Clinical services
Centre for paediatric primary immunodefiency

Primary immunodeficiencies (PIDs) are a broad range of inherited disorders that compromise the normal function or regulation of the immune system. They range from relatively minor antibody deficiencies to severe combined immunodeficiencies (SCID) which are fatal in the first year of life unless effectively treated. These conditions are invariably the result of defects in one or other genes that control immune system development and function and are inherited most commonly in an X-linked or autosomal recessive manner. Although individually PIDs are relatively rare, collectively they constitute a significant paediatric healthcare burden and more so in countries and cultures where there is a high degree of consanguinity. The Immunology services at GOSH have developed over many years to become one of the World’s leading centres for treatment of patients with PID. This is encompassed in a comprehensive and extensive in-patient and out-patient care programme, including national molecular diagnostic testing, antibody replacement, and haematopoietic stem cell transplantation (HSCT) and gene therapy. Alongside the clinical services at GOSH, the Molecular Immunology Unit at the Institute of Child Health (ICH) has been at the forefront of research into the molecular basis and pathophysiology of PID, and the development of novel treatments. This has led to the establishment of a cohesive Centre for Immunodeficiency to oversee both clinical and research activities. This close interface between GOSH and ICH has resulted in a highly active translational research programme and the development of novel diagnostic and management strategies that are not readily available elsewhere. This includes screening for disease causing genes, novel strategies for HSCT, somatic gene therapy, and thymic transplantation.

Out-patient care

Much of the investigation and care of children with suspected or confirmed immune deficiency takes place in out-patient clinics. Each child has a named consultant, who will usually see him/her in clinic, although sometimes a registrar (junior doctor) will see him/her in consultation with the consultant. One or more of the immunology nurse specialists may be involved if an immunodeficiency is confirmed. The care of all children is also shared with a paediatrician at the local hospital and the GP.

Many patients require active care and input despite being outpatients. For this reason we have a dedicated team of clinical nurse specialists who liase closely with families at home and manage their care with the medical staff. This allows greater flexibility for patients and their families and helps limit the length of in patient admission and stay.


Day-cases

Occasionally it is necessary for children to attend the hospital for one or more days to have investigations, see other specialists, or receive some treatment. If there are too many tests and/or consultations to fit into one day, accommodation can be arranged nearby. These visits usually take place on the Safari Day Care unit.


In-patient stays

Any child who needs in-patient admission to GOSH under the care of the Immunology department will usually be admitted to Robin Ward, although occasionally other wards are used. Robin ward has a team of specialist nurses and doctors. The attending consultant, who is responsible for day to day decision-making and care of in-patients, usually changes each month, with four consultants taking part in the rota. However, important decisions always involve discussions with a child’s named consultant.

Robin ward is a specialised unit for children who require protection from infection (and children affected by infectious diseases who need isolation). There are ten individual cubicles. Careful anti-infection precaution must be adhered to, and there are important guidelines concerning visitors. Each cubicle contains a folding bed for one parent to be able to stay with his/her child. Accommodation cannot be guaranteed for more than one parent.

Robin ward has a kitchen and sitting room for resident and visiting parents and families/friends. There is a playroom for children who are able to leave their cubicles. Meals are not provided for parents, but food and drink can be bought from the Peter Pan Cafeteria, hospital canteen, Costa coffee bar, or the hospital shop.