Since the prognosis for SCID is very poor if a matched bone marrow donor is not available, nursing care is directed at supporting the family in caring for a critically ill child. Genetic counseling is essential due to the modes of transmission in any of the forms of the disorder. Nursing goals are directed at helping parents prevent sources of infection in the child, such as careful isolation from crowded facilities and people with active infection, meticulous skin and mouth care, good nutrition and careful supervision during periods of activity to avoid skin trauma. However, even with rigorous environmental control, these children are prone to opportunistic infections. Chronic fungal infections of the mouth and nails with Candida albicans are common problems despite vigorous prevention or treatment efforts.

A hoarse voice can be the result of repeated esophageal and vocal cord erosions by the fungus. It is important to emphasize to parents that such conditions are not the result of laxity on their part in preventing them, but are the result of a serious immune disorder. Parents should be encouraged to immediately notify a physician of any evidence of worsening infection.

Children who receive frequent injections of serum immunoglobulin (ISG or IG) need support during the procedure because the injections are painful. Babies are best comforted by their parents, but toddlers and preschoolers can benefit from needle play. The immunoglobulin is injected deep into a large muscle mass, usually the vastus lateralis. To prevent tissue damage and provide maximum absorption, the entire amount can be divided into two injections and given at two different sites. A register of sites is maintained to ensure a rotating schedule for future injections. An intravenous preparation of ISG (modified ISG intravenous [MISG]) is available that is reported to be more effective and to cause less distress.

A rare complication of long-term ISG administration is mercury toxicity (acrodynia or pink disease) caused by a mercury-containing bacteriostatic agent in the commercial preparation. Nurses working with these patients should be aware of the signs of this unusual reaction, which include pink, scaly, and itchy palms and soles, photophobia, sweating, irritability, and insomnia.

The care of a patient undergoing a bone marrow transplant is primarily directed at preventing infection. Due to the fact that it takes 7 to 20 days before evidence of bone marrow function is evident, hospitalization is long. It is not the purpose of this discussion to detail the care of the bone marrow transplant patient because of the specialized care involved, except to emphasize that the psychological needs of the parents and child are tremendous. For parents, it represents the last hope for successful therapy and survival. For the child, it means sensory deprivation due to isolation, numerous blood tests, and the potential for more pain and suffering if a graft-versus-host reaction occurs. To meet these needs, a responsive and consistent team of nurses who function effectively as members of the total health team is essential.