Sickle Cell Anemia is rampant in Africa. At present, there are two curative Treatments for Sickle Cell Anemia: the Allogeneic bone marrow transplant and the latest Gene Therapy. Not Every Sickle Cell Anemia patient can opt for the curative treatment of Sickle cell anemia. The exorbitant cost of gene therapy and the nonavailability of suitable bone marrow donors are hindrances to getting curative sickle cell anemia treatment. The newer drug research in managing and reducing symptoms of Sickle Cell Anemia has paved the way for many of the latest options in Sickle Cell Anemia management. These new drugs are aimed at significant reduction in symptoms of SCD and offering better quality of life for Sickle Cell Patients.

Table of Contents - Newer Options in Sickle Cell Anemia Management

mon and painful complication of sickle cell disease. In Sickle Cell Patients, the red blood cells which are flexible and disc shaped are mutated to crescent moon or sickle shaped. These Mutated red blood cells also becomes rigid and brittle. The normal flexible disc shaped red blood cells can pass through the tiniest of the blood vessels and capillaries but the mutated red blood cells struck with each other and forms a clump that inhibits the blood flow. The disrupted blood supply in Sickle Cell Patients causes tissue ischemia and pain. The Vaso-occlusive crisis in sickle cell patients cause pain, often in the back, chest, arms, and legs. Sickle Cell Children may experience severe pain and swelling of the hands and feet (dactylitis). The Severity of pain may differ from patient to patient. While some SCD patients may experience moderate pain others may complain of severe excruciating pain. The frequency of VOC also differ among the SCD patients. In some patients the VOC may occur severfal times in a year, while in others it may be less frequent. The long term effects of VOC may cause multiple organ damage and can be life threatening. 

How common is Vaso Occulsive Crisis in SCD patients?

Vaso Occlusive crisis or pain crisis is a common problem with majority of Sickle Cell Patients. VOC are major reasons for hospitalization of Sickle Cell patient and also the cause for increased mortality and organ damage. Many patients may expreince multiple episodes of VOC in a year whereas others may have lower frequency. SCD patients are porn to frequent infections including chest infections like pneumococcal infections, can contribute to the pathogenesis of VOCs due to mechanisms like pneumonitis, pyrexia, and hypercoagulability. 

What are the Options in Sickle Cell Anemia Management?

Sickle cell Disease can be cured with two of the available treatments, the Gene-Therapy recently approved by US FDA and the allogeneic bone marrow transplant. While Gene-Therapy for cure of Sickle Cell Disease is still costing enormous amount of money and staying in the treatment country for almost a year, it is not in reach of majority of the population.

The Allogeneic BMT is also a costly treatment but is affordable to many Sickle Cell Patients. The Major hindrance in Allogeneic BMT for cure of Sickle Cell Disease is unavailibility of a suitable bone marrow donor. 

When the SCD patients can not get a curative treatment, they are advised for for medical management of associated symptoms and avoiding harmful effect of Sickle Cell Disease.

The Conventional Medical Management of Sickle Cell Disease

The aim of Medical Management for Sickle Cell Disease is to alleviate the symptoms and reduce the risk of complications. The conventional management of Sickle Cell Disease is based on following steps:

Hydroxyurea in managing Sickle Cell Anemia

Use of Hydroxyurea is stated to increase the size of red blood cells and keep them flexible and disc shaped thereby lowering the chances of RBCs becoming sickle shaped, brittle and sticky. It is also observed that Hydroxyurea also reduces the episodes of Vaso-occlusive crises (pain crises) by almost half in SCD patients. It has been stated that the usage of Hydroxyurea in Sickle Cell Patients provides following benefits:

• Reduces the episodes of pain crises
• Reduces the incidents of Acute Chest Syndrome, a life-threatening complications of SCD
• Reduces the need of frequent blood tansfusions
• Possible reduction in hospitalization for sickle cell anemia patients

Folic Acid supplement is useful in Management of Sickle Cell Anemia

In Sickle Cell Disease the life span of affected red blood cells is reduced and the bone marrow is required to step up the production of red blood cells. Folic Acid assists the bone marrow in producing more red blood cells to keep up with the increased need of red blood cells in sickle cell patient. In Sickle Cell Anemia the attending clinician will recommend a higher dose of Folic Acis ranging from 1-5 mg daily. Folic Acid supplementation in Sickle Cell Anemia patients is essetial to maintain adequate red blood cell production, prevent anemia, and reduce the risk of certain complications. It's important to follow the clinician's recommendations on dosage and to monitor for potential side effects and other health concerns. 

Prophylactic Antibiotic

Sickle Cell Anemia Children are prone to frequent infections. Many Sickle Cell Children succumb to pneumonia. Pencillin derivatives are prescribed on daily basis to sickle Cell Anemia Patients as a prohylaxis to prevent life-thretening infections. Moreover Pencillin derivatives are found to be particularly effective in children with HbSS (the most common form of the SCD). Other Antibiotics like erythromycin may also be used in some cases, especially if there's a penicillin allergy or if the child has a different type of sickle cell disease with similar severity.  

Increased Fluid Intake helps in management of Sickle Cell Anemia

Adequate hydration is useful for preventing pain crises and associated symtopms in Sickle Cell Anemia patients. Lack of fluid intake can make blood viscous and thicker that can lead to sickle cells clumping together and blocking small blood vessels, causing pain crises. Drinking plenty of water dilutes the blood, making it easier for sickle cells to flow through blood vessels and helps to maintain healthy blood flow and prevents the formation of clots that can cause pain and protects against organ damage. Increased fliud intake is crucial for management of Sickle Cell Anemia. Sickle Cell Patients should drink more water and fresh fruit juices and avoid caffeinated drinks like tea and coffee and alcohol, as they may cause dehydration. The attending clinician may also advise parentral fluid administartion. Patients of Sickle Cell Disease should also avoid excessive usages of diuretics.

L-Glutamine Oral Powder

L-Glutamine Powder taken orally is also useful in managing Sickle Cell Anemia. Daily consumption of L-Glutamine Oral powder in supervision of a qualified doctor helps in making the red blood cells flexible, round, less sticky and brittle and reduces the risk of pain crises and helps in smooth flow of blood in Sickle Cell Anemia Patients.

Splenectomy in Sickle Cell Anemia Patients 

Splenectomy is removal of spleen in some Sickle Cell Anemia Patients. The Sickled red blood cells may clump together in spleen and may reduce the blood flow out of spleen thereby RBC accumulstion in spleen and causng a painful enlarged sleen, sudden fall in hemoglobin and hematocrit levels. This medical condition is known as Splenic Sequestration. In Sickle Cell Anemia Chidren with repeated episodes of Splenic Sequestration, the treating doctors may recoomend Splenectomy for management of Sickle Cell Anemia.

Transcranial Doppler USG for assessment of risk of stroke in Sickle Cell Anemia Children

A brain stroke is a serious complication in children suffering with Sickle Cell Anemia. A Transcranial Doppler USG assists in predicting the risk of a Stroke in Sickle Cell Anemia Chidren. Subsequent Blood transfusion and medical management can prevent a stroke in High risk Sickle Cell Anemia Children.

Latest Options in Sickle Cell Anemia Management

Vaso-Occlusive Crises or Pain crises affects most of the Sickle Cell Patients. Newer drugs like monoclonal antibody Crizanlizumab (Adakveo) in high doses and under the spervision of a qualified hematologist is the latest option in management of Vaso-Occlusive crises in Sickle Cell Anemia Patients. Crizanlizumab significantly reduces the Vaso-Occlusive (Pain) crises in Sickle Cell Anemia Patients. Crizanlizumab is the latest option in management of Sickle Cell Anemia for Children above 16 years and adults in reducing the episodes of Vaso-Occlusive crises.

How Crizanlizumab reduces the Episodes of Vaso-Occlusive Crises in Sickle Cell Anemia?

Crizanlizumab is not a cure for Sickle Cell Anemia; rather, it reduces the frequency of Vaso-Occlusive Crises (Pain Crises) in Sickle Cell Anemia patients. Crizanlizumab neither prevents red blood cell sickling nor increases the hemoglobin levels. It acts by targeting the adhesion process that contributes to vaso-occlusion. 

Who will benefit from Crizanlizuman therapy in reducing Vaso-Ocllusive crises in SCD patient?

Crizanlizumab is indicated in children above 16 years of age and adults esxperiencing recurrent Vaso-Occlusive crises. It reduces significantly the frequency of pain crises in sickle cell anemia patients.

Curative Treatments for Sickle Cell Anemia

At present there are two curative treatment options for Sickle Cell Anemia patients. The recently approved Gene-Therapy and the time tested allogeneic Bone Marrow Transplant. Both the therapies have their own adavntages and disadvanteges. Hematologists and Sickle Cell Disease treatment Specialists will discuss at length with the patient's family and counsel them about which curative sickle cell treatment is better for patient.

CMCS Health, the Best Medical Tourism Provider Agency for International Patients, assists the foreign patients in proper counseling of the Sickle Cell Patients's family with the leading Sickle Cell Treatment specialists for choosing the best Curative Treatment options for the Sickle Cell Anemia Patients.