The Waiting Game

We must be willing to let go of the life we have planned, so as to have the life that is waiting for us. – E. M. Forster
Source: Brainy Quotes

Giving up the life you had planned is one of the hardest parts of having ALS. That is why I named my blog Altering Life Expectations (however I changed the spelling to have the initials ALS). There are lots of other reasons that people’s plans are changed. I don’t know what it would be like to be living with terminal cancer, for instance. And countless humans have had dreams shattered throughout history. 

For me, coming from a family with 3 other ALS patients in two generations, I am waiting for the proverbial shoe to drop, so to speak, because the other three had limb involvement. So far, mine is restricted to the Bulbar region – speaking, swallowing, and some diaphragm involvement. It is possible that it won’t spread to my limbs before my diaphragm and other breathing muscles give up. But it is also possible I could lose the use of my arms or legs. Now that I am sleeping longer since my bipap settings were increased, I am dreaming again. The other night I dreamed that I had trouble getting out of the pool (the way we did as kids – right up over the edge). The pools where I do aqua fitness have stairs or ramps, so this did not fit my reality, but perhaps dreams may be helping me get ready for whatever comes. However, I have also been eating and talking in dreams, too, so there is obviously a lot of memory involved in dreams. In the meantime, I will carry on waiting and doing what I can still do.

I am also waiting to start the new drug that was approved by the FDA in May, Radicava. It will be available in one week. We are waiting for insurance approval and a call from a health educator. I will need to have a PICC line inserted. It is hard to have it so close but not know when I can start. And there is no guarantee that it will work, but the chance of having my progression slowing down 30% is worth the chance, if it is affordable. We don’t know what our insurance company will decide to pay. So I wait.

My stepmom, Anita, had her birthday last week when all of us Macdonalds were out of town. This is a picture of her with her son Brian on her actual birthday.

photo by John Saunders

We were able to go out to celebrate with her one week after her birthday.

photo by John Saunders

Joe and Sheila Erlach joined us at Wild River. Sheila and Anita went to high school together. Joe and Sheila and their five kids are the people I stayed with when I first moved to Reno.

photo by John Saunders

I tried to get a photo of the birthday girl at the head of the table but I had not put the lens on correctly. My dad did get a photo of me when I was trying to get a photo.

photo by John Saunders

But here is the birthday girl with her beautiful new bicycle, in front of their beautiful new home.

photo from Anita Saunders

On Wednesday I went to lunch with two fun friends, Melissa and Sabine. It was fun and relaxing!

Photo by Melissa Knight

On Thursday, I walked with Andy to our neighbors across the street, with two intentions. Andy was selling car wash tickets for his cross country fundraiser and I also wanted him to meet Olivia and her mom because he loves kids and dogs and he is willing to babysit to earn money. Olivia is going into 4th grade and she is the sweetest girl. I know her from working at her school. She had a present for me. I was so touched. Her mom said she was going to ask when she could bring it over.  And they said dad’s car needs a wash.

Olivia made the flowers out of pinecones. I will treasure it.

Stan went to a rocket launch at Black Rock Desert Friday to Sunday. I opted to stay in Reno and get our cars washed at Andy’s fundraiser.

I do have a sore throat and that brings up the ALS Emergency Room and EMT Information sheet I carry in my purse.  All of us humans are waiting to die someday. My someday will probably be sooner than I was planning. It covers:

Shortness of breath – do not give oxygen for shortness of breath or low SpO2 unless I have another respiratory condition that requires it. I may need noninvasive positive pressure ventilation to expel CO2.

Oxygen may not help and may mask respiratory failure. My lungs are healthy, but my muscles, including my diaphragm are weak. I am using a Bipap at home so the settings should be the same, or ideally my Bipap from home should be used. If not using Bipap at home, a Bipap with a pressure of 12/6, backup rate of 10 with titration may help. 

Laying me on my back: It may be difficult for me because of the possibility of CO2 retention due to diaphragmatic weakness and aspiration due to poor ability to protect my airway. I may be able to lay on my back if using Bipap or non-invasive mechanical ventilation.

Avoid: Paralytic or general anthesthetics, narcotics or muscle relaxants unless absolutely necessary. If used, the ability to rapidly assist ventilation non-invasively should be available.

I have a gastronomy tube, please use that for administration of “oral” medications.

I am not able to speak at all, but I UNDERSTAND what you are saying.

Speak to me in a normal voice and ALLOW ME TIME to respond.

My caregivers and I are extremely knowledgeable about my condition, treatment needs and equipment. Please work with us.

In addition I have a written request for 

  • no morphine
  • antibiotics
  • nebulizer
  • bipap from home
  • no tracheotomy
  • no invasive ventilation.

All of these requests are to increase my chance of surviving Pneumonia or Flu.

And then to add some humor to my request, I read this today and it was so funny I want it on my advance directive!

My last wishes are to empty a whole jar of pop corn kernels in my feeding tube, so my cremation will be epic.

Thinking about life and death 

I recently read a book about a year in the life of a medieval woman.

It was very interesting to contemplate what life was like for people back then. The woman’s family lived on a feudal lord’s property. Death of babies and children was so commonplace, it was a wonder why some siblings survived and others didn’t. The town shepherd got a splinter from a split rake handle and it got infected and he was dead within a day. Neighbors of the main character’s family had a mother who didn’t speak clearly  “one could never talk to her for no one could understand what she said, only a gobbledy noise came from her” and she just sat idle in their cottage caring for nothing. Her kids were ragamuffins who begged for food from neighbors. I thought, what if she had ALS? Their neighbors looked upon her family with disdain.

This book made me think about a few different things. One, that I am lucky with technology helping me communicate, suction stuff I can’t swallow , and helping me do therapies to improve my life quality. Also with antibiotics, anti-inflammatories, ALS slowing, and other drugs greatly increasing my quality of life and potentially prolonging my life. Let alone a feeding tube and formula giving me complete nutrition. These are things that weren’t available for most of history. So now we are able to live longer even when sick.

With so many babies and children dying in medieval times, it made me wonder, what is life? My own daughter Anna only lived a moment after birth. What did her life mean?

My older brother died in an accident when he was 19. Why did he get less than two decades when I have had more than five and a half?

These questions can’t be answered. Some religious faiths may give people guidance on these questions, but for people like me with nebulous spirituality, there will be no clarity.

And now we can make decisions about our own deaths. When Stan and I did estate planning about 12 years ago, we both decided our end of life plan would be “no tubes”, imagining us old like our grandparents and intubation prolonging a fairly low quality life. Home health gave me a Physicians Order for Life-Sustaining Treatment form.

My POLST form folded to protect personal information

Obviously I need to update my Living Will because I can still have a high quality life with a feeding tube. I assumed I would be like 95% of ALS patients, and option to not have a tracheotomy to prolong my life. My husband and I had a candid discussion with my nurse practitioner about the implications of signing the form. If I say no endotracheal intubation,  she suggested I might want to look into hospice because I am already having breathing problems and diaphragm spasms. She has a long history of hospital work and was able to explain how patients are weaned off endotracheal intubation – it is turned off to see if the patient can breathe on their own. She worried that I would never be able to wean off it. And it is extremely uncomfortable for conscious patients.

I know that ALS patients are usually able to make the decision to go to a full tracheotomy without having to go through the endotracheal intubation process. I have been worried about the amount of care that will be required to keep me alive in the later stages of my disease. I don’t want to burn through our savings paying for 24 hours care for several years, nor do I want to be a burden to my caregivets.

Some states, including California and Oregon  (I have a sister in each state) have right to die laws. I heard a beautiful story about a woman with ALS in Ojai, California who hosted a 4 day party, culminating with her physician assisted death at sunset on the 4th night.

There are so many profound decisions to be made as humans today. Perhaps it would be easier to live with death much more common as it was in medieval times. You wouldn’t be faced with  moral decisions on the end of life as we are. I have not yet decided and can now see what a difficult decision it will be.

It is sobering to be thinking about these things only 2 months since diagnosis.