Merry Christmas!

Merry Christmas!
We hope you all had a very merry Christmas! We had such a nice time! Kim made Christmas Eve dinner for a bunch of us and it was so delicious. We relaxed and visited with each other. We spent Christmas night at Kim and Wayne’s home. We all used to spend the night at Nana and Papa’s until they passed away in 1994. I picked up Kevin after work about midnight and he spent the night too. Wendy and the kids had been over for dinner but were spending the night at home. Joshua took home cookies and carrots for Santa and his reindeer. I told him that the reindeer also love broccoli but that Santa frowned on feeding it to them. It makes them fart and Santa has to sit behind them....Joshua thought that was pretty funny. Cliff and Jenny spent the night too and we all had fun opening presents in the morning. Kim provides all kinds of breakfast drinks and fixings and is just a warm, fuzzy morning. We take our time opening presents one by one. I really enjoyed Cliff and Kevin. Kevin loves Cliff and they get along so well. Cliff just fits into the family like he was meant to be with us. In the afternoon we went to Linda and Bill’s house to share Christmas with my side of the family and have Christmas day dinner. Linda cooked another huge meal and it was delicious. Sue and her family were there, Thomas was back from Russia, Robert was there with Caleb and Wendy and the kids were there. Larry and Heidi and Karen were there too. I painted and framed a 16x20 deer picture for Larry and he loved it. I liked it so much I wanted to keep it for myself. Brian did great with all the festivities but tired easily. With both of our disease processes we sleep about 12 hours and now Brian is finding he needs a nap as well. Our home is a mess with presents and boxes here and there. I don’t like to put them away right away because I like to look at them and enjoy the memories. Wendy was over today and said she was already taking down her decorations. I like to wait until after New Year’s. Christmas isn’t just a day, it is a season and I am not ready to end it yet!

Grateful

There are so many things to be grateful for this time of year, all year in fact. I am grateful for the warmth of our cottage apartment, especially when it is so freezing outside. Being cold is so painful for me. I lose the circulation in my hands and feet and they turn blue and white. This is from Raynaud’s. It takes a while to get them warm and it is painful when the circulation returns. I know our home is hot to everyone else but it feels so good to me. Luckily, I just got on a discount rate from Smud for having to use oxygen and for scleroderma. Even then, I don’t think my bill will be cheap. But it’s only for a few months and then the sun will shine again.
Kevin spent Monday night with us and I made him and Brian brownies with homemade chocolate icing. They acted like it was the greatest thing since sliced bread. I am so grateful for the time we share with Kevin and Wendy and the kids. Sat. night was our Disney Christmas party and Wendy and the kids were there. Sunday, I treated Kim and I to the Singing Christmas Tree for her birthday and Kevin, Wendy and the kids went too. It is easy to just run through life, hurrying from one thing to another. Brian and I know that we can not take a single day for granted. Each moment is so precious to us and we try to enjoy each and every minute. I made Brian meatloaf for dinner, his favorite meal. He enjoyed it so much. Just a small thing as this is so fulfilling. I am so grateful that I can do little things like this to make him happy. Doing for others, from our hearts, has to be one of the greatest gifts we have as humans. That and the capacity to love. Together, I feel so good!

Christmas Movies

Brian and I watch every Christmas movie we can and many of them several times. Family channel and Hallmark have one or two movies every day. And they all make us cry at the end. If we were like the Grinch and our hearts got 2 sizes bigger every time we felt the Christmas spirit...why our hearts would be...well they would be HUGE! Brian even admits he likes Christmas more than Halloween now. And he is more emphatic about watching Christmas shows than I am. And always at the end, he says, “This is killing me!” The other night we were watching Home Alone which is one of my favorite Christmas movies. (My most favorite is How the Grinch Stole Christmas with Jim Carey. When he mimics Ron Howard’s directing I know I am in on a personal joke!) Anyway, at the end of Home Alone, Brian and I were both crying. Christmas movies are all feel good movies that make you happy to be alive. We have so many warm, fuzzy memories of Christmas’s past. We are so lucky to have such a warm loving family to share our Christmas’s with. Each movie we watch evokes those memories and makes us cry.

Christmas

Brian has always helped me shop for Christmas presents. When Wendy and Kevin were little, we would go to Toys R Us the day after Thanksgiving and shop for them. Brian and I would each have a cart, one for each of the kids and we could fill them up for about $100. each. I admit, that was 25 plus years ago. Brian always went shopping by himself for me. He always got me beautiful clothes and accessories. He has wonderful taste. He would bring his shopping home and delighted in showing his gifts to Kim and close friends. He wrapped his gifts himself and I had so much fun opening them on Christmas morning. Of course those days are over. I no longer work and need nice buisness clothes. Sweats are the norm now. The past few years, Kim has taken Brian to do his shopping and then she wraps his gifts for him. Brian has been fretting for the past two months over what he wants to get me for Christmas. He worries he doesn’t know what to do. I always assure him that Kim will help him and he is satisfied for that moment. We get paid the week of Christmas so Brian has to wait until then to do his shopping. Kim is off work that week so that works out fine. With all the other problems Brian could fret about, that he frets about Christmas Shopping for me makes me cry. It has never been what he has gotten me, but that he takes so much pleasure and makes such a wonderful effort to make sure Christmas is fun for me. That is one of the reasons I have always loved Brian so much.

Daily Living

Thanks to Catherine, Brian has been back on aricept for the past few weeks. However, he continues to show changes from Alzheimer’s. Where as before, the changes were on a weekly basis, I am seeing changes on a daily basis. Each day is a learning process. It is frustrating for Brian when he spills or makes a mess and he is so apologetic, saying over and over again how sorry he is. I tell him he doesn’t need to be sorry, it is his disease process and it is not his fault. But he does feel badly to not be in control of himself. I feel his sorrow for this loss of self and I try to make light of the situation for him. Alzheimer’s is such a mean disease. Brian is conscious that his life and memory are slipping by and he can’t do anything about it. He is not angry about it and only rarely shows frustration. It is scary for him. We continue to joke and laugh about it and that makes him feel better. The other day he said that that president had this. He meant Ronald Reagan. I told him that Ronald did go through the same things as he was experiencing and if Ron and Nancy could do it, so could we. We both thought that was funny and now when faced with a situation, I refer to ourselves as Ron and Nancy. While laughing helps a lot, it doesn’t always work. Yesterday, as I was cleaning up a mess, Brian said he didn’t know how I could handle things every day and he was sorry I had to do this. I told him that I do it because I love him and I know that he would do the same for me if our positions were reversed.

Oh, Christmas Tree

Kevin spent the night again last night and put up our Christmas tree. Our tree is older and is not quickly put together. First you have to figure out how the stand goes together. Directions came with the tree and are around somewhere, but I have always been a firm believer in doing first and reading directions later, if at all. Each layer of branches has a coordinating colored end such as blue, red, etc...But most of the paint has worn off so at best it is a guess which branch goes to which level. Each branch has smaller branches that have to be formed into a realistic looking pine bough. It usually takes me several hours to sort each pile of branches and then go through each pile and form each bough. Kevin, I found, has a different way of putting the tree together. He just kind of eyeballed each branch and put it in a pile of others approximately the same size, put all the branches on the tree trunk and then kind of spread them out. It was much, much faster than the way I have always put the tree up. There was a time when I might have told Kevin the right way to do the tree and insist he do it my way. It was very difficult to learn to accept help when it is offered and to lose control over the way things are done. I was grateful that Kevin did the hard work and I get to do the fun part, decorating the tree. I realize that there are other ways to do things rather than just my way and if the end result isn’t perfect, well, I have never seen a perfect tree.

Christmas trees

Kevin spent the night Monday night. He pulled out most of our Christmas stuff so we could start decorating. When we owned our two-story house, I decorated big time for Christmas after cleaning up the Halloween stuff. Kevin and I put tons of lights on the outside and the roof of the house. Thanksgiving night we turned it all on and it was (we thought) beautiful and festive! I did different things to the inside of the house. One year, I put a big tree in the living room, a tree upstairs in Kevin’s room and a tree on the roof. Looking through the windows from the outside, it looked like one tree went through the whole house. We always had lots of decorations and Kevin and Wendy would help decorate. Kevin was more into decorating though and wanted things in the same place as they had been the previous year. (He still does not like change). We had a Christmas tree in every major room. Kevin and Wendy had smaller Christmas trees in each of their rooms and the lights acted as night lights for them. They decorated their trees with their own ornaments. Kevin always went for lots of tinsel and bling. That is not his style now. Wendy’s style of decorating was more romantic, a style that she continues to this day. So now we have bins and boxes all over and Kevin will be back on Monday to help set up the tree..

Biscuits

Wendy and the Kids were over yesterday. While the Kids and I did crafts, Wendy did some cleaning for me and washed a load of clothes. I made a stoup (cross between soup and stew) and biscuits. Joshua and Melissa say Grandma makes the best biscuits, so I usually make them when they come over. My Grandmother made biscuits every night for dinner. She never measured her ingredients. She had a drawer in her kitchen that was lined with metal and held her flour. She would make a well in the flour and put all her ingredients into it. Then she mixed it up adding her milk until the consistency felt right. She would pull off a chunk of dough, roll it between her fingers into a flattened ball and put it in her metal biscuit pan. The biscuits would cook up light and fluffy. They were the best biscuits I have ever had. When I was a teenager and doing a lot of cooking, I asked Grandma for her recipe. She didn’t have a written recipe and just used a pinch of this and a pinch of that. I sat there while she made her biscuits and when she took of pinch of this and that, I measured it before she put it into the mixture. Grandma is gone but I still have her recipe and think of her every time I make biscuits. Bisquick makes a good biscuit that with a little tweaking, tastes every bit as good as Grandma’s, but I still like to make mine from scratch just like she did. Now, when I make biscuits, Joshua and Melissa bring their barstools over to the counter and make them with me. After washing their hands, we measure and mix and pat and cut. They roll the biscuit dough in butter and place it in my biscuit pan. The biscuits come out light and fluffy and practically melt in our mouths. It makes me so happy to know that Grandma’s biscuit tradition continues with the next generation.

Life

Ever since I was a little girl, I have said my prayers at night. I always thank God for Brian and I having such a good marriage, for my wonderful kids and family. The other night, after I said my prayers, I lay there thinking. I can understand my having Scleroderma and everything that goes with it. I would rather have it than have someone else in my family be sick. But I was wondering why Brian has Alzheimer’s. He is such a wonderful, loving man; why should he have to go through this? I have often wondered this, but until that night I had no answers. Then in a moment of clarity, it came to me. Brian depends on me for so many things. He always has. My love and support have gotten him through some difficult times. We have always faced everything together. We both know that we will not be here 10 years, probably 5 years from now. We have accepted that. But If I go before Brian, he will be devastated. He has family and friends to help him but you have to know Brian to know that he will not want to survive without me. I figure that God has given him Alzheimer’s so that if I go before him, he will probably be living in the past at that time and not in the present. His memories will sustain him until it is his time. I have to believe that there are reasons for everything. I think in Brian’s case, God is being merciful.
I don’t usually talk about my beliefs. When you are facing death, you have to plan ahead so that you can get the best of whatever is possible out of the time remaining. I believe there is a God. A few hours before my mother died, she said she saw "The good Guy", meaning God. She was not a religious person and to say something like this was out of character for her. I cannot look at the sky or at nature without knowing the miracle of life. My Catholic upbringing has given me my beliefs. My beliefs give me the courage and strength to live the best possible life out of my remaining time. All of us are going to die. Some of us are lucky enough to know when and are better able to prepare for it. I read somewhere that without death, life would not be so precious. That is so true.

Caring Voices

The company that gave me the financial assistance for Letairis is called the Caring Voice Coalition and may be found at http://caringvoice.org I thought it was called Caring Hands. My apologies.
According to Jean Sommers, Financial Team Lead, at the website, I will automatically be approved for financial assistance for Letairis in 2009. If I had been approved before Sept., I would have had to apply again. I sure hope that is how it works. It will be very nice not to have to worry about Letairis next year. Caring Voice was established in 2003. They offer financial assistance, education and caregiver support. I cannot say too many times, how grateful I am to them.
It is not just Letairis that is so expensive. Most of the treatments for pulmonary hypertension range from $30,00 to $150,000 per year. I am so happy I don’t have to go on Flolan as yet, if at all.
Flolan is administered through a permanent catheter placed in a vein in the neck or chest. It only lasts 3 to 5 minutes so it has to be continuously infused. A portable, battery-operated pump is worn attached to a belt around the waist or carried in a shoulder pack. It costs about $100,000 per year and as you can see, is very complex to administer. Home health care aids help patients maintain and administer the drug. Infections can be serious. Interruption of Flolan can be life-threatening. Side effects can include jaw pain, headache, flushing, nausea, diarrhea, and vomiting. As with Letairis, Flolan can increase a patient’s life expectancy. So, you can see why I am so grateful for a treatment given in pill form versus a treatment so invasive as Flolan.

Alzheimer's treatments

There are two types of medications approved by the FDA that treat cognitive symptoms of Alzheimer’s disease. Cognitive symptoms affect memory, language, judgment, planning, ability to pay attention and other thought processes.
Cholinesterase inhibitors prevent the breakdown of acetylcholine, a chemical messenger important for learning and memory. They can delay worsening of symptoms for 6 to 12 months, may more, for about half the people who take them. Aricept (Donepezil) has been approved to treat all stages of Alzheimer’s. Exelon, (Rivastgmine), and Razadyne, (Galantamine), have been approved to treat mild to moderate Alzheimer’s. Namenda, (Memantine), works by regulating the activity of glutamate, a different messenger chemical involved in learning and memory. It is the only drug of its’ type approved for moderate to severe Alzheimer;s to temporarily delay worsening of symptoms for some people.
Brian takes Namenda and Aricept.
For many individuals, Alzheimer's disease affects the way they feel and act in addition to its impact on memory and other thought processes. As with cognitive symptoms, the chief underlying cause is progressive destruction of brain cells. In different stages of Alzheimer's, people may experience: physical or verbal outbursts, emotional distress, restlessness, pacing and yelling, hallucinations, and delusions.
There are two approaches to managing behavioral symptoms: using medications specifically to control the symptoms or non-drug strategies. Non-drug approaches should always be tried first. These include recognizing that the person is not trying to be mean but these are symptoms of the disease and changing their environment to resolve challenges and obstacles to comfort, security and ease of mind.
Medications may be appropriate when individuals have severe symptoms or have the potential to harm themselves or others. Medications can be effective in some situations, but they must be used carefully and are most effective when combined with non-drug approaches. When considering use of medications, it is important to understand that no drugs are specifically approved by the U.S. Food and Drug Administration (FDA) to treat behavioral and psychiatric dementia symptoms.
As with all medications, talk to your doctor about what treatment may be right for you or the person in your care. A medication's effectiveness, and the side effects it may cause, can vary from one person to the next. For one individual, one drug may be more effective but have greater side effects. For another person, the same drug may be less effective but have no side effects.

Living with Alzheimer's

Brian’s newest symptom is frustration when trying to make himself understood. He will see something on TV and come find me to tell me about it and by that time he has forgotten the words. If I am watching the same program I usually can figure out what he wants to say. I have to change the TV stations for him so I pay attention to what he watches so that hopefully it is easier for him to make his point. "Writer" is actually David Hasselhoff who is guest staring on a sitcom. Brian recognized him from his old show "Night Rider."
Brian loves to watch certain TV shows and movies, although his attention span for movies now-a-days isn’t very good. He is not able to work the remote anymore, let alone put a movie in and play it. He used to sit and watch the TV for hours at a time, now he is in motion about ¾’s of the time. At first I thought he was just checking to see where I am, but he is up and down even when I am sitting with him. And as I have said before, we go to bed and he is up and down for about 20 minutes before he settles in for the night.
He can’t focus on reading and if he could, he can’t comprehend it. If I point at something, he doesn’t understand where or what I am pointing to. Once in a while when he talks to me, he faces the other way as if I am on the other side of him. He knows I am there, just not sure where. More often than not, I help him with getting dressed and using the toilet.
Brian hasn’t been depressed (that I can see) or angry which are common to people living with Alzheimer’s, or any chronic disease. Brian has always been a "what you see is what you get" kind of guy. He has always depended on me for financial matters, keeping track of social events and running our household. He is very comfortable letting me take care of him. He loves and trusts me and knows no one could do it better at this point in our lives. So he hasn’t had the fears that others have about what to expect with the disease process. His only expectation is that what ever happens, I will take care of it for him. It might sound simple but Brian is not a complicated guy.
Brian lost his job as a forklift operator early in the disease process. He tried temp work but wasn’t able to learn new skills. The last year he worked was in the garden department at Walmart. As his symptoms got worse, I would go in the back and write down his schedule for him and tell him when to work. He liked to get to work at least an hour early to familiarize himself with the store each day. I drove him and picked him up. He got where he couldn’t read anymore and when stocking shelves, just matched like items with like items not code numbers. If he had a major problem trying to find or do something, he would call me for help. He likes people and the other personnel loved him. We managed to keep his disease under wraps until he went out on disability.

Alzheimer's

I have done a lot of talking about my disease processes. Brian wants people to know about Alzheimer’s and living with that disease process.
Alzheimer’s is the most common form of dementia that as many as 5 million Americans have. It destroys brain cells, causing memory loss, changing thinking and behavior. It gets worse over time and it is fatal. It is the sixth-leading cause of death in the U.S.
By the time Brian went in for diagnosis, it was fairly easy for the doctors to see the damage Alzheimer’s had done to his brain. His CT scan showed his cerebral cortex, the outer layer involved in memory, thinking, judgment and speech. had shrunken due to the disease process. He wasn’t able to perform simple tasks such as drawing a clock or remembering three words that the doctor had given him to remember just a few short minutes before. Brian was already at the moderate stage of the disease process.
As we age, we have occasional problems remembering and maybe some slowed thinking. With Alzheimer’s, serious memory loss, confusion and other major changes in the way our minds work are not a normal process of aging. The brain has 100 billion nerve cells (neurons) that communicates with many others to form networks. These networks have special jobs such as thinking learning and remembering. Others help us to hear, see and smell and tell our muscles when to move. In Alzheimer’s, the networks break down and eventually they die. Scientists are not really sure where the breakdown starts.
Two abnormal structures called plaques, (protein deposits that build up between nerve cells), and tangles, (twisted fibers of another type of protein that form inside dying cells), are prime suspects in damaging and killing nerve cells. Scientists are not absolutely sure what role plaques and tangles play in Alzheimer’s disease. Most experts believe they somehow block communication among nerve cells and disrupt activities that cells need to survive.
Brian has early onset Alzheimer’s, meaning it started before he was 65. Brian is only 57. It may affect people as young as those in their thirties.
Alzheimer’s had no current cure, but there are treatments, such as Aricept for the symptoms. Most of what we know about Alzheimer’s has been learned in the last 15 years. There is an accelerating worldwide effort under way to find better ways to treat the disease, delay its onset, or prevent it from developing.

And Then...

In 1992, Kevin called me at work to tell me the fire department was there and I needed to come home. When I drove up, there were three trucks and four fire department cars parked out front. They had heard of our haunted house and wanted to see it. So Kevin and I gave them a tour. They were impressed but also offered several safety suggestions that we took to heart. That year was the first we used walkie-talkies so that we knew where everyone was at any time so in case of a fire we could tell the fire fighters. We made the second floor more accessible to exiting in case of a fire. It was another great year!
In 1993, a few hours before we were due to open for the Haunted House tours, we were told we could not open without a permit. We would have had to wait until Monday to get the permit and of course, by then Halloween would be over. After frantically making some calls, I found out the permit was necessary because it was thought we were charging money. Our signs clearly stated we accepted donations for whatever charity we were giving to that year. No one was required to pay to go through the house. It was just pure fun. We didn’t need a permit after all!
The first year all of our kids got involved, there was a huge storm the night before the party. They had worked tirelessly on a maze out in front of the house and a huge set of experiences from labrynth that went from the side yard all through the back yard. Wayne had built a pirate ship in the pool with skeletons for the crew (we even had a ship launching ceremony!). It was all in shambles and the pirate ship looked like a shipwreck. Everyone rallied together and we were able to repair a lot of the damage in time for the party that evening. The pirate ship we left to look like a shipwreck because frankly, it actually looked pretty good and intentional.
Each year we tried to out do the past year and we all were just getting exhausted. We decided that 1994 was the last year for the party and the haunted house. It was a bittersweet party but we have so many memories to last us a lifetime.

Halloween

Last night was Halloween. We spent the evening at our friends who decorate on a good scale. We go over for dinner and to watch the trick or treaters enjoy the props.
From 1976 to 1994, Brian and I had a Halloween Haunted House that really was incredible. We had a two story house and every room was decorated with a different theme each year. We couldn’t do it all ourselves. We have wonderful friends and family that helped us. We would start planing in April and start gathering supplies. Not having any budget, (or as we called it, "A beer budget"), we grabbed anything we could for free. We made most of our props because the wonderful Halloween props that are available now were not available then. We used tons of cardboard, latex, fiberglass and spray paint.
Work started usually in August with people over working every weekend. Kevin and Wendy were kicked out of their rooms and had to sleep in the family room downstairs for the next few months (they loved it!). At first we stapled and nailed cardboard right to the walls. Later years, we made frames to attach the cardboard to and reduce the damage to the house. I swear, if someone in a helicopter had flown over our house with a giant magnet, our house would be flying! And I am sure the local hardware store had his highest revenues during that time.
Wayne was in charge of building things for us. Charlie was in charge of making things move. Brian was in charge of the prop department, Mike was the Cardboard King and Colleen was in charge of keeping us supplied with chocolate chip cookies. The last Sat. of the month we had a grand party with as many as 250 people. Even though the party was so much fun, the most fun was in those weeks leading up to it. As the party got closer, then we all worked almost every night as well. We never laughed so hard or so much!
Brian and I tried to keep the use of our bed during this time so it was nothing to sleep with hundreds of spiders including a giant spider over our bed, as we did one year. Another year we slept with Lizzie Borden, and another year we slept with the plant from Little Shop of Horrors. At this time we were very involved with the local little league. The league needed to raise money so Brian and I offered to open our house for a few days to accept donations for the league providing the league helped us take tour groups through. We ended up making $700 for the little league that year so we continued to open our house for about 4 days each year to raise money for charity. We raised money for Earthquake Relief, Make-A-Wish and others. Being big Disney fans, we were able to send several ill children to Disneyland through the Make-A-Wish Foundation. We would ask for a $1.00 or $2.00 donation and no one was turned away for lack of money. Many of the neighbor kids came through several times a night. And then......I’ll tell you tomorrow!

Scleroderma and Pulmonary Hypertension

Scleroderma is a connective tissue disease. Collagen is the strong fibrous stuff found in connective tissue. Connective tissue connects and supports parts of the body and is dense with blood vessels. While Scleroderma means "hard skin" this same hardening can happen to an organ or tissue because of rapid growth of too much collagen, which is my case. Most often death with Scleroderma is caused by pulmonary disease. About 150,000 to 300.000 in the U.S. have Scleroderma in the U.S., striking 4 times as many women as men. The cause of Scleroderma is unknown and there is not presently a cure. Scleroderma can lead to PH because it can cause scar tissue to build up in the lungs or heart. Roughly a third of systemic Scleroderma patients may develop mild PH. When mild, it can often go undetected for a number of years, not progressing. When symptoms are prevalent such as increased shortness of breath, it is much easier to diagnose and also much harder to treat. It is not known how often mild PH grows into serious PH as mine has. My pulmonary function tests have shown I have had mild PH in my lungs for several years. If a person has a diffusing capacity of less than 55 percent predicted, they probably have PAH. This is the ability of oxygen to pass from the air sacs of the lungs into the blood.
If a patient does not have a severe form of PH, doctors usually begin treatment by lowering lung pressures with calcium channel blockers, (Norvasc, Procardia, etc.), that come in pill form and are much lest expensive than alternative treatments. Unfortunately, few patients respond to this treatment.
If calcium channel blockers don’t work or a patient is in Class III, then endothelin receptor antagonists (traclear, etc.), some form of prostanoid theraphy (Flolan, Iloprost, ect.), and/or phosphodiesterase inhibitors (Viagra, etc,) will be considered. Letairis, which is what I just started is an endothelin receptor antagonist. Endothelin is present in the blood. Patients with PH triggered by other diseases tend to have higher-than-normal levels of it. It binds to receptors in the layer of smooth muscle cells that surround it. Once it binds. it causes vasoconstriction and retards the growth and development of new smooth muscle cells. It may also increase fibrous tissue formation. Designer molecules made in labs can keep endothelin from binding to receptors and are called endothelin receptor antagonists. Their great advantage is they come in pill form.
Before Flolan was available in 1995, class I or II PH patients lived an median of 6 years . Class III patients (of which I am one) lived 2.5 years and class IV patients lived only 6 months. Scleroderma patients with PH do not appear to live as long even with Flolan. When Scleroderma patients die it is usually from pulmonary disease, particularly pulmonary vascular disease. Letaris has shown that class III life expectancy can be increased to 5 years. I think this is with a patient whose PH is primary, not secondary such as me whose primary disease is Scleroderma.
My references regarding medical information come from the Pulmonary HYpertension Association, the Scleroderma Foundation, The Scleroderma Research Foundation, and The International Scleroderma Network. I am not a doctor, just a patient. Scleroderma affects each patient differently. What works for one patient may not work for another and may even be dangerous. I make an effort to provide reliable information. However, it is up to you to verify all facts and consult your own doctor before starting or discontinuing any treatments.

What is Pulmonary Hypertension?

I thought I would explain what is pulmonary hypertension. When pulmonary hypertension is diagnosed with no known cause, it is called primary pulmonary hypertension. When the cause is known, such as with me, the cause is Scleroderma, then it is known as secondary pulmonary hypertension. It is rare and without treatment it is usually a rapid fatal disease.
Blood enters the right side of your heart carrying very little oxygen. The right side of your heart pumps the blood into the arteries of your lungs. (Here is a fact: this is the only place in your body where your blood is blue). These arteries grow smaller and become arterioles, about the size of a hair. They are so small, the red blood cells can only go through one or two at a time. They then go through even tinier capillaries that surround the air sacs in your lungs. Here they take on oxygen from the lungs, return to the left side of the heart and are pumped out to the rest of your body.
PH usually starts in the lungs. My lungs are scarred from the Scleroderma and they don’t have the elasticity your lungs have nor the ability to move the oxygenated blood through the cells due to narrowing and scarring (fibrosis). My arteries and arterioles are stiff and scarred as well restricting blood flow even more. So I had high blood pressure in my lungs first as my lungs worked harder. When the lungs can’t work well, the pressure backs up into the heart which works harder to compensate for the lungs to push blood through. Eventually, your heart can’t keep up and there is less blood circulating in the lungs to pick up oxygen and you become tired, dizzy, and short of breath. Fainting, edema, chest pain, and dry cough may be present. You may feel like you are choking or hungry for air. I can feel my heart beat, clear into my eyeballs. It will thump, flutter and do other strange things. I have had fainting problems for several years. I get up to do something and I can feel the blackness closing in. I have to lay down for a while then get up and try again. It usually goes away. My oxygen tank has helped a lot.
Not much is known about PH, even less is known about PH with Scleroderma. It is a disease of the blood vessels of the lungs and heart. Tomorrow I will talk about the little bit that is known about Scleroderma and PH.

TV

I love Targets new TV ad showing such things as the new movie night which is staying home and watching a DVD. It shows the new coffee spot which is at a bistro table in your own kitchen. With the drastic changes in the economy the past few weeks, we are all having to make changes.
On the news tonight was a segment about people giving up important medications in order to have money for food. I can identify with that. Even though we have Kaiser (Brian will have it again in January), prescription drugs are a major part of out monthly cost of living. Yes, I did get a grant for Letairis, and Brian will hopefully get his next two months of aricept free from the drug company. There are other drugs we take to help our disease processes. Each month I have to decide which ones I feel I can get by without and which ones I really need. Month before last, I went without prednisone. My doctor freaked when I told him because I guess you can’t quit cold turkey like that. This month I went without my thyroid and my chloresterol meds. Those of you who heard me talk this month probably noticed how gravelly my voice was. This happens when I go without my thyroid med.
The news said that pharmacys are noticing a major change. There are a lot more prescriptions for anti-depressants and a lot of people are not refilling some essential drugs that could mean the difference between life and death, such as high blood pressure pills and anti-seizure medications. The news offered 3 solutions: 1) see if your drug manufacturer offers help in defraying the costs of your meds. 2) See if there are generic drugs available that offer the same benefits as a brand name drug. 3) If you have to cut back on your meds, talk to your doctors about which ones are the most necessary and which, if any are not. What ever you do, do not quit your meds cold turkey for even a short time as I did my prednisone since that is dangerous.
I think the next year will be a series of financial ups and downs. I think we all will rethink our spending habits as the Target ad is already showing. Talk about being in tune with today’s consumer! I commend Target for their insight. We have to decide what is important to living a full-filling life and what is not. I think we are going to find that things we couldn’t live without before are not that necessary now. My hope is that we become happier people without the stress of keeping up with the Joneses. By cutting back on spending hopefully we can cut back on our work hours and take time to enjoy our families and friends and to just smell the roses.

Marriage

I mislead you yesterday when I said my pills were $33 each. That would be true if I had a 3 month supply. I only have a 30 day supply so the cost is $110 per pill. I thank God each time I take one.
There is something to be said for 37 years of marriage. Brian and I were very young when we got married. It wasn’t until a year or two after we were married that our friends started living together without benefit of marriage. That never occurred to us. We both lived at home and then we were married. Now we have been married almost twice as long as we were single. We also waited five years before we had our kids which I think gave us a much more stable marriage. We have had a lot of fun along the way, playing some great pranks on each other and when we had Wendy and Kevin, they were not exempt. I think one of the secrets to our marriage is that we have made it fun. We have both grown as individuals, pursuing things that interested each of us separately. While we are secure in the knowledge that we are not dependent on each other to feel validated, we have found that our lives are much happier doing things together as well. As time went on, we found we were doing more things together than not. It of course helps that while Brian has a temper he has never used it against me. I didn’t even know he had a temper until after we were married. And I very seldom get angry so we compliment each other. We also respect each other. We have never said anything to each other that might hurt the other’s feelings. I, myself have never let those thoughts enter my mind. I think if you look for faults you are going to find them. Being a Pollyana, (put a long blond wig on me and Haley Mill’s cute face instead of my prednisone chipmunk cheeks, I would look just like her), I have always tried to find something good in everything and everyone. So always seeing the good in Brian is a natural for me. We have always said please and thank you to each other for everything, no matter how small. I am a firm believer that family comes first. Too many people treat others nicely because they want people to like them, then treat their family pretty crappy.
Now, even with his Alzheimer’s, Brian still tells me thank you each time I fix him a meal. He always tells me it is the best meal he has ever had, even if it is just soup and a sandwich. Sometimes Alzheimer’s patients personalities change and they lash out at loved ones. If anything, Brian is even nicer and more grateful for everything I do for him. And I am so happy that I can still do the essential things he needs.
This past year, we have both had some embarrassing moments but after 37 years of marriage, they are not embarrassing to us. We just help each other put things right and then laugh about it. I am sure that on this journey of ours we will have many, many more such moments and I hope that we will always be able to laugh.
Brian is having a harder time getting his thoughts across. But after 37 years of marriage, I can pretty much finish his sentences for him. I think this really keeps his frustration level much lower than what some people experience when they cannot get their thoughts across to others.
My hands don’t work right any more. Oh, I don’t have the stiffening and crippling in my hands that most scleroderma patients have. But I have the dropsies. And unlike Phebe on the tv show Friends, I am not very bendy. I can’t bend over to put my shoes on so Brian often has to try and help me. And several times a day I have to call Brian in to help me in my craft room and pick up my messes. We laugh and tell everyone that I am the brains and he is the brawn. Again, we complement each other.
I am sometimes baffled by the fact that we are both hampered by such harsh diseases. We have learned great coping skills throughout our marriage that so far have enabled us to enjoy each day and appreciate our time together.

This N That

I took my first $33 pill Friday night. Most nights I do not sleep well, but I slept well Friday night. Sat. morning, Brian and I got up and got ready to go to our grandson’s birthday party. We picked up Kevin around 12:45 and had a wonderful time at Joshua’s 7th birthday party. He is so sweet and loving. Our grandkids, Joshua and Melissa, are such a joy in our lives.
We took Kevin to work at 4 p.m. and went home. I was exhausted when we got home at 4:30 so after I helped Brian get settled I laid down to read and maybe take a nap. I ended up going right to sleep and other than helping Brian a couple of times, I slept the night away, getting up at 10 a.m. this morning. I thought maybe the Letairis was at fault for my extreme fatigue but I am much better today. I haven’t had any other effects of Letairis (knock on wood).
Fatigue is a major concern for me. I tried to explain to one of my doctors once that some days I am so tired, it is too hard to hold my head on my shoulders. Sleep is another problem. I have always been a night owl. Since Brian has been off work he has gradually come over to the dark side too. I try to go to bed by 2 a.m. or else I get Brian and Belle, our cat, telling me it is time for bed. Brian used to go right to sleep and I take a vicodin and read until my body settles down enough to let me sleep, usually after a couple of hours. Sometimes I think I am ready to sleep, turn off my bedside lamp and immediately I am wide awake again. Some days I don’t get to sleep until 9 a.m. or later. Then I will sleep for 10 or 12 hours at a time.
For about the last week or more, Brian has been having trouble falling asleep. He gets in bed, then he will get out of bed, then in and out and in and out. He kind of wanders a bit but doesn’t know what he wants or needs. After a bit he will finally settle down and sleep. Some Alzheimer’s patients have a tendency to take off and wander outside of their home, getting lost in the process. I have always thought Brian would not be a wanderer but now I wonder if Brian’s behavior now is indicative of a wandering soul in the future. It isn’t just when he goes to bed that he is up and down, he doesn’t seem to be able to sit still for long either and is up and down and walking around a lot in the daytime too. If this is all I have to complain about today, then life is good!

Grant

Great news! I was awarded the grant for Letairis! I received my first 30 day supply by Fed Ex today from Accreedo, the pharmacy that handles the special drugs for PAH patients (pulmonary arterial hypertension). That was so fast! I only sent my form back by mail to my doctor the beginning of last week. I will take my first 5mg pill tonight. It will take several months to see if this drug is working. There are 10mg doses as well if this dosage doesn’t do what we hope. The side effects are: lowering of red blood cell counts, swelling from fluid retention, stuffy nose and inflamed nasal passages, hot flashes, heart palpatations, red and sore throat, stomach pain, constipation, shortness of breath (your patient chart shows sob, so don’t take it personally), and headache. Several of these side effects I already have due to other causes, so I think I can handle these just fine. Some of the drugs I have taken in the past have had to be weighed as to whether the benefits out weigh the side effects. Prednisone is such a drug when taken long term as I do. Letaris doesn’t seem to have that problem. Every couple months I have to perform the 6 minute walk test. I am not sure how I am going to do that when I cannot walk more than a few feet partly because I cannot breathe and walk at the same time but also because of the avascular necrosis. Scleroderma has filled my body with scar tissue and that and prednisone have reduced the ability of my blood to get to my hips and legs. It is very painful to move and walk and weakens my bones. Without prednisone, the alternative is much worse. The 6 minute walk test is to see if the drug is working to slow down the disease process and help me breathe a little easier. I am sure my team of doctors and I will figure out something to test the effects of letaris. Hopefully I will eventually see improvement in my day to day living.
I have a wonderful team of doctors at Kaiser. They include a pulmonary specialist, rhuematologist, gastroenterologist, and a doctor who specializes in critical care. If I see one doctor, within one day all of them have the info from that visit and they regularly communicate with each other about my disease process. I see my gastro doc in a about 2 weeks. Because of my anemia, it is believed I have a slow bleed in my stomach so will probably have a endoscopy scheduled shortly thereafter. I haven’t any major digestive problems for a while so I am definitely due for an endo. For me, it is a great way to nap my day away.
I have always had wonderful care at Kaiser. I suggest if you do not like your doctor or the care you are receiving, find another doctor. They are out there and it can make a huge difference in how you view life.

Is This Enough?

Brian lost his Kaiser coverage last month and this month we had to pay full price for his aricept. It runs over $200 per month. He will get his Kaiser coverage back January first and will go back to paying $50 per month. This is a tier three drug, like an orphan drug but many more people need it so the price comes down. In the meantime, aricept does have a program that we qualify for if you have a certain income level and no drug coverage. They pay the costs of the aricept. Obviously, it is a great program. But isn’t it a shame that people on social security disability have to pay the high costs we pay for our medicines that are literally life saving?
The estimated cost of bringing a single drug through screening processes and FDA procedures to obtain approval is between $350 – 500 million. Companies spend nearly $21 billion just on research and development for treatments and it can take up to 10 years just to bring a single drug into the market. Prior to the Orphan Drug act, very few orphan drugs were brought to market.
In 1983, the Orphan Drug act was introduced to facilitate the development of orphan drugs and other medical products by offering incentives such as seven years of marketing exclusivity after approval of the orphan drug product and offering research grants to reduce the costs incurred in the development of orphan products. A rare disease is considered one that occurs in less than 200,000 individuals in the U.S. Since passage of the Orphan Drug Act, more than 300 drugs to treat rare disease have come to market to help an estimated 12 million Americans. This has been confirmed online by Janet Woodcock, director of FDA’s Center for Drug Evaluation and Research.
My question is, Is this enough? There are over 6000 recognized rare diseases. I am grateful they have Letairis for me (especially if it works) and Aricept for Brian. But my heart goes out to those parents who’s children are diagnosed with a rare disease and there is no drug to help them. I know that companies have to answer to their shareholders to make money and yes the Orphan Drug Act has helped. I can only quote statistics I have found. I don’t have the answers. I just feel so badly for those poor parents and their children who still need a miracle drug. I feel guilty to be so lucky that Brian and I have drugs to help us.

Letairis

Yesterday I told you about the new drug Letairis. It is so expensive that my first co-payment is $3,377.80. No, I am not kidding...Because of my other drugs, I am in the donut hole of my RX coverage but I will reach my cap and my coverage turns to catastrophic and I only pay about $233 for Letairis per month after that until the first of the year when I have to reach my cap again. There are only 8 pharmacies that carry this drug and I was called by one of them on Monday to set up the program. I was refereed to a company called Caring Voice that helps people like me with their co-payments. They are located in Pennsylvania while I am located in California. Anyway, they are writing a grant that will probably cover most if not all of my costs for this drug. Imagine that, a grant that I don’t have to pay back from some folks who don’t even know me. Just when I was just happy that Kaiser will pay a big chunk of this drug that costs $35,000 to $45,000 per year. I should know in the next week if the grant goes through. Yes, George, it is a wonderful life!

Heart Catherization

So, I had a heart catherization procedure about two weeks ago. I wasn’t looking forward to it. Although I live with pain on a daily basis, I am against adding to my pain no matter what the procedure. But my doctors have been after me for the past year to have this procedure so I finally agreed. And you know what? It wasn’t bad at all. In fact, I found it much more ticklish than painful and got laughing so hard I couldn’t keep still. The doctor and nurses couldn’t believe how ticklish I was and said most people just lay there. They never had anyone giggle let alone out and out laugh. They give you something to take the edge off and use a local anesthetic. I think most people go to sleep but I struggled against it and stayed awake. How often do you get to see your own heart, let alone watch a wire in it. I thought it fascinating. The results were not good. In addition to my pulmonary fibrosis (lung scaring from scleroderma, I have pulmonary hypertension, high blood pressure in my heart. Had this happened five years ago, there would have been no good treatments available. But last June, 2007, a drug called Letairis was approved for this rare disease. It has been found to give as much as an additional 2 and a half years to one’s life. And you can see what a difference a few years can make. The downside is that this is considered an orphan drug, and is very expensive. But you do what you have to to buy time in this wonderful world. And today I found out there are more wonderful people out there that I will tell you about tomorrow.

Monday, October 20, 2008
Motorized Wheelchairs
I love my motorized wheelchair. With my avascular necrosis, somedays it hurts so badly to move, let alone walk. And to breathe and walk at the same time...well that is a laugh. To breathe and laugh at the same time...well....anyway, my motorized wheelchair is so great around the house. And it is so very comfortable, too. But what kind of company makes a motorized wheelchair that doesn't fit in a normal car. Yes, while my wheelchair is great at home, I can not take it with me. I have a regular wheelchair that will fit in my trunk, but it is bulky and heavy. And it needs to be pushed which Brian can not do any more. Oh, he can push ok, but he can not maneuver it or understand my directions. My motorized wheelchair would be so wonderful out in the real world. The makers don't come out and tell you that it won't fit in a normal car, you have to find out the hard way. My doctor ordered it, A Kaiser healthcare professional came out to my home, interviewed me, confirmed that I needed one and ordered it. The healthcare professional told me to let my landlord know I needed a ramp for it since every other apartment building has wheelchair ramp access except mine. I guess by law the apartment building has to have so many spaces for so many apartments. My apartment manager showed me an article in an apartment rental magazine that said apartment owners did not have to build ramps for their tenants. She said that to put in a handicap ramp would run $3,500 and that I could do it if I wanted to, but the money would have to go into an escrow account first. Now, I am sorry, Miss Landlady, I do not have $3,500 but you do have about $2000 of our money on deposit. Seems like we could reach a compromise, but no can do....so even if I could get the chair into my car, I don't have a ramp. If I could figure out how to get the chair into my car, I could buy a portable ramp that I could use if space was available. Anyway, I digress....watch the ads for those motorized chairs. They state how wonderful they are for maneuverability in your home. And I tell you, it is so wonderful, it really is...but no where does it state it doesn't work in a car. Easy, you say....Get another car. Yes, I could but this car will be paid for sooner rather than later and another car just means a new loan. I am able to drive now, but with the avascular necrosis in my hips, that may not be a possibility down the line. So, while having the chair with me when I go out would be nice, I can get by without it for now so I just have taken a wait and see attitude. I guess if it works, don't try to fix it.

Friends

Sunday, October 19, 2008
Friends
It just amazes me that we have such amazing friends. Not that they are amazing but that we have so many. Brian and I do not go out much. (Some days we work at the art of doing nothing. Won't be long before we have that perfected!) If we do, it is usually with Wendy or Kevin, our kids, or Kim and Wayne, our best friends who just happen to be Brian's sister and her husband. And they are so wonderful about helping us if we need it. Getting us drinks so that we don't have to get up off our butts. Helping Brian find the bathroom maze that he faces whenever we go out. That is so nice, but they are family and they are more in tune with what we are going through. But our friends do the same things too. Last night at our Disney meeting, if we needed anything, someone was there who was better able to get it for us. If we needed trash thrown away, it was gone. It humbles me when people help us. I had to learn to accept it because, frankly, it sure makes living easier. But our friends do it without thinking twice about it. And that is what amazes me. The capacity of the human heart to care and to love unconditionally. That is one of our greatest reasons to go on battling our disease processes. To all of our friends and relatives...thank you. You are what makes us want to keep going each day. Thank you!