As COVID-19 continues to spread across the globe, more and more people are wondering how this virus might affect them. This is especially true for patients with rare diseases, who are uncertain how their conditions may interact with coronavirus. A recent MedScape article addresses this for patients with thalassemia and sickle cell disease. The article unpacks how a webinar with 3 medical experts described hematological care for patients during this time.
Check out the full webinar entitled “SCD/Thalassemia and COVID-19: Possible Risks and a Proposal for a Patient Pathway during a Pandemic.”
The Webinar: Conditions Discussed
Thalassemia
Thalassemia is a genetic, inherited blood disorder. Patients with thalassemia do not make enough hemoglobin in their blood, which plays a role in red blood cells and helps to carry oxygen throughout the body. So, when someone has issues with hemoglobin, their body is not getting enough oxygen.
Thalassemia can be considered alpha or beta depending on which part of the hemoglobin is defective. With beta thalassemia, it caused by a mutation in the HBB gene. Patients can have minor, intermediate, or major cases; those with minor beta thalassemia often have few symptoms. These symptoms include anemia, fatigue, blood clots, shortness of breath, and dizziness. Additionally, patients may experience excess levels of iron. Learn more about beta thalassemia here.
Sickle Cell Disease
Sickle cell disease is a group of hematological disorders that impact the amount of hemoglobin in the blood. The most common sickle cell disease is sickle cell anemia. With this condition, hemoglobin S forms abnormal strands that cause red blood shells to become crescent-like and misshapen. Rather than travel freely throughout the body, these cells get stuck along vessel walls, blocking the flow of oxygen.
Patients with sickle cell anemia experience symptoms such as hand and foot inflammation, fatigue, jaundice, throbbing pain, vision problems, anemia, pulmonary hypertension, and organ damage. Learn more about sickle cell anemia here.
Thalassemia, SCD, and COVID-19
The webinar was hosted by the Thalassemia International Federation (TIF) and the European Hematology Association (EHA). The speakers included:
Maria Domenica Cappellini, MD, PhD
Dr. Cappellini is a hematology professor who spoke about Italian COVID-19 data. After analyzing data from 11 patients with thalassemia, she noted that patients with comorbidities were more likely to experience severe COVID-19 symptoms. Patients with thalassemia were also believed to be at risk based on prior splenectomies or blood transfusions. Both of these could possibly lead to infections or sepsis.
The 11 patients with thalassemia who also had COVID-19 experienced mild to moderate symptoms. None, despite prior splenectomies, experienced a severe immune reaction. However, about 50% of the patients were hospitalized. But because none of their reactions were severe, none were placed in intensive care. The only patient who needed additional ventilation had previously been treated for diffuse large B-cell lymphoma.
Overall, however, Dr. Cappellini noted that she saw less patients with thalassemia develop COVID-19 than she thought. She believes this is due to earlier isolation and improved patient-doctor communication.
Androulla Eleftheriou, MD
Dr. Eleftheriou is the Executive Director of Cyprus’ Thalassemia International Foundation. She spoke about 51 patients with thalassemia who had later been diagnosed with COVID-19. The patients she analyzed were from different countries including (but not limited to) Cyprus, Italy, Indonesia, France, and the UK. She found the following results:
- 46 patients (90.1%) had mild or moderate COVID-19 symptoms.
- 5 patients (9.9%) had severe respiratory symptoms and required hospitalized. Of the 5, 2 patients were discharged. The remaining 3 died.
Additionally, Dr. Eleftheriou sought the same type of data from 32 French patients with sickle cell disease. She found:
- All patients were treated for COVID-19. However, 17 patients (53.1%) required 10 days of COVID-19 treatment.
- 22 patients (68.75%) were hospitalized. 11 patients, which make up 50% of those hospitalized and 34.37% of all patients studied, were admitted into intensive care.
- 1 patient (3.1%) with sickle cell disease died from COVID-19 complications.
Blood Transfusions and Medical Availability
During COVID-19, Dr. Eleftheriou noted that medical supplies are currently lacking. This includes blood needed for transfusions. She explained that:
- Out of the Thalassemia International Foundation’s 62 member countries, about 46 of them (75%) experienced blood shortages.
- 58% experienced or reported severe blood shortages.
- 35% reported moderate to severe blood shortages.
Blood shortages require innovation to perform transfusions. Potential options include seeking blood donations from friends or relatives, whole blood transfusions, and using blood that is older than one month. Additionally, doctors are now giving patients with thalassemia one unit of blood versus two, with transfusions occurring more frequently.
In addition to the two doctors above, Raffaella Colombatti, MD, the coordinator of the Red Cell Reserve Working Group, also participated in the webinar. Once again, if you would like to watch the webinar, you can find the full recording here.
