As most of you know, we are getting ready to leave for warmer weather in a few days. We rented a bungalow on the beach at Punta Uva near Puerto Viejo, just south of Limon on the east (Caribbean) coast of Costa Rica (in case you are in the neighborhood). We’re hopeful the warm weather will help Phyl’s muscles as the cold has been hard on her. We are back in Massachusetts on March 9.
Yesterday, she had her final visit and evaluation as part of the 6 month experimental drug trial for Amylyx. I thought that her loss of muscle ability during the time she was in the trial was slower than the previous 6 months and the test scores seem to agree. In fact, her muscle strength scores yesterday were no different than in November or January. This was really good news and a huge relief! It is not a “cure” but hopefully has slowed down the progress of the disease.
She is still weak and her stamina is low but she is able to climb stairs (with effort), drive her car, and walk around the grocery store with a cart (and me). We celebrate small victories! Her left hand is pretty useless in the cold but gains function when warmed up (so she can type with two hands on her laptop most days). All in all, she is doing much better than expected based on what we know about ALS. I don’t know if this is due to the Amylyx or if her rate of disease progression is just slow but we are grateful.
Although the Amylyx trial is over and she is off the drug for now, the company is offering to allow us to continue on the drug for the next year. It won’t start until we return from Costa Rica but we are really pleased to have this opportunity! We are watching two other experimental drug trials as well to see when they open and we’re hopeful the stem cell research progresses. Ever since the Ice-bucket Challenge there has been much progress in research which hopefully will result in a treatment someday soon.
AT THE WOMANS MARCH
One of her big days this month was “walking” in the Women’s March in Northampton with her friends who pushed her in her wheelchair. Frankly, this was a big step as she had not wanted to be seen in a wheelchair but she so wanted to join in the march. She was very grateful to be able to participate. Her friends have been great…. lunch, coffee, museums and other outings. The past two weekends we have visited Brian, Belita and Elena in Rhode Island and then Jeremy and Sam in Burlington VT. And Saturday, we head for Somerville to spend the weekend before we leave with Jake, Shannon, Noah and Colin!
Nothing cheers Phyl up more than when friends and family rally around her. You remember the haircutting fundraiser she did for the ALS Association of Massachusetts in October. She loved doing this and felt warmed by all the support. The ALS Association put $3 million into the Amylyx trial, so we feel pretty supportive of this organization. And she is doing another fundraiser, this time a Walk-a-thon, on May 12 in Northampton.
I’ve attached the link to her Walk-a-thon team page below. She named her team “Phyl-in-tropics” and she plans on wearing a flower/tropics shirt to the event. We hope you can “join her team” and walk with her (or help push her wheelchair) and wear your own flower shirt. But if you can’t be in Northampton on May 12, you are invited to make a donation in her honor. Here is the link:
The amount you can donate is much less important than her just seeing your name on the list!
Our next big “test” will be on March 16 when we go back to UMass Medical Center for her ALS Clinic visit and they measure her lung capacity. For now, she is good. And of course I don’t know what the long term will bring, but we are focusing on the near future and warm weather for the next month!
It has been 2 months since I’ve sent you an update on Phyl, so here it is….
It has been relatively quiet lately and we have settled into a pattern of living which I suspect will continue for some time. After the initial diagnosis and scrambling around trying to figure out what we can do to support Phyl, it is kind of nice to have some “boring” days! We wake up with the sun and Phyl gets me the newspaper and prepares our vitamins. I make her a cup of coffee and we discuss our day over breakfast. Some days there is something exciting like a shopping trip or doctors appointment, and Phyl has coffee or lunch with her friends 3-4 times a week. We prepare dinner together and settle down for some television before bed. Simple days…..
Phyl did start back at pottery last month and found it a bit difficult. She has less strength in her left hand and it makes centering the pots difficult. I don’t know where that will go but it will be a big loss if she can’t make pottery any longer. The bigger problem is with walking. She has little stamina and struggles on steps. Just today we got her a second foot brace which put a bit of spring in her step! But walking is surely a struggle.
Her Physical Therapist recommended that we move our bedroom to the main floor and we had a contractor who specializes in accessibility look at the house. He recommended a major renovation of the main floor bathroom to make it power wheelchair friendly. We’ve decided not to do that (yet) both because of the hefty price tag and the hope that an ALS drug will emerge in the next year that might make it unnecessary. We have decided to remodel my office on the main floor however to make it our bedroom, probably over the summer. This will allow her to avoid steps. The power wheelchair requires much more room than a manual model, which I suspect we’ll need sometime within the year. We may also look at a power scooter, both for around the house but also so she can walk the dog. She is missing getting outside to walk Riley!
As you know, she is taking an experimental drug called Amylyx. I know she was disappointed that the drug did not reverse the disease symptoms after being on it for 3 months (we are pretty sure she is not on the placebo). We are hopeful that it is slowing down the progress, but we have nothing to compare it with. The trial will be over in January, but the company has announced that they will continue to allow those in the trial to take the drug if we want. She was pleased and will surely continue as there are no major side-effects and it may be helping a bit. There are several other drugs being tested and we are hopeful one will make a difference someday. It is a bit of a race against the progress of the disease.
Phyl’s attitude goes up and down. When we focus on today, we are fine. Whenever she gets really tired, her legs and left hand don’t work well and it causes her to worry about the future. The initial confusion and anger have largely been replaced by a deep sadness. We could learn to live with her level of ability at present, but the disease is inexorable….. it just keeps progressing. Fortunately it is slow but we continue to watch for yet another loss in physical ability. Its a tough way to live. Her friends help by keeping her occupied.
In the near term, we are excited to be seeing the family around Christmas. We were in Rhode Island last weekend visiting Elena (and her parents) and tomorrow we head for Somerville to celebrate Noah’s birthday and Hanukkah with Colin (and parents). Phyl looks forward to these trips, but they are getting more and more draining on her. Nevertheless, we have plans to spend a month in Costa Rica in February. I hope the warm weather is good for her muscles. The bungalow is 50 steps from the beach, so we won’t be walking too much!
I’m on sabbatical leave until September so I can be supportive. I”m not sure what we will do when I go back to work. At present, she can drive and get around on her own. If she needs help, we’ll have to look into getting an aide to come in a few days a week while I’m on campus. Fortunately, we have a Long Term Health Care Insurance policy that should help pay for assistance when it is needed.
So that’s it for today. Phyl is out with friends this afternoon and I am about to log into a webinar on ALS. I’m becoming something of an expert.
It has been a while since I’ve sent you an update (and many of you have spent time with Phyl/Mom recently). Nevertheless, I thought it as time as we are between adventures!
As many of you know we spent the last two weeks in September “on the beach” in Rhode Island. We rented a small house which was about 50 yards from the beach and had a great time. One week we had the remnants of a hurricane (very exciting) and the next week beautiful sunny days.
Best of all we got to see all of our sons, daughters-in-law, and grandchildren, our niece Corey (and we ate lobster). And next week we are off to Arizona. We rented an RV for two weeks and plan to visit the Grand Canyon, Sedona, Tucson and surrounds. Looking forward to visiting the desert!
In between the two trips, we had quite a few doctors visits (which kind of comes with the territory). We visited both of Phyl’s neurologists. Dr. Jim Russell at the Lahey Clinic in eastern Mass affirmed that Phyl’s lung capacity has not deteriorated in the least since the onset of symptoms. This is really good news. She is losing muscle capacity in her legs and left arm but Dr. Russell said it appears to be slow. We also visited Dr. Bob Brown, the neurologist at UMass Medical in Worcester. Here is a story about Dr. Brown that might interest you: https://alsadotorg.wordpress.com/2017/07/26/new-comprehensive-als-review-published/amp/
Phyl continues on the study trial for the experimental drug, Amylyx, with Dr. Brown (we are pretty sure she is not on the placebo the drug seems to make her tired). She is experiencing a little queasiness and slight headaches which may be associated with the drug but we don’t know for sure. The side effects are minor if the drug does anything to slow down her development of symptoms. We have no idea if it is making a difference however since her symptom development is slow anyway.
The doctor says if the drug shows any sign of positive effect, the company has pledged to continue to make it available to everyone in the study trial after the 6 month study is over. So we continue to hope and pray.
The most fun we have had this month was the ALS Fundraiser that our friends organized for us. They had a goal of raising $3,000 for ALS research and at present they have exceeded $4,700. Phyl had a wonderful day surrounded by friends and family. There were 5 haircutters working mostly full time all day on a Sunday with all of the proceeds going the the Massachusetts Chapter of the ALS Association (which by the way is supporting the Amylyx study trial). They also auctioned off pottery donated by her friends. Thanks to everyone who contributed to the fund raiser. The results are linked here: http://web.alsa.org/site/TR/3rdParty/Massachusetts?team_id=363871&pg=team&fr_id=12690
So that is what is happening. I can also tell you that Phyl has good days and bad. The day of the fundraiser, she was happy and grateful. She got to see Noah, Colin and Elena who always light up her life. When everyone left she had a really tough day. She was exhausted and had difficulty walking which often follows busy days. Yesterday she tried to walk Riley in the fields behind our house and had to turn around rather than walking up a small hill. This is really difficult for someone who was climbing mountains in Colorado last year. She is trying to learn to not push herself so much and take breaks during the day.
Today she went to a yoga class in the morning, ran errands around town, and right now she is cleaning up the garden. She had to ask me to help with some of the gardening stuff and as much as I love helping her, she still wants to do everything herself. This is hard on her.
You can (and do) all help by staying in touch. When I got back from my own errands today, I found her in a lounge chair on the back deck (in the sun) talking with her Aunt Helen. Her voice was animated and engaged. This really helps! She knows she is loved but does appreciate it when someone reaches out to say they care.
So…. that’s it for now. I didn’t think I had much to say, but there it is. I feel ever so grateful that I have a sabbatical leave this year and I can spend so much time with Phyl. We certainly enjoy the vacations but we also love just being together at home, cooking food, and watching TV. Last night we (well Phyl didn’t quite make it to the end of the game at 1:00am) watched the Chicago Cubs win the 5th game of their first playoff round! Go Cubs!
We finally got the appointment to begin the clinical trial on Amylyx on Monday. Phyl will be the forth person in the world to take this new experimental drug. It seems the first three people to get it from UMass Medical found the taste horrible. The company has redesigned the drug to taste better (we hope) and they are ready to continue the treatments again. It is a powder she mixes with water daily. If you want to read about the drug, see:
Phyl is a bit worried about her potential reaction to the drug. It is
composed of two other drugs that have been tested on humans individually but not together. Most people have tolerated the two drugs but some have had nausea. She has a very sensitive stomach and expects to react badly (that is if she doesn’t get the placebo)!
FIRST DRINK OF THE EXPERIMENTAL DRUG – AMYLYX
They hope to signup about 150 participants in the trial from all over
the U.S., with 50 getting the placebo and 100 or so getting the drug.
She took one of the approved ALS drugs a few months ago and reacted badly. The neurologist at UMass Medical, Robert Brown, seems pretty high on this drug so we are trying to be hopeful.
It has been some time since I’ve sent you an update on Phyl. This is mostly because we have settled into a pattern and there is much less “excitement” lately. We are living one day at a time and trying to enjoy life!
Here are a few updates:
Phyl has been getting used to her new carbon, flexible foot brace that gives her left foot more lift. This allows her to walk (mostly) normally without getting too tired. She is wearing it during the day, on errands, for exercise and gardening, but not always around the house. It has made a huge difference in her attitude! We bought her a couple of walking sticks today, to help with her balance. They are less cumbersome than a walker if she is going a distance. Her biggest disappointment has been having to give up walking Riley (our dog) in the fields behind our house because it is just too rough and tiring. I’ve inherited that job! But we have been biking and last week we went river rafting with our niece Corey, Sam and Jeremy. Since the rafting trip required only upper body strength (to paddle) she said she felt “normal” and had a wonderful day on the river.
THE NEW FOOT BRACE MEANS SHE CAN STILL CUT THE LAWN
We visited UMass Medical Center in Worcester last week to be evaluated for a new drug trial, which I suspect she will be accepted into. It is for an experimental drug called Amylyx 0035. It is a combination of two drugs that are currently used for other diseases and seem to have some effect individually on slowing the rate of muscle loss for ALS patients. This is the first time the drugs have been tested together so we don’t know how it will work and we also don’t know if she will be in the placebo group – but it is worth a try. Another aspect of the drug trial is that she will be monitored really closely and given an MRI and PET scan. If there is anything worth noticing about her current health, they will see it for sure. We hope to start the trial sometime in the next few weeks.
We were hopeful of getting into a new stem cell trial which just opened but they are not accepting anyone over 60. She was a bit disappointed as she kind of expects the new experimental drug to cause nausea, since the approved ALS drug Riluzol (which she had to quit) made her sick. We are also questioning the value of taking the newly approved ALS drug, Endavarone, since it is a daily infusion and requires a visit to a doctors office. The effect on ALS was real but minimal. It will be available in October so we will decide then. The Amylyx is a powder taken with water so much easier to take. We’ll see….
HIKING WITH JEREMY IN VERMONT – WITH THE NEW STICKS!
The good news is that the disease progression is very slow, in fact much slower than average. From everything I’ve read, the rate of progression is unlikely to change significantly over time. Also, she has been tested for lung capacity three times over the past year and there is no loss of capacity (this is the big worry). While she is losing strength in one leg, it is slow and other symptoms have not yet developed. I follow the research on ALS and it seems that there are several new drugs being developed along with the stem cell treatment. The basic research on the cause of the disease has exploded over the past few years. The ALS community is truly excited and hopeful. So as long as the disease progression is slow, we remain really hopeful.
The biggest news has been the effect of physical therapy. She has a PT who knows ALS and has given Phyl appropriate exercises. The deal is we want to strengthen the muscles that are functional and not stress those that are struggling. Her back is feeling much better and even her yoga instructor said she is looking stronger. Most days she feels pretty good as long as she limits her activities and is careful walking. Last week she put her foot down while bringing her bicycle to a stop and her left leg gave out. Fortunately, she ended up falling on the grass. As long as she pays attention, she is fine but the weakened left leg can be a problem. Phyl knows her legs will get weaker over time but she is doing everything she can to stay strong as long as possible. Her PT exercises seem to be helping her balance.
HANGING OUT WITH PHYL’S “POPS” IN SOUTH HADLEY
In the mean time, we are living our lives. Jake was here this past weekend with Noah and Colin and we all had a blast, bike riding and hanging out in the pool. On Friday we are picking up the keys for Brian, Belita and Elena’s new house in Rhode Island and we hope to have a couple of beach days while there! Phyl has two groups of girlfriends visiting in August and September, one from high school and the other from college. And this week she is going to a museum in eastern Mass with a gang of her local friends. Right now she is at the corner coffee shop with a few of her friends. She is busy!
I am on sabbatical leave for the next year, so I’m available to provide support 24/7. Today I drove her around on her shopping errands and we went out for lunch. We decided we need to sell one of our cars because she has a bit of difficulty with the clutch on my car and it will get worse (both the car and her leg). She was disappointed as she loves to clutch and shift gears like a race car driver….. but we’ll go look for a sporty automatic so she can still zip around town!
Of course we are not kidding ourselves about this disease. The long term prognosis stinks. Phyl’s attitude is amazing (most days). After a few days of “the sadness” which crept up on both of us last week, she woke up one morning and said “I’m tired of being sad… I’m going to be happy again!” We meditate together and do some mindfulness exercises to try to stay in the day. When we focus on today, things are okay. When we think too much about the long term it becomes more difficult. We had a great day today!
Of course we will prepare the house for her having less mobility when the time comes. We will move our bedroom to the main floor and make room and get some ramps for a power wheelchair to get around. I suspect I’ll need to get a wheelchair accessible van eventually. Folks with ALS who can walk often try to limit their walking as it is so tiring. Someday in the future, hopefully not soon, we will make the adjustments.
WE RODE OUR BIKES UP TO THE MONTAGUE BOOK MILL
But for now we are staying active and enjoying each other’s company. We look forward to two weeks in Rhode Island on the beach in late September, a visit to friends in Arizona in late October or November, and then someplace warm in February. Most days we work in the gardens, hand out by the pool, and run errands together. We’ve become very appreciative of everything we have! We are blessed to have so many family members who live so close. And if you are one of the family members who is far away, please call Phyl to check in. She loves hearing from you….
We had the best weekend in months at 123 Harlow Dr. with lots of family around, the pool is open, good news from the Colorado Gerber/Marines, it was Fathers Day, and Phyl had her energy and appetite back!
For the past week, she has been suffering with nausea and fatigue which we thought might have been caused by the only ALS drug currently available, Riluzole. After consulting with two neurologists we all agreed it was not worth the small, potential benefit from the drug. As soon as she stopped taking it, she felt better. She ate like a horse yesterday – without nausea… AND she woke up this morning with renewed vigor.
She said to me this morning, “life is good when you are feeling good!” This was the cumulative effect of the weekend with our family, Brian and Belita’s good news about having their offer accepted on a house in Rhode Island, no nausea, and she was wearing her new foot brace. She said before she put it on her back hurt, her foot dragged and she was walking really slowly. With the brace on, her back pain went away and there was a spring in her step (literally as the brace gives her left foot lift).
She is working right now with her new Physical Therapist, who has worked with dozens of ALS and MS patients. She will help me figure out what needs to be done to the house (eventually) like ramps, hand rails etc. We like her – and she comes to the house!
That’s the good news…. the sad news, is that Phyl’s Uncle Marty died. Phyl and I used to visit Helen and Marty in Concord when we were both in college and they became very special people to us over the years. When Phyl was first diagnosed with ALS we were in Eastern Mass having met with a neurologist at the Lehey ALS Clinic, who Helen and Martin recommended. After we got the official bad news that day, Phyl knew that she needed to be close to her Aunt Helen immediately. Both Helen and Marty were supportive, caring and concerned – and it helped her a great deal to know how much she is so loved by her Mother’s sister.
On the clinical front….. we applied to be enrolled in a trial of a new drug, AMX0035. It is a phase two trial for safety and efficacy and will only accept 132 people (10 of them from UMass Medical in Worcester). The good news is that it is the beginning of a “cocktail” that addresses multiple causes and both of the drugs in the cocktail have been tested for safety independently. We spoke with the Head of Neurology at UMass Medical last week and he hopes Phyl will qualify for the trial. The weird thing is that her symptoms might not yet be bad enough yet as she needs a definitive diagnosis of ALS. Since the disease is developing so slowly….. we don’t know if she will be accepted. He was hopeful to get her in the trial however. Of course we are happy that the symptoms are developing so slowly…..
That’s my update for today and I’m glad it could be a (mostly) positive report. I am going to help her glaze pottery this afternoon as she can’t lift the glaze containers…. so I’m becoming a potter! We also just signed a contract to rent a house on the beach in Rhode Island (near Pt. Judith Lighthouse) for the last two weeks in August. So, “life goes on” as my Mother says.
It has been a month since my last note to you about Phyl/Mom. While we have been in touch with many of you and thankfully visited with quite a few family members this month, I wanted to bring everyone up to date on where we are at with regards to ALS treatments. Its complicated…..
First, there are several theories on what causes ALS. There does not seem to be one single cause for all people. Also, the disease manifests itself in different ways for different people. The biggest difference is where it shows up first. There is a type of ALS called “bulbar” which starts with the throat (swallowing) and diaphragm (breathing). This is really bad. Fortunately, this is not our story.
The other type of ALS starts in the legs and sometime hands/arms. Phyl has lost a lot of muscle in her left leg, some in her right leg and a bit in her left hand. The uneven gait this produces has also created back pain. The good news is that symptom development has been relatively slow and for most ALS patients, the rate of development seems to be pretty steady over time. Some people have a very rapid loss of muscle control. Again, this is not our story.
So….. the treatments that have been developed and are in experimental stages right now address several of the potential causes. It is likely that the causes interact with each other, and our new best friend, the head of Neurology at UMass Medical Center in Worcester, says any effective treatment is likely to be a cocktail of several drugs. So that is what we are looking at. The potential causes and treatments are:
1. Excess glutamic acid (glutamate). This is a known cause for some ALS patients. Glutamate is a normal body amino acid found in all of us. It is exceedingly high in ALS folks and possibly a cause of motor neuron cell death. The standard ALS drug, Riluzol, is known to reduce glutamate and also delay symptom development. Phyl has been taking it for about a month. She also avoids foods with glutamate (and Chinese food which still has monosodium glutamate in it even though it is no longer legal). Riluzol is very safe and seems to have no side effects.
2. Neuroinflammation – the inflammation of neuron (nerve) cells is almost always seen in ALS patients. The cause of inflamed motor neurons may be external toxins, so we are eating mostly organic, non-processed foods and Belita has taken away all of our toxic cleaning fluids etc. Inflammation can also be caused by excessive free-radicals which oxidize cell components and may cause neuron death. These can come from diet but we also produce free radicals ourselves. She is eating foods high in anti-oxidants and also hopes to start with the new FDA approved drug, Radicava in October. This new drug is a free-radical scavenger and has been shown to slow symptom development in ALS patients. The downside to this drug is that it is administered by infusion into the veins which requires a visit to a medical center – at least at first. Over time, they may give her a port which would allow us to administer the drug at home. It is administered every day for two weeks and then two weeks off… forever. This assumes of course that insurance will pay!
3. Another possible cause is poor function and death of the cellular mitochondria (produces energy in the cell). I really don’t understand the mechanism for this one but there is an experimental drug, Amylyx 35, which will be available for clinical trial in a few weeks that may help. We have a telephone conference call scheduled on Monday to be evaluated for this trial, which includes twice daily medication (a dry powder mixed in water). This one is a combination of two different drugs which I can’t pronounce. Both have been shown to be safe individually and effective in slowing down disease progress. If we are accepted into the trial (and don’t get the placebo) Phyl will be one of 132 people who will test the drugs together for the first time. It seems to work in mice! These drugs are thought to be neuro-protective but the mechanism isn’t clear. Its a gamble and a hope. The folks are UMass Medical are actually pretty excited about this one. There are other experimental drugs but we have to do them one at a time so we are betting on Amylyx 35.
4. Another potential cause is heavy metal toxicity, but this is rare. Phyl is being tested because of her use of pottery glazes. It is unlikely, but the folks at UMass Medical are being really thorough. They have done extensive blood and DNA testing (cost $9,000…. thank God for insurance). More blood tests this week as well.
5. There are other possible causes but no scientific evidence for any. There are also lots of “quacks” who promise a cure. Fortunately the ALS community is strong and communicate with each other. There are several online forums that routinely examine all claims and tend to expose the scam artists. The medical community is supportive of anything we want to try, as long as it does no harm and doesn’t cost us the house. So we continue to explore….
6. One of the other potential treatments that may become available later this year would be stem cell infusions. Our research neurologist who has seen the preliminary data on a treatment called Nurown (they create stem cells from your own bone marrow) encouraged us to get involved in the Amylyx 35 study now and not to wait for the high-tech treatment – so we are following his advice. Maybe later….
Phyl is getting her routine therapeutic care from the Lahey ALS Clinic in Burlington, MA. We really liked the staff and will continue to see them every 3-4 months. She was also examined by the head of neurology at UMass Medical, which is a treatment hospital but specializes in research. That is where we will go for clinical trials. Finally, we saw a Functional Medicine doc last week and are working on cleaning up her diet and taking a few supplements that are believed to support overall health (probiotics, digestive enzymes, fish oil, and some super vitamins). Of course we also have Belita to give us guidance on how to eat healthy and we are making changes in what and how we eat. And today we picked up our first medical marijuana treatment which may have therapeutic value for ALS but is mostly for anxiety. This may allow her to quit the Xanax and help her sleep.
Our objective is to slow down the development of the disease. It is unlikely that any of these steps will cause a reversal of symptoms (although a miracle will be accepted and appreciated). She is losing functional muscles in her legs slowly. We hope to receive a foot brace this week which will provide support for her left leg and make it easier to walk. She also has a new Physical Therapist who she likes and will work on the back pain, that we believe is caused by an uneven gait while walking. She is walking okay… better if she holds my hand. We also have a rolling walker (rotavator) for use if she knows she will be on her feat a long time. So I think we are doing all we can.
There is a lot of research going on – so with these steps and a whole lot of prayers, I remain hopeful. In the mean time, we are planning on spending two weeks on the beach in Rhode Island in September and hopefully a trip to Arizona in late October or early November. If all goes well, I’d like to get to “Saint Somewhere” (warm) in February. All of these trips will depend on the scheduling of the Radicava infusions, so who knows.
This email has been much longer than I expected but I wanted you to know the whole story. If you have questions about any of the treatments or experiments, please let me know. I’m happy to tell you what I know…. as this study has become my life. Well, that and keeping my wife busy and entertained. This weekend Stuart and Gretel visited and we went to see Helen and Marty and family in Concord. We also saw a soccer game in Somerville (Noah was great in the game of course and Colin was trouble) and had dinner and Sunday bagels with Milt and Florrie. It was the best two days she has had since the diagnosis!
We had some good news today and I wanted to share it. In order to participate in a Mindfulness Meditation Study with Penn State University, Phyl needed to have a FVC – Forced Vital Capacity (breathing) test done. We did it this morning and were a bit nervous, as ALS effects the diaphragm and chest muscles which control breathing. Kind of a big deal!
So her test results were completely normal. The disease has not impacted her breathing at all (yet). In fact, her FVC score was exactly the same as it was last August when we were concerned about her breathing. She does have a constricted larynx problem which is made worse by stress (which we have a lot of these days). But this is manageable with breathing exercises and anti-anxiety meds.
Damn good news….. which we really needed!
Otherwise, she is learning to talk about her disease (slowly). When we wake in the morning, it feels particularly heavy but she gets up and gets going and we’ve had some pretty good days. Today, we awoke to crying baby. Our granddaughter Elena is here! That makes her day.
We have our first ALS Clinic on May 15, which is the beginning of regular check ups including Physical Therapy, Occupational Therapy, more breathing tests, and a discussion on potential treatment. We are also on every damn waiting list I can find for Stem Cell research, which is supposed to get started this year at UMass Medical and Mass General. There are also several treatment drugs in the pipeline, but nothing terribly effective just yet. As long as her breathing is good however, we have time. So we get through each day trying to remember to be grateful for all that we have, especially our family.
While we have good moments (like when we are with our grandchildren), there is a heaviness we both feel that won’t go away. Phyl is having more difficulty walking. She is fine in the house and if we walk VERY slowly around the block with the dog she is okay. Yesterday I ordered a rollator, which is a rolling walker because she did much better in the grocery store pushing a cart than walking alone. It was a very difficult decision for Phyl but she was accepting that this was in the future (not too distant). She is struggling with not being able to do all that she wants and was in fact able to do a year ago. That said, she just went off to go swimming….. and she has yoga tonight!
On Thursday to Sunday this week, we will have all three of our sons and their wives in the house and all three grandchildren! We are really looking forward that gang! At the same time, I’m asking all of our family to be aware that Phyl cannot do everything that she used to do. She will need help cooking, cleaning, etc. Lets allow her to play with Elena, Noah and Colin and we need to step it up when it comes to routine chores while you are here. And if you get her to go for a walk with you, please go very slowly.
Please do ask her about the disease as she needs to talk to normalize it a bit. But at the same time, she wants to talk about her grandchildren, our asshole of a President, and the Red Sox! I found this short article about how to interact with family members with ALS, which might help (below).
Phyl asked me to send you an update on what is happening here. You are welcome to share this with others….
As you know, we got a lousy diagnosis on Monday. Phyl has ALS, Lou Gehrig’s Disease, and it is terminal. It was pretty much expected but it was still a shock. This time, unlike the preliminary diagnosis on March 15 (the Ides of March, no less), we got through the shock more quickly. On Tuesday, we had a short bike ride to town for coffee and I know Phyl was pleased that she could do it! She is planning a longer bike ride with Jen, my sister-in-law, tomorrow. The neurologist at Lahey ALS Clinic says to exercise all she wants but not to over do it. Her legs get tired if she pushes herself and then her walking becomes more labored.
Yesterday she went to acupuncture and then to Mass MOCA (art gallery in the Berkshires) with her girl friends, and an exercise (stretching) class in the evening. This morning she is swimming at the health club and plans on a dog walk with Gabe (Jeremy’s mother-in-law). She has a pretty good attitude much of the time but underneath there is a sense of fear that most likely won’t go away. If we focus on today…. life is good.
We are busy putting in place the medical and emotional support that we will need over the coming years. Here is what we are doing:
We signed up for the Lahey ALS Clinic program of visiting every two months to meet a team of experts; nutritionist, occupational therapy, and physical therapy and a few others I can’t remember. The first visit is May 15. They offered us a session on Monday but neither of us are ready yet. I picked Lahey because they focus on patient care and we liked the neurologist who treated her Aunt Helen. The clinic is in Burlington, MA and it took us 20 minutes to get there from Jake and Shannon’s house in Somerville. As long as she can manage the stairs at Jake’s (it is a bit daunting for healthy folks), this means regular visits with Noah and Colin!
She is getting weekly acupuncture from a local guy who happens to be a friend from her pottery studio (and is giving us a discount)! We were accepted into a Mindfulness Medication research study with Penn State University. This is an online training for both of us. They monitor disease progress and emotional health and compare it with a non-participating population of ALS patients. So we are research guinea pigs! We have our “intake” phone call and instructions next Thursday. Our neurologist at Lahey knew the research scientist at Penn State and thought this was a great idea.
We have our first meeting with a Functional Medicine doc on June 1 to begin a nutritional assessment and toxicological screening (thanks to Belita who works for a Functional Medicine doc in Colorado and guided us on the whole process). I”m learning a lot). There is some data suggesting that nutrition can improve quality of life and slow down the loss of muscles. While it is unproven, it is also harmless and the Lahey nutritionist will be consulting with the Functional Med doc so we will have two opinions on what to eat and what not to eat. Weight loss is the primary concern.
Phyl is in the process of lining up a therapist locally who specializes in chronic illness. Having a place to talk about fears and expectations is important. She is able to talk with me about this but it is not easy, as you can imagine. And we have a Mindfulness Medication training on Tuesday night with a woman who also does chronic illness therapy. We have also been doing a guided 15 minute medication each morning which she seems to like. Everyone in the ALS community seems to agree that mental health and attitude is critical. One of her strengths is her positive attitude.
Finally, I am searching for clinical trials and research that we might explore. We don’t expect a cure for the disease but hopefully a treatment to slow down the muscle loss. The most promising work seems to be on stem cell infusions which have been shown to delay the development of symptoms in limited studies. The drug therapy is interesting too but there is only one FDA approved drug and it has minimum effect. The FDA is expected to decide on another drug that is available in Japan in June, but who knows what they will decide. There are lots of snake oil sales folks around who want to sell a cure to desperate people. While I pray for miracles, we are not planning on one.
I am trying to better understand stem cell therapy. While I am surely more comfortable with plant biology, it is still biology – so I’m reading the research papers. I’m signed up for a webinar with Mass General on May 22 to learn more about stem cell research. There are plenty of commercial labs around who will take your money for unproven treatments (cost is about $20,000 and this just pisses me off). The web pages all sound great but they rarely connect you with the original research which makes me suspicious. I am trying to read the research articles and understand what might be expected. Mass General and the UMass Medical Center are among the leading research hospitals in the world and are about to launch a new stem cell trial with an Israeli firm which seems legit. So… we’ll see!
We are on a “waiting list” for information about clinical trials and access to a form of stem cell infusion that has shown some promise at Mass General and UMass…. along with a lot of other folks I expect! The most interesting stem cell work seems to take a person’s own cells, grow new stem cells and “trick” them into making motor neuron cells with your own personal DNA. The difficulty seems to be in delivery to right location. A spinal infusion is a bit un-targeted but its the best there is at present. It seems to be a ways away from any kind of proven success which would be required for FDA approval.
For me, the most important job is to keep her engaged in life and connected to her family and friends. That is where you come in! Please continue to talk to her on the phone or just send her a text from time to time. She smiles when we get at picture of our grandchildren or a text from Jeremy about the Red Sox or from Jake about the Cubs! Texts are great as she is busy during the day.
She is not responding well to advice from folks about cures. We got a long voice mail yesterday from a well meaning person who wanted her to know about all of the research he had done on her behalf, with lots of suggestions about new experimental drugs and what I see as “witch doctors”. Frankly, this isn’t helpful. She has asked her friends to contact me with suggestions, remedies, doctors, research information etc. I am happy to sort through all of the information and share what I find useful with her in a way that she can hear. Phyl gets upset when people feel compelled to share their suggestions about what she should do. She is telling them it is okay to email me.
So that’s the story. Let me know if you have questions. You have my email and my cell is 413-687-7798 if you want to text or call. We are looking ahead at many years of learning how to live our lives together with this disease. Supporting Phyl is now my primary job in life and I am grateful that we have an opportunity to do this together. Our sons have shown a degree of maturity and care which should not surprise me, given who their mother is…. We are both blessed with a supportive family who love us.