Stem Cell Surgery

I finally got the news I’ve been waiting for. All the months of testing, waiting and hoping that I’ve been posting about has resulted in a date set for stem cell surgery at the end of October.

Surgery for the Phase II, Open-label, Dose Escalation and Safety Study of Human Spinal Cord Derived Neural Stem Cell Transplantation for the Treatment of Amyotrophic Lateral Sclerosis.

I’ll be having 4,000,000 stem cells injection directly into my spinal cord, 10 injections on each side totalling 20 injections. I’m experiencing many emotions about it. First I’m honored to have been chosen even though much of it was luck by being at the right stage of progression at the right time and living in the right place. I was still chosen and I take it very serious. I know this trial is being watched very closely and being healthy as possible for surgery and recovery is important to show this is worthy to continue for more people, hopefully sooner. While there will be observations for improvement or slowing of progression, this is still considered a safety trial. Although I have already started eating healthier, I’m definitely kicking that up a notch and doing everything possible to be physically and emotionally prepared for this surgery.

It’s my feeling that participating in this study is not only for me but for everyone living with ALS and those who love them. This is huge for ALS and also for other neurodegenerative diseases such as Parkinson’s and Alzheimer’s disease. A lot will be learned. That’s why I want everyone to know how serious I’m taking this. I want and need to do well for the advancement of treatment for us all. In saying that, I also want to say I can’t predict what will happen in the big picture or when. From all the reading I can get my hands on about this and my personal thoughts, I think this is making much progress in understanding ALS and even if I get no measurable benefit from it, I still believe it’s a huge advancement of learning for researchers and will move treatments forward much faster.

After saying all this, I have to admit to having moments of fear. Thankfully I’m able to express that and keep my mind in a good place. I’ve spent the last few months being tested to make sure I meet the strict criteria and that’s where my thoughts have been. Now that the surgery is actually scheduled, that is where my complete focus is.  There is no doubt huge risks are involved, including my lifelong fear of being instantly paralyzed from the neck down. While knowing these risks, I’m not in the least  deterred. During the procedure I know I will be under the care of what I believe to be the most skilled hands and brilliant minds in ALS research and that care will continue throughout my recovery. This helps keep my fears at bay. Recovery will be far from a cake walk but, I am prepared to take it on.

I want to say to the ALS/MND community as a whole that you can count on me to do everything expected and recommended by the doctors treating me and I’m dedicated to being in the best condition for it. I know many would love to participate in this study. Since it’s so limited at this time, I hope that will bring some comfort to know I’m not just glad to be a participant, I am dedicated and preparing for it. I know that would make me feel better.

Let’s keep hope alive that this and other studies going on will give us a treatment soon. For us as well as people living with other neurodegenerative diseases. Hope is alive and well in me for future treatment.

I have been nervous about this blog post since I received the news. After writing it I feel better and believe I will have the support of the ALS/MND community behind me. I still have a life to live until surgery and for the most part my life will continue as normal.  There are plans to be made for pets and family coming from long distance. Other than that, I’ll be listening to my music, watching movies, loving my pets and being me as each day passes.

Thank you very much for taking the time to read my blog.

Until next time, take care,

April

Unless You Die, You Will Age

Today I’m just rambling on so I can move on. We all know time flies when you’re having fun at any point in life. As we grow older and wiser we realize the passage of time just sweeps you right along, and how the next Christmas will be here seemingly before you’ve dropped your new year’s resolutions, while to the child, the next Christmas seems so far away.

For me the sense of passage of time has been altered since being diagnosed with a terminal illness. With ALS you know there’s a good chance you can count your years living on one hand. A couple of years ago I just superficially didn’t want to be a year older. I didn’t want to get old, feel old, and God forbid, look old. When I reached that pivotal point in life of seeing the signs of age, the first little sign of a dreaded wrinkle on my face, any little cute freckle was now considered an age spot and the skin started losing that natural fresh look, I hit the web for the best anti-aging products out there. Money wasn’t something I had to throw at aging so I spent hours researching DIY treatments. I started using prescription Retin-A religiously because it was scientifically proven to build new collagen and elastin in your dermis. It takes a year to reach full affect and I did it, and continued. It actually did pay off, but I spent more days with burning peeling skin that wasn’t attractive or comfortable just to rid my self of that little beginning of a wrinkle and some skin discoloration.

At the end of the day and after going through all the skin rituals I had with oil concoctions, ferulic acid, everything kept in right light and right temperature. I felt pretty good about what I had accomplished. I did have skin that looked good for my age. I will say again, for my age. I was still aging, there is no going back to 22. There just isn’t. People that spend money on quick fix surgical procedures usually look older even though their skin is smooth with absolutely no discoloration or hint of a line. They usually seem older because of it being so obvious work has been done. Aging is natural and humans intuitively see it. Unless you die, you will age.

I have stopped all of it now. I simply try to be kind to my skin. When I think back to how time consuming all that was, I just think what a waste. Having said all this I think it’s normal and healthy to want to look your best. I would just advise a much simpler regimen starting at a much younger age. Avoid things that you also avoid for health reasons. Always wear sunscreen, don’t drink in excess, and don’t smoke. Be kind to your skin by finding what works for your skin type best at an early age and add it to a simple skin care routine. Then come to terms with aging. I know, easier said than done for some people, like me before ALS, but you can’t stop it and you are missing out on life by obsessing over it.

At 40 you may see yourself as looking very old in a picture. Just wait until you turn 50. You’ll look at the same picture and think how young you look in it. It’s all in perspective. No matter how great your skin looks at 40, to a 20 year old, you still look much older to them. That’s because you are.

Now that I know my years are numbered, I certainly see aging from a new perspective. I would take a face of wrinkles and a flabby body over a death sentence any day now. I know I won’t be swinging on the proverbial porch swing in 30 years with my husband. I won’t see my Grandchildren grow up. I won’t do or see a lot of things I just took for granted that would be before my diagnosis.

Now I’m 41 and I regret that way of thinking. It stings. I doubt I’ll change anybody else’s way of seeing aging, It would be nice to think so but this was just a letting out of my most recent thoughts, regrets. I try not to stay caught up in them, I have a shortened future to focus on. Usually when I let a ramble out in writing a post it helps me move on. I feel I already have.

I’m still a woman and even though how I look has been moved very far back on my priority list, I still find myself some days upset at how drastically my looks have changed. I’m still alive and I want to look and feel like it. By this time next year, I’m sure my perspective will have changed much more.

Anti-aging is a multi-billion dollar industry for a reason. We all want to look our best. My advice is good. It’s what I wrote above. Start young, keep it simple and put enjoying life first and you’ll be much happier for it. That’s the morale of this ramble. Thanks for sticking it out.

To learn more about me and view my image gallery please visit my website, http://thealsexpress.com

Until next time, take care,

April

 

ALS Clinic Day

I’ve had it rough the past few days. In my last blog post I mentioned not resting before clinic day. I’ve been paying the price for that. To make it worse, Friday was the longest and most intense clinic day I’ve had so far. The great news is my breathing is holding up well. I don’t know if there is anything documented on this but I truly believe starting early with help breathing keeps you breathing better, and longer. It’s probably more of an individual thing, as no two people are exactly the same in progression but it can’t hurt. I recommend getting one as early as possible, when you first have measurable decline. I also think the trilogy 100 is the one to get without a doubt. You can go back a few posts and read my experience with it if you haven’t already. I’ve been using it approximately six hours a day give or take, depending on the day, during any given 24 hour period. No more morning headaches or waking to crackling lungs for me.

This ALS Clinic day was very taxing even if I had rested better the day before. There seemed to be a lot more patients there. Every other visit we are called right back and given a room. This time I was called back and after weighing and blood pressure, I was sent back out to wait about an hour. I’m not complaining really, I know clinic days can be overwhelming and you never know what it will be like when well over 100 people are being seen in different stages of progression. Some people take priority and this is one thing in life you don’t want to be given priority status for. I’d much rather wait.

My severe right leg atrophy, and the changes it’s caused in my walking was the most devastating part of the day. I’ve been hoping to be accepted in a clinical trial and you need to be able to walk. I was hoping I was wrong because this is a trial I meet all other criteria for and I want to be in. I haven’t walked for a while now. My left leg is still pretty strong and larger than my right bacause it still has much more muscle. Guess what? That’s right! to my own amazement, I walked. From the exam table to the door and back. Tony walked right beside me, I’m sure like everyone else, he was thinking he needed to be there to help when my right leg carried me on down to the floor. I did it unassisted. I did it!

So this puts me back in the running to be chosen. It’s still far from over. I’m still a very good candidate, but now my records must be reviewed by an independent neurologist. They don’t play or stray at all from the criteria set for clinical trials. That’s a good thing, even though it can be frustrating. If they did, the outcome results could be skewed. Even if I get the go ahead from the independent review, I’ll still have to go through more screening. So I’m far from being in, but I’m not out either.

If it does turn out that I don’t get in the trial, yes I will be disappointed but not defeated. I will search for another. I already know it’s highly unlikely I will personally be saved from the death sentence that is ALS. Even so, as long as I possibly can, I want to be a part of research to end this for others behind me. Do I wish something will help me? Of course, but I make it a point to stay grounded in the reality that it’s highly unlikely.

I think the main reason of all is it keeps my mind busy. I’m already busy with other things I’m focusing on, but I don’t want to give ALS the opportunity to get in my thoughts too often in a way that reminds me of all things I’ll now miss out on in life. Trust me it’s easy to be lead there by ALS. I want to play a part in ALS being stopped from ripping apart more lives and families.  I want to stare my nemises straight in the eye while I deal with it’s destruction day to day. It keeps me living.

I do feel the extreme fatigue and accompanying symptoms like spasticity and cramping letting up. My range of motion exercises also were easier today. I’m loosening back up and ready to get back to my projects. Sorry ALS, you loose again!

ALS is a daily battle. It’s never a good idea for me to think I have the next day defeated. I wait and see what ALS has for me everyday and I decide how I’ll deal with it from there. I have learned to never get too cocky. ALS will fight harder then. I take it day by day and minute by minute. I can do that or sit back and take what it throws at me. Some minutes I don’t have much fight in me but I luckily have the support and encouragement to get back on track. I’m very thankful because I know many people don’t have that.

For now, tonight, I’m going to watch a movie with Tony and during that time put ALS in the background.

I plan, I said plan, to blog tomorrow. Not sure about what. Thank you all very much for reading.

Please visit my website: http://thealsexpress.com

Until next time, take care,

April

Dying Shouldn’t Be Taboo, Everyone Does It

Ironically, putting myself completely out there with my blog and how I’m dealing with ALS is in a way preserving my privacy, something I cherish. I also believe it’s kept my loved ones informed about a lot of things without worrying about how to ask me a question, worrying if it will upset me. I think this is especially so, since I don’t look like I’m on my death bed right now.

Death and dying aren’t easy topics to discuss for most people even when it’s staring them in the face and they have this intense need to know or express something. I completely understand because I’ve always had a hard time expressing myself when someone I care about is in the midst of dealing with a death. It’s a taboo topic that needs to be brought out to mainstream conversation. I’ve heard people regretting that they didn’t express something they wanted a dead loved one to know, including myself, but I’ve never heard anyone regret sharing their feelings and speaking openly with someone at the end of their life.

Right now I need a lot of help and it’s obvious something is wrong, but death? It’s too early for most right now, almost like they feel it would be an insult. Not many have seen me at home in my little ALS headquarters, I jokingly call it, with me unprepared for their visit. Only Tony has seen the absolute worse and me dying is still hard for him to grasp at times. It wouldn’t bother me at all for someone to ask me, but I realize how uncomfortable it is for them. They need more time to process that the losses will continue. ALS will continue until it kills me.

I am thinking and learning about life prolonging, and end of life options. No, not all day every day but there is a part of me constantly preparing. Preparing for the next blow from ALS, and the next hurdle to overcome. For me it’s not much of a choice because I’m feeling changes before they’re apparent to others. My private nature is well known by my loved ones and I think that’s one reason we don’t talk much about it, but I think the main reason is they need more time.

I believe all my loved ones and I are being helped by this blog. I’m able to express my feelings and fears which has turned out to be a great stress reliever for me. They are able to stay informed on what I’m feeling and dealing with. This doesn’t mean all of my posts are easy to publish, they’re not, I always have them and their feelings on my mind. Many days I’ve struggled with whether or not to publish what I’ve written. I have stuck to my word about saying what I feel when I want to. What I thought might be a little selfish has proven to be helpful. I’m happy and proud of that. So during the times we spend together we talk about other things. Right now it’s working great for us all. ALS of course is brought up at times, but everyone pretty much knows where I am at.

I know the time will come when very hard things will need to be discussed. I do wish speaking of death and dying wasn’t so taboo. It can make things much worse in the end. It’s a topic that needs to be brought to mainstream conversation without such discomfort when a person is dying. I think my blog is helping this happen for me and I’m so glad I chose to do it.

This is just something I wanted to express to all my readers and really to my loved ones. I thank you all. I know many people have died with many things left unsaid even though they had time. Hopefully a day will come when it’s common to openly discuss it at the appropriate times. Dying shouldn’t be taboo, everyone does it.

This was written faster than usual. I just wrote what I’m thinking as it came. I do realize all deaths aren’t the same. Some are sudden and with no opportunity to express deep feelings. I’m not about to sit here and say you should have been expressing your deep feelings everyday in case of a sudden death. I’m discussing my death, and the death of anyone dying from a terminal illness.

My last couple of posts have been death oriented. I’ll hopefully have something a little lighter next time. I have to let my thoughts lead the way though.

Please visit my website: http://thealsexpress.com

Until next time, take care,

April

Death: To Know or Not To Know?

I don’t know how common this is to wonder, but I’m sure it’s crossed most people’s minds at some point. I know it has mine occasionally throughout my life. When and how will I die? And do I really want to know? I think it’s fair to say most people want to live a nice long healthy life and then die peacefully in their sleep.

They don’t think much of death until a family member or friend dies. That often has people reevaluate their own lives and priorities for a while, but life eventually gets back in the way. You certainly never stop missing your loved one, but you go on, and with the occasional thought, “I wonder when and how I will die.” The question is, what do you think you prefer? To know or not to know?

Now here I am, short of a tragic accident, I know how I will die. It’s not a pleasant death; that is one certainty. I will continue to waste away and lose all privacy and independence which is something that has always been very important to me. Actually this is the process of leading up to death. The best I can hope for in the final end is that I have a great hospice team to keep me well enough sedated so that I feel as little discomfort as possible. I find myself thinking of those final days at times and it can be scary. I know basically how. I just hope I don’t linger too long in the end.

Like most people who don’t know their fate, I want mine to be quietly in my sleep, albeit drug induced sleep. At this time I have less fear of the actual final day. It’s more the multiple losses and the scary feeling of not being able to breathe because of sticky mucus closing my throat with a congested nose that I experienced not too long ago. I know there will be more scares to come. Scares that can end in the feared nightmare death if not handled properly.

As for when, I’ll probably make it to my mid 40s. If I’m here longer than that, it will most likely be because I chose to have a tracheostomy. I still haven’t made that decision yet though I’m still leaning on the no side. So what is my answer to the question: to know or not to know? For me, in my position I answer yes and no. Yes, since I have been diagnosed with ALS, a disease with no cure and I know I’m dying, I want to know everything. The more I know the more I have control. I’m not a controlling person of other people, but I want to know what to expect for myself. Information is my friend.

If I was 25, or 40 for that matter without this diagnosis, I say no. If you’re alive, live like it!

My Brother and Nephew were in a band called Nevertheless. They wrote a song named Live Like We’re Alive. As a dying person I try to do that with some days being easier than others.

While alive, live like it! Click this link to the Nervertheless song.

My brother is in the process of making his first full length solo album. You can support him by clicking this link.  He only has 7 days left!

 

Please visit my website: http://thealsexpress.com

Until next time, take care,

April

The Trilogy 100

I’ve had the Trilogy 100 for about 3 months now. I wouldn’t want anything else. Now I can’t say I did my research and made the decision after lots of comparisons like with my power wheelchair. This is because I’m at a great

ALS clinic where they stay on top of all patients and take action at the right time. It was my January 2012 ALS clinic day at Emory. When the Respiratory Therapists came to my room for the usual Forced Vital Capacity (FVC) testing that you get at every visit. I told them what I had been experiencing. I was having headaches, especially when I woke in the morning. I also woke about every morning with a feeling of needing to take a deep breath. I would sit up straight as possible with my fists against the mattress and breathe in as hard as I could. For about the last 3 seconds of the breath I would feel a crackling, then it wouldn’t do it for the rest of the day. It made no noise and my husband tried to feel the vibration I was explaining and never could. It wasn’t a crackling like phlegm per

say, like you might have with acute bronchitis. After I had that first deep breath for the day, no more crackles. Tony called Dr. Glass’ nurse and he recommended I see my primary doctor and get a chest x-ray to be sure there was no infection. It came back perfectly clear and nobody thought much when I explained my crackles. Right about the same time I was also beginning to notice a feeling of being out of breath with less exertion than usual. The distance of walking became shorter before I would need to catch my breath, and also during conversations it started becoming sooner that I would sound a little breathy and need to stop for a minute or talk even lower.

These weren’t serious, “Help, I can’t breathe situations,” but when you have ALS you notice these things, and in a different light. You’re very aware that losing the ability to breath and subsequent cases of pneumonia is usually what kills you. I pay close attention to my body while trying not to over think things. It’s not always easy with this scary disease. I gave thought to possibly anxiety being the culprit, but I knew it wasn’t causing the crackling lungs at the end of my first breath of the day, and it became very apparent I needed to take breaks sooner than usual from any exertion, or much talking. Others noticed too.

I told the Respiratory Therapists it wasn’t real bad, but they thought it needed to be looked at closer. They chose to go ahead with some extra testing. They felt it should be done and they believed Dr. Glass would feel the same, so they started with it. The main difference was in my breathing tests while lying down. This explained the morning headaches and even the first breath crackle that had only brought strange looks before when trying to explain to others. They explained that while lying down and sleeping, I most likely was breathing shallow and the lower portion of my lungs were collapsing. They explained my first crackling breathe is when my lungs were opening back up for the day while I breathed deeper. The headaches were likely because during sleep, breathing muscles relax more than when awake and if not breathing in, and especially out correctly a buildup of Carbon dioxide (Co2) will cause headaches. In end stages this can be a cause of death because of carbon dioxide buildup in the blood. Severe Co2 poisoning will result in irregular heartbeat, unconsciousness and death. Respiratory issues are taken very serious in ALS. That’s one discipline you will see at every clinic visit.

When I had my FVC tested sitting for the first time at Emory, the day Dr. Glass confirmed my ALS diagnosis, I tested great. You get three chances to blow out and three to suck in air and they take the highest numbers for each. My first was 101. I thought it would be a long time before any decline. The next visit I think was in the 90s, then a couple 80’s started showing up but I still made a low 90s result. My visit in January was a little shocking when I saw that first result in the 70s. I’m not positive, but I think I made it to mid 80s with my last of three. The real shocker came when I was tested lying down. You can’t lift your head for this and I ended in the low 60s. That not only shocked, but scared me a little. I’ve read of people being in the 30s, 20, and even teens though. I know the lower your FVC score is the more likely you are to be turned down for certain clinical trials and there is a window of opportunity before it gets too low to have a feeding tube placed also.

My trilogy 100 has changed how I feel so much. I do still get really fatigued, but it’s different. I know you can’t regain muscle lost from ALS, but you can slow the loss down and I believe this has been the case with me. I’m feeling good about my FVC scores being tested in a few days when I go for my Validation of bio-marker study. I no longer wake with a headache every morning, and I don’t have the crackles at the end of my first breathe in, and the settings on my Trilogy 100 are still set relatively low.

That’s how I ended up with the Trilogy 100. Now I’ll tell you a little about this amazing device. First of all it is capable of providing invasive and non-invasive therapy. So, if you start off using this device and progress to the point of needing and choosing to have a tracheostomy, you can still use this instead of getting used to a completely different device. It also only weighs in at 11 lbs, so it’s very portable. It fits nicely on the back of a power wheelchair. It includes six hours of battery life, so in the event you’re out and about that’s a long time. You can also purchase extra batteries. It’s very easy for your doctor to keep up with how it’s working for you. It has a simple SD card that your Respiratory Therapist comes to take and replace every couple of months, or whatever is determined necessary for your case. You hit one button to turn it on and two to turn it off. You can’t mess up. It’s capable of several modes of therapy depending on patient needs, but can be left with only two settings for the patient. Without trying to talk over my head mine is with AVAPS setting and BIPAP setting. BIPAP is what is commonly prescribed for ALS patients. Most people are familiar with CPAP – Continuous Positive Air Pressure. Then there is BIPAP – Bi-level Positive Air Pressure. BIPAP allows you to breathe out easier by easing the air pressure when exhaling.

I’m not comfortable explaining what AVAPS setting means except it is: Average Volume Assured Pressure Support. You can learn more by clicking this link.  I can tell you I prefer it much over the BIPAP setting. It senses what I’m going to do and what I need like magic. On the BIPAP there is more of a struggle to get my breath in sync. This has been my experience, and this is the main reason I’m so thankful I wasn’t prescribed a standard BIPAP. This may be different for another person. Since I don’t like the BIPAP setting, I’ll be asking for a prescription for the sip and puff mode. This is a great feature that allows you take a helpful breathe as needed through a harder plastic connection, best way to explain is you take a sip of air as needed. It won’t constantly blow air in this mode. I’m looking forward to replacing the BIPAP with this setting.

I have and recommend a humidifier. Dry air will dry out your sinuses and can be uncomfortable. I had to wait about a month on the humidifier and it made a big difference in comfort. The most dreadful thing, no matter the type of device is finding the right mask for your face. I’ve tried very hard to find and like a full face mask that works well for me with no luck. Its awkward head-gear and the plastic pushing on the forehead begins to really hurt after a couple of hours no matter how soft and I haven’t found one that doesn’t start leaking within two hours either.

My favorite around the house mask is the F&P Pillairo. The nasal pillows are very comfortable and create a great seal. It’s a one size fits all non adjustable band that goes around the head. I thought that would be a problem but I just put it behind my ears and it works like a charm. If I’m in my bed I can just let it hang from my neck between uses. It’s not very pretty though. My next favorite is ResMed Swift FX for her. The nose pillows aren’t as comfortable, going out it’s worth it to me because it covers your face the least. I also have a ResMed SoftEdge Mirage FX. This completely covers the nose. It’s not nasal pillows. I use this occasionally at night. It creates and keeps a good seal and is comfortable. The only reason I don’t use it more often is laziness really. It does involve more time getting it on. When using it I have to also wear a chin strap so that my mouth won’t open in my sleep. At first I didn’t like that because the strap makes my teeth touch in an uncomfortable way. I figured a way to solve this by buying an inexpensive mouth guard you can find at any drug store. You heat it and let it mold to your teeth. It’s a great way to get the same benefits of a full face mask at night with better comfort. I only use this on nights I feel like taking the extra time, which isn’t much, but it is compared to the slip on nasal masks. I’m sure I’ll be trying many more masks, especially as I need the air for longer periods of time.

This has turned into a much longer post than I planned for so I’m going to stop here. I hope this is helpful to someone.

Please visit my website: http://thealsexpress.com

Until next time, take care,

April