January 11, 2023 "Surgical Consult"

95% of people living with Spina Bifida have neurogenic bowel, the loss of normal bowel function. It is caused by damage in the spinal cord nerves that help control the lower part of the colon. These injured nerves affect the ability of the GI tract to function properly, leading to symptoms of incontinence. We have dealt with this issue Rayden's entire life. The solution for many years has involved prune juice, MiraLax and nightly Peristeen enemas. We have done a very good job for 8 years preventing any rectal prolapse or blockages, but lately our efforts have not been too successful. Dr. Ross said it was time to discuss a surgical option, not only for better function, but for Rayden's independence as he gets older. 

We had an initial surgical consult with Dr. Michael Phillips for the MACE procedure. MACE stands for Malone Antegrade Colonic Enema. In this procedure, the surgeon creates a small passageway (conduit) between the skin of the abdomen and the colon (large intestine). This opening would either be at the belly button or over to the right. The passageway can be made two different ways, from the appendix (Appendicostomy) or a small piece of the bowel (Cecostomy).  

An appendicostomy, better known as the Malone Procedure (MACE), is a surgery that creates a pathway between the large intestine and the abdominal wall using the appendix. The appendix is a small tube-like tissue structure that extends off the first part of the large intestine (the cecum). A stoma plug or a tube device might be placed through the appendix pathway. This allows direct passage of irrigation fluid into the large intestine.

A cecostomy is a surgical procedure wherein a channel is created between the abdomen and the first part of the large intestine (the cecum). A tube device is then placed directly into the stoma. This allows direct passage of irrigation fluid into the large intestine.

A tube is inserted in the passageway with a one-way valve. This will keep stool and bodily fluids from coming out from the belly. He can then insert the enema liquid at the beginning of the colon, instead of the end through the rectum. 

There are two types of tubes, the Chait tube or the MiniAce. Both tubes have a trapdoor that remains on the outside of the abdomen. It is easy to swap between the two tubes if one works better than the other. 

The Chait tube is self retaining. It has a long tail that curls to prevent the tube from coming out. This long tube makes it harder to do flushes though. It will need to be changed in the clinic every 6-12 months.

The MiniAce uses a valve that prevents less leakage. It has a short tube to make flushing easier. It uses an inflated balloon to hold it in place. We would deflate it to do the flush and then re-inflate. It would need to be changed every 3 months. 

Of course, our biggest concern is what could go wrong. 

1. The biggest complication is infection. The skin will be connected to the intestinal tract, so the risk of infection is around 5-10%. In most cases the infection would just be superficial, responding well to antibiotics. 

2. The trapdoor could get snagged on a seatbelt, or other things, causing it to get pulled out. A new tube would need to be inserted at the clinic. This makes us worry about his twister cable belt. 

3. Long term complications would be if the passageway becomes tight resulting in challenges with tube changes. This would require a surgical revision. 

Due to Rayden's past complications, Dr. Philllips recommends antibiotics for 24 hours prior to surgery and after, instead of the normal one dose after surgery. This would be a 2-4 day hospital stay. 

I love the way Dr. Phillips talked to Rayden, ultimately it is HIS body anyway. Rayden really didn't warm up to the idea of having something permanently placed inside his body. I know I'll have to explain it to him and remind him of his shunt and tube that have been there since birth. These kinds of decisions are so difficult. Ultimately we want to do what's best for Rayden, making his life easier to manage giving him more independence. Since this is not an emergency we have the luxury of waiting until it is more convenient for our family. So we have time to pray, discuss, research, and decide when the time is right. 

December 25, 2022 "Half-Kickin' Shot Gun"

All Rayden wanted for Christmas was a long gun that didn't kick like his Daddy's. He called it a "Half-kickin' shot gun".  His favorite color is red so this needed to be in the mix. Every single time he told Santa (or anyone else) what he wanted, we had to explain it. Santa got it just right! A red, bolt action, Rascal 22, with a braided red and black strap. It even had a duck feather on the end. He also got some 22 shot shells. When he came into the living room on Christmas morning he went straight to the gun and was tickled to death! He looked around and asked, "Why did Santa bring me all this other stuff?" Rayden is not one bit selfish or stingy at all! He's so precious.  

Michael and Papa took him outside after brunch to 'hunt' birds. When he killed one, I couldn't tell who was more excited!! Three days later, Michael took Rayden duck hunting with his new gun and duck calls. We had one happy boy!! 🦆🦆