Letter to the Editor
Sickle Cell Disease Association of America, Inc. (SCDAA) hopes that the following statement clarifies some of the medical misstatements and confusion regarding the recent unfortunate death of Martin Lee Anderson, a 14-year old young man said to have sickle cell trait.
First, SCDAA wishes to convey its condolences to the family of Martin Lee Anderson. The organization hopes that the family will be provided correct information regarding sickle cell trait as the justice system seeks to establish the actual cause of his death. SCDAA believes that members of the general public deserve to receive information about sickle cell trait so that they can interpret correctly the statements of the medical examiner and those conducting investigations into this matter.
According to media reports, Martin Lee Anderson was beaten by guards within two hours of his arrival at the Bay County Sheriff's Office Boot Camp facility early January 5th, was taken to Bay County Medical Center, and later transported by air ambulance to Sacred Heart Hospital in Pensacola where he died on Jan. 6, 2006.
SCDAA states emphatically that the death of this 14-year old young man cannot be related to sickle cell trait. The organization also asserts that the statements of the County Medical Examiner, Dr. Charles Siebert, associating the death of the young man and bleeding with sickle cell trait, are completely baseless.
Several studies involving thousands of people with sickle cell trait (AS) have confirmed that they do not have lower life expectancy or more complicated health issues than others. People with AS do not have the symptoms of sickle cell disease. In fact, the overwhelming majority of people in the world with AS, and their healthcare providers, are unaware that they have the trait because it does not affect their health.
Over 3 million Americans carry the sickle cell trait. Worldwide, there are more than 200 million people with sickle cell trait. Sickle cell trait (AS) is not a disease. People with sickle cell trait are generally healthy carriers of the sickle cell gene (S). They are said to be AS because they have inherited the normal beta hemoglobin gene (A) from one parent and the sickle cell gene (S) from the other parent. Sickle cell trait is NOT a form of sickle cell disease. People with sickle cell disease have inherited the sickle cell gene from both parents or from one parent and another abnormal hemoglobin gene from the other parent.
The sickle cell gene leads to the production of sickle hemoglobin in red blood cells. Sickle hemoglobin can turn red blood cells into sickle cells. However, in the red blood cells of people with AS, there is always more hemoglobin A than hemoglobin S. This prevents the AS red blood cells from becoming sickle cells under normal conditions. People with AS do not
normally have sickle cells circulating in their blood and they do not have anemia or signs of increased red blood cell destruction, both being hallmarks of sickle cell disease. The finding of sickle red blood cells at autopsy of a person with sickle cell trait is expected. When red blood cells of people with sickle cell trait lose all the oxygen they carry (as would be expected at death), they will turn into sickle cells.
The sickle cell gene first arose in parts of Africa and in the Arabian/Indian peninsula. The gene expanded throughout populations in those regions and later spread to the Mediterranean, Middle East, the Caribbean and the Americas. The sickle cell gene survived and spread because it offered protection against severe malaria in young children who have sickle cell trait. Still today in Africa and elsewhere, children with sickle cell trait are less likely to die from malaria than those with regular hemoglobin in their red blood cells.
However, a few medical reports have sought to associate sickle cell trait with some medical conditions. These conditions include hematuria (bloody urine) related to tissue damage in the kidney, increased frequency of bacteria in the urine (especially during pregnancy), and inability to make concentrated urine. Other rare conditions associated with AS include a very rare cancer of the kidney called renal medullary carcinoma, hyphema (blood in the front chamber of the eye), and glaucoma.
In untrained military recruits, very rare exercise related deaths during basic training were seen more frequently in those with sickle cell trait. Such deaths have not been seen in conditioned military personnel, or in student and professional athletes (including long distance runners) with sickle cell trait. These preventable deaths in military recruits were reduced when training methods allowing for increased drinking of water and recognition of exertional heat illness were instituted.
All these medical conditions also occur in people without sickle cell trait. Their association with people with sickle cell trait is merely statistical. It remains to be proven whether sickle cell trait causes these conditions or whether some other genetic or environmental factors also frequent in people with sickle cell trait are the actual causes of the conditions.
With regard to Martin Lee Anderson, SCDAA wishes to stress first that it is unclear whether the diagnosis of sickle cell trait has been correctly established. Second, it is not possible to attribute his death to any of the very rare conditions associated with sickle cell trait. This has led SCDAA to join other experts in the conclusion that his death is not related to sickle cell trait. Attributing the death of this young man to sickle cell trait, given the physical punishment he was put through, does a disservice to the public and those in the sickle cell disease community. It is our desire that everyone is well educated and that they know that this attempt to declare sickle cell trait as the cause of Mr. Anderson’s death is not medically well-grounded. Justice has yet to be served in this case.
Sincerely,
Willarda V. Edwards, MD, MBA
President and COO
xxxxxxxxxxxxxxxxxxxxxxxxxxxxxxxxxxxxxxxxxxxxxxxxxxxxxxxxxxxxxxxxxxxxxxx
A doctor who says he has studied more than 100 sickle cell trait deaths supports a local medical examiner’s finding in the autopsy of a 14-year-old boot camp detainee.
Dr. John Kark of Howard University Hospital in Washington has been a part of sickle cell trait research for at least 20 years. He said he has studied 3,000 cases of heat illness in military recruits and “more than 100 deaths associated with sickle cell trait.”
Kark wrote in a letter to Dr. Charles Siebert, Bay County’s medical examiner, that the death of Martin Lee Anderson following his first day at the Bay County Sheriff’s Office Boot Camp did have characteristics of sickle cell trait.
“I agree with your opinion that he died from unexpected exertional heat illness from a syndrome that included features of (muscle breakdown) and heat stroke,” Kark wrote. “Please use this opinion as support for your diagnosis of cause of death.”
Kark could not be reached by phone Wednesday for comment or to confirm the letter, which Siebert provided to The News Herald.
Siebert said Kark is trying to stay out of the controversy over this case.
Anderson collapsed at the boot camp on Jan. 5 during a run. Drill instructors were caught on videotape kneeing him, putting him on the ground and striking his arms when he did not or could not comply with their orders to resume the run.
Anderson died the next day at a Pensacola hospital.
Siebert determined that Anderson died of natural causes related to an undiagnosed blood condition of sickle cell trait, which usually is benign. The condition was aggravated, Siebert said, when drill instructors pushed Anderson past his physical limit.
After Siebert released his findings, and the Panama City State Attorney’s Office took itself off the case, Gov. Jeb Bush asked for a second autopsy as part of an independent investigation. Dr. Vernard Adams of Tampa did another autopsy in March, but those results have not been released.
Also in March, Siebert sent his autopsy and medical reports to Kark.
“It’s nice to have a big-name person, an expert in the field, give you his opinion,” Siebert said Wednesday.
He said Kark had seen portions of the boot camp videotape, but Siebert did not send a copy with the autopsy report.
Kark responded with an undated letter that Siebert said he received about three weeks ago. In the letter, Kark talks about the numerous indicators of heat-related illness and sickle cell trait.
“I have indirect evidence that sickle cell trait does not contribute to the onset of mild to moderate exertional heat illness, but rather has an enormous impact on mortality rate when these conditions become life-threatening from other factors,” Kark wrote.
He said it was surprising that Anderson collapsed on a relatively mild January day. Kark guessed that Anderson may have ingested some kind of ephedra compound — Sudafed, for example — that raised his body temperature.
Kark said indicators of heatrelated illness compounded by sickle cell trait were: Anderson’s collapse during the run; “a combative delirium deteriorating into coma;” increased blood acidity; low carbon dioxide; and the late onset of “explosive” internal bleeding that marked the final stages of cellular breakdown in Anderson’s body.
Siebert said Wednesday that he would have kept Kark’s letter, and released it to the media, even if Kark had disagreed with him.
“I probably would have panicked, though,” Siebert said, “about what the hell else it could have been.”
vvvvvvvvvvvvvvvvvvvvvvvvvvvvvvvvvvvvvvvvvvvvvvvvvvvvvvvvvvvvvvvvvvvvvvvvvvvvvvvvvvvvv
I'm rooting for justice. Stan
