Monday, October 31, 2011

Spina Bifida Awareness

October is Spina Bifida awareness month. Yes, I know it's October 31st, but I wanted to get at least one post out there about it.

Spina Bifida Awareness to me, is about making people aware that Spina Bifida is not a terminal condition. That people living with SB, can live long fulfilling lives just like the rest of us.

This is our face of Spina Bifida.

I want to share a quote a fellow SB Mom friend of mine posted on Facebook.

Spina Bifida shouldn't be something scary, sad, foreign, or feared as one would fear a death sentence. And yet every parent who is hears "I'm so sorry -there is something wrong - your baby has Spina Bifida" feels those things. Every pregnant mother who is left with the decision on whether or not to terminate her pregnancy... based on a horrifying description of the "poor quality of life" their child ...will have - feels those things. And it's not only because we are overwhelmed with a diagnosis we didn't expect - it's because we are UNAWARE. We are uneducated, unprepared, unaware of what Spina Bifida really is. What it looks like. What it means for our children. What it means for our futures. We overlook what it CAN be because of what we FEAR it to be. Don't let Spina Bifida continue to be like a ghost in the shadows - shine the light on Spina Bifida. Be aware of the research surrounding it, the miracles born with it, the people who live with it, and the many who strive to REDEFINE it. ~Joanna Penny

I also want to share a post a mom left on a Spina Bifida support forum I belong to. She was given misleading information about her unborn baby, and was lead to believe she had no other option but to terminate. To hear her story, her regret, is heartbreaking. She was given encouragement to get help from the other mothers on the board, and I am proud of them.

I am posting a screen shot of it here, for any mothers that may stumble across this blog in the same position, that they may question those doctors and follow their heart. For my friends and family, it is not for the faint of heart, so please keep that in mind before choosing to read it.

Sunday, October 23, 2011

Neuro Update

So, as I talked about during my last post, Ruth's MRI report from her MOMS trial follow up in San Francisco showed some concerning areas.
  • The quarter size hole in her skull he made to perform the ETV has not closed, in fact the MRI shows there is fluid built up and the bone actually has a slight upward lip to it, indicated there is some pressure.
  • Her ventricles are still enlarged.
  • She has syrinx (build up of fluid) in her spine, and her spinal cord has become tethered to scar tissue at the bottom instead of floating freely.

We expected these things meant a shunt was likely in her future, although we did not talk to a neurosurgeon while in San Francisco, we did already have a scheduled appointment with her neurosurgeon back home the week after we returned. Rachel was nice enough to overnight all the reports and MRI disk (since we had to leave too early to wait for them all), and I called her neurosurgeon nurse and spoke with her. She said as long as Ruth is acting fine there is no reason to bring her in sooner than her scheduled appointment (a week out).

So, the day of the appointment we were all but certain her doctor would be having us schedule a shunt surgery. We were pleasantly surprised! Her head circumference is back on the normal growth curve, and her ventricles are SMALLER than her last MRI before her ETV surgery. He was not happy with the fact the bone has not healed, but unless it gets worse, he can "live with it" like that. So for now, the main things we are watching for is her head circumference to start increasing too fast (go back off the normal growth curve), and the fluid to start bulging out of the opening in her skull. He didn't feel like she would exhibit other symptoms of hydrocephalus, since it is mild and has a very slow progression.

I asked him about the syrinx and tethered cord. He said unless it's causing problems, they do nothing about them. So, that may mean a surgery a few years down the road, or next year, or never. Another one of those wait and see things.

We will continue to watch her closely, and she has a scheduled "rapid" MRI - with no sedation, and a follow up appointment in January. Please continue to pray for no surgery! It is working!

Here is the only photo I took (with my phone) during her appointment. Ruth was sitting in my lap and I was holding my phone out trying to grab a photo of her, and her Daddy decided to be a goof in the background. So, I'm sure he will love that I am posting this, but that's what he gets!! :-)

Sunday, October 16, 2011

MOMS Trial 1 Year Follow Up

Since we participated in the MOMS Trial (for fetal surgery for Spina Bifida), we traveled back to San Francisco for scheduled follow ups. The first one is at 12 months, which they calculate from her due date, and since she was born 2 months early, fell on the end of September.

I already blogged about the fun stuff we did, so here is the rest. It was very emotionally draining for me, which is probably why it has taken me so long to blog about it. I didn't expect all the emotions from last summer to come rushing back to me. It was incredibly hard being back at the hospital there.

I dragged my big camera around with us, but never took it out of the bag. I did, however, take a few random shots with my phone, so I will share those. Forgive the poor quality.

DAY 1:

The first day consisted of a physical exam and developmental exam. Both of those combined took up the entire morning (4 hours!). We then had a short lunch, and she went for a renal ultrasound and back x-ray.

I wrote in my journal about it, so I will share that:

Long day at UCSF. It was nice to see Rachel again, not so nice to be back at the hospital.
The first thing was the physical exam, he tested her vision and hearing. He let us show him that she could crawl, and after we got a lower box, pull up.
He poked her legs with a sharp toothpick to see where she had sensation. He tested her leg movement. Felt around on her belly and tested her bum.
He went over the results with us. He said she has l4 level of sensation since she can't feel the outside of her calves. She has s1 level of motor function since she can wiggle her toes, but doesn't have much ankle movement. Nothing abnormal was noted about her bladder.

The next thing was the developmental exam (baby SATs). She worked really hard, she stacked blocks, played with cups, and did really well with most of the occupational tests. She did not like the tester taking toys away from her. She was soo tired by the time they got to the physical part she didn't really listen, but we did get her to pull up on a small chair. Both exams combined took 4 hours.

We ate lunch then headed to the renal ultrasound. She had the same tech that she had in the nicu! That was neat. After that we headed upstairs for the spine xray. Ruth was so done, we headed back to the hotel and she napped.

We walked around some shops after she woke up and then took the train to eat at one of our favorite places by the family house.

A little about the levels mentioned. Spina Bifida effects every baby differently, depending on where on the spine (the level) the opening was, and what nerves at that opening were effected. Ruth was diagnosed with an S1 opening during pregnancy, but after birth, it was determined by MRI she had an L5 opening, which is worse/higher.

She was given an L4 sensation level. L4. That is worse/higher than where her defect was located. That was very discouraging to hear, but.. we knew she didn't have much feeling in her lower legs. She does have SOME, but I'm guessing not enough for the examiner to count for trial purposes. Her right leg is better off than her left leg, and she can feel when you touch the bottom of that foot. Her left leg is a little worse off, but not terribly.

She was given an S1 motor level. That is BETTER than where her defect was located. She is trying to walk, pulls up, crawls, can wiggle her toes some, and have a little ankle movement.

The developmental exam was exhausting. Ruth got to sit in my lap during the testing, but we couldn't react (laugh, encourage, clap, etc) at all. It was hard, and it was hard on her. She worked really hard at all the tests they laid in front of her, but did NOT like the fact that the tester kept taking the toys away from her! The put the physical part of the exam at the very end, and luckly we were able to encourage her during that part. She was SO DONE by then, and it took a lot for us to get her to show them what she could do (pull up, cruise, climb over things, etc). She did pull up, but refused to cruise, and climb. She kept crawling towards the door (let me out of here!). Poor girl. We did not get the results from that test, but I feel like she did pretty well. She was able to do most of the cognitive tests pretty well. She did not show off her physical abilities too well... but oh well.

I had to remind myself those test have no bearing on Ruth. It is for the trial, and really doesn't mean anything for her. She is who she is, she is going to do what she is going to do, one long morning with random people judging her, well it doesn't mean much. Of course, I want her to show off... But, I tried not to dwell too much on it if there was something she couldn't, or wouldn't, do for them.

DAY 2:

The second day was the urodynamics (study on her bladder), and full head and spine MRI. Since the MRI required sedation, she couldn't eat anything after midnight, and couldn't drink anything after about 8AM. She handled that fine, tho.

Here was my journal entry for the day:

Today was the final day of the MOMS trial follow up. We met Rachel at the fetal treatment center, and she walked us up to urodynamics. We waited a good bit for that to start, enough time for Ruth to get really tired. They took her weight and height (19ish lbs and 28 inches). Once they got us in the room, they had me prep her (empty her bladder and insert the probes). She started to get upset so I put her blanket close to her face and she calmed down. Soon after she fell asleep, and slept through the rest of the test (thank goodness). The test showed she has a normal bladder capacity for her age, and a smooth bladder. It also showed she has a closed bladder neck. All of those things are great for future continence. The reflux that was present last year was still there, but not until her bladder was very full. The doctor was running the test, and it was great to be able to ask him questions. I left there feeling pretty good.

We left there and went back to the fetal treatment center to answer more questions for the trial. Once it got close to 10, Rachel walked us to the mri. We waited, and they finally started at about 11:20. Not too happy about that since Ruth was npo, but she handled it like a champ. She screamed and fought the gas mask to put her under like crazy, which broke my heart. I left her in tears. They told us "about an hour". After 1.5 hours, we had them call back, and was told "another 20-30 minutes". All in all it was almost 3 hours before we saw her again. (They told us the machine kept over heating. Comforting, huh?) It took her a long time to wake up. I nursed her for a while, then headed back up with her to the fetal treatment center to finish the trial questionnaires. She was very grumpy, but we got through it. Once Matt got the disk he joined us, and Rachel told us the radiologist was very concerned about the fluid around the opening in her skull from the last surgery. We knew it was a possibility, but still disappointed. A shunt is likely in her future. He did say he saw the ETV still functioning. We see her nuerosurgeon next week, who knows what he says.

Ruth was very grumpy so we hung out at the hotel for a while before going to eat supper. None of us had eaten, so we were all glad to finally go eat. Ruth made it most of the meal before getting really upset, so I calmed her down outside the restaurant.

She did better once we got back to the hotel, and we all had an early bedtime. So ready to be home.

Photos from before/during the urodynamics. Poor girl was EXHAUSTED.

Photos from before/after the MRI.

A follow up to my note about the MRI - Ruth has since seen her neurosurgeon at home.. it has taken me a while to write about this. I will probably blog separately about all that has happened medically since we got home, so keeping this post from becoming a novel, but as to not worry anyone, Ruth does NOT need surgery at this point. In San Francisco they do not do ETV surgeries for hydro in spina bifida, and the radiologist did not have her most recent MRI to compare against. It does appear Ruth's ETV is doing it's job for now.

One of the reasons we chose to participate in the fetal surgery trial was to hopefully give her a better outcome. Did we? I think so, but it is impossible to know for sure (thus why it was a medical trial, they look at a bunch of kids and the overall outcomes). The two areas the early results from the trial show are (1) better motor abilities and (2) less need for a shunt. Ruth definitely gets around well. She has done everything on track besides walking, and she is getting there. Ruth did not escape the hydrocephalus however. BUT, it is a milder form. Had we not done fetal surgery, she would have likely needed a shunt right away, and had MUCH more fluid draining than she does now. Fetal surgery may not have cured her hydro, but it definitely made it less severe, and thus, she was able to have an alternate surgery (ETV) instead of a shunt. That could change, and very well may, but I still feel we made the best decision for her.

Is fetal surgery the right choice for every baby diagnosed in utero with spina bifida? Definitely not. It is very risky, I know at least two babies have died during the fetal surgery since it left trial, which is absolutely heart breaking. It can also have negative effects on the woman's ability to have more children, since scarring and weakness in the uterus is a possible side effect. Ruth was born premature, but not dangerously so, and we were lucky there. She has no side effects from prematurity.

If we knew then, what we know now, would we have the fetal surgery again? YES, definitely 100% for sure. I have no regrets. It was definitely the harder choice, but I am forever grateful we were given the opportunity, and would do it all again in a heartbeat.

Thursday, October 6, 2011

San Francisco Trip Day 1: Friends & Fun!

We went to San Francisco last week as part of the MOMS trial follow up. They look at the kids that are participating in the trial at 1 year (from their due date), and at 30 months. I want to blog in detail about the follow up, but this post is about our first day there - our free day!

We flew in early Sunday morning, so we would have time to meet up with our friends. They drove from over 2 hours away to meet us.

I missed them so much, I am not sure I would have made it through that long month in the NICU without J!

Ruth & B last summer:

After grabbing lunch, our first stop was the Golden Gate Bridge, I got to drive over it for the first time (last time I never had a car - we always took public transit to get anywhere).

A few photos, of course!

It was a lot of fun seeing the babies together again! And of course, seeing J was awesome.

Next was a stop at the Hospital. This place was our lives for a long time.

Top Floor - NICU. A lot of long hours spent there.

More cute Ruth & B photos!

Kissing babies!! Not much is cuter than that :)

Of course, we had to grab some YUMMY chocolate chip muffins! Oh, how I missed these!