Baby with Chronic Intestinal Pseudo Obstruction

My name is Lucy, and my little girl is 11 months old and has suspected Chronic Intestinal Pseudo Obstruction, present since birth.

Ruby will have to have a full thickness biopsy soon (she has had 2 other types of biopsies both with mixed results) and they say they will do an illeostomy then at the same time. They will do this if/ when she has another blockage. She had her last blockage in december, so this is the longest time she has gone without blocking up and we have been home for 2 months. Ruby was put on tpn at birth, but after clearing the initial blockage she managed to start taking formula. She was ok for a while but then the blockages began, about one every 1 to 2 weeks - it would take up to 6 days to clear her out, she would be on a drip, and look like a zombie with no life in her. We would have about 3 days in between when she would start to look like herself. This has repeated itself about 20 to 25 times. It wasnt until she was 7 months old that she even got a gastro doctor (the kept insisting it was extreme constipation even though i told them her stools were never hard). She is on a milk free diet, just in case. But the doctors are baffled by her because she is now eating well and drinking her formula and has not blovked for 2 months, when shes not getting blocked she puts on weight, but when she has an episode she loses a lot.

They are baffled by the fact that she eats well, has an appetite and responds well to enemas. Tests have ruled out Hirschsprungs and others. She is now on a bowel decontamination regime, hopefully this will help with malnutrition. Does anyone else have experience of anything like this. Can anyone reccommend anything, because if i am honest i dont think our specialists have come across this condition before. This is the first kind of support i have come across, i have never met anyone that has even heard of this condition. I really hope to find people who i can talk to about this, because people really dont understand what its like to see your child suffer like this.

By JenD
Posted April 4, 2008 at 8:06 am
Report post

I'm so sorry to hear about your little one. It is good that she does well sometimes and that you have a GI doc for her. I have gastroparesis and small bowel dysmotility. I really benefit from taking Miralax and other laxatives on a regular basis. I wonder if they could just start her on a daily laxative to help. I take Miralax everyday and without it I get stopped up pretty quickly. I don't know if babies can take Miralax and it will make her stools very soft and messy, but it might keep her from getting stopped up.

It really sounds like Chronic Intestinal Pseudoobstruction (CIPO) which the GI doc should be familiar with. From what I understand a lot of people with CIPO also have episodes like you describe where they are better for a while and then get a bad spell for a while.

I sure hope that things improve for her. Hang in there and know that her condition is not out of the ordinary for motility disorders. Let me know if there is anything I can do to help. Answer questions etc...

Take care,
Jen
http://www.caringbridge.org/visit/jenelle

By Peggypeg
Posted April 5, 2008 at 5:19 am
Report post

Hi!

So sorry to hear of the situation. Really hope things can be sorted so you get the diagnosis soon.

here to chat any time.

Chris
www.ypeggypeg.blogspot.com

By robynr57
Posted June 8, 2008 at 11:50 pm
Report post

Lucy

I am from NSW in australia and there is a professor of surgery at royal Childrens Hospital Melbourne Victoria australia who has diagnosed and pioneered treamtent in a bowel motility diseases not unlike hirsprungs disease. It is called Neuronal Intestinal Dysplasia (NID). his name is Professor John Hutson and is a very approachavle man. My daughter was finally diagnosed by biospy with a mild form of the disease and thnakfully we have not had to go down the surgical tract. Google Neuronal Intestinal Dysplasia NID and I thnk there is a support group and website.
Hope this helps

By td63076
Posted October 7, 2008 at 9:05 am
Report post

I know what you are going through. I have a child that was born with a birth defect,after having his meconium(first stool) weeks after delivery. The doctors were saying that it was only constipation up until Nov. 2007 when my child had a colostomy. He suffered with his stool being able to fuction properly and this chronic illness continues. The doctors here located in Tennessee don't know the next step to take. We are still getting test results for my little man, hoping that the colostomy bag can be removed and everything else reversed. He has episodes where he cant eat, because he feels full. Some days he's just full of gas and others he may eat. His weight fluctuates due to the intake/output. He's six years old now and we still have some surgical procedures to endure. Mom,I want to encourage you to continue to believe, research on this diagnosis yourself. Us, parents have to be the primary advocate for our children, because alot of diagnosis that are out there; doctors are verily new to their existance. I will be glad to discuss this further with you. Be strong and encouraged. You;re not alone. td063076 PS If anyone can help me contact the NidKids network, please let me know

By jessy7743124
Posted November 2, 2008 at 11:54 am
Report post

Hi, I saw your post while I was doing research for my own daughter. She is now 19 months old and she was diagnosed with chronic intestinal pseudo obstruction when she was about 6 months old. She kind of did the same thing your daughter does. For the first 3 months she did ok with eating, but her belly was always distended. Then she had an episode where she got blocked up and was vomiting so they admitted her for a few days, decompressed her, and waited for her to resume eating. We were discharged and she did ok for another 2 months. Her belly was still very distended and she wasn't putting on a lot of weight. Then when she was 5 1/2 months she had another bad episode of vomiting. This time she was in the hospital for 2 months. By the end she had a central broviac line put in so she could be on TPN, an ileostomy, and a G- J tube and diagnosed with CIPO. Over the next months we were in and out of the hospital a lot due to infections, her getting backed up, and issues with her ileostomy and prolapse.

When I first heard about the diagnosis, I was told by a GI doctor that it is very uncommon and there was not a cure, but over the past year I have learned that there are quite a few people out there with this disease, and while there is no cure, there are ways to manage it. Every person with this disease is different and can have different severities of it.

My daughter is now 19 months old and is very happy. She is developmentally delayed due to low muscle tone and being in the hospital so much, but she's catching up. I would love to tell you some of the things that have worked for her, feel free to contact me Jessy7743124@hotmail.com.

By MadelynsMom
Posted November 9, 2008 at 11:19 am
Report post

Hi,
I was wondering if your daughter was on any motility drugs? How many hours of TPN per day and if she gets any feeds thru her G-J tube or by mouth? Do you see a peds GI motility specialist? If so, who, and do you really like them alot?

By Loie
Posted December 19, 2008 at 11:01 am
Report post

Lucy,
I came across your story by accident. I am a 55 year old woman that was born with Idiopathic Chronic Intestinal Pseudo-obstruction. I do not have a child that has the disease. I am very grateful for that! I was diagnosed in 1985 when there were only 100 other cases known.
The symptoms sound the same as I have experienced all of my life...ups and downs. We never know from one day to the next when the shoe will drop.
Even though the drs. say this is still a very rare disease, there is a lot more awareness now and more treatments.
I guess the reason I feel compelled to respond to your outcry is to let you know that I am 55 years old now! In 1985 I was sent home with the only med they knew about at that time (Urecholine) (sp). The dr. told my husband I would be in a wheelchair by the end of the year and die soon after that. NOT SO!
I suggest you get a very knowledgable dr. that specializes with not only motility disorders, but pseudo-obstruction. That is very important. I have come across many GI's that are familiar with motility disorders...but not Intestinal Pseudo-obstruction. The specialists, besides being more knowledgable, are more understand and compassionate as to all that is effected by the disease.
I am sorry that I can't encourage you as a mother, but as a patient/surviror, I hope I helped in even the slightest manner.
If you wish, feel free to contact me. My email address is loies7777@bellsouth.net.
I will be keeping you in my thoughts and prayers,
Lois

By MadelynsMom
Posted December 21, 2008 at 10:41 am
Report post

Thank you so much for your post. Each time I am able to see an adult with CIPO and hear their story it gives me a great feeling of hope! You are an inspiration for us to look to when things are not going well. Thank you so much.

By Mom2Tierney
Posted February 3, 2009 at 11:04 pm
Report post

My daughter was diagnosed with CIPO when she turned 17. This illness is very unpredictable and sporadic in nature - Tierney has had periods of no blockage, sometimes as long as six months, however, she has also had periods of monthly, even weekly, blockage (this illness has no rhyme or reason to it and just when you think you have figured it out, it will throw you for a loop). CIPO is difficult to diagnose as its symptoms are similar to other gastrointestinal disorders, therefore, it can take awhile for diagnostic confirmation (motility testing can confirm diagnosis). If the doctors are unfamiliar with CIPO, you will need to do your research and locate a physician that knows more; I know that there are only a handful of pediatric specialists, for CIPO, in the United States: Dr. Carlo DiLorenzo is one of the top specialists (Nationwide Children's Hospital). Also, if your daughter is a total parenteral nutrition (TPN) patient, you may want to do some research on Omega-6 versus Omega-3 fatty acids due to the fact Omega-6 fatty acids cause liver damage in infants but please do not be frightened by this because Dr. Mark Puder substitutes Omegaven, an Omega-3 fatty acid which prevents fat accumulation and liver injury, with the current Omega-6 fatty acid (http://www.childrenshospital.org/newsroom/Site1339/mainpageS1339P1sublevel2 22.html). Much of what you read on the internet, regarding CIPO, can be overwhelming but know that there is hope in research and medical advances are made every day that can improve patient's lives.

By Mom2Tierney
Posted September 13, 2009 at 5:25 am
Report post

I just wanted to let you know that my beautiful daughter, Tierney, passed away this past March 21, 2009. She did not suffer and as much as I miss her, she is finally at peace. My heart goes out to all who deal with gastrointestinal issues, especially CIP, as it is very difficult to endure the suffering created by this wretched illness. Tierney also suffered from congenital heart defects (holes) and, in the end, that is what took her. If you have been diagnosed with CIP, do not take any other symptoms for granted in the least! Pay extra close attention to any other abnormalities (e.g., Tierney suffered from low blood pressure) and whenever her electrolytes were the slightest bit off, her heart would act up. Also, trust your instincts; if you feel something is wrong, do not take no for an answer and look for a physician who will listen. I cannot stress enough, for those with CIP, how important it is to find a TRUE CIP specialist as they can perform more precise testing to differentiate what form of CIP (muscle or nerve) affects you. Tierney's Godsend, from distention, was her G-tube, therefore, the problems we dealt with (from that point on) were, to a great degree, central line infections. Do not allow the physician to remove you from TPN unless you have another form of nutrition in place as I think removing Tierney from TPN, with no backup in place, was part of her undoing. Please know, I would love to assist anyone with any questions or concerns they might have. I certainly do not claim to know it all, however, I have been though a lot with Tierney, therefore, if I can assist even one patient with a better quality of life, my battles, and Tierney's, will have been worth it. Remember: every patient is different, therefore, what works for one may not work for you. God bless

Welcome to Inspire!

Explore topics in this discussion:

11 replies

Add to the discussion

AGMD on Twitter

Group leaders

You