Only a few decades ago the clinician had little to offer the child with short bowel.There was no effective alternative nutritional support and surgery was inconsistent. The prospects were bleak and the option of  'No Treatment' was often considered to be a welcome and indeed kinder solution for the distraught family. The advent of parenteral nutrition allowed survival and growth, and parents and clinicians dared to hope. However the course was far from smooth and many children came to a tragic end from loss of central venous access and inability to deliver intravenous feeding, or worse still from relentlessly progressive fatal liver disease.

Mr Adrian Bianchi.jpg

Pictured here from left... Mr Adrian Bianchi with his then secretary Carole Jones and Mr Antonino Morabito.

Persistence and creative approaches driven by the belief that children and families with Short Bowel deserved better, led to improvements in parenteral nutrition and better sepsis and central line management such that longer life and actual growth became possible.  Innovative surgery developed the concept of Autologous Gastro-Intestinal Reconstruction (AGIR) with Longitudinal Intestinal Lengthening and Tailoring (LILT) that remodelled the autologous bowel to enhance its tremendous potential to adapt and to increase its absorptive capability.  Clinicians began to speak of Enteral Autonomy with a good quality life, possibly of normal span!  


Rationalization of the available options led in the late ‘90s, to a 'Structured Approach' that combined several techniques.  Thus early Bowel Expansion increased the available bowel that was then Tailored and Lengthened (LILT).  Single or multiple Reversed Antiperistaltic Segments that delayed nutrient passage allowed increased nutrient absorption that was further enhanced by drugs (loperamide, clonidine, GLP-2).  In 2003 the Serial Transverse Enteroplasty (STEP) provided another means for tailoring and lengthening bowel, and another procedure, the Spiral Enteroplasty (SILT), is now in development.  At the end of the line and if no other therapy is possible Small Bowel and Bowel-Liver Transplantation, though complex and potentially hazardous, offers a final but significant ray of hope.  

During such prolonged serialized therapy the parents are empowered to takeover the management of their children through Home Parental Nutrition supported by Specialist Nurse ‘Family Care Support Teams’, thus also developing a normal social dimension. 
Today Enteral Autonomy with good quality life of normal span has become a real and achievable expectation that is indeed the birthright of the child and family faced with Short Bowel.  The dragon has been tamed, and with determined efficient management and appropriate resource organization the future for the child with short bowel is bright and hopeful.

Adrian Bianchi November 2011