Becky's mother was in the hospital with a heart attack. She did pull through, and was even discharged to a nursing home for a few days. However, two days after Becky arrived home, on March 1, 2005, their mother died. Sharon told me, "Well, Mom got her wish. She did not bury any of her children."
You could tell Becky's visit took a lot out of Sharon. She started drifting off to sleep more often, and her appetite had diminished. The hospice nurse was now saying that it could be any day. Back in Minnesota they were holding off on Mom's service because of an aunt being on vacation, but also because they believed we may be having a combined funeral. On March 8, they set their mother's service for that coming Friday, March 11.
That Wednesday, it took especially long to get Sharon dressed in the morning. Amy, who was with us for the month of March, called me home at 11:30 am, scared because Sharon had passed out. She did snap out of it, but her fingers were deep blue, which I knew was a sign her heart and lungs were giving out. We put her oxygen mask on her and turned it up to ten liters, which helped some, but she did sleep off and on all afternoon and evening. Her breathing was hard, and she did not have the energy to hold her head up. At about 10:00 pm, I told Garret and Amy to go in and tell Mom goodnight. They knew what I was getting at. Little JD was staring at Grandma's mask, but she was able to smile and lift her eyebrows at him, which got him to smile. And she was able to answer the kids "I love you."
I went to bed, and I remember looking at the clock at 12:00 am, checking on Sharon, and going back to sleep. But at 12:30 am, all of a sudden I was wide awake. I listened, but heard nothing.
Sharon died on March 10, a day before her mother's funeral. That Sunday, we had an open house in Vegas for our new friends, and her funeral was last Friday, March 18, 2005, in Anoka, Minnesota.
Well, it is after 2:00 am now, and I will wrap up this update, and this story. But before I go, I hope Sharon's ordeal can make everyone with this terrible disease aware of the importance of yearly chest x-rays and echocardiograms, and whatever else your doctor requires. I believe if we would have known Sharon had scleroderma years ago and we would have been doing these tests, Sharon would have had at least a few more years, possibly longer.
~ Update 04-01-05 ~
In Loving Memory of My Mom, by Daughter Amy
My mother passed on March 10, 2005, of this disease. I am her youngest daughter, and I was there when everything went downhill.
My mom was struggling for a good year and a half with this disease. In the past two months of her life she aged quite quickly, and when she passed away she looked like she was pregnant with triplets due to the fact that when her internal organs stopped working for her, they would sit in the pit of her stomach. But we learned to accept that, because it didn't seem to bring my mom down at all and she wanted her family to remember the way that she was before she got so ill.
So, now I'm happy to report that she's in no more pain and that we really appreciated all the support given at the support group we attended and the information we received on this site and all the others. I want to let others know about her struggle, so they realize how serious this disease can be.