Last Friday, I took some time to reflect upon the passing of someone I thought the world of, my girl Cryssy. I laughed, cried and thought deeply about her memory and what kind of legacy what would’ve wanted to lead. And while I know she would’ve wanted all of us to have a good time in her name, I know that she and I talked several times about what I do here and what we both valued the most about the blog, which is the education aspect.
I blog about the things I’ve learned on my journey and I’m protective of it because it doesn’t just reflect tips and tidbits, but it reflects my life. I write about not drinking juice because I don’t. I write about and advocate for full fat foods because I eat them. With pleasure. Because I’m greedy. I live, and living helped me reclaim my health. With health being a big part of what I write about every day, how can I connect that to the legacy that my friend left?
Then, it hit me. If I could help to use this platform to help shed a light on sickle cell anemia, just a bit, maybe she’ll smile down on my daughter and I.
I spent a lot of time reading because I’m a nerd…er, because I wanted to help somehow, but I just didn’t understand. During my digging, I learned a lot that I’m pretty sure the average person may not know, so today’s Friday 5 is dedicated to Cryssy. Here’s hoping I do her justice.
That being said, let’s get down to bidness. Five things every African American should know about sickle cell anemia:
1) What is sickle cell anemia? Sickle cell anemia is a disease that affects blood flow and the delivery of oxygen throughout the body. Every living being has red blood cells, shaped similarly to doughnuts (we don’t do donuts here, but I’m saying’…) that flow through our veins. A person with sickle cell anemia, however, has blood cells shaped more like a sickle (a crescent moon), which not only creates difficulty in passing through the blood stream easily but also impedes the body’s ability to deliver enough oxygen to the body’s organs and tissue.
The anemia comes in because, while the average red blood cell can last about 120 days in the blood stream, sickle-shaped cells only last approximately 20 days, causing the body to exist without adequate amounts of blood cells, also known as “anemia.” Hence, the name of the disease, sickle-cell anemia. (It is possible to have anemia without the sickle-cells, and you can have sickle-cell disease without the anemia, though it usually follows.)
2) Prevalence: While sickle cell also affects Europeans from the Mediterranean, Arabians and Asians, a large number of sickle cell sufferers are African-American. 1 in every 400 African Americans suffer from sickle cell anemia.
Sickle-cell trait, however, is another story entirely.
Really quickly, to carry two alleles for a trait pretty much ensures that you’ll display the full expression of the trait. If you carry two alleles for brown eyes in your genes, you’ll have brown eyes. If you only carry one allele for a trait, then it means that the trait lies dormant – it is not expressed – and you are only a carrier for the trait. This is “fine,” unless you mate and procreate with someone else who is also a carrier for the trait. If you have four children with this person, probability assumes that at least 2 of those children will carry the sickle-cell trait, one child would be spared from the trait entirely and one child will develop the disease.
To carry sickle-cell trait, which basically means that you carry only one marker for the disease, is carried by at least ten percent of the African-American population in America. So, while we may not have the disease ourselves, it is possible that we could procreate with someone else who carries the trait and create a life that does. While many 80s babies and up are now tested for the disease – I’d like to say it’s required but there are lots of things that are required in health care that “everyone” isn’t receiving, so we need to be proactive about this – it’s still important to have a definitive yes or no regarding whether or not you, your partner or your children are carriers of either the trait or the disease.
It’s also important to consider the people we know and love who are as stubborn as a mule and simply refuse to see the doctor, even though they are feeling terribly lethargic, breathless, suffering from stomach pain and writing it off as eating bad food (which… never mind), feeling elevated heart rates and maybe even feeling a bit of pain in their bones, randomly. They might be suffering and not even know it, because of a lack of health care or, even despite having stellar health insurance, a fear of the doctor. (As much as I hate to admit it, we have a long, justified history of not trusting doctors.)
3) Sickle-cell anemia is treatable and curable. Sickle-cell anemia sufferers require frequent treatments as a means of trying to prevent crises from occurring. “Crises,” the name given to when a person suffers from a bout with immeasurable pain from the disease, are treated with blood transfusions to counter the anemia as well as pain medicines. It’s important for all of us to know that symptoms can be treated because it is possible for those of us who only carry the trait – not the full expression of it – to find ourselves suffering from symptoms, as well. It requires special blood testing, administered by your doctor, to determine if you carry the trait.
4) Speaking of which, on to the donation process. It’s not like blood, where you just show up and extend your arm. With bone marrow, you first have to register as a donor. As a registered donor, you may be contacted as a possible match and at that point, if you’re comfortable with going forward with the process (you are a volunteer and are never under any legal obligation to comply), you’d be scheduled for testing to ensure that you’re the best match for the patient in question.
If you are a match, then you’d be informed about your donation, recovery from the procedure and any potential side effects. If you agree, then you’re examined to ensure that the donation process is safe for both you and the life you’re saving. From there, it’s the home stretch.
The donor never pays for the donation or any other expenses incurred. The National Marrow Donor Program donation recipient’s medical insurance covers medical as well as non-medical costs. Donors are also covered by a donor life, disability and medical insurance policy. Neither you nor your family will be financially burdened by your decision to help save someone else.
Donating marrow, according to accounts I’ve seen, feels something like getting a lower-back tattoo. Side effects of donating are anywhere from fatigue and lower back pain to bleeding at the collection site. A donor can experience pain that lasts from a few days to several weeks. Approximately 98.5% of donors feel completely recovered within a few weeks, while about 1.34% experience serious complications.
The peripheral blood stem cell donations, are a bit easier. From Marrow.org:
PBSC donation is a nonsurgical procedure that takes place at a blood center or outpatient hospital unit. For 5 days leading up to donation, you will be given injections of a drug called filgrastim to increase the number of blood-forming cells in your bloodstream. Your blood is then removed through a needle in one arm and passed through a machine that separates out the blood-forming cells. The remaining blood is returned to you through the other arm. Your blood-forming cells are back to their normal levels within 4 to 6 weeks. To learn more, watch the PBSC donation video.
While only around 1 in every 540 of their registered donors in the Be The Match program goes on to donate, knowing the prevalence of sickle-cell anemia in the African American community coupled with (hopefully) an increase in donors, we should all hope to be able to help.
5) We can prevent this. While sickle cell anemia is not contagious, it is easily prevented by being careful with whom we procreate. If you procreate with someone who also carries the trait, you will wind up with a 1 in 4 chance of having a child with the disease. If you aim to be more careful with your family planning, there are means by which one can undergo fertility treatments that prevent passing the treatment on. Treatments are covered by insurance companies, because they’d rather pay to prevent passing the trait on than pay for a child that suffers from the disease. Here’s hoping you’ve got that good health care… because the downfall of all this is that it’s difficult to find someone to cover you if they find out you’ve got the pre-existing condition.
…but we don’t need comprehensive health care reform. Okay.
Please, if there is an inaccuracy or if I’ve misinterpreted something, here, please let me know by way of links, books, any resource other than anecdote, that way I can clarify and add resources for those who want to know more.
I have to admit, it felt good to write this.
What did I leave out?
Sources, of course:
- How To Donate
- Sickle Cell Anemia, from the National Institute of Health
- Frequently Asked Questions About Donating
- What Is Sickle Cell Anemia?
- Sickle Cell Anemia, From The Mayo Clinic
- Sickle Cell Disease Profile
21 comments
Erica, thanks for shedding light on sickle cell disease! I am sorry to hear about the loss of your friend. What a wonderful way to honor her memory!
I support a local charity, the William E. Proudford Sickle Cell Fund, started by my friend Dr. Karen Proudford and her family after the death of their father from sickle cell disease.
You are right…many advances have been made in the treatment of the disease. However, we are far from curing the disease…especially for adults.
If there’s anything I or the Proudford Fund can do to help you spread the word about sickle cell disease and sickle cell trait, please let me know!
Lisa
Thank you for sharing this. I do have some family experience with this but never had this much detail. I am sorry for your loss. You are a great friend.
Love your blog, as always.
Anyway, below are two more links that might be interesting to you (although you may have already found them, so sorry if I’m being repetitive).
This one talks more about the reason’s why (on a molecular level) sickle cells cause disease:
http://sickle.bwh.harvard.edu/scd_background.html
The second one is a PDF from the World Health Organization on Sickle Cell:
http://apps.who.int/gb/ebwha/pdf_files/WHA59/A59_9-en.pdf
And the third is about dominant versus recessive traits (while sickle cell having only one gene means the disease won’t be expressed, other diseases like Huntington’s, you only need one gene to get the disease):
http://www.thetech.org/genetics/ask.php?id=22
http://genome.wellcome.ac.uk/doc_wtd020863.html
Sorry if I went overboard with the links. I think topics like this are really important and very interesting.
And, as always, I value your contributions. 🙂
I clicked the link and I did not realize there would be so many requirements including weight! Just one more reason to get to m goal: to help myself so that I can help others! Thanks, Ericka for the information!
Sickle Cell is more like a group of diseases. For instance, the type of sickle cell that I have is hemoglobin SC. It is considered one of the less severe forms. I rarely have crises and have never needed a blood transfusion. The prevalence and occurrence of symptoms differs by disease type. As an adult with SC, my vision has been more of a concern than pain crises.
Also, while bone marrow transplants have been used to cure sickle cell, it’s still very experimental and is not recommended for all individuals with sickle cell.
The NYT has a pretty good health guide for sickle cell: http://health.nytimes.com/health/guides/disease/sickle-cell-anemia/overview.html
Thank you for posting these facts and sharing your personal experiences with your friends passing.
I have Sickle Beta Thalassemia (which is a rare form of the disease- coupling beta thalassemia [an inability to produce enough hemoglobin] and sickle cell [what little hemoglobin i AM able to produce is sickle-shaped). In March of 2010, i started getting regular “full volume blood exchanges”- a little different from infusions bc all of my blood is taken out and replaced by someone else’s every 2 months. In the two years that ive been getting exchanges, ive been hospitalized ONCE- i used to be admitted into the hospital twice a month and, in 2009, i was plagued with complications like pneumonia, aplastic anemia (my bone marrow wasnt producing anywhere near the needed amount of red blood cells), and acute chest syndrome- the leading cause of death among sickle cell patients. I had all of these complications all at the same time just a few months before i started my exchange regimen so needless to say, im very happy now. I rarely get sick and i can start my life again. Thank you soooo much for posting information about this. When people ask me about my FORMER illness, i always use the same donuts example- now ill just refer them to this post. this whole site is awesome!
Hi Erika, thanks for such an thorough and educated post on sickle cell anemia.. i have two cousins who have sickle cell anemia. my younger cousin wasn’t given much time to live, but he is now 14 years old and going strong. education about sickle cell is so crucial, seeing this post made me realize how much i didn’t know. your friend is smiling down at you right now for you have brought light to an issue that disproportionately affects african-americans.. thank you, thank you for always bringing issues that are of extreme importance to the forefront.. have a blessed and wonderful holiday..
Thank you so much for this. My son has sickle cell and it still amazes me that so many are uneducated about this disorder.
It is so important for people (like myself) with sickle cell to drink plenty of water and eat right. My hemotologist also told me NOT to take multivitamins that have iron in them, to avoid red meat(high iron content), and if I do eat read meat drink some milk or take a calcium supplement to help block iron absorption.
My condolences to you on the loss of your friend.
Thank you for the info about sickle cell. My youngest daughter carries the trait so whenever she decides to start a family, this will be something for her to look out for.
In addition, there is a very rare renal cancer that is associated with sickle cell trait. It is called renal medullary carcinoma. It is very aggressive and is usually diagnosed too late. I have a cousin that died from this two years ago. He passed approximately 6 months after his diagnosis. I share this because awareness will potentially create earlier detection potentially making the disease more treatable.
Thanks for writing this! Sorry for the loss of your friend. You honored her memory and legacy by being a beacon of light on this issue that plagues many of us. My birth mother has sickle cell anemia, however, her hemoglobin never matured and believes that now – in her 50’s – that she is still in utero. So her red blood cells never changed into that “sickle” shape. I guess this left her with no capacity to pass on the disease to her offspring because among the 5 of us, my sister only has the trait. Meanwhile, my birth father has the trait. I was blessed to have received neither the trait nor the disease from either of them. Doctors tested my mother & I repeatedly over the years, concluding that we were biological anomalies.
i find it kind of stupid that this site is for “black” people or “african american” okay eyah they are the majority of people who can have it but i am WHITE, i have grandparents on both sides that were NATIVE american, NOT BLACK and i have it so it kind of ticks me off when is see people talking only about black people having it. open your mind.
Oh. Now, this is interesting.
Even though the majority of sufferers are persons of color, you think all messaging regarding the disease should be targeted to ALL people, not just the majority?
I wonder where I’ve heard such a plea for inclusion before.
first off, Ellie- you might want to calm down with the negative language. This site is from the perspective of, targeted toward, African American women. This post was intended for the massive majority of people who have the disease and might be ill-informed. The exact same way that a breast cancer site targets mostly women even though men can also be diagnosed with breast cancer. second, the best way to present your view is by offering it politely. third, the site administrator and many other people who have become informed on this subject are well aware that Sickle Cell is not a “Black” disease; it is simply an inherited disease that is prevalent among those whose antecedents were from areas wherein malaria was prevalent- so there are many more Italians with Sickle Cell than South Africans. Have a great day, Miss Ellie
Hello!:) I kind of just stumbled on to your blog and glad that I did! Well, for me all my life, off and on Ive been diagnosed with anemia and I dont know why so ive been doing some research to educate myself on the different forms. Well when i first found out i was pregnant with my daughter(who does not have sickle cell…..or trait, not that i know of anyway), I found out I was anemic again and after having her i had to have emergency surgery and after being released I saw a document from the hospital that i was sickle cell anemia positive. This was a shock to me cause i had no idea, no doctor ever told me. So im thinking maybe I must have the sickle cell trait cause i dont think I have sickle cell. Yes i am a 80’s baby, 89 to be exact but the thing is I was adopted when I was a baby so maybe that information was not released, idk…but i will be going to the doctor to know for sure cause ive been having stomach pains and my anemia has gotten worst. I dont understand, if I do have sickle cell trait, why wasnt I told! And if I do have it, does that mean my daughter does too?
This is my first time on your blog and I will be a long time follower. I myself have sickle cell SC. Not only do I have it, but my two children who are 4yrs and 8mths have it as well. Thank you for shedding light on this disease. I’ve only had one blood transfusion and was hospitalized once in my entire 26 yrs. This disease should be as popular as cancer(in my opinion). Whenever I have a crisis and I have to go to the E.R. It’s very rare that you find someone there who understands the disease. I always feel as if I’m educating them on the disease. I thank you again for shedding light on this disease that my children and I live with. SEPTEMBER IS SICKLE CELL AWARENESS MONTH!!!
Oh, wow! I did not know that Sickle Cell was curable and preventable! You learn something new every day.
Thank you for this article Erika. I had a brief over view of what sickle cell anemia was but not an in depth knowledge. However I now need all the information on this disease I can get. I was diagnosed with sickle cell yesterday after a trip to the ER. I was floored because i am 29 and I have 3 children but never knew I had this disease. I had never even had any pain crisis until a few days ago. I woke up in intense pain all over my body. So now my main concern is getting my children tested and getting on a good treatment plan.
Sickle cell anemia is not 100% curable, you need to have a match from a healthy sibling which unfortunately not all sickle cell patients have. I live in Australia and sadly my brother passed away from complications of sickle cell and I also have sickle cell anemia, I have blood exchanges which is a different treatment to blood transfusions, it’s when they take out my some of my blood and replace it with healthy donor blood, this is all done through a red blood exchange transfusion machine. I have asked my doctors about the bone marrow “cure” and they said even if I had a healthy sibling it is a very risky procedure and would only do it in severe cases. I hope and pray that one day they do find a cure that is for every sickle cell anemia patient.
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