Steve’s bowel cancer story
Steve shares his bowel cancer story and shares, “If you have blood in your poo, it needs checking out. Don’t put it off.”
Marking Hirschsprung’s disease awareness day
What is Hirschsprung’s disease?
Hirschsprung’s disease is a condition people are born with. It slows or stops the movement of poo through the large bowel. Ganglion cells, which are responsible for controlling bowel movement, are absent. Because of this, peristalsis, the motion that pushes poo through the anus (back passage), does not work properly.
Hirschsprung’s is often diagnosed right after birth, but sometimes the symptoms are milder. The diagnosis can happen later in childhood in these cases.
About 8 in 10 people with Hirschsprung’s disease have the rectosigmoid affected, which is the last part of the colon. Doctors call this short segment Hirschsprung’s. 15 out of 100 people (up to one fifth) will experience an effect on the bowel further up the sigmoid. In 5 in 100 people with Hirschsprung’s disease, the whole of the large bowel is affected. Doctors call this total colonic aganglionosis.
Hirschsprung’s affects about 1 in 5000 babies. For every girl affected, 4 boys are affected.
What causes Hirschsprung’s disease?
The reasons the nerve cells in the bowel don’t develop properly aren’t fully clear. But we do know it’s not due to anything the mother does during pregnancy. Some gene changes link to Hirschsprung’s disease. This means it can happen more often in certain families. Children with genetic differences, like Down’s syndrome, are more likely to be born with Hirschsprung’s disease.
What are the symptoms of Hirschsprung’s disease?
Symptoms in a baby
- A delay in passing meconium. Meconium is a thick, dark greenish-brown stool that babies usually pass shortly after birth. This is usually noticed during the first 24-48 hours of life.
- A swollen belly (abdomen).
- Vomiting bile (green liquid).
A serious condition called Hirschsprung-associated enterocolitis (HAEC) may occur. This is where the bowel becomes inflamed. Signs of HAEC can include:
- Complete constipation (not passing wind or poo).
- Watery or explosive diarrhoea.
- Distended abdomen (swollen tummy).
- Rectal bleeding (blood from the back passage).
- Fever (high temperature).
- Drowsiness (sleepier than seems normal).
- Signs of sepsis (see below).
Septic shock (sepsis) is a medical emergency. You might notice a child has some of the following symptoms:
- Difficulty breathing. You may notice grunting noises or their stomach sucking under their ribcage. They may have breathlessness or be breathing very fast.
- A weak, high-pitched cry that’s not like their normal cry.
- They may not respond like they normally do. They may not be interested in feeding or normal activities.
- Being sleepier than normal or difficult to wake.
Call 999 or go to A&E if a child is experiencing these symptoms.
Symptoms and signs in an older child can include:
- Abdominal (tummy) discomfort and swelling.
- Vomiting.
- Constipation that continues and does not improve with usual treatments.
- Feeding problems.
- Lack of normal weight gain.
How is Hirschsprung’s disease diagnosed?
A doctor first examines the child’s belly (abdomen) and usually does a rectal exam. Doctors gently insert a finger into the anus (also called the bottom or back passage) to check for any issues. A doctor may do an x-ray of the belly.
Doctors may need to perform a rectal biopsy. This is a small operation where the doctor takes a sample of tissue from the rectum. A laboratory checks this sample to see if Ganglion nerve cells are missing.
How is Hirschsprung’s disease treated?
Surgery is needed to treat Hirschsprung’s disease. Most children have a procedure called a ‘pull through’. The damaged part of the large bowel is taken out, and the healthy sections are joined together.
While waiting for surgery, the medical team may need to provide the following treatments:
- Intravenous fluids (fluids given into a vein through a ‘drip’).
- A nasogastric tube. (A tube passed through the nose and passed down the gullet into the stomach)
- Irrigation. This is when fluid is delivered with precision into the bowel using a tube inserted through the anus. The fluid is then pumped out slowly.
Sometimes surgery happens in stages. This can happen if the child has other issues, such as enterocolitis, or if doctors diagnose Hirschsprung’s later.
In the first stage, a stoma may be needed. This means the bowel is redirected, and waste passes into a bag outside the belly. This allows the lower part of the bowel to rest. The next stage will be an operation to remove the damaged part of the bowel. Then, the healthy sections will be joined together.
How is Hirschsprung’s disease monitored?
Some children living with Hirschsprung’s may have problems with constipation or incontinence. Support is available to help with these problems. There is a thought to be a small possibility that some people are at a higher risk of developing inflammatory bowel disease in later life.
What to ask your child’s doctor about Hirschsprung’s disease?
- What type of Hirschsprung’s disease does my child have?
- What are the benefits and risks of the treatment for my child?
- Will my child need a stoma, and if so, for how long?
- What happens after the surgery?
- How will surgery affect my child’s toilet training?
- Are there any long-term risks?
Why is Guts UK celebrating Hirschsprung’s disease awareness day?
Knowledge and awareness of this condition remains limited, and currently, there is no UK charity dedicated to providing information and support for Hirschsprung’s disease.
As the national charity for the digestive system, Guts UK is proud to provide information and support on more than 40 digestive conditions and symptoms, including Hirschsprung’s disease. As our charity grows, we continue to expand the information and support we can provide, ensuring that we’re there for everyone who might need us.
We are dedicated to improving public education and raising vital awareness for our guts. We’re joining this year’s awareness day campaign to share more information about this condition, including signs and symptoms, diagnosis and treatment.
We’re thrilled to be joining charities and organisations from 18 different countries around the world, who are coming together to raise awareness and increase visibility for the condition. On Monday 15 September, we’ll be joining 25 patient advocacy groups and seven paediatric surgery associations and hospitals across the world in support of Hirschsprung’s Disease Awareness Day, which takes place during Hirschsprung’s Disease Awareness Month in September. Together, we are stronger!
If you’re a registered charity, advocacy group or healthcare organisation that would like to be part of this collaborative initiative, reach out to hirschsprungargentina@gmail.com to find out more.
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