The results are in....

We had a loooonnnnng conference with the pediatric neurosurgeon today who explained from head to toe, beginning to end, how tethered cord syndrome manifests itself. He started at embryonic development and ended sometime in adulthood. He reviewed all of the views that currently exist from aggressive surgical repair to wait and see if there is a problem to a mix of both (which is where his opinion lies).

For Cintia, her MRI appears to give us the ability to wait and not cause undue harm for at least the next couple of years . The next couple of years is important and what we needed to feel comfortable returning to Guatemala in October. Worse case should be a couple of years to complete an adoption. It shouldn't take that long but it could. There are several reasons he thinks we can wait, some of which are subjective and cloudy due to her congenital problems (for those who don't know what that means, it means problems she had at birth that occured when something went wrong during embryonic development). Amazingly, God takes two halves of one cell, puts them together, and makes a human being in 38 weeks time (give or take a few). For all of us pro-lifers, we know that in the first 2 months, a small complete human exists that needs only to grow and develop in Mommy until he/she is big enough to live outside of Mommy in our great big world.

Cintia had a time during her fetal development (somewhere between the 5th & 7th week ) when things went wrong. At the same time her colorectal and reproductive organs were forming, so were her spinal cord and neurological connections to her extremities. She had the obvious defect we knew about and more that we could not see. The lipoma at the base of her spinal cord is not normal. It is a manifestation of tethered cord syndrome and is a common presentation for kids with imperforate anus. At birth and in the early years, a lipoma like Cintia's should not cause problems. It should not cause problems until she has significant growth (normally just prior to adolescence...although it can happen earlier). The first major sign is urinary incontinence. He feels Cintia already has a 25-30% chance of urinary incontinence based on her other problems (imp. anus). Also, he informed us that many kids have neurological deficit like Cintia who were born with imp. anus but not the spinal cord lipoma. Cintia's neurological symptoms may be from the spinal lipoma or could be from other causes (or a combination of both). Is the cord lipoma presenting early symptoms or are her symptoms from peripheral neurological deficit that she was born with? (Peripheral meaning that the nerves to her legs, arms, bladder, intestines, and such are not working right due to being formed wrong when she was an embryo.) He did not mention that it could be cerebral palsy. However, it is a consideration as well according to her neurologist. Unfortunately, we know nothing about Cintia's birth and probably never will.

According to him, there are 4 points to consider....3 of which she has some degree of. The first is urinary incontinence....too early to tell for sure but she does show signs of it. Too early means don't operate until we know. She is simply too young and has had major surgical reconstruction in the area that needs time to heal. We are only one year post op. The next is sensory issues....back pain particularly when sitting for long periods of time which she definitely has. However, she has had major repair down low and has plenty of reason to have pain...but most likely that is the lipoma. The other sensory sign would show a change in the level/location of the end of her spinal cord and/or fluid build-up in the canal around her spinal cord. For now, on the MRI, that appears normal for Cintia. As she grows, that may change due to the cord being bound by the lipoma and not able to ascend into the canal during normal bony growth. The normal level of the end of the varies with every person...but all signs now on the MRI say that waiting is not going to harm Cintia...hers is normal enough to buy us time. The next sign is motor/gait problems that worsen over time. We feel that they have although it could simply be her growth and the fact that she has to adjust to it. That means that since she has grown a lot in the last year the neurological deficit that she was born with are more evident but not necessarily worse. That is a tough one to judge. This is a "which came first, the chicken or the egg" kind of issue. We truly don't know. The last sign is scoliosis. He says her spine is straight...her neurologist says it is not. However, the degree that it is curved is still too small to risk surgery at this time.

All of this said, he recommends waiting for surgery for a couple of years. Unless she improves and we feel waiting is indicated until symptoms justify the risk, we will take that advice. The risk of any further deficit for her considering she already has compounding physical issues from the imp. anus and repair would not be in her best long term interest. The part of us that wants to do the surgery now to give her the best possibility of a good outcome does not want to risk surgery until we have to/need to. All surgery has risk and spinal cord surgery has plenty. However, this surgeon feels comfortable that he can do the surgery with reasonable risk. Waiting 2 years or until we get her back here should not cause her anymore harm. The growth or her spinal cord and bony vertebrae should be in sync enough to not cause significant stretching in that amount of time. At some point, closer to adolescence, her bony vertebrae will grow faster than the spinal cord. At that point, the risk is much greater and her cord could stay bound and stretch instead of rising into the vertebral column. That would cause nerve damage. We want to get it released by surgery before that time since she is already symptomatic. Does she have stretch that can't be seen on the MRI now? Possibly, since the level of the cord varies for everyone. Again, the MRI told him that we are still in the safe zone based on her spinal cord level even given that possibility. The progression is slow enough that we can still wait.

I know this is confusing but it was very good to have someone spend that amount of time with us. He gave us an anatomy lesson and a professional opinion...taking great time to do so. We both feel comfortable letting him do the surgery when we get home with Cintia. We both feel better knowing that we have not harmed her by waiting this long...honestly we were worried we had. Funny how having no health insurance affects how you think and how hospitals and providers think. With insurance, she would have been tested when we got her. Apparently, even with an earlier diagnosis, waiting would have been acceptable and probably advisable.

As for the visa issue, this will not be our ticket home. However, Cintia's well being is okay and we rejoice in that. If God wants us to bring her home in October, He will get the visa for us another way. If He has other plans in mind, we will go where He wants us. God is good and we trust that our plans don't compare with His. He will take us on a grand adventure either way. No worries....just faith. Grandparents....relax. Everything will work out perfectly.

Next update I will share the news from Guatemala and a picture of some of Cintia's best fellows!

Blessings!
April