Sickle Cell Disease – 14 Helpful Steps!

African American Doctor 2Sickle cell disease primarily affects people of African, African American, Mediterranean, Middle Eastern, and Asian Indian ancestry. There is also a growing segment in the Latino-American population particularly those of Caribbean, Central American, and South American ancestry that are affect 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.  In my companion article “African Americans and Sickle Cell Anemia” I covered a lot of material about the cause and symptoms of this disease.  

This article will provide you with 14 helpful steps in managing this disease.  Plus, new research confirms the importance of proper nitric oxide production in helping to reduce the painful symptoms of sickle cell anemia. 

Brief Overview of Sickle Cell Disease! 

Sickle cell disease is 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 much shorter life span than normal red blood cells.  This results in chronic anemia and reduced oxygen to the tissues of the body.  In addition, sickle cells are less flexible than normal red blood cells.  This presents a problem.  They become trapped in the small blood vessels preventing blood flow to the body’s tissues and organs.  This compromises the delivery of oxygen and usually results in pain and potential damage to the associated tissues and organs. 

Recent Research – The Importance of Nitric Oxide! 

Researchers at Duke University and Howard Hughes Medical Institute recently discovered one of the keys to the cause of pain in sickle cell disease.  Their findings were reported in the January 31, 2005 Proceedings of the National Academy of Sciences.  Their research showed that when normal red blood cells move through the arteries they release a signaling molecule that tells the arterial walls to expand.  The signaling molecule is nitric oxide.  Nitric oxide causes the smooth muscle in the wall of the blood vessel to relax.  This helps to open up the blood vessel allowing the red blood cells to pass through it.  

The researchers at Duke University discovered that when red blood cells distort to their sickle shape, the walls of the arteries don’t expand.  This distorted shape of the sickle cells, combined with the fact that they tend to clump together, ends up blocking blood flow through these small arteries and capillaries.  The Duke researchers also noted that as the blood pulses, the walls of the arteries didn’t expand like they do when normal red blood cells are present. 

Their research also noted that the degree of nitric oxide deficiency directly correlated with symptom severity for those with sickle cell disease.  This means that the less nitric oxide produced the greater the pain.  The Duke/HHMI study found that when nitric oxide was administered to people with sickle cell anemia their symptoms were relieved.  One of the conclusions from this study was that abnormal nitric oxide processing may be the real cause of sickle cell circulatory restrictions.  In addition to the Duke/HHMI study, several other studies have demonstrated that when nitric oxide is administrated to people with sickle cell anemia their symptoms are greatly reduced.         

1998 Nobel Prize in Medicine! 

In 1998 the Nobel Prize in Medicine was award to three American researchers.  Part of what came out of their research was that the primary pathway for creating nitric oxide in the body came from an essential amino acid called L-arginine.  L-arginine is called an essential amino acid because your body cannot produce it.  It must be brought into your body through your diet.  L-arginine is found in foods like milk, cheese, yogurt, meat, and other proteins.  In fact, L-arginine is considered one of the most important molecules ever discovered.  Columbia University refers to L-arginine as the “MAGIC BULLET” for the cardiovascular system.  

Genetic Polymorphism and the Importance of L-citrulline! 

Unfortunately, there is a genetic polymorphism common to the African-American community.  Many African-Americans produce too much of an enzyme called arginase.  In the bloodstream, arginase destroys L-arginine so that it is no longer available for the production of nitric oxide.  This compounds the problem of not have a sufficient supply of nitric oxide available to help relax the small arterial walls.  This is especially important when the red blood cells change into their sickle form. 

Fortunately, there is an amino acid that your body can convert to L-arginine to help bypass the arginase enzyme.  It is L-citrulline.  A recent paper (January 18, 2010) entitled Potential utility of full-spectrum antioxidant therapy, citrulline, and dietary nitrate in the management of sickle cell disease by MF McCarty discussed this very issue.  Here is a brief but important quote from this paper: 

“Suboptimal endothelial arginine levels, reflecting increased plasma arginase activity and elevated ADMA, contribute to the loss of NO bioactivity in sickle cell disease; supplementation with the arginine precursor citrulline may ameliorate this defect.” 

This means that proper dietary supplementation using the amino acid L-citrulline can help the body bypass the destructive effects of the enzyme arginase so that proper nitric oxide production can be restored.  Since nitric oxide regulates the muscle tone of blood vessels this becomes extremely important in preventing blockages.  Additionally, nitric oxide prevents blood platelet cells from grouping together in a clot which also helps to prevent blockages in the blood vessels. 

14 Steps to Help Those with Sickle Cell Disease!

Understanding the past and most current research will help to chart a plan of action to help those who suffer from sickle cell disease.  The following 14 steps will help in this process.  However, please remember that these steps are for informational purposes only and is not a substitute for professional medical advice, examination, diagnosis, or treatment.  Always seek the advice of a physician or other qualified healthcare provider with any questions you may have regarding this or any medical condition.  Never disregard professional medical advice or delay in seeking it because of something you have read in this article.  If you think you may have a medical emergency, always call your doctor or 911 immediately. 

Step 1 – Proper hydration.  It’s estimated that 75% of Americans have mild, chronic dehydration.  This will affect blood flow so getting the proper amount of water on a daily basis is critically important.  And, it must be pure water!  Pop, coffee, diet soda, alcohol, or other doctored beverages do not count.  Your body must replace 2-3 quarts of water everyday.  As a rule of thumb, for every 15 lbs of weight, you need 8 ounces (1 cup) of water.   

Step 2 – Stay warm.  Cold reduces blood flow because capillaries constrict.  If you live in a cold environment, then take extra precaution to keep your extremities, especially fingers and toes, properly protected.  

Step 3 – Avoid high altitudes.  Geographic locations with high elevations should be avoided due to the reduce level of oxygen in the atmosphere.

Step 4 – Exercise.  Intense exercise should be avoided but mild to moderate aerobic exercise can be a benefit.  Exercise improves blood flow.  Research has shown that blood flow across the endothelial cells will stimulate nitric oxide production.  Additionally, exercise will produce collateral capillary beds that can aid in providing alternative pathways for blood flow when an arterial pathway becomes blocked.  Collateral capillary beds are extremely important for those with sickle cell disease.  However, before starting an exercise program, you should always consult your physician.  

Step 5 – L-arginine supplementation.  The body utilizes L-arginine to create nitric oxide.  Nitric oxide is necessary for the proper function of the cardiovascular system especially in the control of the elasticity of the arterial walls.  

Now, before you go out and start buying L-arginine supplements you need to be aware of the dark side of this amino acid.  L-arginine ingested in its pure form tastes terrible.  By itself, L-arginine can have some serious side effects.  Because L-arginine can have negative side effects it must be properly formulated if it is to have a positive impact on helping the body create nitric oxide.  It is critical that the company and the person who formulates an L-arginine product have both experience and clinical documentation to its effectiveness.

From my experience, the best L-arginine supplement comes from Synergy WorldWide.  Their product, ProArgi-9 Plus, was developed by Dr. Joe Prendergast who is Board Certified in Internal Medicine as well as Endocrinology and Metabolism.  Dr. Prendergast is an expert in integrative L-arginine therapy and has been using it in his practice since 1991.  As a precaution, you should discuss this with both your physician and pharmacist especially if you are on any type of medication. 

Step 6 – L-citrulline supplementation.  This step is as important as step number 5 and maybe even more important.  Usually L-arginine provides a 30 minute to 2 hour window of improved nitric oxide production.  By supplementing with L-citrulline that window of improved nitric oxide production increases to 24 to 36 hours.  When properly combined with L-arginine and other important heart healthy ingredients, this dramatically improves the endothelial cells ability to properly produce nitric oxide, the master signaling molecule for the cardiovascular system.  This is an important component in ProArgi-9 Plus

Step 7 – Rest.  It’s important to get adequate rest since stress can have a negative effect on your overall health and wellness as well as lowering your immune system. 

Step 8 – Support your immune system.  There is so much that could be said in this area.  Increasing your consumption of antioxidant rich fruits and vegetables would be an important first step.  Daily consumption of at least 500 mg of vitamin C would help both the immune system and liver function.  

Step 9 – Support your liver.  Again, there is so much that could be said in this area.  Adequate amounts of green leafy vegetables with proper water intake are two good first steps to help the liver.  Taking 500 mg of vitamin C will help increase the rate of synthesis of glutathione which is a major component in liver detoxification. 

Step 10 – Avoid people with colds and flu.  Try not to place yourself in situations that allow for infections to occur.  This may require you to use a mask if your work or school environment is compromised with illness issues. 

Step 11 – Immunizations.  Stay up-to-date with the proper immunizations.  Work with your health care provider to set up a schedule so that all the proper steps are taken to stay on top of this area. 

Step 12 – Folic acid supplementation.  Folic acid is a necessary component for the production of red blood cells.  The recommended daily amount for adults is 400 micrograms and 600 micrograms in pregnancy.  If you are thinking about taking a larger amount, then you should discuss this with both your physician and pharmacist.  This is an important component in ProArgi-9 Plus.

Step 13 – Support groups.  Because of the emotional challenges that come with sickle cell disease it always helps to find a support group to share frustrations, challenges, hopes, and successes. 

Step 14 – Family planning.  Due to the genetic nature of sickle cell disease, it’s always helpful to discuss options with a family practitioner who has experience in genetic counseling.  

Because of the genetic nature of sickle cell disease there is no pill to cure it.  This can leave individuals and family members feeling helpless in their battle against the symptoms of this disease.  That’s why it’s important to discuss these 14 steps with a qualified physician.  Together you can develop a plan of action; one that will empower you to have better control over the symptoms of sickle cell 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.

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.