Amyotrophic Lateral Sclerosis (ALS) is a terminal illness of the nervous system, resulting in the degeneration of neurons. When the motor neurons in the lateral spinal cord degenerate, the muscle cannot be stimulated and gradually atrophies. As the disease progresses, the brain cannot initiate and control muscle movement, so voluntary muscle actions, such as reaching for your phone or typing on a keyboard, are lost. Currently there are no treatments or cures to reverse the effects of ALS and the disease process is still widely unknown. Some noteworthy individuals diagnosed with this disease have included Lou Gehrig, Stephen Hawking, Jon Stone, George Yardley, Henry A. Wallace, and Steve Gleason. These are the facts, but I could have never realized what living with ALS was like until I listened to a retired senior, a teacher, and a musician tell me their stories.

The older couple told us of their past year as the husband wrote on an electronic note pad to fill in the gaps of his spouse’s version of their story. They had first noticed his symptoms when he began choking on his food, and they had to adapt as the disease progression got more and more severe. His wife comically said that she doesn’t know where to look when he’s trying to talk to her – “Should I look at his eyes, his mouth, this pad?” Every day, they had to face a new challenge and overcome it together, as a family.

Another patient was a mother, a teacher, now wheelchair bound and with a respirator to help her breathe. Because of her age, none of her physicians expected ALS and thought she was struggling from carpal tunnel syndrome or an ulnar nerve injury. She told us her husband was her biggest caregiver and supporter; her husband joked, “She makes the list and I carry it out.” He talked about his struggles, working full time as well as being a full-time caregiver for his wife and their 3-year-old daughter, as well as still finding time for self-care. Even with all this juggling, he felt guilty. This guilt was expressed by many of the caregivers: some because they didn’t even notice the symptoms and others because they felt like they couldn’t help their spouses. Guilt was felt by the patients too. The mother was upset because her daughter would only remember her in a wheelchair. Despite the difficulties, family was a major support system for each patient.

When asked about how she felt after getting her diagnosis, the musician said, “I felt like a deer in headlights… I never pictured I was going to go out that way… with a terminal illness… Last year I had no trouble walking, singing, cooking, gardening, and this year…” Despite all of this, she said she felt relieved. Her struggle to find a neurologist who respected her was heartbreaking. She had gone to a physician because she felt helpless; she was having trouble playing guitar, she was losing her identity as a musician. However, the physician assumed from her past medical history of anxiety that she was having mental issues with memory, anxiety, and depression. As she continued to receive hostile treatment, she found a second doctor who listened to her. Unfortunately, even though he took her seriously, he didn’t have the courage to diagnose her with ALS, telling her instead that she had a motor neuron disease and referring her to a specialist. When she finally received the diagnosis of ALS, she was relieved. She no longer felt crazy. She was able to be heard. She was able to communicate with someone who wanted to help her. ALS is already a hard disease to diagnose; not having someone on her side only made her situation more difficult. She says now, as each symptom appears, she isn’t afraid anymore, because she knows it’s ALS. This relief of having a concrete diagnosis, of having a diagnosable disease, even one that is untreatable, was reiterated by several of the other panelists.

Although a diagnosis was a relief for many of the patients, they all saw the importance of taking part in clinical trials. They recognized that ALS affects everyone at different times in different ways and hoped that one day they could explain why this illness is so hard to detect and track. Participating in various studies and trials made them feel like they were helping future patients and physicians to be able to better understand why this terminal disease can strike at any time, with no discernable warning signs, affecting each individual uniquely in initial symptoms and progression.

Getting a concrete diagnosis gave these patients the freedom to seize the day and live in the moment. For the musician, as her ability to play guitar diminished chord by chord, and she started slurring her words, preserving her voice and her identity became very important to her. She is using a message banking app to preserve her voice. The software requires her to read thousands of words and sentences. Although she had to accept the things she could no longer do, she realized that she did not have to give up her identity or love of music because of ALS. Overflowing with her love of music, she told us about how when choirs sings together, their hearts become synchronized; and how music lowers blood pressure and stimulates all areas of the brain. As she interwove details about her symptoms with her favorite song, Fire on the Mountain by the Grateful Dead, the two had become part of the same spiritual transformation. Smiling peacefully, she explained, “Maybe nobody else hears it the way I do. I see death as the final healing, finally being released from your body.” She viewed her diagnosis as graduating early and the symptoms that came attached with it as her disappearing slowly, rather than all at once. The unavoidable indignities were just something she had to accept. What mattered most to her was keeping her individual personality in the face of intense daily struggles.

By the end of our discussion, I felt the weight of my white coat as I never had before and recognized that this would be the first of many times that I would have to carry this symbol of patient advocacy. As these patients spilled all their vulnerabilities to a group of 50 students, I realized the most important thing I will learn in medical school is opening my heart without getting crushed under negativity. I learned that, as doctors, we have the tendency to think the ultimate solution is life, when in truth, mortality is the reality. What matters is being present when there is a fire, realizing that “a pail of water” may not be enough to put it out, but continuing to carry that water for your patient.

Long distance runner, what you standin there for?
Get up, get out, get out of the door

There’s a dragon with matches that’s loose on the town
Takes a whole pail of water just to cool him down
-The Grateful Dead

These exalted souls are lovers with angels; they always stand their position, even in the case of possible devastation, collapse, or resentment. They carry out their duties only to please God. They deem themselves responsible towards the community in which they live. They are neither grieved by the disturbance of their work or the disruption of their plans, nor do they panic in the face of dangers descended over their community. Above all, they never fall into despair.

They are patient and skilled, and strong and determined in their relations with others. They prepare and build radiant kiosks, silently but vigilantly, and they wait for their guests. Whoever enters their atmosphere can feel as if they met Khidr, and whoever intermingles and sympathizes with them attains true happiness.

Brightness can be seen in their faces, wisdom in their thoughts, and truth in their speech. Those who enter their private spiritual space burdened with pessimism and hopelessness leave lightened with faith and hope, having acquired the secret to everlasting existence.

Neither the darkness of menacing nights, nor the multiplicity of accumulated problems can discourage them. Even if they were faced with Noah’s flood, they would walk away from it without a drop touching their feet. If the destruction like the one that befell the people of ‘Ad² fell upon them, they would carry on, marching towards their target without loss of resolution or determination. Neither the fire of Nimrod, or the Pharaoh’s arrogance, nor even the atrocious despotism of Caesar could intimidate them.

Their mind has no place for excuses like, “let it be morning, then we can go,” or “let first the cold winter finish, let the snow melt and the spring come, then we can carry on our way.” They are like graceful, inveterate trees whose branches spread beautifully towards heaven, and, by the grace of God, bear fruit in both winter and snow, in both spring and summer.

Owing completely to the All-Powerful in whom they put their trust, they do not expect help, or rely on appreciation. They are not deceived by the capricious attitudes of others. Their vision will not be blurred by the might of tyrants, and their path will not be diverted by promises of wealth or power. Whenever they visualize a terrifying day—when people are desperate and buckle at the knees, when even the proudest crumple—they despise everything about the worldly life, avoid falling into the materialist void, and rise up against the idol of sensuality. Luxury and comfort are the two things they never aspire to. They consider the lethargy of a comfortable life as death for themselves and misfortune for their nation. In the eyes of their community, they strive to accommodate new ideas and perspectives. They seek to engage in relationships with all who are open to healthy, appropriate dialogues.

Throughout history, such immortal souls have never avoided their responsibilities; they have stood firm as a chain of mountains. They were neither distracted by success nor dejected by defeat; they were not misled by the multiplicity of false lodestars, nor left dumbfounded by the destruction of their shrines. Throughout history the light of their moon was extinguished, their sun did not rise, and their stars fell one by one, but they changed neither their direction, nor their path. They have remained steadfast, resolute and truehearted.

They have always been like an army of spirits who carry the vessel of life to their people. In return, the people have always been thankful, and remained faithful.

What about those who give up and stay behind because they have been scared and disheartened when faced with a little adversity? those who always wait for spring to come? those who are shaken, slacken and easily discouraged when confronted with minor oppression? those who want to govern the masses but constantly defraud, mislead and stupefy them? What about those miserable people whose pasts are troubled, whose presents are humiliating, and whose futures are susceptible to unpredictable hysteria?

They are those who behave like daredevils when spring arrives, and hop from branch-to-branch when the sun rises. But when it snows they become sluggish, and when night descends, they, too, fall into languor. They await awards at the frontline, but when the drums of danger beat, they withdraw and vanish into thin air. They are abstinent only when confronted with poverty, but act like Korah³ when showered with abundance. They are dynamic when they are flattered, but lazy and indolent when forgotten. In short, they fall into such disrepute, humiliation, and failure that it would be no exaggeration to describe them as the disgraced of their community.

I am not sure if anything can be explained to those unfortunate ones who feel saturated with thoughts and ideas even before they have awoken to their own person, those who drown in the dullness of habit and routine, those who lose the fervor of their faith and spiritual delights. How I wish even these disordered lines could mean something to them…

¹ Khidr is a spiritual personality and a man of wisdom in Islamic tradition. He lives in an angelic way, serving God in the execution of His commands and succoring people in hard conditions. It is reported that Khidr drank from a fountain in Paradise, granting him immortal life.

² ‘Ad was an ancient tribe from the region of Yemen. The people of ‘Ad rebelled against the Prophet sent to them by God (Prophet Hud, peace be upon him), and did not heed his warnings. Thus, they received the wrath of God and were destroyed in a great storm.

³ Korah was a wealthy person from among the people of Moses, peace be upon him, and his story is told both in the Qur’an and the Bible. According to the Qur’anic description, he was so rich that the very keys of his treasures alone were “too heavy a burden for a company of strong people” (Qasas 28:76). But he falsely claimed exclusive ownership over this wealth which was granted to him by God, and he betrayed his own people.

We vessels can be divided into three main groups in terms of structure and function. The ones with thicker walls, which bring every organ the blood they need from the heart, are the arteries. The pressure inside us is higher and we easily carry blood to the organs. The ones with thinner walls, lower pressure, and larger inner space are called veins. As a matter of fact, both arteries and veins have a three-layered structure that is very suitable for holding a fluid like blood. Since our walls are strengthened with both connective tissue and smooth muscle layers, we bear the pressure coming from the heart and help blood proceed by contracting and relaxing. Since arteries are directly subjected to the strong pressure from the heart, our walls were created in a thicker and stronger form. Since the veins return blood to the heart and thus have lower pressure, we have valves that close after blood passes, so it does not flow backward due to gravity. This is a serious challenge for the blood passing through your legs. Varicose veins might develop due to weight gain from pregnancy or obesity, which increases pressure on the legs, or to hours of standing, walking, or running on hard surfaces.

Capillaries are the most delicate blood vessels, with walls made of a single layer of epithelium, which enables us to exchange substances between blood and tissues. As blood vessels, our total length is about 120,000 kilometers. Try to imagine if a fisherman’s net were made from a rope of this length and how wide it would be! And yet, such a vast network of blood vessels is located in your body, and capillaries take blood to every part, without neglecting an area as tiny as the head of a pin.

The well-being of your organs is directly related to us. If our interiors begin to narrow, because of fatty cholesterol plaque for instance, then we begin to lose our flexibility. This means malnutrition for that organ, since a lesser amount of blood than expected can come. If a blood clot sticks to our wall and blocks the blood flow, the relevant organ may be in terrible trouble. If other arteries supply blood to that organ, then it can handle this, but if a main artery is blocked and if secondary channels do not exist or are insufficient, you experience infarction. Taking this into consideration, you need to be careful what you eat and lead a physically active life. When you get old, if sufficient blood does not pass through us in your brain, failures with brain activities appear and you go senile. As the walls of veins and arteries have a rich network of nerves, we let the suitable amount of blood flow according to the need of the organ we’re serving, under the control of the autonomous nervous system. While blood vessels that are connected to an organ not currently requiring much blood contract to reduce the amount supplied, those that are connected to currently more active organs expand. And dear Peter, the greatest blessing here is that none of these activities require any conscious effort from you; everything works smoothly without your even being aware.

This wonderful network of ours finds its value in the vital fluid we carry. If it weren’t for blood, we would have no value at all, and such a perfect means of distribution would be unnecessary. Even the duty of the heart is to make this fluid circulate throughout the body. Now, let us step aside and allow blood to have the floor.

Blood is a highly specialized tissue circulating throughout the body to carry out essential functions for an organism. Some of the basic functions of blood can be listed as: warming or cooling the body, protecting it against infectious disease, supplying essential ingredients to the cells, getting rid of harmful and unwanted waste from cells, and carrying messengers to initiate physiochemical events at the cellular level.¹ An average adult has approximately five liters of blood which completes its cycle in several minutes.² Blood can be regarded as a flawless servant to human beings with a perfect design to fulfill delicate needs and tasks to maintain their lives. If blood stopped performing just one of these tasks in some way, the survival of humans would not be possible.

Plasma is one of the main components of human blood in which the red and white blood cells are suspended.³ These two “living cells” are responsible for the crucial job of maintaining the balance of the body. Blood cells have a definite life cycle, just as all living organisms do. The most generous and all-compassionate owner, God, even knows the needs of the tiniest creatures and for Him to recreate these two cells is as easy as resurrecting hundreds of thousands of fruits, vegetables and animals every spring. Interestingly, bone marrow acts as a factory to reproduce new blood cells in place of continuously dying cells.³

In order to generate energy required for all cellular processes, oxygen has to be carried into the cell and the resulting carbon dioxide should be carried away immediately. Red blood cells, known also as erythrocytes, contain an iron-rich protein called hemoglobin which performs this duty in an excellent way. Each red blood cell contains approximately 250 million hemoglobin molecules.⁴

Hemoglobin transports oxygen from the lungs to the rest of the body and carries carbon dioxide away from the body to the lungs by consecutive chemical events in harmony. Hemoglobin can bind oxygen and/or carbon dioxide reversibly and the preference for binding to either oxygen or carbon dioxide depends solely on the environment. Upon inhaling the air, the amount of oxygen will increase in the lungs and oxygen will bind to hemoglobin’s iron unit preferentially. Later, the heart pumps oxygen-rich blood all over the body to deliver it to where it is required. As blood travels through the body in artery veins, oxygen will be exchanged with the carbon dioxide, since the amount of carbon dioxide inside cells is higher than oxygen. Then, the bound carbon dioxide will be sent back to the lungs and this process will be cycled over and over again during the course of life.⁵ During these processes a lot of complex chemical and biological changes occur in a systematic way to optimize the speed, effectiveness and quantity of oxygen transportation.

Surprisingly, one hemoglobin unit can carry four oxygen molecules at the same time. However binding of four oxygen molecules does not happen at the same time, they rather prefer binding one after another. One of the most striking discoveries about these processes is that when the oxygen attaches itself to the iron in the hemoglobin, the shape of the hemoglobin changes and this phenomenon facilitates binding other oxygen molecules.⁶

At higher altitudes air contains less oxygen as compared to lower altitudes. In people accustomed to living at higher altitudes the amount of a chemical known as 2,3-BPG in blood was found to be higher than in people living at lower altitudes.6 Researchers showed that this chemical actually binds to hemoglobin to result in easier oxygen delivery in lower oxygen atmospheres. Without this chemical, at high altitudes people would start suffering from oxygen deficiency and some of the vital organs would start dying slowly. It is obvious that this is a decisive and self-evident proof that there is an ultimate power in the universe and He is the one Who is the most Merciful.

Also the hemoglobin in the fetus has a greater affinity for oxygen than its counterpart in adults. Fetal hemoglobin uses maternal oxygen from the mother’s bloodstream and this ability gives the fetus more access to oxygen for better survival.⁷ Otherwise, no baby would be able to grow fully in its mother’s womb. Divine mercy is aware of the need of even an incapable baby in the mother’s womb and His wisdom and generosity provide appropriate tools, decorations and ornaments to whoever is in need of them.

The human body can be seen as a perfect machine equipped with state-of-art components that functions magnificently to sustain human life without any conflict. It is designed to such an extent that even its slightest needs are satisfied with an amazing design planned by great wisdom and engineering. This beauty, extreme skill, and utmost perfection testify to the existence of the All-Wise Maker and All-Knowing Inscriber. Claiming that this masterpiece is not the work of a purposeful artist is as foolish as claiming that a beautiful painting is not the art of a good painter. Even in one of the sub-structures of red blood cells (hemoglobin) the highest degree of mastery and the exquisiteness of administration for each process show an irrefutable wise Creator who has utmost knowledge and proficiency. His unique power for marvelous creation is even more visible on the surface of the earth.

“He Who has created seven heavens in harmony. You do not see any fault or incongruity in the creation of the All-Merciful. Look yet again: can you see any rifts?” (Mulk 67:3)

Mutations somehow alter the sequences of genes responsible for producing hemoglobin and as a result of inheriting these genes, some kinds of hereditary diseases may occur in future generations, such as thalassemia and sickle-cell.⁸ Since hemoglobin in these cases does not have the ability to carry oxygen properly, in some extreme cases blood transfusion is necessary to supply healthy hemoglobin for survival of patients. Instead of producing super quality hemoglobin, mutations lead to malfunctioning of the system. No observable mutation can generate meaningful and healthy changes in an organism. Trying to explain the formation of these beautiful, complex, harmonious, and utterly perfect cells by chance or coincidence and attributing the creation of these systems to unconscious nature as their creator is far beyond any reasonable scientific explanation.

“Was he not once a mere drop of semen poured forth? Then he became a clot clinging (to the womb wall), and He created and fashioned (him) in due proportions.” (Qiyama 75:37-38)

Ibrahim Yildiz is a graduate student of chemistry at the Miller School of Medicine, University of Miami.

Notes

1. Previte, J. J. Human Physiology McGraw-Hill, 1982.

2. Cecie, S., Taggart, R. Biology: The Unity and Diversity of Life. California: Wadsworth, 1989.

3. Jones, B. D. Delmar’s Comprehensive Medical Terminology. Thomson Delmar Learning, 2000.

4. Roberts, M. B. V. Biology: A Functional Approach Cheltenham: Thomas Nelson and Sons, 1986.

5. Mehler, R. E. How the Circulatory System Works Blackwell , 2000.

6. Ganong, W. F. Review of Medical Physiology McGraw-Hill, 2005.

7. Champe, P. C., Richard, A. H. Biochemistry Lippincott Williams & Wilkins, 2005.

8. Steinberg, M. H. Disorders of Hemoglobin: Genetics, Pathophysiology, and Clinical Management. Cambridge University Press, 2001.

In Snow White, the evil stepmother gushes: Mirror, mirror, on the wall, who’s the fairest one of all? Most people think that she is vain, and therefore being vain is bad. But there is more to it than that, and if this is all we can impart to our children then we are robbing them of meaning. Actually, I do not think this is about our children at all, but rather about ourselves as adults. When we read these stories again to our children, its very telling about ourselves if we finally understand these stories or not, now that we have grown up.

If you are searching for wisdom in life, a child’s library of fairy tales is a convenient place to begin. The imagery in fairy tales provides us with archetypes that we can use to organize the lessons that life teaches us.

The notion of the evil stepmother in Snow White talking to her mirror struck me when I was looking at myself in the mirror. We know that some mirrors do a better job for us then others. Mirrors that make us look horrendous put us off. Is it the lighting? Is this mirror warped or what? Do I actually look like that? What’s going on with my hair? How come my pores are so big? But what I really mean is: Am I the fairest one of all?

I wish I had a mirror that did the job like the one Snow White’s stepmother had. It was honest and reliable. But mirrors like that do not exist, at least not for our bathrooms. But we do carry such a mirror, and it is of great help if we know how to use it. To use it properly, we have to ask ourselves the proper questions. The evil stepmother asked the wrong question. She was not evil; she was unenlightened and lost in her own illusions.

Most of us ask the wrong questions but do not think of ourselves as evil. We ask if we are the fairest, the smartest, the happiest, the most contented. The stepmother was told that she was not the fairest in relation to Snow White. She was plenty gorgeous already though, the number two beauty in the land.

When we ask the wrong questions, we become as delusional as Snow White’s stepmother. Unfortunately, many of us do not stop looking for a mirror that will tell us that we are the fairest.

Mirrors only show us who we are, not who we would rather be. We think that a new car would be a good mirror, but it is not. We think that a new, attractive mate would be a good mirror, but it is not. Neither is a high paying job or a lovely home. Such things have nothing to do with finding a mirror that works because this inner reflection, this mirror that we carry, never fails to work. Unfortunately, we simply fail to look, all the while asking the wrong questions to the wrong objects.

To be fair, it is difficult to look straight into our honest reflections. Not only must we contend with our previous illusions of trying to be the fairest, we must now contend with very real failings of character and personality. Guess what? I realized this weekend that I am not such a nice guy. Something like that can be down right depressing, but not if we realize that it is all part and parcel of seeing ourselves.

When we can SEE ourselves, we begin to see others; that is, we see ourselves in others and others in ourselves. We also begin to question ourselves, not to doubt ourselves but to pose questions that require answers from us. That is, we make ourselves accountable. When we are accountable to ourselves, we are essentially free. We no longer look outside for the pre-digested answers so much as inward for both the questions and the answers.