I had written this a while back for a magazine, but alas they opted not to publish it so I thought I would share it with you all.
The day that changed my life was June 12, 1995, a Monday. The phone rang at 5:50am, which is never a good thing. I managed to roll over with my 8-month pregnant belly and answer the phone on the second ring. The voice was familiar but panicked it was my mother. “She is not breathing… your father is on his way…we called 911… the kids are there watching this… I do not know if she is alive… ”. Adam, my husband of 5 years walked in the room as I sprang to my feet with a look of confusion and panic and he knew it was bad news. “Mom we are on our way down we will meet you at the hospital.”
I grabbed one of my husband’s white dress shirts and began to dress, I recall not being able to button it and he helped me. I looked at him and said “Lori, she is not breathing, dad is on his way.” I pulled on pants as I walked through the house with my mind racing. We lived in Sussex County, NJ about 45 minutes from the family home in Rockaway NJ and the hospital was a little further away in Denville. We tossed some food down for the dog and cat while I brushed my teeth and we ran to the car. We were on our way before 6am. The drive seemed to take forever that morning. It was 1995 the days of no cell phones or internet access in a car; we had no way of knowing what was waiting for us at the hospital.
I recall walking into the all too familiar emergency room. My sister was in a critical care bed with a breathing tube, ventilator and cardiac monitors, she looked peaceful too peaceful… I panicked and said “what is her condition?” . “We got her back,” said a voice “Back?” I replied in a stunned voice. All these years later, there are moments of this day, this week that I can recall as clear as looking out my window today. “BACK?” I can hear myself say the word. “Back”, from where and would she leave us again?
A few weeks before this day my sister asked me something, at the age of 36 she looked worried and very serious. “If anything ever happens to me will you take the kids?” She was divorced from their father, a less than responsible individual, and recently remarried to a man with 2 children of his own. I said, “of course, I love them and would never let anything bad happen to them ”. I was 26 years old at the time and a few months pregnant, who would ever suspect that you would ever have to act on that type of a promise. So now, here I stood at the foot of a hospital bed, discussing transport to a trauma center level hospital and wondering when my sister would wake up so I could yell at her for scaring the hell out of us all.
The family all met at the hospital later that day after she was transferred to Morristown Memorial. We were in waiting mode. The doctors advised us to wait for her to wake up to see if there had been brain damage. Watching my niece and nephew ages 10 and 13 sitting at their mothers bedside scared and unsure of anything was just about the most heart wrenching thing anyone could watch. Then reality started to creep in… this scene is not new to my family it’s happened before… how many more would suffer the same fate? Am I next?
In 1953 my grandfather suffered a sudden cardiac arrest at his home at the age of 43 and my father, then 17 yrs old, attempted to do chest compressions to save him, it failed. In 1990, my uncle, age 47, died suddenly while at an event a few hours from his home in Montana and now my sister was laying lifeless in a bed hooked up to machines this time Dad’s CPR worked and we have her back – but was it enough? We all had the same condition – and some of us knew it, others would learn later. We have hypertrophic cardiomyopathy, HCM (in the 1970’s it was called idiopathic hypertrophic subaortic stenosis, IHSS) – in short it is a genetic conditions that causes the heart muscle to become thick and stiff and can cause sudden cardiac arrest. I was diagnosed when I was 12 and had already had a stroke in 1990 weeks after my wedding due to complications from my diagnosis. My niece was diagnosed at 8 and I was carrying a child who had a 50/50 chance of having it too. My nephew’s status was unclear. My father was diagnosed in his late 50’s.
The next 4 days were surreal and heart breaking. There were moments that were gut wrenching – like when my sister Lynn and I took Lori’s jewelry off of her to keep it safe while she was in ICU, we each put on a ring and swore to wear it until she woke up. Then there were lighter moments like bargaining with Lori to wake up – you see Lori had gone grey very young and I had always told her to color her hair to make her look young, she would not do it. So I stood bed side saying things like “ya know if you don’t wake up soon I am pulling out the hair color and lord only knows what you will end up with”. For brief moments it made us smile. We bathed her, we watched, we waited… nothing really changed.
My sister and I had been involved in medical research to learn more about the condition we had in hopes of finding answers to serve the rest of our family. We thought we were in the best hands, it turned out that the best hands had been let go by the institute, the replacement had little experience in our condition, and lead many people astray. On June 12, 1995 I had no idea of this truth.
Wednesday June 14, 1995 the doctors wanted to do an EEG to measure brain activity. There was none to speak of, artifact at best they thought, but let’s give it time they said. We met as a family with the neurologist and discussed the extend of the brain damage – in short there was less than a 5% chance at any meaningful recovery and we would know more with a confirmation EEG in the morning. Lori was unable to breathe on her own and much of her brain was destroyed by the lack of oxygen and length of time to defibrillation. We all knew she was gone, but we all wanted more time. We discussed the option of her saving the lives of others, and we all opted to permit organ donation should she be a suitable candidate.
The rest of the day passes with no change and we arrived at Friday morning June 16. The final EEG is done, there is not activity at 4:36pm her cardiologist walks in and checks her vital signs and does the most compassionate thing I have ever witnessed to this day – he leaned over and kissed her forehead and cried. I walked back into the room after he left. I closed the blinds.. took my sisters hand to my belly and begged and pleaded for her to wake up! I begged for her to see her niece just once, I made deal with heaven and hell and in the end I just cried. It was about 30 minutes later that I realized that the reason the doctor was there was to pronounce my sister legally dead and to begin the process of organ donation. My sister was dead.
Her death lead me on a quest for knowledge to learn all I could about this condition that had claimed the lives of, to my knowledge at that time, 4 family members. I began to ask questions, to dig, to not take no for an answer… my life and the lives of those I loved were on the line and I had lost enough. Damn it I was angry I had lost my sister and a best friend. My digging lead me to learn about other specialists in my condition and that the Doctor we had entrusted was actually not as well regarded as I had been lead to believe. In a strange twist of fate a man I had worked with told me one day in 1994 that his niece suffered from a rare heart problem and it turned out to be the same condition as ran in my family. I had given the name of our doctor to his family and they too had sought care with this man. In November 1995 this little girl, Jennifer, also passed away at the age of 8. First Lori, now Jennifer… who else?
I reached out to reporters to help me learn more and found an amazing man who was at the time working for the Wall St. Journal and a wonderful reporter Michael Moss (now you all know him for the book SALT SUGAR FAT How the Food Giants Hooked Us)– over the next 6 months I dug, read, learned and cried more than any one person should. On the one year anniversary (to the day) of my sisters’ cardiac arrest the largest article ever written in the Wall St. Journal on a medical topic appeared on the front page and my family filed a law suit against the United States of America, because the doctor we trusted was an employee of a government agency. During this same time I became frustrated with the lack of information available for patients with my condition. Then a conversation that I had with my sister a few months before she died came back to me – she had said we should have a support group for people like us. I was never the “support group” kind of person, but I did think a hybrid support and education organization could do a great deal of good. In February 1996 I filed for non-profit status of the Hypertrophic Cardiomyopathy Association and launched our first website shortly thereafter. While the beginning of the organization was nothing more than a website and a few newsletters it soon grew as did my little girl, now 18 and a college student with aspirations to become a nurse. Our first annual meeting took place in 1997 and has grown each year as has our website. To date the HCMA has helped over 5200 families learn about their condition, make wise healthcare choices, educated physicians, families and the public, helped 1000 patients access heart surgery and a similar number access lifesaving ICD therapy. We have created risk assessment tools and hands on approaches to identify those at risk for sudden cardiac arrest in time for action.
I am co-author of A Guide to Hypertrophic Cardiomyopathy for Patients, their Families, and Interested Physicians, now in its third edition. I have also been published in leading medical journals and trade magazines. I have been quoted in hundreds of publications including TheChicago Tribune, The Washington Post, The New York Times, Wall Street Journal, Newark Star Ledger, Sports Illustrated and Men’s Health and has appeared on CBS Evening News, NBC Evening News, News12 NJ, ESPN among many others. I have been as a consultant for the popular television series “Strong Medicine” and for various writers in need of factual HCM-related story lines.
I currently serve and have served on many panels and councils and represent the HCMA in several coalitions. My affiliations include the American College of Cardiology, Heart Rhythm Society Conference on Device Performance, Sudden Cardiac Arrest Coalition, Genetic Alliance, Personalized Medicine Coalition, Coalition for Imaging and Bioengineering Research, National Coalition for Heart and Stroke Research, NHLBI Constituency Group, and American Heart Association Community Strategies Council (North East Region)
So when I look to the day that changed it all…. It was June 12, 1995 and it took me many years to fully understand that my sister did not die without leaving her mark on this world. Each and every person who is helped by the HCMA is helped not because my sister died, but because she lived. There is an expression some call it the chaos factor – a butterfly flaps her winds in Europe and its wind can create a hurricane across the globe – My sister Lori is that butterfly and the winds of change are here – it’s time to not let our loved ones die when options are available to protect them. And yes I am still wearing Lori’s ring.
Lisa – Your Bighearted blogger