SCD-- Where They Are Headed and What They Are Leaving Behind ( A Series Of Discussions)

My sister in law, who happens to be President & CEO of The Sickle Cell Disease Foundation of California was in town attending "The Second National Conference on Blood Disorders in Public Health".  After talking to her my question was, where was SCD research headed and why was there no studies showing advances in Stem Cell or Bone Marrow transplantation.  Her reply was SCD is going Global.  That's where the action is; "New Born Screening".  Consequently, in this posting I will talk about an abstract presented during this session and what it means to adults with SCD.

ABSTRACT #1 (BS8-5)

Clinical Predictors Of All-Cause In-Hospital Mortality In Patients With Sickle Cell Disease in United States-First Reported Results From A Nationally Representative Sample.

RESULTS

In 2007, a multi site study of 166,084 admissions with a diagnosis of SCD was conducted. Males represented 37.5% of SCD related hospitalizations. There was a total of 844 deaths reported in the above admissions with an all-cause in-hospital mortality rate of 0.5%. Of these, 420 (49.7%) were females. These are astounding odds that SCD patients face when they are admitted to the hospital either through pain crisis, acute chest syndrome, and or development of sepsis. Intubation, as a cause of death occurs primarily after the patients organs began to crash in ICU.  In conclusion, the authors propose that early and targeted aggressive therapy based on the presence of these factors should guide the management of hospitalized SCD patients for improved mortality.

MY COMMENTS

As I write this, it makes me wonder why so much money, time and effort is put into research that we already know the answers to.  Fifty years of studying SCD has yet produced viable outcomes or protocols for hospitals to use in assessing patients with SCD.  It pains me as one who's had all of the above diagnosis; death still affects about 50% of those patients admitted with these diagnosis. By the grace of God and the relationships I have with my doctors allows me to post this blog today. 

One of the factors I did not see them discuss is how much of a contributing factor the amount of  pain meds a patient has taken prior to their ER admission. I pose this question because in 2005 I had an episode where after days of self medicating I wound up on a ventilator with Acute Chest Syndrome for almost 10 days.  Many SCD patients do not have the support group I had who could inform the ER doc of what types of pain meds I was taking, how long and the dosing.  This is important information that can go unnoticed and cause mortality.  My sister in law believes the lack of advocacy in the ER by a parent, family or friend to fully disclose pain medication usage causes an alarming amount of unwarranted deaths to those with SCD.   

In conclusion, we must communicate with our physicians (Hem/Oncs) the need for adequate and timely protocols for patients presenting with pain crisis as an underlying symptom of high mortality. What do you think?

Help Support Sickle Cell Camp For Children

I recently wrote about adding more content to this blog. Camp Crescent Moon is the oldest Sickle Cell Camp in the US. What's so unique is it has survived all of these years because of corporate contributions and individual donors who understand the hardship SCD presents to our youth.

My eyes were opened to barriers faced by our youth when I worked in Philadelphia, PA in the Pharmaceutial industry and visited the clinic and hematology floor at Chilren Hospital. What I noticed were children who wanted to enjoy camp with children who are like them. So, below is a link which will allow you to give support to "Camp Crescent Moon".

http://www.firstgiving.com/fundraiser/patriceragin/campwalk