May 24, 2011 -- GI Appointment

This is the appointment that I figured would go well but unfortunately I was wrong. We met with her new GI Doctor, Dr. Masacarehnas. She is very thorough but was full of heartbreaking news! She told us that Bennetts old GI Doctor possibly misread her Pancreatic Stimulation Test. July 2010 Bennett had a Pancreatic Stimulation Test done too see if she was Sufficient or Insufficient. She was taking Creon 12000 since birth and when her CF doctor referred us to her old GI doctor he was astounded by the amount of Creon she was taking for being only 4 years old so he suggested we do this test. Well the results he told us were that she was Pancreatic SUFFICIENT and no longer needed to take the enzymes to stop them immediately. He followed her care for almost a year. From April 2010 to April 2011 she needed to have FOUR NG Tube Cleanouts for bowel/intestinal blockages. Her new doctor Dr. M diagnosed her with DIOS (distal intestinal obstruction syndrome). Yes, we have yet another "disease" under out belts. Lovely huh? Dr. M is having me do a daily diet log & wants a stool sample to run tests on her. Once these stool test results are back we will move onto the next phase of testing. Dr. M believes that Bennett infact isn't Pancreatic Sufficient. She told us by reading her old records from Geisinger that she is Pancreatic Insufficient and perhaps needs to be put back on the enzymes. At this point in the appointment I am in tears because my babygirl has yet another new diagnosis. It is killing me inside. My baby does NOT deserve this.

Unfortunately, I have NOT been able to do the daily diet log because Bennetts appetite has diminished. She hasn't been eating enough to keep a bird alive and her belly is very swollen and hard. Which indicates she is obstructed again. Her last good bowel movement was now 5 days ago. She has taken TWO Miralax cleanouts of 255 grams of Miralax mixed in 64 ounces of juice and has one passed liquid. The liquid is stretching out around the hard blockage. It has been 6 weeks to the day since our last NG admission. This is truly unfair. She takes Miralax 6 times a day, Benefiber 3.5 grams 3 times a day, 2 tablespoons of Milk Of Magnesia, and 2 tablespoons of Fletchers Castoria a day and still has problems. We have tried to change her diet, given stool softeners and everything but with her having DIOS, there isn't much we can do besides surgery and NG cleanouts every 6 weeks for the rest of her life!


Below is a link that has a better explanation of what DIOS (Distal Intestinal Obstruction Syndrome) is:

Distal intestinal obstruction syndrome (DIOS) involves blockage of the intestines by thickened stool and occurs in individuals with cystic fibrosis. DIOS was previously known as meconium ileus equivalent, a name which highlights its similarity to the intestinal obstruction seen in newborn infants with cystic fibrosis. DIOS tends to occur in older individuals with pancreatic insufficiency. Individuals with DIOS may be predisposed to constipation.

Symptoms of DIOS include crampy abdominal pain, vomiting, and a palpable mass in the abdomen. X-rays of the abdomen may reveal stool in the colon and air-fluid levels in the small bowel. Surgery is often required to relieve the obstruction. However, if there is no sign of bowel rupture, a more conservative approach may be attempted. Restricting oral intake, placement of a nasogastric tube for decompression of the stomach and proximal intestines, and administration of laxatives and enemas may resolve the obstruction without the need for surgery. Individuals prone to DIOS tend to be at risk for repeated episodes and often require maintenance therapy with pancreatic enzyme replacement and stool softeners.

http://www.cfeducation.ca/pdf/DIOS_Children.pdf