What is the MACE

When I have been posting about Nick's surgery I have been trying to link back to what the MACE is.
I do have it described within the blog, but no one post that says "this is what Nick had done".
So this post is all about what the MACE is.

MACE stands for Malone Antegrade Continence/Colonic Enema (or just plane Malone) and it is surgery.

Malone is the guy who perfected the procedure, Antegrade means moving forward, the C stands for Contience, which is the goal or Colonic which is the part of the body (colon) involved, Enema is introducing fluid to clear out the bowels of poop.

So how do you actually do this? You use the appendix. That part of the body that no one knows what the purpose is. It has a purpose when spina bifida is involved. It is tubular which is perfect for put a catheter through and into another organ like the colon (it can be used to go into the bladder as well in a surgery called the Mitronoff, but can only be used once).

So the MACE uses the appendix to move an enema solution forward through the colon to provide continence and prevent constipation, in a way that provides easy access for the individual and leads to independence.

So how is this done?
First some biology. The appendix is attached to the colon. The colon, also known as the large intestine is where poop has difficulty moving, moves slowly and constipation is when the colon fills with poop that doesn't move and clear out. And the end of the colon is the rectum and bum.

Our goal for continence with spina bifida is to clear poop out the colon and be able to time when it is filled again. This allows a clean colon during the day hours so there is no poop to leak out (incontinence)

A traditional enema puts in fluid through the bum to work it's way backwards through the colon, collect all the poop and then poops it out. An antegrade enema moves fluid forward through the colon and poops out the bum. It doesn't need the time or fluid amount to works it's way up through the colon, because it starts at the beginning.

The MACE surgery involves bringing the appendix to the belly button to create a stoma or a surgical opening/hole. Usually this hole is hidden in the belly button. It is tightened inside so that fluid can go in, but nothing drains out.

A catheter goes in through the stoma, through the appendix and into the colon. Fluid goes through the catheter and into the colon flushing it forward and out.
This pictures shows the stoma in the belly and not the belly button. It shows the ileon (small bowel), colon, appendix, stoma and catheter. (all labelled in ?Spanish)

A foley catheter (latex free of course) is used, which has a balloon on the end to keep it in place in the colon while the fluid is going in. At the end of the flush the balloon is deflated and the catheter comes out. Leaving the stoma hidden in the belly button.

So that is the end result. But first is the surgery. It takes about 2 hours under general anesthetic. They can do it laproscopically, which means there is no large incision, but a smaller incisions (with 2 stitches) that allow the doctor to work. It won't interfere with shunt tubing either.

With laproscopy, there is no large incisions, less pain and a quicker recovery. And less of a hospital stay. 

We stayed in hospital 2 nights/3 days and were ready to be discharged home. By the time we went home Nick didn't need any more morphine, and just using Tylenol/Advil as needed (But he did need them the first night). The hospital stay and recovery is different for every person and every surgeon. It depends on techniques, complications and pain control. The more invasive the technique, the longer the recovery and the more the pain.

For the recovery Tylenol and Advil work at home. And a foley catheter is left in place to keep the stoma open and let it heal. (The stoma is like an ear piercing and will try to close if you don't keep in place or pass a catheter through it regularly).

There is some of patient information available on the internet.

This is a patient education package for patients from Urology department in San Fransisco. It has pictures and describes the procedure and recovery. It is very similar to the package that we received when we left the hospital gave to us prior to discharge.

I found another pdf document that talks about parent experiences and opinions with the MACE. It was very helpful for me to try to figure out what to expect and what complications there could be and also the opinion of those who are actually living with it.\

So that is the MACE. There are other options out there, some are surgical and some are non-surgical.
We worked our way up to surgery, and did not make the decision lightly.

Sharing my take on Bowel Continence

*Updated with some parent resources*

Trying to become socially continent is a journey and a challenge. There is no right answer, there is just a lot of trial and error as well as a progression through various steps. This post isn't about constipation - that would take a whole different post. It isn't about consistency of poop, but about consistency in timing.

I call the bowel a stubborn teenager that yells "I won't do it! You can't make me!" And folds it's arms and stomps its feet until you wear it down but doing the same thing over and over again.

So anything that you doesn't happen over night. To say that something was a fail means that you tried it for weeks before saying that it didn't work.

My knowledge comes from my own experience and what I have learned over the last couple of years. And it is by no means extensive.

We progress through the poop steps of least invasive to most invasive.
There is wide ranges to spina bifida and neurogenic bowel, so there would be wide ranges to the ability to become socially continent.

• Diet - I had a great dried fruit combo for baby food that kept Nick regular when he was a baby, but this was more about keeping him from getting constipated than getting him clean. Because as a baby diapers is expected. 
• Staying away from cows milk is also a biggy in our house. For milk we use almond milk or goats milk. Goats milk is easier to digest than cows milk and less constipating.
• Oral laxative - PEG flakes (Restoralax) we give every morning, in oatmeal, it is quick and easy, doesn't have a bad taste. We can adjust it up and down depending on what we need. Right now we are at 1tsp every morning (but have gone up to 3Tbsp when we really need it). And as I've heard there isn't a maximum dose.
• We have tried some other medications like lactulose (didn't like it, didn't work, it was a liquid and he needed to take it too often for it work into our lifestyle)
• Senna we haven't tried. I've heard very negative things about senna and want to stay away from it for now. I've heard that it is habit forming, which doesn't really make sense because the neurogenic bowels need the habit.
• Omega-3 and probiotics, we used this as a supplement for a while and it made a huge difference, but we still needed the restoralax. We are on a break right now, but I find it makes Nick smell like fish for a while. Will probably start it again soon
• Stimulation. You are stimulating the muscles to work. This can happen by gently pressing around the anus
• Suppositories. Again medication. It seems like this has a couple of steps
• Glycerin is the first step, but we found it didn't do anything to help.
• Ducolax is a medicated suppository, the medication in it also stimulates the bowel to move. This has been great for preventing or keeping him from getting constipated, and when we do it every day or every other day we get things moving, but I found he is leaking all the next day. So it just isn't working for getting him clean.

• Enema. This is using liquid (with or without something added to it) to clean out the colon more completely than the suppositories will do. The more comes out at one time with the enema the less that can come during the day. This is a retrograde enema - so it works in the colon from the bottom (literally) up.
• There are different types of enemas.
• A regular tip enema, but I found that using this (like a fleet enema) doesn't work. You have to have the muscle control to tighten around the tip, which Nick does not have, so the fluid doesn't stay in, which is the whole point. And just squirts right back out. So this hasn't worked for us.
• A cone enema, is the step we are currently on. It is typically used for cleaning out colostomies. And it is cone shaped to fit into the hole of the colostomy, but we are using it for a different reason. The cone shape means that we can create a stopper without the muscle control. So the fluid that goes in, stays in to work, and when the fluid comes out, so does all of the poop in the colon.

• A catheter type of enema - this is newer and more expensive. But it works to do the same things as the cone. Instead of the cone shape it is a tube with a balloon at the end to keep the tube in place and keep the water from coming out.

 

• Once all of the fluid has been put in, the tube or cone or whatever comes out. This way the bowel is clean as far up as the fluid can go.
• Surgery. This is the final step. There are 2 different types of surgeries, it takes the enema solution and starts at the top of the colon, (called an antegrade enema) around where the appendix is, and cleans the colon from that point and down. It seems to me to be the most effective way, but also the most invasive - because it involves surgery.

• MACE
• What the MACE stands for is Malone (the guy who discovered it in the 80's) Antegrate Continence Enema. So they take the appendix (which is already attached to the top of the colon) and make it so that it connects to the belly button, so that a tube can go into the colon and put the enema solution from the top and clean out the entire colon. It is a laproscopic surgery, it will not interfere with the shunt tip, he says there is a quick recovery from it. On the outside the belly button will just look like an inny (this is how our urologist explained it - I tried to find some pictures to see what the results actually looks like, but I can't fine one). There is something about a flap or something that comes down - so that things don't back up the wrong way.

You need to have an appendix to have this as an option.
This is a chapter in a textbook. It has pictures
http://www.biliaryatresia.org/reference/Malone-procedure.pdf

A helpful parent information package is available http://www.urology.ucsf.edu/sites/urology.ucsf.edu/files/uploaded-files/basic-page/ace_malone_2.pdf

• Ceacostomy
• The other kind of surgery isn't really surgery. It is a ceacostomy button. It is when they take a tube, and through radiology guided the poke the tube from the belly into the colon. Then the tube stays in, and on the outside is a button that stays closed when you don't need it. This is just like a g-tube. If it not needed it can be removed.

The urologist was not very positive about this (but he is a surgeon that does the MACE, so I'm not surprised).
The button seems less invasive, but if we are putting something foreign into the body it can get infective, whereas the MACE is more invasive but less likely to get infected or have the body 'reject' it.

There is another parent information package available
http://www.hamiltonhealthsciences.ca/documents/Patient%20Education/CecostomyTubePORTRAIT-lw.pdf


This is so far the extent of my research to share. Other sites I found useful are:

http://www.mededmasters.com/anterograde-continence-enema-ace-procedure.html
http://addberbe.hu/templates/ja_zeolite/images/cecostomy-picture
http://www.docstoc.com/docs/93351272/Information-for-Malone-MACE-Appendicostomy