[Mike Minardi]

Can’t tell your daily dosage from the message. My wife is taking the combination of sodium phenylbutyrate and tudca as mentioned in another post. Her SP dosage is 6 grams / day (12 500mg capsules which we dissolve in water and give through G-tube) plus 2G of Tudca (4 500 mg capsules per day).

We source the SP capsules from Johnson Compounding Pharmacy in Waltham, MA. They are an outstanding compounding pharmacy and they are licensed in Illinois which is where we live.

1000 500 mg capsules, we currently pay $1450. If you are dosing 6g / day like my wife, then I think you are getting a very good price. If you need more information, please send me a private message.

[Mike Minardi]

My wife has bulbar ALS and was diagnosed in 2018. Her speech and breathing were impacted right away. Fortunately she is still breathing on her own but she cannot lie down so she always sleeps in an inclined position. We believe one of the key factors for maintaining her ability to breath is a therapy she has been receiving since June of 2019 through the Healey Center”s expanded access program at Mass General. If you would like more information about that, please send me a private message.

[Mike Minardi]

You might want to look at a shower / potty chair. We use a shower buddy chair and it also tilts. We have added a Roho commode cushion to prevent pressure sores.

[Mike Minardi]

Here are a list of meds / supplements my wife takes in addition to Riluzole. Her neurologist has prescribed a number of these and she is also fortunate to be part of the MGH / Healey Center expanded access program.

Sodium Phenylbutyrate + Tudca – SP has to be prescribed and is not covered by insurance and Tudca can be purchased on Amazon
Metformin – prescription
Mexilitine – prescription
Neudexta – prescription
Baricitinib (Olumiant) – prescription
Acteyl L Carnitine – OTC – Amazon
Elysium Basis – OTC Supplement
Theracurmin – OTC Supplement
RNS60 from Revalesio – Expanded Access Program

As others have stated, please confer with your neurologist. If you want more information, please send me a note.

[Mike Minardi]

For swelling, you might ask your Dr for a water pill (furosemide). You certainly don’t want to risk DVT (blood clots) due to lack of mobility. You can also buy compression boots on Amazon. They run about 20 minutes and then you remove them. They are much easier to put on than compression socks

[Mike Minardi]

I would recommend getting a Philips Lifeline. It has fall detection and if you fall, a Lifeline operator will come on quickly and ask you if you need help. If you are unable to answer, Lifeline will automatically contact 911 and EMS will come to your home and you can designate friends to get a call at the same time.

The other thing Lifeline offers is a key lockbox which you can keep on your front door so that your friends or EMA can unlock your door.

Alexa / Amazon Echo is a nice back-up, but I would encourage you to look at a Philips Lifeline service. If you were to become unconscious, Alexa is not going to help.

Stay safe

[Mike Minardi]

Hi Steve, just saw your question on dosage. Yes, the dosage was ordered by Dr. Merit Cudkowicz at Mass General. We mix 6 500 mg SP tablets with 2 capsules of 500mg Tudca twice a day, morning and evening. She takes quite a few different scripts (all ordered by Merit Cudkowicz), each one backed up by a research study. All with the hopes of slowing this down. My wife actually has the C9 mutation and we are hoping some of the therapies in the pipeline will arrive sooner rather than later. If you would like to discuss, please feel free to give me a call, I am in Illinois and my mobile is 630.768.8616

[Mike Minardi]

My wife has been taking Sodium Phenylbutyrate and Tudca since January. While Tudca is available on Amazon, you do need a script for Sodium Phenylbutyrate. She currently takes 6g of SP per day (12 500 mg tablets) along with 2G (4 500 mg capsules of Tudca) per day. Her neurologist at Mass General / Healey Center prescribed the SP. Insurance will not cover it and it is expensive, but you can get a discount through GoodRX. you need to go through a specialty pharmacy such as CVS Specialty. We have seen no side effects and while Kathy’s ALS was already far along, she has remained stable. Find a neurologist who will prescribe it. In our case, Kathy is fortunate to have the Director of the Healey Center as her neurologist, Dr. Merit Cudkowicz.

Good luck!

[Mike Minardi]

Before you pick a trial. See if you can talk to Merit Cudkowicz at Mass General. She isn’t just one of the best, she is the best.

[Mike Minardi]

Get a peg sooner rather than later, even if you can still swallow. And as suggested above, after a couple of months get a mic-key. Our recommended supplement is Kate Farms Peptide 1.5. a non-soy-based supplement with excellent vitamins and minerals and each carton is 500 calories. And for hydration, my wife consumes a combination of gatorade and pomegranate juice. And all meds are mixed with water and given through the peg.

 

[Mike Minardi]

My wife started slurring her speech in the spring of 2018 with right hand and arm weakness. She was diagnosed with bulbar ALS at the end of Sept 2018. She can no longer speak or swallow, but she is still breathing on her own and we have gotten great support from the team at Mass General and the Sean M. Healey and AMG Center for ALS in Boston. She has been on an experimental treatment (RNS60) through the Healey Center expanded access program since June 2019. We know her condition is caused by a genetic mutation (c9) and we remain hopeful that one of the genetic therapies in the pipeline will become available and effective sooner rather than later. Wishing you all the best.