Hirschsprungs: Our Journey with a Crappy Disease

*Fair warning: When I say this is a crappy disease, I mean it. Literally. This post is mostly about poop and how our life, Jett’s life (my 3 year old) revolves around it. I will share the perspective I have gained on caring for a child who is sick, as well as go into detail about his disease in an effort to bring some awareness to his condition.

                A few days ago, we celebrated Mother’s Day. To me, Mother’s Day is not only a day to celebrate the mother’s in our lives, but to celebrate being a mother. I think of each of my children and am filled with joy and gratitude for the opportunity I have to be their mother. Being a mother is H.A.R.D. work! Although each day isn’t filled with gum drops and rainbows, each day truly is a gift. Some of us mothers know all too well how quickly that gift can be taken from us. How the light of life can be snuffed out too quickly, too soon. Mothers who care for a child who has special medical needs know this truth all too well. It is a unique and terrifying experience to have a child who has been ill and almost died. For some, it is an isolated event that you can move past once it resolves and your child is healed. For others, it is a daily battle to keep your child healthy. Either way, once you experience it, it changes you- forever. Nothing can give you more perspective on life than the threat of losing a life. I applaud all mothers who make sure not a single stone goes unturned, who work tirelessly to make sure their child has every opportunity at having the maximum health their bodies will allow. It is a difficult, emotional, and often heartbreaking road on which we travel.

                As a mom, I have done things I never imagined when I would daydream about motherhood.  As a mom to a child who is sick, (by the way, I prefer to say “a child WHO is sick,” as opposed to “a sick child.” The same way that you should say “the man who is paralyzed,” not “the paralyzed man.” My child is first a child, who happens to be sick. Moving on…) however, I do things I couldn’t have even fathomed!

                My son has a bowel disorder. My senses have been numbed and I have forgotten that there is such a thing as “too much information” when it comes to talking about pooping habits. Sometimes I forget that not everyone is interested in hearing about my toddlers poop. Oops! Seriously though, all you mothers of regular-poo-pooing children don’t know how good you have it! I have been known to:

* smell my child’s poop and think it smells “good” (as opposed to infection-laden smelling poop, a sign something is seriously wrong),

*video tape my child pooping as encouragement for him to “push it out so he can watch himself later”

*dramatically fall on the floor just to make my child “toot” more,

*ask my neighbor (bless her heart!) if my child can poop in her toilet because he won’t poop and we are desperate to do anything that will get him to go.

*Say things REAAAALLY enthuastically, like it is the best thing EVER such as “Jett! Do you want to poop at the Aquarium? Sooo exciting! Come on!” “Oooo! Let’s poop at the Library! The grocery store! Church! Yay! How fun!”

*Be terrified, because I have lost Jett at the library and have everyone searching for him, only to be found by a librarian in the bathroom with poop ever where! Jett’s response at 2 ½ years old? “I had to poop weally, weally, bad Mom!” I was equally relieved that he was found AND he had pooped. Dodged a few bullets that day!              

                I will begin Jett’s story where it started, in the womb. When I was pregnant with our 3^rd child, we found out at the 20 week ultrasound that his life was in extreme danger. There was a problem with the womb, part of the amniotic sac had separated, and fallen, like a sheet, on top of our baby. The risks were anything from webbed fingers or toes, to deformations of the skull/brain, to self-amputation of limbs and…death. The remaining months of my pregnancy was like a roller coaster. I knew that my child’s life was in the Lord’s hands. Although this dilemma had nothing do with his eventual Hirschsprung’s diagnosis, it played an important role in my life as a mother, shaping my viewpoint of how each day I have with my children is a blessing, and a gift from God. It was the first time that I felt the delicacy that life truly is. 17 anxiety filled weeks and more than 20 ultrasounds later, our perfect baby boy was born into this world, unscathed by the band that threatened him inside the womb. It was a miracle, given by God. We knew it, and the doctors knew it.

                Fast-forward 4 days. We found ourselves in the Neonatal Intensive Care Unit, with our baby, who was placed there initially for his inability to maintain his body temperature and had a swollen leg (both issues resolved on their own within hours) but he was kept because he refused to eat and was force fed by an NG tube. The neonatologist told us he hadn’t pooped in more than 24 hours, hadn’t returned two suppositories, and his stomach was extremely distended making him at risk of perforation of his bowels. My husband and I waited on pins and needles for the EMS transport team to arrive and take him to another hospital that had the doctors he needed. We watched as the paramedics loaded him into an ambulance and drove away, without us. A few days later, we received his diagnosis: Hirschsprung’s Disease (HD). Never heard of it? Neither had we. HD is a disease in which ganglion nerve cells in the colon are absent, leaving parts of the intestine essentially paralyzed and causing an inability to poop.  

*Jett @3 days old, prior to any knowledge of a bowel problem

                At 10 days old, he had a surgical “pull through” procedure that removed the diseased intestine and then pulled down and attached the functioning intestine. Although surgery removes the diseased portion of the bowel, it is not a cure. There is no cure for Hirschsprung’s. Luckily, most cases do not require much care past surgery. Unluckily, a small percentage of children still experience a great deal of suffering, despite the operation. Jett does not suffer from the majority of these problems, but for some it may include repeated infection, chronic constipation, chronic pain, additional surgeries, strict diets, incontinence, delayed potty training, ostomies, medications, botox injections, irrigations, multiple hospitalizations, bowel management clinics, etc.

*Jett @5days old, pre-diagnosis

                Jett’s surgery was very successful, and 99% of the time we do not have problems. Every day, however, we do take measures to ensure that he stays healthy. All we have to do is make sure he poops. Easier said than done though! If you’ve ever tried to force a child to poop, you know that it can be tricky. I’ve had days where I wondered how I would get Jett to poop the next day, because I was completely out of ideas, but somehow, the next day would come and I would have a new creative way to get him to poop!

                When he was a baby, he pooped 10+ times a day and all we had to worry about was managing his severe diaper rash (which is a common problem with HD). At 21 months old, however, things changed and he suddenly stopped pooping, stopped eating, vomited continuously, and became very lethargic. He was hospitalized and treated for enterocolitis, a very serious infection that can lead to sepsis, perforation, and death if went untreated. Enterocolitis is the leading cause of death for HD patients.  

                Just today, while Jett sat on the toilet downstairs, I sat on the toilet upstairs and we had a race to see who would push their poop out first. Yeah. I know. It’s weird. And if you know me, you’ll probably never look at me the same way again, but desperate times call for desperate measures! At any given moment, we figure Jett has about 3 days before he will become critically ill and need invasive, traumatic treatments and hospitalization if he doesn’t poop. We will do ANYTHING to keep him from getting to that point, although sometimes it is still out of our hands. If Jett hasn’t pooped, by day 2 his belly is distended, he complains that it hurts, and his appetite diminishes. By the end of day three he is vomiting and lethargic. We never wait that long to take action though. By the end of day 2, we are usually irrigating his colon and manually getting the toxic poop out ourselves. If we have to do more than three irrigations we have to call his surgeon. If Jett is already lethargic, vomiting, and feverish we would need to take him to the emergency room. 

*Jett @21mos. Leaving for Baltimore via ambulance                                                             *Jett @27mos. Being treated for enterocolitis

                

Treatment for enterocolitis includes strong antibiotics, being hooked up to a suction to keep all gastric juices out of the gut, and irrigating the colon. Irrigation, especially when done in the hospital by the surgeon, is hell on earth. I hate to say it, but whenever he has to have it done, although it is very crucial to his well-being, I feel like he is being assaulted. And worse, I think he feels like he is also. It involves a catheter being threaded into the intestine via the anus and saline being pumped through it in an attempt to “wash out” the colon. A dilator (rod) is inserted and, while applying a great amount of pressure on the abdomen, is taken out, forcing stool and fluids to come out. If enterocolitis is present, the stool often comes out in a sort of “explosion,” peppering anything within a 5 mile radius (and the answer is YES! Jett has nailed the doctors and nurses present in the room before!). Repeat multiple times a day. Might I add that several adults are needed to hold him down, and he will not only scream and cry during it, but also for a long time afterwards, and won’t let anyone touch him.  It is because of the risk of enterocolitis that I will do absolutely anything to encourage Jett to poop.

                Through our experiences, I have had countless people tell me how “strong” I am. I cringe whenever someone says that to me, because I know it is not true. I am not strong.  I am weak. However, I have come to rely on the scripture Philippians 4:13 “I can do all things through Christ, which strengtheneth me.” It is because of Christ, because of his atoning sacrifice, and His ability to know perfectly how to comfort and sustain me, that I have been able to continue on, in faith, during difficult times. 

*Jett @ 3 years old

                Am I grateful that my son has this disease? No. Of course not. Am I grateful for the things that I have learned along the way? Absolutely! Perhaps if I could see “the whole picture” I would be grateful for the disease too. After all, I do credit his Hirschsprung’s with saving his life when 3 months old and had severe RSV. His Hirschsprung’s got him to the right hospital, with the right doctors, at just the right time, before his condition became critical. What if he hadn’t of had HD? Would he have received the care he needed in time? I really don’t think so, but that’s another story. What I do know, is although I am weak, my faith is strong. Despite sometimes having walked into the hospital and being unsure if we would walk out again with our son, despite those times of uncertainty, I am continually comforted by the knowledge that families can be forever. Each of us as mothers will face our own challenges and difficulties on this journey of raising our children. May we each take the time to relish in the joy that it is to be a mother, even when we want to lock ourselves in the bedroom because our children are driving us completely insane. If you made it to the end of this post, I thank you! May your days be filled with happy pooping and bum wiping!