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sickle cell disease

Sickle Cell Disease: Types, Causes, Treatment & Management

Do you know that Nigeria leads the world in the number of patients with sickle cell disease (SCD)?

Around 150,000 babies are born with SCD every year in the country and 70-90% of them die before the age of 5.

Until a few decades ago there was no treatment option for this disease and doctors used to prescribe medications to lower the symptoms and prolong life.

Luckily, with the advancement of technology, SCD can be treated by bone marrow transplant. To know more about SCD, let’s go deep into what EXACTLY it is and how it can be treated.

But before going further, let us tell you the success story of one of our patients from Nigeria.

Apunanwu, an 8-year-old girl from Nigeria, had been battling SCD since birth. Her life was a constant struggle with pain and fatigue, and her mother, Daluchi, and father Akunna, watched helplessly as their daughter’s health deteriorated day by day. In their small village, healthcare options were limited.

One day, Daluchi was chatting with a neighbor, Chinwe, who heard wonderful things about Medico Experts. Chinwe explained that MedicoExperts had helped her friend’s child receive life-changing treatment in India at a very reasonable cost.

Intrigued, Daluchi asked for more information and learned that it is a global virtual hospital specializing in connecting patients with world-class medical care, including stem cell treatments or bone marrow transplants.

sickle cell disease
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With newfound hope, Daluchi and Akunna reached out to MedicoExperts, and our team quickly responded. They explained the process of getting Apunanwu the treatment she needed. It involved a trip to India, where a team of skilled medical professionals would perform stem cell therapy, a potentially life-saving procedure.

Though it gave them hope, Daluchi and Akunna started developing cold feet thinking about the trip and the cost related to the treatment. 

Thankfully for them, their daughter was in the right hands.  MedicoExperts went above and beyond to help the family. The organization’s support was a lifeline for the desperate parents.

Once in India, Apunanwu underwent stem cell treatment under the care of a highly experienced medical team. The procedure held its risks and challenges, but Daluchi and Apunanwu’s determination and faith never wavered. They watched their daughter endure the process with a mixture of fear and hope, knowing that this was their best chance for a brighter future.

Months passed, and slowly but surely, Apunanwu’s condition began to improve and her energy levels started to rise. The transformation was nothing short of miraculous. Daluchi and Apunanwu could hardly believe their eyes as their daughter’s smile returned and the sparkle in her eyes grew brighter with each passing day.

MedicoExperts continued to support them throughout the recovery process, ensuring that Apunanwu received the necessary follow-up care and medications.

Inspiring, isn’t it?

Now let’s learn what sickle cell disease is.

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What is Sickle Cell Disease

What Is Sickle Cell Disease?

Sickle cell disease (SCD), also known as sickle cell anemia, is a genetic blood disorder that affects the shape and function of red blood cells. 

Normally, red blood cells look like flat disks or doughnuts and are flexible, allowing them to flow smoothly through blood vessels, carrying oxygen to various parts of the body. However, in individuals with sickle cell disease, a genetic mutation causes their red blood cells to become rigid and take on a crescent or “sickle” shape. 

These abnormally shaped cells can get stuck in narrow blood vessels, leading to reduced oxygen supply to tissues and causing severe pain, anemia, and various complications. 

Sickle cell disease is a chronic and often painful condition that requires ongoing medical management, and it is more prevalent in certain populations, including those of African, Mediterranean, Middle Eastern, and South Asian descent.

You will better understand this disease if you learn about its symptoms. So, let’s learn.

What are the Symptoms of SCD?

The early symptoms of this disease are

  • Extreme tiredness and fussiness because of the anemia
  • Swelling and painfulness in hands and feet
  • Yellowish skin or white of the eye due to hemolysis (breakdown of red blood cells) in a large number of red blood cells.

But when you see the following symptoms you should immediately consult a healthcare provider:

  • If the patient has a fever of more than 101.3 degrees Fahrenheit
  • The patient shows signs of severe anemia like fatigue, dizziness, shortness of breath, irregular heartbeat, etc. 
  • If the person complains of chest pain and shortness of breath along with fever and coughing
  • If the patient is showing the signs of a stroke like numbness in one side of the body, sudden weakness, and problems in speech, sight, and hearing. 

If you or anyone in your close circle is showing these symptoms, seek medical care or call emergency services. 

Diagnosing it at the right time can save you and your near and dear ones from these life-threatening situations. 

Symptoms Sickle Cell Disease
Sickle Cell Disease

How to diagnose this condition?

If you or your child has symptoms of this condition, your healthcare provider will use one or more of these tests to diagnose it:

  • Blood test 

A blood test is done to check for the presence of the abnormal hemoglobin that causes the disease. This test is usually done soon after a baby is born in many places, as it’s important to catch the condition early. If someone is older and has symptoms like pain or anemia, a blood test can also be done to look for abnormal hemoglobin. 

  • Genetic Test

You may also have a genetic test to make sure that your genes are not carrying or have the traits of sickle hemoglobin that you could pass on to your child.

  • Newborn screening

Newborn screening for sickle cell disease is typically conducted through a simple blood test shortly after birth. A few drops of blood are collected from the baby’s heel and analyzed in a laboratory. 

This test measures the presence of abnormal hemoglobin, specifically hemoglobin S, which is characteristic of sickle cell disease. If the test results indicate the presence of hemoglobin S, further confirmatory tests may be performed to confirm the diagnosis. 

Early detection through newborn screening is crucial as it allows for prompt medical intervention and management to improve the health outcomes of infants with sickle cell disease.

  • Prenatal screening

The doctors can diagnose whether the babies have sickle cell disease before they are born. This test can be done as early as eight to ten weeks of your pregnancy. 

For this test, the doctors will take a sample of either your amniotic fluid (the fluid in the sac surrounding the embryo) or tissue samples from the placenta (the organ that attaches the umbilical cord to the womb).

What are the different types of SCD?

There are many types of SCD but the most common are the following:

When children inherit a sickle cell gene from their parents, they have sickle cell anemia. These deformed blood cells can block blood flow, leading to pain, anemia, and various complications. 

Sickle cell anemia is one of the most common forms of sickle cell disease and is caused by a mutation in the hemoglobin gene.

Sickle Hemoglobin-C Disease (SC) is a genetic blood disorder that results from inheriting one copy of the HbS gene (sickle hemoglobin) and one copy of the HbC gene (hemoglobin C).

It leads to the production of both abnormal hemoglobin S and hemoglobin C, causing red blood cells to assume irregular shapes under certain conditions, potentially leading to pain, anemia, and other complications, although usually less severe than classic sickle cell anemia (HbSS). SC disease is one of the many variations of sickle cell disease and can vary in its clinical manifestations.

Sickle Beta-Plus Thalassemia is a genetic blood disorder that combines features of both sickle cell disease and beta-thalassemia. Individuals with this condition have one copy of the HbS gene (sickle hemoglobin) and one copy of a mutated beta-thalassemia gene.

This combination results in the production of abnormal hemoglobin and decreased production of normal hemoglobin, leading to a range of symptoms similar to those seen in sickle cell disease, including anemia and the potential for painful sickle cell crises.

Sickle Hemoglobin-O Disease is a rare genetic blood disorder that occurs when an individual inherits one copy of the HbS gene (sickle hemoglobin) and one copy of the HbO gene (hemoglobin-O). This combination results in the production of both abnormal hemoglobin S and hemoglobin O.

People with this condition can experience symptoms similar to those with sickle cell disease, including pain crises and anemia, although the severity and clinical presentation can vary. Sickle Hemoglobin-O Disease is one of the many different forms of sickle cell disease.

Sickle hemoglobin-D disease is a rare genetic blood disorder caused by inheriting one copy of the HbS gene (sickle hemoglobin) and one copy of the HbD gene (hemoglobin-D).

This combination leads to the production of both abnormal hemoglobin S and hemoglobin D. Individuals with this condition may experience symptoms similar to those of sickle cell disease, including pain crises and anemia, though the severity and clinical manifestations can vary. Sickle hemoglobin-D disease is one of the less common forms of sickle cell disease.

How to Treat SCD?

Treating sickle cell disease involves a combination of managing symptoms, preventing complications, and improving overall quality of life. Here’s a concise overview of the key treatments and strategies:

A bone marrow transplant can potentially cure sickle cell disease. This procedure involves replacing the patient’s unhealthy bone marrow, which produces the defective sickle hemoglobin, with healthy bone marrow from a compatible donor. The transplanted marrow produces normal hemoglobin, effectively eliminating the root cause of sickle cell disease.

However, it’s essential to note that bone marrow transplantation is not without risks and challenges. Finding a suitable donor match, often a sibling or unrelated donor, can be difficult. 

The procedure itself carries risks, including complications from the transplant process and the need for lifelong immunosuppressive medications. 

Due to these factors, bone marrow transplantation is typically considered for individuals with severe sickle cell disease and life-threatening complications when other treatment options have been exhausted. Nevertheless, when successful, it can offer a potential cure for the disease.

Pain is a common symptom of sickle cell disease, often due to blocked blood vessels. Doctors use  Medications like opioids or nonsteroidal anti-inflammatory drugs (NSAIDs) to manage pain during crises.

Hydration and warm baths can help alleviate pain by relaxing blood vessels.

This medication can increase the production of fetal hemoglobin, which reduces the likelihood of sickle cell crises. It’s particularly effective in preventing severe complications and reducing the frequency of pain episodes

In severe cases, regular blood transfusions may be necessary to replace sickled red blood cells with healthy ones. This can help prevent organ damage and improve overall health.

Sickle cell patients are at higher risk of infections. Vaccinations and prophylactic antibiotics can help prevent bacterial infections like pneumonia.

Non-pharmacological approaches like relaxation techniques, physical therapy, and counseling can complement medications for better pain management.

Staying well-hydrated, avoiding extreme temperatures, and maintaining a balanced diet can help reduce the frequency of sickle cell crises.

Frequent check-ups and monitoring of blood counts, organ function, and overall health are essential for early intervention and management.

Patients and their families benefit from education about sickle cell disease, including its management, signs of complications, and available resources for support.

Here, you need to remember that timely diagnosis and treatment of this disease can prevent a huge number of deaths. 

What is the cost of SCD treatment?

In India, the expenses for treating sickle cell disease vary depending on the recommended treatment tailored to the patient’s specific condition. When it comes to addressing this challenging ailment, a bone marrow transplant emerges as the most promising solution. 

The cost of this transformative procedure in India can commence at range from $22,000 USD to $60,000 USD, although the exact expenses depend on the specific type of bone marrow transplantation required.

Cost of Sickle Cell Disease
Sickle Cell Disease

Why go to India for SCD treatment?

At the beginning of this year, India’s Finance Minister Nirmala Sithraman said that the Indian Government will work in “mission mode” to eradicate sickle cell disease by 2047 from the country. 

Apart from the push from the government, India is increasingly recognized as a favorable destination for treating sickle cell disease due to several key factors. 

Firstly, the country boasts a vast pool of highly skilled medical professionals, including hematologists (the study of blood and blood disorders) and transplant specialists, who have extensive experience in managing hematological disorders. Their expertise, combined with state-of-the-art medical facilities and cutting-edge technology, ensures that patients receive world-class care.

Secondly, India offers cost-effective healthcare solutions, making it an attractive option for individuals seeking treatment for sickle cell disease. The affordability of medical services, including bone marrow transplants, is significantly lower in India compared to many Western countries, making it accessible to a wider range of patients. 

This combination of medical expertise and cost-effectiveness makes India a great choice for those seeking effective treatment for sickle cell disease.

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Final Thoughts

Remember, the treatment plan for sickle cell disease should be tailored to each individual’s specific needs and may evolve over time as new therapies become available. Regular communication with your healthcare providers is essential for optimizing care and improving the quality of health for those living with this condition.

MedicoExperts, the global virtual hospital, is your trusted partner in bridging the gap between Nigeria and India for the treatment of sickle cell disease. With a mission to provide top-notch healthcare at affordable prices, MedicoExperts connects you with a network of highly experienced super-specialized doctors and well-equipped hospitals in both countries. Through online video consultations, you can obtain a second opinion from these experts, ensuring you have access to the latest and most advanced treatments with success rates that meet international benchmarks.

What sets MedicoExperts apart is its commitment to offering multiple cost options depending on the hospital facilities, all while keeping the same doctor you trust. Moreover, with treatment options available in multiple cities, states, and countries, you have the flexibility to choose what suits you best. Trust MedicoExperts to provide you with peace of mind as you embark on your journey towards effective sickle cell disease treatment. Your health is our priority, and we’re here to make your path to recovery as seamless as possible.

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Frequently Asked Questions and patient concerns:

Q1. Can a sickle cell patient live long?

People with SCD can live an active life if they consult with a doctor and go for check-ups regularly. With proper care and management, sickle cell patients can live long, fulfilling lives.

Q2. What is the new drug for sickle cell disease?

Crizanlizumab-tmca is the new drug for sickle cell disease. It is an injection that is approved for adults and children of 16 years and above. 

Q3. Can sickle cell be treated with diet?

A study conducted in 2021 revealed that by addressing nutritional deficiencies linked to Sickle Cell Disease (SCD), it is possible to potentially alleviate the severity of SCD symptoms and enhance an individual’s overall quality of life.

Q4. What should sickle cell patients avoid?

Sickle cell patients should avoid situations that can trigger a sickle cell crisis, which includes extreme temperature changes, dehydration, and high-altitude locations. 

Additionally, they should steer clear of excessive physical stress or strenuous activities that can increase the risk of red blood cell sickling and pain episodes. It’s crucial for them to stay well-hydrated, maintain a balanced diet, and follow their healthcare provider’s guidance to manage their condition effectively.

Most importantly, they should avoid alcohol and smoking, as alcohol can cause dehydration and smoking can trigger serious lung problems.

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