Muscat: A baby girl suffering from Severe Combined Immunodeficiency (SCID) undergoes stem cell transplant at the Royal Hospital (RH).
The Royal Hospital, represented by Clinical Immunology department with support from the RH’s Pediatric Intensive Care Unit (PICU), and the Bone Marrow Transplantation unit in the Sultan Qaboos University Hospital (SQUH), performed the operation.
The child was diagnosed with SCID when she was two-month old. This is an inherited primary immunodeficiency disease (PIDD) that typically appears in infancy and results in profound immune deficiency conditions leading to a weak immune system that is unable to fight off even mild infections.
After diagnosis, a treatment plan was prepared and initiated immediately.
In order to reduce the chance of both regular and opportunistic infections, the affected infant was given oral microbial antibiotics daily in addition to a monthly Intravenous Immunoglobulin (IVIg) treatment provided by the Clinical Immunology department.
As conventional treatments failed to control the severe bacterial pneumonia and blood infection as well the associated hyper immunity of the child (Fajer) and her condition was deteriorating, the team decided to cure the primary disease- (SCID).
The most effective treatment for SCID is bone marrow transplant (also known as a stem cell transplant). In this treatment, an infant with SCID receives healthy stem cells from a matched donor, usually a healthy brother or sister.
By identifying a matched donor – Fajer’s big brother, preparation was made for stem cell transplantation. The procedure was performed in the RH’s PICU after receiving the stem cell from SQUH. Following transplantation, a substantial improvement was noted in the patient’s health.
While this kind of treatment is offered in SQUH and is currently not available at the Royal Hospital, the procedure was performed within exceptional conditions that can be highlighted in the following:
The child’s health condition was unstable to be transferred to the SQUH’s Bone Marrow Transplantation department for direct observation. Therefore, there was a possibility of complications occurring in case the child receives stem cells from the donor without the direct supervision of the bone marrow transplant team.
The RH’s PICU team is not familiar with this type of treatment.
Lack of necessary laboratory examinations to identify the progress of the transplant procedure and predict its complications.
Despite the challenging circumstances, the PICU has spared no efforts to provide utmost-specialised distinct health services to the patient. Surly, this procedure presents a qualitative step in introducing similar therapies for children with primary Immunodeficiency in the future.
Now in her tenth month, Fajer is enjoying good health with her parents and brother- the donor. The Pediatric Clinical Immunology department is following up closely her health condition.
It is worth mentioning that SCID is considered to be the most serious PIDD. It may lead further to an uncontrolled Hyperimmunity. Hyperimmunity in children with severe combined immunodeficiency causes the immune system - which is damaged - to attack the body itself causing other health conditions as Hemophagocytic lymphohistiocytosis (HLH), bone marrow immunosuppression, or the development of blood and lymphatic system cancer.
Hyperimmunity poses a real threat to a child’s life. This type of disease is very rare. However, due to the increase of relative-marriage rate in Oman and the neighboring countries, it is estimated to affect 4.6-5 of every 100,000 Omani newborn per year.