Re: DIOS

[Guest guest]

,

I'm sorry to hear that Ashton's having trouble. Here's some

additional information I found. I hope he's feeling better soon!

J Pediatr Gastroenterol Nutr. 1983 May;2(2):299-301. Related

Articles, Links

Incidence of distal intestinal obstruction syndrome in cystic

fibrosis.

Rosenstein BJ, Langbaum TS.

A variety of intestinal complications, including constipation,

abdominal pain, palpable cecal masses, intestinal obstruction,

intussusception, and volvulus, have been observed beyond the

neonatal period in patients with cystic fibrosis (CF). In a

retrospective chart review of 63 patients with CF, we found evidence

of one or more of these complications in 26 patients (41.3%). The

incidence of intestinal complications was not related to overall

disease severity, pulmonary exacerbations, history of meconium ileus

at birth, or dose or type of pancreatic enzyme replacement. There

was no change in the incidence of intestinal complications after

patients switched to a pH-sensitive enteric-coated microsphere

enzyme preparation.

PMID: 6553601 [PubMed - indexed for MEDLINE]

Distal Intestinal Obstruction Syndrome

by Rock, M.D.

Distal intestinal obstruction syndrome (DIOS) was previously called

meconium ileus equivalent. What is DIOS? This is a condition that is

unique to CF patients in which there are episodes of thick stool

that obstructs the distal part of the small bowel (the distal part

of the small bowel is the last portion of the small bowel just

before it connects to the large bowel). For most CF patients

(approximately 90%), pancreatic insufficiency exists resulting in

malabsorption of fat in the stool. This malabsorption can be

controlled well with pancreatic enzymes. However, in addition to fat

malabsorption, there are also abnormal intestinal mucins, abnormal

intestinal water and electrolyte transport and perhaps a prolonged

transit time from the mouth to the beginning of the small bowel. All

of these factors may contribute to the development of DIOS.

Patients with DIOS clinically have the complaint of crampy abdominal

pain, often in the right lower quadrant, abdominal distention and

often loss of appetite. On physical exam, the physician may often be

able to feel a mass of stool in the right lower quadrant of the

abdomen. These findings alone may be enough to establish the

diagnosis. If the diagnosis is unclear, then the most useful

laboratory test is an x-ray of the abdomen. In DIOS, the x-ray shows

an abundance of stool in the intestine (particularly bubbly/granular

material in the right lower side) and the presence of air fluid

levels with a variable degree of small bowel dilatation.

The treatment for DIOS is the administration of large volumes of

balanced polyethylene glycol electrolyte solution. (Colyte or

Golytely; these solutions were originally developed to clean out the

bowel prior to endoscopy procedures or abdominal surgery.) This may

be given orally if the patient can drink large quantities (two to

six liters); otherwise, it is given by nasogastric tube.The endpoint

of treatment occurs by the passage of stool, disappearance of

symptoms, and disappearance of the mass that could be felt in the

right lower quadrant. If the polyethylene glycol-electrolyte

solution is not effective, then other methods of clearing out the

bowel include enemas. The radiologist can give a gastrograffin enema

in the x-ray suite. Gastrograffin is a hyperosmolar solution,

meaning that it draws water into the bowel which results in

liquefying and loosening the stool. For this procedure to be

effective, the gastrograffin must reach the small bowel and area of

stool that is causing the obstruction. Because use of such

hyperosmolar contrast agents results in large shifts of fluids and

electrolytes, there must be close monitoring of the electrolytes in

the blood. (The oral polyethylene glycol-electrolyte solution has

the advantage of not adversely affecting the electrolytes in the

blood.) Gastrograffin enemas have clearly become a back-up choice in

treating DIOS. Lastly, in rare cases, neither of the above maneuvers

relieves the obstruction and surgery is necessary.

Because recurrences of DIOS are common, the following maneuvers are

done. First, patients should be sure to always take their

recommended enzymes with meals and snacks. The physician might

prescribe other products (such as TUMS, or acid blockers like

omeprazole or ranitidine) to maximize the effect of the enzymes.

Patients should drink plenty of fluids in order to avoid dehydration

and thus thicker stools. For many patients, physicians will

prescribe laxative agents or stool softeners.

Distal intestinal obstructive syndrome home

Jan, 2001.Distal intestinal obstructive syndrome [online]. Seacroft

and St 's University Hospitals, UK. Available from

http://www.cysticfibrosismedicine.com

Acute abdominal pain, distension and vomiting may result from

intestinal obstruction due to impaction of undigested bowel

contents. This is often referred to as distal intestinal obstructive

syndrome (DIOS) or meconium ileus equivalent (MIE). The condition

may result from inadequate or excessive dosing of pancreatic

enzymes. The problem is much less frequent in patients whose

absorption is monitored regularly and whose enzyme doses are

adequate, although such patients may also be affected. Initial

treatment with fluid replacement (if necessary intravenously) and

oral gastrografin diluted with fruit juice or water usually relieves

the obstruction (O'Halloran et al, 1986). Treatment with a balanced

electrolyte solution (Klean-Prep) has proved effective for those not

responding to gastrografin (Cleghorn et al, 1986; Koletzko et al,

1989). Some patients may require nasogastric administration of this

balanced electrolyte solution to achieve an adequate intake. After

the acute episode has resolved the pancreatic enzyme dose should be

reviewed and altered as necessary. Where the problem is recurrent,

despite these measures, the regular use of Cisapride has been

helpful in some (Koletzko et al, 1990). Investigation of patients

who are prone to attacks of DIOS should include serum amylase and

abdominal X-rays. If the condition is not responding to medical

treatment, a contrast enema should be performed. Ultrasound may be

helpful in identifying the obstructing masses, but cannot be relied

upon to exclude other serious causes of pain and obstruction such as

intussusception. If gastro-intestinal investigations are negative,

enzyme therapy is optimal and significant malabsorption persists,

addition of ranitidine, omeprazole or lansoprazole to reduce gastric

acid secretion (Heijerman et al, 1990; Heijerman et al, 1993) or

taurine ( et al, 1994) may improve absorption.

Distal Intestinal Obstruction Syndrome

Distal Intestinal Obstruction Syndrome (DIOS) or Meconium Ileus

Equivalent is characterised by repeated episodes of partial or

complete intestinal obstruction in later life. The incidence has

vbeen reported to range from 2.1-47% (21,22) of CF patients and is

probably related to inadequate doses of pancreatic enzymes

particularly as the individual's growth leads to a relative decrease

in the number of enzyme capsules/Kg body weight (22). It may present

with a right iliac fossa mass and can be difficult to distinguish

from appendicitis, intussusception, volvulus, Crohns disease,

fistulae, neoplasm's or ovarian conditions.

Interestingly, the incidence of DIOS is increased after lung

transplantation. Minkes et al identified a 10% incidence of

obstruction with in a short time of transplantation(23).

They cited a number of possible factors including -

1) Prolonged bed rest

2) Use of azathioprine (impairs motility)

3) History of previous abdominal symptoms

4) Conditions surrounding surgery e.g.. Dehydration, General

anaesthesia, analgesics

DIOS can normally be managed conservatively. Treatment involves

rehydration and the use of oral gastrograffin or N-acetylcysteine.

However, in the above series of 70 lung transplant patients the 7

who developed DIOS were managed surgically. Five patients had

preoperative hypaque enemas which did not relieve the obstruction

and it was felt that all of the patients were too ill to safely

undergo further attempts at non-operative management. The authors

suggest a pre-transplant regimen of bowel washouts and if possible

peri-operative prophylactic N-acetylcysteine in order to prevent

post operative DIOS. A high index of suspicion should be maintained

at all times in this population of patients.

PS: Give Ashton an extra hug from us... We're thinking of him!

[Guest guest]

Cody has suffered from this for a long. long time, since birth (had MI).

The only way we kept it under control was to give him Mucomyst 20%

(acetylcysteine solution) in his g-tube 2-3 times daily, and when he would

begin to have a blockage, which we could tell if he began having decreased

stools or an upset stomach, we would increase it to up to every two hours.

Currently he is on 15 cc/twice daily, and I see this as something he will

always be on. We tried going off of it, and within a few weeks, he had a

really bad blockage. Many of them required hospitalization when he was

little, especially if he got ill with a cold or flu of any sort, as he would

get them much worse when he got dehydrated, and he dehydrates VERY quickly.

Now, though, he hasn't had one for about 3 years--well, one very mild one,

but we got that under control very quickly--so it is finally under solid

control.

A couple of times he had to have a gastrographin enema, but I hope THAT

never is necessary again! YUK!!

Best of luck, and if you have any more questions, let me know.

S., mom to Cody (7, pwcf), DJ (9, nocf), and a (14, nocf)

DIOS

> Has anyone been diagnosed with DIOS? Distal Intestinal Obstruction

> Syndrome. My 28 month old son, Ashton, has been vomiting half of

> feedings (all g-tube and liquid luckily) over the past 3 weeks. It

> has been a messy time in our house--thank goodness for hardwood

> floors! The vomit and his stools were very mucuosy and gross. We

> finally got to see his GI doctor today and he thinks it is DIOS.

> Ashton did have a meconium ileus at birth (caused him to be

> premature) that required surgery. He also has a g-tube. He didn't

> have the distended belly that I see is a normal symptom of DIOS. The

> doctor has prescribed Miralax (a laxative we have used before) for 4

> days to totally clean him out. He will have diarhea for several days

> (ugh!). I am just wondering if anyone else out there has had this

> and what they did to treat it and if vomiting was one of the

> symptoms??

>

> mom to Ashton 28 months wcf