Managing Expectations After Surgery for Hirschsprung Disease

Hirschsprung disease is typically diagnosed within the first 24-48 hours of a newborn’s life, and naturally, parents can be a little overwhelmed upon receiving the diagnosis. It’s not a condition that is spoken about openly and like with many conditions diagnosed around birth, mothers often question if they did something to cause it.

While we don’t know exactly what causes the condition, we do know that mothers have done nothing wrong during pregnancy and that in some cases it may be associated with a genetic mutation.

Hirschsprung disease is a condition in which some of the nerve cells do not form properly in the intestine during fetal development, causing stool to back up and potentially leading to bowel obstruction and a bacterial infection in the digestive tract. All babies born with Hirschsprung disease will need surgery to remove the portion of the intestine that is not working properly.

This can be a scary time and parents typically have a lot of questions upon receiving the diagnosis. One of the most commonly asked questions we receive from parents is if surgery is absolutely necessary and then whether or not their child will be “cured” after it. This is also a common misconception.

Many parents have been led to believe that the surgery, called a pull-through procedure, will completely “fix” the problem and their child will not have any bowel issues post-surgery.

The reality is that even after surgery, the majority of children will still have some constipation issues. This is why it is incredibly important to keep in close contact with your colorectal surgeon. If she is aware of any constipation issues after surgery, she can recommend a regimen of laxatives and/or stool softeners – called a bowel management program – which can help your child to achieve “normal” functioning and a good quality of life. We consider “normal” bowel functioning to be 1-3 bowel movements each day with no accidents. All children with Hirschsprung disease should be able to reach this goal with the proper post-operative management.

In other words, a diagnosis of Hirschsprung disease does not mean that your child will have life-long, life-altering issues, so long as your child is followed closely by a colorectal team familiar with the condition and who partners with families to manage issues as they arise. It is a condition that may change periodically through your child’s life and your child’s regimen may need to be tweaked along the way.

But a good partnership between the team and your family can help resolve these issues fairly seamlessly.

This is why I think it is incredibly important for your child’s colorectal team to manage your expectations accordingly before the surgery. Surgery to correct Hirschsprung disease is an absolute necessity, but it isn’t a cure-all. Your child may have new issues that arise periodically, but they can be controlled with the proper bowel management regimen.

If you don’t feel like your child is where he or she should be, don’t be afraid to ask for a second opinion. We have trained several pediatric surgeons and collaborated with up and coming colorectal centers regarding surgical techniques for Hirschsprung disease as well as bowel management post-surgery. Ask your surgeon where he learned this procedure, how many he has performed it, and post-operative success rates. If you run into any road blocks, our team here is always happy to help consult and/or evaluate your child.

If you have questions, or would like to request an appointment, please contact our Colorectal Center.

Editor’s note: Lyndsey Jackson, RN, BSN, CPN, and Dr. Jason Frischer hosted a live, online Q&A for parents of infants and children born with Hirschsprung disease on June 10, 2015. Here is a link to a recording of it

Lyndsey Jackson, RN, BSN, CPN

About the Author: Lyndsey Jackson, RN, BSN, CPN

Lyndsey Jackson, RN, BSN, CPN, has been a pediatric nurse at Cincinnati Children’s for 11 years, and has spent the last eight of those years with the Colorectal Center. She enjoys her role of patient care coordination and the long-term connection she is able to build with patients and families who have colorectal issues, working to ensure their highest quality of life possible.

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Comments

  1. Barb Heidy May 22, 07:40
    My son is now 13 yrs old. He is very aware of his condition and how important he lets me know if he ever has any problems going to the bathroom. We have been very blessed with the outcome at this point. If I feel there is a problem espically with projectial vommiting I take him to the local ER and request an xray due to his condition and fear of obstruction they never say no. We make sure he has alot of vegtables in his diet and fiber. We have had no issues for at least 6 years. Is there any info on adults still taking care of this and is there a major risk for his children if he wishes to have any?
    • Lyndsey Jackson, RN, BSN, CPN
      Lyndsey Jackson, RN, BSN, CPN Author May 29, 08:52
      Hi Barb, I think you might find participating in the live event (June 10 from 7-8 pm) helpful for answering some of your questions. https://liveevent.cincinnatichildrensblog.org/events/hirschsprung As always, if you would like to seek care for your son for some of his recent concerns, we would be happy to see him. Our phone number is 844-803-6419. Best,
  2. Kim May 22, 08:48
    This article is somewhat misleading. Really only talks about having one surgery. In lots of cases, depending on the severity of Hirschsprungs, it may take several surgeries. Also doesn't talk about the potential for have a temporary or permanent ostomy. This is done fairly often. With my Grandson, he was about 1 1/2 weeks old when he had his first surgery. He had no normal large intestines so doctors removed them all. They were able to save the rectum and a very small segment of the large intestines attached to the rectum, called a stump. When he was just 1, doctors thought they could do the pull through surgery and he'd manage great. First though, we had to do the rectal dilations on him daily. This was mortifying to all involved!! Surgery went well. They were able to reattach the small intestine to the rectum. My grandson immediately started "dumping" and struggled to gain weight. We tried multiple medications and diets with no luck. He was a frequent visitor to the hospital, mostly do to dehydration or bowel obstructions. His poor, poor little bottom was so raw all the time because of having such frequent stools. No medications or creams would help either. He was miserable all the time. Diaper changes and bathes were always so painful that he screamed and cried. He had a miserable first 3 years. Finally our doctor, who we loved and who has been there from day one, send him over for a bowel study. Needless to say, study showed he didn't have any normal bowel. Stated his bowels never rest. They recommend for him to have his ostomy back again. Not the news we wanted to hear, but we felt it was coming. So, November 2014, he went in for yet another surgery. This had been the best thing for him. He is now thriving and happier than he's ever been. Yes the bag is an inconvenience, but it's worth it to see him happy. He now plays and laughs so much and that warms my heart :) He is 4 now and can't manage the bag yet by himself but has adjusted so well to it. Even he knows what a difference it made in him. Anyway, wanted to share our experience with Hirschsprungs with you. No offense to the article, but this story depicts the reality of having a child with Hirschsprungs.
    • Lyndsey Jackson, RN, BSN, CPN
      Lyndsey Jackson, RN, BSN, CPN Author May 26, 16:03
      Hi Kim, I apologize for the misleading nature of the blog post. This was not my intention; it was my hope to answer one of the most common questions we are asked related to Hirschsprung disease -- will their child be cured after surgery -- and to help manage expectations post surgery. You are absolutely right, many patients have a difficult time following surgery for Hirschsprung disease and there are many variables and variations. We hope that folks will join us for the live Q&A where we will address avoiding and treating those variables: https://liveevent.cincinnatichildrensblog.org/events/hirschsprung Related to your grandson, we would be happy to see your grandson to determine if and when it would be most appropriate to close his stoma and what his care would look like after stoma closure. Our phone number is 844-803-6419.
  3. LaTosha McGuire May 22, 12:41
    Thanks for sharing this. My daughter is 8 now and we have major issues with her health pertaining to this Hirschsprung's disease. Enterocolitis and c diff is often the problems which I was told she would only have to worry about those right after surgery, surgery was 7 years ago ( pull through). Will this get better for her?
    • Lyndsey Jackson, RN, BSN, CPN
      Lyndsey Jackson, RN, BSN, CPN Author May 26, 15:44
      Hi LaTosha, Certainly every child is different and there are a lot of variables with Hirschsprung disease. However, in general, a patient should not be having enterocolitis and c diff issues at eight years of age. I would recommend that your daughter be evaluated by someone who is experienced in managing patients with Hirschsprung disease who are not doing well after surgery. We would be happy to see your daughter here; a significant portion of our patients with HD had their operations elsewhere and were not having an ideal outcome. Our phone number is 844-803-6419 if you would like to see how we might be able to help your daughter.
  4. Blessy July 17, 03:11
    I newly hear about this disease. This article gives an awareness about Hirschsprung disease as well as provided the remedy tips. It's really useful. Thank you
  5. Kathie December 04, 09:32
    I had Hirschprungs as a baby, as did my brother. My son was born with it. I have never been constipated a day in my life, on the contrary I never have solid stool and have had accidents my whole life to one degree or another. My son is the same way. He is 10 and has been having accidents at night and during the day at school. He is having abdominal pain. His doctor has sent him for stool samples, x-rays and is now having us do enimas for every other day for 2 weeks, and now 5 days a week for 2 weeks. His accidents at night have stopped (how can he have one when he has water diarrhea every night from the enimas) but he still has some small accidents during the day and painful tummy. I am so frustrated. I am tired of hearing that he is "constipated" and the accidents are leakage. He has NEVER had a solid stool. They are all loose or water. He also formed a pouch on his colon about 4 years ago, and at the time they said he would have to have it fixed, but now he is just pushing the enimas. Help! By the way, he is not my sons original surgeon, he is a pediatric tummy doc.
    • Lyndsey Jackson, RN, BSN, CPN
      Lyndsey Jackson, RN, BSN, CPN Author December 15, 08:29
      Kathie, I’m so sorry to hear about your struggles with Hirschsprungs; we would be happy to help all family members. Many patients need a bowel management regimen after surgery for Hirschsprung Disease. However, it is important to first determine the reason for the soiling. Our typical approach is to order a contrast enema so that we can see the anatomy of the colon and to perform an exam under anesthesia so that we can check the area of surgery and be sure that there isn’t a stricture or narrowing, determine that the sphincters or muscles are intact and then we can discuss what the child needs in order to be continent of stool. If you are interested in having your son seen here, we will need some demographic information and to obtain medical records for our doctors to review and develop a plan of care for your son. Here is a web page with contact information: http://www.cincinnatichildrens.org/service/c/colorectal/contact/
  6. Bigdaddy517 September 27, 20:13
    Hello r my youngest who is now 17 years old had a pull through at 4 days old yes scary and back then hard to find information but doing as docters said with post surgery dilation and watching foods (Kool aid and some juices cause an acidity stool) he is doing find today he is currently 6 ft 10 in tall 350 lbs of x box playing young man.. Remember do as docters suggest don't read horror stories online and things will work out and remember all children are special in there own way not every child is the same. Last thing is if yoyr child has this learn how to spell it and ask your docter for information to share with others. God bless everyone especially the medical personal that knows of this condition.. and it I s NOT the father's fault either it is genetic
  7. MSQ February 09, 17:30
    My son was born with hirschprung in 1973. Our local doctors didn't know what his problem was. They only knew he was going to die if he didn't get a proper diagnosis. They sent him to Childrens Hospital in Pittsburgh. Thank god they did. The doctors looked at the xrays the doctors sent with him and immediately knew his problem. These doctors in Pittsburgh told informed us our son had only 24 hrs to live without the surgery. Our son had a colostomy for one year. Our son was lucky he only had a small portion of bowl that needed removed. He is now a healthy grown man with a son of his own. He is a nurse in Philadelphia and we are so happy he is with us. At the time he was diagnosed no one here ever heard of the problem. We even had to instruct our local community nurses as to how to treat him and get him ready for the pull through surgery. It was a learning experience for us all. I cannot thank the doctors in Pittsburgh enough for saving our son. They are angels from heaven.
  8. Evans mom April 25, 16:20
    My son was diagnosed at about 1 week old, surgeon performed the pull through method at almost a month old. We have been through alot in the last 5 years trying to get him "regulated" and trying to figure it out. Potty training wasnt easy at all. We are still learning. He is on a Miralax regiment. If you have any advice on how to help him please help. He still continues to have accident in his pants. Things havent been easy. Im hoping we get into a groove and by 6 or 1st grade we will be good.
    • Rachel Camper
      Rachel Camper April 26, 06:55
      Hi Evans Mom, If you haven't already, you might want to check out this webpage about our Bowel Management Program: https://www.cincinnatichildrens.org/service/c/colorectal/treatments/bowel-management To learn further about the program, feel free to call 513-636-3240 or fill out this online request form: https://www.cincinnatichildrens.org/service/c/colorectal/contact