Here is the perfect illustration that in life, we must never give up on any obstacles but also diseases. Here is the example with Léah, a 5-year-old girl with severe congenital neutropenia who continues to spread her good humor in her hospital room and on the social networks on which she is followed and supported by thousands of people.
Léah, a five-year-old American girl, has severe congenital neutropenia that has already required two bone marrow transplants and requires her to be hospitalized most of her time.
What is severe congenital neutropenia?
Severe congenital neutropenia is a blood disease that induces a fertile ground for recurrent infections in people who have it. It is characterized by a deficiency of neutrophils, which are part of leukocytes and are a type of white blood cell, which are born in the bone marrow before being released into the blood and lymph and play a leading role in inflammation and in the fight against infections.
Severe congenital neutropenia can result from mutations in one of many different genes that play a role in the maturation and function of neutrophils, which are cells produced by the bone marrow. These genetic mutations cause the formation of neutrophils that decline rapidly or do not work properly.
Congenital neutropenia can occur from birth and causes repetitive infections in early childhood, including lung, liver and sinus infections, as well as conditions such as osteopenia, osteoporosis, and severe bone without missing the risk of contracting certain cancerous diseases of the blood such as leukemia or myelodysplastic syndrome (insufficient production of healthy mature blood cells by the bone marrow).
In the case of Léah, it presents difficulties to eat properly because of the problem of its absorption by the intestinal tract.
What is a bone marrow transplant?
Léah's congenital neutropenia required two bone marrow transplants within two years, which caused the girl to have a graft versus host disease (GvHD), a major medical complication in a row. bone marrow transplants characterized either by the presence of immunocompetent cells in the graft capable of reacting against the histocompatibility antigens of the host, or by a difference in histocompatibility (compatibility rate between two organs or tissues, allowing a transplant not to be rejected) between donor and recipient, or the inability of the recipient to reject the transplant.
Marrow transplantation; where the cells are made (white blood cells, red blood cells and platelets), is a transplant involving the transfer of bone marrow from one individual to another suffering from a pathology of his / her marrow, as it is the case with the neutrophil deficiency and which is the subject of the severe congenital neutropenia of which Léah suffers.
The bone marrow present inside the bones makes the blood cells; red blood cells that carry oxygen, white blood cells that fight against infections and platelets that intervene in coagulation, and lymphocytes, responsible for recognizing and annihilating any foreign element introduced into the human body. Bone marrow transplantation is therefore essential for patients with serious blood diseases and can save their lives.
In any case, neither the congenital neutropenia, nor even the grafts that Leah has undergone, have tainted the ability to sing, to demonstrate each day of joy, good humor while benefiting the medical profession. Oakland Hospital. A beautiful lesson of courage at such a young age.
