Posts Tagged Lou Gehrig’s Disease

“The Theory of Everything” – Movie Review

In 1960s, a brilliant young man introduces himself as a cosmologist, to the girl who later becomes his wife.  When she asks what it means, he says, “it is a kind of religion for intelligent thinkers”.  And there begins the true story of one of the most brilliant thinkers in history, Stephen Hawking (Eddie Redmanyne) and his sweetheart, wife and mother of his children, Jane Wilde (Felicity Jones).

Right before Jane and Stephen got married, Hawking was found to have motor neuron disease (also called amyotrophic lateral sclerosis or ALS or Lou Gehrig’s Disease), and received prognosis of two year survival (see my article  on possible novel treatment for ALS). Jane married Stephen in 1963, despite being warned that the “weight of science was against her”.  In a race against time, Stephen, with Jane’s help, started working on science.  Stephen is miraculously still alive today and is in his 70s.  In their life’s journey, Stephen and Jane encountered many trials and tribulations, became parents of three children and are now grandparents.  They married, they loved deeply, they eventually divorced, and they continue to remain friends.

English: Stephen Hawking giving a lecture for ...

English: Stephen Hawking giving a lecture for NASA’s 50th anniversary (Photo credit: Wikipedia)

Interwoven with the great love story is the story of science which began with Hawking’s search of a “single unifying equation that explains everything in the universe”.  In his earlier work, Hawking developed detailed mathematical models to explain the property of black holes from which nothing escaped.  But later, explaining from combined general theory of relativity and quantum mechanics, he opined that black holes are not black, after all.  Hawking explained that these so called black holes glow with “heat and radiation and eventually everything blows up in a spectacular explosion”.

Jane explains Quantum theory, versus Relativity theory with the help of peas and potatoes, to Jonathan Hellyer Jones (Charlie Cox), a family friend.  Hawking had begun revising his thinking.  He had always been an atheist and in the conversation with Jonathan, Hawking explains, quantum theory by itself was falling short in the explanation, “so God is once again on endangered species list and physics is back in business”.  With his “no boundary theory”, Hawking explains in his new book “The Brief History of Time”, that time and space would have no boundary or edge and the laws of science would hold everywhere, including at the beginning of the universe.

Jonathan becomes a close family friend and eventually, he and Jane develop feelings for each other.  Also Hawking develops a new relationship with his therapist, Elaine (Maxine Peake).  During a visit to Geneva, Stephen contracted pneumonia and only survived with a tracheotomy, which also resulted in him losing his speech entirely.  Even though Stephen and Jane go through many challenges that take a toll on their 25 year old marriage, it is clear that their relationship was not only built on deep emotional love but it was a meeting of the minds; their conversations provided intellectual stimulation and they got creative insights from togetherness, in their space and time.  This movie, adapted from Jane Hawking’s book, “Travelling to Infinity: My Life with Stephen”, celebrates their incredible relationship.

Hawking is indeed a remarkable human being.  He is incredibly brilliant and he has defied all odds and beaten all predictions made about his disease and quality of life.  More importantly, the woman who stood by his side and enabled his most meaningful contributions in the field of science, also added richness and meaning in his personal life.  Although Hawking continues to remain an atheist, he seems to have become mellower and more reflective.  Recently , he mentioned through social media that he was moved by watching the movie and it gave him an “opportunity to reflect” on his life.  In the movie, towards the end, Hawking was asked if he had a philosophy for life and he replied, “just as universe has no boundary, there is also no boundary to human endeavor; where there is life, there is hope.

Director James Marsh has done a brilliant job of capturing the complexity of Jane and Stephen Hawking’s beautiful relationship, of Stephen’s disabling body, and a brilliant mind, of wit and humor with which he approaches life.  The movie does not overplay the pitiable aspects of the devastating disease and balances the shadow of the disease in Stephen and Jane’s relationship, with love and romance in their life. From a young man in love, to a man who is slightly clumsy at the onset of ALS, to a man who ages, whose marriage is getting old, and who struggles through the progression of the deadly disease, Radmanye, who lost 15 lbs. for the role, met 40 ALS patients, trained with a dancer to control his body, and stood in front of the mirror for hours contorting his face, gives an absolutely incredible performance.

And while the movie focuses on the everyday aspects of Stephen and Jane Hawkins’ life, family, and children, it also provides some powerful metaphors (e.g. peas and potatoes, wind back the clock), so characteristic of Hawking, to shed light on his theories.  Unfortunately, the film only provides a drop from the vast sea of science produced by Hawking.  What I would have loved to see is more science and more explanation of significance of Hawking’s contributions.  This still remains a fantastic movie about the life of two amazing people and on my scale of 1-5, with 5 being excellent, I give it a rating of 4.8.

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Treating ALS – The Story of Neuraltus

Rich Casey, President and CEO of Neuraltus (formerly Chairman and CEO of Scios), talked about treatment option for ALS (Amyotrophic Lateral Sclerosis), also known as Lou Gehrig’s Disease, a fatal neurodegenerative disorder of unknown etiology, at event.

Typically, the age of onset of ALS is anywhere between 40 and 70 years.  Life expectancy after the diagnosis is between 2 and 5 years, although in very rare cases (e.g. in case of Stephen Hawking), it could be an extremely slow progression.  No effective treatments for the disease exist.  Motor neurons degenerate and early symptoms include muscle weakness, specifically involving arms and legs, lack of hand grip, deep tiredness and it progresses to difficulty in swallowing, breathing, and garbled speech.


Macrophage (Photo credit: Wikipedia)

Neuraltus’s lead product,  NP001 is in clinical development, targeted for the treatment of ALS.  While the exact cause of the disease is unknown, there is fair amount of evidence that points to increasing levels of inflammation in the macrophages.  This increased inflammatory activity results in the release of factors in the central nervous system that leads to damage of motor neurons.  Macrophages are cells produced by differentiation of monocytes in the tissues.  Macrophages are highly specialized in removal of dead cells or debris.  Additionally, macrophages also “present” antigen that plays a crucial role in initiating an immune response.  There is an increasing evidence implicating neuroinflammation with the progression of ALS.

English: Amyotrophic lateral sclerosis MRI (ax...

English: Amyotrophic lateral sclerosis MRI (axial FLAIR) demonstrates increased T2 signal within the posterior part of the internal capsule, consistent with the clinical diagnosis of ALS (Photo credit: Wikipedia)

Neuraltus’s novel, proprietary drug, NP001 regulates macrophage activation and converts the activated inflammatory macrophages from activated, neurotoxic inflammatory state to a neuroprotective state.  It thus normalizes the critical cellular environment.   Phase I, single dose study indicated that that there are no safety concerns and the drug is well tolerated.  Further, in a Phase II safety, tolerability and preliminary efficacy study, after administration of NP001, there occurred a close dependent reduction of inflammatory macrophages.

The dosing regimen is somewhat cumbersome, said Casey.  First time, the patients come in the hospital and need to stay for 5 days and subsequently for 3 days.  At low dose, phase II study results indicated positive trends in the ability of NP001 to slow the rate of disease progression but did not reach statistical significance.  But at high dose, the drug freezes the disease in a 3rd of the patients.  Additionally, the drug is found to be safe and well tolerated.  This clearly looks like a very exciting potential treatment for a devastating disorder, ALS.  The company is currently looking for funding.  The talk was followed by Q&A.


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