An ALS diagnosis in the family is a life altering event not just for the patient, but for everyone living at home, and involved in the care. Nothing in life prepares you for what is to come with an ALS diagnosis. Unsurprisingly many people living with ALS (PLWALS), caregivers/families find themselves struggling to deal with the situation, and end up helpless and demotivated. A strong, proactive, and involved caregiver, and careful coordination with PLWALS is very crucial to prolong the length of survival, and for making life livable for PLWALS. For all the things that are out of control for PLWALS and caregivers, there are still quite a few things that are well within their control.
Below is a list of things within their control, that can help PLWALS and caregivers develop an approach to dealing with the unpredictable situations that will arise over the course of the disease. None of this is new information, and many of you might already be practicing this, but it is well worth reiterating.
1. Understand that ALS progression is non-linear and unique to each patient
What that means is that the rate of progression is not constant throughout the disease - the disease might be progressing rapidly at one stage but gets slower at another stage, and vice versa. Also each patient's journey is unique to them. So even if you've been told by the doctors, or read on websites about the typical stages of ALS (Early, Middle, Late and End), each patient will experience them differently. Thus it's important to provide the best care throughout.
2. Know that plateaus are real
Due to the non-linear nature of the progression, some patients do experience temporary slowing down or plateaus in their disease progression. These plateaus have been observed to range from 6 months to 18 months.¹ It is unclear what causes these plateaus, but there are real.
3. Do your own research
Due to ALS being such a complex disease, it is not well understood by a lot of medical professionals, including many neurologists who are not neuromuscular/ALS specialists. Also, unless you are consulting a multidisciplinary specialty ALS clinic, many a times even specialists like Pulmonologists, Physical Therapists etc. are not aware of the right treatment protocol for ALS patients.
Read research/review articles, get to know about treatment protocols, learn the operation and settings for medical equipment, and daily procedures that will be required to be done. Do not completely rely on the homecare staff.
4. Turn to Patient/Caregiver forums
Patient forums contain a wealth of practical information, some of which you won't find anywhere else. Refer to places like national/domestic ALS groups, ALS Support Group on Facebook, r/ALS on reddit, alsforums.com etc.
5. Act as a Project Manager
The caregiver in coordination with PLWALS needs to take a role similar to that of a project manager
- Take ownership of the situation and take proactive decisions as per the wishes of the PLWALS
- Make contingency plans for various possible scenarios
- Know the inside out of the treatment protocols and operation of various equipment/home procedure
- Be a keen observant about changes in disease progression
- Carefully listen to what PLWALS has to say, and communicate at every step
- Document everything
6. Assemble a team
Talk to your friends, and family and try to have people ready in case of emergencies like hospital visits, homecare staff on leave, need help procuring some emergency medical equipment/medicines etc.
7. Recognize urgency, avoid complacency
Certain medical situations will require urgent attention, and immediate action. Some of these include:
- Difficulty breathing and drop in SpO2 levels below 90
- Significant decrease in food intake due to difficulty eating (chewing/swallowing)
8. Be prepared to challenge healthcare authorities
This mostly applies to patients from developing nations where the awareness about ALS is severely lacking, with no access to multidisciplinary specialty care hospitals.
- Keep your own non-invasive ventilation (NIV) equipment handy
- Ensure the treatment protocols used at the hospital are in-line with your research and advise from patient groups
- Get to know the treatment details and clarify any queries with the doctors/medical staff
9. Do high impact things (80/20 principle)
Few things (~20%) have a disproportionately high impact (~80%) on the rate of progression. They are:
- prevent weight loss²: get a feeding tube at the earliest signs³. Eat calorie surplus, high fat, high protein diets. Of course there will be loss of muscle, but that needs to be made up with gain in fat to avoid weight loss
- prevent falls: get a wheelchair, support sooner rather than later
- timely respiratory support: Initiate NIV at the earliest signs⁴, followed by invasive ventilation (if chosen)
10. Get all necessary medical equipment before trying alternative therapies
Before trying out alternative therapies, and other treatments with mere anecdotal evidence, ensure that you first buy/rent all the necessary medical equipment, and have sufficient funds saved for home care.
11. Seek professional help from a psychologist/therapist
Get rid of reservations or stigma (if any) that you have in mind. ALS is a lot to deal with. Go see a therapist. It may genuinely helps.
12. Anything that helps the mental and psychological wellbeing of the patient and caregivers
Develop healthy, non-destructive coping mechanisms. Praying helps? Do it. Enjoy a glass of whiskey? Pour yourself a double. Anything that keeps your spirits up.
13. Take care of yourself
Take breaks between caregiving, and look after your own health as well. Caregiving for a PLWALS is not a one man's jobs - ask for help whenever you feel overwhelmed.
- Vasta, R., D'Ovidio, F., Canosa, A., Manera, U., Torrieri, M. C., Grassano, M., De Marchi, F., Mazzini, L., Moglia, C., Calvo, A., & Chiò, A. (2020). Plateaus in amyotrophic lateral sclerosis progression: results from a population-based cohort. European journal of neurology, 27(8), 1397–1404. https://doi.org/10.1111/ene.14287
read now↗ - van Mantgem, M. R. J., van Eijk, R. P., van der Burgh, H. K., Tan, H. H., Westeneng, H. J., van Es, M. A., ... & van den Berg, L. H. (2020). Prognostic value of weight loss in patients with amyotrophic lateral sclerosis: a population-based study. Journal of Neurology, Neurosurgery & Psychiatry, 91(8), 867-875.
read now↗ - Fasano, A., Fini, N., Ferraro, D., Ferri, L., Vinceti, M., Errals, & Mandrioli, J. (2017). Percutaneous endoscopic gastrostomy, body weight loss and survival in amyotrophic lateral sclerosis: a population-based registry study. Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration, 18(3-4), 233-242.
read now↗ - Dorst, J., & Ludolph, A. C. (2019). Non-invasive ventilation in amyotrophic lateral sclerosis. Therapeutic advances in neurological disorders, 12, 1756286419857040.
read now↗