Since the prognosis for SCID is very poor without a compatible bone marrow donor available, nursing care is aimed at helping the family care for a seriously ill child. Genetic counseling is essential due to the modes of transmission in any of the forms of the disorder. Nursing goals are aimed at helping parents prevent sources of infection in the child, such as cautious isolation from crowded facilities and people with active infection, meticulous care of the skin and mouth, good general nutrition, and careful supervision during periods of activity to prevent 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 frequent problems despite vigorous prevention or treatment efforts.
A hoarse voice can be the result of repeated erosions of the esophagus and vocal cords caused by the fungus. It is important to emphasize to parents that such conditions are not the result of laxity on their part to prevent them, but the result of a serious immune disorder. Parents should be encouraged to notify a physician immediately of any evidence of a 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. Immunoglobulin is injected deep into a large muscle mass, usually the vastus lateralis. To prevent tissue damage and provide maximum absorption, the total amount can be divided into two injections and administered at two different sites. A registry of the sites is kept to ensure a rotating schedule for future injections. An intravenous preparation of ISG (modified intravenous ISG [MISG]) is available which is reportedly more effective and causes less distress.
A rare complication of long-term ISG administration is mercury toxicity (pink disease) caused by a commercially available mercury-containing bacteriostatic agent. Nurses working with these patients should be aware of the signs of this unusual reaction, which include itchy and scaly pink palms and soles, photophobia, sweating, irritability, and insomnia.
The care of a patient undergoing a bone marrow transplant is primarily aimed at preventing infection. Due to the fact that it takes 7 to 20 days before evidence of bone marrow function is obvious, hospitalization is long. It is not the purpose of this discussion to detail the care of the bone marrow transplant patient due to the specialized care involved, except to emphasize that the psychological needs of the parents and the child are tremendous. For parents, it represents the last hope of successful therapy and survival. For the child, it means sensory deprivation due to isolation, numerous blood tests, and the possibility of further pain and suffering if a graft-versus-host reaction occurs. To meet these needs, it is essential to have a coherent and responsive team of nurses who function effectively as members of the total healthcare team.