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.