sickle cell anemia

Among the many types of anemia, sickle cell anemia is the most serious. It is an inherited disorder of red blood cells, which causes chronic anemia. 

Fortunately, there are a number of options available for treating sickle cell anemia. Some of them involve dietary supplements, but there are also medications that you can take to treat the disease. 

These treatments are used to improve blood flow, lower high blood pressure, and prevent the growth of clots.

Understanding Sickle Cell Anemia

Swollen Hands and Feet

Symptoms of sickle cell disease may include pain, swelling, fever, and anemia. The disease is an inherited disorder that causes sickle-shaped red blood cells (RBCs) to break down faster than normal red blood cells. 

These sickle cells can block blood flow, causing tissue damage and anemia. These cells can also cause infections, which can be life-threatening.

Children and adults can develop pain crises. These episodes vary in severity depending on the organ involved. They usually occur due to injury or illness. Pain crises may require hospitalization and require analgesia to relieve the pain.

If the pain crisis is severe, it may require the use of analgesics, electrical nerve stimulation, or biofeedback. These treatments can be helpful in relieving the pain and preventing further injury.

In children, swollen hands and feet may be the first symptom of sickle cell disease. Children may develop this condition as early as six months, although some may not show symptoms until after ten years.

Leg Ulcers

Despite the progress in treatment of sickle cell anemia (SCD), leg ulcers continue to occur, especially in patients aged 15 years or younger. 

Leg ulcers are disabling, painful, and slow to heal. They can result in complications such as pulmonary hypertension and thrombosis of the lower extremity. In addition, the onset of leg ulcers may be associated with local trauma.

The National Health Service reports that more than 60% of all leg ulcers occur as a result of venous leg ulcers, which are the most common type.

Leg ulcers are often chronic and may persist for years. Although they are painful, they can be managed with transfusions, surgical procedures, and dermatology. These treatments involve debridement, wound care, and education.

The incidence of leg ulcers among patients with SCD is about 10 times higher than the general population. There is also a high mortality rate among patients with leg ulcers.

Leg ulcers are categorized as acute, chronic, and recalcitrant. The recalcitrant group is defined as leg ulcers that are present for six months or longer.

Deep Vein Thrombosis

Thrombotic complications occur in patients with Sickle Cell Disease (SCD) and increase morbidity and mortality. There is evidence that venous thromboembolism (VTE) is a significant contributor to the overall progression of SCD. 

However, there is limited information on the relationship between SCD and VTE. Consequently, there is a need to evaluate the risk of thrombosis in SCD patients to determine those at highest risk.

The underlying mechanism of sickle cell disease is the polymerization of hemoglobin S (HbS). This causes sickled red cells and poor microvascular blood flow. The result is vasoocclusion, which promotes thrombosis. In SCD, thrombosis is mediated by thrombin, a coagulating protein.

Chronic activation of the coagulation system is a possible explanation for increased risk of VTE in patients with SCD. Several biomarkers indicate hypercoagulability.

Treatments for Sickle Cell Anemia

person with sickle cell anemia

The only cure for sickle cell disease is a blood and bone marrow transplant. After early diagnosis, a healthcare provider may prescribe medicines or transfusions to treat complications.

In addition, the following treatments may be used:

  1. Hydroxyurea (Droxia, Hydrea, Siklos). In the body, hydroxyurea increases the size of red blood cells.
  2. L-glutamine oral powder (Endari).
  3. Crizanlizumab (Adakveo).
  4. Voxelotor (Oxbryta). 
  5. Pain-relieving medications. During sickle cell pain crises, your doctor may prescribe narcotics to alleviate pain.

Does Voxelotor Increase Hemoglobin?

Earlier this year, the US Food and Drug Administration approved the use of the drug Voxelotor for sickle cell anemia. This drug is a molecule that inhibits the polymerization of hemoglobin S. Sickle cell anemia is a genetic disorder that causes rigid red blood cells.

This disorder is a severe illness that affects many parts of the body, including the brain. The disease causes chronic pain and other complications that may lead to disability. 

To manage complications, patients often need blood transfusions or opioids. However, these medications can cause side effects, including nausea and headaches.

The US Food and Drug Administration approved Voxelotor in November 2019. It is now available as a tablet, in a grape-flavored suspension. It should be taken at the same time each day.

Is Glutathione Good for Sickle Cell Patients?

Glutamine is a conditionally essential amino acid. It is a precursor of nicotinamide adenine dinucleotides (NAD). 

It has been shown to increase the proportion of the reduced form of nicotinamide and adenine in sickle cell erythrocytes. 

It also plays an important role in synthesis of antioxidants and nitric oxide. It is also known to help HIV/AIDS patients absorb food better.

Oxidative stress is a major contributor to sickle cell disease, which leads to hemolysis. L-glutamine therapy improves oxidative stress in sickle cell erythrocytes. Glutamine increases NAD redox ratios and red cell glutamate. These changes may reduce vaso-occlusive crises in sickle cell disease.

Pharmaceutical-grade L-glutamine is effective in adult patients with sickle cell anemia. In a clinical trial, the group treated with L-glutamine showed a decrease in acute pain crises and pain-related hospitalizations.

What is the Main Cause of Sickle Cell Anemia?

Symptoms of sickle cell disease usually appear in the first few months of a child’s life. They can also be present later in a person’s life.

The main cause of sickle cell disease is a change in the hemoglobin gene. Hemoglobin is a protein that carries oxygen from the lungs to the tissues. The change in hemoglobin causes red blood cells to become rigid and sickle-shaped. This causes blockages in blood vessels, leading to anemia.

What Happens to a Person With Sickle Cell Anemia?

sickle cell anemia life expectancy

Several different complications can result from sickle cell disease. Some of them include lung and kidney damage, painful leg ulcers, and even strokes. 

These symptoms vary from one person to the next, and they can have a major impact on a person’s life. It is important to seek medical care if you are experiencing any of these symptoms.

How Long Can You Live With Sickle Cell Anemia?

Sickle cell disease patients can live full lives and enjoy most of the things people do in their normal lives. 

CDC report states that nearly 95% of people born with SCD in the United States reach 18 years of age; however, people with the most severe forms of SCD have a life expectancy 20-30 years shorter than those without SCD.

According to a study published recently in Rare Disease Advisor, people with sickle cell disease normally live for 54 years. This is approximately 20 years shorter than the life expectancy of people without sickle cell disease.

Final thoughts

Those suffering from sickle cell anemia have chronic anemia that leads to pain and fatigue. Often the pain lasts for days or weeks. This is why regular medical care is recommended.

People with sickle cell anemia have a greater risk of developing infections. They also have a higher risk of stroke. When red blood cells get stuck in the blood vessels they form a sickle shape and block blood flow. This can increase the risk of infections and put people at risk of kidney damage.