Hirschsprung Disease
Ultimately, HD is trial and error and the mantra we now live by is to take one day at a time - Joanna Scott
image by: Hirschsprung's Community
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Dear Malachi…
:
Dear Malachi,
Every morning that I awake I stare at your perfect little face. Peaceful and happy in a deep slumber, I am reminded of our journey thus far. The many ups and downs, the tears and belly laughs, the frustration and love. I know without a doubt that you have been my greatest teacher. When you were born I stood next to your crib in the NICU, all 10.5 pounds of you (which by the way you looked utterly ridiculous next to all the preemies) and felt completely helpless.
Terrified, weak, and unsure of the future, I could barely control my emotions. The doctor stopped making eye contact with me in case it triggered another sobbing episode. I was not sure…
Resources
REACH
Research, Education & Awareness for Children with Hirschsprung Disease... REACH is a non-profit organization committed to improving the lives of children and families affected by Hirschsprung Disease by increasing awareness, promoting education, connecting families and supporting various research around the world.
Diagnosis of Hirschsprung Disease
Although HSCR has been historically considered to be a disease of the neonatal period, a retrospective review of national inpatient databases reported that only 6.5% of patients present within the first week of life with mean and median ages of presentation to be 3 years and 1 year, respectively.
Hirschsprung's Disease: What You Need to Know
The most common symptom of Hirschsprung’s disease is when a baby fails to pass meconium stool (a baby’s first stool) within the first 48 hours of life... Nearly 9 out of every 10 cases of Hirschsprung’s disease are diagnosed in newborns or within the first several months of life.
We are the real village people.
I have lived it with my Children. When Malachi was born with Hirschsprung’s disease I was beside myself. The first month I barely remember one day from the next.
Hirschsprung's Disease
Hirschsprung’s disease is a congenital condition where nerve cells of the myenteric plexus are absent in the distal bowel and rectum. The myenteric plexus, also known as Auerbach’s plexus, forms the enteric nervous system. It is the brain of the gut... The severity of the presentation and the age at diagnosis varies significantly depending on the individual and the extent of the bowel that is affected. It can present with acute intestinal obstruction shortly after birth or more gradually developing symptoms:
Hirschsprung’s disease explained
The earliest reference to a possible Hirschsprung’s disease (pronounced hirsh sproongz) case comes from Europe in 1691, when a Dutch anatomist Fredericus Ruysch described a young girl with abdominal pain and an enlarged colon. Following that, many other cases appeared in the medical literature concerning children with similar symptoms. But it wasn’t until 1888 that Harald Hirschsprung presented his work on two infants, who had died with enlarged colons, and gave the disease its name.
A quick run down of what Hirschsprung's Disease is!
When Rory was 3 days old he was diagnosed with Hirschsprung’s disease. The main symptom of HD in newborns is not being able to go to the toilet after they are born. Rory’s HD diagnose to us was a complete shock and we had never even heard of it before.
Hirschsprung Disease: It’s all about the poop
Simply put, HD happens when the lower intestines do not grow ganglion cells, the cells that are responsible for peristalsis, during fetal development. They simply do not grow.
Hirschsprung Disease: One Mother’s Story of Her Newborn’s Rare Disease
Pregnant for the fourth time, Megan and James Watson felt pretty familiar with the ins and outs of having a baby. But Hirschsprung disease? They’d never heard of it.
My little HD Hero: Living with Hirschsprung's Disease one day at a time
Ultimately, HD is trial and error and the mantra we now live by is to take one day at a time.
What is Hirschsprung Disease?
Constipation is a very common aliment in babies and young children. It is often down to poor diet or can just be idiopathic, meaning there is no reason for the constipation but should only be tempory and will usually sort itself out after a couple of days. However there are some babies/children (1 in 5000 in fact) that suffer with an abnormal but treatable condition called Hirschsprung’s disease and is considered a birth defect.
Dear Malachi…
Every morning that I awake I stare at your perfect little face. Peaceful and happy in a deep slumber, I am reminded of our journey thus far. The many ups and downs, the tears and belly laughs, the frustration and love. I know without a doubt that you have been my greatest teacher.
Roo's Journey
A blog dedicated to my son, Malachi ("Roo"), and his journey with Hirschsprung's disease.
GI Kids
Boys are more commonly affected than girls. HD may affect multiple members in a family. In fact, 10%–33% of individuals with HD also have other family members with HD. The exact causes of HD are unknown, but genetic mutations likely contribute. HD is common in individuals with genetic conditions like Down syndrome, Waardenburg-Shah syndrome, Smith-Lemli-Opitz syndrome, neurofibromatosis, and multiple endocrine neoplasia type 2.
Help For Hirschsprung Disease
I have set up a non-profit organisation called Help for Hirschsprung Disease to help other families who’s children also suffer from HSCR / bowel disease. This website is an avenue to become informed, connect and share stories, hopefully making the experience for others dealing with HSCR a little easier.
Hirschsprung's & Motility Disorders Support Network
The Hirschsprung's & Motility Disorders Support Network (HMDSN), is an organization that makes it possible for of ordinary people and families, who's children suffer from Hirschsprung's Disease and other Gastrointestinal motility disorders to support each other. We want to help parents with children who suffer from these Secret Diseases
Hirschsprung's Community
Hirschsprung's Community serves as a forum for anyone with a family member living with Hirschsprung's disease.
Hirschsprung's Disease Family Support Group
HD develops in children before they are born. It is not caused by anything the mother did while pregnant. And effects 1 out of 5,000 kids.
The Hirschsprung's Disease Foundation
Providing Hope to families affected by Hirschsprung’s Disease.
About Kids GI
The treatment of Hirschsprung’s disease is primarily surgical. The goal of surgery is to remove the abnormal bowel and attach the normal bowel to the anus just above the sphincter. The three commonly performed operations, known as pullthrough procedures, are called the Swenson, Duhamel, and Soave operations. The long-term results appear to be similar whichever operation is used.
NIDDK
The main signs and symptoms of Hirschsprung disease are constipation or intestinal obstruction, usually appearing shortly after birth. Many healthy infants and children have difficulty passing stool or infrequent bowel movements. However, unlike healthy children and infants, kids with Hirschsprung disease typically do not respond to constipation medicines given by mouth.
NORD
Hirschsprung disease (HSCR) is a birth defect. This disorder is characterized by the absence of particular nerve cells (ganglions) in a segment of the bowel in an infant. The absence of ganglion cells causes the muscles in the bowels to lose their ability to move stool through the intestine (peristalsis)
ScienceDirect
HSCR pathology is characterized by the absence of enteric ganglia in a distal segment of the gut, resulting in tonic contraction of the aganglionic segment and intestinal obstruction.
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