Sickle cell disease (SCD) is an inherited blood disorder in which oxygen carrying capacity of blood reduces significantly. Sickle cell anaemia is one of the SCD disorders, defined by the alteration in the shape of red blood cells (RBCs) from a disc shape to a sickle-like, crescent shape.
An estimation suggests that blood related disorders are the most common genetic diseases in Turkey with the 10.0% incidence of sickle cell trait.
The average cost of sickle cell anaemia treatment in Turkey typically ranges from USD 50,000 to USD 75,000. This includes the cost of BMT, medications, blood transfusions and other medical consumables.
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Haemoglobin (Hb), the most vital protein of RBCs, is composed of two alpha and two beta chains.
Hb binds to oxygen and carbon dioxide and then carries them to different parts of the body. In normal conditions, RBCs are flexible disc-shaped and move easily through the blood vessels.
Some mutation (sudden genetic change) in the beta chain of haemoglobin (HBB), the shape of RBCs becomes abnormal and clumps together, causing the RBCs to turn sickle shaped.
The major complications with sickle-shaped RBCs are:
Sickle-cell anaemia is an inherited disorder, which means that this genetic defect is inherited from the parents to their children. Based on the allele, it can appear in two forms:
A child born from two HBB-carrier parents has a higher chance of sickle-cell anaemia.
The diseased person suffers from hypoxia and a range of complications, such as stroke and sometimes even organ damage. Now, let’s understand the symptoms of SCD, which are listed below.
Dactylitis
This is one of the earliest symptoms seen in infants suffering from sickle cell anaemia where the accumulated sickled RBCs leads to inflammation in hands and feet.
As compared to normal RBC with a lifespan of 120 days, Sickled RBCs have a shorter lifespan of only 10-20 days. Moreover, sickled cells are more prone to destruction as well. This causes chronic anaemia and symptoms such as dizziness, shortness of breath, fatigue etc.
Due to sickle-shaped RBCs, the blood flow is blocked in a particular area, often causing intense pain. Pain accompanied by swelling is most commonly observed in the chest, arms, and legs of the patient.
A life-threatening complication of sickle cell disease, acute chest syndrome occurs when sickle cells block blood vessels in the lungs. This condition is often triggered by an infection that leads to fever and severe pain.
Sickled cells, when accumulated in the spleen, cause a sudden drop in haemoglobin levels, leading to a life-threatening situation
Sickle cells block major blood vessels of the brain, leading to severe brain damage. Individuals who have had one stroke are at increased risk of subsequent strokes due to sickle cell anaemia.
Sickle cells may cause a painful obstruction of blood vessels in the penis. If not treated effectively, it can lead to impotence.
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Sickle cell disease is an autosomal recessive disease. It occurs when the patient inherits two abnormal genes, one from each parent.
After genetic testing, if doctors confirm sickle cell, haematologists analyse the patient’s health condition and derive a treatment plan which includes:
In SCD, patients suffer from vaso-occlusive crisis (VOC) and neuropathic issues. To address acute and chronic pain, doctors recommend non-steroidal anti-inflammatory drugs (NSAIDs), hydroxyurea and other over-the-counter drugs to manage moderate-to-severe pain.
This treatment is done to prevent chances of subsequent stroke in children who have had a first stroke and thus reduces the complications of vaso-occlusion.
Also known as stem cell transplant, BMT is a procedure in which healthy blood-forming stem cells are replaced with defective bone marrow cells in the patient’s body. This defective bone marrow produces defective sickle-celled RBCs for which BMT is performed. BMTs are of two types:
Allogeneic Hematopoietic Stem Cell Transplantation (HSCT): It involves replacing a patient’s damaged bone marrow with healthy stem cells from a sibling or unrelated donor.
This aims to restore normal blood cell production, including haemoglobin.
While this procedure can be life-saving, it also carries risks, such as graft-versus-host disease, graft rejection, infections, and other complications.
Allogeneic HSCT is typically considered for patients with severe sickle cell disease who have a compatible donor and are at high risk if not treated on time.
Autologous Hematopoietic Stem Cell Transplantation (HSCT): It utilises the patient’s own stem cells.
These are collected prior to chemotherapy or other preparatory treatments. This procedure is considered for patients who lack a suitable donor for allogeneic HSCT.
During sickle cell anaemia, there is an increased risk of infections due to their compromised immune system. Doctors recommended a complete immunisation plan to help patients fight infection.
The ultimate goal of any treatment strategy is to alleviate symptoms associated with sickle cell disease, prevent complications, and improve the patient’s quality of life.
Sickle cell anaemia is not a single, one-time intervention and requires a series of ongoing treatment procedures to improve the patient’s quality of life. The cost of treatment for sickle cell anaemia in Turkey depends on the specific treatment approach which could be medications, blood transfusions, bone marrow transplantation or combination of any of these.. Some other factors are listed below.
The type of transplant recommended by BMT transplant specialists is influenced by factors like the patient’s disease condition, the availability of a compatible donor, their age and general health, and the potential advantages and drawbacks of each treatment option.
For the treatment of sickle cell anaemia, allogeneic BMT is done.
| Type of BMT | Cost in USD |
|---|---|
| Allogeneic BMT | 65,000-75,000 |
The choice of hospital facility claimed by the patient, including the hospitals’ location, infrastructure, their accreditation and their quality of care, can incur additional costs to the treatment.
The medications such as pain relievers, hydroxyurea, and other therapies used to treat sickle cell anaemia, may impact the overall cost of treatment.
The cost of diagnostic tests and imaging studies used to track the progression of the disease and identify complications can increase the overall treatment expense.
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TEM Avrupa Otoyolu Göztepe Çıkışı No: 1, Bağcılar 34214| Cost of Allogeneic BMT in Different Countries | Average cost in USD |
|---|---|
| Turkey | 62,500 |
| India | 32500 |
Sickle cell anaemia develops as a result of genetic defect, so before starting the treatment, it’s necessary to determine the severity of the disease. The severity of the disease is revealed by performing genetic and haematological evaluation of the patient.
Some tests and follow-ups required that may add to expenses which can be classified as:
After the treatment, managing sickle cell anaemia becomes crucial. The management focuses on minimise associated complications, which includes-
Routine blood analysis is conducted to monitor levels of haemoglobin, white blood cells, and platelets. Any deviations or indications of issues like anaemia or blood infection may require immediate medical attention.
Since the condition makes the patient anaemic, sufficient nutritional intake and proper vitamin supplements help to improve RBC production.
In order to minimise post-treatment risks and complications, doctors provide some suggestions such as:
Quit smoking and drinking
Maintain a healthy diet
Maintain body weight
Take a good amount of sleep
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