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/about/profile/mark-hyman/
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 when we went in for our first trach change. It’s recommended you change your trach every month, but many insurance plans pay for only one trach every 3 months. So you have to figure out where you can purchase trachs. We use Health Products For You quite a bit: https://www.healthproductsforyou.com/
We’ve heard they’ve stopped making the comfortable trach (flexible, and low profile).
Things that are terribly broken in ALS land
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 (very 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.
- Hospital staff is overworked and cannot remember anything that is not standard protocol. Standard protocol can 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. A trained caregiver can get the patient’s airway cleared under 20 seconds, including face muscle 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.
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 is poised to break this pattern–NurOwn. The NurOwn 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 is 100% proof it works. Yet CBER/ the FDA drags and drags its feet and ALSA National (the only large ALS non-profit not to support NurOwn approval before the EAP results) 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 drug 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.
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 sign over all your belongings to medicaid.
This sad state of affairs results from the care being intense and requiring intense training. Care companies like Visiting Angels don’t allow their caregivers to do trach suctions and such, 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 extensive experience with that site). And for the privilege of training and managing them yourself you also get to pay them nearly double the going rate for caregivers.
VOCSN – all ALS patients should qualify
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 a long time AND that you can pop out and replace with fully charged batteries. They are much more comfortable for patients. They 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 you can simply take my word for it–it is impossible.)
Getting to the root cause
Even though there are 57 documented reversals, nearly all of which used the same basic protocol/practice to reverse as 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. 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, because at the same time you are researching ALS you are also 1) getting 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. 2) trying to tell your loved ones you’re dying while trying to make fond memories with them/bucket list 3) researching how to deal with/equip needed for each month’s new massive function loss (and learning that equipment–also, you are largely on your own trying to get and learn this equipment) 4) processing the loss of each function/your life 5) managing your new business that makes no money – interviewing caregivers, scheduling them, doing payroll, fundraising for your care, 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 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, the most comfortable trach (low-profile cuff and flexible) has just had production stopped on it. No other company is picking up the ball to make these trachs. I don’t believe any org is trying to fix this. My guess is because there aren’t many diseases that require long-term use of trach tubes or insurance won’t cover the comfortable ones. At any rate — BROKEN!
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.)
Articles or reports of treatments having “potential” or “promise”
This is gripe is not one of the “terribly broken” things, but articles geared toward ALS patients that discuss trial results need to 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 3) 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)