I’m capturing stuff here that I don’t want to lose track of. Most of these items I’d like to turn into blogs some day for the newly diagnosed with ALS or newly trached ALS patients.
For now, this random melee is what I have time for.
Link to functional medicine doctors: https://www.ifm.org/find-a-practitioner/
Newly trached or g-tubed? Be sure to hold on to your prior trach or g-tube for training caregivers. Also, as soon as you can, start working on acquiring your next trach and g-tube (or having a backup trach and g-tube). We were surprised to find out that we were expected to have our own trach tube and trach tube change kit when we went in for our first trach change. It’s recommended you change your trach every month, but some insurance plans pay for only one trach every 3 months. So you may have to figure out where you can purchase trachs. We use Health Products For You quite a bit: https://www.healthproductsforyou.com/. However, our trach (the only sensibly designed trach in our view) has been discontinued–details below.
Things that are terribly broken in ALS land
Please note that these things were all true pre covd and post covid. Covid did make the staffing and supply issues worse, but these problems were severe before and after covid.
Hospital dangers
Many (all?) hospitals know nothing about how to keep an ALS patient alive. We have heard our same complaints from ALS patients across America, so this is not from only our experiences. The below combination is deadly:
- Hospital staff doesn’t understand that when a patient has absolutely no way to signal AND aspirates 5 to 12 times per hour (common in ALS!!!) it means that that patient must be monitored 24/7. No alarms go off when an ALS patient chokes/suffocates because they cannot cough strongly enough.
- Hospital staff doesn’t understand that by “monitor” we mean looking at the patient’s face every 30 – 60 seconds AND interpreting the patient’s subtle facial muscle movements. A patient with only subtle facial movements as communication can say 50 or more things in his/her “facial language” to someone who has been trained to speak that language. Most importantly, the patient can tell you what is needed to clear their airway (rainout removal, trach suction, cough assist, neb, etc.) which saves a minimum of 10 minutes while choking/suffering.
- Hospital staff is overworked and cannot remember anything that is not standard protocol. Standard protocol can injure or kill most advanced stage ALS patients.
- Hospital staff believes that ALS patients don’t have any feeling in their body. (!!!) Even after you repeatedly tell them, they forget.
- If a trained caregiver/interpreter isn’t bedside at all times (including for tests like MRIs, getting positioned for surgery, getting wheeled to recovery room, and being in recovery room), it can be deadly for the patient and at the very least injurious, torturous, and terrifying.
- Hospital RTs (at least in America) for the most part don’t seem to be trained on cross-contamination or efficient and safe trach suction techniques for long-term trached patients. A trained caregiver can get the patient’s airway cleared in under 20 seconds, including subtle face-movement language interpretation time.
- Hospital staff is not trained on the importance of a patient’s yes and no signal nor trained on how to use a letterboard. Nor how to ask yes or no questions. Even if you train them, they forget, time after time.
- Hospital rolls are very dangerous for ALS patients. We hear this over and over and Pat actually got a slipped disc in his neck from his first hospital roll.
- We have heard this from several RTs who are very knowledgeable about ALS and who have worked in different hospitals. It is maddening to them (and us) that RT staff in hospitals don’t understand that the rules on trach suction frequency change for people who cannot swallow saliva (pALS) and who can feel pain and suffer. pALS can aspirate up to 12+ times per hour and cannot be told “oh you just did a trach suction an hour ago so we need to wait another hour” while the pALS is choking. RTs will sit and watch a patient choke and suffocate thinking the patient will “re-stabilize” magically somehow. They don’t understand that at home, when the patient is having a “juicy” couple of hours, we are suctioning every 5-20 minutes. (Note that hospital RTs are trained to hit the carina during trach suction–completely unnecessary and could be deadly for ALS patients due to the frequency of trach suctions and being trached long term.) We have to have this discussion with every new RT and it simply doesn’t stick bc it’s not in their training.
Access to treatments that work
ALS patients cannot get access to treatments that have proven to slow and/or stop ALS, such as NurOwn, even when that treatment has been proven to work on particular patients in its trial or EAP. Young adults in the NurOwn EAP are being denied a drug that the evidence says works on THEM SPECIFICALLY. Not only that, the FDA won’t even agree to listen to the ALS experts and trial primary investigators on the matter. This 30+ year pattern of failure of the FDA not approving drugs that work on ALS patients must stop. A perfect drug was poised to break this pattern–NurOwn. The NurOwn data (minus primary endpoint), trial PIs, the participants, the EAP, and the trial’s peer review, all say the same thing–it works! Absolutely no one refutes that the EAP data is 100% proof it works. Yet CBER/ the FDA drags and drags its feet and ALSA National sits idly by to this day.
Once a drug/biolgic from small pharma gets approved by the FDA, it opens the floodgates for the hundreds of other promising treatments you’ve read about over the years from small pharma. If you wonder why for 30+ years these haven’t seen the light of day, it’s because small pharma doesn’t have the money to take the drug to the next phase, especially given the likelihood that it will get rejected by the FDA for no good reason. We just need one small pharma drug–maybe NurOwn– to show that the FDA will (FINALLY!!!) follow its own guidance document where it promises to give regulatory flexibility to deadly diseases with unmet needs.
Clinical trials
We have heard this a lot with the ALS patients we work with–clinics do not have a way to help you navigage clinical trials. YOYO (you are on your own).
Caregivers
There is absolutely no way to pay for 24/7 care needed once trached except to burn through your life savings and ask friends and family for help (or if you bring in less than $2k per month, you have the privilege of being able to sign over all your belongings to medicaid for caregivers…but even then, you still have to train them, find them, etc.). Most people we talk to don’t understand that care duty is an intense job, even when the ALS patient is “sleeping.” If somehow as the spouse you can magically keep your “real” job to keep benefits and be care duty for 8 hours per day (ie do 16 hour days min), then you STILL need to cover 16 hours of caregiving EVERY DAY unless you don’t plan to sleep, eat, or do your own personal hygiene in the remaining 8 hours per day.
This sad state of affairs is intensified from the care needs requiring rigorous training. Care companies like Visiting Angels don’t allow their caregivers to do trach suctions, g-tube feedings, etc., which means they are of little use as a caregiver. And private duty nursing companies are unable to find staff willing to work with ALS patients (and let’s face it–they have trouble finding staff to just do the easy jobs!)
All this adds up to requiring you to find and train from scratch your own caregivers on sites like indeed.com (we recommend avoiding care.com based on our experience). If you go through a care service, you get the privilege of training and managing them yourself WHILE paying the third-party fee. You pay more for essentially the same thing and your caregivers make less money than if you would find them yourself.
One option I want to look into more for early-stage ALS patients or patients with simply no one to serve as their care coordinator is the convalescent home. I have heard these provide more care than some SNFs.
VOCSN – all ALS patients should qualify and they should keep making them
ALS patients should all qualify for VOCSN ventilators, and there should be an effort underway to help create more of these ventilators and supplies for them. Reasons include: they ingeniously are small yet have 5 machines in one. They have batteries that last much longer than the other available vents AND the batteries pop out so you can easily swap them in instead of having to swap in a backup vent. VOCSNs are much more comfortable for patients–more settings. They display the leak value. They also have a quiet (and more powerful than any standing machine) suction machine as one of the 5 machines–critical when you aspirate every 5 – 10 minutes. Standing suction machines sound like jackhammers and they are right beside the patient and caregiver’s ear. The VOCSN suction is a whisper. (If you’ve never slept with a jackhammer going off every 5-10 minutes for years, you haven’t lived!)
We heard from a DME that Ventec, the makers of the VOCSN, recently sold to another company that doesn’t plan to focus on the 5-in-1. If the 5-in-1 fades out, so do the chances of ALS patients ever leaving their house. You cannot pack 5 machines in your van and 5 backups (you need a backup of each machine if you want to survive your trip). It is not practical to pack 10 machines and 10 sets of tubing but it is practical to take a VOCSN (5 machines) and a backup VOCSN (5 machines).
Getting to the root cause
Even though there are 57 documented reversals, many of which used a similar basic protocol/practice to reverse as basically documented by healingals.org, very few if any clinicians investigate these or try to get to the bottom of each patient’s ALS, which is the first step of each of the reversals. Body Science is now taking this approach too and having some success. Otherwise, the ALS patient is on their own to do all this research. ALS is YOYO as Sandy Morris put it (You are On Your Own).
While many health issues and disease states make you do your own research, it typically is not AS burdensome as it is for ALS. With ALS, at the same time you are researching you are also having to quickly 1) losing function, day to day 2) get your affairs in order bc you don’t expect to live past a year or so – and good luck wading through SSDI, insurance, retirement, etc. 3) try to tell your loved ones you’re dying while trying to make fond memories with them/bucket list 4) research how to deal with/equip needed for each function loss (and learning that equipment–also, you are largely on your own trying to acquire and learn this equipment) 5) processing the loss of each function/your life 6) managing your new business that makes no money – recruiting/interviewing caregivers, scheduling them, figuring out how to pay them, figuring out employment laws, etc.
No resources to give your boss
As a patient or caregiver, there is absolutely no resource, like a video or document, that you can send your boss when diagnosed that explains what you are up against. They simply have to take your word for it, even though you are just starting to understand this new nightmare yourself.
Home health needs serious help
It’s tough to get nurse visits, PT visits, and supplies through home health agencies and DME providers. We were lucky to find 2 rockstar DMEs recently, but we struggled for years before that. Even with our amazing DME providers today, we cannot get the most basic and critical-to-life supplies we need. For example, when the most comfortable trach (low-profile cuff and flexible) started becoming scarce, DMEs couldn’t go looking for them from other suppliers bc they are under contract. There’s no place for DMEs or home health agencies to go to report that a critical piece of equipment disappeared. It doesn’t help that not many diseases exist that require long-term use of trach tubes. Another problem may be that is that insurance won’t cover the comfortable ones which are only a few dollars more per year. At any rate — BROKEN!
If you get a bed wound and need wound care supplies, the only way to get these covered is through a home health nurse service. There are other essential things ALS patients need from a nursing service, such as access to a nurse 24/7 for questions. We’re told that it’s illegal for a nursing service to drop you, but it has happened to us several times. The only time we can get a nursing service to contract with us is if my husband has had a hospital stay and the hospital sets it up. (Be sure to ask for a home health nursing service when the hospital asks, “Is there anything else you need before we discharge you?”)
There is a connector called “flex trach adapter” that makes it much easier to put the vent on and off a patient — plus it is more comfortable than any connector because it is lightweight where it connects to the trach–doesn’t pull the trach. Just recently these have become–can you guess–discontinued.
The list goes on and on (humidifiers that heat the tubing AND let you control the water temp have been discontinued, red caps to keep tubing clean while not in use–discontinued, heated wire tubing itself is scarce, etc. etc.) Risk of discontinued VOCSN as explained above… etc.
They stopped making the only sensible trach!!!!!!!!
There is only one trach type that makes sense for long-term trach tube use–the flexible, low-profile Portex Bivona. This is because it is soft, plus gets even softer once inside your body. Imagine the difference between PVC pipe consistency and baby-bottle-tip consistency sitting beside your gag-reflex and pushing on your trachea walls. Which would you prefer?
Low profile means that when the trach tube is taken in and out of the trach, there is no big lip that is painful when it pops in or out of your trach. The Portex Bivona has absolutely no lip–it is completely smooth–so the monthly trach change doesn’t set you back a week. DISCONTINUED!
Update: I called the company in Oct. 2023 (Smith’s) and they said “the discontinuation must only be temporary” because she had not heard about a discontinuation. But then she said they are just getting reorganized after a merger so maybe she is not fully in the know. Our DME said they heard it was permanent and that’s why they cannot order it through insurance for us (and our respiratory DME is the best there is).
Equipment–communication devices, hoyers, hospital beds, wheelchairs, shower chairs, and more
We have had ALS therapists (PTs, speech therapists, RTs) knowledgeable in ALS equipment and who proactively work to get equipment ALS patients need and we have had and therapists not knowledgeable in equipment who take the easiest possible path. The knowledgeable, proactive therapists create a good quality of life for an ALS patient, helping them get the best equipment possible, while the unknowledgeable, easiest-path ones are close to worthless to an ALS patient.
We would like to see a world where each patient does not have to research each piece of equipment on their own and try to figure out how to acquire it. We’d also like to see a world where insurance is not allowed to stall until the patient no longer needs the equipment.
Two quick stories of our dozens of equipment battles:
Shower chair — Most insurance does not cover a shower chair, even though a tilt, gel-seat, head-support shower chair is critical for ALS patients to go to the bathroom and get a shower before they are completely bed-bound (before they enter “the wet noodle” phase of ALS where getting in a shower chair becomes much more difficult). However, back when we first needed a shower chair (and this is still true for hospital beds and other equipment today), even if you could afford to buy it yourself, no supplier would sell it to you unless your insurance approved it. Talk about a vicious (and absurd) circle! This needs to change. Because of this absurdity, my husband lost 9 months of having a daily shower and being able to go to the bathroom in a normal way. He loved getting showers and going into the bathroom to get his morning care done (vs stay in bed). At the time we could afford to buy the shower chair, but no one would sell it to us unless we went through a DME but no DME would purchase it for us unless insurance would cover it, but our insurance didn’t cover it. We finally found a DME who would purchase it for us and let us pay for it. If I had found this DME in the beginning, Pat wouldn’t have lost 9 months of much higher quality of life. Why does each patient have to figure this stuff out for themselves?
ALSO, OF COURSE there is no reason loaner closets, DMEs, and clinics can’t work together to get every ALS patient the equipment they need WHEN they need it! This also needs to change.
Another issue for us was getting a med surg bed. Once bed bound, a hospital-grade low-loss air mattress is critical to keep severe bed wounds at bay. It is also critical for rolling the patient (fast max-inflate is required, lowering bed is required, if the primary caregiver is elderly, turn-assist is required, etc.) We were so desperate for this type of bed, we were willing to raise the funds to purchase it used at $60k. But again, absolutely no one (Hill-rom at the time nor Stryker nor any 2nd-hand bed shop) would sell it to us if insurance didn’t cover it. I’m not making this up! Of course our insurance did not cover it so I spent 6 months of near 8-hour days trying to get Pat a bed. Why is this research foisted on the caregiver who is already so snowed under with care tasks. insurance paperwork, trying to keep their job, etc. Incidentally, Hill-rom was the only company in 2021 who would even provide a med surg bed to a home and we were told we were the very last home customer they would provide a bed to.
Articles or reports of treatments having “potential” or “promise”
This is gripe is not one of the “terribly broken” things, but is pointing out that articles geared toward ALS patients that discuss trial results should cut to the chase. Patients don’t need to read through paragraphs on how the trial is conducted, how many are in the trial, bla bla bla. The lead needs to be 1) Drug XYZ showed ABC possible benefit and 2) How can I get this drug while the long absurd and dead end process of getting it approved takes place (off label, mix supplements myself, etc.) 3) Drug XYZ is or is not damaging to the liver, kidneys, etc. (ie what are the drawbacks of taking the drug)
Mouthguards
I’ve seen in forums several patients who needed a way to avoid clamping down on their tongues or grinding their teeth, which makes sense if you know how ALS works.
It seems absurd to me that a mouthpiece for this has not been invented. When I was a kid, after my braces came off I was given a very hard mouthguard-like thing made of hard plastic that I was to “bite on at every opportunity.” It would be the perfect buffer for my husband when he uncontrollably bites down or grinds his teeth because there is no way the thing would break. I wore it much later in life because it also protected my tongue from sharp spots on my teeth when I would have night teeth grinding phases.
Farmstrong 