Tuesday, May 26, 2009

Thalassemia and Sickle Cell Disease

Picture this, you have Sickle Cell Disease (a disease of the red blood cell) and you marry a man who is diagnosed (at age 44) with Beta Thalassemia (also a disease of the red blood cell). Now you think to yourself; why didn’t this man know he had a blood disease?

Often people who have Thalassemia don’t know they have it. Sometimes they’re told, “You’re anemic,” and that’s all they’re told.

Thalassemia is similar to Sickle Cell Disease. It is also a genetic defect of the hemoglobin within the red blood cell. Thalassemia, however, is the defect in the number of hemoglobin made in the blood, where Sickle Cell is a defect of the hemoglobin (sickle shape). Either or both of these conditions cause anemia. Some people may have both of these conditions at the same time.

This is where my utter shock comes in. My husband was told by his parents that he was anemic, but at 44 he was officially diagnosed with Beta Thalassemia. My husband is African American and the doctor who diagnosed him is Chinese. The doctor informed him that they have “the same type of Thalassemia.”

Are you getting this? A Chinese doctor and an African American man have the same blood disorder.

There are two issues that come to mind when I remember this story. Firstly, this is another example of my main argument that blood disorders are diseases of humans, not of one race or another. And, secondly, I see the need for better testing/education practices. People should know what their health limitations are, and in result, understand the impact on them having children.

People living with Thalassemia suffer just like people living with Sickle Cell Disease. Just imagine a child of ours having Beta Thalassemia AND Sickle Cell Disease. Luckily, we do not have any children, and thank God I didn’t have to find out what that would be like.

Get informed.
Get tested.

Tuesday, May 12, 2009

Patient Centered Care

I was recently sent an email where I was asked my opinion regarding sickle cell management in the US and abroad, as it relates to improving care for sickle cell disease. The question was posed by suggesting more of a cultural/social model of care rather than a medical one.

I am just a patient with Sickle Cell Disease, and an advocate for people living with the disease. I am not a medical professional, I am not an expert, nor do I profess to know what a “medical model” is verses a “cultural/social” one. All I know is my experience with having Sickle Cell Disease.

As a child, I received treatment by medical professionals within my “cultural” community and the care I received was gentle, compassionate and well trained. It was not until I grew up, began to travel and moved to different communities. More than once I’ve been told by uninformed medical professionals, “What do you have? Sickle What?”

I was disgusted when doctors would tell me, “Oh, you have Sickle Cell, only black people get that disease.” Or, doctors would say, “Oh, we don’t get many black patients here, so we don’t see cases of Sickle Cell.” This calls for more training of medical professionals.

Just for the record, my niece looks white. She is ¾ Anglo & ¼ African American. Because we have a family history of Sickle Cell, she asked her doctor to perform an electrophoresis test to see if she was a carrier for Sickle Cell Disease. He told her, “You’re white, you don’t have that disease.” She insisted, because of her family history (her mother has it). Low and behold, was tested and found to have the Sickle Cell trait. So much for “you’re white, you don’t have THAT disease.” Again, this calls for more training of care providers.

For this very reason, I believe Sickle Cell care should be approached in a patient centered model; (as a disease of humans), not as cultural model (a disease of one racial group). Sickle Cell Disease is creeping silently into all populations, all races (because of mixing) and all cultures. Now, that’s my opinion.