Glanzmann Thrombasthenia: Treatment, Cost and Side Effects

Glanzmann disease or Glanzmann’s thrombasthenia is an extremely rare condition in which the blood in the body is not able to clot properly. This bleeding disorder is present since birth and is known as a congenital hemorrhagic disorder. Glanzmann Thrombasthenia takes place when the blood does not have enough amount of glycoprotein which is a protein found in the blood platelets. Without the presence of enough glycoprotein in the blood, the platelets are unable to clot together properly. Large quantities of blood can be lost in case of emergencies like a serious injury or in case of surgeries making this condition very serious.

The cause of the problem is still not known. However, doctors are trying to figure out the primary cause in order to find better treatment methods for the problem. It is believed that the defect in the genes which are carried on chromosome 17 to the DNA can cause this condition of Glanzmann Thrombasthenia. This autosomal recessive condition can be inherited by you if your family has a history of Glanzmann disease or any other related disorder. This long-term disorder does not have any definite cure and patients suffering from the problem need to come in terms with living with the disorder.

Glanzmann’s disease needs to be diagnosed with the help of a simple test like platelet aggregation test which is performed to find out how well the platelets clot in your body. A complete blood count is important to determine the number of blood platelets in the body, a prothrombin time and a partial thromboplastin time is needed to find out how long it takes for your blood to clot. Some tests of your close relatives may also be needed to identify if they have the disease which has contributed to your disorder.

No specific treatment method is decided upon when it comes to Glanzmann disease, however, doctors usually recommend a blood transfusion, or using an injection of the patient’s blood in cases where there are serious bleeding episodes. Replacing the damaged platelets with normal platelets has also helped patients with Glanzmann’s disease have less bruising and bleeding. Patients should avoid taking any medication like ibuprofen, warfarin, blood thinners, aspirin and other anti-inflammatory drugs as these medications stop the platelets from clotting any further and this can cause more bleeding as well.

Individuals who show symptoms like frequent nose bleeding, heavy menstrual bleeding, excessive bleeding during or after surgery and easy bruising and bleeding gums should get themselves tested as they may have the disorder.

Individuals who do not show symptoms of the disease should not get themselves treated. Patients who are taking medications like ibuprofen and other blood thinners should also avoid taking them in order to regulate their condition.

Certain medications required for patients suffering from this disorder include hormonal contraceptives which have side effects like headaches, migraine, breast tenderness, nausea, weight gain, mood changes among other issues. Other Antifibrinolytic drugs have side effects like allergic skin reaction, nausea, vomiting and diarrhea.

Patients who have glanzmann’s disease cannot be cured as there is no treatment available for the disorder. However, patients only need help during times when they are bruised, injured, bleeding or are undergoing a surgery. Avoiding the use of anti-inflammatory drugs are very important for patients suffering from Glanzmann’s disease.

Patients cannot completely recover from this disorder as it is a rare genetic disorder. However, taking care of yourself and avoiding getting bruised and injured is very important to prevent the matter from getting serious.

A single transfusion episode can cost you around Rs 10,000 or above, depending on the facility you choose. The platelet concentrate per unit costs around Rs 500-600.

The results of the treatment are permanent as the treatment is only required in cases of bruising and bleeding. However, the condition on its own cannot be completely cured. Patients need to have platelet transfusion regularly all throughout their lives.

There are other treatment considerations which can also be followed. Getting vaccinated against Hepatitis B to keep safe from infectious risks which are associated with multiple transfusions, considering oral contraceptives and not using medications which intrude in the functioning of the platelets are all very important.