Difficult changes as ALS is progressing

We have been adjusting to our schedules with school and sports and life this fall. However, over the last month or so, Eric has been having problems with coughing and once and a while choking on food or excess saliva. I have been concerned about this and asked Airway Oxygen to come out to the house and check Eric's breathing. They came out last Monday and measured his forced vital capacity (FVC) and it was at 16% which is very low. His NIF measured -13. They explained that a hospital would use this measurement to see if he could be weaned off of a ventilator if he was placed on one in an emergency situation. He would need to be at least -20 to be weaned. I want to be clear that this doesn't mean he needs to go right on a ventilator at this point as he is still breathing on his own. This was hard news for us to hear and last week we had some very emotional times taking about the fact that the disease is progressing in an area we do not want to see happen. Airway gave us a lot of equipment to put into our daily routine which would be like "push-ups" for his lungs. We are now using a nebulizor with duoneb medicine and a cough assist maching twice daily and are trying to incorporate a bipap into our day and eventually throughout the night. We are doing well with the first two, but struggling with getting the bipap to be tolerable right now. We have had some serious conversations the last two weeks about a feeding tube, a trache with ventilator and even funeral arrangements at 2:00 in the morning when Eric couldn't sleep. We really haven't discussed his wishes in these areas before and as much as I didn't want to talk about it, I realized that I don't want to be faced with it if he should pass away and not know where to start. We believe this is something every couple should discuss before it is time as well as having a will and trust in place, guardians for your kids, etc. because none of us know when it will be our last day on this earth. More importantly, do you know Jesus as your Savior? It brings our whole family so much peace to know where Eric is going when he dies and that he will have a new heavenly body without ALS. We recently read about this in 2 Corinthians 5: 1-9:
Our Heavenly Dwelling
1Now we know that if the earthly tent we live in is destroyed, we have a building from God, an eternal house in heaven, not built by human hands. 2Meanwhile we groan, longing to be clothed with our heavenly dwelling, 3because when we are clothed, we will not be found naked. 4For while we are in this tent, we groan and are burdened, because we do not wish to be unclothed but to be clothed with our heavenly dwelling, so that what is mortal may be swallowed up by life. 5Now it is God who has made us for this very purpose and has given us the Spirit as a deposit, guaranteeing what is to come. 6Therefore we are always confident and know that as long as we are at home in the body we are away from the Lord. 7We live by faith, not by sight. 8We are confident, I say, and would prefer to be away from the body and at home with the Lord. 9So we make it our goal to please him, whether we are at home in the body or away from it. 10For we must all appear before the judgment seat of Christ, that each one may receive what is due him for the things done while in the body, whether good or bad.

This is so encouraging to us and our goal is to please Him while we are here on this earth. We had already committed to speaking at Celebrate Recovery group at KCC the night we got this news and we knew we had to testify to what God has done in our lives in so many powerful ways. We have decided to change some of our priorities in our week too. Eric has quit some weekly appointments we have been trying since the risk and time away from family outweighed the potential benefits. We want to spend our time with family and friends and be obedient to God when he asks us to do something in his name. We want to keep being in his Word and continue to grow.

We have told Zach about the changes that are happening and not as much with Maddie as Zach is so much more aware of what is going on. Eric was talking to Zach before bed the other night last week and Zach prayed for God to send him a good friend. Zach is doing so well at school, he was named Student of the Month in October and also made the basketball team. We decided to have a bunch of 7th graders over last Friday for a party and they had a blast. There were 14 boys here and they ate pizza, played video games and went through the woods behind our house on a "scare walk" that Eric asked some people to help throw together. It was a fun night!
This week we went to the ALS Clinic at Mary Freebed and were there for 4 hours meeting with all of the disciplines there (ALS Dr, Respiratory, Dietician, social worker, Physical therapy, and a pulmonologist) We retested his breathing and it actually improved to 18% this week so the treatments may be helping. The doctor also said Eric may not have the capacity to take the tests to get an accurate read and may be breathing better the tests show. At the clinic we talked more about a feeding tube and how to go about putting one in since his breathing is lower than they like to do it at. We have to be prepared that they may need to put a trache in during that procedure if his breathing becomes compromised. We really need to be prepared to have more care in place in case this is done. We had an X-ray taken of Eric's lungs to be sure there is no infection or bronchial problems, an ultrasound on his legs for the extreme swelling, CO2 level and other bloodwork done the next day at St Mary's and are waiting for the results. Lots of big decisions to be made so we need your prayers.
We are having Eric's family over tomorrow to celebrate Thanksgiving and Christmas (yes, Christmas) together and they are spending the night so we are really looking forward to that. Maddie and Grandma put the tree up together tonight. I love decorating for Christmas so I am excited to have the decorations out early.
My family received some more bad news today. My Grandpa just discovered he has colon cancer and my sister's CA125 number went up again to 74 I believe. They are both also in process of deciding what to do from here. I keep thinking about our eternal perspective in a world that is full of sickness. Fix our eyes not on what is seen, but what is unseen, for what is seen is temporary and what is unseen is ETERNAL!! Thanks for reading our very long update, it has been a while again and so much has been happening in the last few weeks. Despite the tough changes, we are really still doing remarkably well. Thanks so much for your prayers!!
We love you all,
Lori and Eric