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Navigating High Risk Pregnancy With Essential Thrombocythemia

Essential Thrombocythemia (ET) is a rare blood disease caused by an excess of platelets produced by abnormal stem cells in the bone marrow

The experience of becoming a mother and having a child is a remarkable journey filled with excitement and joy. This journey can be filled with additional complications for women who have been diagnosed with the uncommon condition Essential Thrombocythemia (ET). 

Essential Thrombocythemia (ET) is a rare blood disease caused by an excess of platelets produced by abnormal stem cells in the bone marrow. Platelets are blood cells that form blood clots to slow or stop bleeding. It is a form of Myeloproliferative Neoplasm Essential Thrombocythemia. It develops due to mutations in certain genes, making it an acquired genetic disorder. It is more prevalent in women. Although 20% of patients are women of childbearing age, the average age of ET patients at presentation is around 60yrs old. 

Recently, the team at Fortis Hospital Mulund helped a 31-year-old woman, Sarika (name changed) have a successful pregnancy, after her treatment of Essential Thrombocytosis had stabilised her disease. Despite having lost two pregnancies in the past, Sarika was prepared to try again when her health was better. 

Women with Essential Thrombocythemia (ET) should visit their Obstetrician and Haematologist when planning their pregnancy. This comprehensive approach guarantees optimal management of the condition and the pregnancy. 

Sarika's thorough assessment revealed a protein S deficiency, posing a heightened risk of blood clots and early miscarriages. Therefore, it was crucial to monitor and manage her condition constantly. When Sarika got pregnant, in addition to the advised course of treatment for Essential Thrombocytopenia, she was administered injections to ensure the foetus had access to blood. Along with that, Sarika was also given medication to prevent heart attacks, strokes, and angina & to maintain Folic Ccid to support her overall health and fertility.

Pregnancy with Essential Thrombocythemia comes with maternal complications; foetal risks are considerable, with a potential 43 per cent chance of first-trimester miscarriage. Later complications may involve growth restriction, stillbirth, placental abruption, and pre-eclampsia. Interferon treatment, though essential, may induce side effects like high-grade fever.

During Sarika's pregnancy, her platelet levels were closely watched and controlled with interferon therapy. The blood clot controlling injections were also important in supporting the pregnancy. At 20 weeks, Sarika developed Oligohydramnios, a condition where there's low amniotic fluid, which needed close monitoring and treatment. Later on, she developed Intrahepatic Cholestasis of pregnancy, a liver condition that was carefully managed to ensure a full-term delivery.

Throughout Sarika's pregnancy, she received close supervision, with regular monitoring and prompt interventions to address any complications. After birth, her baby underwent screening for platelet issues and was confirmed to be healthy. Sarika's journey underscores the significance of careful medical attention and monitoring in managing a high-risk pregnancy, with essential Thrombocythemia and Protein S Deficiency. Her successful pregnancy showcases the effectiveness of a coordinated treatment plan and attentive prenatal care. --

Sarika's journey embodies hope, resilience, and the impact of specialised medical care. It highlights that with an appropriate treatment plan and careful monitoring, navigating essential thrombocythemia during pregnancy is possible. It also encourages others facing comparable challenges by demonstrating that the goal of having a healthy baby can be accomplished with determination and guidance from professionals.

The author is Consultant Gynecology & Obstetrics, Fortis Hospital Mulund 

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