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Iron Healh Alliance voices
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speaker 1
dr. Hendricks speaking
speaker 2
Annie's father speaking
speaker 3
Annnie speaking
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Acute Chest Syndrome: Acute chest syndrome is a lung-related complication of sickle cell disease that can lower the levels of oxygen in the blood and can be life-threatening. It is the second most common cause of hospitalization in patients with sickle cell disease.


Acute hemolytic crisis: An acute hemolytic crisis is characterized by an accelerated rate of red blood cell destruction leading to anemia, jaundice and reticulocytosis (an excess of immature red blood cells in the blood).


Acute Myeloid Leukemia: Acute myeloid leukemia (AML) is a cancer of the blood and bone marrow. It is characterized by an increase in the number of myeloid cells in the marrow that do not mature and interfere with the production of healthy blood cells. The marrow eventually stops working correctly, leaving a person with an increased risk of bleeding and infections. When the bone marrow continually fails to produce enough healthy platelets, red blood cells and white blood cells over time myelodysplastic syndromes (MDS) will progress into AML.


Anemia: Anemia is a condition in which there is less than the normal number of red blood cells or less than the normal amount of hemoglobin in the blood.


Beta-thalassemia: Beta-thalassemias are a group of hereditary blood disorders characterized by ineffective production of red blood cells. This results in different types of the disorder which range in severity from patients with severe anemia to others with no symptoms.
-- • Beta-thalassemia major (TM) is characterized by profound anemia, often requiring medical attention in the first two years of life and leading to a dependency on blood transfusions.
-- • Beta-thalassemia intermedia (TI) is characterized by less severe anemia that appears later in life. Patients may not require blood transfusions, or may only require them occasionally but can still suffer serious complications.


Blood transfusion: Transfusion is the process of transferring blood or blood components (e.g., plasma, blood cells) from one person into the circulatory system of another.


Bone marrow: Bone marrow is a flexible tissue that fills bone cavities and produces most of the blood cells for the entire body. Bone marrow transplants may be used in the treatment of patients with thalassemias, such as non-transfusion-dependent thalassemias (NTDT), and in some cases in patients with MDS and sickle cell disease.


Chelation: Chelation is a process in which a chemical compound, or chelating agent, is introduced into the body. This compound tightly binds to metals such as iron, which are subsequently excreted through body waste.11 Chelation is an important treatment to remove excess iron from the body in patients with thalassemia, sickle cell disease and MDS.


Genetic disorders: Genetic disorders are caused by abnormalities in genes or chromosomes. They can be inherited or caused by environmental factors.14 Sickle cell disease and thalassemias like NTDT are inherited genetic disorders.


Genetic screening: Genetic screening is a test to determine if a person has an abnormal gene that may be passed on to children. Genetic screening can also be used to diagnose severe diseases even in the absence of symptoms, including some types of thalassemia, sickle cell disease and sickle cell trait.


Endemic: An endemic disease or condition is one that is commonly found among particular people or in a certain geographical area. Thalassemia is endemic to South and Southeast Asia, the Middle East and the Mediterranean region. Sickle cell disease is endemic to tropical Africa, the Caribbean, the Middle East and the Indian subcontinent.


Ferritin: Ferritin is the major iron storage protein. The blood level of ferritin serves as an indicator of the amount of iron stored in the body.


Hematopoiesis: Hematopoiesis is the formation of blood or blood cells in the body.


Hemoglobin: Hemoglobin is a protein in red blood cells that contains iron and carries oxygen throughout the blood.


Hemoglobin E-beta-thalassemia: Hemoglobin E-beta-thalassemia occurs predominantly in people of South and Southeast Asian descent.24 The severity can vary significantly, with some patients experiencing no symptoms and others requiring regular transfusions.


Hemoglobin H disease (HbH-alpha-thalassemia): Hemoglobin H disease (HbH-alpha-thalassemia) occurs predominantly in people of Southeast Asian, Middle Eastern and Mediterranean descent.


Hereditary disease: A hereditary disease is passed on from parent to child. Thalassemia and sickle cell disease are hereditary diseases.


Iron: Iron is an essential element in the human body mostly found in red blood cells. Iron, as part of the protein hemoglobin, helps cells “breathe” by carrying oxygen to cells and tissues, and is essential to giving the body energy and having a properly functioning immune system.27 A lack of iron is a common cause of anemia. Patients with sickle cell disease, MDS, and thalassemias, such as NTDT, can accumulate too much iron in their body and develop iron overload.


Iron chelator: An iron chelator is any agent that binds to excess iron in the body. The excess iron is then excreted through body waste.


Iron metabolism: Iron metabolism is the regulation of normal iron levels within the body.


Iron overload: Iron overload is an accumulation of excess iron in the body. Under normal conditions, very little iron enters and leaves the body. Chronic iron overload is a complication of conditions that require multiple blood transfusions, like sickle cell disease, myelodysplastic syndrome and thalassemia, and is also a complication of non-transfusion-dependent thalassemia.


Iron toxicity: Iron toxicity occurs when chronic iron overload begins to cause tissue damage.


Myelodysplastic Syndromes: Myelodysplastic syndromes (MDS), also called preleukemia, refer to a category of diseases that affect the bone marrow and blood. In MDS, the blood-forming cells in the marrow slow down, or even stop making the three types of blood cells. Most patients with MDS will develop anemia and may need blood transfusions as a result.


Non-transfusion-dependent thalassemia(NTDT): Non-transfusion-dependent thalassemia (NTDT) syndromes encompass several types of thalassemia, a diverse family of genetic disorders affecting red blood cell production, causing anemia.
Unlike patients with other types of thalassemia, those with NTDT syndromes can live without regular blood transfusions. However, even without transfusions, NTDT patients still accumulate excess iron from increased absorption through the stomach and intestines leading to debilitating health complications.


Non-transferrin-bound iron (NTBI): Non-transferrin-bound iron (NTBI) is the toxic, free form of iron which easily penetrates tissue barriers and forms iron deposits that lead to organ damage and dysfunction.


Preleukemia: Preleukemia is another word for myelodysplastic syndromes (see MDS definition).


Red blood cells: Red blood cells are the most common type of blood cells and the body's principal means of delivering oxygen to the body tissues in the circulatory system. Production of red blood cells is compromised in sickle cell disease, MDS and thalassemia patients.


Serum: Serum is the clear liquid that can be separated from clotted blood. Serum differs from plasma, which is the liquid portion of blood containing red and white blood cells and platelets.


Sickle cell disease: Sickle cell disease is a hereditary blood disorder characterized by sickle-shaped red blood cells. It is a chronic, life-long, debilitating disease with many forms that can range in clinical severity from asymptomatic to life-threatening.


Splenectomy: A splenectomy is a surgical procedure that partially or completely removes the spleen, an organ that acts as a blood filter. In thalassemia and sickle cell disease patients, the size of the spleen can increase over time, resulting in worsening anemia and the need for more transfusions. Removing the spleen can reverse these effects, but can lead to serious complications including blood clots, pulmonary hypertension and silent stroke.


Stroke: A stroke occurs when the blood supply to part of the brain is interrupted or severely reduced, depriving brain tissue of oxygen and nutrients. In sickle cell disease this is caused by the occlusion of small vessels from deformed sickle-shaped cells.


Thalassemia: Thalassemia refers to a diverse family of genetic disorders characterized by an underproduction of hemoglobin. NTDT is one type of thalassemia.

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SCD Voices is based on the original idea of Agence Catalpa, and conducted on behalf of the Iron Health Alliance.

Coordination, interviews, writing, scenario: Géraldine Genin, Audrey Andrianarivo
Photographs and scenario: Thomas Troadec
Graphic design: Axel Perignon
Sound editing: Christophe Lalanne-Claux
Web engineering: Florian Gracy
Production year: 2014

The content of this website is protected by the terms of the Intellectual Property Code. Any reproduction, total or partial, or any imitation, of its form or its content, without prior written consent given by its authors and creators, is strictly forbidden.

Disclaimer: This is an international website for Iron Health Alliance, and is intended for healthcare professionals outside the US. The information on this site is not country-specific and may contain information that is outside the approved indications in the country in which you are located.

Copyright © 2014 Novartis AG. All Rights Reserved

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The Project

Sickle cell disease (SCD) is a group of inherited genetic disorders characterized by defective hemoglobin synthesis. By causing, from early childhood onwards, chronic anemia, acute painful episodes known as vasoocclusive crises, infection risks and organ damage, this disease impacts significantly on daily life, and presents important challenges for the people who are affected, their families, and the medical community.

Although improvements in patient care have had a significant influence on prognosis, with a substantial increase in life expectancy, long-term health, quality of life and lifestyle choices still depend on access to care, constant caution and close monitoring. Global awareness and action at all levels are still necessary to achieve better understanding, better access to treatment and better management.

From this challenging context emerged the idea of SCD Voices: an international human adventure initiated in 2013, a journey designed to collect and share individuals’ stories about SCD. The web documentary here features testimonies of patients and physicians encountered in this journey. It shows the diversity of SCD, and the ways people cope with this disease in daily life, in different parts of the world. This project is designed to raise awareness through a series of portraits showing how SCD impacts on people’s daily life.


Human ties and dialogue at work

SCD Voices is also about the people who have united their efforts and collaborated across geographical boundaries throughout this global journey:
--• Project managers interacted with local SCD experts, who showed strong dedication: first in drawing willing patients in the project, then in taking part in interviews, talking about their experience, and finally in providing scientific highlights and ways to talk about the disease, to contribute to better understanding.
--• Local advisors and coordinators worked together to enable experts and patients in each country to understand the nature and importance of this project, arranged the preliminary encounters, and provided onsite reporting.
--• The moments of privileged exchange between patients, the interviewer and the photographer gave birth to the rich and sensitive material that you can now discover on this website.

Throughout its development, the SCD Voices project has been a sharing experience, conceived to give sensitive, human perspectives on the disease, in order to allow better understanding, and generate more actions.


Partners: Novartis and the Iron Health Alliance

Novartis Oncology established the Iron Health Alliance in 2011 as a way to help improve the standard of care for people diagnosed with chronic iron overload. The Iron Health Alliance is a partnership that raises the standard of care of chronic iron overload by supporting education, diagnostic access, and patient management for optimized outcomes.

The Iron Health Alliance provides support through three core pillars of care to improve outcomes:
--• Increased awareness and knowledge of diagnostic methods to detect and measure iron overload
--• Increased awareness of anemias
--• Increased awareness of the importance of treatment adherence

It is as part of this dynamic that the Iron Health Alliance has given impulse to SCD Voices, allowing Novartis to sponsor this web documentary, which is an important step towards global SCD awareness.


Acknowledgements

This adventure would not have been possible without:
--• the four patients, whose names will not be disclosed in respect for their privacy, who agreed to share their experience as part of this project of dialogue and international communication
--• the local SCD experts, whose human and scientific involvement was remarkable:
-----Pr Rodolfo Cançado, Sao Paulo, Brazil
-----Dr Marc Hendricks, Cape Town, South Africa
-----Pr Marianne de Montalembert, Paris, France
--• those who allowed the encounters to take place in the best conditions, particularly the local Novartis teams, whose support made this project possible.