SCID is the acronym for severe combined immunodeficiency disorder, an inherited, rare and potentially fatal immune system malfunction. The disorder is marked by lymphocyte abnormalities of both your T cells and B cells, hence the reason it’s entitled combined.
T and B lymphocytes are specialized white blood cells that are produced in your bone marrow to fight infectious disease. So, if you suffer from a severe combined immunodeficiency disorder, it’s nearly impossible for your body to combat virus, bacteria and fungus infections and win.
There are several causes of SCID, the most common being a problem with a gene found on the X chromosome. And because males only have a single X chromosome, this cause is limited to males. However, females can be carriers. This immunodeficiency is often given the ”boy in the bubble” reference.
Another SCID cause is an adenosine deaminase deficiency. This is far less prevalent, but causes an infection fighting issue in both male and females.
Typical symptoms of SCID are recurring serious infections, chronic diarrhea and failure to thrive. The types of infections may involve:
Severe combined immunodeficiency requires diagnosis and effective treatment as soon as possible after birth. Otherwise, an infection coupled with your failure to thrive symptoms can be fatal.
The most effective treatment for SCID caused by defective chromosome is a bone marrow transplant, usually finding the best match from a sibling. This treatment’s goal is for the introduced stem cells to build your immune system. And the earlier the better because the first few months of life is when your immune system is developed.
Some cases of SCID requires chemotherapy first to destroy already developed immune cells to prevent an attack on the introduced cells and to make room for the donated cells. This treatment may not be necessary for those with relatively few immune cells.
Post transplant, additional SCID treatment may involve antibiotics or immunoglobulins. And sometime in the future, gene therapy may replace the need to find a bone marrow donor for treatment.
If your SCID is caused by a missing enzyme, then replacement treatment is given weekly via an injection. Unfortunately, this is not a cure and regimented injection treatment is required for living a healthy life.
