What we do
Regional, National, and International Referral Centre
The Primary Immmunodeficiency (PID) program at The Hospital for Sick Children is the largest centre in Canada. It provides consultation services to over 2,000 outpatients referred from physicians and hospitals across Canada and in-patients with highly complex disorders such as Severe Combined Immune Deficiency Syndrome, receiving highly specialized bone marrow transplant procedures. This centre will ensure that SickKids' renowned bedside to bench to bedside process continues, allowing the hospital to produce new generations of research and clinical leaders who know why, for example, a common infection causes only a cold some individuals, while in others it causes devastating meningitis.
- Adelle Atkinson MD
- Eyal Grunebaum MD
- Chaim Roifman MD
At the most extreme end of inherited primary immunodeficiencies, are children with Severe Combined Immunodeficiency (SCID). These children have a complete absence of both T and B lymphocyte function, which is essential to lead a normal life. The absence of these types of functional white blood cells puts the very young child at risk for serious life-threatening infections, specifically opportunistic pathogens such as fungus, viruses and pneumocystis jiroveci pneumonia (PJP). Clinically, these young infants have recurrent severe infections, failure to thrive, diarrhea and sometimes skin rashes. Treatment options for SCID are currently limited to bone marrow transplantation, which involves infusing stem cells from a healthy donor who matches the patient. These transplants are either full-matched sibling transplants or matched unrelated donor transplants.
The Bone Marrow Transplant Program for primary immunodeficiencies at The Hospital for Sick Children is the only one of its kind in Canada. It is a leader in the field of Transplantation for SCID both in Canada and around the world. In the late 1970s the first national transplant for primary immunodeficiencies was performed at The Hospital for Sick Children, and since that time approximately 50 or more similar transplants have been performed. This is an extremely significant number given the rarity of these conditions. The first national matched unrelated donor transplant was also executed at The Hospital for Sick Children in 1990 in a patient with Omenn’s Syndrome, and since then SickKids holds the highest survival rate for these transplants at 77 per cent compared to 61 per cent in European centres. This transplant program provides care to children all across Canada with a wide variety of immunodeficiencies such as IL-2R deficiency and ADA deficiency, and many types of autosomal recessive and X-linked diseases. Both before during and after the actual Bone marrow Transplant, the Immunology Team at The Hospital for Sick Children is very involved with each patient and their family in terms of locating a potential donor and devising a plan of care. After the transplant the Immunology Team follows the patient very closely to provide complex post-transplant care. With a true "bench to bedside" approach to patient care, this program provides genetic diagnoses and outstanding therapeutic practices/clinical care for these patients, while adding to the growing body of knowledge about patients with these inherited disorders.
IVIG Interdisciplinary program
- Chaim Roifman MD
- Brenda Reid RN
There are a number of inherited disorders of the immune system that are characterized by patients lacking the ability to produce antibodies. Patients with documented antibody deficiency syndromes are enrolled into the gammaglobulin treatment program at The Hospital for Sick Children. Once stable on therapy patients may receive their treatment in a community hospital that is closer to home and be monitored by a community care physician. The program team consists of Dr. Roifman and an advanced practice nurse. They monitor the patients’ monthly laboratory results from the community and assess the patients at routine clinic appointments at SickKids. The Immunology team is always available for consultation to the patients and physicians in the community. This treatment model allows for patients to have the expertise of the Immunology team at The Hospital for Sick Children, while at the same time allowing for care to be delivered in a convenient location for patients and families. Patients in the program are also monitored for infections by history, pulmonary function tests, and CT scans of their lungs to determine if lung disease develops. As well, liver function tests and viral screening are performed to monitor the safety of gammaglobulin therapy, by screening for possible viral transmission through the blood product. With a long-term follow-up on over 150 patients, the Hypogammaglobulinemia Program at SickKids is one of the largest treatment programs for this patient group in the world. In being viewed as world leaders in the management and treatment of hypogammaglobulinemia, Dr. Roifman and the Clinical Team involved in this program have been invited to participate in National and International Consensus Conferences on patient management of hypogammaglobulinemia and gammaglobulin therapy.
National (cellular, molecular, genetic) Diagnosis Centre
- Chaim Roifman MD
- Harjit Dadi PhD
- Andrea Newell
Here at The Hospital for Sick Children diagnostic information for patients with primary immunodeficiencies in Ontario, and across Canada will be provided via techniques such as protein identification, cloning and sequencing, and a state of the art flow cytometry facility. This strictly pertains to service rendered for already known entities. Therefore as new types of PIDs are discovered, the service will have to adapt quickly and implement accurate and reliable tests. This facility currently includes a modernized immunology service laboratory, located near relevant research activities to their mutual benefit.