The Ice Bucket Challenge, sometimes called the ALS Ice Bucket Challenge, was an activity involving the pouring of a bucket of ice water over a person's head, either by another person or self-administered, to promote awareness of the disease amyotrophic lateral sclerosis and encourage donations to research. After a while, the true reason behind the challenge was forgotten. Many have no idea as to why they are doing it, and believe it to be a random trend.
The challenge went viral on social media in 2014 when ALS research became the main beneficiary. A response in South Africa led to contributions to the Motor Neurone Association of South Africa. Since then, it has not had much impact and nothing materialised locally.
Amyotrophic lateral sclerosis (ALS) is a nervous system disease that weakens muscles and impacts physical function. In this disease, nerve cells break down, which reduces functionality in the muscles that they supply. The main symptom is muscle weakness. Over time, patients will lose small motor functions, gross motor functions, lose their ability to eat or move without help, and to breathe. Unfortunately, the disease is terminal, and has no known cure.
One of the most prominent figures of our time to suffer from ALS is theoretical physicist, cosmologist, and author, Stephen Hawking. Joost van der Westhuizen, former Springbok Rugby player, succumbed to the disease as well.
Dr Franclo Henning, neurologist at Tygerberg Hospital/Stellenbosch University, and national chairperson of the MND/ALS Association (MNDA), and executive vice-chair Sheila Slabber shared their expertise on ALS with Mopani Pharmacy.
What is the difference between ALS and MND (motor neuron disease)?
“MND is a broader term, while ALS refers to the most common form of MND (about 90% of cases). Most people use the 2 terms interchangeably, depending on where in the world you are. In the USA, it’s also referred to as Lou Gehrig’s disease”, explained Dr Henning.
How common is ALS?
“Globally, the incidence is about 1.5 to 2 new cases per 100 000 of the population, per year. It appears to be lower in South Africa, we have roughly 1 new case per 100 000, per year.”
Are there risk factors that will cause the onset?
“Smoking appears to be a risk factor, but has not been shown to cause MND. There are no other proven lifestyle or environmental risk factors.”
Read more: Trying to quit smoking?
“Genetic factors likely play a very important role, probably the most important. However, this is very complicated. About 10% of cases have a genetic mutation that is known to cause MND. The cause in the other 90% is still uncertain, but is likely either related to:
- a combination of genetic and some as yet unknown environmental risk factors
- a number of genetic risk variants. The more of the genetic risk variants you have, the higher the chance that you would develop MND.
However, this is all still very unclear, and a hot topic in research.”
Are there risk factors that will worsen the symptoms / progression?
“Weight loss is associated with faster progression. it seems one can slow the disease down to some extent by maintaining weight as much as possible.”
Have there been made any advances in the treatment of ALS?
“There are two drugs that are approved by the FDA: Riluzole and Edaravone. Both only slow down the progression to some extent, and do not stop the disease altogether. There have been some recent exciting findings from Scotland. These are still only laboratory-based research, and still have to be tested in clinical trials.”
We are however, seeing more devices and leaps in technology and treatment that assist patients in their daily lives.
A recent newsletter from the MNDA explained:
“Patients with progressed ALS often struggle with eating, as they have problems with swallowing. Once it becomes serious, patients may opt for a percutaneous endoscopic gastronomy (PEG).
It involves a thin, flexible tube being inserted directly through the wall of the abdomen into the stomach through which soft, liquid foods, fluids and/or medications can be administered to those who are struggling to swallow.
It is a simple, effective procedure that helps remove the anxiety caused by trying to eat with the constant fear of choking. It is also invaluable in ensuring that you keep otherwise healthy and well-nourished in order to cope with the many other challenges of living with MND.
Even after opting for PEG, some people still manage to eat and drink a little by mouth afterwards. Medications that are not available in liquid form can also be given through the PEG tube after being crushed and administered in water.
A new smartphone app called Look to Speak has been launched by Google, promising to help those with speech and motor impairments communicate with eye movements.”
With this app, you simply have to look to the left, right or up, to quickly select what you want to say from a list of phrases. The phrase is then spoken aloud by an automated voice.”
What is the typical prognosis?
“On average, survival is 3-5 years from symptom onset. But this is variable. About 10% of people will live for longer than 10 years from onset,” said Dr Henning.
Stephen Hawking outlived his prognosis, he was given only two years to live. He was diagnosed at the tender age of 21, and lived another 55 years before passing away at age 76. It remains a medical mystery to this day. Joost van der Westhuizen was not as lucky, he sadly passed in 2017, six years after his diagnosis.
How does the MNDA support patients and their families?
Executive vice-chair Sheila Slabber explained, “The MNDA is an NPO, based in an office in Pinelands, Cape Town. The organisation employs part-time, home-based advisors across the country to provide counselling (through home visits or telephonically); to advise on care; and to organise the loan, purchase and installation of aids and equipment to ease the lives of patients and their families and caregivers living with this degenerative, incurable neuromuscular disease.”
“We currently have patient advisors that cover different areas in the country.”
Currently the MNDA has:
- Three in Gauteng (based in Pretoria, Johannesburg and East-Rand respectively)
- One in KwaZulu-Natal (Durban-based)
- Three covering the Cape Peninsula
- One covering the southern parts of the Cape region
“I look after the southern regions of the Cape and oversees all the patient advisors nationally. I also provide support to patients countrywide in outlying areas, telephonically.”
“As part of its mission to inform and educate the public about MND / ALS, the MNDA supplies new patients and doctors with reading material on the disease, its effects and solutions to its every-day challenges.”
“The MNDA of SA is able to disseminate the latest information on international (as well as local) research, raise public awareness of MND and encourage fundraising activities. Our detailed recordkeeping of patients and their progress is a valued source of information to medical researchers.”
Has the pandemic affected you?
“We were not able to have any patient support group meetings or patient home visits by our advisors during the various lockdown period and to date. MND patients are in the high-risk bracket, and we also needed to keep our patient advisors safe. However, our advisors continued to support and advise patients by email, telephonically and via WhatsApp”, said Slabber.
“Home visits, following strict sanitation and mask-wearing protocols, were gradually resumed towards the end of last year. Changing lockdown regulations allowed this, and state hospital MND clinics attended by our patients, were resumed. We hope to re-introduce support group meetings once we feel it is safe for our patients to attend. Provision of equipment on loan to ease the lives of those living has also been resumed.”
For more info on how you can get involved or get support, contact MNDA on mndaofsa@global.co.za or 0215316130.
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