Brilliant intelligence of Sir, Albert Einstein and quickness of wit of Mr Sherlock Holmes

  I was always mesmerized by Sir, Albert Einstein, and his extraordinary intelligence being always fascinated by the genius of his mind which remained a mystery. 

I always kept a picture of the revered man pasted on the wall of my study table. In the house I grew up.

I hoped if only I could have a magnificent brain like him. But I ended up with one scattered throughout with tumours.

I fancied his intelligence but not being stupid, dull or clumsy I ended up with an average intelligence to help adaptability with a rare disease and the conditions of life which brought me from riches to rags.

But I survived a hellish trek for which there was no map or previously beaten path. I was lucky to be surrounded by supportive, loving and optimistic Mum. In the vacuum of non awareness where people are too complicated to be figured out objectively, plus they are known to change their mind! I moved on with complete sang froid.

I investigated several articles on what made Albert Einstein such a genius.

Einstein’s brain had an extraordinary prefrontal cortex and that inferior portions of the primary somatosensory and motor cortices were greatly expanded in the left hemisphere. 

The corpus callosum is the largest nerve fibre bundle that connects the cortical regions of the cerebral hemispheres in human brains and it plays an essential role in the integration of information transferred between the hemispheres over thousands of axons.

It is a large C shape of white matter that divides the cerebral cortex into the right and left hemispheres.

 

Einstein’s corpus callosum circularity is significantly larger.

In most of the genu, midbody, isthmus, and part of the splenium, Einstein’s corpus callosum is thicker than normal but thinner in the most rostral body.

Einstein’s brain weight was very similar to the mean brain weight of the elderly control group. Einstein’s brain was normal for his age

Einstein’s corpus callosum in the genu(knee-like anterior curvature of the corpus callosum of the brain, ending in the rostrum or beak of that organ: as, the genu of the optic tract)is wider.

The corpus callosum (The anterior one-fourth of the callosum is considered the genu. The rostral body begins directly behind the genu, extending back to include the anterior one-third of the callosum. The center one-third of the callosum is split into two equal sections, the anterior and posterior midbody. The isthmus extends from the posterior one-third to the posterior one-fifth of the callosum. Finally, the most posterior one-fifth is considered the selenium)is the largest bundle of white matter neural fibres in the brain that connects the interhemispheric cortices, and it may be involved in any neuroanatomical substrate of hemi­sphere specialization.

The fibres that pass through the callosal ros­trum and genu appear to connect the interhemispheric regions of orbital gyri (The orbital gyri are located on the inferior surface of the frontal lobe. There are four gyri and they are divided by the H-shaped orbital sulci. They have a role in the perception of odors) and prefrontal cortices corresponding with the left and right Brodmann areas 11/10(Brodmann’s areas are typically shown on a map of the brain surface, but each region is continued through the depth of cerebral cortex), which are involved in planning, rea­soning, decision-making, memory retrieval, and executive function.

 

• Brodmann areas 1, 2 & 3: primary somatosensory cortex (postcentral gyrus)
• Brodmann area 4: primary motor cortex (precentral gyrus)
• Brodmann area 5: somatosensory association cortex (superior parietal lobule)
• Brodmann area 6: premotor cortex and supplementary motor cortex 
• Brodmann area 7: visuo-motor coordination (superior parietal lobule)
• Brodmann area 8: frontal eye fields
• Brodmann area 9: dorsolateral prefrontal cortex
• Brodmann area 10: anterior prefrontal cortex
• Brodmann area 11 & 12: orbitofrontal area (orbital gyri, gyrus rectus, rostral gyrus and part of superior frontal gyrus)
• Brodmann area 13 & 16: insular cortex
• Brodmann area 17: primary visual cortex (V1)
• Brodmann area 18: secondary visual cortex (V2)
• Brodmann area 19: associative visual cortex (V3, V4 & V5)
• Brodmann area 20: inferior temporal gyrus
• Brodmann area 21: middle temporal gyrus
• Brodmann area 22: superior temporal gyrus (including Wernicke area)
• Brodmann area 23, 24, 28 to 33: cingulate cortex
• Brodmann area 25: subgenual area
• Brodmann area 26: ectosplenial portion of the retrosplenial region of the cerebral cortex
• Brodmann area 27: piriform cortex
• Brodmann area 34: dorsal entorhinal cortex
• Brodmann area 35 & 36: perirhinal cortex & ectorhinal area
• Brodmann area 37: fusiform gyrus
• Brodmann area 38: temporal pole
• Brodmann area 39: angular gyrus
• Brodmann area 40: supramarginal gyrus
• Brodmann area 41 & 42: primary auditory cortex (Heschl gyrus)
• Brodmann area 43: primary gustatory cortex
• Brodmann area 44: part of Broca area (pars opercularis, part of the inferior frontal gyrus)
• Brodmann area 45: part of Broca area (pars triangularis, part of the inferior frontal gyrus)
• Brodmann area 46: dorsolateral prefrontal cortex
• Brodmann area 47: pars orbitalis, part of the inferior frontal gyrus
• Brodmann area 48: retrosubicular area
• Brodmann area 52: parainsular area

There is a hypothesis consistent with the finding that Einstein had relatively expanded prefrontal cortices. The morphology of both his corpus callosum and prefrontal cortex may have provided the foundation for his exceptional cognitive abilities and remarkable thought experiments.

The neural fibre bundle that passes through the callosal midbody and isthmus mainly connects corresponding interhemispheric pre-motor cortices (Brodmann area 6), primary motor cortices (Brodmann area 4), primary somatosensory cortices (Brodmann areas 1/2/3), secondary somatosensory cortices (Brodmann area 5) and parts of the parietal region. These fibres have the largest and most heavily myelinated axons, which transfer information faster. Einstein had an enlarged omega-shaped fold in his right primary motor cortex, which probably represented the motor cortex for his left hand, an unusual feature that may have been associated with the fact that he was a right-handed violin-player from childhood. 

Fibers of the posterior isthmus and splenium are thought to connect corresponding parts of the superior parietal lobules (Brodmann area 7), inferior parietal lobules (Brodmann areas 39/40), and temporal cortices (Brodmann areas 20/21/37), whereas other fibres of the splenium have been shown to connect extensive cortical regions including the occipital cortex (Brodmann areas 17/18/19).

The superior parietal lobules are involved in visuomotor coordination, spatial attention, and spatial imagery. Recent functional MRI studies indicate that the superior parietal lobule and the intraparietal sulcus (groove)are both activated during mental arithmetic and digit memory tasks. The inferior parietal lobules are concerned with language, mathematical operations (especially on the left), spatial perception, and visuomotor integration. The occipital cortices are in charge of visual processing and can be activated during imagery with eyes closed. The inferior temporal gyri (Brodmann area 20) are involved in high-level visual processing, recognition memory, face and body recognition, and processing of color information. The parietal lobes of Einstein’s brain were 15% wider. Einstein’s right superior parietal lobule (Brodmann area 7) was considerably wider than the left, his right intraparietal sulcus was highly unusual, his left inferior parietal lobule appeared to be relatively expanded compared to the right, and the cortical surfaces of Einstein’s occipital lobes were very con­voluted. The ratio of glial to neuronal cells was significantly greater in Einstein’s left compared to right Brodmann area 39 and relatively increased in the bilateral temporal neocortices. The glia affects neuronal excitability, synaptic transmission and coord­inate activity across networks of neurons.

It was observed significant positive correlations between posterior callosal thickness and intelligence measures. Einstein’s extraordinary spatial imagery and mathematical gifts were grounded on definable neurological substrates. Although the intelligence of human beings cannot be fully explained by regional cortical volumes, Einstein’s extraordinary cognition was related not only to his unique cortical structure and cytoarchitectonics, but also involved enhanced communication routes between at least some parts of his two cerebral hemispheres.

Thus Einstein’s cerebral hemi­spheres by comparing the morphology of his corpus callosum with that of 15 elderly healthy males and 52 young healthy males. We found that Einstein’s corpus callosum was thicker in the vast majority of subregions and that Einstein’s corpus callosum was thicker in the rostrum, genu, midbody, isthmus, and (especially) the splenium. These findings show that the connectivity between the two hemispheres was generally enhanced in Einstein compared with control The results of our study suggest that Einstein’s intellectual gifts were not only related to specializations of cortical folding and cytoarchitecture in certain brain regions but also involved coordi­nated communication between the cerebral hemispheres. Last but not the least, the improved approach for corpus callosum measurement used in this study may have more general applica­tions in corpus callosum studies.

~ From Brain a Journal of Neurology

The corpus callosum of Albert Einstein‘s brain: another clue to his high intelligence?

To explain the relationship between intelligence and the internal and external world of the individual; the analytical, creative, and practical comprise each sub-theory category respectively.

Understanding the nature of the components of intelligence is not alone sufficient to understand the nature of intelligence because there is more to intelligence than a set of information processing components. Sternberg held that we could scarcely understand what makes one person more intelligent than another, by only understanding the components of processing based on an intelligence test.  

For example, someone may be book smart but lack creativity and street smarts. 

Another person may be creative, but lack analytical and practical skills; another may be quite practical but lack the creative and analytical abilities others have.

By incorporating analytical, critical, and practical intelligence you can achieve successful intelligence.

Most intelligent people fail due to: a lack of motivation, a lack of impulse control, a lack of perseverance, a fear of failure, procrastination, the inability to delay gratification, and due to having too little or too much self-confidence.  

By incorporating analytical, critical, and practical intelligence you can achieve successful intelligence.

It’s little known that Einstein was an accomplished violinist, and even less known that had he not pursued science, he said he would have been a musician:

"I live my daydreams in music. I see my life in terms of music." said Einstein.

Music can be thought of as a complex stimulus able to enrich the encoding of an event thus boosting its subsequent retrieval. However, several findings suggest that music can also interfere with memory performance. A better understanding of the behavioral and neural processes involved can substantially improve knowledge and shed new light on the most efficient music-based interventions.

. 

Dr. Watson wrote in The Red Headed League: "Holmes was an enthusiastic musician, being himself not only a very capable performer of no ordinary merit." The detective enjoyed music of all kinds, including opera, concert music, and obscure compositions. His special devotion to music was clear from the number of references in the stories.

Watson mentions Holmes’ violin playing several times.

Music enhances our prefrontal cortex.Mind, was thought of as the complex of faculties involved in perceiving, remembering, considering evaluating, and deciding. The mind is in some sense reflected in such occurrences as sensations, perceptions, emotions, memory, desires, various types of reasoning, motives, choices, traits of personality, and the unconscious. 

Years ago there was little knowledge of the functions of

the subregions of the human prefrontal cortex. 

Most intelligent people fail due to: a lack of motivation, a lack of impulse control, a lack of perseverance, a fear of failure, procrastination, the inability to delay gratification, and due to having too little or too much self-confidence.

Being a bibliophile, loving knowledge, and having a chronic disease abliophobia I believe in esteemed Mr.Sherlock Holmes' words,

“I consider that a man’s brain originally is like an empty attic, and you have to stock it with such furniture as you choose. A fool takes in all the lumber of every sort that he comes across, so that the knowledge which might be useful to him gets crowded out, or at best is jumbled up with a lot of other things, so that he has a difficulty in laying his hands upon it. Now the skilful workman is very careful indeed as to what he takes into his brain-attic. He will have nothing but the tools which may help him in doing his work, but of these he has a large assortment, and all in most perfect order. It is a mistake to think that that little room has elastic walls and can distend to any extent. Depend upon it there comes a time for every addition of knowledge you forget something you knew before. It is of highest importance, therefore, not to have useless facts elbowing out the useful ones.”

My mental states and processes are supposedly made up of a different sort of stuff and take place in a different sort of realm. I disregard the view of this prevalent tendency to equate mind with brain, and the accompanying assumptions about the spatial boundaries of the human cognitive realm. My brain is naturally adaptive and the natural plasticity and unique brain's circuitry, partially busted makes it plastic par excellence and makes me endure anything and not be a victim but a Warrior 👸 Princess.

I am very stimulated. My mind never stops.

Do you have the nerves to control your guts?

 As a whole, the human nervous system operates in two modes: sympathetic, commonly known as ‘fight or flight’ when the body perceives a threat (stress), and parasympathetic, or ‘rest and digest’ mode. When the sympathetic mode is switched on, heart rate and breathing accelerate, and blood is directed toward the muscles for fast action, inhibiting digestion. In parasympathetic mode, we are relaxed and gastrointestinal secretion and motility increase.

As a whole, the human nervous system operates in two modes: sympathetic, commonly known as ‘fight or flight’ when the body perceives a threat (stress), and parasympathetic, or ‘rest and digest’ mode. When the sympathetic mode is switched on, heart rate and breathing accelerate, and blood is directed toward the muscles for fast action, inhibiting digestion.

 In parasympathetic mode, we are relaxed and gastrointestinal secretion and motility increase. 

The organs of digestion are served primarily by the Vagus nerve, one of the larger nerve networks in the body.

It’s divided into nerves in the submucosal layer that stimulate secretions and nerves deeper within the muscles of the gut which stimulate peristalsis. These nerves of course communicate with the brain through the central nervous system, but they are also rich in interneurons, or cross-links that enable the GI system to make most of its own decisions.

The cranial nerves associated with the swallowing process are the trigeminal (V), facial (VII), glossopharyngeal (IX), vagus (X), accessory (XI) - usually not considered - and hypoglossal (XII). It should be emphasized that the structures involved in the swallowing process are pairs, both anatomically and/or functionally, due to the dual-side innervation. Anatomically unique, the tongue, palate, pharynx, and larynx are functional pairs, each side having independent innervation.

From receptors on each side of the oral cavity, the trigeminal (V), facial (VII), and glossopharyngeal (IX) nerves conduct information to the brainstem. These mixed nerves lead sensitivity (afferent pathway) and motor command (efferent pathway). The afferent pathways of the anterior two-thirds of the tongue are supplied by the lingual nerve, which associates the trigeminal (general sensibility) with the facial nerve (taste). In the posterior third of the tongue, both the general sensibility and taste are conducted by the glossopharyngeal nerve.

The trigeminal nerve (V) has three branches; upper (ophthalmic), middle (maxillary), and lower (mandibular). The upper and medium are exclusively sensitive, and the inferior, mixed. The sensitive fibers of the three branches innervate the face in transverse bands of representation. Regarding the oral cavity, the middle branch (maxillary) has sensitive responsibility for the upper arcade teeth, upper lip, cheeks, hard palate (mouth mucosa), and mucosa of the rhinopharynx. The sensitive portion of the lower branch (mandibular) is responsible for the sensitivity of the lower arcade teeth and lower mucosa of the mouth, as well as by the general sensitivity of the anterior 2/3 of the tongue.

From the trigeminal ganglion to the brainstem, all the sensory pathways will end in the posterior portion of the brainstem, over the trigeminal sensitive nucleus that occupies the medulla oblongata (spinal tract nucleus of the cranial nerve V), the pons (main sensory nucleus of the cranial nerve V) and the midbrain (midbrain nucleus of the cranial nerve V). Centrally the sensitive fibers divide into short, ascending branches that end in the main sensorial nucleus, to attend to tactile sensibility, and into long, descending branches that serve to tact, temperature, and pain, also providing collateral pathways to the spinal nucleus of the cranial nerve V.

It is believed that proprioceptive fibers from the midbrain nucleus of the trigeminal nerve in synapse with its motor nucleus located in the upper portion of the pons, would be able to integrate important chewing reflex arcs. Unless expressly desired, these arcs allow reflex modulation of chewing intensity based on bolus consistency variations, even during the voluntary bolus chewing preparation.

The motor root of the trigeminal nerve emerges from the ventral portion of the pons and runs through the mandibular root to innervate the chewing muscles, the mylohyoid, the anterior belly of the diagastric, and the tensor muscle of the palate.

The facial nerve (VII) is a mixed one, considering its motor root in association with the sensitive root given by the intermediate (Wrisberg) nerve1. The taste of the anterior two-thirds of the tongue on each side are its responsibility. From the tongue, this afferent, pre-ganglionic route follows through the lingual nerve (association of nerves V and VII), and afterward through the tympanic cord nerve (facial branch), to make synapses on the geniculate ganglion. Through the intermediate nerve, the postganglionic fibers (afferent visceral special - gustative route) synapse in the solitary tract nucleus of the medulla oblongata, associated with the general afferent visceral fibers, providing sensitive innervation to the mucosa of the nasal cavities and soft palate.

The parasympathetic efferent fibers of the facial nerve, originating from the upper salivary nucleus located on each side of the upper portion of the medulla oblongata, run through the intermediate nerve and afterward through the tympanic cord nerve to make synapses in the submandibular ganglion. Thence, through postganglionic fibers, they stimulate salivary secretion of the submandibular and sublingual glands.

The motor portion of the facial nerve has its nucleus on the ventral portion of the pons. Its fibers stimulate the skin-inserted muscles in the face, neck, and scalp, as well as the posterior belly of digastric and stylohyoid muscles.

The glossopharyngeal (IX) nerve comes out of the skull together with the vagus (X) and accessory (XI) nerves. The visceral general afferent and the visceral special afferent fibers of the glossopharyngeal nerve are associated. The visceral general afferent fibers are responsible for the general sensitivity of the oropharynx mucosa and the posterior third of the tongue, and the special visceral afferent fibers, for the taste of the posterior third of the tongue. These preganglionic fibers make synapses with the upper ganglion. The postganglionic fibers will end at the solitary tract nucleus.

The glossopharyngeal nerve’s efferent pathways come from two distinct nuclei of the medulla oblongata, the salivary inferior (parasympathetic) nucleus and ambiguous motor (special visceral efferent) nucleus. The parasympathetic fibers stimulate the salivary secretion after synapses with the optic ganglion, from which postganglionic fibers emerge to innervate the parotid gland1,29,31.

The glossopharyngeal nerve’s only motor role is with the stylopharyngeus muscle. Nevertheless, it has already been considered as motor to the superior pharyngeal constrictor muscle, whose activity had been previously attributed to the vagus nerve, responsible for the motor innervation of all pharyngeal constrictor muscles.

The vagus (X) nerve has relationships extending from the cervical region to the abdomen (transverse colon). Its sensory afference (sensory pathway) connects with the solitary tract nucleus located in the medulla oblongata. The visceral special efference (motor pathway) comes from the ambiguous nucleus in the ventral region of the medulla oblongata, and the parasympathetic fibers (visceral general efference), from the dorsal motor nucleus of the vagus.

Motor neurons that direct digestive gland activities (secretion) and smooth muscle contraction (peristalsis)     Some of the neurotransmitters at work in the ENS include acetylcholine, norepinephrine, GABA, serotonin, substance P, and vasoactive intestinal peptide. We recognize some of these also as hormones, and communication chemicals important also in mood, stress, and immune response.     Hormones orchestrate much of homeostasis, or the maintenance of balance in the human being. This is quite a complex feat, and it’s the balance, or relationship of hormones to one another that facilitates equilibrium. Hormones from many different glands, including the kidney, heart and organs not usually associated with the endocrine system, all function in relationship to one another, for example high levels of some feed back to reduce levels of others. That’s how something like stress can impact other hormonal functions, like a late menstrual period or even changes to one’s cycle.

There are two ways that the condition of the gut tissue impacts, and can be impacted by the condition of the rest of the body. One is reflexive action – because the GI tract arises from the same cells in the embryo as respiratory and urinary tissue, they are all linked.

Where there is tension, stagnation, weakness or hypersensitivity become generalized throughout the body. In this case, normalizing the overall tissue state will address both nervous and digestive systems, along with the rest of the body. For example stimulating sluggish digestive, liver and circulatory function will also stimulate better, quicker nerve and hormonal communication.

Because digestion is controlled by both nervous and endocrine sytems, both can impact how well our GI tract functions. It’s well known that stress is positively correlated with several GI conditions, including ulcers, irritable bowel syndrome/spastic colon and inflammatory bowel diseases including ulcerative colitis. Especially chronic stress promotes the body’s secretion of inflammatory mediators – communication chemicals that the ENS recognizes that can initiate or exacerbate inflammation.     The state of the digestive organs and their function can also, conversely, affect one’s mood. For example, stagnation in the gut—gas, bloating, constipation – can lead to feelings of gloom and depression. Irritation to the intestines, from food allergens, junk foods or poorly digested food, can result in irritability of the emotions as well. 

The visceral special afferent (taste) and visceral general afferent (sensibility) pathways of the vagus nerve, after synapses in a peripheral ganglion (lower or caudal), have their postganglionic fibers end at the solitary tract nucleus, similar to that observed in the intermediate portion of the facial nerve and in the glossopharyngeal one. The visceral general afferent fibers conduct impulses related to the sensitivity of the pharynx, larynx, trachea and esophagus, and the visceral special afferent route lead taste stimuli from receptors on the vallecula and from a small posterior area of the tongue next to the vallecula.

The visceral general efferent (parasympathetic) fibers of the vagus nerve originate in the vagus dorsal motor nucleus, and from it, on each side, they gather in a single-trunk, descending pathway, emitting branches in the cervical, thoracic and abdominal region, where they end. These preganglionic fibers will establish synapses in peripheral ganglia of the parasympathetic vegetative or autonomous nervous system, close to, or even inside, the viscera walls.

The visceral special efferent (motor) fibers of the vagus originate in the ambiguous nucleus, and are responsible for innervation of the striated muscles of the pharynx, larynx and esophagus.

The Hypoglossal (XII) nerve, a motor one, has an individualized nucleus on the ventral-medial portion on each side of the medulla oblongata. It is responsible for the tongue extrinsic and intrinsic muscles. In addition, fibers from the cervical plexus in association with the hypoglossal nerve form the ansa cervicalis, from which a branch from the cervical plexus, usually C1, will innervate the geniohyoid muscle, one of the responsible for the hyoid-laryngeal displacement.

The pharyngeal plexus (glossopharyngeal, vagus and accessory though vagus) is considered responsible for the pharyngeal reflex phase, where afferent information from the pharynx reach the brainstem, generating efferent stimuli to the pharyngeal structures involved in this phase of the swallowing process.

The pressure transfer from the oral cavity to the pharynx by distention would produce afferent stimuli that would reach the brainstem, in special the sensitive (solitary tract) nucleus. From the sensitive nucleus, through interneurons of the reticular formation, the ventral motor (ambiguous) nucleus of the brainstem generates efferent motor stimuli to the pharyngeal structures. Several structural movements initiated during the voluntary oral phase, remain in progress until the end of the pharyngeal phase, such as hyoid-laryngeal elevation, swallowing apnea and tongue posterior projection, to pharynx, started during the oral ejection, without considering the palate tension produced by the trigeminal nerve. In this way, several elements of the oral phase incorporated by the pharyngeal reflex phase allow us to consider the pharyngeal phase as dependent on the cranial nerves V, VII, IX, X, XI and XII of both sides.

To command your guts you need

•  Carminatives are aromatic herbs containing volatile oils. These small molecules help relieve gas, tension and spasm. They help the digestive process function smoothly and with ease. 
•   Bitters are stimulating to the secretions of stomach, pancreas, liver and small intestines, and increase our ability to digest and assimilate our food.
• Demulcents are slimy, mucilaginous herbs. They are made up of branched carbohydrate  molecules that easily trap water and other molecules, causing them to swell and become like soft sponges. These herbs are very soothing to the gut walls and the nerves.
• Nervines are relaxing herbs that are often tonic or supportive to the nervous system.

Carminatives are used when there is gas, irritability, fluctuating stool frequency and/or consistency, bloating, cramps or flatulence.

Bitters are used when there is indigestion, heartburn, poor fat digestion or constipation.

Demulcents are used  when any of the GI tract mucous membranes are irritated, ulcerated or inflamed – from the throat on down, and for watery stools.

Carminative nervines - lemon balm, chamomile, lavender, hawthorn, rosemary, valerian, peppermint, hyssop, celery seed

Carminative bitters – turmeric, angelica, elecampane, wormwood, mugwort

Bitter nervines – motherwort, blue vervain, hops, skullcap

Demulcent nervines – linden, oats Other demulcents – marshmallow, slippery elm, plantain, flax

Beneficial Herbs for Digestion

Our DIY digestive bitters recipe uses the following beneficial herbs:

ANGELICA. Angelica is a stimulant, which aids in gastrointestinal secretions, absorption, and elimination. It relieves gas and the feeling of fullness. (Avoid if pregnant).

GENTIAN. Gentian is a simple bitter that has been used for over 3,000 years to revitalize the gastrointestinal tract by optimizing levels of stomach acid and digestive enzymes. Gentian is especially helpful for protein and fat digestion. (Avoid if pregnant or on H2 receptor antagonists, like Zantac, or other antacids).

DANDELION. Dandelion is high in nutrients and is bitter, which stimulates the cascade of digestive secretions. Dandelion also has a significant cleansing effect on the liver by stimulating bile production, which is important for fat digestion.

GINGER. Known as the “universal medicine,” ginger is warming and carminative, which means it’s great at relieving bloating and gas. Ginger also supports healthy bowel movements.

World Health Day

                                     

                                   When I look back at all I've been through

wars and horror I've endured

With a happy smile underneath the sky so blue

I fight my malady which has no cure

I think of the willfully ignorant world that the same trouble I face with vivacity

Might befall them someday 

I wonder when they face the same will they run away?

An old man with failing health was once a stout man

Similarly, the hell inside of me

I face every hero, philosopher, demon 

As I gain skills to fight demons with procedures or surgery

With pluck, and luck

Spirit and with

I fight every myth

As Mum stays with 

Me giving company

I have faced horridest days

On this ordinary day

Only the rich can stay

Wise, wealthy, and healthy

Tell me if nobody would help me

 Why is today world health day?

It should come when authority

Offers help good and gay 

To stay active, pain-free, and healthy 

Yet here, I am surviving

Because I am meant to 

When everyone's treatment is the same regardless of the socioeconomic position or age there will be justice.

 I am not Benjamin Button with a rare ageing ailment that makes the baby born begin life as an old man and proceed to age backwards. 

It's a ghastly joke of fate along with all the coexisting rare diseases the Creator forgot to add this one. If he had an alert mind he would have done so and I would have benefited from NATIONAL POLICY     FOR     RARE DISEASES, 2021 where only those diseases diagnosed as a baby or child are included and only those who don't look smart are denied but funny are accepted. I never had any delay in intellectual and physical development. I would have never been chosen. Everything is inside. My missing liver which has been stitched back with a replacement; my left kidney, part of which is gone forever; my brain which has been burnt by radiation beams. resulting in an ischemic brain and the tumours scattered inside sparkling like a star punched sky yet the "little grey cells" had worked out an exceptional detective book during lancinating pain with one-eyed vision and typing with the index finger of the right hand because after the hand got palsy and was treated with high-dose steroids the hand doesn't work like before and the left hand after recovering from paralysis didn't get back the sense of touch and both halves of brain lost coordination and refuse to work together. Also writing for Times of India digital without honorarium.

I can work out more books and encouraging posts for society if my compressed nerves are restored into functional mode.

 

NPRD is a game of charade. This newfangled policy doesn't seem fair.

In their policy, all diseases to get support chosen by the committee and the ministry are pediatric diseases and prevalent in children. 

I was once a child because I am not Benjamin Button. I grew up suffering all the time from a variety of symptoms and fatal diseases.

Adulthood diseases are only

• Osteoporosis

• Tyrosinemia - a metabolic disorder

• Pompe disease - a lysosomal disorder in juvenile/adult form

• GSD may appear in adults

All diseases are metabolic disorder or fall under lysosomal storage disease

This is a loathsome distinction.

"Support those interventions that would provide more  number of healthy life years for a given sum of money while simultaneously looking at the  equity i.e., interventions that benefit poor who cannot afford healthcare are prioritized."

The idea is that you take the cost of any treatment and then calculate not just how many  lives it saves, but the quantity of life it saves which is absurd. ( This looks like a business strategy)

The beautiful simplicity of the ideas astounds me. Getting only one-time treatment for little ones, lifetime or long-term treatment and treating them cheap.  

We are poor and abandoned with only Rs 7000 mother's pension which is spent mostly on food and my mother's health needs as she is almost a septuagenarian and the nagging worries about rent, electricity and water bills, food and then medicines with surveillance and doctor's fees and conveyance charges. My personal income is almost nil after my rare thoughts and manuscript of a good book " Adventures of Mum and Princess" - an anthology detective stories was stolen by the owner of 3 English speaking schools for the purpose of syllabus of classes 8, 9,10. I wrote this book at the peak of pain of Trigeminal Neuralgia.

My personal income never was more than Rs2000. Under current situation it is further reduced. I beg from Facebook acquaintances and some say " will send you in a few days" but that never happens

 Health authorities offered a policy with negotiated contracts where hospital becomes a shop, all kinds of treatment becomes a piece of merchandise, and normal business practices prevail : pile 'em high and sell' em cheap because diseases treated by enzymes or food are only chosen.

They don't understand when medicine is socialized, then you have true health care. When everyone's treatment is the same regardless of the socioeconomic position or age there will be justice. Otherwise, there will always be a tormenting distress about someone somewhere else getting what you are not.

This newly minted policy is an irony that the government is taking care of handful of citizens.

This is not helping, but  swamping those who are rare and need to fight daily for life, not just recuperate but also keep thinking about how to live, how to arrange food, medicines and a roof above the head and how to be relatively healthy. It's tragic that the government thinks "life and health of a citizen an unfair burden". 

Government support for those listed in the groups with only 20 lakhs, compels me to say this government does not value human life.

It is unfair even for those chosen.

Disorders that are amenable to other forms of therapy (hormone/ specific drugs)

i) NTBC (Nitisinone) for Tyrosinemia Type 1  --₹ 50,000/ Vial-manufacturer -Roche- on demand

ii) Osteogenesis Imperfecta –  $100- $200 for Bisphosphonates therapy

 iii) Growth Hormone therapy for proven GH deficiency, Prader Willi  Syndrome and Turner syndrome, Noonan syndrome. -cost for a 20 kg child would be Rs 200,000 per year.

 iv) Cystic Fibrosis- Pancreatic enzyme supplement  -The cost of enzyme replacement treatment was US$ 6881,63 ± 2334,04/year; US$ 6778,19 ± 2339,26/year in the tumor group; US$ 7096,78 ± 2356,17/year in pancreatitis group.

 v) Primary Immune deficiency disorders -Intravenous immunoglobulin -Bharglob 16.5% Inj. 2ml    Serum 200 International    and  sub cutaneous therapy (IVIG) --$5736 per first 3 years of therapy replacement eg.   etc.

vi) Sodium Benzoate( ₹ 300.00) , arginine (₹ 1,699.00), citrulline (₹ 2,500.00), phenylacetate --₹ 1,000/ Kilogram (Urea Cycle  disorders), carbaglu (Rs 80000/kg), Megavitamin therapy--Rs 8000- Rs 18000, says Dr. Mittal(Organic acidemias,  mitochondrial disorders)  

vii) Others - Hemin --costs British pound sterling 1125 for 4 x 10 ampoules.(Panhematin-313 mg is around $7,558) for Acute Intermittent Porphyria, High  dose Hydroxocobalamin injections- 5.35 Euro(30mg/ml formulation – not  available in India and hence expensive if imported)

  viii) Large neutral aminoacids( ₹ 1,189.00), mitochondrial cocktail therapy ($1,000 per month),  Sapropterin (Rs 4000/bottle) and other such molecules of proven clinical management  in a subset of disorders

Based on the literature sufficient evidence for good long-term outcomes exists for  the following disorders 

 

     1. Gaucher Disease (Type I & III {without significant neurological impairment}) 

Gaucher disease is categorized as a lysosomal storage disorder (LSD)

Enzyme replacement therapy for Gaucher’s disease, which is available in India, costs about Rs. 40 lakh to Rs. 1 crore a year, depending upon the weight of the child.

 2. Hurler Syndrome [Mucopolysaccharisosis (MPS) Type I] (attenuated forms)  

MPS I is member of a group of hereditary metabolic diseases known as the mucopolysaccharidoses which, in turn, are part of a larger group of diseases known as lysosomal storage disorders (LSDs) - 46 lakhs approximate annual cost per 10 kg child

3. Hunter syndrome (MPS II) (attenuated form)- 1 crore approximate annual cost per 10 pkg child

  4. Pompe Disease (Both infantile & late onset diagnosed early before  development of complications)  This disorder belongs to a group of diseases known as lysosomal storage disorders. 49 lakhs approximate annual cost per 10 kg child

 5. Fabry Disease diagnosed before significant end organ damage.  Cost of ERT- 20 lakhs approximate annual cost per 10 kg child(This disorder belongs to a group of diseases known as lysosomal storage disorders.)

6. MPS IVA before development of disease complications.- 1.3crores approximate annual cost per 10 pkg child

7. MPS VI before development of disease complications.- 1.1 crores approximate annual cost per 10 pkg child

To almost all of them it will not suffice ( expenses are an approximate value)

What is required

Financial support upto Rs. 20 lakh under the Umbrella Scheme of Rashtriya  Arogaya Nidhi shall be provided by the Central Government for treatment, of  those rare diseases that require a one-time treatment (diseases listed under  Group 1) is Disorders amenable to one-time curative treatment:

Disorders amenable to treatment with Hematopoietic Stem Cell Transplantation  (HSCT) –

Disorders amenable to organ transplantation

I had a liver transplant give me back my money along with expenses of surveillance. 

State Governments can consider supporting patients of such rare diseases that  can be managed with special diets or hormonal supplements or other relatively  low cost interventions (Diseases listed under Group 2) is ridiculous.

Keeping in view the resource constraints, and a compelling need to prioritize the  available resources to get maximum health gains for the community/population,  the Government will endeavour to create alternate funding mechanisms through    setting up a digital platform for voluntary individual and corporate donors to  contribute to the treatment cost of patients of rare diseases. 

Well, then Coca-Cola, IAM, Being Human, Tata trusts, Hans Foundation etc should support for life without delay. But I am certain this would never happen because Director and ambassador of Coca-Cola was in school with me, Tata trusts vaguely denied in 2020, IAM helped me once but now according to them they only help children, Being Human is unreachable, Hans Foundation approved my cyber knife but then denied because cost was very high 5 lakhs in 2020.

These are useless ideas.

What the close-fisted government ought to do is

Like Mexico's legislation for rare disease that authorized Seguro Popular, a national health insurance, this ought to be done in this country for all ages but not for a handful of diseases and amount ought to be enough to meet the expense of every patient after calculating every expense of surgeries, procedures, surveillance, medicines.

The plight of rare disease patients with caregivers is known to doctors and the government.

It should be acknowledged the needs of everyone touched by rare diseases and provided all factors of care.

We need a plan to better coordinate efforts by governments  involved in addressing the  challenges and ensuring that all people with rare disorders across the country can enjoy the same timely and high-quality health and social care as patients with more common diseases.

A clear personal care plan for every patient that brings together health and care services.

• Making sure patients, their families and carers have the information they need, are listened to and consulted.

• Developing better methods of identifying and preventing rare diseases.

• Improving diagnosis and earlier intervention for those with a rare disease.These days diagnosis is improved in my opinion. I could find a lot of Indian research done. 

According to Dr. Rajiv Sarin they have found 40-50 VHL patients in Tata Memorial. 

• Books are written on Trigeminal Neuralgia by AIIMS doctors.
•  Hypoparathyroidism is an uncommon disorder of calcium metabolism characterized by hypocalcemia, hyperphosphatemia, and reduced level of intact parathyroid hormone (iPTH).

Indian journal of anaesthesia and Indian journal of endocrinology and metabolism contains few (10)such articles where I see in Army hospital parathyroid injections are available not for civilians

• Better education and training for health and social care professionals.

After several misdiagnosis and diagnostic dilemmas in 2008  I was diagnosed with a very rare disease hardly known in India, called VHL or von Hippel Lindau during my landmark liver transplant. I can still recall the faces of the radiologists checking my tumour studded liver with their probes and looking at my file with a weird name “VHL” with vacant faces and regarding me quizzically. Yes, the liver transplant was a life and death affair and we had no money to get the liver transplant done which was a whopping> 30 lakhs!

Therefore I believe there's already enough awareness amongst health care professionals but not those who run NGOs amongst common people. Few can't distinguish between immunosuppressants and immunotherapy!

• Building on research to improve personalised approaches to healthcare for those with a rare disease. 

NPRD gently and politely smashed hopes for continuing my treatment by choosing a handful of diseases by foretelling and quantifying the quality of human life.

Patients with rare diseases like me received a misdiagnosis from multiple physicians. Ultimately was diagnosed during a liver transplant. Considering a bacterial infection Tuberculosis I was misdiagnosed and underwent treatment in vain for 2 years until proper medicines were given by Dr Randeep Guleria. History repeats and once again was misdiagnosed going around all hospitals in Delhi when Prof HariHara Dash confirmed bilateral TN. But due to this misdiagnosis I suffered pain in teeth and going to the dentist under antibiotic coverage got 105 F fever and 3 lymph nodes stood out in the chin.  This delay in diagnosis adds to the costs of the disease incurred by my parents. Doctors need to know more about diseases like Trigeminal neuralgia and MDR TB.

• Serious health priority patients with rare diseases should have equitable access to effective services. By bringing in a lasting change offering better health and quality of life for individuals and families affected by rare diseases would lead to a positive change. By ensuring that patients and families living with rare conditions have equitable access to high-quality services, treatment and support.

• Can this orphan drug be imported for me?

https://www.drugs.com/cdi/sirolimus-oral-solution.html

• To improve the quality of life of patients with rare diseases, as follows:

  • Subsidize patients by reducing the burden of medical expenses
  • Provide information about rare diseases
  • Help regional patients by reducing indirect expenses

This is a very rare thought, not asking the patients to beg and die eventually.

Not a mockery like NPRD.

Though in the last two decades, due to advancement in technologies, understanding of  the pathophysiological mechanisms of rare genetic disorders has somewhat improved,  yet the treatment modalities are few and the available therapies may not lead to “cure’. Here I think I have heard AIDS doesn't have cure for almost half a century but gets support and rehabilitation.

About "clinical trials and studies." I might say I have been into studies where with my knowledge I can preserve my life and there have been several studies cited in Part 1.

Central nervous system hemangioblastomas are cardinal feature of VHL syndrome and occur in 60-80% of VHL patients, with cerebellum being the most common 

site . VHL syndrome associated hemangioblastomas frequently expresses SSTR . Ambrosini et al.  have previously demonstrated in vivo SSTR expression in VHL associated hemangioblastoma with 68Ga-DOTANOC PET-CT. In the present case 68Ga-DOTANOC PET-CT detected previously unknown cerebellar hemangioblastoma, which was confirmed on contrast enhanced MRI. Retinal angiomas (hemangioblastoma) are the most common presenting feature of VHL disease as was in the present case, though not recognized at that point of time. 68Ga-DOTANOC PET-CT detected the retinal lesions and were subsequently confirmed with MRI. To our knowledge, there is no previous published report of imaging retinal hemangioblastoma with 68Ga-DOTANOC PET-CT.

~ Von Hippel-Lindau Syndrome: Demonstration of Entire  Disease Spectrum with 68Ga-DOTANOC PET-CT Punit Sharma, MD, Varun Singh Dhull, MD, Chandrasekhar Bal, MD, Arun Malhotra, PhD,   Rakesh Kumar, MD, PhD All authors: Department of Nuclear Medicine, All India Institute of Medical Sciences, New Delhi 110029, India

This is the most important study because it establishes my leptomeningeal hemangioblastomas.

Increased levels of growth factors lead to angiogenesis. While HIF-1 a inhibits cell growth it is HIF-2a that drives tumour progression. In RCC there is hypoxia associated factor which drives increased levels of HIF-2a. They play a crucial role in tumour promotion in other cancers including breast, brain, colon, gastric, lung, skin, ovarian, prostrate, renal and pancreatic.

ICMR has funded research on VHL and is well aware HIF is involved in GBM (Glioblastoma can be difficult to treat and has a high recurrence rate. Treatments may slow down the progression of cancer and reduce signs and symptoms. Glioblastomas are often incurable and fatal too).

I am citing an example where it says cure isn't very far away

Here I may say “Von Hippel-Lindau disease is a rare genetic condition for which there is no systemic treatment option available and is associated with a high risk of cancer development in multiple organs. In fact, up to 70% of patients with VHL develop renal cell carcinoma during their lifetime,” said Scot Ebbinghaus, MD, vice president, clinical research, Merck Research Laboratories, in a press release.

MK-6482 is a selective inhibitor of HIF-2α, which is a protein that can accumulate in patients when VHL is inactivated. Without regulation, this accumulation can cause the stimulation of several oncogenes associated with cellular proliferation, angiogenesis, and tumour growth.

https://www.pharmacytimes.com/view/fda-grants-mk-6482-nda-priority-review-for-von-hippel-lindau-disease-associated-rcc

Mutations of VHL genes are also part of causes of other tumours including breast, colon etc.

VHL is the key to understanding how tumors grow and how potential theories can inhibit angiogenesis.

According to Dr. Rajiv Sarin they have found 40-50 VHL patients in Tata Memorial.

I feel sorry there's no advocacy group for such a serious and high priority disease.

Mr. Narendranath Vikkath, biotechnologist and lecturer at Amrita institute of medical sciences said,

"In India we don’t have data i guess. May be you can get individual case reports"

Other names of VHL gene

• elongin binding protein
• pVHL
• VHL1
• VHL_HUMAN

von Hippel-Lindau tumor suppressor, E3 ubiquitin protein ligase

When the VHL protein is altered or missing, the VCB-CUL2 complex cannot target HIF-2α and other proteins to be broken down. As a result, HIF can build up in cells. Excess HIF stimulates cells to divide abnormally and triggers the production of blood vessels when they are not needed. Rapid and uncontrolled cell division, along with the abnormal formation of new blood vessels, can lead to the development of cysts and tumors in people with von Hippel-Lindau syndrome.

"Considering the limited data available on rare diseases, and in the light of  competing health priorities, the focus would be on prevention of rare diseases as a  priority for all the three groups of rare diseases identified by Experts.  Public Health and  hospitals being a State subject, the Central Government would encourage & support the  States in their endeavour towards screening and prevention of rare diseases through  Centres of Excellence under Rare Disease Policy and Nidan Kendras under Department of Biotechnology."

- Unless new fields are explored how will knowledge increase. I have leptomeningeal HB due to the fault of the doctor during a surgery. How will he realize? I got back from the jaws of death in the debridement but until the doctor had sudden doubt something had gone wrong and called his senior how would he have known? Instead of being a doctor he would have turned out to be a killing machine.

Nidan Kendra will be effective for the generation after the next.

Even if there's no cure they are treatable and treatment increases quality of life so why not help to get treatment. Focussing on treatment which is most effective is better idea.

Tobacco increases propensity to TB can you stop Shah Rhukh Khan from being a chain smoker else he will end up with TB so day. Can you predict that?

ICMR has funded research on VHL and is well aware HIF is involved in GBM (Glioblastoma can be difficult to treat and has a high recurrence rate. Treatments may slow down the progression of cancer and reduce signs and symptoms. Glioblastomas are often incurable and fatal too).

There's a good chance of getting old with silver hairs if my venerable disease gets an opportunity for good treatment, my potentials are realized. 

" Thus, interventions that address health problems of a much larger number of persons by  allocating a relatively smaller amount are prioritized over others such as funding  treatment of rare diseases where much greater resources will be required for addressing  health problems of a far smaller number of persons."

There are many NGOs in our country who care about orphans, child education, farmers but no one cares for a rare disease patient. Most of them prefer to help on a mass scale to get their names printed on the front page of the leading dailies. Certain individuals become "God-like" but if you try to contact them it's bogus...the general mentality of the country.

Unless pVHL is studied a solution to cancer will never be found.

"Enable access to affordable health care to patients of rare diseases which are amenable to one-time treatment or relatively low-cost therapy."- This won't work, rare disease treatment is mostly expensive and " EQUALITY of status and of opportunity;" from the preamble vanishes into thin air.

"Premarital, post-marital, pre-conception, and post-conception screening", this is supposedly not going to work in a country with a population of over a billion people where you can't explain why the citizens should wear a mask, why there's a need to maintain a distance during a pandemic despite attempts to video shoot aerosols because even educated individuals are ignorant. If any couple wants to marry and have a child they will defy any rule and do so even if marriage and birth certificates aren't given. The population is huge and it's difficult to make them understand.

Instead of that, strategies like: government may create laws aimed at reducing the occurrence of rare diseases that have a preventable cause, such as neural tube defects (NTDs). In 1998, a regulation issued by the US Food and Drug Administration (FDA) went into effect that mandated the addition of folic acid to cereal grain products labelled as enriched in the US. The objective of this regulation was to provide women with an avenue for increasing dietary intake of folic acid, which can help prevent NTDs. As a result of this regulation, just over 1,300 more babies were born without a NTD each year from 1999 through 2011. To date, mandatory folic acid fortification of grain cereals now exist in 86 countries. Can also create awareness.

Reflecting the needs of the rare disease work of the government should be to carry out activities to capture the experiences of those living with a rare disease. This includes conducting surveys of patients and families, consulting with a wider audience on the issues that affect them, to gather evidence and collect opinions.

Voluntary crowd-funding for treatment

Human beings are no longer humane and they don't work hand in hand now and if you ask twice get irritated. They love a lavish life and collecting money. Therefore crowdfunding is an outrageous thought and every platform, Milap, Ketto has their own charges unless you collect a large amount it's useless. The decayed society with affluence. Crowdfunding for 5 lakhs becomes difficult for cyber knife of Trigeminal Neuralgia and when someone donates from abroad gateway fee etc by RBI deducts most of the sum.

The responsibilities and activities of the COEs , I hope these government hospitals will bring a cyclotron or synchrotron in a few days, for those who are in great shape it's okay to wait but for those who are progressing towards the advanced stage need treatment immediately.

All which is needed to lengthen the period and quality of life is a good doctor who is thinking about your health and happiness and not treating you like a guinea pig and not working for his own good and fame. I got the opportunity to choose between good and better doctors four times in my life, during my liver transplant- who didn't say transplanting the liver will cause the tumours to grow once again and it will be a futile effort;

Payel Bhattacharya with Padmasree Dr.Arvinder Singh Soin

MDR TB treatment-cured by Dr Randeep Guleriaa  which other doctors couldn't treat for 2 years at Medanta and because those doctors failed because of whom I can't walk properly as I had bone TB and I still need the help of a stick to walk;

Payel Bhattacharya with Director and Prof. Dr Randeep Guleria at AIIMS

my kidney cancer- who understood before taking out the cancerous tumour in kidney doing fine needle biopsy could spread cancer to other parts of the body

Dr Sanjay Gogoi

and trigeminal neuralgia-understood risks of MVD surgery on a leptomeningeal hemangioblastoma patient.

Payel Bhattacharya with Dr.Sankar Vangipuram and team before cyberknife for Trigeminal Neuralgia at HCG Khubchandani Cancer Center Colaba Mumbai.

Perhaps the medical community and the country needs to hear your thoughts, experiences and suggestions in order to change for the better. Instead of accepting whatever the oppressors decide about your life, and health your voice needs to be heard.

To make voices heard

Pluck up the nerve and get together and say where the commodity involved is human life - could never be quantified, quality of life is something you could not put a price on the right to life and healthcare is a fundamental right. Where quality adjusted life years is a pathetic policy, to bring in change you have to be the change.

• Patients should come together, represent the views of the rare disease community in key political debates, making sure that your interests are represented on the broad political issues that affect you on the issues that matter to you.

• Government should improve the lives of those affected by rare diseases. 

• Patients enable them  to raise awareness. Building up through interactions with patients, families, and treating doctors have more knowledge.

• The more we can together raise our voice the greater the likelihood that it will be recognised as a serious health priority and patients and families living with these conditions will have equitable access to effective services. Help us to build the pressure that will lead to positive change!