From Patient to Student; A Different Side of Stanford

The last time I was at Stanford, it was for a check-up with Dr. Steinberg to ensure that my operations back in 2008 were working. I remember an angiogram, an MRI, and too many IV’s for my liking. The time before that had been another scheduled check up, and the time before that had been my surgery. Essentially, my only experiences with Stanford thus far have been related to Moyamoya and keeping it at bay. This all changed a few weeks ago.

Back in early February, I was contemplating what I was going to do with my summer. Several of my friends had already secured internships, decided to go back to their camps as CIT’s, or pursue studies at summer programs. One day, while in Chemistry class, I saw my friend filling out an application. My curiosity sparked a conversation, and soon I became aware of one of the summer programs that Stanford offers.

“You better hurry up,” my friend said, “the app is due in a few weeks and there’s a ton to write.” I do not remember ever feeling such a drive to write. Not only did the course I chose interest me, but there was this desire to see the “other side” of Stanford: one of students, classes, dorms, and a campus; not of doctors, nurses, IV’s, and sterile white hallways. My understanding of Stanford — the thoughts that came to mind when the name was mentioned — was different than most going into this summer.

Several months later, I arrived at my dorm, excited and nervous. This was the first time I had been away from my family for over a week. I was there to study International Relations at one of the summer programs for high schoolers. Over the course of the next few weeks, I met intelligent, inspiring people from all across the world, made lifelong friendships, and learned a lot. My first time walking across the campus — I got lost due to my terrible sense of direction — I was awestruck at its size and unique beauty. The red tiled roofs and palm trees, as well as the fountains completed the relaxed yet lively environment. The people I met at Stanford transformed my experience. While one may be able to see all that I did as a tourist or visitor, the professors, graduates, students, and fellow classmates that I interacted with expanded my perspectives on life and taught me more about myself in the process. Sure, I learned tons about the global economy and the ways that countries interact with each other, but I also learned what it meant to be independent, make lasting friendships of my own, and explore a sprawling campus and decide what my place there looked like. In short, I experienced, albeit for a brief time, what it felt like to be a college student.

Moyamoya Day Reflections

Note: Earlier this month, California officially recognized May 6th as Moyamoya Disease Awareness Day (http://scopeblog.stanford.edu/2017/05/04/may-6-officially-recognized-as-moyamoya-disease-awareness-day-in-california/)

This past Saturday was World Moyamoya Day — but only to those within the tight-knit Moyamoya community and their friends/family. There has been an effort to get the World Health Organization (WHO) to recognize May 6th as “World Moyamoya Day”, yet the WHO’s decision that the disease is not “major” enough to receive recognition in this form presents a roadblock. Because of this, there is a petition, one spearheaded by Tara Macinnes, to persuade the WHO (World Health Organization) to formally set May 6th as “World Moyamoya Day”. The link to the petition is under the “Important Links/Other Sites” page of this website, but I have copied it below as well:

http://www.thepetitionsite.com/831/455/587/moyamoya-disease-awareness-world-moyamoya-day-may-6th/

Getting as many signatures as possible — telling friends, family, or peers about this petition — is absolutely crucial to the spread of awareness of Moyamoya. There are around 5,000 signatures needed for the petition to be legitimized. I write often about the need for increased awareness about Moyamoya, yet do not regularly explain why it is so necessary. I was misdiagnosed almost a dozen  times by otherwise qualified doctors and neurologists before being succesfully treated simply because of the “rarity” of Moyamoya cases; however, a trained or aware doctor could have observed and picked up on the rather textbook signs of Moyamoya that I exhibited in MRI’s. The issue is not necessarily  with the doctors themselves — who are representative of the general medical community and was as the public’s knowledge of Moyamoya — but with the fact that almost nobody a) expects to see cases of Moyamoya or even b) knows what it is. Fortunately, I was able to be seen by one of the leading experts in Moyamoya, Dr. Lori Jordan (see my interview with her a few blogs down) and was properly diagnosed.

However, the sad reality is that not everyone has been/will be as fortunate as I was; The correct diagnosis of Moyamoya disease is not difficult to make when a doctor is informed and aware; with proper resources such as an MRI or an angiogram, the signs of Moyamoya in the brain’s blood vessels can be easily distinguishable.

All current statistics indicate that close to 1 in every 2,000,000 people in the world have Moyamoya — hence its status as a “rare disease”. However, factors such as misdiagnoses have led researchers at the Stanford Moyamoya Center to estimate that the actual number is closer to 1 in 200,000. The  astounding difference in the two estimates of Moyamoya cases per population concentration is incredibly worrying, especially given the progressive nature of Moyamoya. The longer the disease  goes untreated, the more one is vulnerable to repeated strokes which could lead to increasing cognitive and physical damage  or death.

This is why raising awareness for Moyamoya is so important; this is why we need to convince the WHO to recognize May 6th as World Moyamoya Day. It is tragic how an unknown number of people die every year because of stroke or hemorrhage due to an undiagnosed or belatedly diagnosed case of Moyamoya.

Another reason that signing this petition — taking 5 minutes of your day (maximum) in order to further a life-saving cause — is necessary is the funding for research. Just as a WHO-recognized awareness day would raise awareness for the disease, it will attract attention towards the research being done at leading clinics around the world. There is currently no cure or prevention method for Moyamoya, and there only exist surgeries to aid in improving cerebral vascularization. The problem is, however, the lack of access that many people have to get the MRI’s and angiograms, not to mention surgeries, in order to beat back Moyamoya. Drawing more attention to the severity and relevance of Moyamoya disease everywhere around the globe could potentially fuel research to find a cure.

 

Baseball and Gratefulness

One of my favorite things to do is play baseball. I have done so for years, and am part of my high school’s baseball team. I love the community, excitement, and competitiveness that the sport provides, and hope to continue to play for as long as possible. Although I am not a freak athlete or a stand-out prodigy, I still value its role in my life and continue to play.

Baseball is only one of the activities that I enjoy and am fortunate enough to pursue. I run Cross Country, teach and play chess, play guitar, participate in Model Congress conferences, and more. Nearly none of my growth and experience in any of those fields would be possible without my successful surgeries. I would not be able to do much of what I now love if I continued life undiagnosed and untreated like many out there. The minor strokes that I had would have gotten worse and more frequent the longer I went without treatment, and that is unfortunately a reality for many of those with Moyamoya. The rarity and lack of awareness around Moyamoya is ultimately the deadliest factor, and one that led to several of my misdiagnosis.

Earlier this week, during one of the first games of our school’s baseball season, I dove to catch a fly ball. After making the play, I felt a rush of gratitude. I tend to take my health for granted, and not think about the very real possibility that I could have suffered a major, paralyzing stroke before I was successfully treated — or even that my operations almost a decade ago did not produce the optimal results they so far have. It is too easy to get lost in comparisons to my peers on the team, or players on the opposing team. This leads to a disregard for how far I have come since my first TIA at the age of 5.

The attempt to more consistently recognize how privileged I am to have been diagnosed and treated before any serious, lasting damage — physical and mental — has led to a more humble attitude as well. Rather than accrediting any achievement solely to myself and getting self-absorbed, I instead think about all of the kind nurses at Stanford, Dr. Steinberg and his skills/expertise, my parents, and all of my friends and family that supported me through a time of need — all of whom contributed in their own ways. It is important to keep in mind that it is a blessing to be healthy, especially if you have a disease such as Moyamoya that does not have a definitive cure and lacks awareness.

 

Dehydration and Everyday Life

As someone with Moyamoya disease, staying hydrated is especially crucial to my health. However, as a relatively active and busy teenager, it can become easy to forget to drink water and stay hydrated. Usually I try to carry around a water bottle during school, as it provides a physical reminder to drink. In engaging and stimulating settings such as school, social situations, or sports, getting carried away and neglecting to stay hydrated can be easy — bringing a water bottle with me makes keeping hydrated easy and simple. I often find myself in situations where I have been concentrating on one thing for such a long time that when I finally get something to drink, I realize how thirsty I am. For example, during a packed and busy day at school, there can be lots on my mind other than drinking water. Even when eating lunch, I tend to drink less hydrating, sugary drinks such as lemonade when my body needs water.

The irony lies in the accessibility of water around me. Despite the privilege of usually having a source of clean water, I do not often take advantage of it. This is not in an attempt to scorn my body, but rather a lack of habit and a preoccupation with something else. I find that I am often slightly dehydrated when there is a source of water nearby me. In my mind, there is somewhat of a procrastination towards staying hydrated: “I will just get some later.” And this is fueled by how easy it is for me to access drinking water. The availability of water often circumvents my hydration levels, and causes water to be low on my immediate list of priorities on a given day.

Just to clarify, I am almost never dangerously dehydrated. Many of the instances that I have referenced above are cases of mild dehydration, not anything life threatening. However, it is still imperative as someone with Moyamoya to keep hydrated. One easy way for me to tell if I am hydrated is urine color. The darker and more yellow my urine is, the less hydrated I am. That, and thirst levels, are last resorts for me in terms of staying hydrated. For me, it is ideal if I do not have to rely on these signs of dehydration and be proactive in drinking water instead.

Although I am not necessarily the model, hydrated person, I have been striving to stay hydrated recently and want to share some quick, easy tips:

  • As mentioned before, keep a BPA-free water bottle on person throughout the day, and on trips
  • If you notice any of the tell-tale signs of dehydration, (see https://medlineplus.gov/dehydration.html) drink water immediately
  • Stay away from sugary, caffeinated beverages as they are not as hydrating as water
  • Stay away from salty, high-sodium snacks
  • Remember to drink water after exercising, especially when the weather is hot.

 

Haircuts

As someone who has had clearly visible scars on both sides of my head since the 2nd Grade, I know how it feels to be self-conscious. Many times people have asked me about them, often thinking that my scars were a hairstyle preference. Of course, I do not disdain or dislike those who ask me about them, but the questions often make me feel uncomfortable. At some points in my life, I have let my hair grow out in order to have my scars less noticeable; I suppose that I thought it would make things more convenient.

It all probably started when I met my brother and sister at the airport after returning from Stanford. My sister looked at me, — two years old at the time — with a innocently frightened face, and pointed to where my scars would be on her head in confusion. At the time, I just smiled and thought nothing of it. Only weeks after my surgeries, my scars looked absolutely disgusting and intimidating — especially for someone young. Since then, it has seemed like an endless stream of people asking me about my scars. My answer has always been the same: “These are scars from my brain surgery in Second grade.”I usually say this casually, and matter-of-factly, just to try to get over it. Many times, this has the effect of shocking the person, and often prompts them to say something like “I’m sorry to hear about that.”

In the past several years, I have had this pattern occur: First, I would get long, shaggy hair and decide to cut it; then, I would see how visible my scars are and feel hesitant and irrationally insecure. In my head, I would think that everyone would notice and comment on my scars, — which is not a big deal at all — something that I am unreasonably afraid of. Sometimes, I would get a few questions about my scars from people who had not even noticed they existed, but things always go normally.

My insecurity spawns from the natural desire to want to fit in with everyone around you. I have always wanted to be known for my merits, and actions rather than the fact that I have Moyamoya disease. It is an important part of my identity, but not one that I want to define me before my personality. My scars are something that clearly separates me from 99% of those that I have met, and that can make me feel uncomfortable. Although my insecurities certainly still do exist, this past year I have started to care less and less about trying to cover my scars. I have come to accept that they are a part of my life, represent a key experience that I had, and am proud to bear them.

 

Living With A Rare Disease

Wedged in between Mowat-Wilson and Moynahan alopecia on the National Institute of Health’s “Rare Disease List”, Moyamoya disease sits as one of the 7,000+ conditions that affect less than 200,000 people in the United States.

Living with a rare disease, one that almost no one — other than doctors, family members and close friends of those who have Moyamoya — I have met has recognized or heard of, can be difficult. Even as I type this post, the term “Moyamoya” is not recognized as a word and has irritating red dots underlining it.

When explaining Moyamoya, I often give an oversimplified version of a complicated cerebrovascular disorder while also explaining how that corresponds to the scars on both sides of my head. I do not even know very much about this disease — other than what I have sought out to learn —  and hardly have the expertise to give a concise and accurate description to someone just finding out about it.

I have encountered countless situations where I have been going about my day, when I get a gaze at my scars. That usually leads into a conversation, where “These are scars from when I had brain surgery in the Second grade” is met with surprise. Several people who have asked about my scars — which I do not condemn as an rude or insensitive action — have even thought that I was sporting a strange hairstyle.

Sometimes, people think that I am joking, or pranking them — I assume due to the rarity of Moyamoya combined the rarity of a Second grader going through brain surgery. These moments of doubt are clearly well-intentioned, yet are vaguely annoying. Although there is really no way for anyone other than an expert in cerebrovascular diseases to recognize Moyamoya disease in my scars, it is unlikely someone would lie about such a life-changing and terrifying part of their life.

The real source of my frustration lies in the lack of awareness of Moyamoya. Research, as well as my own experience, indicates that cases of Moyamoya are actually far more plentiful than shown by statistics. Even in cases — such as mine — with “textbook” symptoms of Moyamoya, they are often overlooked and more common, easily treated diseases are diagnosed. Especially in areas with meager medical resources, a rare disease such as Moyamoya is not likely to be seen or properly diagnosed. Several accomplished doctors in the NYC area completely misdiagnosed me, and one almost gave me steroids to treat something that was not there.

Minor strokes, or TIA’s are one of the principle symptoms of Moyamoya. Yet, one who experiences TIA’s is unlikely to ask for an MRI or an angiogram — surefire ways to confirm a case of Moyamoya. The chances that someone who has a developing case of Moyamoya reaches out to a doctor before a seriously debilitating stroke and is successfully diagnosed is slim.

Nowadays, with expanding and improving Moyamoya treatment centers, breakthrough research, and highly successful operations the issue lies in awareness. If no one knows about the disease, what use is a top-notch clinic at Stanford or Boston going to be?

This is one of the reasons that I created this blog in the first place. I was one of the lucky survivors who was diagnosed in time before a serious stroke. I feel as though it is my responsibility to attempt to prevent a tragic case of somebody suffering a paralyzing or deadly stroke simply because nobody around them knew about Moyamoya. Moyamoya is a deadly, progressive disease; it gets worse and worse over time without proper treatment, with strokes escalating in damage until death.

Although awareness is one of the key components of preventing unnecessary loss at the hands of Moyamoya disease, research for a permanent cure — something that does not currently exist — is crucial.

There is a Moyamoya research fund at Stanford University Medical School dedicated to finding a cure. I will post a link to donate at the top right of my blog next to “Contact” and “About”.

MRI’s and Me

From blood tests, to routine vaccines, to IV’s, there has never been a shortage of needles in my life.

When I had my surgeries in Stanford almost 10 years ago, I remember holding both of my arms out straight in fear that the two IV’s on each arm would snap inside me if they bent. At that time, having at least one IV was a regular ordeal, and I simply dealt with it. Back then, I had not really thought too much about them, simply a source of discomfort.

But ever since my surgeries, and the series of various needles following, I have developed somewhat of a fear of needles. Before getting an injection or my blood drawn, I would freak out — internally, of course — and play the scenario over and over again in my head before hand.

Around a month ago, I had an MRI done for a regular post-surgery check-up. I knew beforehand that I would need an IV for contrast, as I had been through the ordeal many times. However, several unpleasant experiences with IV’s still haunted me, and I walked into the prep room nervous.

Seeing the IV itself in the plastic wrap caused an involuntary tremor within me, and a familiar knot began to form in my stomach. As the spot of the injection was swabbed down, after I meekly informed the nurse that I would rather have it in my right arm, I had brief flashbacks from similar situations.

Despite the knowledge that it would not hurt, and the stinging sensation would be gone at within the minute, the psychological block remained. Something about the process, the lack of control, and the childish fear of even the slightest bit of unavoidable pain prevented the transcendency of the mental barrier.

This time, I can luckily say that, in retrospect, the actual MRI was the worst part. The cacophonous variety of sounds pounded in my ears for what felt like an eternity, while in reality lasting 40 minutes. The IV injection went smoothly, and the blast of relief that I felt following the quick procedure felt great. I immediately murmured something along the lines of “That wasn’t that bad”, and vowed to remember that, and internalize it.

Although I have not gotten my irrational, but deeply entrenched fear as of late, the recent MRI gave me a note of hope, that the annoying and gripping panic leading up to a simple injection would one day go away forever.

An Interview With Dr. Lori Jordan

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Dr. Lori Jordan is currently the Assistant Professor of Pediatrics, Director of the Pediatric Stroke Program, and Director of Pediatric Neurology Residency Program at the Vanderbilt University School of Medicine. She used to work at John’s Hopkins, where she diagnosed me with Moyamoya.

Dr. Jordan has been immensely kind to me and my family over the years, carving time out of her busy schedule to meet with me. She has had an immeasurable impact on my life, and I will always remain grateful for her help.

Over the course of the last month, I have been in contact with Dr. Jordan via email, and assembled somewhat of an interview with her. I also met with her in person for a checkup at Vanderbilt several days ago, and was able to ask a few more questions.

I wanted to have this interview to be more informed about Moyamoya, especially because it provided me the opportunity of learning from one of the country’s foremost experts.

Here is the interview:

Me: Regarding simple statistics about Moyamoya, (ex. How many people have it, what is the demographical distribution, what are several definitive leading factors, etc.) where should one look to find accurate and updated information?

Dr. Jordan: Finding up to date information is challenging for rare conditions.  I looked at many websites. Most hospital based websites have not been updated significantly in the last 5 years. NORD, the national organization for rare diseases is a good source.

Three good sources are below:

http://www.ninds.nih.gov/disorders/moyamoya/moyamoya.htm
http://www.childrenshospital.org/conditions-and-treatments/conditions/moyamoya-disease
https://rarediseases.org/rare-diseases/moyamoya-disease/

I tend to look at many sources and compare them. Probably the most reliable source for medical information is the New England Journal of Medicine. They published a review article on Moyamoya in 2009 written by Dr. Scott and Dr. Smith from the Children’s Hospital of Boston. Though a little older now, this is an excellent article.

Me: I know that you have been researching various aspects of pediatric hemorrhagic strokes, such as risk factors and outcomes, and I was wondering if you could elaborate on the goals and direction of your research, as well as any results that you have gathered.

Dr. Jordan: My early research focused on non-traumatic intracerebral hemorrhage (hemorrhagic stroke) in children, helping to understand causes of intracerebral hemorrhage in children as well as predictors of neurological outcome. Our research showed that hemorrhage volume is a major predictor of outcome, described a bedside method to assess hemorrhage volume, and reported the frequency of hematoma expansion in these children. A hemorrhage grading scale was developed for children called the pediatric ICH score similar to score that has helped improve care in adults.  We reported the incidence and impact of seizures and epilepsy after ICH in children.

With a team of collaborators, I have studied the neurological complications of sickle cell disease. Children with sickle cell disease (SCD) have a 100-fold increased risk for stroke compared with healthy children. Working in collaboration with hematologists, I served as a site neurologist for two multicenter stroke prevention studies in sickle cell anemia. I am currently the chair of the neurology committee for several stroke prevention studies in pediatric SCD, one based in the US in very young children and one based in Nigeria.

Finally, I collaborate with neuroimaging researchers to study of novel MRI techniques to predict stroke risk in children and young adults with sickle cell disease assessing cerebral hemodynamics and cerebrovascular reserves.  These are my main grants right now.

Me: What are some general statistics on how common Moyamoya is within the population?

Dr. Jordan: A study that analyzed hospital discharge data from Washington and California in the western United States found an estimated overall Moyamoya incidence of 0.086 per 100,000 people per year, lower than in Japan.

Among ethnic groups in California, the Moyamoya incidence rate for Asians was 0.28 per 100,000 people per year, similar to the incidence in Japan. The incidence rates were lower for blacks, whites, and Hispanics (0.13, 0.06, and 0.03 per 100,000, respectively).

**Incidence is new cases of Moyamoya.  Prevalence is all the people known to have Moyamoya (not just new cases. The prevalence of Moyamoya is 3.2 to 10.5 per 100,000 people.**

Most people with Moyamoya are diagnosed between ages 5 and 14 years or in the 30s-40s.

 

 

 

 

A Lot to Be Thankful For

This year, my family and I drove down to Maryland to visit my Grandma, Grandpa, and cousins. I had a great time, and especially enjoyed spending time with my Grandparents and Maryland cousins, neither of which I see often during the year. I also played with my two baby cousins, both cute little boys at ages 2 and 4.

Having quality family time this Thanksgiving made me truly appreciate how lucky I am. Nobody from my Dad’s side of the family had passed away yet, and all were healthy and happy. Looking around the table at a lively, charismatic, and loving family at dinner on Wednesday — a delicious, highly anticipated crab feast — gave me a deep sense of gratefulness.

I am most thankful for my family’s supportive and fiercely loyal nature. During the undoubtedly roughest point in my life, when I was diagnosed and treated for Moyamoya disease, my family stood with me. My uncle helped spend time with my siblings while my parents — both sources of happiness and good in my life — flew with me to Stanford. My Grandpa, then the head of the Anesthesiology department at John’s Hopkins Hospital, allowed my case and MRI’s to be seen by one of the country’s leading experts on Moyamoya, Dr. Lori Jordan. The rest of my family, as well as my friends, provided immense love and support for me during my surgeries and treatment, and made my experience with Moyamoya immeasurably better. Without them, I would not be who I am today.

Not to say that Thanksgiving is the only time during the year to be thankful, but it certainly brought to my attention all to that there is to appreciate. I tend to forget about and take for granted things that I should be grateful for, and things that I rely upon every day. In the midst of a fairly busy and preoccupied life, it is easy to ignore the factors that contribute to success.

To my family — especially my beloved Mom and Dad — and my friends that support and love me, thank you.

 

 

 

Great News for the NYC Community

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This month, NewYork-Presbyterian hospital, with the help of Weill Cornell Medicine, Columbia University Medical Center, and the Fire Department of New York (FDNY), has launched a Mobile Stroke Treatment Unit (MSTU). This will be the first of its kind on the East Coast, and will be deployed and utilized through the New York’s 9-1-1 system.

There has long been a need for something of this sort, a way to minimize damage done by strokes. According to Matthew Fink, M.D, the Chairman of the Department of Neurology at NewYork-Presbyterian’s Weill Cornell’s Medical Center, nearly 800,000 people in the US will have a stroke. MSTU’s are vital in stroke cases due to their speedy treatment services.

The MSTU’s in NYC will be run by two paramedics, a CT technologist, and a neurologist. The vehicle will be equipped with a CT scanner, that wirelessly transmits the images to a neuroradiologist. The MSTU’s will be efficient in treatment, and effective at minimizing the time between a stroke starting and treatment. The time saved be a MSTU — rather than being transported to the emergency room after the stroke has hit — can be the difference between a healthy recovery, paralysis, or death.

When I found out about the launch of this initiative, I was overjoyed. There is a real need for a function of this kind in the city, and one that I sympathize with. Although I have not suffered any serious strokes before and after my surgeries, it was very lucky that that was the case. If I had been misdiagnosed, as I was the first few doctors visits, I would most certainly have suffered a stroke.

This unit will be crucial for people like me who have Moyamoya, but also for anyone who has a stroke. What surprised me and pleased me the most about this discovery was that there was this much attention drawn to the stroke community and stroke awareness. I believe that this is an important milestone for the community and one that could have a positive impact.

Check out these websites for more info!

http://www.emsworld.com/video/12268181/mobile-stroke-treatment-unit-launches-in-new-york-city

http://www.ems1.com/ems-products/ambulances/articles/134147048-FDNY-launches-first-mobile-stroke-ambulance/

http://www.nyp.org/news/NYP-Launches-First-Mobile-Stroke-Treatment-Unit-on-East-Coast