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.

Excellent Video

Here is an excellent video of doctors who are participating in the MOMS study explaining spina bifida (myelomeningecele). The MOMS study stand for Management of Myelomeningocele Study  and is in the US that is looking at the effects of prenatal surgery to repair the lesion. It has been going on for many years.
Pretty much at 25 weeks the mom (and baby) undergo surgery that would have been done on the baby within the first day or two to close the back. If you participate in the study you are randomly selected to either the postnatal surgery or the prenatal surgery group.

I found a video that is really excellent in explaining a myelomeningocele and everything that goes with it. If you are interested - please check it out!
http://www.chop.edu/video/moms/management-myelomeningocele-study.html?item=1

This video explains how the postnatal repair is done - this is the surgery that is done on every baby that is not participating in the study (as well)
http://www.chop.edu/video/moms/management-myelomeningocele-study.html?item=4

This video explains - and shows video - how the prenatal surgery is done. These pictures are amazing! It also reviews the risks and benefits of prenatal surgery
http://www.chop.edu/video/moms/management-myelomeningocele-study.html?item=5

Learning About SB: The Repair

I have a lot of people asking me how they fixed his back, but I didn’t really know how to answer. Then I found some pictures.

When you are pregnant, you wonder about what will happen in those first few days after he is born. And the answer is major surgery. A way to fix his back, this surgery will fix his back, but will not fix the nerves. In fact that first surgery can actually damage the nerves – that is one of the risks. But really there isn’t a choice.

A baby that was born with spina bifida has part of their spinal cords outside of their body and that needs to be cared for and repaired. It is called a myelomeningecele repair (MMC repair for short). Nickolas had his first major surgery when he was less than 24 hours old, and this surgery lasted almost 6 hours. I didn’t really know how it was repaired other than they put the spinal cord back in and covered it with muscle and skin. Well I found a couple of good pictures and thought – huh – this is how it was repaired (you learn something every day). Even though I had an idea, it was helpful to see pictures.

I do not have a picture of Nickolas’ lesion, I never thought of it at the time and wasn’t able to visit anyways (the joys of H1N1). There are pictures you can view online, some are large, some are small. But really it only looks like that for the first 24 hours and then it is repaired.

Nickolas has quite a big scar. and one part of it got infected, so it didn't heal very well, he stayed on his belly for 6 weeks until it cleared. And that part now has an indent of scar tissue when it healed. I think his scar looks beautiful now. I’m amazed at the work of the doctors, and his plastic surgeon.

Pictures came from: http://www.seattlechildrens.org/medical-conditions/chromosomal-genetic-conditions/myelomeningocele/