Till Death Do Us Part

“I want to go home and take care of my husband.”

That was what Dorothy* said just shortly after we took her off the ventilator. She was an elderly woman in her late 70’s  who was admitted in our ICU after suffering a major heart attack. Her heart rate went berserk like a runaway train that we had to slow it down. She also developed heart failure, causing fluid to build up in her lungs like a dam, giving a sensation of drowning.

As she was extremely struggling to breathe spontaneously, we placed her on a non-invasive ventilator. This ventilator is like having a jet-fighter pilot’s mask strapped snugly in your face and then hooked to a strong blower machine, forcing air and oxygen into the lungs. This is similar to the machine that people with sleep apnea use at night.

Besides the cardiac and respiratory failure, Dorothy also suffered mild kidney and liver injury from the poor blood perfusion to these organs as her heart was like an overwhelmed central pump, barely sputtering to adequately supply its vital tributaries.

For three days Dorothy was on this non-invasive ventilator to assist her breathing. But with great care, supportive interventions and medications, she slowly improved. She improved enough that we were able to liberate her from the breathing machine. Now, all she just wanted was to go home, and be with her husband.

After talking to her at length, I learned that Dorothy was married for 56 years. A long time indeed. Her husband, believe it or not, was more sickly than her. She was supposed to be the healthy one of the pair. In fact, she was the primary care giver of her ill husband for many years now. She devotes her time and energy in taking care of him, so much so that she sometimes neglects taking care of her own self. Such dedication. Such love.

And now this happened. Who will take care of her husband now?

But Dorothy willed herself to get better. She was determined to get stronger. She will survive this heart attack. She will get over this congestive heart failure. She will recover from this respiratory failure. She will be going home to be with her husband once again.

The next day, Dorothy was out of bed and was sitting in a chair. She looks so good and was really doing fantastic. She improved so much that we told her that we were moving her out of the ICU, and if she continued to do well, she might go home in a couple of days. She was ecstatic.

This proves that many times it is really mind over matter. Our willpower can heal our ailing body. Our resolve can overcome our weakness. Our determination can make us stronger. We can will ourselves to get better. And in this case, love was the motivating force. Like some old songs would say “that’s the power of love,” and “love will find a way.”

Not too long after we left Dorothy in her ICU room, her daughters came. They came with a sad news. Terrible news! Her  husband – the one that she dedicated her life and strength, the one that she love in health and in sickness, the one that she willed herself to get well in order to come home with – suddenly collapsed at their home. He was dead on arrival at the hospital.

Two hours later, Dorothy was placed back on the ventilator. Life can be so cruel at times.

(*not her real name)

(**photo was taken inside the great hall of Salisbury House in Des Moines)

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*)