Difference Between Sickle Cell Anemia and Sickle Cell Disease: A Guide for Nigerians

Confused about the difference between sickle cell anemia and sickle cell disease? This guide explains it clearly for Nigerian patients and families.

Difference Between Sickle Cell Anemia and Sickle Cell Disease: A Guide for Nigerians

The sickle cell conditions are one of the important inherited blood disorders that impact the families in the country of Nigeria and the rest of Africa. But sickle cell anemia and sickle cell disease are sometimes used the same way which can cause confusion.  For patients and families, it is important to know the difference between sickle cell anemia and sickle cell disease to better understand the diagnosis, symptoms and complications of the disease and options for treatment. This is particularly crucial for Nigerians who have been affected by sickle cell and for those who are considering getting specialized treatment in other countries.

What is Sickle Cell Disease?

Sickle cell disease (SCD) is a collection of inherited blood disorders that result from a change in the gene that makes hemoglobin. The protein in a red blood cell that transports oxygen around the body is called hemoglobin. In individuals with sickle cell disease the abnormal hemoglobin can make the red blood cells hard and bend into a sickle or crescent shape. Such cells can deteriorate and can also clog small blood vessels. If a child receives two abnormal genes that code for hemoglobin, it is known as sickle cell disease. The condition may occur in a variety of different ways due to different genetic combinations. The most frequent and serious is called HbSS and is what people often refer to as sickle cell anemia.

What is Sickle Cell Anemia?

A type of sickle cell disease is called sickle cell anemia. It is typically linked with the "HbSS" type of gene where a person receives two sickle hemoglobin genes. Sickle Cell Anemia can lead to chronic anemia as the red blood cells with sickle hemoglobin can have a shorter life. The abnormal cells can also block blood flow and may cause the person to experience periodic, severe pain and other problems.

Symptoms include:

  • Persistent anemia and weakness.
  • Pain crises
  • Hands and feet swelling (especially in children),
  • Frequent infections
  • Jaundice
  • Children growing slower than normalChildren's growth is slow
  • Shortness of breath
  • Organ complications (kidneys, lungs, eyes, brain, etc.).

Symptoms may be mild to severe and are not always present in a person.

The difference between Sickle Cell Anemia and Sickle Cell Disease

The easiest way to grasp the difference between sickle cell anemia and sickle cell disease is to consider these two conditions as a family. There are a number of inherited hemoglobin diseases all of which are classified as sickle cell disease. One of those conditions is sickle cell anemia and it is generally taken to be the most severe.

Sickle cell disease can be accompanied by:

  • Sickle cell anemia (HbSS) is the name given to this disease.
  • HbSC disease
  • HbS beta-thalassemia
  • Other less common sickling disorders
  • So, all people with sickle cell anemia have a form of sickle cell disease, however, not all people with sickle cell disease have sickle cell anemia.

This distinction is important because the symptoms, complications and treatment requirements may vary from one person to another based on their specific genotype.

Why is it important to know the difference between Sickle Cell Anemia and Disease?

The distinction between the sickle cell and sickle cell disease is important because the treatment of the individual must be tailored to the specific diagnosis not just that of the general term sickle cell. For instance, a person with HbSS may have more vaso-occlusive pain crises and may be severely anemic whereas another individual with a different form of SCD may not have any pain crisis or be as anemic.

Therefore, doctors might want to consider:

  • Hemoglobin genotype
  • The occurrence of pain attacks, and how often they happen.
  • Hemoglobin levels
  • Previous complications
  • Organ function
  • History of blood transfusions
  • A person's age and general health and fitness.
  • A prior exposure to treatment.

Doctors can make a treatment plan through a comprehensive evaluation that is appropriate to the person.

What are the signs that might help detect Sickle Cell Disease?

The diagnosis typically is based on blood tests to determine what type of hemoglobin is present in the blood. Determination of haemoglobin electrophoresis or other specialised haemoglobin analysis may be helpful to diagnose sickle cell disease and confirm the genotype. Sometimes, genetic testing can also be suggested. Newborn screening can lead to the child being identified early and medical teams can start preventive care before any serious complications occur. The early diagnosis may be of great importance in Nigerian families because periodic monitoring and preventive treatment can help to minimize the risk of serious complications.

Treatment options for Sickle Cell Disease

No one treatment fits all patients. The treatment of sickle cell disease is dependent on the genotype of the patient, the symptoms, the age, the complications and overall health. Treatment can consist of medication, preventive measures, blood transfusion, management of acute complications and in selected patients, potentially curative therapy.

Hydroxyurea

  • Hydroxyurea is a proven drug that is already being used by many sickle cell disease patients. Can raise fetal hemoglobin levels and may help to decrease the incidence of some problems such as painful episodes in suitable patients. Patients are regularly monitored by doctors during treatment with the medicine.

Pain Management

  • One of the most well-known complications of SCD is pain crises. Treatment will include hydration, suitable pain medications, oxygen (if clinically appropriate) and treatment of underlying triggers. If you suffer from severe pain or atypical pain, it is advisable to see a health professional instead of trying to treat all of your pain episodes yourself.

Blood Transfusions

  • In special cases, such as very low blood counts or some serious complications, blood transfusion might be suggested. Repeat transfusion may be necessary for some patients and these may need to be monitored for iron overload and other transfusion-related problems.

Preventive Care

  • Preventive care is an important aspect of sickle cell disease treatment. This may involve vaccinations, infection prevention, frequent medical check-ups, organ screenings and proper hydration depending on age and individual needs.
  • Children with a sickle cell disease will need careful monitoring as complications can occur in childhood.

SCT or BMT

  • In some patients, a bone marrow transplant, or Hematopoietic Stem Cell Transplantation (HSCT), may be a viable option for treatment that can cure sickle cell disease.
  • But it is a big surgery and it may not be suitable for all. They take into account the age, the severity of the disease, the health of the patient, availability of donors and risks of treatments.
  • More advanced treatment options, such as transplantation may be considered at specialized centers.

Emerging Gene-Based Treatments

  • Selected patients with severe sickle cell disease have been the recipients of new approaches in advances in genetic medicine. Some gene-based therapies alter the blood-forming stem cells in a patient to make them capable of making healthier red blood cells or to boost fetal hemoglobin.
  • All countries have different availability, eligibility, long-term follow-up criteria, cost and regulatory approval. Patients who are thinking of using advanced therapies will need to have a specialist center evaluation.

Seeking Sickle Cell Treatment in India

Nigerian patients seek specialist care in India should they require complex procedures, advanced evaluation, special assessment for transplantations or specialized hematology services. Patients are advised to request their clinical history, previous blood tests, genetic testing results, drug history, transfusion history and any specialist or imaging reports prior to traveling. Medical tourism facilitator can assist in arranging doctor visits, medical advice, travel arrangements, hotel stays and more. GetWellGo caters to international patients who require specialized medical treatment in India by assisting them in arranging their treatment process from consultation to treatment at the hospital and travel arrangements.

Conclusion

It's important for patients and families to understand the difference between sickle cell anemia and sickle cell disease. The term sickle cell disease is used to describe all the inherited disorders while sickle cell anemia is usually used to describe the HbSS form of the disease. The difference between sickle cell anemia and disease also underscores the importance of knowing the exact genotype of a patient. The clinical course and treatment of the various forms of sickle cell disease may vary. Today, prevention, medications including hydroxyurea, pain relief, blood transfusion (as needed) and in some patient’s stem cell transplant are used to treat sickle cell disease. There are also newer gene-based therapies that are providing an increasing number of options for those who qualify. GetWellGo may facilitate specialist consultation and support patients' journey for advanced sickle cell evaluation or treatment in India.

Difference between sickle cell anemia and sickle cell disease GetWellGo

GetWellGo is regarded as a leading supplier of healthcare services. We help our Nigerian clients choose the best treatment locations that suit their needs both financially and medically.

We offer:

  • Complete transparency
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  • 24 hour availability.
  • Medical E-visas
  • Online consultation from recognized Indian experts.
  • Assistance in selecting India's top hospitals for sickle cell disease treatment.
  • Expert hematologist with a strong track record of success
  • Assistance during and after the course of treatment.
  • Language Support
  • Travel and Accommodation Services
  • Case manager assigned to every patient to provide seamless support in and out of the hospital like appointment booking
  • Local SIM Cards
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