Disease Progression Summary (Draft...):
Mumma (born on Dec 12th 1948 ) has been very active, humble and caring throughout her lifetime. She used to manage home, office along with upbringing of kids perfectly well with love and care.Our life was moving fine like any other family having their usual ups & down. Mumma was always available for support needed by anyone and never took any stress. She served 35+ years in Central Govt Job..
As expected in later stages of life with any regular person, mumma was also experiencing continuous pain in knees for all those years. Orthopedic and gynecology doctors suggested that this is due to calcium deficiency OR after effects of hysterectomy procedure performed during 1997. Due to this, mumma was regular visitor for consultation on these knees problem at rehabilitation center of AIIMS with respected Dr. U Singh HOD of that deptt.. She took voluntary retirement from post of Library Information officer in Feb 2005 from Central Sectt Library, Deptt of culture. So 2005 was year of lot of change in her routine of life
Few important points from life history
• In late 1970s Sciatica problem (which got cure with medication and time in few years)
• 1997 Hysterectomy Operation.
• 1997-2000 HRT (Hormonal replacement therapy)+ Calcium tablets
• 2000-2007 Stopped HRT, -Sever Pains most of times in legs, 3 times locking in knees and cartilage breakdown, (Catiligen forte for 4 months which helped) Regularly on Calcium.All time with medicines from orthopedic (AIIMS)
• Feb 2005 – Took voluntary retirement from central Govt job.
After taking retirement as well, she kept all those active life by visiting gurdwaras, reciting religious shabad and other house chores.
ALS –Symptoms Started
During Jan 2008, mumma mentioned that she can’t clearly sing lines while reciting shabad and facing some difficulties also while eating and seems her tongue was going numb. Dr. U singh might have noticed something and suggested some tests and advised to consult neuro doctor.
June 2008 -
• MRI –Brain done – Reports all clear.
• Sometimes shared problem in swallowing and fasciculation’s in body.
• Mumma started walking slow. We always accompanied whenever she moved out. One day there was jerk in hand due to imbalance holding in Market. We noticed problem in thumb after this. Thumb showed sign of curling inside, mumma started facing difficulty in picking up anything or while buttoning.
• Muscles in left hand palms seems thin.
2008: thumb started curling inside, also facing fasciculations across various body parts , excessive saliva and some change in voice. Dr. U Singh referred us to Neurology deptt. AIIMS for Neurological review.
We kind of surprised that what is relation of curling of thumb with that of Neuro doctor. So it got delayed for another 3-4 months. In Nov 2008 during general visit to AIIMS (for our father medicines) , I showed mumma same thumb condition along with voice changes and fasciculation problem in body to Dr Sumit Singh Astt professor Neuro deptt. I don’t know what came to Doctor’s mind who despite really busy prepared new consultation card for mumma and mentioned priority admission for his Deptt to undertake. He shared he wanted to keep mumma in observation and perform some tests during 3-4 days of admission so as to rule out some serious problem he was expecting. And to our surprise we got admission date of 16th Dec (which was just 3-4 weeks away) from Neuro deptt at AIIMS where people wait for months and years. This made us really think this topic seriously.
Before planning for admission in AIIMS (which looks quite big) for few minor problems (like changes in voice, knees pain, thumb curl which we thought of jerk happened in market), we thought of getting checked from nearby hospitals. Before going for first opinion, I sat on internet for hours and scrolled about the word ALS which Dr Sumit Singh mentioned with '?' character in AIIMS consultation card. I had interest in medicine and kept exploring medical news, but never heard of this 'ALS' term before. I was shocked to see what was the disease and its symptoms which were quite matching with mumma present condition at that time. Knowing that ALS will slowly take away strength from limbs, and ability to speak, swallow and breath and finally life in short period of time...Life was going blank in front, still we recollected and made assured, that it’s not something which mom will be having as that disease is very rare.
First we went to Jaipur Golden Hospital where a senior Neuro doctor recommended EMG test and showed him report on same day. It was quite painful test, however after lot of discussion with mumma and my sister and thinking of avoiding AIIMS admission, we got test done and showed reports to doctor. He suggested mumma to sit outside and gave us this devastating news of ALS. I was in total non-convincing mode throughout the discussion. He mentioned one statement which I still remember and give it to everyone, I come in contact with ALS patients and their families i.e. “TODAY IS BETTER THAN TOMORROW”. He gave us overview of medical details in few minutes and still suggested that we should get final decision from AIIMS and must get admission.
As there were few weeks still left, we took second opinion from another senior Neuro doctor at his residence who also did some locomotors movements’ tests and made mumma speak few selective words from his collection. At end of 1 hour of discussion and study he told about the same incurable disease which is ALS.
2008: AIIMS. (DOA -16th Dec08, DOD-20th Dec 08). Diagnosis results: Amyotrophic Lateral Sclerosis (ALS).
5 days admitted in AIIMS for diagnosis. Following tests were conducted:
MRI
ECG &Chest XRay Normal
Thyriod Elevated (last three tests results : 6.8, 7.6, 7.2 )
All blood tests normal
Mumma was quite fine and no-one could judge who is patient in that big private AIIMS nursing room in which mumma and either of us (me and my sister) was staying(ref: 2008 pic above taken at AIIMS ward). And final results were still the same which we never wanted to hear.( Concluding remarks- ALS). During stay at AIIMS. I took all my researched documents to Dr. Sumit Singh who was reviewing mumma case along with his team. He is exceptionally talented doctor. He patiently listened to my queries and gave me few lines of explanation for ruling of each and every mimicry disease I had quoted. For few things , he appreciated these notes and shared that caregivers in such kind of incurable diseases are more knowledgeable and updated then the practicing doctor. He suggested to start Rilutor 50 tab BDS in case we wish to start.
We started Rilutor 50 BDS after reaching home. Mumma being knowledgeable about disease and explored in internet, but did not showed any kind of depression or cried whatever we remember. She started experiencing few other symptoms like- uncontrolled laughter and emotions sometimes.
Our life anchor is mumma and hearing the news that slowly and slowly her body will become motionless and all those dreaded things which were stated to happen during course of progression led feelings of depression and kind of feeling our life is also coming to end. I feel good that we never ever showed our low emotional periods to mumma. But it was quite tough initial day/weeks/months to digest this bitter fact of life.
Feb 2009- Planned out of station trip to Jaipur resort. Was really nice experience. Regret now we did not planned such trips earlier.
Mid 2009:
Mumma deterioration continued(or even became faster …with Rilutor administration) every passing week, saliva increased –she need to wipe her face with dropping saliva while sleeping, she needed support while walking (first needed walking stick) and later in couple of months required left body support along with complete care on moving right foot forward due to initiation of foot drop condition. She experienced fasciculation’s in various parts of body at random times. She started needing assistance in wearing cloths, buttoning and fingers were getting curled, palms were getting thin. Also there was increased difficulty in swallowing food –it took half an hour and more to complete the meal and require immediate attention and care all this time as drinking liquid was getting troublesome. So we required to be seated alongside mumma for feeding assistance through day and night.
It was getting difficult to make her walk over to washroom for daily chores so we got folded lavatory chair and bring that up whenever needed. For getting bath- we used to make her sit in plastic chair and take that chair in bathroom and do her bath. While for brushing and hand wash – we got big bowl, kept below the chin and do these procedures. Mumma was able to do few things from hands, as hands were still able to move however curling of fingers inside making it difficult to hold brush or spoon.
June 2009
• Saliva secretion increased, more evident in early morning. Need bucket at bedside to split saliva.
• Frequency of Fasciculation/twitching in body muscles/tongue got increased.
• Continuous feeling weakness in whole body. Severe pain in one side of back (kamar)
• Movement reduction in left hand’s thumb and in index finger. When mumma tried to hold up anything, fingers kind of curled up inside
• Knees – Weakness most of time. (No pain)
• Fatigue after 10 mins of walking. (Imbalance walking). She used to walk perfectly fine before Jan 2008.
• Difficulty in speaking.. slurred speech..slow speech
• Difficulty in Swallowing food and water, tongue movement reduced…(Eat slowly and now on semi-solid food)
• Problem in left foot started since March 2009. It looks swollen and detached from the ankle. Walking became dragged.( Foot DROP)
• Experienced cramps while curling of nerves in full hands and legs whenever any specific movement happen (buttoning , holding heavy weights)
Voice reduced and unclear significantly since May, Most of the lines needed to be repeated. Weight looks reduced.
During mid 2009 Our wish for any other mimicry disease was still intact , so kept searching for supremo in Neuro filed in India specially in ALS or MND cases. We found Dr Gauri Devi (Dr. Gauri Devi (MD, DM(Neuro), Chairman Deptt of Neurophysiology, SGRH, Professor & HOD Neurology-NIMH Bangalore, President Neurological Society of India.) and visited her in May 2009, that time mumma was needing quite a support to walk so took her to wheel chair for appointment room. Dr Gauri discussion continued with mumma and both of us sisters for 2 hours and she also came to same conclusion of ALS.
Dec 2009
• In addition to above symptoms following starts appearing :
• More frequent difficulty in swallowing food . Now take almost 30-45 mins to consume 1 roti dip in daal. Few challenging situations happened while swallowing food – got choked and got breathless for few seconds while eating. (One should go for PEG ASAP)
• Not able to take tablets; need to break into small pieces. Sometimes need hands to move food from one portion of mouth to other as tongue was difficult to move.
• Difficulty in taking clear liquids (water, coconut water, milk) more prominent.
• Fasciculation/twitching in usual body muscles reduced. Now moved to those parts of body where it did not happened before.
• Weight reduced 8-10 Kg as food intake reduced. (<50 KG)
• Walking reduced a lot. Left feet is almost dragging( due to foot drop)
• Speech: slurred and reduced. Mom usually said single word instead of complete sentence. Very difficult to understand, need repetitions.
• Tongue movement reduced. Tongue usually looks swollen with some swollen nodes on its surface.
• Weakness in whole body, walking with back slightly lowered. Was unable to walk straight.
• Left hand : all fingers curled inside (spasticity) Muscle mass reduced a lot from left hand palm. Right hand –thumb also curled and looked stiff.
• Legs : Muscle mass reduced significantly from lower and upper portion of leg. More significant in left side. Left foot need to drag (Foot drop)
Difficulty in doing Respiratory exercise
Mar 2010- Could not walk , needed wheel chair.
2010 Oct : Swallowing difficulty progressed and weight lost significantly. Consulted Dr. Monika Jain (MD, DM Gastro) and got PEG (Percutaneous Endoscopic Gastro procedure) done in October.
2010 Oct: Started facing respiratory difficulty and got admitted in at Hospital and stay there for 3-4 days. Given Meropenum IV and got fine and was discharged. Advised O2 1-2 L/min at home by O2 concentrator, Monitoring by pulsoxymeter).
2010 Nov : Extreme Respiratory difficulty O2 value dropped from 98 to 88. Frequent urination, anxiety-admitted immediately at Hospital. Started with BIPAP support: could not sustain and intubated in late Nov.Weaning off was tried but could not sustain and was again connected to mechanical ventilator.
Tracheostomy was done on 30th Nov 10. In view of continuous ventilator support, we took trial of Porta Vent Trilogy 100 in in hospital for 4 days and got discharged on 12th Dec 10 on Ventilator only.
Medication Management during progression early 2009-mid 2010
- Riluzole (50 mg one daily) : Started in January 2009 but since then progression seems to speed up . So from last Sept 2009 we completely stopped.
- Homeopathic medicines between Jan 2009-Sept 2009(Dr. A.K. Gupta of Ovihams). No prescription detailed available. Waste of money and time there. Homeopathic medicine. Based on someone advice, Started Ammonium Causticum (one drop every 2 hours since 25th Dec evening). Ability to swallow seems to be benefitted by that. Consulted another homeopath (Dr. Pankaj Aggarwal) in Jan 2010 who asked to stop ammonia causticum and started with IGn (olfaction) every 3 hours. And this went till March when Dr. Khan started new medicine. But no benefit of these medicines, so stopped.
- Physiotherapy – No significant improvement and neither helped in reducing progression.
- We got done few sessions of Panchkarma oiling therapy in March 2009. – No improvement however mumma felt nice doing that.
- Speech therapy – No significant improvement.
- Alternate day -mustard oil from massage lady but tapers its frequency 2009 end.
- Bijnor renowed vaid medicines -visited 4-5 times .. it seem to help in first month but later no difference so stopped.
- Nutralite powder in feed mixture.
- Milk with turmeric(few weeks): started but could not continue as faced difficulty while drinking.
- Tried Milk with Chawanprash as well for few weeks.
- Some multivatims syrups.
- Cod Liver oil – 1 spoon daily
Besides medication or trying alternative therapy, we have spend money in superstitious things as well during 2009 (we did not believed in even one single superstitious throughout life ) But when one looses hope from everywhere, tends to fall trap of such things as well and we must admit that we did such things like buying and wearing precious gem stones, doing some activities which astrologer advised , visit here and there after receiving advise from many people. But nothing helped and within very less times we realized our mistake and got back to our basics and values.
Above is just draft version, there are lot of missing details which we may add later