Tag Archives: Sickle Cell Anemia

Straight, No Chaser: Sickle Cell Disease/Anemia

sca

I don’t type this post with any amount of joy. Can you imagine an existence in which your blood cells are deformed to the extent to which they resemble pointy knives poking at you? That’s a grotesque oversimplification, but it does capture the essence of those suffering from today’s topic. World Sickle Cell Day was earlier this month, seeking a heightened awareness of Sickle Cell Disease. Here are some answers to basic questions meant to provide you information about the disease.

  1. What Is Sickle Cell Disease (SCD)?

Sickle cell disease is a group of disorders of the red blood cells (RBCs). The RBCs have abnormal hemoglobin (the protein responsible for carrying oxygen throughout your bodies to nourish various tissues).

  1. How do I get Sickle Cell Disease?

sickle-cell inheritance

The conditions defining SCD are inherited. It is not a disorder you can individually obtain from interacting with someone affected (i.e. it is not contagious). In order to inherit SCD, you must receive abnormal genes from both parents. Sickle cell anemia is more common in certain ethnic groups, including those of African descent, Hispanic Americans from Central and South America, and those of Middle Eastern, Asian, Indian, and Mediterranean descent. One in twelve African-Americans carries a sickle cell gene.

  1. Is this the same as Sickle Cell Anemia?

sickle_cell_disease_sm

In sickle cell disease, a form of hemoglobin known as hemoglobin S is formed through inheritance from one parent. If the genes passed down from both parents create hemoglobin S (a designated known as hemoglobin SS), the resulting condition is known as sickle cell anemia (SCA). SCA is the most common and severe form of SCD. It should be noted that other forms of sickle cell disease exist, representing other combinations of hemoglobin and relatively different amounts of hemoglobin S. These include the following:

  • Hemoglobin SS
  • Hemoglobin SC
  • Hemoglobin Sβ0 thalassemia
  • Hemoglobin Sβ+ thalassemia
  • Hemoglobin SD
  • Hemoglobin SE
  1. What does it mean if I have Sickle Cell Disease?

The problem is simple and relatively easily understood. Red blood cells (RBCs) carry oxygen from your lungs to tissues around the body. Normally hemoglobin is disc-shaped and quite able to form in ways allowing it to maneuver throughout the body in its quest to deliver oxygen. If the hemoglobin is deformed, it is less able or unable to carry oxygen. If you can’t carry oxygen, it’s not getting delivered to your organs and tissues. If it’s not getting delivered, there are consequences.

  1. Why do I get symptoms? What symptoms would I get if I had SCD?

sickle-cell blockage

It’s all about the lack of oxygen. The pain results from the equivalent of your body screaming from its absence. These pain crises are sudden, severe and often unrelenting, requiring ER visits and dramatic doses of medicine to reverse symptoms. These crises can occur anywhere such as the brain or lungs, but are typically seen in joints.
Symptoms may result from the rupture of these deformed cells (hemolysis). Sickle cells only last 10-20 days, compared to a normal lifespan of 90-120 days for normal cells. The lack of effective RBCs in your body is what’s known as anemia. Those of you who bleed monthly or otherwise have conditions affecting red blood cells recognize all too well the fatigue and energy loss associated with low RBC levels. Furthermore, the abnormally shaped cells themselves (in combination with the relative lack of oxygen delivery) can damage organs, most notably including the spleen but also including the brain, eyes, lungs, liver, heart, kidneys, penis, joints, bones, or skin.

  1. How is SCD treated?

On a relative scale, there’s much better news than existed a generation or two ago. In the US, back in 1973 the average lifespan of a patient with SCD was only 14 years old; today it is approximately 40-60 years. SCD produces a life-long illness. Today, stem cell transplantation of those cells producing normal red blood cells (called hematopoietic stem cell transplantation or HSCT) represents a cure for those that can obtain it. The rate limiting consideration for many is most sicklers are either too old or don’t have an appropriate healthy genetic match to receive a successful transplant.
In the absence of a cure, for most patients, management involves regular healthy measures and regular medical care to prevent complications. Special attention is given to maintaining hydration, appropriate blood cell counts and pain management when needed.
A truly unfortunate part of the disease is the relative lack of compassion offered to sufferers. These patients live a life of pain and go through life treated as drug-seekers in a way we never see others treated (e.g. those with cancer or Lupus, for example). Most have had surgery before they reached age 10. Patients with sickle cell anemia suffer from a decreased life expectancy. If you’ve made it to this point in the post, consider yourself aware. I hope you care enough to lend a hand when needed.
Feel free to ask your SMA expert consultant any questions you may have on this topic.
Take the #72HoursChallenge, and join the community. As a thank you for being a valued subscriber to Straight, No Chaser, we’d like to offer you a complimentary 30-day membership at www.72hourslife.com. Just use the code #NoChaser, and yes, it’s ok if you share!
Order your copy of Dr. Sterling’s new books There are 72 Hours in a Day: Using Efficiency to Better Enjoy Every Part of Your Life and The 72 Hours in a Day Workbook: The Journey to The 72 Hours Life in 72 Days at Amazon or at www.72hourslife.com. Receive introductory pricing with orders!
Thanks for liking and following Straight, No Chaser! This public service provides a sample of what http://www.SterlingMedicalAdvice.com (SMA) and 844-SMA-TALK offers. Please share our page with your friends on WordPress, like us on Facebook SterlingMedicalAdvice.com and follow us on Twitter at @asksterlingmd.
Copyright © 2018 · Sterling Initiatives, LLC · Powered by WordPress

Basic Questions and Answers about Anemia

What is anemia?
Anemia is a condition defined by blood containing a lower than normal number of red blood cells or if the blood cells present don’t contain sufficient hemoglobin (the protein that carries oxygen throughout the body). Anemia from iron deficiency is the most common nutritional deficiency.

What causes anemia?
There are many causes of anemia and many different types of anemia, but the most common causes are blood loss (the moss common cause), a lack of red blood cell production and higher than normal rates of destruction of red blood cells.

What are the symptoms of anemia?
One of the major points of blood is it is the vehicle for carrying blood and removing carbon dioxide (waste) from your body. The presence of anemia means the absence of sufficient oxygen through the body. This produces symptoms such as fatigue (the most common symptom), weakness, shortness of breath, dizziness, headaches, cold feeling in the hands and feet, pale skin and chest pain. Severe or long-lasting anemia can cause actual damage to your heart, brain, and other organs in your body and can lead to death.

Are there risk factors?
Practically, the biggest risk factor is being a woman of child-bearing age because of the ongoing blood loss that occurs from menstruation. Additional major risk factors include a poor diet (meaning one low in iron, vitamins or minerals), blood loss from surgery or an acute injury, long-term or serious illnesses and infections, and a family history of inherited anemias (e.g. sickle cell anemia or thalassemia).
How is anemia diagnosed?
It’s important to note that all anemia is not created equal. Whether or not suggestive symptoms are present, anemia is rather easily identified with a simple blood test (the complete blood count, aka CBC). In many instances, that’s the beginning of the assessment. Additional tests may be needed to identify the specific test of anemia.
How is anemia treated?
Believe it or not, in many instances, the treatment of anemia isn’t as simple as taken an iron supplement, and thus medical assessments should be considered essential. Treatment for anemia depends on the type, cause, and severity of the underlying condition. Anemia treatment may involve dietary changes and/or supplements, but it may require other medicines, procedures, or surgery to treat blood loss.

What can I do?
Focus your efforts on these specific actions:

  • Prioritize getting routine evaluations and evaluations as needed in the midst of suggestive symptoms.
  • If you fall into a risk category, your diet and iron supplementation matters, as iron is needed to make hemoglobin. You can enhance iron absorption by eating red meats, chicken, turkey, pork, and fish/shellfish. If you don’t eat meat, foods that are good sources of iron include dark green leafy vegetables such as spinach, tofu, peas, dried fruits (prunes, raisins and apricots), prune juice and iron-fortified cereals and breads. Maintaining Vitamins B12, Vitamin C and folic acid, are also important in maintaining healthy cells and absorbing iron.

The good news is quite often anemia can be easily identified, treated and controlled. As with many other conditions, early diagnosis and treatment are key for improving one’s quality of life and life expectancy.
Feel free to ask your SMA expert consultant any questions you may have on this topic.
Take the #72HoursChallenge, and join the community. As a thank you for being a valued subscriber to Straight, No Chaser, we’d like to offer you a complimentary 30-day membership at www.72hourslife.com. Just use the code #NoChaser, and yes, it’s ok if you share!
Order your copy of Dr. Sterling’s new books There are 72 Hours in a Day: Using Efficiency to Better Enjoy Every Part of Your Life and The 72 Hours in a Day Workbook: The Journey to The 72 Hours Life in 72 Days at Amazon or at www.72hourslife.com. Receive introductory pricing with orders!
Thanks for liking and following Straight, No Chaser! This public service provides a sample of what http://www.SterlingMedicalAdvice.com (SMA) and 844-SMA-TALK offers. Please share our page with your friends on WordPress, like us on Facebook SterlingMedicalAdvice.com and follow us on Twitter at @asksterlingmd.
Copyright © 2017 · Sterling Initiatives, LLC · Powered by WordPress

Straight, No Chaser: Sickle Cell Disease/Anemia

sca

I don’t type this post with any amount of joy. Can you imagine an existence in which your blood cells are deformed to the extent to which they resemble pointy knives poking at you? That’s a grotesque oversimplification, but it does capture the essence of those suffering from today’s topic. World Sickle Cell Day was earlier this month, seeking a heightened awareness of Sickle Cell Disease. Here are some answers to basic questions meant to provide you information about the disease.

  1. What Is Sickle Cell Disease (SCD)?

Sickle cell disease is a group of disorders of the red blood cells (RBCs). The RBCs have abnormal hemoglobin (the protein responsible for carrying oxygen throughout your bodies to nourish various tissues).

  1. How do I get Sickle Cell Disease?

sickle-cell inheritance

The conditions defining SCD are inherited. It is not a disorder you can individually obtain from interacting with someone affected (i.e. it is not contagious). In order to inherit SCD, you must receive abnormal genes from both parents. Sickle cell anemia is more common in certain ethnic groups, including those of African descent, Hispanic Americans from Central and South America, and those of Middle Eastern, Asian, Indian, and Mediterranean descent. One in twelve African-Americans carries a sickle cell gene.

  1. Is this the same as Sickle Cell Anemia?

sickle_cell_disease_sm

In sickle cell disease, a form of hemoglobin known as hemoglobin S is formed through inheritance from one parent. If the genes passed down from both parents create hemoglobin S (a designated known as hemoglobin SS), the resulting condition is known as sickle cell anemia (SCA). SCA is the most common and severe form of SCD. It should be noted that other forms of sickle cell disease exist, representing other combinations of hemoglobin and relatively different amounts of hemoglobin S. These include the following:

  • Hemoglobin SS
  • Hemoglobin SC
  • Hemoglobin Sβ0 thalassemia
  • Hemoglobin Sβ+ thalassemia
  • Hemoglobin SD
  • Hemoglobin SE
  1. What does it mean if I have Sickle Cell Disease?

The problem is simple and relatively easily understood. Red blood cells (RBCs) carry oxygen from your lungs to tissues around the body. Normally hemoglobin is disc-shaped and quite able to deform in ways allowing it to maneuver throughout the body in its quest to deliver oxygen. If the hemoglobin is deformed, it is less able or unable to carry oxygen. If you can’t carry oxygen, it’s not getting delivered to your organs and tissues. If it’s not getting delivered, there are consequences.

  1. Why do I get symptoms? What symptoms would I get if I had SCD?

sickle-cell blockage

It’s all about the lack of oxygen. The pain results from the equivalent of your body screaming from its absence. These pain crises are sudden, severe and often unrelenting, requiring ER visits and dramatic doses of medicine to reverse symptoms. These crises can occur anywhere such as the brain or lungs, but are typically seen in joints.
Symptoms may result from the rupture of these deformed cells (hemolysis). Sickle cells only last 10-20 days, compared to a normal lifespan of 90-120 days for normal cells. The lack of effective RBCs in your body is what’s known as anemia. Those of you who bleed monthly or otherwise have conditions affecting red blood cells recognize all too well the fatigue and energy loss associated with low RBC levels. Furthermore, the abnormally shaped cells themselves (in combination with the relative lack of oxygen delivery) can damage organs, most notably including the spleen but also including the brain, eyes, lungs, liver, heart, kidneys, penis, joints, bones, or skin.

  1. How is SCD treated?

On a relative scale, there’s much better news than existed a generation or two ago. In the US, back in 1973 the average lifespan of a patient with SCD was only 14 years old; today it is approximately 40-60 years. SCD produces a life-long illness. Today, stem cell transplantation of those cells producing normal red blood cells (called hematopoietic stem cell transplantation or HSCT) represents a cure for those that can obtain it. The rate limiting consideration for many is most sicklers are either too old or don’t have an appropriate healthy genetic match to receive a successful transplant.
In the absence of a cure, for most patients, management involves regular healthy measures and regular medical care to prevent complications Special attention is given to maintaining hydration, appropriate blood cell counts and pain management when needed.
A truly unfortunate part of the disease is the relative lack of compassion offered to sufferers. These patients live a life of pain and go through life treated as drug-seekers in a way we never see others treated (e.g. those with cancer or Lupus, for example). Most have had surgery before they reached age 10. Patients with sickle cell anemia suffer from a decreased life expectancy. If you’ve made it to this point in the post, consider yourself aware. I hope you care enough to lend a hand when needed.
Feel free to ask your SMA expert consultant any questions you may have on this topic.
Take the #72HoursChallenge, and join the community. As a thank you for being a valued subscriber to Straight, No Chaser, we’d like to offer you a complimentary 30-day membership at www.72hourslife.com. Just use the code #NoChaser, and yes, it’s ok if you share!
Order your copy of Dr. Sterling’s new books There are 72 Hours in a Day: Using Efficiency to Better Enjoy Every Part of Your Life and The 72 Hours in a Day Workbook: The Journey to The 72 Hours Life in 72 Days at Amazon or at www.72hourslife.com. Receive introductory pricing with orders!
Thanks for liking and following Straight, No Chaser! This public service provides a sample of what http://www.SterlingMedicalAdvice.com (SMA) and 844-SMA-TALK offers. Please share our page with your friends on WordPress, like us on Facebook SterlingMedicalAdvice.com and follow us on Twitter at @asksterlingmd.
Copyright © 2017 · Sterling Initiatives, LLC · Powered by WordPress
 

Straight, No Chaser: Sickle Cell Disease/Anemia

sca

I don’t type this post with any amount of joy. Can you imagine an existence in which your blood cells are deformed to the extent to which they resemble pointy knives poking at you? That’s a grotesque oversimplification, but it does capture the essence of those suffering from today’s topic. World Sickle Cell Day was earlier this month, seeking a heightened awareness of Sickle Cell Disease. Here are some answers to basic questions meant to provide you information about the disease.

  1. What Is Sickle Cell Disease (SCD)?

Sickle cell disease is a group of disorders of the red blood cells (RBCs). The RBCs have abnormal hemoglobin (the protein responsible for carrying oxygen throughout your bodies to nourish various tissues).

  1. How do I get Sickle Cell Disease?

sickle-cell inheritance

The conditions defining SCD are inherited. It is not a disorder you can individually obtain from interacting with someone affected (i.e. it is not contagious). In order to inherit SCD, you must receive abnormal genes from both parents. Sickle cell anemia is more common in certain ethnic groups, including those of African descent, Hispanic Americans from Central and South America, and those of Middle Eastern, Asian, Indian, and Mediterranean descent. One in twelve African-Americans carries a sickle cell gene.

  1. Is this the same as Sickle Cell Anemia?

sickle_cell_disease_sm

In sickle cell disease, a form of hemoglobin known as hemoglobin S is formed through inheritance from one parent. If the genes passed down from both parents create hemoglobin S (a designated known as hemoglobin SS), the resulting condition is known as sickle cell anemia (SCA). SCA is the most common and severe form of SCD. It should be noted that other forms of sickle cell disease exist, representing other combinations of hemoglobin and relatively different amounts of hemoglobin S. These include the following:

  • Hemoglobin SS
  • Hemoglobin SC
  • Hemoglobin Sβ0 thalassemia
  • Hemoglobin Sβ+ thalassemia
  • Hemoglobin SD
  • Hemoglobin SE
  1. What does it mean if I have Sickle Cell Disease?

The problem is simple and relatively easily understood. Red blood cells (RBCs) carry oxygen from your lungs to tissues around the body. Normally hemoglobin is disc-shaped and quite able to deform in ways allowing it to maneuver throughout the body in its quest to deliver oxygen. If the hemoglobin is deformed, it is less able or unable to carry oxygen. If you can’t carry oxygen, it’s not getting delivered to your organs and tissues. If it’s not getting delivered, there are consequences.

  1. Why do I get symptoms? What symptoms would I get if I had SCD?

sickle-cell blockage

It’s all about the lack of oxygen. The pain results from the equivalent of your body screaming from its absence. These pain crises are sudden, severe and often unrelenting, requiring ER visits and dramatic doses of medicine to reverse symptoms. These crises can occur anywhere such as the brain or lungs, but are typically seen in joints.
Symptoms may result from the rupture of these deformed cells (hemolysis). Sickle cells only last 10-20 days, compared to a normal lifespan of 90-120 days for normal cells. The lack of effective RBCs in your body is what’s known as anemia. Those of you who bleed monthly or otherwise have conditions affecting red blood cells recognize all too well the fatigue and energy loss associated with low RBC levels. Furthermore, the abnormally shaped cells themselves (in combination with the relative lack of oxygen delivery) can damage organs, most notably including the spleen but also including the brain, eyes, lungs, liver, heart, kidneys, penis, joints, bones, or skin.

  1. How is SCD treated?

On a relative scale, there’s much better news than existed a generation or two ago. In the US, back in 1973 the average lifespan of a patient with SCD was only 14 years old; today it is approximately 40-60 years. SCD produces a life-long illness. Today, stem cell transplantation of those cells producing normal red blood cells (called hematopoietic stem cell transplantation or HSCT) represents a cure for those that can obtain it. The rate limiting consideration for many is most sicklers are either too old or don’t have an appropriate healthy genetic match to receive a successful transplant.
In the absence of a cure, for most patients, management involves regular healthy measures and regular medical care to prevent complications Special attention is given to maintaining hydration, appropriate blood cell counts and pain management when needed.
A truly unfortunate part of the disease is the relative lack of compassion offered to sufferers. These patients live a life of pain and go through life treated as drug-seekers in a way we never see others treated (e.g. those with cancer or Lupus, for example). Most have had surgery before they reached age 10. Patients with sickle cell anemia suffer from a decreased life expectancy. If you’ve made it to this point in the post, consider yourself aware. I hope you care enough to lend a hand when needed.
Feel free to ask your SMA expert consultant any questions you may have on this topic.
Order your copy of Dr. Sterling’s new book Behind The Curtain: A Peek at Life from within the ER at jeffreysterlingbooks.com, iTunes, Amazon, Barnes and Nobles and wherever books are sold.
Thanks for liking and following Straight, No Chaser! This public service provides a sample of what http://www.SterlingMedicalAdvice.com (SMA) and 844-SMA-TALK offers. Please share our page with your friends on WordPress, like us on Facebook SterlingMedicalAdvice.com and follow us on Twitter at @asksterlingmd.
Copyright © 2016 · Sterling Initiatives, LLC · Powered by WordPress

Straight, No Chaser: Sickle Cell Anemia

sca

September represents Sickle Cell Anemia (SCA) Awareness Month. In the context of discussing diseases that present to the emergency department with pain, the Pain (Vasoocclusive) Crises of SCA stand out. In my experience, I can’t identify another physiologically understood disease in which patients are as commonly treated as if they are less than deserving of treatment. That certainly isn’t the case with patients needing pain control from diseases such as cancer and lupus. This is a quite good (and unfortunate) example of biases creeping into medical practice. Can you imagine an existence in which your blood cells are deformed to the extent to which they resemble pointy knives poking at you? That’s a grotesque oversimplification, but it does capture the essence of those suffering from a Pain Crisis.

  1. What Is Sickle Cell Disease (SCD)?

Sickle cell disease is a group of disorders of the red blood cells (RBCs). The RBCs have abnormal hemoglobin (the protein responsible for carrying oxygen throughout your bodies to nourish various tissues).

  1. How do I get Sickle Cell Disease?

sickle-cell inheritance

The conditions defining SCD are inherited. It is not a disorder you can individually obtain from interacting with someone affected (i.e. it is not contagious). In order to inherit SCD, you must receive abnormal genes from both parents. Sickle cell anemia is more common in certain ethnic groups, including those of African descent, Hispanic Americans from Central and South America, and those of Middle Eastern, Asian, Indian, and Mediterranean descent. One in twelve African-Americans carries a sickle cell gene.

  1. Is this the same as Sickle Cell Anemia?

sickle_cell_disease_sm

In sickle cell disease, a form of hemoglobin known as hemoglobin S is formed through inheritance from one parent. If the genes passed down from both parents create hemoglobin S (a designated known as hemoglobin SS), the resulting condition is known as sickle cell anemia (SCA). SCA is the most common and severe form of SCD. It should be noted that other forms of sickle cell disease exist, representing other combinations of hemoglobin and relatively different amounts of hemoglobin S. These include the following:

  • Hemoglobin SS
  • Hemoglobin SC
  • Hemoglobin Sβ0 thalassemia
  • Hemoglobin Sβ+ thalassemia
  • Hemoglobin SD
  • Hemoglobin SE
  1. What does it mean if I have Sickle Cell Disease?

The problem is simple and relatively easily understood. Red blood cells (RBCs) carry oxygen from your lungs to tissues around the body. Normally hemoglobin is disc-shaped and quite able to deform in ways allowing it to maneuver throughout the body in its quest to deliver oxygen. If the hemoglobin is deformed, it is less able or unable to carry oxygen. If you can’t carry oxygen, it’s not getting delivered to your organs and tissues. If it’s not getting delivered, there are consequences.

  1. Why do I get symptoms? What symptoms would I get if I had SCD?

sickle-cell blockage

It’s all about the lack of oxygen. The pain results from the equivalent of your body screaming from its absence. These pain crises are sudden, severe and often unrelenting, requiring ER visits and dramatic doses of medicine to reverse symptoms. These crises can occur anywhere such as the brain or lungs, but are typically seen in joints.
Symptoms may result from the rupture of these deformed cells (hemolysis). Sickle cells only last 10-20 days, compared to a normal lifespan of 90-120 days for normal cells. The lack of effective RBCs in your body is what’s known as anemia. Those of you who bleed monthly or otherwise have conditions affecting red blood cells recognize all too well the fatigue and energy loss associated with low RBC levels. Furthermore, the abnormally shaped cells themselves (in combination with the relative lack of oxygen delivery) can damage organs, most notably including the spleen but also including the brain, eyes, lungs, liver, heart, kidneys, penis, joints, bones, or skin.

  1. How is SCD treated?

On a relative scale, there’s much better news than existed a generation or two ago. In the US, back in 1973 the average lifespan of a patient with SCD was only 14 years old; today it is approximately 40-60 years. SCD produces a life-long illness. Today, stem cell transplantation of those cells producing normal red blood cells (called hematopoietic stem cell transplantation or HSCT) represents a cure for those that can obtain it. The rate limiting consideration for many is most sicklers are either too old or don’t have an appropriate healthy genetic match to receive a successful transplant.
In the absence of a cure, for most patients, management involves regular healthy measures and regular medical care to prevent complications Special attention is given to maintaining hydration, appropriate blood cell counts and pain management when needed.

scdaa

A truly unfortunate part of the disease is the relative lack of compassion offered to sufferers. As previously mentioned, these patients live a life of pain and go through life treated as drug-seekers in a way we rarely, if ever, see others treated. Most have had surgery before they reached age 10. Patients with sickle cell anemia suffer from a decreased life expectancy. If you’ve made it to this point in the post, consider yourself aware. I hope you care enough to lend a hand when needed.

sca research

There are good and important organizations that are dedicated to the study and treatment of SCA, including the Sickle Cell Disease Association www.sicklecelldisease.org and the Foundation for Sickle Cell Research www.fscdr.org.
Thanks for liking and following Straight, No Chaser! This public service provides a sample of what http://www.SterlingMedicalAdvice.com (SMA) and 844-SMA-TALK offers. Please share our page with your friends on WordPress, like us on Facebook @ SterlingMedicalAdvice.com and follow us on Twitter at @asksterlingmd. Preorder your copy of Dr. Sterling’s new book Behind The Curtain: A Peek at Life from within the ER at jeffreysterlingbooks.com.

Straight, No Chaser: Sickle Cell Disease/Anemia and World Sickle Cell Day

sca

I don’t type this post with any amount of joy. Can you imagine an existence in which your blood cells are deformed to the extent to which they resemble pointy knives poking at you? That’s a grotesque oversimplification, but it does capture the essence of those suffering from today’s topic. Today is World Sickle Cell day, meaning it’s a day calling for awareness of Sickle Cell Disease. Here are some answers to basic questions meant to provide you information about the disease.

  1. What Is Sickle Cell Disease (SCD)?

Sickle cell disease is a group of disorders of the red blood cells (RBCs). The RBCs have abnormal hemoglobin (the protein responsible for carrying oxygen throughout your bodies to nourish various tissues).

  1. How do I get Sickle Cell Disease?

sickle-cell inheritance

The conditions defining SCD are inherited. It is not a disorder you can individually obtain from interacting with someone affected (i.e. it is not contagious). In order to inherit SCD, you must receive abnormal genes from both parents. Sickle cell anemia is more common in certain ethnic groups, including those of African descent, Hispanic Americans from Central and South America, and those of Middle Eastern, Asian, Indian, and Mediterranean descent. One in twelve African-Americans carries a sickle cell gene.

  1. Is this the same as Sickle Cell Anemia?

sickle_cell_disease_sm

In sickle cell disease, a form of hemoglobin known as hemoglobin S is formed through inheritance from one parent. If the genes passed down from both parents create hemoglobin S (a designated known as hemoglobin SS), the resulting condition is known as sickle cell anemia (SCA). SCA is the most common and severe form of SCD. It should be noted that other forms of sickle cell disease exist, representing other combinations of hemoglobin and relatively different amounts of hemoglobin S. These include the following:

  • Hemoglobin SS
  • Hemoglobin SC
  • Hemoglobin Sβ0 thalassemia
  • Hemoglobin Sβ+ thalassemia
  • Hemoglobin SD
  • Hemoglobin SE
  1. What does it mean if I have Sickle Cell Disease?

The problem is simple and relatively easily understood. Red blood cells (RBCs) carry oxygen from your lungs to tissues around the body. Normally hemoglobin is disc-shaped and quite able to deform in ways allowing it to maneuver throughout the body in its quest to deliver oxygen. If the hemoglobin is deformed, it is less able or unable to carry oxygen. If you can’t carry oxygen, it’s not getting delivered to your organs and tissues. If it’s not getting delivered, there are consequences.

  1. Why do I get symptoms? What symptoms would I get if I had SCD?

sickle-cell blockage

It’s all about the lack of oxygen. The pain results from the equivalent of your body screaming from its absence. These pain crises are sudden, severe and often unrelenting, requiring ER visits and dramatic doses of medicine to reverse symptoms. These crises can occur anywhere such as the brain or lungs, but are typically seen in joints.
Symptoms may result from the rupture of these deformed cells (hemolysis). Sickle cells only last 10-20 days, compared to a normal lifespan of 90-120 days for normal cells. The lack of effective RBCs in your body is what’s known as anemia. Those of you who bleed monthly or otherwise have conditions affecting red blood cells recognize all too well the fatigue and energy loss associated with low RBC levels. Furthermore, the abnormally shaped cells themselves (in combination with the relative lack of oxygen delivery) can damage organs, most notably including the spleen but also including the brain, eyes, lungs, liver, heart, kidneys, penis, joints, bones, or skin.

  1. How is SCD treated?

On a relative scale, there’s much better news than existed a generation or two ago. In the US, back in 1973 the average lifespan of a patient with SCD was only 14 years old; today it is approximately 40-60 years. SCD produces a life-long illness. Today, stem cell transplantation of those cells producing normal red blood cells (called hematopoietic stem cell transplantation or HSCT) represents a cure for those that can obtain it. The rate limiting consideration for many is most sicklers are either too old or don’t have an appropriate healthy genetic match to receive a successful transplant.
In the absence of a cure, for most patients, management involves regular healthy measures and regular medical care to prevent complications Special attention is given to maintaining hydration, appropriate blood cell counts and pain management when needed.
A truly unfortunate part of the disease is the relative lack of compassion offered to sufferers. These patients live a life of pain and go through life treated as drug-seekers in a way we never see others treated (e.g. those with cancer or Lupus, for example). Most have had surgery before they reached age 10. Patients with sickle cell anemia suffer from a decreased life expectancy. If you’ve made it to this point in the post, consider yourself aware. I hope you care enough to lend a hand when needed.
Thanks for liking and following Straight, No Chaser! This public service provides a sample of what http://www.SterlingMedicalAdvice.com (SMA) and 844-SMA-TALK offers. Please share our page with your friends on WordPress, like us on Facebook @ SterlingMedicalAdvice.com and follow us on Twitter at @asksterlingmd. Preorder your copy of Dr. Sterling’s new book Behind The Curtain: A Peek at Life from within the ER at jeffreysterlingbooks.com.