Hello Friends from your Sweet Caroline!

Its been a while since Ive said hi.

I am so comfortable at this moment I can hardly keep my eyes open. Ive been drifting in and out of sleep all day. Its been wonderful to feel so peaceful, especially during the evening time. It seems we have finally crested the hill past the anxiety and discomfort I was feeling. I still listen to my music and Harry Potter, I am almost through book #4.

Don't feel sorry for me. Look how good my life is right now!

I have cute boys that sing to me and give me kisses on my mask...


Loved ones who stare at me adoringly and tell me stories of memories we share.


And if that's not enough I get foot rubs all day long.


Don't despair for me...I am at peace!
Love you all!

The beginning of the end

I was completely taken by surprise when we had a light rain this morning. The weather seemed a fitting tribute to the mood of our home. It was somewhat somber but also incredibly peaceful for the first half of the day. My parents had a talk this morning and with my Dads blessing my Mom has decided no longer take any food or fluids.

Hospice came and gave her a catheter and we increased her medications to make her more comfortable. She can not be without her mask for more than a few minutes and we all agree that dehydration is a much more peaceful way to pass than waiting for her breathing to claim her - which is not far off.

This comfort care business is not easy. Her evenings are bad. When we finally got the right medications into her to make her comfortable they had been in long enough to cause nausea. Even with the comfort care nausea medications she threw up - which when you have no diaphragm strength is something like a small burp.

Thankfully we survived the evening and she is sleeping peacefully now after having received a blessing. Tomorrow she will be getting a morphine pump. My comfort care is coming in the form of my Aunt, the nurse, who will be here within the hour and staying with us and guiding my Mom through until the end.

While we have prepared ourselves for this point there is still a sense of disbelief when we find ourselves standing at the threshold. Tonight our hearts resonate with Hymn #128 - When Faith Endures

I will not doubt, I will not fear; God's love and strength are always near.
His promised gift helps me to find an inner strength and peace of mind.
I give the Father willingly my trust, my prayers, humility.
His spirit guides, his love assures that fear departs when faith endures.

Changes

In the last 2 days we have changed my Moms nurse, pain meds, and anxiety meds. We have been busy! Some of these posts may seem mundane and boringly detailed but I am trying to give details for those who are also dealing with ALS. One of the most frustrating things for me as a family member of someone with ALS was having no rough guide as to what kind of process to expect. I know that everyone case is different but I would have loved some idea of what kind of road bumps to expect. I know there are several readers that have found this blog because they have ALS or are the caretaker of someone with ALS. These kinds of posts I hope will help serve as a rough guide for others.

First of why we changed our nurse: I'm sure our first nurse is a great nurse for some people but her personality was not the best for my Dad and I which is really important since we are ones that work with her the most. We asked for a change and the Nurse they sent yesterday was great. She was much more calm, very organised, and very methodical. We are happy we made the change because we need someone that brings us and our home a sense calm. Darlene is the right fit for our family.

Second, we changed her from morphine to methadone. We were needing to go up on the morphine every couple of days and they recommended we try methadone instead because it is longer lasting and it can be given every 8 hours and should keep her more evenly comfortable. We can still use the morphine for breakthrough pain and discomfort.

We also switched her from Seroquel to Lorazepam to treat anxiety. My Mom did not do well on Xanax. We were told that it can make some people act like a mean drunk - she wasn't that bad but it was enough that we didn't want to use Xanax. That's when they gave us the Seroquel which worked but I'm not sure why they tried that one next. In the comfort pack hospice brings they use Lorazepam and it is suppose to work really well with morphine and methadone. They are suppose to each make the other more effective.

Now our only problem is that she woke up this morning with something similar to lock jaw. We have someone coming here soon to check her out. Guess the G-tube is a blessing once again :) My Mom is taking about 1-1.5 cans of Ensure a day and probably around 3-4 cups of water total. But then again the Lorazapam and the methadone are given in the cheek in liquid form so we really need to figure out what is wrong with her mouth.

Her strength has decreased so much that we are almost to the point that she is dead weight when we lift her. We will have to start using the lift we have soon or she will have to stay in bed with a catheter.

Her breathing has changed significantly. As of Thursday night when she first had that breathing attack she has been wearing her mask almost all of the time. She takes it off for about 5-30 minutes but that's about all. Before that she was only wearing it during the night, naps, and very sporadically in between. We have her pressure up to 14.8 now. Manny said he actually has someone at a pressure of 20 so we will have to see what she can tolerate. There will come a point when the pressure is too much that its uncomfortable or she wont be able to keep the seal closed. When that point comes Darlene said we will put her into a very comfortable coma so she wont feel like she is suffocating.

The nurse also heard a wheeze in her lungs yesterday for the first time. That's not a good thing. From what we understand that might be the start of fluid in her lungs. If she gets fluid in her lungs they can not do anything to help her and it will turn into pneumonia. This last Monday the admitting hospice nurse said she might expect to see fluid start in my Moms lungs in about a week and it seems she wasn't far off. I tried to listen today and thought I would be able to hear the wheeze since I am use to listening for it in my asthmatic son's lungs. I could hardly hear anything at all, let alone a wheeze. When I could hear anything it was only when the stethoscope was up near her shoulder blades.

It seemed for a time that my Moms lack of strength and lack of appetite might actually claim her life before her breathing did. With the changes we have seen in the last couple of days that doesn't seem to be the case anymore.

We feel very confident that with Darlene and hospice's help that we will be able to make it through while keeping my Mom as comfortable as possible. My Dad has been a hero in the last week. He is even more patient and more loving with her. He cares deeply about how she is doing and is methodical about her, her care, and he is advocating for her. It is very touching to watch.

Thank you all for your prayers and kind e-mails. I am sorry to say that we have been horrible at thank you cards or responding. Please know that each comment, letter, e-mail, meal brought in, soda delivered, babysitting act, and countless other little act help lift our burden and make it feel lighter. All the movies, games, books on tape that have been lent have done so much to help keep us all sane. Your smiles and hugs are making it possible for us to make it through this process. I often think of Moses in Exodus 17.

11And it came to pass, when Moses held up his hand, that Israel prevailed: and when he let down his hand, Amalek prevailed.

12But Moses’ hands were heavy; and they took a stone, and put it under him, and he sat thereon; and Aaron and Hur stayed up his hands, the one on the one side, and the other on the other side; and his hands were steady until the going down of the sun

Our sincerest thanks to all of you for keeping our hands steady!

The Myth of Morphine

There is a prevailing myth in the world about hospice and morphine. I know I believed it and hospice tells us they encounter this almost every time they go into a home. I knew hospice came in before the coma stage but the myth we all believed was that when the morphine started we were essentially starting to put her into a medicated coma and she was going to start sleeping a lot.

NOT TRUE!!!!

We would have started the morphine weeks earlier had we know that she would not only be alert but happy again! Its not a drugged silly stupor either. She is comfortable enough to be herself. How I wish we had known! The last few days have been wonderful. Smiles are back and she just seems....comfortable. I feel like we are abusing that word but its really the best description. They mean what they say in the term comfort care. From what I understand when the morphine levels get really high there is a lot of sleeping, but I'm not sure if at that point its from the morphine or because the body is shutting down.

We are lucky we had started the medication and had her comfortable because she had what I would describe as her first experience with "air hunger." It wasn't horrible but we should have figured something was wrong earlier. She was telling me that she hadn't been given her anxiety meds and I was telling her we had given them to her but she was still really frustrated and agitated. She seemed really uncomfortable and fidgety. I could kick myself for not evaluating her all overall but we were working so hard to distract or make her comfortable that we were missing how hard she was breathing and that her retractions were really deep.

She finally told us she couldn't breath when we looked it was shocking. We put her mask on her and she calmed down and we eventually got her comfortable again. Its been busy with all of the different hospice workers coming in and we had a new bed delivered that is the TURBO comfort low air loss alternating mattress. She was having a sore spot on her tailbone and thighs, a sure sign of a pressure sore starting, but that is already getting much better just after her nap in the bed.

It feels good to know we were on top of things enough that when we hit this first air hunger type of moment she was already medicated enough that it was not too traumatic. (Sigh) At the same time its like we just got everything figured out. There were smiles and peace for just a brief moment and now we are moving right along into the next stage. I guess that's good, its what she wants. The process is just exhausting! Its hard when you see her uncomfortable and so you do everything you can to make it better but you get so caught up in the pressure when all you really need to do is take a step back. That is the hardest lesson to learn, and just when you think you've mastered it everything changes again.

Hospice

7 months ago we found that our family was taking a voyage aboard a ship and we were not just going for a ride we were going to be the the crew. There was only one problem - none of us has ever sailed before. We voraciously studied charts of the waters we would need to navigate through trying to become familiar with what we might encounter. We read the journals of others that had sailed this route before and scoured them for tidbits of knowledge that might warn us of hidden dangers. But the sea is unpredictable and with variations in tides and weather conditions each crew must face the unknown as best they can. And with inexperienced crews there naturally comes navigational errors, improper training, poor judgement calls, and worst of all fear. All of this makes the journey so much scarier and that much more perilous. We knew that our ship was nearing the most treacherous waters in our journey in the shallowest waters with the most hidden wrecks, rocks, and unexpected sandbars. We were scared and so we studied harder and prayed more.

And then suddenly a Captain boarded our ship. Our captain has navigated these shallow waters many times before and helped many scared first time sailors make it through where our ship must also go. They know what hidden traps might set our ship off course or run our ship aground. With our new Captains experience, confidence, and patience our crew feels a greater sense of calm and we feel more confidence that we can see this journey through safely.

Hospice is our Captain, and we are elated to have them on board.

Update

I just returned from Utah and in those 6 days my Moms overall condition decreased dramatically. She doesn't type on her ipad anymore instead she has been drawing letters with her toes on the ground. There has definitely been a decline in her breathing. She wears her breathing assistance devices more and her BiPAP machine is now at a pressure of 14. The respiratory therapist confirmed to my Dad that she is officially the fastest case he has ever seen.

We did get some really good news. The first hospice company made a mistake and we actually have around 70 days of hospice coverage! Grrrrrr! That makes a huge difference! She should have a speaking device shipping this week and we are waiting for that to come before calling hospice in. If we call hospice in before we get that device the insurance company wont pay for it. Since communication is our biggest challenge we are waiting for the device to come and then we will call hospice. We don't necessarily need hospice because we have the comfort pack medications already, but it will still give us peace of mind to have hospice on board.

The biggest change is in her strength. She has very little left and she seems listless now. She hardly seems to have the energy to nod her head yes or no to our questions. She is much more uncomfortable and she has a lot more anxiety; which is a normal side effect of her breathing limitations. We are using the comfort pack anxiety medication to treat it and today seems to be much better than yesterday.

Weak is the best description for her overall condition. Not just a physical weakness but a deep weariness seems to have fallen over her. She doesn't seem to have the same sparkle in her eye. Smiles that use to come frequently and easily, are now fewer in number and much harder to come by. The one thing that always brings a smile to her face are the letters and comments from all of you. One of those in particular brought a smile to her face today. One of my Moms readers has a secret....one that lasts around 9 months long!!! A big congrats from my Mama to those soon to be parents of a little sesame seed sized secret. Don't worry your secret is safe with her - even if she wanted to spill the beans she couldn't :)

Comfort Care

We had my Moms appointment yesterday and here are the results compared with her numbers from 3 weeks ago:

Forced Vital Capacity: 40% three weeks ago, down to 25%
Negative Inspiratory Force: 28% three weeks ago, down to 12%
Sniff: 32% three weeks ago, down to 22%

They were right about where we expected them to be after seeing the changes in her over the weekend. Everything we talked about with the nurse was for comfort care. I like that term. It brings to mind peace and compassion. The head nurse at the clinic told us she will never forget her first appointment with my parents. She had never sat in an initial visit with a new patient and spouse where they seemed so accepting of the diagnosis.

It is true, my Mom is not afraid to die. That does not mean she is not afraid. She is terrified of the process to die. And honestly, who wouldn't be? I cannot imagine how heavy the burden would be to know when and how one would die. When I really take the time to think about how difficult that would be my respect grows even more for the way my Mom has handled this.

Nothing in our life changes except perhaps how we will be defining our days. Good days will be days where my Mom is comfortable. She use to love getting out and doing things but it is just getting to hard. Her body is trying to conserve its limited energy for the essentials - like breathing. She enjoys sitting and listening to music and relaxing with one of us by her side. She and I had the best night simply sitting and hanging out.

Everyone tries to ask in a roundabout way what is coming, but the fear of the unknown is usually worse than the actual knowledge. As her ability to breathe decreases she will wear her BiPAP and Sip and puff machines more often because they will give her relief from feeling depleted of oxygen and will temporarily relieve her of the exertion that it takes to breathe. These are not life sustaining devices, despite what we had previously been told about the sip and puff. There will come a point where the machines will no longer be effective enough on their own to keep her from feeling air hunger. That does not mean she will feel it or ever be aware it is happening - we will not let that happen. We have in place the medications needed to relax her body and mind to eliminate any awareness of those sensations. She simply sleeps through it. As her carbon dioxide levels continue to climb her body will continue to be more sleepy and relaxed. She will slip deeper into her own deep sleep and it will be incredibly peaceful for her.

Don't get me wrong, we are by no means there! Right now she is happy and alert. We still laugh and enjoy her companionship during the day. While before it was her family restricting visitors to preserve her energy, she has now requested to not have visitors. Our lives are a fluid routine that provide her with a feeling of peace and stability. While visitors are sincerely enjoyable it is the difficulty of falling back into her familiar routine that is the problem. When the balance is interrupted it can cause us all anxiety. Even the anticipation of un-routine things, like her Drs Appointment this week, made it so she could not sleep the entire night before. We appreciate your understanding and help in maintaining her feelings of security that the consistency of her routine provides.

We also recognize it can be hard to feel cut off, even for her family when we are away. A good friend from our Ward, Amelia has been a heaven sent resource for our family. Her Dad passed away from ALS and they used an instant message system that allowed friends and loved ones to send messages that would pop up on the screen and could be read throughout the day. My Mom said she would love something like that and so we have enlisted Brian and Mark's techy talents to figure that out. When they have it all set up we will get instructions out on how to send her messages. You can still send them to my e-mail and we will read them to her. If you really want to make her day - comment on her blog. No really, she loves to see that people stopped to check on her. Even if it is just says hi. If you don’t have a Google account and it is making you choose anonymous, just sign your name.

We have a lot of things to catch up on, some events are still from Christmas. Stay tuned I have posts set to go up automatically since I am writing this post from my red rock home here in UT where I plan on playing nonstop with my boys. My Aunt, Mark, and Brian will all be with my Mom over the weekend and I will be back next Monday evening. She is in good hands...but I still wanted to wish her goodnight!

Dear Momma,

I miss you already but it feels good to be home. I forgot I still have my Christmas decorations to take down :) Oh well, they may be up until this summer because tomorrow we are chillin' in our jammies and letting the house be a wreck, even Caleb is playing hookie. Our drive was good but I didn't want to stop and by the time we hit the gorge the kids were done and really wanted to get their point across!

I brought your Phantom of the Opera CD and just before I hit insanely crazy I put it on and turned it up loud. I mean L-O-U-D. As in drowning out any possible noise that could escape the back seat. Don't worry only the front speakers were on. I sang on the top of my lungs. I looked in the review mirror after a few minutes expecting to see crying tear streaked faces, and instead I saw three wide eyed, open mouthed, quite boys. I can’t decide if their shock was from the knowledge that there is something in this world louder than them or from my awful singing! Caleb loved the main title so much and he was pretending to play an imaginary guitar and yelled I love ROCK! Don't worry we will be working on their cultural sophistication and won’t let you down.

Do you remember when we went to the Phantom? We went twice but I especially remember the time you let me wear your black dress with teal sequence. I felt so grown up. Thanks for making sure we had all sorts of experiences growing up. As Caleb would say - you ROCK!
Night Night! Love you!

Retraction

I am going to have to retract my earlier statement that we had not seen much digression in my Moms breathing. As I sat by her at church her breathing felt off to me and when we got home I examined her closely and sure enough she was retracting. Moms with asthmatic kids develop a sixth sense for retraction. Trust me, I'm a Pro!

Retraction is when the body has to use the accessory muscles to breathe. From the outside it looks like the skin sinks in at the base of the neck and also at the rib area when breathing occurs. It means the body is having to work really hard to breathe. So chances are good her numbers will not be good tomorrow. Her fasciculations have also become more pronounced and have spread to most of her upper body and can be seen almost all of the time.

Today she became too weak for the stairs. We moved her room downstairs to the family room and created a playroom in the master bedroom. Actually the Grange boys from our Church came and moved both rooms in about 5 minutes. They were amazing! They must have learned it from their amazingly hardworking babysitter growing up. Oh wait, that was me! Just kidding, well not about being their amazing babysitter...its obviously much to late for me to be updating this blog!

My Mom had a great day Thursday but since Friday morning she has been so weak its hard for her to even walk and lift her feet. She has a hard time keeping her head up and so she walks looking at the ground. Its like shes literally shrinking.

I know I don't even have to ask, but please pray for her.

Medical Update

Physically my Moms body is very weak and we continue to see her strength decline day by day. She has lost almost all use of her right arm and hand. She can no longer grip with her right hand which means she can no longer write. We are getting along okay with the use of her ipad and hopefully the people responsible for her speaking device will get their act together and actually submit it to insurance - a whole month later! Grrrrr! Not having a grip is also making it extremely difficult for her to hold the wand for her suction machine which is complicating things. When I have tried to help her maneuver it I trip her gag reflex....so we are still working on that one! So far she has been fine driving her wheelchair by placing the joystick in the hallow of her thumb and pointer finger. If that eventually becomes to hard they can adapt her wheelchair to be maneuvered with her chin which is pretty amazing.

When she was first diagnosed they put her on Rilutek which is the only drug used to treat ALS. "Treatment" for ALS means maybe extending life for 3-5 months by slowing the progression. At that time she said she only wanted to take Rilutek until her quality of life was getting poor and then she wanted to go off of it. This week she made the choice to stop taking it.

As for her breathing its hard to tell if there has been much of a change. If she is changing it is definitely not as noticeable as it was before. Although she is not attempting any physical activity anymore so we just might not be able to tell. Today we did increase the pressure on her cough assist machine to make her cough still effective. Her BiPAP machine has been at a pressure of 12 but today she felt like she wasn't getting enough air to be comfortable so we bumped the pressure up to 13. She has an apt on Tuesday afternoon at the ALS clinic and they will perform another Pulmonary Function Test. That will give us an indication for how much lung capacity she has lost over the last 3 weeks.

She got a sip-and-puff machine right before Christmas. A sip-and-puff machine is a non-invasive ventilator and can sustain her life indefinitely by breathing for her. The upside of the sip-and-puff machine is that it can be used as a portable BiPAP machine. This will allow her to leave the house and keep her oxygen saturation levels up. The down side is that it is a ventilator and by definition can bring heavy choices with it. While there are many people who choose to use ventilators it just wasn't what my Mom wanted and she has put that in her Advance Directive. For now she doesn't wear it very often, but that could change in the future.

She still has some use of her legs but even their strength is diminishing. She is still able to do the stairs with assistance as long as she has not taken Xanax. For the last few weeks she has been taking a small dose of Xanax some evenings to help her relax and get rid of anxiety. It makes her feel better but it also makes her body go limp and getting her up the stairs is not easy!

The good news is that she is no longer having the sympathetic nerve pain in her arm and shoulder. Once she stopped trying to sleep on her side and got use to the hospital bed, her pain in the night diminished. She still has aches and pains from her muscles deteriorating but we can give her something and it works to take that pain away. Its nice that when she is losing so much she is also losing the bad things :) We will celebrate that loss for sure!

Emotionally this has been a rough week and while we expected it, its still hard. We are having to evaluate the necessity of caretakers as well as if, when, and how long I will be going home for. There is nothing easy about these decisions and its made everyone a little bit edgy. The Holidays are over and its one more milestone that has flown by. There is nothing we can do now but give ourselves time to find new footing and meaning in all of this.

I know we all will, we just need time.

Good news...

Just wanted to let you know that today my Mow was up and feeling better. Looks like it was a little virus and we are grateful that she seems to be bouncing back pretty quickly!

Medical Update

There are more special events to share from our Christmas weekend but first we wanted to give you an update on My Mom since there have been a few changes.

Its looking like the continued writing on this blog will be mostly from me. My Moms right hand has continued to deteriorate and typing gets frustrating for her. I'm hoping she will be up to write a few sentences here and there but this week she has not been up to it.

Yesterday we had an appointment at the UCI Palliative Care Clinic and it was a good experience for us. This is how they explain palliative care:

"Palliative Care is comprehensive treatment that aims to improve the
quality of life of patients and their families when facing a serious or life
threatening condition. The word palliate means to ease or comfort through the
prevention and relief of suffering. It focuses on early assessment and treatment
of pain and/or other emotional, spiritual, or physical problems."

A palliative care Dr does not treat actual diseases they treat the symptoms and side effects of the disease to keep patients comfortable. We really liked the Dr and he informed us that the pain she is experiencing in her left arm each night is what they call sympathetic nerve pain. All of the painkillers we have are completely ineffective on that type of pain but we do have a few options. The most promising is a nerve block which she will getting on the 5th.

The palliative care clinic also has an anesthesiologist who makes sure that the medications we are using are the most effective as well as making sure that the medications do not interfere with one another. They changed a couple of my Moms medications yesterday and we will see how she transitions onto them.

While ALS is obviously not a party to deal with, it gives us peace to know that it is not a painful disease. The pain she is having in her arm is from the atrophying in her muscles and her inability to move her arm in the night. Most of us naturally move our body while sleeping if we are uncomfortable. When her arm becomes uncomfortable in the night her nerves tell her arm to move but since there is a disconnect to the muscle her arm does not move. The pain builds through the night until it wakes her up. During the day her pain in that arm is between a 0-1. When she wakes up in the night her pain is between 5-8. Hopefully the nerve block will eliminate that.

We also started Home Health Care with St Joe's this week and we are happy to have a nurse coming in and regulating her progress as often as they think necessary. I mentioned earlier how we are unique because my Mom is not on Medicare. At this point most people with ALS would likely go to hospice but with private insurance we will go through Home Health for now and eventually switch to hospice. So far we have been really happy with Home Health. They have been attentive and they will be sending the same nurse each visit so that she will be able to recognize changes in my Moms lungs as she listens to them each visit. She is on palliative Home Health Care which means they too are focused on her continued comfort.

The best news is that we have my Moms sister Pam here to stay with us during the week from now on. Two caregivers can care for my Mom but with my kids here its hard to have us both available. My Aunt will be staying with us through most of the week and then either John, Mark, Lisa, Brian, or Kate be here for the weekend to be our third caregiver. My Aunt is the best not only because she is incredibly fun but she is also a nurse! Even better for 10 years she worked for St Joe's Home Health and Hospice and still knows many of the people there. She brings my Mom a sense of comfort with her presence and its been great for my Dad to feel at ease enough to relax a little and get more rest.

My Aunt could not have come at a better time either because my Mom woke up dizzy and nauseous today. We are not sure if it is because she has a virus or if it is because we changed her medications yesterday. My Aunt knows what my Mom needs and worked with the Dr's to get it for her. She keeps my Mom comfortable and her sense of calm and patience keeps us from getting to worried. This is the first day my Mom has not been able to get out of bed all day.

She was running a slight fever at the Dr yesterday - 99.3 which is marginally high for her since all the women in our family consistently run around 97.6. That seems to indicate that this is probably just a virus and we are optimistic that tomorrow she will wake up feeling better. We took her off all the new medications just in case and after she feels better we will start them again, one at a time.

We are keeping her comfortable and with your combined prayers I'm sure she will be up and riding around in her new custom wheelchair that is being delivered tomorrow! Watch yourself on the roads in Brea! She hasn't been able to be independent for a while and you should have seen her cruising Lambert yesterday on our walk!

Medical Update

Once again, this is Lisa. I know you all come here anxious to visit with my Mom but you keep seeing more and more of me. Unfortunately that is because my Moms energy is decreasing daily. Even though our days are pretty empty, we seem to hardly have a free moment. Many of you have called or e-mailed in the last week - we appreciate it more than you will ever know. I'm so sorry that we have not been able to respond individually. This has been a really difficult week. My Mom has been delighted with the cards, e-mails, and comments from family, friends, people who know her children, and even strangers. Truly they light up her day! We thank you and hope you understand we wish we could do more to express our thanks right now!

The last week has been a frustrating and emotional one here at our house due to many different factors so bear with me as I try to get you all caught up...

The ALS clinic usually sees patients every two months to track their progress and get their patients the resources they need at that point in their care. For my Mom this timeline has not worked well because of the speed of her case. She has not been seen frequently enough for them to comprehend how quickly she is progressing. It was frustrating for us to feel that no one was monitoring her progress and walking us through this step by step.

We saw her becoming more uncomfortable this last week as her pain started increasing, which also started causing her some anxiety. We didn't feel like we were equipped to get her what she needed when she needed it. That feeling of helplessness is pretty brutal on us as her caregivers. Her pain is mostly at night and it has been keeping her from sleeping. As her muscles are deteriorating they spasm and leave her without the ability to shift her weight. That builds on her pressure points and was then compounded by her lack of sleep. It left her drained when the day began and made her dread going to sleep at night.

One side of our frustration is that as a professional stay at home Mom she does not have enough work credits to qualify for Social Security and disability benefits, which then makes her ineligible for Medicare. ALS is one of 2 diagnosis' that waves any sort of waiting period for Medicare benefits. The day you are diagnosed is the day you become eligible. Everyone in the medical field assumes we are on Medicare because everyone else is and with medicare everything is covered and there is no thought of pre-qualifying or limits. Thankfully my Dad has good insurance but we are having to play their game and jump through the right hoops with the insurance company. It is maddening when her condition is changing so quickly.

A friend of my Moms lovingly joked with her that, "even in death my Mom is an overachiever!" Her progress has been nothing short of shocking and its no wonder that we and her doctors can hardly keep up with her. The last time she was seen at the clinic her feeding tube surgery had been performed one week earlier. During that apt they did whats called a Forced Vital Capacity test. The Forced Vital Capacity test is one test in a group of breath capacity tests that can be a rough guideline for how a patients lungs and diaphragm are digressing. The test requires the patient to exhale as hard and long as they can into a mouthpiece or mask that is attached to a recording device.

For people with ALS the weakness and loss of their diaphragm muscles is what will eventually take their life. Without the ability to move air in and out of the lungs, oxygen levels drop and they can not breath out the carbon dioxide which builds up in their bodies. Some people choose to have a ventilator and a tracheotomy but my Mom does not want that. There is no magic number of vital capacity that life is lost because each persons body has a different tolerance. Some people can live for months on 15% capacity while others can not handle 25% capacity. The only concrete information I have been able to find is that below 30% can be life threatening.

Exactly 3 months ago my Mom had her 1st test and her FVC was 106%. Once again a overachiever, how exactly does one get over 100 %??? A month later she was down to 98%, which let us know that her diaphragm was starting to be affected by the disease. A month ago her FVC was 70% which was a significant drop for just 1 month. But that was the test we questioned the accuracy of since she had just had surgery and her stomach muscles were sore, which could have affected her ability to perform the test.

Since Thanksgiving we have noticed her breathlessness increase dramatically and her strength has decreased dramatically. We have been trying to have her FVC done again for some sort of indication of where she was at! We were finally able to get in today to have the test done and have her seen at the clinic by the head nurse. She blew a 35% on her first blow and then a 40% on her second. She is down 30% in 1 month! Her 106% was blown on 9/21 and that means she is down 66% in exactly 3 months! That's an average a 22% loss each month!

These tests are not perfect and ALS patients can plateau, or so we've heard - we have yet to see one! Please understand that this test is not a perfect prediction of what she will continue to do. There are a lot of factors that still have to be considered. We don't know what tolerance her body has overall and she might be able to tolerate living at a very low FVC. We also don't know what her tolerance to the BiPaP machine will be.

What the BiPaP machine does is inflate her lungs so she can exhale more than she could on her own. An ALS patient can not just be given oxygen because they don't have the ability to breathe it in deeply enough. That is what the BiPaP does for her. She wears it at night and then as much as she needs to and can during the day. If you want to see what it looks like look back a few posts and there is a picture of her with her mask on. It is pressurised normal air and she has done really well adjusting to the machine. The pressure can be very hard for people to tolerate. She started on the machine with a pressure level of 8. A couple of weeks ago she felt like she wasn't getting enough air while wearing the mask. We learned that when that happens we need to increase the pressure because it has to blow harder to get air to the bottom part of her lungs (which is where the deterioration starts). She is now at a pressure level of 10. The respiratory therapist told us the highest pressure he usually sees people tolerate is around16 but her machine can go up to 24. We don't know how high she will be able to tolerate the pressure.

All we can really walk away with from yesterday and the test results is the knowledge that her diaphragm and lungs are losing their strength as quickly as her body is. Yesterday, we ordered all of the medical equipment she will ever need. We have a gel pad and hospital bed being delivered today and hopefully this will help her with her pain at night. Her custom ride (electric wheelchair) cleared insurance which is great news and should be here next week! A loaner wheelchair should be here today or tomorrow. She also will be seen at a pain clinic starting next Tuesday which will help as this progresses to manage her pain and keep her comfortable.

There are simply to many factors for the clinic to give us any sort of reliable expectation for how much time she has left. Using averages and subtraction we can deduct that if she were to continue at the same rate she has so far, we have around 4 weeks left, give or take a couple of weeks either way. NO medical professional has ever told us that doing that kind of estimation is a reliable or even logical thing to do so please don't misunderstand that to be reliable medical information.

Even when we know what our path is, the reality check of where we are on that path can still be hard and even shocking at times, at least for those of us traveling with her. My Mom would have honestly been more heartbroken if they had told her that her FVC was at 65% capacity. She and I were talking this week about how a spirit as strong as hers simply can not exist inside her newly broken body.

She told us from day 1 that she had ALS.
She told us from day 1 this would be her last Christmas.
And before her test yesterday she told us that her numbers were going to be really low.
She hasn't been wrong yet and I'm not sure when we will start actually believe her :)

And now....shes telling us that her time is short.

Its amazing that this doesn't make her despair. She didn't flinch at her numbers today. It was like she was hearing the weather report. Actually, with all of this rain shes more distressed over the weather report because its interfering with her Christmas lights and weekend! That is not to say that she doesn't have moments full of anger, fear, and anxiety. If she didn't have those moments we might be insulted that she is so happy to leave us :)

But overall she is at peace. She does not want to suffer. She does not like feeling weak and dependent. In the beginning of this disease I was hounding her for the things that she had always wanted to do that we could accomplish before she died. Every time she would smile and say, "Ive done everything I ever wanted to do. I'm content."

Today all 3 of her kids will be here with our families for a 4 day Christmas extravaganza! I'm very confident that nothing in this world could compete with the joy that will bring her!

Truly she is content, and that is where we find our source of peace and comfort.

Genetics

Today we had an apt with the geneticist to help us decipher the results we got back on my Moms genetics (or at least try to). This may only be interesting for Caroline's relatives...for the rest of you - sorry this may be loooong!

Some facts to start out: Only 5-10% of ALS is passed through genetics and they classify those patients as having "familial" ALS. Of that 5-10% that have familial ALS they can only identify the gene responsible in about 25% of those cases. If they can not find it on a gene that does not mean that it is not familial. Familial ALS does seem to be more aggressive than non-familial ALS. They also usually classify it as "familial" if 2 or more people in a direct line have ALS. My Moms Aunt died of ALS, and it is suspected that my Moms Aunt's cousin also died of ALS. They have assumed because of the family history and the speed of my Moms disease that her ALS is familial, although my Moms Aunt lived for 3 years after symptoms developed so even compared to her Aunt my Mom is going fast.

Now for a quick biology recap: Each human has 23 pairs of chromosomes. They inherit one from each parent making a total of 46 chromosomes all together. On these chromosomes we have "genes" and there are over 30,000. A gene mutation or variation can cause genetic disorders either through a deletion in the sequence or additions. ALS is classified as autosomal dominant which means that only 1 parent has to pass the bad gene, a bad gene will dominate a normal gene. Theoretically this means that if someone has it on their gene - they will get it.

The majority of familial ALS is found on the SOD1 sequence. They are finding new gene mutations that can also cause ALS but these are still very new. In my Moms case there is no sequence alteration on her SOD1, TARDBP, ANG, FIG4 but on her FUS sequence there is a sequence alteration. They have it listed as FUS Variant 1: 3 base pair duplication of CGG. What does that mean? It means there is an amino acid change of unknown clinical significance. And what does that mean??? A genetic mutation of the FUS sequencing has only been linked to ALS in the last year, maybe two. There is simply not enough information to determine if this is what has caused her ALS. She did point out that a 3 base pair duplication is better, or less significant, than a 1 or 2 base pair duplication since it keeps the amino acid chain intact (if you understand what that means feel free to explain it to me)!

The only way to find out if that mutation is significant to her ALS would be to test her Dad and find out if he has this same genetic mutation - because it was his sister that died of ALS. Although if he passed this mutation onto my Mom, and it is a mutation that causes ALS, her dad "theoretically" should have gotten ALS because he would have to have the gene and the gene is suppose to be dominant. Only problem with that is he is 86 and does not have ALS. They explained that all of the genes interact with each other in different ways so someone can have a dominant gene and have it be dormant because of other genetic factors.

What we can conclude: a whole lot of nothing! We don't know if my Mom has familial ALS or not. She does have a genetic sequencing alteration that "could" cause ALS. It can only be proven by finding that genetic mutation in my Grandpa, to prove that the link between his sister and my Mom and also have one of my grandpa or his sisters descendants also get ALS - who also have this same genetic mutation on the FUS sequencing. That would show a direct link between the mutation and ALS. There are Dr's salivating over getting mine, my brothers, my Aunt, and my Moms cousins DNA...at least for me, its not going to happen.

No matter what my brothers and I have a 50/50 chance of having this sequence alteration. My mom has 2 chromosomes to pass so she either passed us her good or the altered. My Moms sister has a 50/50 chance of having that same alteration since one of her parents has to have the mutation. My cousins could have a 50/50 chance of having it - but only if their Mom inherited it like my Mom did. My Moms cousins have a 50/50 chance of having a ALS mutation but only if their Mothers ALS was actually caused by genetics.

It doesn't change anything about the way my Mom is treated, they just are looking for subjects that can help further their understanding of ALS. It is such a unknown disease and we have so little knowledge about what causes it. They are going to do another test on my Mom and look for yet another genetic alteration that they are just discovering the link between that sequencing and ALS.

For now, only time will be able to tell and there is no use worrying about it :)

Medical update

How do I start? I guess its time to be honest. My case of ALS will go down in history as being a very fast case. Here is how I see it. The ALS clinic is barely keeping up with my changes as their clinic is held every 2 months. They have no clue how fast I am deteriorating.

My muscles are atrophying to the point I am becoming hunched back. I can no longer stand up straight. This makes my shoulders and neck ache constantly.

I can no longer talk. I can't answer the phone, because you wouldn't understand me. Today we had an appointment with the Speech clinic and I now have a mountable computer on order that can be calibrated to read my eyes for typing! How cool is that?

My left arm is dead weight and swollen, but you already knew that. Now my right arm is almost gone and my right hand still works, for the most part, but my right hand is very weak. I can't really write my name anymore! Here it is Christmastime and I can't wrap or unwrap the presents. But guess what? It gave Ashlynn, my 10 year old granddaughter the opportunity to practice wrapping, and she did a great job. Thanks to everyone who has helped me wrap my presents!

My legs still work, sort of. I have to be helped into a standing position from a sitting position. Yep, it means I can't go potty alone anymore, I need some to pull down my pants, pull me from the toilet after my business is done and then pull my pants up again. sounds invasive and it is!The good news is that I have one of those fancy electric wheelchairs on order and it should be here just before Christmas! Watch out world, here comes Hot Grma Silly Caroline, get out of my way!

As for food, I still try to take a few bites of food, but I am getting most of my nourishment through my feeding tube. The good news here is that I have always considered eating a waste of time! Don't get me wrong, I like good food, it's just my motto has always been "I eat to live, not live to eat"

The only really scary part for me is the ability to breathe. At my 1st appointment at the ALS clinic they tested my breathing and I was at 106%. After my feeding tube went in, I was at 59%. Now I am guessing I'm about at 40%. Since the Dr. is on vacation until Dec 20, I can't have it tested again until then. This means my diaphragm is now affected. Meanwhile they suggest I keep my Bi-Pap machine on at all times. Can you imagine sitting around all day and all night hooked up to a machine with a pilot mask attached to your face, smashing the bridge of your nose as it keeps slipping down on my face, with a harness going around your head holding it in place??? I can't even unhook the strap anymore to free myself when I get an itchy nose, therefore I feel like a caged animal.

(A visual of her mask)

Oh, but it keeps me breathing! I decided when I first learned I had ALS that I would NOT be tracheated! Although my breathing is harder, I stand by that statement. No heroics for me, there are a lot of things I am afraid of, and being just a living vegetable is one of them, Heaven is not!

C C C machines

This week we had a delivery. A very nice respiratory therapist named Manny came by the house and dropped off 3 different machines I now need to use. A C-pap machine for oxygenating me, a Cough Assist machine and a mouth Cleaning machine for clearing out things that get caught in my mouth or throat.

As I learn to use machines to help me live, I often think that life isn't much fun anymore. I have realized that the only true pleasure I get is when I am doing something for someone else. I know I must now be served, but it is so totally unnatural for me! And I'm getting noticibly weaker. Can you tell I'm going through a rough period right now? Prayers wanted and I hope accepted.

Feeding Tube

As you can see, I went into the UCI Hospital to get my feeding tube put in. Although this is a state of the art Medical and Research hospital, The beds are still not comfortable. If you know me well, you know I am almost always cold. Here at the hospital I can say I was actually hot. The surgeon was wonderful, the nursing staff overworked but nice. I can tell you that I thought I had died and woke up in hell. I lost 3 pounds while there for my 42 hour ordeal. You can't believe how thirsty I was! I didn't sleep the entire time I was there except for short snippets that left me sore and uncomfortable. I came home much more exhausted, thirsty and hungry than I went in.

I had wonderful support while there from Larry and Linda Howson (Larrys sister) Denny Howson and my dearest Sister Pam and my niece, Kelly. Linda wiped my eyes dry, Denny kept an eagle eye lookout while Larry smuggled in the contraband. Kelly gives the most Wonderful neck, back and legs and my sissy knows whats really going on.
I am so glad that's over. I got a great nights sleep (11 hours) and I'm finally no longer thirsty! Part of my swallowing problems and thick saliva was from being so dehydrated. I'm good to go now!

Surgery update from Lisa

Just got word from my Dad that the feeding tube surgery was a success. They will most likely be keeping her over night. They have lots of help and comfort from my Aunt Linda and Uncle Denny who are vising from Washington.

Thanks for keeping my Mom in your prayers!

Lisa here...

I wanted to let you all know that I have stolen my Mom this week so that we could enjoy setting up the Christmas decorations together. It has been a priceless week for me to spend time reminiscing and learning from her.

Her strength continues to slide. We have had a few sad milestones this week. The first is that she no longer has the strength to push herself up onto a stool or get up from the ground unassisted. This has been hard in light of the fact we are decorating but she adapts with a good attitude. She does an amazing knee walk on the ground to the nearest chair or furniture available.

The second milestone is a little harder and was learned a little harder too. She can no longer drive and go out without assistance. Unfortunately this lesson was learned after taking a fall in a parking lot yesterday evening. Her left arm has no mobility left so she could not break her fall at all. Not to mention since she can no longer get herself up she was quite literally stuck on the ground with no cell phone and no one around to help her.

She can no longer lift her left arm and uses a swinging motion to get it where she wants it. Eating has also become much more difficult and we are hoping that her feeding tube will be put in a week from tomorrow.

That all comes out sounding so bleak and yet for us its normal day to day life. She goes about it all with a smile on her face and joking about the changes that have come. Last night we got laughing so hard all over something so silly and we couldn't stop laughing.

That's one of the hardest things about ALS - while her speech slows and her limbs lose strength her mind is the same and sometimes its hard for us to remember that. My mom is not an old lady, nor is she mentally handicapped. She is as smart and sharp as ever, if not more so from the unique perspective that she has. She is amazing and being here with her this week has been a joy and blessing for me.

Sometimes people tell me they don't know how to act and I think the most important thing is to remember to let her do for herself what she can and not talk down to her. Slow down, go her pace, and just take the time to enjoy the process and not worry about being productive. That's what it is about, the joy of the process - not always the ending result.

“Anyone who imagines that bliss is normal is going to waste a lot of time running around shouting that he has been robbed. The fact is that most putts don’t drop, most beef is tough, most children grow up to be just like people, most successful marriages require a high degree of mutual toleration, and most jobs are more often dull than otherwise. Life is just like an old time rail journey … delays, sidetracks, smoke, dust, cinders, and jolts, interspersed only occasionally by beautiful vistas and thrilling bursts of speed. The trick is to thank the Lord for letting you have the ride.”

-President Gordon B. Hinckely

Tests, Doctors and Results

Today was one of those days I usually despise and for good reason. 9:15 am ( I'm mot a morning person and have never been) Went to a really nice Dr. to talk about My Thyroid nodule, He said its nothing to worry about.

At 11:00 am we went for my Barium swallow, all I can say is that the test didn't look perfect. As I swallowed different thicknesses of Barium, which were all sickeningly sweet and chalky, from a honey consistency to a cracker with Barium spread on it and every thickness in between. It wasn't as bad as the needle and shock test, but I gaged on the pudding consistency. Every time I swallowed, the Dr and Speech Person would say "look, she gets most of it down the right pipe, but a small amount is going down the wrong part" or something like that. It lasted for 15 minutes or so. The bottom line is: Get a feeding tube put in while you still have your health, don't Wait until the last minute. So I'm in the process of scheduling that right now. I don't have to use it, just have it in place in case I wake up one morning and can't swallow anymore.

Then Larry took me to lunch at the Main place Mall food court. Because it was close to our 2:00 pm Dr. appointment with Dr De Silva. We had some time to kill so we went into Penny's Store and bought me 6 pair of "pull-up" old ladies slacks. Yep, I'm now officially Grma Irene!

When we got to Dr Desilva's office, he told us the genetic test was back. SO.....I do not have the common familial kind of SOD1, but I have the rarer kind of familial kind known as FUS, leave it to me to have the RARE kind of ALS!

Thick it

My swallowing is not good with thin liquids, in fact I choke almost every time I drink water! I used to drink about a gallon of water a day, simply because I love water, but since I got sick, I'm lucky to get a small water bottle down!

I have always taken pills by throwing them into the back of my throat and then guzzling water to wash them down, my record was 10 pills at one time, but no more! I was struggling to get down 1 pill at a time and choking every time I took a pill. This is not fun.

Then Larry bought me "Thick-It". At first I told him I don't need that yet. But he asked nicely if I wouldn't try it in some water. So I did!!!!!!!!!!!!!!!!!!!!!!!!! To my surprise it really has no taste and no gritty granules. It just made the water the perfect consistency for me to be able to swallow! Now I am a believer in this stuff, and I won't leave home with out it!

At today's assessment here is how I stand: My left hand continues to loose mobility every day a little bit, it is atrophying or clenching up on me and frustrates me when pulling up my pants.
I still can eat everything under the sun, but I'm slower at eating and sometimes I have to hold a napkin over my mouth because it seems like my tongue doesn't move the food around correctly. My legs still work well and so does my right hand, that is how I'm typing this blog. My speech has plateaued, but at night, when I get tired, I slur more. My end take on life right now it: Its GREAT!