Let me just state that I am not a doctor or nurse. I have no degree in medicine at all. We have a child who was born with Long-segment HD and we have been through ALOT in 6 short years with this. I am doing this to help provide support and information for others out there. These are things we have been told and have gone through since he was born.
FACTS ABOUT HIRSCHSPRUNG'S DISEASE
It occurs in 1 in 5000 live births
There are nerves in your intestines that have ganglion cells in them to help move everything along the way out. With HD while the whole digestive system is developing these nerves will stop developing at a certain point in the intestines. Most cases happen in the large intestines, rare cases will affect both small and large intestine. Even more rare, about 2%, will have spotted HD. ( We know this first hand because our son not only had it affect both intestines but also had spotted HD in his small intestine)
If this affects both small and large intestine it is called Total Colonic HD. Have also heard it called Short Gut Syndrome.
It occurs more in boys then girls. This does not mean girls can not get it.
It is a genetic disease. (This is what our surgeons have told us)
If you have one child with HD, there is a 3%-12% chance that another baby will have it
This is a disease that a baby is born with. It is not a disease that will show up later in life.
80% of babies will show signs in the first 6 weeks.
Most babies will show signs in the first 24 hours. The biggest sign is not having a bowel movement within 24-48 hours.
Other signs is yellow-green vomit, along with swelling of the abdomen.
Once signs have shown the doctors may do one of the following to determine if it is HD:
abdominal xrays
contrast enema-a contrast is given into the rectum to coat the inside of the organs so that it will show up on xray.
rectal biopsy
Treatment of HD in all cases is surgery. The segment of intestine that is bad will be removed. In most "easy" cases the intestine will then be pulled down to the anus. In other cases they will create an ostomy. The ileostomy is where they pull the small intestine through the abdomen to create a stoma. A colostomy is where they pull a part of the large intestine through the abdomen. You will then use a bag around it to collect the poop.
*for our son we actually use a urostomy bag because his poop is not formed enough and it is more liquid. A urosotmy bag is easier for us to empty. It is a simple valve you turn to either open or close the bag. It has been wonderful. We also use and overnight bag connected to the urostomy bag for it to drain overnight. Otherwise his bag would leak and was needing to be changed daily.
FACTS ABOUT HIRSCHSPRUNG'S DISEASE
It occurs in 1 in 5000 live births
There are nerves in your intestines that have ganglion cells in them to help move everything along the way out. With HD while the whole digestive system is developing these nerves will stop developing at a certain point in the intestines. Most cases happen in the large intestines, rare cases will affect both small and large intestine. Even more rare, about 2%, will have spotted HD. ( We know this first hand because our son not only had it affect both intestines but also had spotted HD in his small intestine)
If this affects both small and large intestine it is called Total Colonic HD. Have also heard it called Short Gut Syndrome.
It occurs more in boys then girls. This does not mean girls can not get it.
It is a genetic disease. (This is what our surgeons have told us)
If you have one child with HD, there is a 3%-12% chance that another baby will have it
This is a disease that a baby is born with. It is not a disease that will show up later in life.
80% of babies will show signs in the first 6 weeks.
Most babies will show signs in the first 24 hours. The biggest sign is not having a bowel movement within 24-48 hours.
Other signs is yellow-green vomit, along with swelling of the abdomen.
Once signs have shown the doctors may do one of the following to determine if it is HD:
abdominal xrays
contrast enema-a contrast is given into the rectum to coat the inside of the organs so that it will show up on xray.
rectal biopsy
Treatment of HD in all cases is surgery. The segment of intestine that is bad will be removed. In most "easy" cases the intestine will then be pulled down to the anus. In other cases they will create an ostomy. The ileostomy is where they pull the small intestine through the abdomen to create a stoma. A colostomy is where they pull a part of the large intestine through the abdomen. You will then use a bag around it to collect the poop.
*for our son we actually use a urostomy bag because his poop is not formed enough and it is more liquid. A urosotmy bag is easier for us to empty. It is a simple valve you turn to either open or close the bag. It has been wonderful. We also use and overnight bag connected to the urostomy bag for it to drain overnight. Otherwise his bag would leak and was needing to be changed daily.