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*The following story was shared with us by Giana's mom, Geng
Giana was diagnosed with a rare case of Hirschsprung Disease and had an Ostomy surgery at 3 days old. We were devastated to find out the defect affected her entire colon and an immediate surgery was our only option in order to save Giana's life. In Giana's case, the treatment required more than one surgery, delicate home care procedures and regular doctors appointments. I just wished that there was an established medical center or facility for colorectal health and ostomy care and management that could have provided an all-inclusive service in the Philippines where we live because little did we know that the post operative home care and Ostomy management would be very difficult and traumatic for Giana.
Hirschsprung Disease is a congenital defect of the intestine that causes obstruction. It occurs approximately 1 in 5,000 live births. In Hirschsprung Disease, special nerve cells called Ganglion cells that help the intestines squeeze with a wave-like motion to move stool along the digestive tract are missing typically, in the bottom segment of the colon. But in rare cases (about 5% of the cases), ganglion cells may be missing in the entire colon and part of the small intestine. Hirschsprung Disease is a life-threatening illness and treatment requires immediate surgery.
Giana was born on May 03, 2015. It was observed that she was unable to have her first bowel movement within the first 48 hours after birth and her abdomen was beginning to swell. Immediately, a colon X-ray called Barium Enema was performed by injecting a liquid into her rectum through a soft tube to examine her colon. She cried during the whole procedure and sounded like she was in so much pain. It was so hard to see her in that state.
The colon X-ray and biopsies confirmed Hirschsprung Disease, her whole colon was affected. Initially, we were presented with a staged repair plan consisting of at least 3 to 4 stages of surgeries with 6 to 12 months intervals in between surgeries. The 1st stage called Ostomy surgery was performed right away, where a healthy part of her small intestine was brought out to her abdominal wall creating a temporary passage for her stool. The temporary passage is called a stoma. She needed to wear a pouch/appliance outside her body for stool collection up until the defect was restored. The daily post operative management at home became very difficult for her.
We worked closely with our doctors in the succeeding months to ensure we cover all options before Giana was supposed to undergo the 2nd stage - the removal of her entire colon. We were afraid of the potential long-term complications of this illness, specially without her whole colon. Our doctors ordered a more in depth biopsy of all 27 slides of sample tissues previously extracted from different parts of her colon to determine presence of ganglion cells. These were the same slides that were initially evaluated during the Ostomy surgery where no ganglion cells were seen. But God was on our side! We were so thankful because this time around, all results came back positive for ganglion cells. It was truly a miracle and this gave us so much hope for a better future for Giana.
Consequently, this changed and shortened the course of our treatment plan. Her colon was not removed. Our Pediatric surgeon recommended we do the pull through surgery or the closure stage next. But in preparation for the closure, he prescribed Giana to undergo a colonic irrigation 2 times daily within the next 3 months. I had to give this at home so I needed to get some training on the said procedure. I was scared the first few times I was giving irrigation though our surgeon assured us that the procedure would only cause Giana some discomfort.
However, in the course of the irrigation phase, we were again faced with another problem when Giana was diagnosed with a type of imperforate anus, called Rectal Stenosis, a stricture in the rectum. This had to be repaired first either through anal dilation or through a surgery called Post Posterior Sagittal Anorectoplasty. We opted to do the former since it is non-invasive; it would spare Giana of undergoing too many surgeries. Anal dilation is done by inserting a metal instrument called Hegar Dilator into the anus to slowly stretch the stricture in the rectum. The size of the dilators is increased on a weekly basis until the recommended opening size is reached. I had to get some training on this as well as this is a very delicate procedure. However, during the first month, I noticed Giana would be in so much pain during the procedure, and somehow it didn't seem to help open up the stricture. Again, we wanted to spare her from the trauma of a long painful procedure so we decided to just proceed with the surgery (considered 2nd stage). Two weeks after the surgery, we resumed with anal dilation as an after care procedure to help keep the opening in the rectum to the right size. We did it diligently, 2x a day everyday for another 4 months. This time, we knew that it was not as painful as before though Giana still cried during dilations.
Finally, on August 16, 2017, the pull through/closure was completed. The ostomy was closed and her colon was reconnected to the other section of her healthy small intestine. Post surgery was again very difficult as she experienced diaper rash and severe maceration due to diarrhea. She cried at every bowel movement and every diaper change. But overall, we consider the surgeries successful.
The complexities and post operative management and Ostomy care needed at home were the most difficult, demanding and enervating challenges we faced every single day. The whole process was overwhelming -- from changing and cleaning pouches, to peristomal skin irritations due to leaks, to watching her diet, to bleeding stoma, to giving the colonic irrigation and anal dilation. The first few months were the most insane and emotional part for me. We encountered problems of leaking since her stool was often loose that it caused severe painful irritations and lesions around the peristomal skin. Literally, she would cry for hours and I wouldn't know how to comfort her. Unfortunately, there were times when even our doctors could not provide help for us. I felt helpless, there were times I just wanted to give up. But I decided to do my own research about Hirschsprung disease and eventually, I found a local support group who extended their help, gave advices and provided solutions to our problem.
However, 5 months after Giana's operation, I, myself, had an upper cervical spine surgery and was hospitalized for a month leaving her under the care of my old mother and my younger sister. Good thing I was able to teach them how to change and clean her pouch earlier on so they were able to do it while I was in the hospital.
It has only been a few months since the final surgery, Giana is now 2 years and 7 months old. I do not know how she was able to put up with all the pain and those medical procedures but she had been amazingly brave and strong all throughout her journey. No more pouch hanging outside her body, no more bleeding stoma. I cannot say that her bowel is already functioning normally at this point. Her stool is still generally loose to semi formed and sometimes comes out 5 to 6 times in a day. We are currently discussing a bowel management program for her with our doctors. Also, the whole experience was very traumatic for her not to mention those numerous rectal examinations she went through. At around 7 months old, she would cry at the sight of doctors/medical staff in white coat or blue scrub and was fearful to enter hospital/clinic premises. But she is slowly forgetting about the experience and I know that she will eventually overcome the trauma.
The surgery left a three-inch incision mark in her abdomen. We call it her victory mark. She is our brave little warrior.
Be unrelenting in your faith. Faith moves mountains, indeed!
Be inquisitive. While it is important to find a doctor whom you can trust and are comfortable with providing care for your child, it also helps to understand and learn everything you can about the illness of your child. Do your own research, ask for a second or third opinion if you deem it necessary. Look for organizations or advocates for the same illness or a support group where you can seek help, advices or meet other parents who have gone through the same situations. It is comforting to know that you are not alone in your battle.
Be sensible and objective as much as you can. I know in situations like these it is hard not to get emotional but we need to have a clear and sound mind to be able to make better judgement and important decisions pertaining to our child's medical condition and needs.
Be prepared. Establish a functional support system at home specially if you are a hands on mom like me. Train your spouse, your nanny or at least one or two members of your family on Ostomy care and management. They will come in handy during emergencies or when you just need some time alone to rest, distress and recharge. Remember, you need to take good care of yourself too so you can better take care of your sick child and family.
*You may reach Geng through her email firstname.lastname@example.org and FB account https://www.facebook.com/gengoteyza. She posted several helpful and very informative links about Hirschsprung Disease and more details about their journey on her timeline.