Turning A Bedroom Into The Safest Home ICU

How two sisters mastered the technology of critical care to help their mother live with ALS.

Darji was a profoundly devout man. His piety was not limited to his daily visit to the Gurdwara; rather, it was a permanent resident in his heart. His eyes saw only the good in his fellow human beings.

Darji worked for the government and lived with his family in a two-bedroom flat. Like the gurdwara, his abode was an open house. The small flat magically transformed into a mansion to accommodate any number of guests. In such a blessed home was born Darji’s oldest daughter, Swaranjit Kaur.

During his one-hour lunch break at work, Darji darted off to the Bangla Sahib Gurudwara in Delhi. There he sang kirtan in the precious thirty-minute slot allotted to him. He met many regulars, one of whom was a young man. One day as they were exchanging pleasantries the young man asked Darji to help him find a mate. Darji, always the benevolent soul, offered him the hand of his own eldest daughter! And that was how Mumma and Papa got married in 1972 without ever knowing what the other was like.

Mumma had inherited Darji’s temperament and she too saw no wrong in anyone. Hers had been a middle-class upbringing, and she had just cleared Bacherlors in Home Science when she was married off. She had no inkling about the hardships that awaited her in her husband’s home. Papa’s family had migrated in 1947 from Sargodha in Pakistan and were in severe financial difficulties. This was further compounded by the untimely death of Papa’s father, leaving him to take care of his five younger siblings. Papa took odd jobs to make ends meet, but also continued his studies to obtain his double M.A. degree. When he got married, all he could offer his beautiful bride was a one-room house in Kalkaji with no electricity or running water. Mumma’s sunny temperament helped her weather those initial years of extreme struggle when she had to make do with a lantern at night, and with soaked sheets hung across the window to get some cool breeze. Every morning, she had to wait in a long queue to fill water for the family from a single community tap. This was where Satvinder was born three years after her older brother Mandeep.

Darji, Biji, Satv and Mumma

Mumma and Papa decided to improve their lot by preparing for UPSC clerical grade exams to help them secure government jobs. Both had their day jobs, and two little children to bring up; yet they found the time and energy to clear the UPSC exams. Papa got posted to the ministry of Post & Telegraph and Mumma got a job in a library which was part of the Ministry of Culture. Nine years into their marriage, through their sheer grit and determination, they not only had secure government jobs, they started constructing their new house in Paschim Vihar DDA colony. This coincided with the birth of their second daughter who brought them the ‘sukh’ of their own house, and they named her Sukhvinder.

Mandeep and Satvinder (Satv) were acutely aware of the hardships their parents were going through. That was not to say that there was any dearth of fun. Every Sunday Papa would haul Mandeep and Satv on each shoulder, with Mumma in tow, and they would trot off to the nearest cinema hall to watch a movie. Mumma possessed Darji’s natural gift of a melodious voice and kept singing Gurbani, religious shabads or old film songs while working in the kitchen. As Sukh grew up, the five of them formed a beautiful family unit. They did everything together, be it household chores, weekend shopping trips or entertainment. The parents never imposed a hierarchical order, and the children were equal participants. They became independent from a young age. By the time parents returned from office in the evening, the kids would have finished their school homework and were ready to spend time with parents, do Rehraas Sahib paath together, play ‘antakshri’ with Mumma in the kitchen, and be her helpers as she cooked dinner.

Mumma (bottom left), in school.

In 1995, when Satv was in her teens, Papa developed serious mental health issues due to office-related stress, and had to be hospitalized to undergo psychiatric treatment for bipolar disorder. With help from her children Mumma took over Papa’s share of domestic duties, and the family emerged out of this setback. Papa could continue with his job, although there were regular episodes of mania and depression. On such occasions Satv would spend hours counselling Papa and convincing him to come out of depression, take his meals etc.

Satv passed her graduation with medical stream (B.Sc Gen), followed by Master’s in computer management. The latter was a very practical decision as she quickly secured a contract job with CMC. She was with the organization for four years and got promoted, after which her project required that she move to the US. While many would have celebrated such an opportunity, Satv couldn’t bear the thought of moving away from her dear parents, as her family was her entire world. In fact, even within India she wanted to be only in Delhi where she grew up. This strong filial sentiment perhaps arose from a subconscious sense of foreboding.

Satv decided to leave her job at CMC, and after a short stint in another firm, she finally landed a job in IBM, in the year 2005. The job came with a good pay package, and even more importantly, very progressive, pro-women policies which would be Satv’s lifeline just a few years later. (Satv believes that God was paving the path for a rocky road ahead). This was also the time when marriage proposals were coming thick and fast as Satv had all the virtues that a man would seek in a bride. But Satv was in no hurry to accept. For her the Mr. Right Guy, in addition to all other virtues, should preferably be residing in Delhi.

The year 2005 turned out to be a watershed year for the family. In February, Satv landed the IBM job with a decent pay package in comparison to what the family had been earning so far. The same month Sukh also joined her first corporate job as HR executive. They now persuaded Mumma to take premature retirement and ‘enjoy life’. They reasoned that with no financial compulsions, Mumma did not need to continue with the daily grind of a 9-5.30 job. She could relax and rest, as she had lately been complaining of knee pain as well. Mumma strongly disapproved of this proposal. She did not consider her job as a daily grind; rather she enjoyed being productively engaged in the library, where her colleagues were like a second family. In the end, however, the daughters prevailed and Mumma formally retired from work. Meanwhile, the parents received a proposal for their son, Mandeep. Soon he was married off, with all the pomp and excitement deserving of the first wedding in the family. Mumma exerted herself the most, planning and executing all tasks for the marriage.

Mumma & Papa on Mandeep's wedding.

Early symptoms of a deadly disease

Mumma had never been a home body, and even after retirement she found ways of slipping out of home every day to run errands with Papa as company. Yet the retirement wasn’t turning out to be as restful or interesting as the daughters’ love-soaked heart for their mom had envisioned. Rather, Satv could sense a certain listlessness and anxiety in Mumma behind her cheerful façade. Two years rolled by and Mumma’s life adopted a new normal with her homely daughter-in-law, and the wonderful company of her granddaughter.

January 2008

Mumma had been singing kirtan frequently in the Gurdwara and loved it immensely all her life. For some time now, she had been noticing that her tongue would become stiff and would sometimes stall while singing. At first no one took much note of it, hoping that the issue would resolve on its own.  When that did not happen for some time, Satv started getting concerned and made a mental note of consulting a doctor.

October 2008

From the time he had first developed mental health issues in 1995, Papa had been undergoing regular follow-up treatment at the Department of Neurology, AIIMS, New Delhi. Satv often accompanied her parents on these trips to the over-crowded neurology OPD for Papa’s checkup. It used to be teeming with patients, and the junior doctors had an uphill task to maintain silence so that the senior doctor could examine patients in relative calm.

Obviously, the examination of each patient was a crisp affair, done in the shortest possible time, so that maximum patients could be accommodated within the OPD timings. On this visit in October 2008, when their turn came, the doctor repeated the same prescription for Papa as there were no new adverse issues. Within 2 minutes they were done. But, on a whim Satv requested the doctor to see Mumma as well since she was developing a speech problem due to the stiff tongue. The doctor asked her a few questions. He also noticed that her thumb was stiff, which the family had thought was because she had recently fallen during a shopping trip to the market. In addition, the doctor noticed somewhat excessive salivation. To Satv none of these appeared serious issues, so she was surprised when the doctor quietly took out a new OPD card and filled it for Mumma, a process which would normally require standing in serpentine queues at the registration counter and waiting your turn. On the card he wrote ‘ALS?’ ‘Admission on priority basis, 16th December’. There was no time to ask questions about what ALS was and why it needed priority admission. The doctor had already gone beyond expectations to examine Mumma out of turn; so, without further probing the family came away mildly bewildered. Mumma appeared normal except for a few issues which seemed minor; so why was the doctor keen on admitting Mumma. Were they planning to do some research on her? Use her as a guinea pig?

November 2008

Over the next month Mumma’s health issues began to worsen rapidly. She felt very weak while walking, and so much saliva had started dripping that she needed a handkerchief even at night. This was no longer minor, and the siblings became concerned. They decided to check ALS on the internet. The family owned one desktop computer which was kept in the drawing room on the first floor where Mandeep’s family lived; while Mumma, Papa and the two sisters lived on the ground floor. The worst evening of their life that they would not wish on an enemy

Back from work, sitting at the computer upstairs Satv, Sukh and Mandeep googled ALS. As the words that appeared on the screen registered in their brain, their hearts began to pound frantically. All the listed symptoms matched with Mumma’s. The disease was a death sentence, with no known cure, and a life expectancy of just 2-5 years. Mumma, their closest buddy, their security blanket, gone in 2 years… they sat in a numb stupor and suddenly the flood of tears began, till they heard Mumma’s voice calling them downstairs for dinner.

A ray of hope - could the diagnosis be wrong?

The next morning, absorbing the shock of the previous night, Satv was battle-ready. What if the diagnosis was wrong? Even the AIIMS doctor had placed a question mark after ALS. Maybe there were other neurological diseases with overlapping symptoms that were not fatal. She decided to take Mumma to a renowned private hospital for a diagnosis. The doctor ordered some tests, including an EMG. The next day when they went to collect the reports, the doctor politely asked Mumma to wait outside while Satv and Papa went into his office.

He confirmed the diagnosis of ALS and offered them no hope of recovery.  He strongly suggested that they go to AIIMS and get her admitted on Dec 16. But Dec 16 was still a few weeks away. Satv could no longer sit still. She looked for ALS specialists in private practice and took Mumma to one of them for consultation. He not only concurred with the ALS diagnosis, he also convinced Satv that AIIMS was the best place to get a confirmed diagnosis. He reasoned with her that the level of experience AIIMS doctors had was simply unmatched, especially in rare diseases like ALS where other doctors saw very few patients. She was now reassured, and waited for 16th December, secretly hoping that the diagnosis would still turn out to be a treatable condition.

December 16, 2008 - The Diagnosis

Mumma was admitted to AIIMS and was lucky enough to get a room in the private ward. She was in good spirits and, sitting on the sofa she looked radiant. So much so that when the team of doctors came to her room, they had to confirm whether she was the patient! Soon a battery of tests started, including MRI, CT, EMG-NCV and more. The doctors asked many questions to accurately record her disease symptoms and progression. After 5 days she was finally discharged with a confirmed diagnosis of ALS. The only drug prescribed was Riluzole which could, at best, improve survival by three months. They were asked to report back to AIIMS in 3 months for follow up.

What next…?

Confronting the reality of the diagnosis, Satv set to work. She decided to apply all her might to defy the verdict. At least Mumma would not go for want of effort on her part. She set out to learn anything and everything about ALS that she could find on the internet. She gathered information from blogs, joined international ALS forums, and went through scientific papers. She visited sites where people narrated their experience with steroids, homeopathic drugs, and other alternative therapies. Well before the 3-month follow up visit was due in AIIMS, Satv had compiled this information into a 20-page document. This mainly contained information about ALS mimic diseases (which can confound the diagnosis), and positive results from some ALS drugs in clinical trials. But she needed expert advice to know whether her understanding of ALS was correct. So, she sent an email to the best expert she knew- the senior doctor who diagnosed Mumma at AIIMS. Knowing how busy he was, she somehow picked up the courage to request him for a 10-minute meeting with her alone, without Mumma. She needed his undivided attention. Sensing her sincerity he granted her the appointment. She was to meet him in the OPD, and call him on his personal phone before coming. In the noisy OPD he shooed everyone into silence and patiently went through the queries posed by Satv from her 20-page document. Along the way he kept explaining to her about why the mimic diseases quoted by her would not apply to her mother. She kept taking notes of all his comments. Finally, he complimented her that she need not come to AIIMS or visit any doctor any more as she knew more about ALS than many doctors in India. Rather she should continue with her self-study and wished that she could manage her dear mother herself on this journey!

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What is Amyoptrophic Lateral Sclerosis (ALS)?

ALS is a severe neurodegenerative condition caused due to progressive loss of motor neurons which control the movement of our voluntary muscles. The name is derived from the Greek: a- no; myo- muscle; trophic- nourishment; that is muscle malnourishment or wasting; lateral refers to the location of affected neurons in the spinal cord; and sclerosis is scarring due to death of motor neurons. ALS belongs to the class of motor neuron diseases (MNDs) and is also sometimes called MND, or Lou Gehrig’s disease (after the U.S. baseball player who died of ALS in 1941 at age 37). It is generally a late-onset disease, appearing most often at ages 50-75 years. (The ALS group started by Satv and Sukh has data of >650 Indian patients which shows that early-onset disease at 20 to 40 years of age is not uncommon in India).

With increasing death of motor neurons, muscles stop receiving signals, become progressively weak and lose function. Early symptoms include muscle weakness, fatigue, difficulty in swallowing and slurred speech. Eventually one loses the ability to eat, speak, move and even to breathe. Once breathing is severely affected the patient needs permanent ventilator support to survive. Since ALS attacks only motor neurons, the sense of sight, touch, hearing, taste and smell are not affected and other brain functions remain sharp. A small percentage of ALS patients may, however, develop frontotemporal dementia. The average life expectancy from diagnosis is 2- 5 years, although with assistive breathing, feeding devices and care, survival could exceed 10 years. The estimated number of people affected by ALS worldwide is 60 to 90 per million individuals.

Causes - ALS is not attributed to a single, straightforward cause. For ~10% cases a genetic cause is known, and there is a family history (familial ALS). Of the 30-odd genes that could be involved, the four most common are SOD1, FUS, C9orf72, and TARDBP43.

The majority of ALS cases are sporadic and may be caused by a combination of pre-disposing genes and a host of environmental factors. Strenuous physical exercise is considered a risk factor. Some personality traits have also been linked, of which, interestingly, a study showed higher incidence of ALS in ‘particularly nice’ people! (The protagonist of this story was certainly a particularly nice lady).

It is thought that many sporadic cases could, indeed, be familial but may be missed due to early death of affected individuals by other causes. Some sporadic cases may also carry de novo genetic mutations, which could be inherited by the progeny. As gene therapy for ALS is under active development, it may benefit patients of sporadic ALS to get tested for possible mutations in the ALS-associated genes.

Pathology - Pathology is caused by neuronal death; however, which key cellular function leads to this is not clear. A common feature in all ALS cases is the appearance of protein aggregates in the cytoplasm, which form inclusion bodies. The most common component in inclusion bodies is the TDP-43 protein. Interestingly, misfolded TDP-43 is found in inclusion bodies in a number of neurodegenerative diseases, including Alzheimer’s and frontotemporal dementia, and inside muscle cells in a number of myopathies. These diseases are sometimes called TDP-43 proteinopathies, and it is possible that a common treatment may emerge for them. Like TDP-43, mutated SOD1 protein can also misfold and form aggregates. Further, misfolded proteins could propagate in a prion-like manner and spread across cells, speeding up disease progression.

Judging from the normal function of the four major genes mutated in ALS, one or more of the following molecular processes could be affected- protein degradation, RNA processing, mitochondrial function and oxidative stress. Both TDP-43 and FUS are RNA-binding proteins. They promote target RNA processing, essential for various cellular functions. The C9orf72 gene contains a string of hexanucleotide repeats. Abnormal expansion of the number of these repeats could lead to pathology by multiple mechanisms, including mitochondrial dysfunction.

Another cause of neuronal death in ALS is excitotoxicity. It occurs due to excessive stimulation of motor neurons by the neurotransmitter glutamate, which then elevates the level of intracellular calcium ions. Motor neurons are highly sensitive to high calcium levels. Riluzole, an approved drug for ALS, works to reduce glutamate levels at neuronal junctions.

Diagnosis - The initial clinical symptoms of ALS overlap with a number of other diseases. To arrive at a definite diagnosis, physicians perform neurological tests (like EMG, MRI and nerve conduction velocity) at regular intervals to monitor the course of disease which is more specific for ALS. Some of the mimic diseases with which ALS could share symptoms include neurological disorders like peripheral neuropathy, multiple sclerosis, spinal cord tumour, spinal muscular atrophy, Guillain-Barré syndrome etc. Others are infectious diseases like HIV and syphilis, and some myopathies.

Treatment - In the absence of cure, it is important to provide supportive care like ventilation to assist in breathing, feeding tube to provide adequate nutrition once swallowing function is lost, and regular physiotherapy to maintain muscle function. A few drugs have recently been approved for clinical use (Riluzole, Edaravone, and AMX0035), but these have limited efficacy. A large number of drug candidates are under development and one can expect more effective drugs to be available in the coming years.

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Trying every possible treatment: Jan to July 2009 - Homeopathy

Mumma’s condition began to gradually worsen. Satv and Sukh were Mumma’s main caregivers but they also had full-time jobs. Luckily for Satv her employer (IBM) allowed a lot of flexibility and she started working from home. But Sukh had no such luck and had to be in office 6 days/week.

Satv remained glued to Mumma round the clock. She could sense Mumma losing her ability inch by inch. She started needing assistance in wearing clothes, going to washroom, and eating her food. Her fingers were getting curled, and palms were getting thin. Salivation had increased to the point that she needed a pan on her bedside to spit saliva during the night. Due to weakness in the leg muscles, she developed foot drop, for which the AIIMS physiotherapy unit gave her a splint, which was of limited help. Most alarmingly, she started having difficulty swallowing her food. Under the circumstances it made sense to try out all possible treatment options that were being touted on the internet, just in case one of them worked for Mumma. A highly reputed homeopathic doctor in Delhi, who had received prestigious awards, claimed to have a treatment for ALS. But he never divulged the name of the medicine, which had to be directly procured from him. Could he be a sophisticated quack? Maybe, but the family decided to try it out anyway. From Jan to July 2009 Mumma took this medicine. It did not help at all. Her condition continued to worsen and they stopped this treatment.

Feb 2009 - Mumma knew about the seriousness of her disease but showed no visible signs of depression. Yet, the mood at home had never been so sullen. Satv sensed that a break would do them all some good. Mumma could still walk, although with care and support. Satv planned a family trip to Jaipur. Charmed by the sights and sounds of the pink city, Mumma and Papa looked so happy on that trip. ALS was momentarily forgotten, and they returned home feeling relaxed and rejuvenated. Satv highly recommends these outings to ALS families in their group now. The Mantra is- Today is better than tomorrow.

May 2009 - Mumma started experiencing new symptoms, like uncontrolled laughter at times. Since ALS symptoms do overlap with a number of other treatable diseases, Satv and Sukh wanted to give a last shot at re-confirming the ALS diagnosis. They made an appointment with one of the top Neurologists of the country. The Doctor discussed the case history with them for over 2 hours, and finally came to the same conclusion of ALS.

Around this time the family made another trip together to Chandigarh to attend a wedding. Satv had heard about a stem cell treatment being developed for ALS at PGI, Chandigarh. She visited them to understand the protocol in detail, and realized that the risk/ benefit ratio was not favorable for Mumma.

Meanwhile Mumma’s speech was getting more and more slurred. She started receiving speech therapy at a nearby hospital. However, within a few months the speech deteriorated to the point where she was almost unintelligible.

August to Dec 2009 - Switched to a Hakim

By now Mumma was wheel-chair bound. She could walk a few steps with help, and could sit in the chair with support, but mostly she was lying in bed. She would experience fasciculation (muscle twitching) in various parts of the body where the muscles would begin to thin out. She could no longer swallow solid food. It was getting difficult for her to use the washroom as well. There was some strength in her hands, but due to curling of fingers, she could not easily hold a brush or spoon. In short, her level of dependence was now almost complete.

At this time, they learnt about a certain Hakim from Bijnor who was known to cure the toughest ailments. They hired a taxi and took Mumma in her wheel chair for the four-hour journey to Bijnor. The Hakim was very considerate and came out to check Mumma in the car itself. Simply by examining her pulse he told them that she had a neurological problem, and that she had undergone surgery at some point. (She had, indeed, undergone a hysterectomy many years ago). The surgery was a crucial issue as the Hakim’s medicine was not as effective in a body that had suffered such tampering. Nevertheless, he gave them the medicine which Mumma took for the next four months. However, there was no improvement and the treatment was discontinued.

Unani, Panchkarma, and Homeopathy again

In medical parlance Mumma’s symptoms of difficulty in swallowing, breathing and loss of speech are indicative of bulbar palsy. In her regular internet searches Satv found a Unani medicine for bulbar palsy which could be obtained from old Delhi “dawai-e-luknat”. It was an ointment to be smeared thoroughly inside the mouth. It did seem to help Mumma initially, but later the effect wore off. Perhaps Mumma’s condition was too advanced, and the medicine might be effective if used early in progression.

Mumma was also taken to an Ayurvedic hospital in Delhi for a 15-day session of panchkarma. This included special oil massage followed by steam exposure. Mumma felt better and could even walk a little after the massage, but the effect did not last. Not wanting to leave any stone unturned, they tried homeopathy yet again with another reputed homeopathic doctor. Mumma took the medicine for three months. Alas, again there was no improvement.

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Current efforts in drug development for ALS

ALS is one of the most actively researched rare diseases. A large number of candidate drugs that target a variety of molecules and processes implicated in ALS pathology are being tested.

Three of these have been approved for clinical use: riluzole, edaravone and AMX0035. Riluzole ameliorates excitotoxicity by reducing glutamate release into the synaptic cleft.

Clinical trial results showed modest benefit, with survival being enhanced by 3 months on average.

Edaravone, an antioxidant, was tested in clinical trials in Japan. It was administered intravenously for 14 days per month, for six months. It could slow down disease progression in selected patients, although its effect on survival is not known. An oral formulation of edaravone has recently been approved in the USA.

AMX0035 is an oral, fixed-dose combination of sodium phenylbutyrate and taurursodiol. Sodium phenylbutyrate is a histone deacetylase inhibitor that upregulates protein chaperones, thereby reducing misfolding, while taurursodiol inhibits mitochondrial associated apoptosis. Clinical trial results showed enhanced survival by 6.5 months. The drug has been approved by USFDA.

Apart from these, there are as many as 91 ongoing programmes to develop drugs for ALS treatment. These include novel formulations, as well as repurposed or reformulated drugs. The drugs being tested target one or the other known causes of ALS pathology. Sixteen of these target neuroinflammation. Antisense oligos (ASOs) are being tried to knockdown the expression of toxic mutant proteins encoded by C9orf72, FUS and SOD1. One ASO targets Ataxin2. Depletion of ataxin 2 was shown to limit TDP-43-mediated toxicity in model systems.

Some of the drugs in active clinical trials

Tofersen is an ASO being developed for the treatment of ALS patients with a confirmed SOD1 mutation. It binds to SOD1 mRNA and reduces the synthesis of the toxic mutant SOD1 protein. It is given monthly by intrathecal injection.

NurOwn is a cell-based therapy in which adult stem cells from the patient are manipulated in the lab to secrete neurotrophic factors, and transplanted back into the patient. The neurotrophic factors can stimulate new motor neuron growth and promote neuromuscular junction reinnervation.

Aldesleukin is being investigated as a drug to suppress neuroinflammation. It is a recombinant human IL-2. Low-dose IL-2 is known to enhance regulatory T cell function in autoinflammatory conditions.

Verdiperstat is an orally administered small molecule inhibitor of the enzyme myeloperoxidase (MPO). MPO increases oxidative stress and inflammation in the brain, which leads to neurodegeneration.

With so many promising drugs under clinical trial, why is it that THE drug that could substantially prolong and improve the quality of life with ALS is not yet available? While ‘failure’ is an integral part of research, it invariably leads to the way forward. Scientists have analysed the reasons for poor success of the ALS clinical trials so far. Some of these are listed below.

Reasons for poor success of ALS clinical trials so far

-        ALS is not a homogeneous disease. Hence patients recruited for trials should be subclassified according to the underlying disease mechanism, for example known genetic cause, and rate of disease progression. Treating all patients alike may confound clinical trial data.

-        Nonavailability of suitable biomarkers to measure drug efficacy. Better biomarkers are being developed. 

-        Nonavailability of appropriate disease models. The SOD1 transgenic rodent model has been used extensively so far for preclinical testing, but it is not appropriate for non-SOD1 origins of ALS. Improved disease models are being developed.

-        In trial design insufficient attention has been paid to determine if the drug indeed had the desired effect on its target molecule. Specific biomarkers are needed to monitor target engagement by the drug.

-        Most ALS patients are diagnosed when the disease is well advanced, at which stage drugs would have limited efficacy. One needs to administer the drug at early stages of disease. The use of biomarkers like NFL (NeuroFilament Light; its levels go up rapidly in plasma as neurodegeneration sets in), for early diagnosis may allow earlier intervention. Validation of these biomarkers is under way.

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2010 - The journey downhill

Mumma’s voice was almost gone. To communicate, they started using an alphabet board over which they guided her hand to point to the right alphabets and form the word. She had completely lost the ability to swallow solid food. So, they started blending her rotis with subzi/ dal and feeding her spoonfuls of slurry. Slowly, her food intake started reducing as she could not move the food in her mouth. It was taking 30-40 minutes to feed her, and swallowing even liquids was getting tough. There were also some instances of severe choking. And so it came to pass in October 2010 that for 3 days in a row Mumma could hardly take in any nutrition at all. She had been losing weight rapidly, and the situation was becoming alarming. It was now necessary for her to be fed through a feeding tube.

October, 2010 - Potential dangers of feeding tube insertion and respiratory complications

Satv & Sukh rushed Mumma to a hospital to get a feeding tube inserted. Satv was on her guard, as she was aware of potential dangers of such a procedure.

They noticed that after the feeding tube was inserted, Mumma was breathing with difficulty and gasping for breath. The doctor did not seem concerned, but they insisted to check Mumma’s SpO2 levels. Sure enough, the levels had dropped. Mumma was put on oxygen, and in a few hours she recovered.

Back at home, Mumma was feeding well with the tube. However, she now needed a much greater level of care. Sukh decided to give up her corporate HR job to take care of Mumma full-time and provide support to Satv. The two sisters were her constant companions and caregivers at home, bathing and feeding her regularly. Although fully dependent and unable to speak, Mumma nevertheless retained her cheerful disposition and stayed in high spirits. But the decline was relentless. Due to progressive muscle weakening, she was now developing breathing difficulties and was not able to cough out phlegm.

Severe respiratory complications- After the feeding tube was inserted, all seemed to go well for fifteen days, till Mumma developed respiratory problems. Her SpO2 levels, which they constantly monitored by an oximeter, suddenly dropped to 80. She was immediately shifted to a hospital where they treated her with antibiotics and put her on a BiPAP machine which applies bi-level airway pressure to open the lungs and assist in breathing. In a matter of 3 days Mumma responded and came back home. Upon discharge, the doctor advised to use oxygen at home (whereas they should have been advised to use BiPAP at home as well).

November 2010 : Harrowing experience in the Intensive Care Unit

In a few days the respiratory issues of Mumma flared up again with low SpO2 levels and chest congestion. This time she had to be admitted to the Intensive Care Unit, where family members or attendants were allowed entry very briefly, only at designated times in the morning and evening. One may expect then, that each patient in the ICU would get personalized attention from the ICU nursing staff. This ICU like many others nowadays, was a large hall with 35 beds, and the number of nursing staff was just too small to permit constant attention for each patient. Satv and Sukh panicked as Mumma was unable to speak or call out for help. She had lost movement, and her fingers were too stiff to press the bell for assistance. They gave her a rattle (toddler’s toy) which she could move to call for help. However, the nurses would put the rattle away, out of Mumma’s reach. When Satv and Sukh went to meet Mumma at the allotted hour, she kept crying and shared with her daughters through the alphabet board that she had to remain in wet and soiled diaper for long hours. Mumma, the ever-cheerful lady, was now forlorn and desolate. Satv and Sukh realized that they had to be with Mumma in the ICU to care for her as they did at home. After much running around and pulling strings, they could finally obtain special permission for one of them to be in the ICU with Mumma at all times, except when the doctors on duty visited the ICU for their rounds.

And so the ICU, and its large waiting room outside, became Satv and Sukh’s residence for the next three weeks. In spite of their best efforts, however, on the fourth day in the ICU, Mumma entered an emergency situation. It happened during the hours when the doctors were doing the rounds and Mumma was alone. When Satv went back to the ICU, she shockingly found Mumma gasping for breath. Her SpO2 level was 74, but there was no one to attend to her. She reported it to the nearest nurse who was too busy to take any action and said this patient is not mine. It needed some running around and screaming for help from Satv to attract the attention of a group of nurses and doctors at the far end of the ICU hall. By then her SpO2 level was 54 and she was getting critical. The whole bunch of staff then descended over Mumma’s bed. An immediate decision had to be taken to place her on invasive ventilator, for which the family’s consent is required. Satv and Sukh had no time to make an informed decision, and signing the consent was a mere formality. Satv shivered at the thought that had she been delayed in going back to the ICU even for a few minutes, Mumma could have lost her life; and the hospital would have attributed it to a heart attack or multiple organ failure, the common reasons given to families of deceased patients.

The two sisters now redoubled their efforts to monitor Mumma constantly. They communicated with Mumma through the alphabet board. Thus, when she guided them to the alphabets to form the word GAS they knew that the food was not agreeing with her. Satv managed to tailgate into the hospital’s kitchen and found, shockingly that the PEG feed was being prepared under most unhygienic conditions, although the attendants while serving it would wear gloves etc.! They obtained special permission to get their own PEG feed for Mumma, which brought her digestion on track.

The surprise came when the word that Mumma wrote on the board was COLD. The ICU was warm enough and Mumma had a couple of blankets to cover her. But the word reappeared on the board repeatedly. They now correlated that she wrote COLD whenever the technical staff performed X-Ray scans on her using a portable machine at her bedside between 1 AM-2AM. Obviously, the whole procedure caused her a lot of discomfort.

Scan or Scam?

Why did the doctors keep ordering scans and blood tests so regularly? Satv and Sukh were no longer alone to ponder over such questions. The waiting hall of the ICU was now their extended family. Attendants of other patients were also camping there for weeks together and they had moved beyond formal pleasantries in their relationship. Apart from sharing food, tea and snacks, they would share their concerns about the medical treatment being provided to their loved ones in the ICU, and the heavy bills being raised every day. A general scenario that seemed to emerge was that no patient was discharged from the ICU with a bill of less than Rs. 2 lakhs, and extra tests were ordered to reach that ‘goal’. Out of frustration, one family called a news media to cover their ordeal, and Satv was amongst the vocal few to share their issues. But the whole affair became an exercise in futility. It only served to make the treating doctor boil with rage, especially against the flagbearers. In that agitated state he blurted out to Satv, “Your mother is only a body, there is nothing in her to survive; you can take her home”. This remark was to come to Satv’s aid very soon.

November 2010 - How long more in ICU?

Mumma had already spent two weeks in the ICU. She had responded to the antibiotics and her pneumonia was controlled. For the last ten days she had been intubated for ventilation (a procedure where a tube is inserted into the trachea, or wind pipe, through the mouth/ nose to keep the airways open). Intubation requires medical supervision and a patient with intubation cannot be discharged from the hospital. A decision was now to be taken whether to wean her off, or continue on the ventilator through a tracheostomy (a procedure where a hole is made through the throat and a tube is inserted into the trachea to keep it open for breathing). A patient on tracheostomy can be discharged from the hospital and cared for at home, albeit with a strict regimen to prevent infection.

Satv had been intensely browsing the internet to learn from international ALS patient groups about the best course of action under Mumma’s condition. The consensus was to continue the patient on ventilator through tracheostomy, and not wean them off. Satv suggested this to the Doctor to which they agreed and the tracheostomy was done the next day. Removal of the intubation tube brought much relief to Mumma.

To remain on the ventilator, however, meant continuing in the ICU, which to Mumma was akin to a living hell. Next day she wrote on the board DIE AT HOME.

Outside, in the ICU waiting room, Satv resolved quietly that Mumma will go home soon, and that she will not die. She set to work scanning the internet to study all about home ventilators and installation of an ICU setup at home. She made detailed notes about how to operate a ventilator, the critical parameters to be considered, and settings to be changed as required. It was not trivial, but it was also not rocket science. With some determination one could learn it. When Satv suggested this to the ICU doctors, they openly laughed at her ridiculous idea. A home ventilator was unheard of in India at that time, although abroad the use of portable home ventilators had begun to gain popularity. In India, however, the prevalent notion was that a patient on ventilator needed critical care which could only be provided in the hospital ICU.

Apart from the mocking by the doctors, another demoralizing factor was friends and relatives who thought that the girls had cared enough for their mother. They should now follow the doctors’ advice and leave the rest to God. After all, they needed to move on with their lives as well.

All this, instead of dampening their spirt, further strengthened Satv’s and Sukh’s resolve. They were determined not to give up without trying. Satv taught herself all about home ICU setup from the internet. She also learnt by observing the ICU procedures in the hospital, and by discussing with the few responsive junior doctors in the ICU. However, she found that some of the ventilator procedures being followed in the ICU were incongruous with those she read on the internet, and could even be dangerous for the patient. So, she kept an open mind and drew her own logical conclusions.

Satv and Sukh now decided to rent a Philips ventilator and switch Mumma to this ventilator in the hospital itself. This would be the safest way to take Mumma home. Also, they could take help from hospital staff for initial operation of the ventilator. But the treating Doctor would have nothing of it. This was not safe and was contrary to their protocol. At this point Satv gently reminded the Doctor, “Sir, to you she is only a body. Please allow it”. And so, Satv did the unthinkable. Mumma would become, possibly, the first known person in India who would be on a home ventilator without the continuous support of doctors/nurses.

Dec 12, 2010 - After three weeks in the ICU, back home on a ventilator

On Dec. 9, while Mumma was in the ICU, she had been put on the rented Philips trilogy 100 ventilator. After testing for 3 days Satv was getting confident of its operation. The nurses and ICU staff had never seen such a portable device and were not aware of this single limb circuit ventilator; so, they looked on with curiosity. Dec. 12 was Mumma’s birthday. A cake was brought for her in the hospital. After cake cutting and making amends with the ICU doctors and staff, Mumma finally returned home. Before her arrival, Mandeep and his wife Kuldeep had gotten the home freshly painted and deep-cleaned, and fitted with all the gadgets needed for ICU care. Mandeep helped in Papa’s caregiving as he too was now dependent and Kuldeep came to support Mumma’s needs whenever required.

To hire a nurse to help them, they interviewed various nurses provided by a certain agency to check their technical knowledge in critical respiratory care, but did not find anyone who inspired confidence. They finally zeroed in on one person who looked somewhat sincere.

For the family the battle had just begun. They had taken it upon themselves to provide critical care to Mumma at home. Now they had to measure up to it. Those three weeks in the ICU had created a unique bond between the two sisters. They realized that they instinctively complimented each other. Sukh was a natural caregiver. Satv did the cerebral part, gathering information and deciding about the best course of action. In turn, both worked on every procedure and discussed everything together, before putting it all into practice.

2011-2012: Mastering the art and science of home critical care

When Mumma was brought home on the ventilator they had no great expectations from themselves. If Mumma could survive even for a few days at home they would have fulfilled her last wish to die at home rather than a cold ICU bed.

They knew critical care was not trivial and they were mere novices. But they were ready to acquire the knowledge and skills for it. At stake was their Mumma, to whom they were indebted for every happy moment of their life. Even now, lying motionless on her bed, the smile had never left her face. Her body may be vegetating, but the brain was fresh as a child’s. Even now, they turned to her for solace.

But mere emotions would not take them far. On the very first day when the nurse they had recruited was asked to perform the suction procedure to clear the trachea, Satv saw his hands trembling with nervousness. It seems he had no practical experience at all, and only knew the theory, which he was honest enough to admit! Satv immediately took charge and did the suction herself! In the days to come both sisters were on their toes, spending sleepless days and nights, dealing with scores of unknown procedures headed their way. But they desperately needed expert guidance. The inexperienced nurse did them one favor. Before leaving he gave them the contact number of a very good ICU nurse named Ajin. As soon as the two sisters met Ajin they knew he was their savior. Although it was very difficult for Ajin to do house duty for them, seeing their utter desperation he agreed to do an eight-hour shift at their place for the next ten days. Under Ajin’s tutelage Satv and Sukh learned the various procedures and could now perform them with confidence. The bond they developed with Ajin, their guide, in those days of acute nervousness, stays strong to this day.

Satv and Sukh now chalked out a daily routine for Mumma’s critical care. In Sukh’s words they were following the path of ‘Kaizen’, a Japanese term for ‘continuous improvement’. They continuously reviewed the methods they were using for each procedure, and introduced changes for further fine-tuning. Ultimately, the level of refinement they achieved by acute observation, and logical reasoning of why Mumma’s body reacted to each procedure in a particular way, earned them the deep admiration of health professionals who occasionally visited their place.

The most common cause of death in ALS is respiratory failure, resulting from gradual loss of voluntary muscle function of the lungs and diaphragm. In addition to breathing difficulty, there can be critical choking of the airways as patients progressively lose ability to cough and clear mucosal secretions of the lung. Many mechanical devices are available to assist the patient, but they need to be operated at settings that are optimal for the patient on a given day (and night). This needs close monitoring on a 24x7 basis, which is a major challenge for the caregiver. It was tough for them, but when work is done with love and devotion, it gets done on its own.

Birthday party in Mumma's Room.

Taking care of every contingency

Satv and Sukh had various emergency measures in place. They always had an oxygen concentrator handy, along with backup oxygen cylinders in case of power failure. For every device and medical supply they had a backup in place, and sometimes backup of a backup as well. One parameter they monitored closely was Mumma’s chest congestion. Any change in her breathing pattern, or in the sound of her cough put them on alert. By feeling her chest, they knew when Mumma needed suction to remove excess secretions that could be blocking her airways. Sometimes she would need suction every few minutes, at other times just once a day.

Another challenge was to maintain a high level of personal hygiene and avoid bedsores. Since Mumma could not indicate when she needed to relieve herself, they would place bedpan every few hours, and rest of the time she had a diaper placed loosely around.

Another issue was to provide adequate nutrition to Mumma through her feeding tube so that she was not malnourished. Electrolyte balance was also an important factor, for which Satv incorporated one feed of a solution of 1 teaspoon sugar and a pinch of salt every day. Mumma also had an exercise routine. Every morning and evening she would be given short physiotherapy sessions to move every body part.

Experiences with Crisis Management

In their early days of home critical care, they had to face many crises. A few weeks after Mumma’s return from ICU, just as they thought they were getting the hang of ventilator operation, they had a confidence-shattering experience. An emergency situation arose on Feb 1, 2011. Mumma started turning pale and her SpO2 levels dropped to 42. Somehow, they managed to improve her breathing through nebulizer. Satv, having silently taken a vow not to take Mumma to the ICU again, realized that the problem had arisen due to low humidity in the inhaled air. Immediately they placed an urgent order for humidifier which got delivered at 1 in the night. Attaching the humidifier brought much relief to Mumma. In normal breathing the air gets warm and moist as it traverses the nasal cavity before entering the lungs. But the air that the ventilator pushes into the lungs through a tracheostomy tube depends on the ambient conditions, which in winters would be cold and dry. The Fisher and Paykel MR 850 humidifier which they purchased simulates the nose to an extent, such that the air reaching the trachea is warm and moist. However, it also entails greater risk of infections as the humidifier tubes, being constantly moist, provide fertile ground for bacterial growth. Hence strict cleaning procedures have to be followed, besides ensuring that the humidifier chamber is filled every 2-3 hours round the clock.

Satv was deeply involved in learning about the operation of each machine, and how to optimize it for Mumma’s critical care. She would frequently be on the phone with the technical care experts of ventilator and humidifier devices with a barrage of queries. They were always supportive of her. Her level of engagement was so exceptional that her suppliers did not treat her as a mere customer. The manager of Fisher and Paykel arranged for Satv to attend a critical care conference which gave Satv a lot of exposure, and good contacts with dealers in home ICU. This helped her later in their journey, as she could share these contacts with the larger ALS community.

In spite of continuous monitoring, on 3-4 occasions Mumma experienced severe episodes of pneumonia (or could be sepsis?) where the whole body was cold and profusely sweating, and Mumma was unresponsive for 2-3 days. The doctor who came to examine her gave no hope of survival, but Satv and Sukh continued to follow their routine. On these occasions they would put Mumma on supplemental oxygen attached to ventilator. The suction aspirate would be sent to a lab for testing drug resistance of the bacteria in it. They had two families of antibiotics in their armor. Accordingly, Mumma would be put on a suitable antibiotic. And on the fourth day, she would open her eyes and the two sisters would take a bow and say, “what are you made of-you are back again!”.

2012-2014 : Feeling comfortable with home ventilator care

Ventilator care meant there was no telling when a critical situation might arise. Even though they had kept a backup for everything (even themselves, as they were two!), there was no scope for complacency. Nevertheless, the comfort level which Satv and Sukh had begun to feel while taking care of Mumma was gradually increasing. They could now dare to think of their own lives which had been relegated to the back burner. Sukh had given up her HR job to give full-time support to Mumma in 2010. In 2012, she now found a teaching job in a reputed school very close to their house. This was convenient, as in an emergency she could rush back home as well. They hired a full-time house help who could also give supportive help for Mumma’s care. Satv had been working from home, which was extremely practical at this time, but she did miss the office atmosphere and socializing with colleagues. Sukh began to occasionally take a day off to allow Satv to visit her office.

At work both sisters walked the extra mile to become assets for their respective organizations, and so they never had professional insecurity.

Mumma’s indomitable spirt : Living on a ventilator with no regrets

It is one thing to merely exist on a ventilator, with a tube permanently stuck in your trachea; and quite another to treat the machine as an extra organ and make the most of life. When you have one tube in your stomach to feed you and another to make you breathe, it is not hard to imagine the level of discomfort you must be enduring every moment. It is a tribute to Mumma, her daughters and her whole family who could together retain the happy, boisterous atmosphere of a typical Punjabi home in the face of this harsh calamity. The family was clear from the very beginning that Mumma would not merely exist, she would live life, whatever the number of days destiny provided to her.

The level of care that they provided to her has been documented in detail on their site alslifemanagement.weebly.com/daily-routine.html

In the morning, before Sukh rushed off to school, she would brush Mumma’s teeth and clean her face. Satv and the house help would give Mumma a comfortable sponge and dress her up nicely. Then Kuldeep would give her the PEG feed for breakfast, liquefied to be fed through the tube. Before blending the food, they invariably showed it to Mumma to retain her sense of involvement. From 9-11 am Mumma would watch Gurbani channels on TV.

After lunch Mumma would usually sleep for a couple of hours. Satv, sitting in Mumma’s room would do her office work while keeping an eye on Mumma’s parameters all day. Every evening at 4.45 pm Mumma would listen to Gurbani live telecast from the Amritsar golden temple, a most uplifting experience. Later in the evening both grandchildren would visit, and she truly cherished their company. Mumma’s room was the family’s party place. Every birthday party, Mother’s day, Diwali festivities and other events would be celebrated in her room. Even the neighborhood kids would join in and play board games in connected rooms and dance there, and Mumma, paralyzed except for face and eye movement, participated with joy shining through the eyes.

At night Mumma, Satv and Sukh would watch TV together for a while, after which they would dim the lights and play “Sukhmani Sahib” paath to create a soothing atmosphere before going to bed. Satv and Sukh took turns to watch over Mumma while the other sister slept in the adjoining room. Their minds were acutely conditioned to react to every abnormal sound emanating from Mumma, be it of dripping saliva or a change in breathing pattern.

While taking care of Mumma the family would be constantly chatting with her and seeking her advice on matters. The expression in her eyes was sufficient for them to gauge her opinion.

Mumma; a million reasons to be happy!

Big B writes to Mumma!

Another source of joy for Mumma was watching her favorite programs which included Salman Khan movies, Kapil Sharma show and Amitabh Bacchhan’s KBC show. To give her an extra dose of pleasure Satv decided to write to these three greats to send Mumma a personalized note which would make her feel special. Big B did oblige and sent her a very sentimental letter. Mumma read the whole letter moving her eyes slowly over every word.

The sparkle in her eyes was worth a thousand words of gratitude.

2015-2018: Sharing knowledge with the larger ALS community

When Mumma got diagnosed with ALS in 2008, Satv and Sukh wanted, desperately to meet other ALS patients in India to learn from their experiences. But that did not happen and they had to fight their battle alone, learning from their mistakes the hard way. Now with several years of experience and deep self-learning about critical care at home, they felt it was time to share their successful journey with other ALS patients in India and help them cope with this disease.

And thus was born ALS Care & Support (ALSCAS) with the mission to provide knowledge and guidance so that persons with ALS could manage their disease and live with dignity. They started documenting their vast experience in alslifemanagement.weebly.com, which helped to connect with other ALS patients. Soon a network of patients and caregivers began to form. They started the ALSCAS whatsapp group with 5-7 members, which now boasts of over 650 members (and growing). They call themselves CALS (caregivers of ALS) and PALS (person with ALS). Hundreds of CALS and PALS have initially benefited from the personal guidance of these two sisters. Subsequently, the collective, ever-expanding knowledge of CALS on their group has been constantly shared among fellow members.

More than 200 home ICUs have been supported through their Home-ICU group. In 2022, seeing the rapid growth of the India-wide whatsapp group, Satv divided all Indian states into 8 subgroups so that the families in each subgroup could locally connect and support each other.

Detailed accounts of how to set up ventilator and accessory critical care facilities at home, and the management of crucial issues that arise with ALS patients have been diligently recorded on their website. Procedures that were considered impossible to execute at home, like changing the PEG tube, were also mastered by them. Many in the ALS group now avoid going to hospitals and use this shared knowledge of the group.

In sharing their experiences, Satv has poured her heart out to provide hope and inspiration to others newly treading the path. To Satv and Sukh, even though the path was challenging, with round the clock procedures, many life-threatening situations, several instances of out-of-the-box thinking to resolve crises, yet it contained some of the most memorable, and fulfilling moments of their life.

Sept 2018 : Mumma bids adieu

For the past few months Mumma had been slowly declining, giving the indication that the journey’s end was approaching. Then, on that long September night, her face bearing a calm, serene expression, her children around her, she breathed her last. She radiated so much peace that when they made her ready for the final sponge without all the tubes sticking from her body, she looked like an angel from heaven. There was no need for grief.

Life after Mumma

Every night before going to bed, Satv and Sukh would kiss Mumma on the forehead saying ‘Guru Rakha, Rabb rakha’, and place her hand on their head to receive her blessings. This remains unchanged, as they can feel that gentle touch every night when they seek her blessings. They are in silent communion with her and she continues to be their guiding force.

Indeed, Mumma has now returned in the form of Harneet Kaur, a 3-year-old irrepressible girl whom Satv and Sukh adopted through CARA in July 2023. She has filled their home with cheer just the way Mumma used to. Both mummas of Harneet (Satv and Sukh) are enjoying the blissful life of a family through her.

Collage of ALSCAS Group

The ALS Care & Support Family, India

Satv and Sukh feel connected with their ALS patients and caregivers of India as their extended family. It is their endeavor that every patient who joins the group, receives the best advice and care to prolong and improve their quality of life through the dynamic knowledge base of ALSCAS. They believe that they have found their purpose of life with ALSCAS mission. Some of the activities of ALSCAS are listed below.

Engaging with Neurologists in India - Realizing the lack of awareness about proper management of this rare disease even amongst health professionals, they are engaging with Neurologists as well. Doctors are beginning to recognize the knowledge and understanding this community has about disease presentation and management. In 2022 they were invited to the 29th Annual conference of the Indian Academy of Neurology (IANCON).  To the attendee Neurologists, it brought alive what living with ALS means. ALSCAS's caregivers shared their personal journeys – the emotions, the daily challenges, the innovations of CALS to make the life of PALS more comfortable, and above all the triumph of hope over despair. In 2023 they received the "NeuroRehab Initiative of the Year" award from the Indian Academy of Neurology.

Engaging with the Government - They have been in discussion with the Ministry of Health since 2021 to include ALS in rare disease policy. It will help to raise awareness about the disease and to fund research for treatments.

Moving beyond ALS - Over the years, the ALSCAS group has supported so many ALS caregivers to set up long-term critical care at home, that Satv believes their learnt knowledge and tested protocols could save not only ALS patients but many others admitted to ICUs. From their experience they have seen that a large number of ICUs in the country are inadequately staffed. Where an illness requires a prolonged stay in the ICU, it stands to benefit both the patient and the caregiver to set up the necessary facilities of critical care at home.

International recognition -

At the 13th World Congress for Neurorehabilitation WFNR at Vancouver, Canada they presented a poster on innovative low-cost communications solutions for people living with ALS and other neurodegenerative diseases.

Recently, ALSCAS has attained full membership of the international alliance of ALS/MND association as their India arm.

Satvinder’s Top 10 Pointers for the ALS Journey -

1. Just Diagnosed - Consult 3-4 doctors to confirm the diagnosis and rule out mimic diseases. Must join an ALS support group and remain closely connected, maintain a positive home environment, and plan some interesting things to keep spirits high.

2. Stay Active - Engage in light exercises to remain active but avoid strenuous physiotherapy or hard massages. Gentle oil application is okay.

3. Mobility Impact - Use aids like walkers, wheelchairs, and railings to prevent falls. If bedridden, an airbed can improve blood circulation.

4. Speech Impact - Practice speech exercises and explore communication tools like bells, eye trackers, laser pens, alphabet boards etc.

5. Nutrition Impact - Required to churn food? OR taking 30 mins and more to eat food? OR portions got reduced? OR chocking episodes? OR significant weight loss? Please consult a gastroenterologist and consider PEG tube feeding. If surgery is not an option, a Ryle tube is a substitute.

6. Breathing Issues & Non-invasive ventilation - Regularly monitor SpO2 levels. Prepare for home BiPAP and oxygen concentrator use if levels drop below 96 when awake or 94 when sleeping. Start using breathing assistive devices intermittently as needed. Ensure the device has BiPAP ST mode with AVAPS/IVAPS features. Refer to ALSCAS resources for more information.

7. Tracheostomy/Invasive Ventilation - If BiPAP usage is increasing or required nearly all day, a planned tracheostomy should be preferable. This approach can prevent the need for emergency ventilator and the associated ICU trauma during lung infection episodes. Instead of weaning off the ventilator in ICU, opting for a tracheostomy as a second step is advisable. Keep device on Bipap ST mode with avaps/ivaps feature ON.

8. Home ICU Setup - Refer to the ALSCAS Home ICU guide resources. Caregiver must understand and learn most important tracheal suction procedure besides every other needed. Have backups for all essential devices and train nurses/attendents/caregivers on managing pneumonia and other issues.

9. Role of caregiver -  in the ALS journey is crucial, providing essential physical, medical, and emotional support, including assistance with mobility, personal care, and communication. Caregivers must take small breaks to manage stress and avoid burnout.

10. Multidisciplinary specialists - Please find and get in touch with good physicians, Physiotherapists, speech therapist, Gastroenterologist, pulmonologist, ENT consultant and consult them whenever needed.
    

ALS affects the body, not the mind. With proper management of nutrition and breathing by taking PEG & Bipap/Ventilator decisions at right time (pt 5,6 & 7), one can live a long, fulfilling life. Stay employed if possible, as policies may entitle you to full salary. Plan for financial management and decision-making throughout the journey. Our ALSCAS family is a live example of hundreds of patients living for months/years in home ICU setup. You are not alone in the journey. Till the time there is no cure, there is care & Support (ALSCAS).

Stephen Hawking - The man who defeated ALS

In the scientific world Stephen Hawking is not only a marvel for his contributions to theoretical physics and cosmology, but also for being the longest known survivor of ALS. He was diagnosed with the disease at 21 years of age, and according to his doctors he had just a few more years to live. Sure enough, the disease starting taking its toll and as the progression continued it left him almost completely paralyzed and without his voice. But the mind was constantly at work solving some of the toughest mysteries of the universe. He used his still-active cheek muscle to communicate through a computer-generated voice. Defying ALS, he lived on to enrich the world till he was 76.

There is no clear explanation of how Stephen Hawking could survive the disease for over five decades. Although the type of early-onset ALS he had is generally slow in progression, he lived on in a paralyzed state for decades. Being a celebrity he had the resources and connections to receive the best round-the-clock medical attention, which surely would have helped brave many a crisis. But perhaps the one factor that trumped them all in contributing to his longevity was his mental attitude and complete devotion to his work. In Hawking’s own words, “When you are faced with the possibility of an early death, it makes you realize that life is worth living and that there are a lot of things you want to do.”

Satv and Sukh couldn’t agree more. The positive mental attitude, and atmosphere of love and care around the patient are the magic ingredients to fight ALS or any debilitating disease. Love and care are the medicine that works wonders when doctors give up.

Their message to others- Do find the time to take personal care of your sick and elderly parents. When you care for them with your own hands, they respond magically. They nurtured you day and night when you needed it. Now it is time to pay back.

When being strong is the only choice you grant yourself, nothing remains impossible. References: ALS_FactSheet-E_508C 2020 https://www.als.org/understanding-als 

Mead et al. Nature Rev Drug Dis. 2023.- https://doi.org/10.1038/s41573-022-00612-2.