Tuesday, 28 March 2017

ALS, in the words of Judy Starritt

This is an address which was delivered by PAL Judy Starritt to Dalhousie Medical Students in 2015. At the time Judy’s voice was weak so the address was spoken by her husband, Cameron Starritt. Rumor has it that Judy did very well animating her words while Cameron delivered it.  Judy continues to be an inspiration to all who meet her.  Thank you, Judy, for letting us share your story:

I was very pleased to accept the invitation to be here today but was quite amused when I read Dr. Gordon's email that said he would like me to address the students at the beginning of the panel. I was amused because I find it difficult to whisper even for a short time now. That is what happens when you have bulbar ALS as I do.

So I immediately thought of a Plan B and this is it. My husband, Cameron, will speak my words. Over the years, many, many times he has had to listen to me, rambling on and I have often spoken for him.  He has definitely become more outspoken since I have had ALS.

Let me first tell you a little about my background. Something you should know about me is that I am a retired high school math teacher and an official math nerd. This fact gives me an advantage over many others. I know who you are! My favorite class to teach was Pre-Calculus and you were the people that challenged me to be the best I could be. I knew I was just a stepping stone for you but I wanted to give you a firm foundation for what would lie ahead of you in the future.  The challenge of pre-cal was to bring in unexpectedly the topics that you had covered in previous years of math. I would sneak them in wherever  I could. I would spend hours making up questions that would expand your brain. You would finish them in 10 or 15 minutes. Then I would look forward to see how you answered them. We would share with the class the various approaches that were given. You were the students that, when you got 97 on a test, you looked for where you went wrong and vowed never to make that same mistake again.  Some of you made stupid mistakes like saying 2 times 3 was 5 and wondered how you could possibly have made that mistake. Some of you came for extra help to see if you could glean just a little something more. Some of you never came to extra help! That's OK too.  Most of you are the ones that did your homework! You were the ones that kept me on my toes, mathematically speaking .  All of you questioned and have continued to strive for excellence. It is not an easy journey getting to where you are sitting today. You, in my eyes, are the best of the best.

Here is something else you should know about my life. I have lived a truly blessed life. There hasn't been a day in my life that I have not felt loved. Family and friends mean everything to me. I lived a very healthy, busy life and didn't take this for granted. I knew I was blessed. Two years ago in July I woke up with a swollen tongue and I spoke with a lisp. It didn't get better. My voice would get weaker and weaker as the day went by and by the end of the day, I found it hard to talk at all. I began my quest to find out what was wrong. First I went to my doctor, took antihistamines in case it was an allergic reaction, had a Cat scan, got referred to a neurologist, and was warned it could take 6 to 9 months to see him. My family and friends were becoming very concerned and finally, in December, I met with the neurologist who administered an EMG test and I had a private MRI done that resulted in me being sent to Dr. Timothy Benstead for a second opinion on February 28, 2014.  By then, other things were beginning to happen: shortness of breath, a tightening in my diaphragm, the feeling that my energy was slowly draining from my body. I knew there was something wrong, seriously wrong, but I still didn't suspect ALS. You see, I didn't go on the Internet and self diagnose myself as so many people do. When I heard the first neurologist say that my tongue wouldn't be swollen if I had ALS, that the opposite occurs, I had ruled out that possibility. After all, I was an optimist. But after the appointment on February 28th when Dr. Benstead said that I definitely had a neurological disease and further testing would have to take place, I did go home and go on the internet and my heart began beating faster and anxiety began to creep in. My symptoms mirrored those of ALS. We looked at the symptoms of MS and hoped that might be the problem. Research had shown that the older you are when diagnosed, the less severe MS can be, according to one study. On March 26th , after further testing by Dr. Benstead, he told us in a very compassionate and sensitive way, that I was in the very early stages of bulbar ALS. We left the hospital numbed, shocked, gob smacked.

The next 3 months were ones of constant adjustment. My future had just imploded. Severe twitching began in my muscles, especially in my legs. Breaking the news to our family and friends was difficult. How do we tell our three sons? How do I tell my 90 year old mother? It was done in as gentle a way as possible. That was hard. Cam began searching the web for information and found some alternate  forms of treatment. Dr. Benstead had mentioned a drug, Riluzole, that could be taken to lessen symptoms in patients with bulbar onset of the disease by 3 to 6 months. After clinical trials, it was first approved for use in the US in 1995. It was discouraging to know that nothing had been found to help in the last 20 years.

And so I began living my new life. Looking back, there are three words that summarize my new journey. The first is ACCEPTANCE. This was a totally unexpected road I was traveling. But I was not alone. When I had to tell this devastating news to my family and friends. I knew that how I handled this was important to pave the way for this new road ahead. I made up a motto for myself and shared it with others, what I have, what I am and what I want. “I have faith and hope, I am optimistic and I will find joy every day.” I  had to repeat it to myself several times during each day until I began to believe it myself. I looked for joy- “unexpected happiness” and began to find it in the most unexpected places.

I had been told by Dr. Benstead that the ALS Society would be a wonderful resource for information and support but it took me almost four weeks before I made the initial phone call to them. It was Easter Monday but Kim Carter, the ALS CEO was in the office and told me to come in . She would be there all day. I went. The office is on the left as you come off Magazine Hill before you get to Akerley Boulevard, a 15 minute drive from my home in Beaver Bank. I spotted the sign  ALS NS and the slogan “Care and Hope”. I went in. I talked for two hours! I so wanted to let her know who I was before I “changed”. I told her I didn't want to be known as the ALS lady. I was already the Angel Lady. You see, I had started a Nova Scotia branch of African Aids Angels in 2009 making angels as a thank you for donating $10 to children affected with AIDS in sub-Saharan Africa. We have raised close to $24 000  since then. I told her I had promised  my friends that they would not have to participate in a walkathon because we already walk in support of ovarian cancer, a cause close to our hearts because our dear friend, Cathy, lost her battle to this disease. I did allow Kim to talk too!! She was calm, reassuring, comforting, smiling, knowledgeable and said exactly what I needed to hear. She told me everyone has their own journey with ALS and gave me a binder of all the information, solid information that we would need, that answered questions and gave us contact numbers and reliable research information. She told me about the loan program that provides equipment to ALS patients. The ALS Society works hand in hand with the ALS clinic at the Rehab (I would be going there every six months for assessment and re-evaluation) and the ALS Society provides the necessary equipment to meet the patient's needs through Terri Cooper who is in charge of Client Services. Kim told me about Dr. Victor Rafuse who is the director of the Brain Repair Centre at Dalhousie University and very involved with the activities of the ALS Society.  She talked about the group PALS, People living with ALS, that meet every second Tuesday of every month and invited both Cam and I to attend. One of the members, Mike Watson, has had bulbar ALS for over 12 years. I came away hopeful and comforted in the knowledge that they were there for me and others like me.

Meanwhile at home, Cam was fast tracking plans for our trip to Europe that we were planning to take in the fall but booked in May instead. We guessed we would have the summer but who knew after that. I would begin taking Riluzole when I got back. Before we went on our trip, we went to our first PALS meeting. It was our first time seeing people in the more advanced stages of the disease. They were very helpful but I left, knowing a line had been crossed that day. 

Upon returning home on May 19th from Europe I had many more questions to ask, especially about what I should be eating.  I was always a healthy eater but now I took it to extremes. I was fearful if anyone would have a negative reaction to a drug, it would be me.  I have always been sensitive to chemicals. I was having difficulty swallowing at times. Later I attributed a lot of that to my anxiety, my not knowing what to expect or when to expect it. I was losing weight. I had an appointment with a dietitian who told me now was not the time to lose weight and told me to adjust my eating to include high caloric and high fat foods. She recommended I use a thickener in my fluids to help me swallow fluids without difficulty. Have you ever tried anything thickened with thickener? Gross is not an adequate word to describe it. No words are adequate. Was this what my future would be like?

I was becoming more anxious, especially since my three sons were coming home in mid to late June to see me. We have always been a close family even though we are spread from coast to coast here in Canada. I wanted to be my best. Instead, our oldest son, Tim,  arrived just two days before I was admitted to the hospital with severe dehydration and a resulting urinary tract infection. I was in the hospital for two weeks when my second and third son came home, plus two grandsons.  This is when my second word, CARE, really crystallized. In the hospital, I was immediately hooked up to an IV and within a very short time, was being cared for. Every nurse, doctor, medical student, nurse's aid, even the kitchen staff, bent over backwards to give me the best treatment possible. I had lots of time to lie there and count my blessings. I particularly enjoyed the young pre-med students that came trailing in after the lead doctor. It reminded me of a mother duck and her ducklings. “Do you mind if we discuss your case?” Hell, no! This was a chance to be part of the teaching process again. I was the unexpected. They thought they would be seeing a stroke victim ( I was in the 8.1 section of the Infirmary)  but instead, I was able to expose them to ALS. I learned to relax again. I realized my anxiety played a huge part in my body's response to my disease. Through further testing, it was found  that I could still swallow fluids. While there, I was given a PEG to accommodate a feeding tube. I was also introduced to Nutren, a supplement I now take twice a day but later will be be my only source of nutrients. My family visits filled my soul with happiness. I left the hospital SO thankful for the loving care that was given me. I had finally accepted my new path. I left with the added strength and knowledge that was needed to continue in my new life.

Before I get to my third word, I want to summarize what has happened in the last year. When I came home from the hospital, Cam and I got in a new routine and I began gaining weight and feeling much stronger.  By mid-August I had gained back half the weight I had lost and  when we went to Vancouver to see our family, Andrew, our second son, was shocked when he saw me walking towards him at the airport, looking SO much better. That was just our first trip. We traveled extensively. our last trip in June being a family trip to Hawaii.  I went parasailing with my son and two grandsons there. Heaven! Who would have guessed? Since our cruise in December I use a scooter to get around but still can walk short distances. I budget my energy and now I certainly talk less and listen more!! I cherish the time with family and friends with a new intensity and lots of laughter. As I got stronger last summer, I began to eat “regular” food and have recently discovered Miss Vickie's potato chips. I am aware of my muscles weakening and no longer can stick my tongue out at you. On a lighter note, I CAN still give people the middle finger...with a smile, of course. Changes are happening daily but slowly and that brings me to my third word that involves YOU and why I am here today.

My third word is HOPE. And where does my hope lie? In new research that hopefully can affect me and if not me, others. In my initial motto, I said I had hope but if you look at what is known about ALS, it IS a fatal disease. So is life! But there IS a big difference. 80% of the people diagnosed with ALS die within the first 5 years.  In my optimist math mind, I thought that that means there are 20% of people that live longer. Another article stated that 10% lived longer than 10 years. Hope was creeping in. I decided, “ I am going to be a positive outlier!! Why not strive for the best results?” But how can I get there? Not by myself. My attention became drawn now to what the researchers were doing. Quotes about brain research kept popping up everywhere now.

One article called How Canada's Remarkable Research Provides New Hope For Breakthroughs in an independent supplement by Mediaplanet to the National Post caught my attention.

Quoting “The current economic burden of neurodegenerative diseases is estimated to be $33 billion per year. Brain and mental health disorders, which already affect one in three Canadians, are the major health challenge of the 21st century." Later in the article -  “Ninety percent of what we know about the brain was discovered in the past 20 years”. And finally-  ”Overall, Canadian researchers rank fifth in the world for the high quality of their brain research.”

Another newspaper article caught my eye in the August 4th edition of the Chronicle Herald. It was titled  Scientists find genetic ties to Cerebral Palsy by Sheryl Ubelacker. Her opening paragraph reads ”Cerebral palsy, the most common  cause of physical disability in children, had long been thought to result from brain injury in the fetus. But new Canadian research is challenging that notion, finding that at least one in 10 cases likely have an underlying genetic cause.“

Second paragraph “Our findings were shocking, actually,” said principal investigator Stephen Scherer, director of the Centre for Applied Genomics at Toronto's Hospital for Sick Children. So ingrained. has medical dogma been around the root cause of cerebral palsy that “when I showed the results to our clinical geneticists, initially they didn't believe it,” he said.

The rest of the article was as fascinating to me as the opening paragraphs and the parallels between the research for cerebral palsy and ALS  were eye opening for me. 10% of  people diagnosed with ALS are of the inherited variety called Familial ALS. But there are so many differences as well, the biggest one being that cerebral palsy affects children from birth whereas ALS develops in adulthood. I was one of the fortunate ones. I had just turned 65 when I was diagnosed. I already had a full, complete life behind me. An estimated 50 000 Canadian children and adults have cerebral palsy. Approximately 2 000 to 3 000 persons in Canada currently have ALS.

The biggest similarity is that we share the same HOPE for the future. That hope is in the hands of researchers. People who are always asking “What if? ” 'I wonder why?” “ Why not?” “Could this be the reason?” The people who question the parameters and think outside the box. People who see problems and absorb them into their system, who subconsciously are always looking for solutions. They never know when a possible question might be answered or a new question formulated. Their minds are working 24-7 but on the outside, they appear “normal”. They look like you and me.

How many of you are sitting there, wondering if I am going to bring up the ice bucket challenge?  Of course I am!  I was blown away at the timing of the ice bucket phenomenon. It was starting to take off just before we went out to Vancouver to see our family. Before I left, I nominated  my son, Andrew, and our two grandsons, Findlay and Eamon, to do the ice bucket challenge but to wait until we got out there so we could watch. Andrew is a fireman and I knew he would do it in style. I was right. He rose to the occasion. Check it out later on YouTube under Hot Firefighter ALS, one of the 17 million ice bucket videos on YouTube. If you know Vancouver, my grandsons, their mother and our son, Tim,  took the challenge at the fountains in Queen Elizabeth 11 Park. On our way home Cam, Matthew and his lovely wife, Laura got wet and cold at their place in Toronto. By then, my whole family and thousands of others were dumping ice cold water over their heads. Awareness of ALS was growing exponentially (Great math question there! )  For me who had been struggling with this relatively unknown disease for the past four months it was “refreshing” for want of a better word to know that now so many people would be more knowledgeable and accepting of this disease. It was like ALS had just come out of the closet.  And the donations that began coming in to the ALS Society! In Canada, the final tally saw over $10 million raised through the ice bucket challenge going to research alone. Brain Canada matched every dollar raised so the end result was $20 million for research. The rest of the near $7 million raised was used by local ALS Societies to enhance the loan equipment program.  $220 million was raised worldwide. The ALS Society will continue these challenges every year under the heading “Every August til a Cure".

Are you wondering how much Nova Scotia and New Brunswick raised?  $775 000.  $415 000 of that went to research and the rest to the purchase of new equipment and to enhance their work.

Now, put yourself in the position of an ALS researcher. Until then, researchers across Canada shared about $2 million per year for research. The ALS Society is a non profit organization that is constantly raises money for both equipment and research. Since the unexpected awareness last summer, more and more donations are still coming in.

I began thinking of Dr. Rafuse, working here in Halifax on ALS research. I decided I had to meet him and offer myself as a guinea pig if necessary. I pictured him in an old fashioned lab leaning over a microscope with a red geranium in the window. On April 9th of this year, the ALS Society had their annual meeting at the Brain Repair building. A tour of Dr. Rafuse's lab was part of the agenda. I could hardly wait!! To my amazement, I found a huge modern building. When Dr. Rafuse came down and asked if anyone wanted a tour of the lab, I eagerly scootered behind him, boarded the elevator and was introduced to a modern, well equipped lab with a million dollar plus microscope that filled its own room! Facts were coming at me nonstop and the enthusiasm in Dr. Rafuse's confirmed we had something in common. He had the same kind of enthusiasm for research that I had for math! I didn't see a red geranium in the window but I did see my first real brain and spinal chord and the shared knowledge of some of the research that is being done around the world to find a cause and an eventual cure.

On August 8th, the ALS Society had a huge fundraiser called Bucket List Dreams Night. Several people including myself painted buckets and they contained various items to be auctioned off. I was the highest bidder on the bucket that was sent from Dr. Rafuse's lab. I now have a T-shirt with a large neuron on it, lots of lab related things, information I could pass on to you which would only make this speech longer, plus a day in the ALS lab with Dr. Rafuse. I have to finish writing this speech in order to start typing the questions I want to ask him.

Getting back to my journey, we were assessed to see what adaptations would have to be made to our home in the future. Like so many other diseases, ALS doesn't have a time line. It was recommended last October that we build a ramp before winter set in. Terri Cooper at the ALS Society said “Leave it to me” and within a very short time, we were contacted by The Ramp Project, a group of young people who gave their time and knowledge to built ramps for people with MS and ALS. This group was started because a young resident, Marie Claire, met an ALS patient who couldn't go home because she didn't have a ramp to get into her house. Marie Claire reached out to her friends and the first ramp was built. On Sunday, November 23, nine young people arrived and built us OUR ramp. Every day whether I am walking on it or using my scooter or just looking out my window I am thankful for the compassion of this lovely lady and her friends who came into my life.

When I was curious as to who you are, sitting here, listening to this speech and what road after high school you had to travel to get here today, I emailed her. Her reply boggled my mind. Quoting her, “The path to being a doctor varies- but to be in medical school you need to have finished a 4 year undergraduate degree. You need ridiculously high marks and you need to have aced an exam called the MCAT. Some people do additional masters degrees to increase the likelihood that they will be accepted. In addition to ridiculously good marks during your undergrad degree and on your MCAT - you need to have done a lot of community stuff - or have shown that you want to be a positive influence in the world.”

Then, to become a doctor, “minimum is 4 years undergrad, 4 years medical school and 2 years of family medicine.  Normal is more like me. 4 years of undergrad, 2 years masters. 3 years medical school (I went to the medical school that was 3 years with no breaks) and 5 years of residency.” Marie Claire also said, “Medical school is super challenging but the first years don't know that yet. They are just ridiculously thrilled that they are going to be doctors in 4 years.”

You have already started to climb the medical team's ladder. Getting here is just the first rung. Some of you will become family doctors who solve so many people's medical problems. Others will decide to study for another five or more years and become more specialized. Before ALS happened to me, I didn't give research as much thought as I have in the last year. I thought being referred to the neurologist would be my final step, my “last rung”. But so many of these specialists still have nagging questions. They want to give their patients definitive answers. As Dr. Scherer said in the article on cerebral palsy, “I can't imagine having a child who's got a disorder and not having some type of an explanation as to why. So I think it's pretty exciting that we can actually provide for these spontaneous cases that the parents didn't do anything wrong. They didn't make a wrong decision during pregnancy or eat the wrong food or expose the fetus to the wrong thing. It was just a random genetic change.” Research provides an “extra rung” to climb. The proven knowledge that clinical studies provide filters down through the medical profession and to all of their patients.

An excellent article by Aaron Hutchins in August 6th Maclean's online magazine A cold day: The ice bucket challenge, one year later  quotes  Dr. Lorne Zinman, a neurologist and director of the ALS Clinic at Sunnybrook hospital in Toronto. “The huge influx of cash has brought forward ideas that researchers previously balked at even attempting—like stem cell transplantation— because they figured trials would be far too expensive for approval. But with the ice bucket challenge money, anything feels possible. ALS Canada has already approved a $500,000 clinical trial for pimozide, in hopes that the anti-psychotic drug proves that it strengthens where motor neurons connect the brain to muscles, thereby allowing muscles to function longer.”

I will leave here today and continue to live my life to the fullest. I have discovered I haven't changed, just my body has changed. I am more intensely aware of both the changes in my body and the joy that surrounds me. I am SO loved! The man of my dreams I met while going to Acadia University is still beside me and I love him more each day. I plan to get my handicap sticker that has an expiry date of July, 2019 renewed. That will  put me in the top 10%. I will volunteer to be included in clinical trials and pray that I won't get the placebo. I will pray that in my lifetime, ALS will become treatable, not terminal. I will participate in the ALS walkathon next year. I will be both the angel lady who has ALS and an ALS spokesperson that makes angels. As my body changes, I will be the same old me.

I love quotes. This is one I found in a National Geographic magazine: “There are more neurons in your brain that there are stars in the Milky Way”.

From the deck at out cottage in Bass River there is an unrestricted view of the Milky Way on a clear, starry night. Last summer I imagined that every twitch that I felt in my body was a star in the Milky Way being turned off. But since visiting the lab in April, I have another analogy. As the last researcher turns off the lights in the lab before going home on this side of the world, there is another researcher on the other side of the world, probably with a cup of coffee in her or his hand, turning on the lights of their lab to begin their day.

So many people with stories of their own, so many support groups providing funds and care, so many researchers trying to find answers.



  1. what a wonderful story Judy! Love the words you choose to live by! Sending loving energy your way, one PAL to another!

  2. Always an inspiration...from the moment I met you. I think of you often. Wilma C

  3. Always an inspiration...from the moment I met you. I think of you often. Wilma C