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Short bowel syndrome is a condition of which patients
either have lost a large amount of their intestine, or they
were born with an insufficient amount of intestine,
or their intestine just simply doesn't work well enough.
And the end result is they can't absorb enough nutrients from
eating, or from feeding through a G-tube or a J-tube.
To the point that they need,
supplemental nutrition through an IV line.
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There are a variety of causes.
The causes are different in children and adults.
In kids and babies in particular, the number
one cause is necrotizing enterocolitis, which is a severe
infection that's most common in premature infants.
And this infection can be devastating.
The intestine can get diseased, and
then frankly get necrotic and die.
And then the surgeon has no choice but
to take out the diseased segment to save the baby's life.
But then the children are left with short bowel syndrome.
Other kids are born with an insufficient
amount of intestine.
That's known as intestinal atresia.
Still, other kids may have a normal amount of intestine at
birth, and it works well for a month or two, or
even a few years, and then one day it just twists.
And that's because it wasn't configured properly
when they were inside their mom in the womb.
That condition is intestinal malrotation
with midgut volvulus.
And the problem with the twist is that they could lose most,
if not all, of their small intestine, and
be left with shortgut syndrome.
Other kids will have enough length of intestine but
it won't work.
One disease is Hirschsprung's disease,
where the intestine's not innervated properly.
That can infect a small portion of the intestine normally, but
it can also infect a large portion,
if not all of the intestine.
And those kids will need that intestine resected, and
then they're left with short bowel syndrome.
Another cause that we see in babies is gastroschisis,
which is a hole in interior abdominal wall,
next to the umbilical cord.
And when the babies are in-utero their intestine will poke out
through that hole.
And then the blood supply to the intestine will get choked off.
And then that intestine will die and they'll be left with
insufficient amount of intestine.
Adults on the other hand have different causes.
They usually have had multiple operations.
Either for bowel obstructions from adhesions or from
Crohn's disease, where they had multiple intestinal resections,
or they've had trauma.
Other causes for adults would be malignancy, or
complications from radiation, or ischemic vascular disease.
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The most frequent symptom of short bowel syndrome
are frequent loose stools, which can also contain blood or mucus,
which are signs of inadequate absorption.
With that, we'll get diaper rash in babies,
or perineal excoriation in older patients.
Also, they will often have abdominal distention
and bloating.
They can also have pain with that as well.
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Short bowel syndrome patients are treated with a combination
of medications and
feeding strategies with the goal being trying to augment
the amount of absorption that their intestine can achieve.
So the intestine will normally adapt after a large amount of
it's resected, and we try to optimize that.
And through these strategies, we're able to dial down
the intravenous nutrition to the point that we can actually
graduate the kids off the intravenous nutrition so
that they're exclusively fed by mouth or by G-tube.
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Johns Hopkins is a prime place for patients to come if they
have complex diseases, especially short bowel syndrome.
Because this place has the expertise and
the people, the infrastructure, and
the resources in order to take care of complex diseases.
This past year we opened a multidisciplinary intestinal
rehabilitation program,
which there are very few of in the country.
This is a multidisciplinary clinic that involves pediatric
surgery, pediatric gastroenterology with Dr.
Darla Shores.
Nursing specialists, nutritionists, pharmacists,
speech therapists, occupational therapists, and
behavioral psychologists all working together
to take care of patients through one clinic visit.
The parents like this because it streamlines their care.
And it's also been shown nationally in studies to improve
outcomes because you're able to provide a uniform care plan.
We call our center the circus clinic.
And the inspiration for
circus came from the big apple circus clowns,
which are in the hospital, and they entertain the kids.
And they give parents and
kids a few minutes to forget about their disease.
And so that was the inspiration for the name.
The acronym actually has a lot of meaning.
The Center for Intestinal Rehabilitation and
Cure Using Science.
The cure is in fact what happens to most patients.
Most patients don't need a transplant.
Most patients after a month to a couple of years will be
off of TPN and off their IV nutrition.
And then science, we use the scientific method in the clinic
to study our outcomes.
So we can keep track of what works and continue that.
But at the same time, if our management, something we do is
not helping, then we can get rid of that and move on.
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