Severe Combined Immunodeficiency Disease
Since the prognosis for SCID is very poor if a compatible bone marrow donor is not available, nursing care is directed at supporting the family in caring for a fatally ill-child. Genetic counseling is essential because of the modes of transmission in either form of the disorder. Nursing goals are directed at helping parents prevent sources of infection in the child, such as cautious isolation from crowded facilities and individuals with active infection, meticulous skin and mouth care, good general nutrition, and careful supervision during periods of activity to prevent skin trauma. However, even with exacting environmental control, these children are prone to opportunistic infection. Chronic fungal infections of the mouth and nails with Candida albicans are frequent problems despite vigorous efforts at prevention or treatment.
A hoarse voice may result from repeated esophageal and vocal cord erosions from the fungus, It is important to stress to parents that such conditions are not a result of laxity on their part in preventing them but are the result of the severe immunologic disorder. Parents should be encouraged to immediately notify a physician regarding any evidence of a worsening infection.
Children who receive frequent injections of immune serum globulin (ISG, or IG) need support during the procedure because the injections are painful. Infants are best comforted by their parents, but toddlers and preschoolers may benefit from needle play. Immune globulin is injected deeply into a large muscle mass, usually the vastus lateralis. To prevent tissue damage and provide maximum absorption, the total amount may be divided into two injections and given in two different sites, A record is kept of the sites to ensure a rotating schedule for future injections. An intravenous preparation of ISG (intravenous modified ISG [MISG]) is available that reportedly is more effective and causes 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 signs of this unusual reaction, including pink, scaling pruritic palms and soles, photophobia, sweating, irritability, and insomnia.
Care of a patient undergoing bone marrow transplantation is mainly directed at preventing infection. Due to the fact it takes 7 to 20 days before evidence of bone marrow functioning becomes obvious, hospitalization is long. It is not the purpose of this discussion to detail the care of the patient with a bone marrow transplant because of the specialized care involved, except to emphasize that the psychological needs of the parents and child are tremendous. For the parents, it represents the last hope for successful therapy and survival. For the child it means sensory deprivation because of isolation, numerous blood tests, and the possibility of more pain and suffering if a graft-vs-host reaction occurs. To meet these needs, a sensitive, consistent team of nurses who function effectively as members of the total health team is essential.
