If your medical condition falls into one of the following categories, you may be a candidate for an intestinal transplant.
TPN related complications
- Parenteral nutrition-induced liver disease. Liver failure is the worst complication induced by parenteral nutrition. An increase in bilirubin, ALT, AST and Alkaline phosphatase may represent the first signs of liver failure. Liver failure is responsible for a large number of deaths caused by intestinal failure.
- Central venous catheter (CVC) related thrombosis of two or more central veins. If you lose access to one or more central veins from thrombosis (or clotted veins) secondary to a parenteral nutrition line, you may be at risk of not being able to access a central line for nutrition and hydration.
- Frequent episodes of central line sepsis. This could include two or more episodes per year of blood stream infection secondary to an infection of a central venous line requiring hospitalization or a single episode of line-related fungemia (infection caused by fungus).
- Frequent episodes of severe dehydration despite intravenous fluid administration in addition to parenteral nutrition.
Underlying disease with an increased risk of morbidity
- Desmoid tumors associated with familial adenomatous polyposis.
- Congenital mucosal disorders (i.e., microvillus inclusion disease, tufting enteropathy).
- Ultra short bowel syndrome (gastrostomy, duodenostomy, residual small bowel <10 cm in infants and <40 cm in adults).
Intestinal failure with intolerance to parenteral nutrition
- Intestinal failure with high morbidity (frequent hospitalization, narcotic dependency) or inability to function (i.e., pseudo-obstruction, high output stoma).
- Patient’s unwillingness to accept long-term home parenteral nutrition (i.e., young patients).
Intestinal rehabilitation is the science that stimulates the amazing ability of the intestine to adapt to different and unexpected medical and surgical conditions. It encompasses pharmacological, dietary, and surgical options that can return the intestine to a normal function.
When the intestine is irreversibly affected and rehabilitation is not possible, parenteral nutrition is the following step. Parenteral nutrition (PN) saves thousands of lives each year, but is not tolerated by everyone. Patients with a very short length of bowel, those who develop recurrent catheter related blood stream infection (CRBSI), or those who experience multiple central vein access thromboses may be at a higher risk of developing life threatening complications from the use of long-term PN. Indications for intestinal transplantation vary in the adult and pediatric populations.
In the adult population, intestinal failure is usually the result of one of the following:
- Short Bowel Syndrome (SBS). This is a malabsorption disorder caused by the surgical removal (resection) of large sections of intestine. Most cases are acquired due to removal of diseased bowel, intestinal trauma, or loss of blood supply to the gut; although some are born with a congenital short bowel. The degree to which patients suffer the consequences of SBS depends largely on the remaining intestinal anatomy. A large jejunal resection should not disturb absorption substantially because of the ability of the remaining ileum and colon to absorb increased fluid and electrolytes, maintain bile salts, and prolong movement of food and fluid through the intestine. A large ileal resection leads to significant fat malabsorption, and if the colon also is resected, fluid and electrolyte balance can be severely impaired.
- Motility disorders may also be referred to as Chronic Intestinal Pseudo-obstruction (CIPO). The anatomy and length of the bowel may be preserved, but the function (the way the small bowel moves) is impaired. Symptoms may be similar to a bowel obstruction and can include severe abdominal pain and distension, severe bloating, nausea, vomiting and the inability to eat.
- Intra-abdominal non-metastasizing tumors are tumors that grow locally and progressively obstruct the bowel. When growth occurs close to the intestinal blood supply, tumor removal can only be possible by resecting the entire intestine and replacing it with a transplanted intestine.
A list of the most common causes for intestinal failure in the adult population is listed below:
- Crohn’s Disease
- Motility Disorder
In the pediatric population, intestinal failure is characterized by the following:
- Short bowel syndrome and dysmotility disorders occur in the pediatric population as they due in the adult population.
- A large number of pediatric SBS cases are caused by necrotizing entero-colitis (NEC), gastroschisis, intestinal atresia, and other congenital disorders.
A list of the most common causes for intestinal failure in the pediatric population is listed below.
- Necrotizing enterocolitis
- Intestinal atresia
- Intestinal pseudo-obstruction
- Microvillus inclusion disease
- Intractable diarrhea of infancy
- Autoimmune enteritis
- Intestinal polyposis
Diagnosed with Intestinal Failure?
If you are diagnosed with intestinal failure, the first step is to contact Cleveland Clinic's Intestinal Rehabilitation and Transplant Center. Having a diagnosis of intestinal failure does not mean that you need an intestinal transplant; only a small number of patients with intestinal failure need a transplant. You may be an excellent candidate for intestinal rehabilitation and adaptation and be able to avoid or eliminate the need for parenteral nutrition.
Cleveland Clinic has one of the largest and most successful intestinal rehabilitation programs in the world. There are essentially two ways of optimizing the intestinal function in patients with intestinal failure: medical/dietary treatment and surgical treatment. Medical/dietary treatment includes diet adjustments and medications that are prescribed to improve digestive and absorptive function of the remaining bowel (growth factors, drugs that increase or decrease the intestinal motility, etc.). A small number of patients are found to have out-of-circuit bowel as a result of previous surgery that can be put back in continuity with the bowel that is exposed to food so that absorptive function can be improved. Furthermore, new surgical procedures (STEP, Bianchi and tapering procedures) are offered at our center to enhance the residual intestinal function. If existing medical and surgical treatments do not improve intestinal function, the patient is evaluated for PN and possibly intestinal transplantation.