African Americans and Sickle Cell Anemia!

Concerned Woman.Recent research on sickle cell anemia has shown how improving nitric oxide production can make a significant difference in symptom severity.  I’ve written about this in my article “Nitric Oxide and Sickle Cell Anemia!”  The purpose of this article is to provide a basic understanding of this genetic disease. 

Although African Americans are affected the most by sickle cell disease, it can affect Mediterranean, Middle Eastern, and Asian Indian ancestry.  There is a growing segment in the Latino-American population particularly those of Caribbean, Central American, and South American ancestry who are also affected by this disease. 

In the United States one out of every 400 births has this disease.  The most common type of sickle cell disease is sickle cell anemia. 

Definition and Description of Sickle Cell Disease 

Sickle cell disease is a group of inherited blood disorders that center on red blood cells.  These red blood cells can function abnormally resulting in small blood clots, chronic anemia, painful events, and potential complications associated with tissue and organ damage.  These blood disorders include sickle cell anemia, Mediterranean blood disease, and the sickle beta thalassemia syndromes. 

All types of sickle cell disease are caused by a genetic change in the hemoglobin portion of the red blood cell.  Hemoglobin is the oxygen-carrying protein inside the red blood cell.  Normal red blood cells are oval and flexible.  Red blood cells in sickle cell disease have a tendency to reshape themselves into rod-like structures that resemble the curved blade of a sickle; thus, the term sickle cell.  

Sickle cells have a shorter life span than normal red blood cells.  This results in chronic anemia and reduced oxygen to the tissues of the body.  Sickle cells are sticky and less flexible than normal red blood cells.  This presents a problem since they can be trapped in the small blood vessels preventing proper blood flow to the tissues.  This compromises the delivery of oxygen and results in tissue pain.  It also has the potential to damage the associated tissues and organs. 

Carriers of the sickle cell gene are referred to as having sickle cell trait.  Most of the time sickle cell trait does not cause health problems.  In fact, sickle cell trait can be beneficial because it provides protection against malaria, a disease caused by blood-borne parasites transmitted through mosquito bites.  It is estimated that one in 12 African-Americans has sickle cell trait. 

The Cause of Sickle Cell Disease! 

The hemoglobin molecule of a red blood cell is made up of three components:  

  • Heme
  • Alpha or alpha-like globin
  • Beta or beta-like globin.   

Sickle cells contain a genetic change in the beta globin component of the hemoglobin molecule.  This is caused by a change in the genetic coding on chromosome 11.  One small change in a single DNA nucleotide results in a different amino acid being inserted into the beta globin protein of the hemoglobin molecule.  This results in the unique properties of sickle cells.  For simplicity we will call this altered gene the “sickle cell gene” and the regular gene the “normal red blood cell gene.” 

For most individuals, they have two copies of the “normal red blood cell gene” to produce normal beta globin resulting in typical red blood cells.  Individuals with sickle cell trait have one “normal red blood cell gene” and one “sickle cell gene” so they produce both normal red blood cells and sickle cells in roughly equal proportions.  Because of this they do not usually experience significant health problems as a result of having sickle cell trait.  Those with sickle cell anemia have two “sickle cell genes.” 

Genetics play a significant role in both the disease, symptoms, and in family planning.  If both members of a couple have sickle cell trait, then there is a 25% chance in each pregnancy for the baby to inherit two sickle cell genes.  The resulting child will have sickle cell anemia.  Correspondingly, there is a 50% chance the baby will have sickle cell trait and a 25% chance that the baby will have the “normal red blood cell genes”.  

If both members of a couple have sickle cell anemia, then the baby will have sickle cell anemia 100% of the time.  If one member of the couple has sickle cell anemia and the other has both “normal red blood cell genes”, then the resulting child will have sickle cell trait 100% of the time.  Finally, if one member of the couple has sickle cell trait and the other has both “normal red blood cell genes”, then the resulting child has a 50% chance of having normal red blood cell hemoglobin or a 50% chance of having sickle cell trait. 

The Need for Oxygen! 

Oxygen is necessary for life and the optimal function of all cells.  Red blood cells transport the oxygen from your lungs to the tissues of your body.  It’s the hemoglobin molecule that binds oxygen to itself in the lungs.  It then releases oxygen to the tissues for proper cell respiration.  However, once the oxygen is released by the sickle cell hemoglobin it can cause the red blood cell to alter its normal oval shape into the rigid, sickle shape characteristic of sickle cells.  Low oxygen can be a trigger for this change.  Studies also seem to indicate that cold temperatures and dehydration can be additional factors in triggering this change. 

Normal red blood cells can survive for approximately 120 days where as sickle cells typically last 10-12 days.  This is an important factor because it leaves the bloodstream chronically short of red blood cells and hemoglobin which leads to anemia.  This creates its own shortage of oxygen which could trigger a shape change in the oval red blood cell to the sickle shape.  This rigid, sickle shape causes circulation problems especially in small blood vessels.  

In addition, there are altered chemical properties that develop which increases the cells’ “stickiness”.  This is why sickle cells tend to adhere to the inside surfaces of small blood vessels, as well as other blood cells, resulting in blockages.  These blockages prevent oxygenated blood from reaching the tissues resulting in pain.  If kept without oxygen long enough possible organ and tissue damage can occur. 

Symptoms of Sickle Cell Anemia! 

Common symptoms for those with sickle cell anemia include the following: 

  • Bloody urine
  • Bone and/or abdominal pain
  • Chest pain
  • Delayed growth and delayed puberty
  • Excessive thirst
  • Fatigue, breathlessness, rapid heart rate
  • Frequent urination
  • Increased susceptibility to infections, fever
  • Pain which can vary from moderate to intense
  • Paleness, yellow eyes and/or skin, jaundice
  • Poor eyesight or blindness
  • Ulcers on the lower legs usually in adolescents and adults 

For those with sickle cell anemia, the severity of symptoms varies widely and cannot be predicted solely on genetic inheritance.  Some with sickle cell anemia develop health and life threatening problems in infancy while others only have mild symptoms throughout their lives.  Others experience various degrees of health issues as they age.  Certain variations of sickle cell disease tend to have less severe symptoms than other types of sickle cell disease. 

Organs Affected by Sickle Cell Anemia 

Various organs and body systems can be effect by sickle cell disease.  As you will see from this list, sickle cell anemia has a wide range of effects on the body.  The bottom line is that any tissue that needs oxygen and adequate blood flow can be at risk. 

  • Acute Chest Syndrome – Acute chest syndrome or ACS is a leading cause of death for those with sickle cell disease.  It takes place in the lungs.  Rapid diagnosis and treatment is very important.  ACS can occur at any age.  It is similar to pneumonia in symptoms but distinct in its damage. 
  • Anemia – As we learned early, sickle cells have a life span of 10-12 days resulting in a deficiency of red blood cells in the bloodstream.  It is the hemoglobin of red blood cells that carry oxygen, so with this deficiency there is a reduction in oxygen to the tissues.  Common symptoms of anemia include fatigue, paleness, and a shortness of breath.  The heart rate will increase.  This circulates more blood helping to make up for the lack of oxygen to the tissues. 
  • Delayed Growth – Because of the short life span of sickle cells, the demands on the bone marrow to produce more red blood cells compete with the demands of a growing body.  Children with sickle cell anemia may experience delayed growth and reach puberty at a later age.  However, by early adulthood, they catch up on growth and height but may still remain below average in weight. 
  • Infections and the Spleen – Children under the age of three with sickle cell anemia are particularly susceptible to life-threatening bacterial infections especially from Streptococcus pneumoniaeUnfortunately, 15% of these types of cases result in death.  Since your spleen helps to fight bacterial infections, it is particularly vulnerable.  For those with sickle cell anemia, it is not uncommon to see the loss of spleen function by late childhood. 
  • Jaundice and Gallstones – Jaundice is indicated by a yellow tone in the skin and eyes due to increased levels of bilirubin.  Bilirubin is the final product of hemoglobin degradation when red blood cells are destroyed.  Bilirubin is  removed from the bloodstream by the liver.  Elevated levels can increase the chance for gallstones.   
  • Joint Problems – The blood supply to the connective tissues, especially in the hip and shoulder joints, can be blocked by the sickle cells resulting in bone damage and poor healing.  This complication can affect an individual’s physical abilities and result in substantial and chronic pain. 
  • Kidney Disease – Kidneys are particularly prone to damage from sickle cells.  Adults with sickle cell disease often experience reduce kidney function which can progress to kidney failure. 
  • Painful EventsThis is the hallmark symptom of sickle cell disease.  The frequency and duration varies tremendously from individual to individual and over an individual’s lifetime.  These painful events are also the most common cause for hospitalization.  This hallmark symptom results when the small blood vessels become blocked by the sickle cells preventing oxygen from reaching the tissues.  Although pain can affect any area of the body, the most frequent sites are the extremities, chest, abdomen, and bones. 
  • Priapism – Only males have to deal with this since it is a condition characterized by a persistent and painful erection.  Blood vessels become blocked by sickle cells so that blood is trapped in the tissue of the male’s organ.  It’s extremely painful and can result in damage to this tissue causing impotence.   
  • Retinopathy – The blood vessels that support the tissue at the back of the eye may be blocked by sickle cells resulting in this condition.  Regular ophthalmology evaluations and effective treatment can help a person avoid permanent damage to their vision. 
  • Stroke – This is the most concerning complication of sickle cell disease.  Approximately 11% of individuals with sickle cell anemia will have a recognizable stroke by the age of 20.  A stroke in a person with sickle cell disease is usually caused by a blockage of a blood vessel in the brain by the sickle cells.  This results in lack of oxygen to the affected area of the brain.  The consequences are far ranging from undetectable effects, to apparent or subtle learning disabilities, to severe physical or cognitive impairment, to life-threatening situations. 

Diagnosis and Treatment of Sickle Cell Anemia 

The inheritance of sickle cell disease or sickle cell trait cannot be prevented but it can be predicted so screening is recommended.  If you exhibit symptoms, then contact your physician so that accurate tests can be done.  These tests can determine if you carry the “sickle cell gene” and what level of risk you are at.  For newborns, more than 40 states include sickle cell screening as part of the battery of blood tests.  However, don’t just assume the test is done.  You must always be proactive. 

Hemoglobin trait screening is always a good choice for any person of a high-risk ethnic background especially if you are planning on having children.  If you and your partner are found to have sickle cell or any other hemoglobin traits, then you might want to receive genetic counseling to better understand the risk of sickle cell disease for your offspring.  There are various testing options available to you to help you in your planning. 

There are various treatment options to help prevent some of the symptoms and complications of sickle cell disease.  These treatment options can include: 

  • Access to comprehensive health care
  • Adequate nutrition
  • Avoiding stresses and infection
  • Blood transfusions
  • Bone marrow transplantation
  • Getting proper rest
  • Good hydration
  • Hydroxyurea
  • Pain management
  • Proper immunizations
  • Supplementation with folic acid
  • Support groups
  • Surgery
  • Use of preventative antibiotics

As with any disease condition, you want to always work with a qualified health professional to develop a course of action that best fits your individual situation. 

New Research on Nitric Oxide! 

There is new research that shows a direct correlation between nitric oxide deficiency and symptom severity for those with sickle cell anemia.  I will discuss this in my companion article “Sickle Cell Disease – 14 Helpful Steps!”  This article discusses the latest research.  It also provides 14 suggestions you can use to help those with sickle cell disease. 

I hope this article has given you a better understanding of sickle cell anemia.  I encourage you to take the steps to educate yourself and any loved ones who may have this health issue.  We can help empower them to take the necessary steps to improve their overall health and wellness. 

Together we can work to save a million lives! 

Dan Hammer 

Dan Hammer has a background in biology, chemistry and exercise physiology.  He used to run one of the largest health club operations in the Chicagoland area and has been helping people with their wellness issues for more than 25 years.   
The information contained in this article is for general information purposes only and never as a substitute for professional medical advice or medical exam.  The information contain in this article has not been evaluated by the Food and Drug Administration and should not be used to diagnose, treat, cure or prevent any disease without the supervision of a qualified medical doctor. 

African Americans and Cardiovascular Disease!

African American DoctorCardiovascular disease is the number one killer of people worldwide.  Unfortunately, it’s even worse for the African American community.  There are several reasons for this and hopefully this article will help begin the process of changing this sad reality.   To address these reasons I’ve broken this article into three parts:  The Bad News, Several Underlining Causes, and Steps to Decrease Your Risk! 

The Bad News! 

It’s important for you to understand the depth of cardiovascular disease confronting the African American community.  Below is a brief summary of the ailments that directly affect African Americans:  

  • Diabetes.  Type 2 diabetes has grown to epidemic proportions in the United States.  People with diabetes have a greater risk for stroke, heart disease and circulatory issues.  Most diabetes-related deaths are due to cardiovascular disease.  Twice as many African Americans will develop diabetes when compared to the Caucasian community. 
  • Erectile Dysfunction.  Erectile dysfunction affects 50% of the male population over the age 40 and is even greater in the African American community. 
  • Heart Disease.  African American women in the age range of 25-44 have a 2.5 times greater risk of coronary heart disease and African American men have a 1.5 times greater risk than the Caucasian community. 
  • High Blood Pressure.  The number one risk factor for stroke is high blood pressure, and 1 out of every 3 African Americans suffers from this ailment.  The American Heart Association estimates that 28% of African American adults and more than 66% of African Americans over the age of 60 have high blood pressure.  
  • High Cholesterol Levels.  High blood cholesterol is a significant risk factor for heart disease.  Unfortunately 50% of African American men and 54% of women have too much cholesterol circulating in their blood stream. 
  • Sickle Cell Anemia.  It’s estimated that one in 12 African Americans has sickle cell trait and one out of every 400 births have sickle cell anemia. 
  • Stroke.  African Americans are twice as likely to die from a stroke as Caucasians.  The rate of having their first stroke is almost doubled that of Caucasians.  One half of all African American women will die from either a stroke or heart disease.  For those with sickle cell anemia, 11% will have experienced a stroke by the age of 20.  

That’s quite a list of ailments affecting African Americans.  If you look closely at all seven health issues you will see that the common denominator is your cardiovascular system.     

The key for reducing your health risk for these cardiovascular diseases is to make sure your cardiovascular system is as healthy as possible.  This article will provide several steps to address this but before we do you need to understand some of the underlining causes of why cardiovascular disease is so prevalent in the African American community. 

Several Underlining Causes! 

Within your cardiovascular system there is one particular essential amino acid, one critical signaling molecule, one critical cellular structure and one detrimental enzyme that directly impact its overall health.  They are: 

  • L-arginine – Key Essential Amino Acid
  • Nitric Oxide – Key Signaling Molecule
  • Endothelial Cells – Key Cellular Structure
  • Arginase – Key Detrimental Enzyme 

The first three keys of L-arginine, nitric oxide and endothelial cells are interrelated so we will examine them as a whole.  The endothelial cells form the interior lining of all your blood vessels.  These cells ultimately determine your cardiovascular health.  One function of these endothelial cells is to take the essential amino acid L-arginine and convert it into the signaling molecule nitric oxide.  Very simply, you couldn’t live without nitric oxide! 

Nitric oxide is the master signaling molecule of the cardiovascular system.  It regulates blood vessel tone and flexibility.  Its production is completely dependent upon the health of your endothelial cells and an adequate supply of the essential amino acid L-arginine.  Here are some of the benefits of nitric oxide: 

  • Nitric oxide regulates the muscle tone of blood vessels to have a major impact on controlling blood pressure.  This directly relates to high blood pressure.
  • Nitric oxide causes penile erections by dilating blood vessels.  This directly relates to erectile dysfunction.
  • Nitric oxide prevents blood platelet cells from grouping together in a clot.  This minimizes blockages in the blood vessels to reduce the risk for heart attacks, strokes and complications from sickle cell anemia.
  • Nitric oxide promotes vascular reparative mechanisms and is one of the keys to reversing atherosclerosis.  This helps to reduce the damage caused by high cholesterol levels and assists in preventing the vascular complications of diabetes. 

Nitric oxide is literally involved in all cells to help keep you fit and healthy.  It’s important to understand that this interrelationship between the endothelial cells, L-arginine and nitric oxide production is common for all races.  

What is not common is a genetic polymorphism inherent to the African American population.  This genetic polymorphism causes African Americans to have higher levels of an enzyme called arginase in their blood stream.  Arginase destroys L-arginine which is the main ingredient for creating nitric oxide in the body. 

For the African American community, nitric oxide production is compromised on both sides of the equation!

On the front end, the enzyme arginase destroys L-arginine which interferes with the production of nitric oxide.  On the back end, the health of your endothelial cells determines how much of the remaining L-arginine can be converted to nitric oxide.  Unfortunately, your endothelial cells are damaged by high blood pressure, high sugar levels, high cholesterol, smoking, and oxidative stress.  This damage reduces the production of nitric oxide which compounds the problem and increases the risk for cardiovascular disease. 

Steps to Decrease Your Risk! 

Given the above information there are several important steps to decreasing your risk for cardiovascular disease.      

Step 1 – Monitor your blood pressure.  High blood pressure damages your endothelial cells.  If you do not know what your blood pressure is then you need to go to your doctor’s office, local pharmacy or health clinic and get your blood pressure taken.  If you have high blood pressure, then you need to take steps to bring it back into a normal range.  This is your first and most important step.  It’s so important that the Black Barbershop Health Outreach Program was established to exclusively address the issue of high blood pressure on a national level.  For more information on participating barbershops, go to http://www.blackbarbershop.org/ .

Step 2 – Monitor your blood sugar and cholesterol levels.  High sugar and cholesterol levels damage your endothelial cells.  Diet is extremely important for both of these areas.  One simple but highly effective step to stabilize your blood sugar levels and help your body naturally reduce its cholesterol levels is to increase your fiber intake.  Including dry beans or legumes into your diet is a quick and delicious way to increase your fiber intake. In fact studies have shown that consuming dry beans four times or more per week, compared with less than once a week, lowered the risk of cardiovascular disease by 22 percent. 

Step 3 – Stop smoking!  Smoking damages your endothelial cells.  This is a nasty habit that is extremely hard to break.  Part of the process is having a motivation greater than the habit.  

If your very life isn’t motivation enough, then maybe for the men your sex life is! 

Your endothelial cells control your nitric oxide production.  Your nitric oxide levels control an erection.  No nitric oxide no sex!  Every puff on your cigarette is destroying your endothelial cells which lowers your nitric oxide level and reduces your chances of having good sex.  

Step 4 – Increase L-arginine intake!  In addition to protecting your endothelial cells, you need to get an adequate intake of the essential amino acid L-arginine which is found in foods like milk, cheese, yogurt, meat, and other protein sources.  Most people take in enough L-arginine to meet basic bodily needs.  However, when your diet is poor and/or your stress level is high, then this essential amino acid will be lacking.  Couple this with the enzyme arginase that destroys L-arginine and the potential risk for cardiovascular disease is increased. 

Step 5 – Use L-citrulline to bypass Arginase!  Your body will convert the amino acid L-citrulline into L-arginine to help maintain its production of nitric oxide.  This process also helps to bypass the arginase enzyme since it has no effect on L-citrulline.  Having a good supply of L-citrulline provides the body with a 24 to 36 hour window in the production of nitric oxide.  This is important for all population groups but it’s critically important for African Americans especially those with sickle cell anemia.  Unfortunately, L-citrulline is not prevalent in most foods we eat.  Thus, supplementation is usually required. 

Step 6 – Consider ProArgi-9 Plus!  There is a clinically proven nutritional supplement called ProArgi-9 Plus which has the ability to bring in the proper balance of L-arginine and L-citrulline.  This product also combines other heart healthy ingredients to significantly improve the health of the endothelial cells and their ability to properly produce nitric oxide.  The most recent validation for this product is the remarkable results obtain for 33 congestive heart failure patients in the High Desert Heart Institute study.  

Conclusion

You have the power to directly improve the health of your endothelial cells to significantly reduce your risk for cardiovascular disease.  And, for those with sickle cell anemia, you can help to reduce your symptoms.  It all centers on helping your endothelial cells function optimally, with an above average supply of L-arginine, and a way to bypass the arginase enzyme so that nitric oxide is properly produced.  Improved production of nitric oxide is critical to helping African Americans decrease their risk for cardiovascular disease!

Together we can work to save a million lives! 

Dan Hammer 

Dan Hammer has a background in biology, chemistry and exercise physiology.  He used to run one of the largest health club operations in the Chicagoland area and has been helping people with their wellness issues for more than 25 years.   
The information contained in this article is for general information purposes only and never as a substitute for professional medical advice or medical exam.  The information contain in this article has not been evaluated by the Food and Drug Administration and should not be used to diagnose, treat, cure or prevent any disease without the supervision of a qualified medical doctor.