The new normal

[PiratesWife]

I would like to start by thanking you all for being here, I understand your time is precious and I appreciate the support offered here so much. My 47 year old husband was diagnosed on Jan.5/2022 after months of referrals to different specialists , his neurologist finally narrowed it down to ALS and it was confirmed by our local ALS clinic.

We have a baby under one year and a 10 year old, it breaks my heart when I think about what the future will and won’t hold for them. It’s been less than two weeks since D day and my husband still isn’t ready to talk about it in depth and refused to let me attend any appointments with him. I know I need to give him time to grieve this devastating news.

This has been frustrating since I was a disability care aid for over a decade and I know I can be a great CALS with all my experience advocating for my various clients over the years. All I know is what he tells me, apparently he may have a slow progression, currently his voice is slurred but understandable and he has muscle weakness and loss in his right arm and hand which has been happening for over a year now. He gets up and works harder than most every day, eating all the food, and most people wouldn’t know anything is wrong yet.

He doesn’t want to take the prescribed riluzole and he can’t deal with the idea of having a port for the radicava, hoping the oral form of that will be available in time?

He would probably hate that I’m posting here and is understandably feeling very private about his diagnosis, which has been awful for me to lie to all our friends when our life has just been imploded by this news.

Has anyone here been following the research in potential for ketamine as a treatment to slow progress in early stages? Thoughts? I’m thinking at the worst it will help with depression which can’t hurt?

 

[lgelb]

A sad welcome, PW. It sounds like your husband is still processing, and that will take time, though I know it is difficult to see. You have a great background for helping him through this, but he has to come to that point of "letting you in" at his own pace. It may be bit by bit.

It has been less than two weeks, so maybe in a couple more he will be ready to talk about sending this news further afield. I certainly wouldn't feel you have to lie for him. You can always say, "I can't talk about that right now -- we are still gathering information" if you are asked about his status. Of course, the ten-year-old should know first.

The FDA's action deadline (which doesn't have to be final approval) is May 12. Not sure of the Canadian timeline. If he might be interested in a current trial, though there is an equal chance of getting a placebo, here is that study. There are Canadian sites.

Re ketamine, yes, the patent holder has received an IND after receiving an orphan drug designation (note that an ODD means absolutely nothing in terms of safety and efficacy). It's a pretty heavy-duty drug, with effects that can include nausea, vomiting, dizziness, and disassociation, so that would not be my first move. The forthcoming Phase 2 study is a single-center titration study with 36 PALS at the University of Missouri; that suggests a light budget and limited expectations.

Since PharmaTher has opted not to publish the preclinical studies, it is hard to evaluate how on track the theory might be.

Ask us anything, any time.

Best,
Laurie

 

[johnny5]

Oral Radicava has been submitted for approval in the US. Expect to see results in or around May (re: ALS news today).

Riluzole was causing my liver function tests (AST and ALT) to double and triple, they took me off of it. Everyone is different.

Dr. Bedlack does a lot of investigations on alternative treatments at ALSuntangled. You can find a ton of info on what everyone is dealing with that is not generally approved for the treatment of ALS.

I have noted a lot of pALS go through a mourning-type process when they are diagnosed. You may want to speak to him on that topic. ALS generally goes too fast to drag that out. Finding joy and happiness these days is an important factor in staying healthy (as much as possible). Some studies have hinted that depressed pALS progress quicker.

Education is the key and gentle persuasion.

 

[PiratesWife]

Thank you so much for your reply and insights Laurie.

I’m not sure when the right time is to tell our 10 year old, I just want things to be as normal as possible for as long as possible for her… my instinct is to wait until it’s obvious that something is wrong. That being said kids are very smart and I’m sure she knows something is up.

Back in our “party” psychedelic exploration days (long before we had our kids) we recreationally used ketamine and are fairly familiar with it as a substance, my husband has never had any adverse reactions to it. To be clear I am not suggesting or condoning that anyone else use it, as many people definitely do not do well with it.

Other options we (by we I mean I, am grasping at any hope here with hours of mostly pointless internet searches) are exploring are ibogaine, lions mane mushrooms, even ayahuasca. I realize that these are not regulated or proven safe options and again I’m not here to suggest that any one else should try them. It seems like even the approved options are a shot in the dark at best.

 

[lgelb]

It's only natural to look for the overlooked treatment. The problem with nutritional mushrooms and psychedelics is that motor neurons are completely separate from the biochemistry of mood and thought, controlled by the brain.

Also, all rx, supplements, vitamins, and food have to be metabolized by the body, whereas ALS is a disease at least in part of hypermetabolism. These substances also often interact and cancel each other out, wasting the energy of metabolism. And the less energy the body has to support motor function, the faster it can be lost. So it's not true, as some will say, that "anything is worth trying."

Alleviating some of the symptoms of ALS that are mediated by brain function is not the same as slowing progression of the death of motor neurons.

Apart from the approved rx for ALS, that have obviously limited efficacy, here are some of the ways that PALS here have extended/improved their lives.

Active-to-passive range of motion exercises at least daily, depending on the PALS' capabilities
Massage to prevent/reduce edema
Respiratory support (BiPAP) as soon as needed, with adequate secretion removal via self-or mechanically-assisted suction/coughing
Mobility devices to avoid falls, likely culminating in a power wheelchair with elevation, tilt, recline, power foot rests, and a powered patient lift for transfers
A bed that allows for an optimal position to avoid pressure injuries and facilitate lift transfers. A hospital bed should have reverse Trendelenburg capability in addition to independent bed, head, and foot height adjustment.
Use of a Hoyer-style lift as soon as other transfer methods become painful or risky
Adequate postural support in bed, wheelchair, and chairs
Maintaining body weight, through nutrition and hydration, via feeding tube if/when needed, using a non-corn syrup based formula or blending real food. If not using a tube, adjusting diet to weakened musculature as needed, e.g. reducing kernels, skins, leafy foods, and tough meats
Judicious use of supplements (several threads; use the search box top right)
Adequate sleep, social/nature/arts interaction, pursuit of hobbies/interests in whatever possible form(s)

 

[lgelb]

As regards your daughter, many of us have found that since kids do see more than we sometimes think, it's best not to carry on a pretense past their first suspicions. Just as it was hard on you before you knew what was wrong, the same applies to them.

 

[PiratesWife]

Thank you Johnny5 for your response. I hope it’s ok if I ask what you are currently doing for your own treatments? Thank you for the lead to ALS Untangled, it’s a lot of info to wade through. I’m looking into the TUDCA option, we have a zoom meeting this week with his team from the ALS clinic, speech therapist, nutritionist and nurse. I’m trying to get my ducks in a row here with things I can ask them, although I’m not sure really what they can do to help.