Three papers have been published recently in the scientific literature describing: 1) gastrostomy tube (G-tube) safety and satisfaction in patients with A-T; 2) the care of critically ill A-T patients and 3) the care of patients with A-T undergoing operations and the possible risks associated with the use of anesthesia for surgical procedures.
G-Tube Placement and A-T
Maureen Lefton-Greif, PhD, CCC-SLP, BRS-S, swallowing specialist at the A-T Clinical Center at Johns Hopkins Hospital, published a 2011 study in the Orphanet Journal of Rare Diseases entited “Safety and caregiver satisfaction with gastrostomy in patients with Ataxia Telangiectasia.” Dr. Lefton-Greif and colleagues found that G-tube placement was well tolerated and associated with easier and more enjoyable mealtimes when placed in young patients with A-T, before the development of significant nutrition and respiratory problems. A caregiver survey also demonstrated that patients had more energy to participate in their daily activities after receiving G-tubes. The risks and benefits of obtaining a G-tube will be different for each patient and should be thoroughly discussed with the physician. It is recommended that G-tube placement occur at a tertiary medical care center (a health center that has extensively trained specialists and advanced medical technologies). [1]
Care of Critically Ill A-T Patients and Anesthetic and Surgical Risks in Patients with A-T
In 2012, a team of clinicians from the Departments of Anesthesiology and Critical Care Medicine and the A-T Clinical Center at Johns Hopkins Hospital published two studies examining care for critically ill patients with A-T and the risks associated with anesthesia and surgery in these patients.
The study on critically ill A-T patients examined the course of seven patients admitted to the Pediatric Critical Care Unit of a single tertiary hospital. The most common admitting diagnosis to the intensive care unit was respiratory distress, and suspected or confirmed bacterial infections were common. These findings illustrate the relationship between acute life-threatening illness, chronic lung problems and immune deficiencies in A-T. Although previous reports indicated that A-T patients admitted to the intensive care until had very rapidly progressing, severe and often fatal pulmonary complications, a large number of patients in the current study survived to discharge. The clinicians suggest that this improvement in survival may be due to family support of patients, multidisciplinary medical care, and better diagnosis. They recommended that future studies of critically ill patients with A-T investigate: the effects of limiting exposure to ionizing radiation; methods for short and long term ventilator support; optimal management of immune system problems and the effects of nutritional status. [2]
Anesthetic and surgical risks for patients with A-T were studied in a single tertiary care center. In this patient group there were no deaths, no unplanned hospital admissions and no major complications. These results suggest that general anesthesia and mechanical ventilation associated with surgical procedures can in general be tolerated by patients with A-T, with only minor concerns (such as mild hypothermia) occurring after surgery. Noting the lower body mass index percentiles in the patients with A-T as compared to healthy age and gender matched individuals, and because nutritional status has been correlated with wound healing and infection, the Hopkins team recommended that nutritional support for the A-T patient be maximized both before and after surgery. Health centers providing medical care to A-T patients from the time of admission for surgery through discharge should be aware of the complex and multisystem problems that can occur in these patients. Further studies examining anesthetic and surgical risks for patients with A-T should focus on optimization of nutritional and pulmonary support and the risks associated with sound infections and other immune deficits. [3]
References
1. Lefton-Greif, M.A., et al., Safety and caregiver satisfaction with gastrostomy in patients with Ataxia Telangiectasia. Orphanet J Rare Dis, 2011. 6: p.23.
2. Lockman, J.L., et al., The critically ill patient with ataxia-telangiectasia: A case series*. Pediatr Crit Care Med, 2012. 13(2): p. e84-90
3. Lockman, J.L., et al., Anesthetic and perioperative risk in the patient with Ataxia-Telangiectasia. Paediatr Anaesth, 2012. 22(3): p. 256-62.