Shooting Star

I arrived in our outpatient clinic the other day and the local TV News crew was there waiting for me. No, I have not become a celebrity. Far from it. A fugitive maybe? What I learned was that they were not really after me, but was following the patient who was referred to us, whom I was seeing for the first time.

Before I walked into the exam room where my patient was, the TV news reporter whom I immediately recognized, as she was also the local evening news broadcaster, greeted me and asked permission if they could shoot footage of my interview and examination of the patient. How could I say no, when the camera was already there in front of my face?

The TV reporter then put me at ease, as maybe she sensed the tension in my face, and said that I should just do my regular routine as if there was no camera recording me. Easier said than done. I just wished they notified me a few days in advance so I could have at least prepared and had my hair done too. Oh I forgot I have no hair. I checked then if there was something in my face or something stuck in my teeth, so at least I won’t look gross on camera. They hooked a small microphone on me, and said I was clear to go. It was show time!

I entered the room with the camera rolling and following me. I met my patient. He was a young man, in his early 20’s, tall and muscular, but walks with a cautious gait. I also noticed some weakness with his handshake. With a nasal-twang voice, he told me that he was trying to increase awareness of his devastating disease and that he was raising funds for a foundation he had set. Such a noble cause. Such a noble gentleman. That was the reason the TV crew was there.

My patient has ALS.

ALS or Amyotrophic lateral sclerosis is a relentlessly progressive, presently incurable disease. It was first described in the 19th century, long before the famous New York Yankee baseball player Lou Gehrig was afflicted with it, for whom the disease was now named after. It has an incidence of 1 to 3 cases per 100,000 people worldwide.

(image from here)

The disease affects the ‘motor’ neurons (nerve cells) in the brain and in the spinal cord. These neurons supplies the muscles that are responsible for our movement (hence the name ‘motor’). Thus the common presentation of patients with the disease is progressive weakness – may first present with clumsiness in writing or inability to hold a glass of water and advance to full paralysis of the arms and legs, that they become wheelchair-bound.

They also present with hyperreflexia and spasticity as if they are performing Michael Jackson’s dance moves. Their speech becomes garbled as if they have swallowed their tongue, for our tongue is a muscular organ and thus gets affected. Eventually their muscles atrophy as it loses its function.

It is rare that ALS would impair the cognitive or intellectual functions. Though about 15 % or more will develop some form of dementia. So the sad part is that they are fully alert, with their mind so clear, while their body gets weaker to the point that they could not move, imprisoning them inside their own body.

So you may ask, what was the patient, with a purely neurological disease, doing in a our pulmonary clinic?

Sadly to say, the life threatening features of the disease involves pulmonary. Weakness involves the muscles of respiration too, therefore breathing becomes more labored as the disease advances. Progressive respiratory failure, in fact, is the most common cause of death in ALS. That must be an awful feeling, as if you are drowning and you are not even underwater.

They can also have problems with swallowing as time goes on, making eating difficult, if not impossible. This trouble with swallowing and problems clearing their throat, combined with ineffective cough, predisposes them to aspiration with resultant pneumonia, which is another common cause of their demise.

I had the patient perform a breathing test in our office. This was to assess if his ventilatory capacity was already compromised. Once the lungs’ vital capacity drops to 50% of normal, it is associated with significant respiratory symptoms and would need intervention. If it drops to less than 30%, the risk for respiratory failure or sudden death is quite high.

What intervention can we do? Patients with significant respiratory compromise can be placed on Non-invasive Positive Pressure Ventilation (NIPPV) when they sleep, as well during the day if desired. It is like putting a fighter pilot mask and attaching it to a blower. This is similar to the Continuous Positive Airway Pressure (CPAP) machine that people with sleep apnea use. The NIPPV helps them breathe better and improves their quality of life, though whether it prolongs their life is still debatable.

Of course if the breathing status gets really bad, a tracheostomy can be done and they can be hooked permanently to a mechanical ventilator. Though only fewer than 10% of patients with ALS choose to have this. I guess people accept the fact that these invasive intervention, like tracheostomy, is just prolonging the agony and does not prevent the inevitable, that is death.

ALS is indeed a ravaging disease. It is an unrelenting condition, and ends only with dying. The mean life span of patients with ALS is 3 to 5 years after the diagnosis. And at this point, there is no known cure. It is a death sentence whatever angle you look at it.

After examining my patient and reviewing his breathing test, I told him that his vital capacity was still way above 50% and that he does not need any intervention…..yet. A glimmer of good news in an otherwise gloomy outlook.

Life is short. In my patient’s case, it is short-er. Much shorter.

Live life to the fullest, even how short it may be. For it is not how long our light shines, what matters is how bright it glowed in the dark night. My patient is a shooting star.

“I might have had a tough break, but I have an awful lot to live for.” – Lou Gehrig

*****

P.S.#1: I hope I have raised awareness of this disease on my own, in behalf of my patient.

P.S. #2 : I hope I see myself on TV. (*mulls over a showbiz career*)

16 thoughts on “Shooting Star

  1. Tell him to stop drinking milk and other dairy products.

    —————————–
    How many people are aware of the dairy consumption link?

    Multiple sclerosis affects approximately 300,000 Americans. Two-thirds of those diagnosed with MS are women. Most researchers believe that MS is an autoimmune disease. Auto means “self.”

    It is interesting to note that Eskimos and Bantus (50 million individuals living in East Africa) rarely get MS. Neither do those native North and South American Indian or Asian populations that consume no cow’s milk or dairy products.

    The British medical journal Lancet reported that dairy-rich diets filled have been closely linked to the development of MS. (The Lancet 1974;2:1061)

    A study published in the journal Neuroepidemiology revealed an association between eating dairy foods (cow’s milk, butter, and cream) and an increased prevalence of MS. (Neuroepidemiology 1992;11:30412.)

    MS researcher, Luther Lindner, M.D., a pathologist at Texas A & M University College of Medicine, wrote:

    “It might be prudent to limit the intake
    of milk and milk products.”

  2. In 1976, the journal Neurology suggested that Lou Gehrig’s
    disease might be induced by an autoimmune response to bovine
    proteins (Volume 26:2, 167-172).

    Frank Oski, M.D., once Chief of Pediatrics at Johns Hopkins
    Medical School wrote on page 63 of his best selling book,
    “Don’t Drink Your Milk”:

    “It may be more than coincidence that a group of
    investigators from the Baylor College of Medicine in
    Houston, Texas, also identified milk consumption as a factor
    in…amyotrophic lateral scleroses–also popularly known as
    Lou Gehrig’s Disease after the famous athlete who was a
    victim of this disorder. The neurologists analyzed many
    variables in twenty-five patients with this disease and
    compared the patient’s histories with twenty-five healthy
    individuals of similar sex, age, racial background, economic
    status, and education. The factors that set aside the
    patients with amyotrophic lateral scleroses from their
    normal counterparts was that the patients reported an
    increased incidence of exposure to lead and mercury, more
    participation in sports, and higher ingestion of cow’s milk.
    More food for thought.”

  3. We are a small group of volunteers who formed ALS Treat Us Now. our mission is to have the FDA and Pharmaceutical companies help ALS patients have access to some of the 42 treatments showing safety and effectiveness in early trials.
    We need many more volunteers. Check us out:
    http://www.facebook.com/pages/ALS-Treat-Us-Now/220968231317148?sk=app_4949752878
    and:
    http://www.change.org/petitions/corporate-citizens-authorize-and-make-available-compassionate-use-drugs-for-als-patients-now
    email: treat.us.now@gmail.com

  4. i have known ALS, of course from harrison’s internal med book and other references i’ve read. unlike you, i have not yet encountered anyone with such.

    i sincerely hope your patient goes through with ALS, for the lack of better word, with less difficulty.

    i also wish doc, that your shining moment on tv would not be edited and be shown even on youtube. (kidding!).

    • Since the news clip will be really about my patient, and the news crew have been following him for a while, who knows his visit with me could be really edited out. I just hope they show me even for a split second. :)

  5. Lou and his faith in life and all that it has offered is touching and inspiring. A noble man who taught us that life is not about quantity but of the quality we give to it. He did so much that a lot of us won’t be able to achieve even if we live past 60. Let his story be an inspiration for all. This struck me, “Live life to the fullest, even how short it may be. For it is not how long our light shines, what matters is how bright it glowed in the dark night. My patient is a shooting star.” He is…a star that will shine for a long , long time….

  6. I know that you mentioned somewhere that you are against euthanasia and the like. I wonder if it may be the way euthanasia is defined/painted as in euthanizing animals like old sick dogs or injured horses.

    I want to call your attention to the case of Sue Rodriguez. She was a Canadian woman who lived with ALS in the late 80’s and had started the movement for the right to die when ready. She was heavily supported by a lot of older-age groups and gay rights groups back then, because of their vested interests in wanting do die with dignity. ALS patients pretty much drown in their secretions. Not a pretty sight nor experience, I’m sure. My husband and I followed her as she brought her cause to the Parliament and to the courts until it reached the Supreme Court. She lost, of course, and in a few years, she passed away. But all her work did change how Canada treat the people who are dying or have a long lingering debilitating illness. Entire programs were set-up to address the need to feel that patients still have some degree of control over their waning faculties, their pain, their comfort, and if and when they really do not want to fight anymore. We saw the programs develop over time (in fact, some of our own colleagues were involved quite heavily) until we moved from Canada to the U.S. in ’97. We need to allow people to die with dignity. It’s very easy for us to aggressively preserve the status quo. It’s very hard for us to come to terms with letting go and not feel that we have failed them.

    That was my previous life.

    • When I use the term “euthanasia,” I strictly limit it to the physician-assisted suicide or as you said it, as in”euthanizing animals like sick dog or injured horses.” In this context, I don’t agree with it.

      However, just like you, I believe in “allowing” people to die peacefully, or letting nature takes its course. Of course we can provide comfort and pain measures as they pass on with dignity. I am a strong proponent of Palliative Care medicine.

      However these “terms” has a lot of gray areas and can be quite controversial.

  7. Wow showbiz ka na Doc.

    Nalaman ko ang sakit na ito noong napanood ko ang ‘Tuesdays with Morrie’ starring Jack Lemon. Very touching ang movie kasi true story. May book din ito.

    Kung may gustong makapanood available ang movie sa youtube(11 parts).

    “Live life to the fullest.” Amen!

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