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Hirschsprung's Explanation Episode · Tom's Hirschsprung's Podcast

• 0:05
  Speaker 1: You are listening to Tom's Hirshprung's Podcast, where I provide
• 0:08
  Speaker 1: a look into the remarkable experiences of the most inspiring
• 0:11
  Speaker 1: people around Hirshprung's disease and sports. Hello and welcome to
• 0:39
  Speaker 1: a short but sweet episode of Tom's Hirshprung's Podcast. This
• 0:44
  Speaker 1: episode won't have a number because it's not an interview,
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  Speaker 1: but this is just going to be a series of
• 0:51
  Speaker 1: clips where myself, Luise and Carla, my other two co founders,
• 0:55
  Speaker 1: are going to explain what hirshprunks is, the symptoms and diagnosis,
• 1:02
  Speaker 1: and also what types of treatment you can expect to
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  Speaker 1: find as well.
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  Speaker 2: So kicking us off. Hirshprung's disease is a birth defect
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  Speaker 2: that occurs in around one in every five thousand babies.
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  Speaker 2: During the fetal development, a certain type of nerve cell,
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  Speaker 2: often called the ganglion cells of the intestine from the
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  Speaker 2: top of the digestive tract will fail to form. These
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  Speaker 2: ganglion cells tell they tell the muscles of the intestine
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  Speaker 2: to relax. In Hirshbrng's disease, these nerve cells fail to
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  Speaker 2: develop in certain portions of the small and or large
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  Speaker 2: intestine because the nerve cells are absent, the intestine fails
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  Speaker 2: to relax. Therefore, stall and gas cannot pass, and the
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  Speaker 2: blockage may occur when the disease put when the diseased
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  Speaker 2: portion starts. When this occurs, it may be difficult or
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  Speaker 2: near impossible for the effected child to pass stall. In
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  Speaker 2: terms of types of Hirshprung's disease, there are five main
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  Speaker 2: types or five five types in total. Some will have
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  Speaker 2: different percentages in terms of the amount of people that
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  Speaker 2: are affected, and these percentages are rough estimates rather than
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  Speaker 2: sort of absolute guarantees. But the first one is an
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  Speaker 2: ultra short segment r SHPRUNGS otherwise I'M known as U
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  Speaker 2: s SHD. This will be that the child is missing
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  Speaker 2: the gang beIN sales and the last one to two
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  Speaker 2: centimeters of direct This may not require surgery, but also
• 2:43
  Speaker 2: may do as well, but this is probably the least
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  Speaker 2: slightly to require surgery. Secondly, you have short segment Hirshprung's
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  Speaker 2: disease s SHD, which I have. This is the most
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  Speaker 2: common form of Hirshprung's disease where you are missing the
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  Speaker 2: ganglion cells in the rectum and the sigmoid polon this
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  Speaker 2: will affect around about eighty percent of cases. Thirdly, long
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  Speaker 2: segment Hirshprung's disease l SHD. This is where you are
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  Speaker 2: missing ganglion cells in the first third of the colon
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  Speaker 2: and can affect fifteen to twenty percent of people. Would
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  Speaker 2: have hirschbrngs fifteen to twenty percent of cases. Total colonic
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  Speaker 2: Ersprungs TCHD, where you will be missing ganglion cells in
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  Speaker 2: the entire polon, so this is round about five percent
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  Speaker 2: of cases. In rare cases, the small valve may also
• 3:45
  Speaker 2: be affected. Lastly, you have total intestinal a ganglianosis. This
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  Speaker 2: is very rare, that not a percentage on it. From
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  Speaker 2: what I can see, this is a very rare type
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  Speaker 2: of hirshprungs. Obviously, Hershprungs itself is very rare, and this
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  Speaker 2: is a rare type of a rare condition, so ultra
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  Speaker 2: rare if you will, this will be the absence of
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  Speaker 2: ganglion cells from the duodenum to the rectum. So hopefully
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  Speaker 2: they excuse you a bit more of a clue as
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  Speaker 2: to what Hirshprungs is and the types of hersprungs that
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  Speaker 2: can be present as well. And as I said, Louisa
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  Speaker 2: will talk through the symptoms and the diagnosis next, and
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  Speaker 2: then finally Carla will talk about possible treatments that you
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  Speaker 2: may will see.
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  Speaker 3: Hi, my name is Louisa and I am a Hirshbruns survivor.
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  Speaker 3: Some symptoms of Hirshbrungs include a swollen bareley, constipation so
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  Speaker 3: they're unlikely to be able to pool, vomiting, and second
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  Speaker 3: and your little one will probably have difficulty feeding. These
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  Speaker 3: can vary based on the type of Hushbrungs they've got
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  Speaker 3: and just because your newborn has these symptoms, it doesn't
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  Speaker 3: necessarily mean that it is Hushbrungs, but please ask your
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  Speaker 3: nurse or doctor to have a look into it. In
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  Speaker 3: the UK, these symptoms are typically picked up on and
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  Speaker 3: further tests are done, but this will probably differ in
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  Speaker 3: other countries depending on the medical resources available. In terms
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  Speaker 3: of diagnosis for Hirshbruns, a rectal biopsy will need to
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  Speaker 3: be done and this is where they just take a
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  Speaker 3: little sample of cells from your little one's bow well
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  Speaker 3: and they have a look at them to see what's
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  Speaker 3: going on. I know it sounds really really scary. It
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  Speaker 3: will provide the medical team to get the information they
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  Speaker 3: need styleth is hairshprungs or not, and then they'll be
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  Speaker 3: able to get the right treatment they need.
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  Speaker 4: Hi, my name is Carla's Caramella, and I will provide
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  Speaker 4: you some details about the treatment of hershburn disease. Hershbron
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  Speaker 4: disease always needs surgery at least one, but can be
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  Speaker 4: more than one, depending on the portion of colon affected
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  Speaker 4: by the heirshprum, the time on which the hersheprin disease
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  Speaker 4: is diagnosed, and the approach of the pediatric surgeon involved
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  Speaker 4: in the surgery. Typically, when hershburn disease is diagnosed in
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  Speaker 4: newborns and the portion affected is suspected to be a
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  Speaker 4: short segment harshprin disease, the treatment would be rectal irrigations,
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  Speaker 4: which consists of liquid injections to cleanse an empty the bowel,
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  Speaker 4: and this would be done periodically by parents until the
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  Speaker 4: baby reached the age or the weight to be done
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  Speaker 4: the corrective surgery, which is traditionally called a pull through surgery. However,
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  Speaker 4: for some cases which are diagnosed at a later stage
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  Speaker 4: in life, or for heirshprun that are considered to be
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  Speaker 4: long segment or have a more portion affected of the colon,
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  Speaker 4: a colostomy or iliostomy would take place. This is a
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  Speaker 4: surgery that makes the colon or the ilion go out
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  Speaker 4: through the belly and in that way, the baby or
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  Speaker 4: the child would be able to pull properly until the
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  Speaker 4: corrective surgery, which also would be a pull through through surgery,
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  Speaker 4: would take place at a later stage when the surgeon
• 7:59
  Speaker 4: decides to do it. The pull through surgery should always
• 8:06
  Speaker 4: be held by qualified pediatric surgeons specialized in colorectal care.
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  Speaker 4: In addition, during the surgery, the surgeon will count on
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  Speaker 4: a specialized pathologist who would tell him up to which
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  Speaker 4: portion of the affected colon should be removed and in
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  Speaker 4: order to have an optimal surgery done.
• 8:30
  Speaker 3: Hi, my name is Louisa and I am a Hushbrans survivor.
• 8:36
  Speaker 3: Some symptoms of hush brands include a swollen barley, constipation
• 8:42
  Speaker 3: so they were unlikely to be able to poo, vomiting,
• 8:46
  Speaker 3: and secna. A new little one will probably have difficulty feeding.
• 8:53
  Speaker 3: These can vary based on the type of hush brunks
• 8:56
  Speaker 3: they've got, and just because your new born has has
• 9:00
  Speaker 3: these symptoms, it doesn't necessarily mean that it is hushbrngs,
• 9:05
  Speaker 3: but please ask u nurse or doctor to have a
• 9:09
  Speaker 3: look into it. In the UK, these symptoms are typically
• 9:14
  Speaker 3: picked up on and further tests are done, but this
• 9:18
  Speaker 3: will probably differ in other countries depending on the medical
• 9:23
  Speaker 3: resources available. In terms of diagnosis. For hirshbruns, a rectal
• 9:31
  Speaker 3: biopsy will need to be done and this is where
• 9:34
  Speaker 3: they just take a little sample of cells from your
• 9:37
  Speaker 3: little one's bowell and they have a look at them
• 9:41
  Speaker 3: to see what's going on. I know it sounds really
• 9:45
  Speaker 3: really scary, but it will provide the medical team to
• 9:50
  Speaker 3: get the information they need to tell if it is
• 9:53
  Speaker 3: hushbrungs or not, and then they'll be able to get
• 9:58
  Speaker 3: the right treatment they need.
• 10:00
  Speaker 4: If to pull through surgery, some children might experience severe
• 10:04
  Speaker 4: diaper rush due to the frequency and consistency of the depositions. Also,
• 10:10
  Speaker 4: these children are at the higher risk of developing entrocolitis.
• 10:15
  Speaker 4: Symptoms of enterocolitis may include fever, abdominal distension, lethargy, and bombits.
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  Speaker 4: This event requires immediate attention because if it's left untreated,
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  Speaker 4: it might lead to a septic job. Some of the
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  Speaker 4: long term challenges people with herpron disease might experience include
• 10:36
  Speaker 4: food intolerances, constipation, and issues in vowel management. However, not
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  Speaker 4: all people with herpron experience these events. Overall, people with
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  Speaker 4: Herberan disease can live happy and full lives.
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  Speaker 1: Thank you for listening to the podcast. Find us on
• 10:59
  Speaker 1: Instagram Underscore Hirshbrun's Underscore podcast. Listen to previous episodes on
• 11:04
  Speaker 1: Spotify by searching Tom's Hirshbrun's podcast.
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  Speaker 2: H