Ethical considerartions in the clinical management of infants with severe combined immunodeficiency

Severe combined immunodeficiency (SCID) is an inherited primary immunodeficiency syndrome characterized by a profound deficiency in T-lymphocytes and variable defects in the B-lymphocyte number. The present study aimed to evaluate the ethical aspects of the clinical management of children with SCID. This report is based on the case of a 6-month-old male infant with SCID diagnosis presented during the Medical Ethics Grand Rounds in the Children's Medical Center in Tehran, Iran.The patient had a positive history of recurrent infections and frequent hospitalization. His parents were consanguineous and came from a low socioeconomic level of the community. He was the fourth child of the family. The first and second children were healthy girls, while the third child was a boy with a history of numerous problems post vaccination who had eventually died of widespread infections. The physician had recommended bone marrow transplant, but the parents had been directed by a neighbor to visit a religious healer.Timely management of severe combined immunodeficiency can reduce complications and improve the patients’ quality of life. The need for early screening tests is therefore ethically justified, although ethical issues surrounding the subject should not be overlooked. Instances of these issues include: neglecting the scientific criteria necessary to conduct the tests; the economic burden imposed on the family of the child; failure to provide sufficient information to parents and ignoring their consent to conduct testing and treatment; lack of equitable access to diagnostic and treatment facilities; and lack of attention to the child’s autonomy. It should, however, be noted that in certain cases, healthcare providers could perform their management activities with an ethical and supportive approach, taking into account all physical and psychological needs of infants with severe combined immunodeficiency, as well as those of their families.