I wanted
to take a few minutes to talk about COMPLICATIONS from Hirschsprung's Disease.
I have heard from a lot of people that the internet hasn't been of much help
for education. My point exactly. I read a lot of the sites and the stuff they
say is pretty off - as far as complications go. That's why I am asking more
families like ours to post about their stories. Hirschsprung's is a VERY tricky
disease. Almost ALL kids with it suffer
complications. The internet makes it seem like this disease is an easy fix,
have a surgery and they are cured. NOT AT ALL. This is a LIFELONG illness. A
LIFELONG disease that we battle EVERY SINGLE DAY. This blog entry is for
education on complications and to talk about some struggles we have had, and
some struggles some of our fellow HD families have had.
First I would like to cover some general education on
Hirschsprung’s Disease. There are 2 main types of Hirschsprung’s Disease, known as Short
Segment Hirschsprung’s Disease (SSHD for short) and Long Segment Hirschsprung’s
Disease (LSHD for short) which are defined by the region of the intestine
lacking nerve cells.
In short segment disease, nerve cells are missing from
only the last segment of the large intestine. This type is most common,
occurring in approximately 80% of people with Hirschsprung’s Diease. For
unknown reasons, short segment is 4 times more common in men than in women.
Long segment occurs when nerve cells are missing from
most of the large intestine and is the more severe type. Long segment is found
in approximately 20% of people with Hirschsprung’s and affects men and women
equally.
The rarest forms of Hirschsprung’s are called Total
Colonic Hirschsprung’s Disease (or TCHD) or Total Intestinal Aganglionosis
(TIA). In Total Colonic the nerve cells are missing from the entire large
intestine and sometimes part of the small intestine. And in Total Intestinal
ALL of the nerve cells are missing from BOTH the small and large intestine.
Depending on what type of HD you have, really means
what your outcome will be. Kids with short segment seem to fare better. Also,
there is different protocol for surgery, care, etc depending on what form you
have. A lot of the time kids with short segment just get the pull through
surgery. Kids with long segment may need a colostomy bag or an ileostomy before
they can have their pull through surgery. Isaac did not have a colostomy so I
am not well versed in this topic. I am hoping that more HD parents will come
forward with their stories so I can post more about kids with colostomy bags
and such. What I can do, is talk about Isaac’s surgery and talk about his
complications he has experienced.
In children who cannot receive the pull through directly the surgery is
done in steps. First, the abnormal portion of the colon is removed and the top,
healthy portion of the colon is connected to a small hole (ostomy) the surgeon
creates in the child's abdomen. Stool then leaves the body through the ostomy
into a bag that attaches to the end of the intestine that protrudes through the
hole in the abdomen. This allows time for the lower part of the colon to heal.
Ostomy procedures include:
- Ileostomy. With
an ileostomy, the doctor removes the entire colon. Stool leaves the body through
the end of the small intestine.
- Colostomy. With
a colostomy, the doctor leaves part of the colon intact. Stool leaves the body
through the end of the large intestine.
Later, the doctor closes the ostomy and connects the healthy portion of the intestine to the rectum or anus. Which is called the pull through (or they sometimes call it a BOWEL RESECTION), which is what Isaac had. He had the pull through – which was one surgery. Even with the pull through there are many factors and techniques for that surgery. The surgical techniques are called the Swenson, Suave or Duhamal. Each of these operations has different versions. Which type is best for your child depends on their condition. Also there are different ways the surgery is performed.
- Minimally invasive procedure. The surgeon
makes a few small cuts (incisions) in your child's belly. Then, they insert a
thin, lighted tube with a camera and their surgical tools. This is sometimes
called laparoscopic surgery.
- Open surgery: The surgeon uses a single cut
to get to the intestines that need to be removed. The incision is usually
larger than those used in minimally invasive surgery.
- Transanal pull-through: Sometimes the surgeon
can reach the intestines through your child's anus. Then, they remove the unhealthy
section and pull the remaining intestines down.
- Combination: In some cases, surgeons use
laparoscopic or open techniques along with a transanal operation.
Isaac had the Swenson technique and it was
done as a combination surgery. His surgeon made a small incision below his
belly button, she pulled the intestine out, did a biopsy in the operating room
to make sure the area she was connecting to had plenty of ganglion cells (which
are the cells that are missing from the diseased portion of the colon), then
she proceeded to perform the trans-anal pull through.
Now that I have covered some basics about the
different types of Hirschsprung’s and surgeries they perform, I will now talk
about the complications that kids can and DO experience. It really irks me that
most of the informational stuff on the internet makes Hirschsprung’s seem
manageable, easy to treat, and basically no lifelong complications. WRONG. SO
WRONG.
One big
complication that is on the forefront for many kids is Enterocolitis. Children who have Hirschsprung's disease are prone to a
serious infection of the intestines called enterocolitis.
Enterocolitis is caused by stool backing up behind the
immobile section of colon. The stagnant mass of stool provides a fertile
environment for bacteria to grow. As the stool mass expands, it presses on the
blood vessels in the walls of the colon. Decreased blood flow can lead to a
breakdown of the lining of the colon (mucosa), making it susceptible to
infection. It can be a life-threatening complication. It's treated in the
hospital with colon cleaning (rectal irrigations) and antibiotics.
Isaac has never had enterocolitis (thank God) but I know
MANY HD kids that have. It is really scary. Every time Isaac has a stomach bug,
he has to go into the ER and is almost always admitted to be monitored to make
sure it’s not enterocolitis. This infection can come on really fast and be very
severe in a short amount of time. It usually begins with foul smelling
diarrhea, loud audible stomach noises, lots of gas, abdominal distention,
fever, possibly vomiting. HD kids can get dehydrated very easily and much
quicker than a normal child would. So IV fluids are almost always a must, even
when it is just a stomach bug. So simple childhood illnesses like
gastroenteritis (stomach flu) and viral illnesses are taken very seriously with
HD kids because those illnesses show the same symptoms of enterocolitis. That’s
why Isaac has to go into his surgeon or the ER every single time he has a
stomach illness. To rule out enterocolitis. This is why we are almost always
admitted a couple times a year. This is why we were just in the hospital last
week.
I also read on a website, this information.. I am
absolutely IRATE with the first sentence... Most
patients treated for Hirschsprung’s disease do not have complications.
However, up to 10 percent may have constipation, and less than 1 percent may
have fecal incontinence.6 Enterocolitis
and colonic rupture are the most serious complications associated with the
disease and are the most common causes of Hirschsprung’s-related mortality.
Enterocolitis occurs in 17 to 50 percent of infants with Hirschsprung’s disease
and most commonly is caused by intestinal obstruction and residual aganglionic
bowel.5,6 Infants should continue to be monitored closely
for enterocolitis many years after corrective surgery because the infection has
been reported to occur up to 10 years later. However, most postoperative
enterocolitis cases occur within the first two years of ileoanal pull-through
anastomosis.6
Are they serious? Most children do not have
complications? Let me see, EVERY SINGLE HD kid I know has had some complication. MY CHILD HAS.
The information online makes it seem like this disease is so cut and dry. And
there is no long-term side effects or issues. Most of the websites don’t even
list anything more than Enterocolitis as a complication. Yet, there are many
more things that HD kids can experience. More surgeries, bowel obstructions,
kinks in the bowel or bowel rotations, malabsorption issues, weight gain or
feeding issues, other GI issues like reflux, food allergies, dehydration,
constipation, incontinence, I can’t even name all the things I have seen or
heard about HD kids having issues with.
All the reading material online is passive. This is one
site I visited it and this is what it shows are long-term lifestyle changes for
managing the disease or what the general outlook is..
Children
may experience constipation after surgery. To help manage constipation:
- Serve high-fiber foods. If your child eats solid foods, include high-fiber foods as part of your child's diet. For instance, offer whole grains, such as whole-wheat bread. Reduce servings of refined grains, such as white bread. Encourage your child to eat fruits and vegetables. Cut down on fatty animal-source foods, such as meat and butter. Be aware, though, that a sudden increase in high-fiber foods can make constipation worse — so add high-fiber foods to your child's diet slowly. If your child isn't eating solid foods yet, ask the doctor about formulas that might help relieve constipation
- Increase fluids. Encourage
your child to drink more water. One of the colon's jobs is to absorb water from
food in the last stages of digestion. If a portion of your child's colon was
removed, your child may have trouble absorbing enough water. Drinking more
water can help your child stay hydrated, which may help ease constipation.
- Encourage physical activity. Daily
aerobic activity helps promote regular bowel movements.
- Ask your child's doctor about laxatives. Certain
laxatives — medications to encourage bowel movements — might help relieve constipation.
Ask the doctor about the risks and benefits of laxatives for your child.
SERIOUSLY?!? This is what you
“need to do” to manage it. Managing constipation for an HD is not simple. These
guidelines for managing constipation are for a child with a NORMAL COLON. Yes,
these things are beneficial for an HD child, but not at all “the fix.” Children
with HD have a colon that does not function normally. It never will. Even with
surgery. There is something structurally wrong with their colon.
This is why HD is a lifelong condition. The surgery works yes, but it does not
cure Hirschsprung’s.
Last year when Isaac turned 2, he started complaining about stomach pain. We took him into his surgeon who did some xrays and determined he was having issues with constipation. She suggested that we try dietary changes – adding in chia seeds, maybe some prune juice. Things like that. We then had a recheck in a month which determined those measures were still not helping. Still constipated. The issue with constipation is the longer it goes untreated, the more likely the colon will stop functioning properly. When you don’t have a system that is functioning properly, all kinds of other issues come up. One being, that if you let the issue go, the colon with basically will get lazy causing the colon to be stretched out. Obviously which makes it not work properly. It also puts the child at risk for another surgery. That’s when a mom friend of mine suggested we see a nutritional practitioner. I
posted a blog about what we were trying and what we were doing under her care,
which is located here:
I
won’t go into detail about it on this post since it was already a blog entry
for us. The things we learned, and still learn, from here are so valuable. She
told us that a strict NON GMO diet was in order because people with GI
disorders, especially ones like HD, should not consume GMO’s. In case you are
unfamiliar with GMO’s I suggest you read up on them on this website (http://www.nongmoproject.org). In short, GMO’s causes inflammation in the intestines and digestive tract. Can you see
why it wouldn’t be good for HD kids?? She also really outlined a diet for us,
what stuff to avoid, what stuff to stick to, and she also prescribed a lot of
supplements to start to “heal” his gut. When we started working with her, our
surgeon had put us on a low dose of senna. Which is a bowel stimulant. Not a
laxative, a stimulant. This helps aid in the process of elimination (pooping)
by basically stimulating your bowels or forcing them to empty. It wasn’t that
his poop was hard, it was not moving through fast enough. We went back and
forth on the dosage. Up down, up down. Sometimes it really upset his stomach.
Lots of gas, lots of pain. That’s when we went to see Isaac’s GI dr. After
speaking with him, he suggested we start seeking more alternative care, since
that’s what we were now leaning towards. I would not accept that my son would
need a bowel stimulant everyday just to poop. Especially one that hurt his
stomach. But what do you do? Either him be in pain from not pooping or be in
pain from the senna. And there was nothing more really the surgical team could
do because there was nothing really structurally wrong, it was just his system
not processing it fast enough. It needed stimulation. That’s when our GI
suggested chiropractic care. Which I just wrote an entry about that as well.
Which I won’t get into too much on this entry. You can read about it here:
I
wanted to point out that NONE of the alternative therapies are covered by
insurance. His nutritional practitioner, his chiropractic care, none of that.
That is all out of pocket expenses we pay for. I decided to not go back to work
when we found out Isaac had Hirschsprung’s Disease. And let me tell you, I made
the right choice. There were plenty of days, weeks, months that I had to
administer treatments at home. Irrigations amongst other things. Doing this is very common for HD kids. We often have to do them when
our kiddos get sick as well. And especially when they get enterocolitis. Some
parents have to do them every day just to get their kids to poop. With that
being said, a daycare would never be allowed (or even want to) perform these
treatments. Isaac also struggled with food allergies until he was about 2 years
old. HD kids really don’t just have Hirschsprung’s, they have a whole host of
other issues too. So putting your child in daycare is not really something we
can do. Some parents HAVE to, but I guarantee they don’t want to. HD kids need
to be monitored. They need to be treated as an individual. We have to document
what they eat, how much they drink, how much they are pooping.. What color the
poop is, the consistency of the poop, does it smell funny, do it look funny,
the list goes on and on. How are we supposed to put our kid under the care of
someone else who has no experience with this disease??? It’s not as cut and dry
as you think. Also, with the special diet, that means virtually no eating out.
No garbage food. Isaac couldn't have dairy, soy, grains, gluten, nuts, or eggs
until he was tested at the age of 2. His diet was so strict. And it still is..
We have to eat whole, organic foods. Which comes at a large cost. But I will do
anything to help my son. ANYTHING.
Another
issue is financial problems for HD families. Like our family, we live on one
income. Isaac's dad works a lot. Sometimes his schedule is not accommodating to
Isaac’s issues, but the company he works for has been such a blessing for us.
They understand Isaac’s condition and always try to accommodate when they can.
But most employers do not do this. What about the families that HAVE to go to
work and cannot miss work or they will get fired?? The other day our insurance would not pay for a 29$ bill to the pharmacy for
special spray and cream my son needs for his butt. 29$. They didn't want to pay
for it because it is considered something the pharmacy can dispense over the counter. But you cannot go into a store and just buy it. You have to have a
prescription for it. So how is that “over the counter?” We have tried every
single over the counter, buy in the store diaper cream. HD kids don’t get a rash
per say, the poop burns their butt. Basically a burn. They only thing that works
for Isaac is to have this special cream and spray. Our surgeon used to supply
it for us but they have cut back their expenses and cannot give it out anymore.
So now we have to pay $14.50 for a 1 oz (ONE OUNCE) bottle of spray and $13.50
for a tube of barrier cream. We use about one per week. So we are looking at
around 110$ a month. Do you know how long I spent on the phone on Monday with the
insurance company? 2 hours. TWO hours to not get anything taken care of, to get
a recording that said the phone lines are experiencing a high volume of calls
and to call back later. After being transferred to 5 different places. To hear
that recording. 2 hours. Do you know how long my fellow HD mom spent on the
phone last week with insurance? 6 hours. SIX HOURS. Who can have a full time job when you have to
spend 6 hours on the phone with insurance? How can someone even take care of
their child or children when they have to be on the phone for 6 hours. What
about if their spouse works? Or what if they are a single parent?? Our system
is so flawed. This is why I want to raise awareness for Hirschsprung’s. So they
medical system, insurance, whoever else will recognize the struggles that
families go through - not just HD families. On top of spending hours and hours on the phone a week
with insurance or whatever, we also have doctor appointments. Sometimes
multiple appointments a week. A mom I spoke with said her kiddo has 6 to 8
appointments a WEEK. A WEEK. That is like 2 or 3 a day. How can a parent of a
medical complex kiddo have a job that would accommodate a schedule like that??
There is no point in working because you spend all day with doctors. What about
all the other things life throws at you? Cleaning house, cooking, taking care
of your other children. Or even remembering to eat or shower. This is why HD
parents feel so alone. I admire HD parents (and other medically complex kiddos) because we
ALWAYS put our kids first. We don’t worry about how the bills are going to get
paid, or about when our house will be cleaned, or a shower. Or even thinking
about the future. HD is a day to day illness. We fight every day for our kids. It’s so hard to be
an advocate for your child when there is no one listening. No one there to
help. Hirschsprung’s families feel alone a lot of the time because people
generally don’t understand our struggles. Actually a lot of families feel alone
when their kids have medical issues. Not just Hirschsprung’s families. But I
want to raise awareness to increase the funding for research, to push our
government to have better programs for families like mine. Hirschsprung’s is
about 1 in every 5000 live births, that means there is about 40 families like
mine living in Tacoma (give or take with a population of about 202,000), I need
the help of all of you to link us all together so that we can tell our stories.
I only know ONE family locally. I need to know EVERY family in Tacoma. Every
family in Washington would be my goal. But without the help of all of my
friends, family, strangers, I cannot fight this battle alone. I hope after
reading this blog post you will decide to share my ILoveRare page one more
time. And to encourage all your family, friends, strangers on your Facebook to
do the same. I thank everyone who has already helped us. What you've done is invaluable to me. Here is the link to our Facebook page..
