by Myra Christopher

book-signing-225Last Sunday, I had the privilege of speaking at a book signing in Kansas City.  I had not met Carolyn Johnson, the author of Sickle Cell Disease and My Life Struggles, until then, but I know her brother, Jonathon, pretty well.  Jonathon is a member of the PAINS Citizen Leader Advisory Group.

This group is made up of about 50 people in our community who live with chronic pain and/or their family members. By sharing their experiences and working with us to incorporate the challenges they face into our efforts to “transform the way pain is perceived, judged and treated”, they provide a valuable service to the Center for Practical Bioethics and to the PAINS initiative.  Members of the Citizen Leader Group have not only been advisors; they have also worked with us on grant development, served as spokespeople with the media, and participated as faculty in educational programs focused on chronic pain.  Jonathon has done all of the above as a volunteer; so, when his sister called to ask me to speak, of course, I quickly said yes.

book-signing-224As Sunday progressed, however, the wind began to blow, the temperature dropped and it began to sleet.  I must admit that when it was time to leave my home, I was not entirely enthusiastic about doing so.  But I did, and I am so grateful that I did so because I saw a room filled with family and friends who understand chronic pain in a way that most of us never will and because I had one of those “aha!” moments which I often have when working with people living with chronic pain.

Only 70-100,000 people in the US live with Sickle Cell Disease.  Another 3,000,000 have the Sickle Cell Trait.  Sickle cell is a rare inherited blood disorder.  I learned from Carolyn’s book that there are several “types” of sickle cell problems, but they are all awful – frequently causing chest and joint pain, various infections, renal failure, cancer and other problems throughout one’s life.  Pain is of special concern for those who live with Sickle Cell Disease.  The National Mortality Study for Sickle Cell Disease some years ago demonstrated that the number of pain crises experienced by patients is directly correlated with survival.  Although life expectancy has been increasing because of improvements in care, sickle cell patients who have three or more crises in one year are at risk of dying.

One aspect of Carolyn Johnson’s book that gives me hope is that when hospitalized in institutions that are knowledgeable about her illness, rather than being treated as a “frequent flyer”, “drug seeker” or “malingerer”, she has experienced compassion, quality care and improved outcomes.  I believe this is true for all those who struggle with chronic pain and complex illnesses that include chronic pain and want to believe that we are on the precipice of positive and dramatic change as we move toward a patient-centered, bio-psychosocial approach to chronic pain.

In the US the prevalence of Sickle Cell Disease is approximately 1:5,000.  However, among African Americans the prevalence rate is 1:500.  Although Sickle Cell Disease is historically very important because it is one of the first diseases which was shown to have a genetic basis, there has not been a lot of research and drug development to address this disease.

However, in 2014 a Phase 1 Gene Therapy Clinical Trial was launched. With the coming of personalized or “genomic” medicine, it is the hope of many that new treatments and therapies will be available to those who struggle to live with so called “orphan” diseases.

My epiphany came yesterday when I realized that I was among four generations of a family that have struggled with this terrible disease and chronic pain associated with it.  When one of the speakers talked about the importance of testing before choosing a partner with whom to have children, I got a glimpse into the complexity of this disease that many others living with chronic pain will never have to consider.

Among those present yesterday was a precious little girl only a few months old.  Despite having an NG tube taped to her nose, I have never seen a more beautiful little face or prettier smile.  She is related to the author who said that she prays that, if this baby lives, her life will be easier than it has been for others in her family.  We know full well that when even one family member lives with chronic pain, an entire family can be devastated.  It appeared to me that this family which has lived with illness and chronic pain generation after generation has figured out how to remain intact in spite of all the challenges Sickle Cell Disease presents.  I think we have much to learn from them.