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

Surgery. #6

October 14th - Today was the day. Isaac was 6 days old. For 4 days he has been poked and prodded almost on a continual basis. We've had to weave through leads, IV's, cords of all kinds just to be able to hold him. I wanted so badly to hold him like I did for the first 2 days of his life. With nothing attached, nothing to be careful of, nothing to restrict us to his bedside. I felt this was precious time I was losing. Bonding time with my son. But I also knew that he would remember me being there all the time. The only time I really left was when he was asleep. I asked all his nurses to call me as soon as he had woken up so I could make my way back there.. If I wasn't there already. And they always did. They were all so good to us. One of his nurses even drew him a picture of a ladybug and posted it on the plexi glass next to his head. He loved looking at it. I made sure to pack it in our bag to take home so we could have that for his baby book.

Surgery was scheduled for 9am. They told us that around 7am they would prepare to take him to pre-op. So we made sure to get there extra early to spend some time with him before. He was sleeping when we got there so we let him rest. I did hold him before because they told me once they got him up to pre-op he would remain in his transport incubator type thing until they were ready to take him back to the OR. I don't remember much of this morning. I only remember going up to pre-op. We loaded the elevator. Isaac was wide awake and being a squirmy wormy. He was so precious. We got off the elevator and got to our little room.They told us Dr. Acierno, the anesthesiologist, and the rest of the surgical team would be by to see us. We had requested a nurse, Kristin, from the NICU to go to surgery with him. She was there with us the whole time. She was our favorite. Isaac had so many nurses, at least 4 a day, but we LOVED all of them. But Kristin made me feel confident and comfortable with everything that was going on. I trusted her. She was supposed to be on vacation that Friday but she requested to be on the schedule and to work that day so she could take him to surgery. She did this without even telling us. That's how special and kind this lady was. I had asked someone the day we found out he had Hirschsprung's if it was possible to request a certain nurse to go with him to surgery. When I found out we could, I immediately asked Kristin. I felt better because she was there.

I can't even remember what time the surgical team came by.. Probably around 8:30am. Dr. Acierno gave us a run down of how the surgery would go. She also told us that Kristin would be calling us from the OR and updating us as each step was completed. The plan was to do a small incision under his belly button so she could do a biopsy and have the pathologist review the sample right there to check for ganglion cells. The closer the cells were to the end of his large intestine, the better it was. That meant he could have the pull through procedure because he had what was considered "short segment." If that was the case, she would go ahead and do the pull through. She couldn't give us an exact time frame because she really didn't know. If he had a larger area affected, she may need to do a colostomy. Which would make the surgery more complicated. As she finished the anesthesiologist came in and introduced herself, along with a surgical nurse named Shannon. We were pretty quiet and didn't ask that many questions. We really didn't know what to ask. We just wanted it to be over and done with.

I could barely look at Isaac. I felt so bad. I felt like it was my fault. It was the worst feeling in the world. But I just kept telling myself, this HAS to be done. He NEEDS this surgery. After all, I didn't have a choice. Kristin then told us a few things about what to expect after surgery. She told us he would still be on a ventilator and that he would be very out of it. She said he might be twitching or making odd movements. She said as the day went on it would wear off and he would be back to normal. She basically just gave us a run down of what he would look like and what they would be watching for. She told us that after a surgery she would only have one patient and they monitor them minute by minute. She would only have him for the day. That made me feel better. She would be right by him all day and so would we. I heard people coming down towards our room again and I knew it was time for him to go to the OR.

We kissed him and told him we would see him soon. I promised him that he would get to home in a few days but we just had to do this first. I promised I would be by his bedside when he woke up and I would rock him like I always did. It was the hardest thing I've ever had to do. Fear set in. I began to let my mind run in a million different directions. I burst into tears as I watched them wheel him away. He was happy and wiggling all around. I was like how terrible of a mom am I to send him away like that. He didn't even know what was going to happen. I didn't want him to feel scared. I didn't want him to think we weren't there for him. It was so hard... I reminded him that I loved him and I would be waiting for him.

We went up to the surgical waiting area. At that moment I felt really calm. I felt confident. I knew that this was one step closer to bringing our baby home.  We checked in at the front and gave them our number and name. Each patient is given a number and there is a tv screen that you can watch to see what phase they are in during surgery. Also, we had to give our names and number so that when the OR called to update us they could get us on the phone. Dr. Acierno told us it would take her about 30 minutes to get him set up. And then about 30 minutes to do the biopsy. So we wouldn't hear anything for about an hour. I just kept thinking an hour? An hour? What am I going to do for ONE long HOUR? Ug I hate having a time put on something. That meant clock watching minute by minute for an hour.

Every time the phone rang up at the reception desk, I would jump. My heart would race, my face would feel hot, I felt faint. When the phone rang and it was for us, I jumped up so fast. I didn't know if we would be getting good news or bad news. When I got on the phone Kristin told us that they had done the biopsy and on the first sample there were "lots of ganglion cells." That meant he only had a very small area affected. I was so relieved. She said now they were going to proceed with the pull through. She said he was doing very well. I let out a sigh of relief and hung up. This was the best possible outcome we could have asked for. Now we had to wait for the next call. I figured it would be like at least 2 hours. Blah.. Time.

About 40 minutes later the phone rang for us. Kristin told us that they were done and the surgery went very well. She said Isaac was doing great and that they were taking him back to the NICU to recover and that Dr. Acierno would be up to see us and tell us how everything went. He was in surgery for under 2 hours. I figured it would have taken a lot longer. I was so relieved that it was finally over. I was so eager to hear from the doctor and to get down to see Isaac.

When we met Dr. Acierno in the post op conference room she told us that she had removed about 5cm. She said he had such a minimal area affected that she didn't expect any complications. She said that it was a text book case and ideal surgery. She expected him to do very well. Finally I could breathe again. The worst part of our journey was over.

As we got to Isaac's bedside I felt an instant rush of relief. I was no longer worried or scared. I was so happy, like a huge weight was lifted off my shoulders. When we walked in he was laying on his back.. He was still on a ventilator. And that was hard seeing him like that. But he didn't look bad at all.. I thought he would look horrible. Thankfully, he didn't. I spoke softly to him. We were told to not speak loudly or to talk too much to him because the anesthesia and surgery was overwhelming for him. We actually didn't stay that long because they told us to let him recover and to let him sleep as much as he could. We didn't want anything to hinder his recovery. I asked Kristin how he was doing and if he was stable. She told me he was doing excellent and that they didn't expect any issues.  So we kissed him, told him we loved him, and stayed for about 20 minutes just so he knew we were there for him. I felt so much better now. It was a total turn around. The past 4 days seemed like weeks. All the hours ran together. I was hoping that we would get some normalcy back. This was such a traumatic event that we had not planned for. But who ever does, right? As we walked out of the NICU I imagined what it would be like to walk out of these same doors with Isaac in our arms and for us to head home. That didn't see like a dream anymore.. It was becoming a reality.