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Pour des professionnels de santé en dehors des Etats-Unis
Iron Healh Alliance voices
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speaker 1
Dr. de Montalembert
speaker 2
Agathe's father
speaker 3
Agathe's mother
speaker 4
Agathe
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background glossary




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 est basé sur une idée originale de l’Agence Catalpa et mené au nom de l’Iron Health Alliance.

Coordination, entretiens, écriture, scenario : Géraldine Genin, Audrey Andrianarivo
Photographie et scenario : Thomas Troadec
Design Graphic : Axel Pérignon
Montage son : Christophe Lalanne-Claux
Développement Web : Florian Gracy
Année de production : 2014

Le contenu de ce site est protégé par la loi du 11 mars 1957 et les lois internationales sur le droit d’auteur et la protection de la propriété intellectuelle. Il est strictement interdit de le reproduire, dans sa forme ou son contenu, totalement ou partiellement, sans accord écrit et préalable des auteurs et créateurs.

Avertissement : ce site Web international consacré à l’Iron Health Alliance, est destiné aux professionnels de la santé situés hors des États-Unis. Les renseignements donnés sur ce site ne sont pas propres à un pays et peuvent mentionner des indications non approuvées dans votre pays de résidence.

Copyright © 2014 Novartis AG. Tous droits réservés.

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Le projet

La drépanocytose recouvre un groupe de troubles héréditaires caractérisés par un défaut de synthèse de l’hémoglobine (Hb). En provoquant dès la petite enfance, une anémie chronique, des épisodes aigus et douloureux identifiés comme des crises vaso-occlusives, des risques d'infection et des dommages sur les organes, cette maladie a un impact considérables sur la vie quotidienne des patients et présente des défis importants pour les gens qui sont touchés, leurs familles, et la communauté médicale.

Bien que les améliorations dans les soins apportés aux patients aient eu une influence significative sur les pronostics, avec une augmentation substantielle de l'espérance de vie, et de la santé à long terme ; la qualité et les choix de mode de vie dépendent encore de l'accès aux soins, d’une constante prudence et d’une surveillance étroite. Une sensibilisation et des actions globales à différentes échelles sont aujourd’hui encore nécessaires pour parvenir à une meilleure compréhension, un meilleur accès au traitement et une meilleure gestion de la maladie.

De ce contexte difficile a émergé l'idée de SCD Voices, une aventure humaine internationale initiée en 2013, un voyage conçu pour recueillir et partager des histoires individuelles sur la drépanocytose. Le web documentaire propose ici des témoignages de patients et de médecins rencontrés dans le cadre de ce voyage. Il montre la diversité des parcours, et comment des personnes touchées à travers le monde font face à cette maladie dans leur vie quotidienne.


Liens humains et dialogue à l’oeuvre

Ce sont les équipes qui ont collaboré à travers le monde pour offrir ce voyage interculturel qui ont permi que SCD Voices soit rendu possible:
--• Les responsables de projet ont pris contact avec des experts médicaux, qui ont fait preuve d’une véritable implication: d'abord en invitant des patients à prendre part au projet, puis en participant aux entretiens, en parlant de leur expérience, en fournissant des apports scientifiques et en posant des mots sur la maladie, dans une logique de meilleure compréhension globale.
--• Les conseillers locaux et les coordinateurs ont travaillé ensemble pour permettre à des experts et des patients dans chaque pays de comprendre la nature et l'importance de ce projet, organisant les rencontres préliminaires et apportant leur appui lors des reportages.
--• Les interactions entre les patients, l'intervieweur et le photographe ont permi qu’une matière riche et sensible soit présentée sur ce site.

Tout au long de son développement, le projet SCD Voices a été une expérience de collaborations, conçue pour offrir des perspectives sensibles et humaines sur la maladie, afin de permettre une meilleure compréhension et d’encourager les actions.


Partenaires : Novartis et l’Iron Health Alliance

Novartis Oncologie a créé l’Iron Health Alliance en 2011, afin d’œuvrer à l’amélioration de la qualité des soins pour les personnes dont le diagnostic montre une surcharge en fer chronique. L’Iron Health Alliance vise à améliorer la prise en charge de la toxicité ferrique en faisant progresser les connaissances, en améliorant l’accès au diagnostique et en créant des partenariats avec des acteurs clés.

Trois piliers fondent l’action de l’Iron Health Alliance:
--• Faire progresser les connaissances sur les méthodes de diagnostic visant à détecter et mesurer la surcharge en fer
--• Améliorer les connaissances sur les anémies
--• Sensibiliser à l'importance de l'observance des traitements.

C’est dans cette dynamique que l’Iron Health Alliance a donné l’impulsion nécessaire à la réalisation du projet SCD Voices, permettant à Novartis de parrainer ce web documentaire.


Remerciements

Ce projet n’aurait pu voir le jour sans :
--• Les patients, dont les noms n’ont pas été dévoilés par respect pour leur vie privée et qui ont accepté de partager leur expérience
--• Les experts dans leurs champs respectifs, dont l’implication personnelle et professionnelle furent remarquables :
-----Prof. Rodolfo Cançado, São Paulo, Brésil
-----Dr. Marc Hendricks, Le Cap, Afrique du sud
-----Prof. Marianne de Montalembert, Paris, France
--• Ceux qui ont permi que les rencontres se passent dans les meilleures conditions, en particulier les équipes locales de Novartis, dont l’appui a été primordiale.