Complications. An educational post.. #51

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

(Referenced from http://www.aafp.org/afp/2006/1015/p1319.html)

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: 

http://sweetbabyisaac.blogspot.com/2014/01/managing-hirschsprungs-disease_21.html

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: 

http://sweetbabyisaac.blogspot.com/2014/05/chiropractic-48.html

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.. 

https://m.facebook.com/hirschsprungsstrong

Sweet Baby Isaac.. #50

The other day I posted this status to my Facebook...

With all this ALS stuff going on, makes me want to start raising awareness for other rare diseases that no one knows about. How about Hirschsprung's Disease. That's what my son has. And it virtually gets no funding for research, no shout outs, no challenges, no news time, no media time, nothing. Nothing. Yet these kids are just as strong, endure just as much - maybe more - than any other person fighting any other disease. As do their parents. So who will repost this to honor my son? Or post a sign for him saying Way To Go Buddy, you survived your surgery at 6 days old. Or donate money to fund Hirschsprung's research? No one cares because it's rare and not that important because it's not an "epidemic." Help me raise awareness for Hirschsprung's Disease. Share my status.

With this picture of Isaac. I asked all my friends to share my status and to keep sharing it. I got such a huge response, emails, messages, calls about what we could do to raise awareness. I have so many people willing to put in the work to raise awareness, start a non profit organization,  start challenges like the one currently circulating for ALS. With that, I created this Facebook page to raise awareness for Hirschsprung's Disease.  And a lot of other rare diseases that do not get ANY media coverage.

Pop over and like our page and share it far and wide!!! This is just the beginning people.. Lots more to come. Here is the link to our Facebook page.. 

Sweet Baby Isaac on Facebook

End of summer check in.. #49

The summer has been great to us. We have spent a lot of time swimming and doing yard work in our yard. After starting chiropractic care for Isaac things did 180 degree turn. For the better. He now has normal bowel habits. He is completely off the senna and we are seeing his surgeon every 3 months instead of multiple times a month or monthly. We made it over that hump! I believe whole heartedly chiropractic care was the missing piece to our puzzle. Another great doctor to add to our team.

In February we are going to Disneyland!! 15 of us. My side of the family has planned a big family vacation and we couldn't be more excited. This will be our first big family vacation. Planning for that has already started. It is leaving me so excited but so anxious because February in the past has been a bad month for us. Just with all the viruses going around. We spent a few days in the hospital last February with a stomach virus. Really hoping we can be 100% healthy so we can enjoy our vacation. But we will probably have to talk to our surgeon about worst case scenario options for when we are in California. It's really hard being a parent with a special medical needs child sometimes because you always have to plan and think worse case scenario. Which means I will need to get hospital names and drs there. In case we have an issue arise. It has limited us as far as going on vacations because I guess we have always been worried about the what if. Which is not good. I really hope we can stay healthy and go enjoy this vacation :)

I just wanted to give a brief update and let everyone know we are doing well!! I hope to write again soon.. Before Isaac's 3rd birthday. Yep, he will be THREE.. 3.. THREE in 2 short months. Where has the time gone?!? I can't believe my baby will be 3.