More than half of our blood is composed of plasma — the pale component of blood, most of which is water, while the rest is crucial proteins.
The proteins are crucial because they play a big role in what makes our immune system tick. It is these proteins that facilitate the detection and destruction of pathogens — foreign microbes that cause diseases. The presence and functioning of these proteins, hence, is what constitutes our immunity.
The C1 Esterase Inhibitor is one such protein.
The C1 Esterase Inhibitor, or C1-INH, is a protein that controls the C1 protein. The C1 protein is one of the nine major proteins (C1, C2...C9) that are critical to the working of the complement system — a sub-system of the immune system responsible for the strength of antibodies in fighting infections. In fact, the complement system also regulates proteins responsible for the transport and disposal of waste materials, such as damaged cells.
Therefore, the absence or low levels of C1-INH make our immune system unable to carry out these vital functions.
The consequence is a rare disease called Hereditary Angioedema (HAE).
As the name suggests, HAE is a rare hereditary disease caused by a genetic mutation that leads to low levels of C1-INH. Its chief symptoms are sudden, and often painful, attacks of swelling, which affect the face, throat, hands, arms, legs, feet, and abdomen.
The swelling is the result of blood vessels and capillaries becoming leaky, allowing fluids to collect in the surrounding tissues.
Hereditary Angioedema is rare, and typically affects children and young adults under the age of 20. It is hereditary, as explained since its defect lies in the DNA, which is inherited from a parent. However, Angioedema can also be acquired. Acquired Angioedema typically affects adults over 40, and is likely to affect people suffering from cancer or autoimmune diseases.
Hereditary or Acquired, Angioedema is treated with plasma therapies based on C1-INH.
The therapies are manufactured from plasma donated by healthy, voluntary donors — plasma which is rich in C1-INH.
The proteins are separated and concentrated and administered intravenously. They provide an external supply of C1-INH, which makes up for its low levels.
These patients, some every single day, rely on the generosity of regular plasma donors to live a 'normal', healthy life. You make it possible.
Learn more about plasma at www.iplasma.life