When becoming a parent there are many new things to learn, one of which is Hirschsprung Disease which is a rare disease.
Often called Hirschsprung’s disease in babies, this is a rare disease that causes problems with defecation (BAB).
What causes it and how to fix it? Here is a complete explanation,Parents.
What’s thatHirschsprung’s Disease?
Reporting from Mayo Clinic,Hirschsprung’s Diseaseis a congenital or birth condition of the large intestine that causes problems with defecation.
Nerve cells in the large intestine that control muscle contractions to move food are not fully formed.
Without contractions, stool cannot be expelled from the body and remains in the large intestine.
This condition occurs when the nerves in the intestines (enteric nerves) do not form properly during development before birth ( embryonic development ).
This condition is usually identified within the first two months of life , although less severe cases may be diagnosed later in childhood.
Newborns suffering from this disease cannot defecate in the first days of their life and require surgery to cut and remove the affected part of the large intestine.
Types of Disease
There are two main types of Hirschsprung disease determined by the area of the intestine that lacks nerve cells, namely:
Short Segment Disease
In short segment disease, nerve cells are missing only from the last segment of the large intestine (colon).
This type is the most common, occurring in about 80 percent of people with Hirschsprung disease.
For unknown reasons, short segment disease is four times more common in males than in females.
Long Segment Disease
Long segment disease occurs when nerve cells are missing from most of the colon and is a more severe type.
Long segment disease is found in about 20 percent of people with Hirschsprung disease and affects men and women equally.
Very rarely, nerve cells are lost from the entire large intestine and sometimes part of the small intestine (total colonic aganglionosis) or from all of the large and small intestine (total intestinal aganglionosis).
Reason
Until now, it is still not known for certain what causes Hirschsprung’s disease.
In some cases, Hirschsprung Disease is associated with genetic mutations. The disease can also be inherited from parents.
In Hirschsprung’s disease, these ganglion cells are missing from the end of the intestine, extending upward from the anus, the opening at the bottom through which feces pass.
Ganglion cells are intestinal muscles that are controlled by nerve cells.
For some reason, cells do not develop in that area when the baby is growing in the womb .
It’s not clear why this happens, but it’s not thought to be caused by anything the mother did while pregnant.
Risk Factors
If you have had a child with this disease before, you are more likely to have another child with this disease.
Other factors that can increase the risk of Hirschsprung disease are:
- Gender . Hirschsprung’s disease is more common in males.
- Having other inherited conditions . Hirschsprung’s disease is associated with certain inherited conditions, such as Down syndrome and other abnormalities present at birth, such as congenital heart disease .
Symptom
National Health Service explains, symptoms of Hirschsprung Disease are usually seen soon after a baby is born, although sometimes they are not seen until the child is one or two years old.
Usually, the most obvious sign is that the newborn baby is unable to defecate within 24-48 hours after birth.
Signs of the condition in babies include:
- Failure to pass meconium within 48 hours, dark stool passed by a healthy baby soon after birth (although some babies later diagnosed with Hirschsprung disease do pass meconium )
- Distended stomach
- Vomiting green fluid (bile)
- Constipation or gas, which may make a newborn fussy
- Diarrhea
Signs in older babies and children include:
- Bloated stomach and stomach ache
- Persistent constipation that does not improve with usual treatment (chronic)
- Not eating well or not gaining much weight
- Extreme fatigue
Diagnosis
The child’s abdomen will usually be examined and sometimes a rectal examination may be performed.
This is where the doctor or nurse inserts a finger into the back passage (rectum) to feel for abnormalities.
If a child is suspected of having Hirschsprung’s disease, an X-ray may be performed to show blockages and bulges in the intestine.
The diagnosis can be confirmed by performing a rectal biopsy, which involves inserting a small instrument into the child’s bottom to take a small sample of the affected bowel.
This is then examined under a microscope to see if nerve cells are missing.
Frequency of Occurrence
Most cases of Hirschsprung’s Disease tend to occur in boys.
Children who suffer from Hirschsprung’s disease are susceptible to serious intestinal infections called enterocolitis.
If action is not taken immediately, enterocolitis can cause death.
Hirschsprung disease is a rare disease.
Hirschsprung’s disease occurs in about 1 in 5,000 newborns. In Indonesia, there are less than 150 thousand cases per year.
Complications
Children with Hirschsprung disease are susceptible to a serious intestinal infection called enterocolitis.
Enterocolitis can be life-threatening and requires immediate treatment.
Prevention
Based on American Academy of Family Physicians,Because the cause of Hirschsprung’s disease is unknown, parents cannot prevent or avoid it.
However, parents who have this disease can unknowingly transmit it to their children.
However, for children old enough to eat solid foods, a high-fiber diet can relieve and prevent constipation.
Drinking plenty of water is also important, and helps prevent dehydration .
The large intestine helps absorb water from food, so dehydration can be a concern for children who have had part of their intestine removed.
Additionally, encourage your child to do physical activity every day to help facilitate regular bowel movements.
Treatment
Meanwhile, for most people, treatmentHirschsprung’s Diseaseis surgery to cut or remove the part of the large intestine that lacks nerve cells. A
There are two ways this can be done: pull-through surgery or ostomy surgery.
OperationPull-through
In this procedure, the lining of the diseased part of the intestine is stripped away. Then, the normal part is pulled through the colon from the inside and attached to the anus. This is usually done using a minimally invasive method (laparoscopy), surgery through the anus.
Ostomy Operation
In seriously ill children, surgery can be done in two steps.
First, the abnormal section of colon is removed and the upper section of healthy colon is connected to an opening the surgeon makes in the child’s abdomen.
The waste then leaves the body through an opening into a pouch attached to the end of the intestine that protrudes through an opening in the abdomen (stoma).
This gives the lower part of the colon time to heal.
After the colon has had time to heal, a second procedure is performed to close the stoma and connect the healthy part of the colon to the rectum or anus.
Apart from that, there is a story of struggle related to children with this disease condition.
TeamtheAsianParentIndonesiahad the opportunity to interview a mother who has a child with this condition.Hirschsprung’s Disease. What was the story of the experience like?
Story of a Child Suffering from Hirschsprung Disease, No Defecation After Birth
Eka Meilinda Sari never thought that her first son would suffer from…Hirschsprung’s Disease.
His son’s condition was first discovered when the little one was born in the hospital.
“In a case like my child, the symptoms were visible when he was 3 days old. Usually, newborn babies up to 3 days old will definitely have black or meconium-colored stools, but my child didn’t have any stools at that time.” Lia, her nickname, began the story.
After giving birth by caesarean section in 2015, she did not do any rooming in or being in the same room with her baby.
At that time, the nurse came to give news that the son had passed meconium, but in small amounts.
“Three days after giving birth, the doctor allowed me to go home, but my child was not. That’s when I became more suspicious, but the doctor still couldn’t give a definite diagnosis. I insisted on staying another night in the hospital and my child was X-rayed,” said Lia.
At that time, the doctor who examined the son was still unable to provide certainty about the actual condition.
The family did not know what kind of treatment to provide and finally decided to go home.
“Her stomach did look more bloated and tight. We thought maybe it was just normal bloating. We insisted on going home, after 2 days at home it didn’t get better, so we checked her again at a different hospital which could be said to have much more complete facilities,” Lia said.
After 3 days of hospitalization, the Hirschsprung diagnosis was still not given by the doctor. On the 4th day, the child was allowed to go home. However, it turned out that his condition suddenly dropped .
“I was happy , I wanted to pick him up even though the stitches from the surgery were still stinging a little. But when I arrived at the hospital, his pediatrician said he was dropping again. Then, I was told to meet a pediatric surgeon. There, I got a little enlightenment that my child most likely had Hirschsprung Disease,” she said.
In order to establish this diagnosis, the son had to undergo a series of examinations such as x-rays and biopsies.
Finally, Lia’s son underwent surgery to connect his intestines to his anus.
Children’s digestion becomes sensitive becauseHirschsprung’s Disease
Even though surgery has been performed, it turns out there are side effects from the condition.Hirschsprung’s Diseasethat Lia’s son suffered during his growth and development.
One of them is that the child’s digestion becomes sensitive.
“If it’s for growth and development, there must be a slight effect because of HD. For example, when it comes to toilet training, my child is a bit slow because he has to learn to control the movement in his anus. In addition, his digestive process is a bit different, so he is more sensitive than normal children. His poop is never hard so sometimes if he loses control, he can lose it like that.” Lia added.
The son becomes more prone to diarrhea, bloating, and other digestive problems.
To overcome this, Lia tries to get her son to always eat home cooking.
“Even when I go to school, I bring my own lunch from home. I rarely buy street snacks, not because I’m being hygienic, but because the conditions need to be monitored. I also limit packaged foods, because the sugar content can make your digestion upset, such as diarrhea or bloating.” She explained.
This dietary regulation is part of Lia’s parenting . She stated that she is not the type of parent who strictly forbids her child from eating this and that.
“I’m not the type who is strict about this or that food, but more about it being allowed, but I determine the amount. At least , he tries it and knows the taste, so when he eats, he doesn’t eat too much,” Lia explained.
Lia’s method ensures that her child never throws a tantrum when he wants food like his other friends.
According to him, his son was not curious because he was still given the opportunity to taste it.
If it turns out that the child likes the food, Lia usually gives it to him infrequently so that his digestive health is maintained.
Due to her baby’s digestive condition which requires special attention, Lia routinely consults a doctor every few months to take prebiotic vitamins.
Becoming a Parent of a Child with DisabilitiesHirschsprung’s Disease, “The Key is Enthusiasm, Patience, and Prayer”
As a parent with a child who has a special condition, Lia said that the key to accompanying the child is Hirschsprung’s Diseaseis with enthusiasm, patience, and don’t forget to pray.
“At the beginning, there must be something that makes you down . For example, before the operation, after the operation there are still many challenges waiting. In my case, for example, there was a problem of rashes on the skin after the stoma bag was installed, after the closed colostomy there were still many.” Lia said.
According to him, parents must be proactive during consultation sessions with doctors to get a clear picture of their child’s condition.
“Always consult a doctor, just pepper the doctor with questions that we want to know. Everything will pass, it feels difficult at first because we don’t understand what to do. But, if you already know and understand, it will be easier to accompany your child,” he concluded.
He also left a message for parents who have children with special conditions such asHirschsprung’s Diseaseor others to keep up the spirit.
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Hopefully Lia’s story can be useful for all of us, especially for those who are also struggling with special child conditions.
That is the inspiring story of Bunda Lia’s struggle to care for a child suffering from Hirschsprung’s disease .
Hopefully, the little one will always be healthy until he grows up. And Mother Lia will remain enthusiastic in raising her child.
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Again, Hirschsprungs Disease is a serious disease if left untreated.
So, it is important to get help as soon as possible. Take your child to a specialist doctor immediately if they experience some of the above symptoms.
Hope this information helps!
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