Sickle Cell Disease Treatment for Nigerian Patients

Sickle cell disease treatment

Sickle cell disease (SCD) is a hereditary blood disorder that occurs when hemoglobin is abnormal and the red blood cells become hard and sickle-shaped. These cells will obstruct blood flow leading to pain crises, anaemia, infections, stroke, acute chest syndrome, etc. Treatment depends on the individual's age, the type of SCD, the frequency of pain crises, the anemia, organ complications and previous treatments.

Sickle Cell Disease Treatment options:

Hydroxyurea

• Hydroxyurea is a very significant long-term therapy for many patients. It could improve fetal hemoglobin and lower sickling thus decreasing the rates of acute chest syndrome, hospitalizations, transfusion requirements and pain crises. It needs to be monitored medically regularly.

Medicines for pain management

• Depending on the severity of a pain crisis, it may need treatment with non-opioid pain relievers, prescription pain medications or other medications. If severe, may need hospital care including pain and fluid to be given intravenously.

Blood Transfusion

• Severe anemia, stroke, acute chest syndrome, surgery or other serious complications may be indications for red blood cell transfusion. Complications like iron overload and immune reactions should be monitored in patients who receive repeated transfusions.

L-glutamine and Other Medicines

• L-glutamine could be a helpful treatment for decreasing the incidence of a pain crisis and hospitalization in qualified patients. In some situations there's also another treatment called crizanlizumab. Treatment options will vary based on patient factors and availability of local regulatory treatments.

Preventive/Supportive Care

Long-term management may involve:

• Adequate hydration
• Vaccinations and infection prevention are recommended
• Regular blood tests and monitoring of organs
• Children's Stroke-risk screening by transcranial Doppler ultrasound
• Eye examinations
• Treatment of other complications, including kidney, lung and heart.
• Stay away from the known causes like dehydration and extremes of temperature

Bone Marrow Transplant

Selected patients may be able to be cured of sickle cell disease by a hematopoietic stem cell transplant. It is only used when the disease is severe and there are complications and a suitable donor is present. Ideally, a sibling that is close in age is the person who would donate.

There are numerous risks to transplantation such as infections, graft-versus-host disease, infertility, transplant failure and more. So, health professionals narrow down the pros and cons before offering advice on its use.

Gene Therapy

New treatments using genes are available for a few patients with severe SCD. Some acceptable options include removing a patient's blood-forming stem cells, changing them and re-transplanting them following a conditioning chemotherapy course. Eligibility criteria include age, severity of the disease, previous treatments and availability of regulation.

Malaria treatment in sickle cell disease

Prompt diagnosis and treatment of malaria is important in individuals with sickle cell disease (SCD). Malaria can exacerbate anemia and cause complications like a vaso-occlusive pain crisis, acute chest syndrome, dehydration and organ dysfunction, given that SCD is already a condition that causes chronic anemia and red-cell breakdown. When serious, malaria is a medical emergency.

Malaria Treatment in Sickle Cell Disease (SCD):

Confirm malaria quickly

• Malaria RDT or blood microscopy is the best way to diagnose a patient with SCD and fever as soon as possible. 

Treat uncomplicated malaria

• Plasmodium species, geographic region and local resistance patterns determine the antimalarial medicine. Artemisinin-based combination therapies (ACTs) are recommended as the primary treatment for P. falciparum. This includes artemether-lumefantrine and dihydroartemisinin-piperaquine.
• The disease of sickle cell does not require a totally different regimen of antimalarial treatment. But there is a need for clinicians to be more vigilant as problems can present quickly.

If the malaria is severe, it should be treated in a hospital

Warning signs include:

• Severe anemia
• If breathing difficulties or sudden chest symptoms occur.
• Confused and/or seizures/loss of consciousness
• Damage to the kidneys or decrease in urine production
• Shock or extremely low blood pressure
• Anemia with other symptoms of severe malaria
• High parasite levels

If the malaria is severe, intravenous artesunate is used followed by the appropriate complete oral antimalarial therapy once the patient can swallow the medication.

Concurrently treat sickle cell complications

Additional conditions that may need to be controlled are also:

• Severe anemia and may require blood transfusion
• Pain crisis
• Dehydration
• Fever and infection
• Acute chest syndrome
• Low oxygen levels
• Kidney complications

Care should be taken to monitor the patient's hemoglobin and the patient's clinical condition, since both malaria and SCD may be causes of anemia.

Prevention of Malaria

• Children and adults with SCD who reside in or visit malaria endemic areas are especially at risk for prevention. These can be insecticide treated bed nets, mosquito control and where appropriate and recommended by local guidelines, malaria chemoprevention. 

Treatment of sickle cell disease in Nigeria

Sickle cell disease (SCD) is a lifelong inherited blood disorder that needs routine preventative care, treatment for acute complications and surveillance for organ damage. Nigeria ranks among the top countries with high burden of SCD in the world.

Treatment of sickle cell disease in Nigeria includes the following:

Hydroxyurea Therapy

Hydroxyurea is an important disease-modifying treatment. It stimulates the production of fetal hemoglobin (HbF) which can decrease red-cell sickling and help to limit pain crises and complications. Hydroxyurea is part of Nigeria's national guideline for patients with more severe disease complications such as recurrent crises, abnormal transcranial Doppler (TCD) results, acute chest syndrome, and stroke. It should be monitored regularly with blood counts and clinical monitoring in patients receiving it.

Treatment of Pain Crises

A vaso-occlusive crisis can lead to sudden pain in bones, chest, abdomen or joints. Treatment may include:

• Appropriate pain-relieving medicines
• Adequate hydration
• Treatment of the infections or other triggers.
• Oxygen as clinically indicated
• Hospitalisation for serious or complicated cases

If there is a need for long-term treatment to change the course of the disease, this should be reviewed if the crisis is repeated or severe.

Blood Transfusions

Severe anemia, stroke, acute chest syndrome, major surgery or other serious complications may require blood transfusion. Sometimes, exchange transfusion may be necessary and not just simple transfusion in some patients. Patients receiving repeated transfusion should be monitored as repeated transfusion may lead to iron overload and other problems.

Infection Prevention and Treatment

SCD individuals have an increased risk of severe infections. Management can include:

• Childhood/Adult Immunization Schedule
• Suitable antibiotic pre-treatment in certain cases
• Early treatment of bacterial infections
• Prevention and prompt treatment if appropriate, for malaria.

Monitoring for Complications

It is important to follow-up with a hematologist or SCD specialist regularly to detect complications early. Monitoring may include:

• Complete blood counts
• Assessment of hemoglobin and HbF.
• Prevents kidney and liver damage.
• Screening in children who are at risk of stroke using transcranial Doppler.
• Eye examinations
• Diagnosis of avascular necrosis
• Recurrent pain or acute chest syndrome - evaluation

Stem Cell Transplantation

In the right patients, SCD may be curable through hematopoietic stem cell transplantation (HSCT). It is usually advised if the disease is serious and the benefits are fairly high when compared to potential transplant risks. Transplantation is recommended only by a specialist transplant centre after assessing conditions like disease severity, complications in the organs, availability of donors, age and health.

Conclusion

Treatment for sickle cell disease is ongoing and should be comprehensive because the treatment goal is to minimize pain crises, prevent complications and enhance the quality of life for a person's lifetime. Treatment can include hydroxyurea, pain management, blood transfusion, preventing infection, managing malaria, frequent check-ups and referral to a specialist in Nigeria. However, in select cases, a potential cure is available for patients with severe disease – hematopoietic stem cell transplantation. A timely diagnosis, periodic follow-up and availability of experienced hematology specialists are crucial to improve the long-term outcome. In other cases, when advanced treatment options or transplant services are needed, patients in Nigeria with complex or severe sickle cell disease can explore specialist treatments available abroad.

Sickle Cell Disease treatment for Nigerian patients in India 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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• 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
• Currency Exchange
• Arranging Patient’s local food

FAQ

Can sickle cell disease be cured?

• SCD is a lifelong disease. But, in selected patients, the disease can be cured using hematopoietic stem cell transplantation. In certain countries, newer treatments that are based on genes are also emerging.

Can malaria make sickle cell disease worse?

• Yes. Malaria may lead to or exacerbate anemia and can lead to serious complications in those with sickle cell disease. Immediate testing and adequate treatment for the malaria is essential.

Is there a treatment available for a child with sickle cell disease?

• Yes. Special care is needed for children including vaccinations, infection prevention, regular monitoring, disease modifying treatment and screening for complications like stroke.

What can be done to avoid complications of sickle cell disease?

• Periodic medical monitoring, use of medications as directed, good hydration, immunizations, infection control and prevention, antibiotic treatment for infections (if applicable) and treatment of complications can minimise health risks.