Rhonda Smith sits with her 3-year-old daughter, Destiny. When Destiny was born, doctors detected that her small bowel was blocked because the lumen of the bowel was not formed. She has undergone about a dozen surgeries and in January 2011 received a small bowel transplant at Seattle Children’s. Watch her story.
For more than three years, Destiny Smith’s routine consisted of daily intravenous nutrition, frequent trips to Seattle Children’s Hospital, about a dozen surgeries and a waiting game on the transplant list. For the 3-year-old’s mother, Rhonda Smith, that waiting game seemed like it would last forever. But in January 2011, the wait ended.
“I just thought this was the life that she had and that we’re going to appreciate the life that we shared with her and whatever happened, happened,” says Smith. “We never thought we were going to get a transplant. Even when they called me, I thought it was a joke.”
But it wasn’t a joke. Destiny was getting a small bowel transplant.
While Smith was pregnant, an ultrasound detected a potential blockage in Destiny’s small bowel.
When she went into early labor at 32 weeks, doctors quickly detected the blockage, which was caused by the lumen, or inner cavity, of the bowel not forming. This is known as intestinal atresia and meant that Destiny would need an operation shortly after birth to connect the short length of small bowel to her large intestine.
The small intestine, or small bowel, is a hollow tube about 20 feet long that runs from the stomach to the beginning of the large intestine. The small intestine breaks down food from the stomach and absorbs much of the nutrients from the food.
Destiny had short bowel syndrome, meaning her small bowel was too short to digest and absorb food. As a result, she needed to be nourished by intravenous feeding, known as total parenteral nutrition (TPN).
The function of the small bowel (also known as the small intestine) is to absorb fluids and nutrients, says Dr. Simon Horslen, medical director for Liver and Intestine Transplant at Children’s, professor of pediatrics at the University of Washington School of Medicine, and a member of Destiny’s medical team.
Destiny spent about a month at a local hospital, then transferred to Children’s where she stayed until she was about 5 months old. Since her birth, Smith says, Destiny has had more than 12 surgeries and the family has made more than 50 trips to Children’s from their Kent home.
TPN is life-saving in situations like Destiny’s, but it carries the risk of serious complications, including severe liver disease. This occurred with Destiny, so she was put on the transplant waiting list for a combined liver and small bowel transplant.
While Destiny was on the transplant list, her medical team started her on a promising new form of intravenous fat, called Omegaven, which was available through a study funded by Children’s. Omegaven is derived from fish oil, as opposed to the soy oil used in traditional preparations of intravenous fats, and has shown promise at reversing the liver disease associated with long-term TPN use.
When Destiny was about a year old, her liver disease had improved so much that doctors took her off the liver transplant list.
In January, Smith got the call that changed everything: a small bowel was available for Destiny. After a long (almost 10-hour) surgery at Children’s, Smith says the journey toward a more “normal” life for her young daughter began. In fact, she says Destiny will soon eat all her food by mouth instead of through her gastrostomy tube. Once that transition is made, the tube will be removed.
“There is light at the end of the tunnel,” says Smith. “Never give up hope.”
What is intestinal failure?
Dr. Simon Horslen
The most common cause of intestinal failure is short bowel syndrome. Children with this syndrome have often lost more than half of their small intestine through a resection to treat a disease, through an injury, or because they were born without a properly functioning small bowel, as in Destiny’s case.
Short bowel syndrome can also be caused by diseases or conditions including gastroschisis, intestinal atresia, necrotizing enterocolitis, volvulus, inflammatory disorders, trauma or thrombotic disorders. Other diseases leading to intestinal failure include motility and absorptive disorders.
At Children’s, about 80% of patients with intestinal failure have some form of short bowel syndrome, says Horslen. Although the number of children with intestinal disorders has not increased over time, he says providers have become more aggressive in saving patients with these “intestinal catastrophes.”
He says a lot has changed in the last decade; Dr. Jorge Reyes, director of Transplant Services at Children’s and chief of the Division of Transplantation in the Department of Surgery at the University of Washington School of Medicine, agrees.
To transplant or not to transplant
Dr. Jorge Reyes
Destiny is the fifth patient to receive a small bowel transplant at Children’s. These types of procedures are rare because there are few patients with irreversible intestinal failure. They may become even more uncommon in the future, thanks to advances by Children’s team of intestinal rehabilitation experts.
Although Children’s is the only intestinal transplant program in the Northwest, the region does not receive a large number of organ offers compared to more densely populated areas like the East Coast and California, says Reyes. As a result, our donor organs often come from distant places.
Also, because small bowels can only stay viable outside the body for about 11 to 12 hours, we can only go as far east as Chicago to receive these vital organs.
These restrictions limit the number of small bowel transplantations our world-renowned surgeons can perform. For that reason, Children’s providers are working on ways to rehabilitate damaged intestines, limiting the need for a bowel or liver transplant.
Patients like Destiny, who have little or no small bowel, are given TPN to get nutrients into their bodies. But prolonged use of TPN carries risks – besides potential liver disease – including infections in the line that delivers nutrition and blood.
Over time, these infections become more difficult to treat and more dangerous for patients. To address these infections, Children’s providers have begun to lock ethanol into the intravenous lines when not in use to clean the bacteria build-up. The ethanol sits in the line and does not enter the patient, says Horslen.
Bacteria tend to stick to the plastic lines and the ethanol unsticks and kills the bacteria. This innovation, which is only used in patients who have the most recurrent infections, is proving successful.
“Despite transplants being my business, if I can manage a patient without doing a transplant, then I consider that the ultimate success,” says Horslen. “The fact that we are getting patients that 10 years ago we would have transplanted and now we’re getting them to avoid transplant and they’re doing well, that’s great news.”
Reyes, whose job it is to procure organs and perform the transplant surgeries, is thrilled with the direction in which the management of intestinal failure is headed.
The future of intestinal disease
Children’s healthcare providers have become very good at treating intestinal failure and performing bowel transplants, but Reyes says there is a lot of work still to do in the field. In fact, transplantation of the small bowel only dates back to the 1990s.
“Losing the bowel is still something we’re learning the impact of,” says Reyes. “We call it intestinal failure, but it isn’t just the fact that you’re on TPN. Suddenly, your stomach isn’t working well; you may become a diabetic; and, of course, there’s the issue of the liver.”
Since the field has progressed so much during the last 20 years, Reyes believes healthcare providers will gain a better understanding of immunology and the normal physiology of the bowel over the next two decades.
Other new territory already presenting itself may be tissue engineering to clone cells so patients can grow their own intestine back, he says.
“As these operations become more commonplace and we have more survivors, we’ll probably be able to avoid transplant in many patients,” says Reyes. “Even though I’m a transplant surgeon who puts in these organs, it’d be great if I could be put out of business and reinvent myself because there’s no more need for transplant.”
For Destiny, the future is bright, thanks to intestinal rehabilitation and her small bowel transplant. The lively 3-year-old girl, who loves playing with her brothers and watching “Dora the Explorer,” can, with the aid of immunosuppressive medications to prevent the rejection of the organ, live a normal life.
“Now I look forward to regular things you look forward to for your child – kindergarten, first grade, being just like my son who is in second grade,” says Smith. “I look forward to her having a great life like nothing ever happened.”