Personal stories of courage and fear living with sickle cell disease

     Doctor Marjorie Dejoie was diagnosed with sickle cell disease in her early twenties, when she was a medical student at The University of Pennsylvania.  She is currently the Medical Director of The Sickle Cell Disease Association of America, Philadelphia/Delaware Valley Chapter. (Kimberly Paynter/WHYY)

    Doctor Marjorie Dejoie was diagnosed with sickle cell disease in her early twenties, when she was a medical student at The University of Pennsylvania. She is currently the Medical Director of The Sickle Cell Disease Association of America, Philadelphia/Delaware Valley Chapter. (Kimberly Paynter/WHYY)

    Dr. Marjorie Dejoie uses fitness training and nutrition to fight sickle cell disease and to help coach others through their own symptoms. Dejoie reached out to her contacts and colleagues for commentaries about their personal experiences with the disease. 

    September is Sickle Cell Awareness Month. This the third in a series of three posts about sickle cell disease (SCD).

    Dr. Marjorie Dejoie, the medical director of the Philadelphia and Delaware Valley chapter of the Sickle Cell Disease Association, was born with sickle cell disease in 1971. She uses fitness training and nutrition to fight SCD and to help coach others through their own symptoms. Dejoie reached out to her contacts and colleagues for commentaries about their personal experiences with sickle cell disease. The following are excerpts from their remarks.

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    ‘I worry about her every day of my life.’

    I became associated with the Sickle Cell Disease Association and how this terrible disease affects millions of people. Some of the issues are, but not limited to, weakness, severe migraines, paleness, total exhaustion, and exquisite pain throughout the body. When patients get attacks they are usually bedridden for days sometimes a week. Recently, there have been instances where patients have lost fingers and limbs just to stay alive.

    One woman, my dear friend, has a constant ringing in her ear, which to this day she has not been able to get rid of. To make matters worse, the average life expectancy for someone who has sickle cell is the mid-40s at a time when the national average is approximately 78. My friend is 43ish, and I worry about her every day of my life.

    To add insult to injury, to there isn’t any cure. Also disturbing: My research has found that this disease was once thought of to be found in black and Turkish people only. However, there is now evidence that it also affects Hispanic people.

    I am only one person but I try to comfort my dear friend. I attend sickle cell charitable functions. I make donations to this charity, and most importantly, I pray for a cure.

    —Rob Truck, partner with Samantha Associates and president/CEO of Logistical Outsourcing Group

    The importance of compassion and understanding

    My best friend of 16 years has sickle cell disease. I am also a social worker and have worked in both adult and pediatric hospitals. It has been an interesting journey over the years both from a personal perspective and as a professional. Admittedly, at times it is hard to separate the two.

    Personally, of course, it is difficult to see someone you care for in any type of pain. Your reaction is to try and remove it, fix it or simply wish it away. I have had to do a lot of self examination to move away from my own concepts of what should and should not be done to care for people, realizing that to support and offer space for someone means to accept their ideas of care and help.

    In my professional life as I have grown, and as my understanding has grown, working with individuals and how they manage pain can really only come from a place of compassion. Working with patients is to advocate in all ways possible.

    —Alyson B. Watkins, MSW, social worker at St. Christopher hospital

    Talk to people with sickle cell and open your eyes

    For over a decade, I have had a friend with [sickle cell disease]. I have noticed times when he hasn’t come on trips, and someone would casually say “altitude” or “not feeling well.” I have heard about him being in the hospital over the years. Sometimes I have called, sometimes I have not. I knew some of the basics about sickle cell disease, but honestly, no more than that.

    Then I had a professional opportunity working with a pharmaceutical company investigating new sickle cell treatments. I am a market researcher with a focus in pharmaceuticals, so at first, it sounded like another opportunity — an exciting “disease state” that I had not worked in previously.

    To prep for the project, I asked my friend to tell me more about his experiences, and slowly my eyes began to open. He told me about the pain. He talked to me about the mistreatment in the ER. Despite his education, despite his professional successes, despite his beautiful family, he has been treated inhumanely by health care providers who have acted as though his life was meaningless.

    He shared experiences about being told he would be dead before he was 30 and, even though he has made it well past that age, all of the things he has missed out on because of his illness. And technically, he is considered to be have a “milder” form of sickle cell.

    I began working on my project, interviewing nearly 75 individuals living with sickle cell and another 30 health care providers and advocates. I have heard stories of abuse, despair, pain beyond measure, disappointment, depression and people feeling isolated in a world where society is so misinformed they think people with sickle cell are nothing but contagious, lazy, drug addicts.

    But I have also heard stories of triumph, hope, inspiration, strength, faith, courage and an amazing will to do more than survive — to live. I have fallen in love with some of the people I met, experienced a connection that I could not explain, other than to say I was drawn to their spirit. Now, I not only want to keep the client happy with a job well done, I want to push, pull, lead and tug this client and anyone else to help people living with sickle cell. They deserve better.

    Just like ALS patients and women with breast cancer and babies born into incubators, they deserve compassion and fair treatment and media attention and research dollars. They deserve a place on the national and international health agenda.

    So now, I am trying to do my part. Hopefully my work makes a difference and helps a big pharmaceutical company do more. Hopefully being able to share their story with someone who cared enough to listen helped someone with sickle cell sleep a little better tonight. Hopefully telling my kids about sickle cell, so they grow up to be more compassionate and understanding than if they were still ignorant of sickle cell disease will make a difference. And hopefully, I am a better friend.

    — Nishea Clark, NVC Consulting LLC

    Ideal health depends on mind, body and soul

    As a person living with Multiple Sclerosis for over 15 years, I was looking for help in maintaining my overall health. What struck me most when I first started to train with Marjorie DeJoie was her level of stamina and energy! Marjorie did not fit the image of those with sickle cell disease. She was not hindered by multiple medications and rounds of steroids to manage the pain.

    Marjorie taught me the use of supplements and vitamins, what vegetables and foods to use, and what types of exercises would support my body. We used massage therapy along with acupuncture to manage symptoms when going through rough periods. Her plan was not just what I could do with exercise, but rather we looked at our work together from a holistic perspective, realizing that the mind, body and soul has to be engaged for ideal health. This was in alignment with my own work with people who have mental health and medical disorders and what makes for the best treatment options.

    Working with Marjorie has helped shape my work. When addressing the complications of mental health and medical conditions with physicians, social workers and nurses, I see a great opportunity to share how others are living with illnesses such as sickle cell disease.

    —Carol Bylan, Psychiatric RN, Hahnemann Hospital

    Diversify the blood supply to help children with sickle cell disease

    With teary eyes and my heart racing, at the age of 11, I witnessed my older cousin Betty doubled over in a fetal position attacked with sudden, excruciating pain while screaming, gasping for air and pounding the floor with her fists. I was abruptly removed from playtime with Betty’s daughter as we ran to the phone and called a relative for help.

    My aunt finally arrived at Betty’s home along with the ambulance. Calming the panic that almost overtook me, my aunt held me in her arms as neighbors gathered and watched the medical team gently lift Betty onto the stretcher and into the emergency vehicle. Frightened by the ordeal, yet too numb to cry, I was confused and plagued with questions wondering what just happened.

    That was my first and last time witnessing a sickle cell crisis.

    More than two decades later, I currently serve as program manager of community outreach for the American Red Cross Penn-Jersey Region, advocating for patients who need blood to live, including those with sickle cell disease. The Red Cross provides dedicated blood transfusion support for over 2,000 pediatric patients with sickle cell disease. Through community outreach efforts, the Red Cross collects approximately 18,000 pints of blood each year to specifically support sickle cell patients.

    Our blood supply must reflect the diversity of our community to best meet the needs of hospital patients. Sickle cell disease is a genetically inherited disease and genetically similar blood is best for those who need repeated or large volumes of blood transfusions for illnesses like sickle cell and other blood diseases.

    —Qiana Riley Cressman, program manager of community outreach, American Red Cross, Penn-Jersey Region

    To learn more about the American Red Cross Sickle Cell Donor Program, please email Qiana Riley Cressman.

    The Annual Walter E. Brandon Sickle Cell Walkathon, in Fairmount Park on September 27, 2014, is a major fundraiser sponsored by the Sickle Cell Disease Association of America, Philadelphia/Delaware Valley Chapter (SCDAA/PDVC) and AFSCME District Council 33. For more information, and to register, visit the SCDAA/PDVC website.

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