Frequently asked questions
Aplastic anaemia is a rare disease wherein the bone marrow fails to produce enough blood cells. Bone marrow is a spongy tissue inside the bone that contains stem cells that develop into various types of blood cells. Due to certain reasons at times the bone marrow fails to produce enough of blood cells. This condition is termed bone marrow hypoplasia. When the bone marrow stops producing blood cells at all, the condition is termed bone marrow aplasia.
In cases of bone marrow hypoplasia and aplasia, the blood cells formed are normal, but there is a significant reduction in number. RBCs, WBCs, and platelets are all deficient, giving rise to a variety of complications. Decrease in RBCs result in reduced oxygen carrying capacity and resultant anaemia. A decrease in WBCs results in increased susceptibility to infections. Similarly, a decrease in platelets results in bleeding disorders.
The treatment for the disease depends on the severity. In mild cases, the approach is to wait and watch whether the situation improves by itself. Blood transfusions and immuno-suppressants and bone marrow stimulants are recommended for moderate to serious cases. Severe cases may require bone marrow transplantation.
Blood Transfusions: Blood transfusions are not a cure for aplastic anaemia, they merely help in relieving some of the symptoms and in controlling the bleeding. For most aplastic anaemia patients, the blood needs to be irradiated to reduce the chance of getting graft vs host disease.
Bone Marrow Transplant: In bone marrow transplant, stem cells are introduced into the bones after suppressing the diseased bone marrow through chemotherapy and radiation. The success of the transplant is higher if the bone marrow donor is a close relative like siblings. The success rate is higher in young patients. Usually, the aplastic anaemia life expectancy is about five years. The patient will need to take immuno-suppressants for life. Bone marrow transplant is done in various hospitals in India. The cost for the same is only a fraction of that in developed countries. In effect, the aplastic anaemia treatment cost in India is much lower compared to that in many other countries.
Immunosuppressants: For patients with aplastic anaemia, who cannot get a bone marrow transplant, immunosuppressive therapy can be used. Patients are given immunosuppressants which suppress the activity of the immune system, and thus, stop them from attacking the blood cells. This helps the bone marrow to heal and generate more blood cells.
Iron Chelation: This procedure may be required by patients if they’ve had too many red blood cell transfusions. Too many transfusions can result in iron overload in the body and iron chelation is used to treat it.
Dos and don'ts during treatment:
• Avoid contact sports that can result in injury
• Proper body hygiene should be practised
• Avoid places from where it is easy to pick up infections
• Prescribed medicines should be taken as directed
• Vaccinations should be taken to prevent communicable diseases
A small minority of Aplastic anaemia sufferers inherit it by birth. This condition is called hereditary Aplastic Anaemia. The reason is attributed to faulty genes that carry the disease. They also suffer from certain other genetic or developmental abnormalities that contribute to the disease. Some inherited genetic conditions that cause Aplastic anaemia are-
• Fanconi anaemia
• Schwachman-Diamond syndrome
• Dyskeratosis congenital
• Diamond-Blackfan anaemia
A huge majority of the sufferers acquire the disease during their lifetime. Children, young adults, and older adults can all develop the disease. In about eighty percent of the cases, the cause is idiopathic in nature. That means there is no evident cause for the disease.
Symptoms of Aplastic Anaemia:
Some of the common symptoms of Aplastic Anaemia are:
• Shortness of breath
• Exertion while breathing
• Irregular or rapid heartbeat
• Paleness of the skin
• Increased frequency of infections
• Increased duration of infections
• Frequent nose bleeds
• Bleeding gums
• Tendency to bruise easily
• Skin rashes
A diagnosis is usually arrived at by corroborating the findings of medical and family history, physical examination and presenting symptoms with medical lab reports. Some diagnostic tests are complete blood count [CBC] to measure the number of RBCs, WBCs, and platelets, a reticulate count to measure the number of young RBCs etc. Bone marrow biopsy is done to confirm the disease.
Acquired Aplastic Anaemia is considered as an auto immune disease. In auto immune diseases, the immune system of the body targets its cells mistaking it for foreign bodies. In aplastic anaemia, the target of the body is the bone marrow and hematopoietic stem cells. But why the body acts like this remains unclear even today.
However, there is some knowledge regarding certain trigger factors for aplastic anaemia. Some of them are-
• Toxins like arsenic, benzene, and certain pesticides
• Radiation and chemotherapy treatments for tumours and cancers
• Treatment for certain autoimmune diseases like rheumatoid arthritis and SLE
• Pregnancy, but the condition is very much reversible after childbirth
• Viral infections with hepatitis, Epstein Barr, Cytomegalovirus, Parvovirus, and HIV
• Metastasis or spread of cancers to the bone marrow
So, Aplastic Anaemia is not a form of cancer. However, even though it is not a malignant disease, it is a very serious disease.
Aplastic Anaemia can be classified into three types:
• Non-severe aplastic anaemia: In this type, the blood cells count is low, but not too much. There may be no symptoms displayed and doctors may not prescribe a treatment plan yet.
• Severe Aplastic Anaemia: In this type the neutrophil count is very low as is the platelet and reticulocyte count.
• Very Severe Aplastic Anaemia: In this type, the neutrophil count is less than 200/microliter. Apart from this, all other counts are similar to severe aplastic anaemia.
Bone Marrow Transplant is the treatment for aplastic anaemia that is undertaken in many hospitals in India. The pricing for BMT treatment in India is much lesser than most countries around the world.
Quick break-up of costs:
• Initial Consultation: 1500 USD
• Chemotherapy (before BMT): 7500 – 10,000 USD for 3-5 cycles
• Autologous BMT Treatment: 29,000 USD approx.
• Allogenic BMT Treatment with matching donor in family: 35,000 USD approx.
• Allogenic with donor from registry: 35,000 USD + (17000 to 25000) = 52000 to 77000 USD. The cost varies based on registry.
Apart from these, patient will also need to factor in additional costs such as travel fare, hotel stay, food and medications.