Saturday, October 31, 2009

Spina bifida 101

We've been home for two weeks now, and are loving every second of it.  We're still amazed at how nice it is to have our own kitchen and bathroom, ceiling fans, TV, cars in the garage (as opposed to parked six or eight blocks away), and the list goes on.  Mostly, we are enjoying having our family all together in one place.

Kaitlyn is now five and a half weeks old.  Next Friday, the 6th, would have been her due date, and she is determinedly packing on the pounds.  There's no way she'll catch up to Damon's pace (17 lb at 2 months!), but she's doing pretty well.  At her last checkup on October 20, she weighed 6 lb 6 oz, so she had gained about an ounce a day since she left the hospital.  At that rate, she's probably around 7 lb today.  She's a very good baby, calm, has her alert times, and is occasionally smothered by her brother and sister.  A few pics are posted below.

We wanted to give a quick summary of how she is doing in each of various areas that are commonly affected by spina bifida.  (Tamara, Rachel, and any of you parents out there, hopefully we're not too far off in how we describe things below.)  We'll try to do the same thing every so often down the road, just so you all can get a picture of her status as compared with what one might expect.
  • Hydrocephalus:  Up to 90% of children with spina bifida have some degree of hydrocephalus, or fluid buildup in the brain.  Kaitlyn technically has this -- her ventricles have measured a little high (see here) -- but it is not symptomatic.  We're watching her carefully for symptoms like excessive vomiting or fevers, measuring her head size, making sure her fontanels don't feel full, making sure she's alert, etc.  We've read that for babies who had the prenatal surgery, the median length of time after birth for ultimately needing to have a shunt placed is about 180 days, so we'll need to keep watching this for a while to come.  But so far so good.
  • Chiari II malformation:  Where the brainstem is pulled down into the cranial vertebrae, leading to the hydrocephalus described above.  Children with this can also develop problems with swallowing, breathing, etc.  Kaitlyn had this malformation in the womb, but it was reversed by the prenatal closure, as we had hoped.  (We wrote about this in our  October 7 post.)  
  • Leg mobility:  The most obvious symptom of spina bifida is some degree of paralysis and loss of sensation in the legs.  Kaitlyn's level of myelomeningocele (hers was from the L2 to S1 vertebrae) would be expected to need crutches and long leg braces up to the thigh or waist, and would probably need a wheelchair for long distances (see here).  But we, and all of the nurses at UCSF, are amazed at how strong and active her legs are.  She kicks very vigorously at times, moving her hips, knees, ankles, and toes, and appears to have at least some sensation when we tickle her feet and legs.  We'll have to just wait and see when she gets older how well she does with crawling and attempting to stand or walk.  
  • Orthopedic problems:  Children with spina bifida are more likely to have clubbed feet, hip dislocation, and scoliosis.  Kaitlyn does not have any of these issues at this point.
  • Bladder issues:  Nearly all people with spina bifida have some bladder dysfunction.  When Kaitlyn was born, she had some slight hydronephrosis (swelling of kidneys), so they had us regularly catheterize her each time we changed her diaper.  The day she was discharged we took her to get urodynamics, where they fill her bladder with a dyed liquid and take X-rays to see how her bladder reacts.  There was some good news:  they did not observe any reflux of urine back up to the kidneys, and her bladder looked smooth and elastic like it should be.  But they did observe what they call dyssynergia; when the bladder constricts to push out urine, the sphincter at the bottom should relax to release it into the urethra, but Kaitlyn's constricted instead for a few minutes, so she wasn't able to release it when she wanted to.  She does usually urinate on her own, but we'll keep catheterizing her (5-6 times a day) just to make sure she voids as she should.  As of now, this is the only major difference between her and our other newborns.
  • Bowel issues:  Children with spina bifida often have bowel problems leading to chronic constipation, and often need a bowel program to gain social continence.  This has not been a problem for Kaitlyn yet; usually this is not an issue until they start eating solid foods.  Many newborns with spina bifida do tend to void all stool constantly, creating severe diaper rashes.  But Kaitlyn doesn't have a rash yet, and it looks to us like she is intentionally pushing out her BMs; we see her grunting and pushing.  We don't know if this means anything yet, but we're hopeful because it appears that she does have some feeling and control in that area.
  • Tethered spinal cord:  This is where the cord gets tugged by scar tissue near the site of the surgery, which can over time lead to further nerve damage.  If any such problems develop in the future, she may need surgery to release the tension.
  • Visual problems:  Children with spina bifida often have strabismus, where one or both eyes turn in or out.  Kaitlyn's daddy had that condition (and even had two eye muscle surgeries to correct it), so we're definitely not counting that out yet.  But so far her eyes don't appear to drift too much.
  • Learning difficulties:  Learning difficulties can be caused by hydrocephalus, infections, etc.  We obviously won't have any way of knowing whether Kaitlyn has any such problems for awhile.  Yet another "wait and see" issue.
  • Latex allergies:  Up to half of all kids with spina bifida are latex sensitive, so we will need to be very careful to avoid any products -- medical supplies, rubber bands, tires, balls and other toys, etc. -- that may contain latex. 
So there's a sum-up of the various issues we will be watching as Kaitlyn grows.  So far she seems to be doing very well, and we are so grateful for that.  As always, we thank you all for your prayers, understanding, love, and support.  Looking back on how scared we were when we first got the diagnosis, it's incredible how much more hope and optimism we have that she can have a happy and fulfilling life.  She is a beautiful little girl, and we are so glad to have her in our home.  And she is cute, right?



























Wednesday, October 14, 2009

HOME!

  I can't believe the day has finally come.  I fly home to Houston with Kaitlyn in a few hours, and Kendall will make the LONG drive back after dropping us at the airport.  Kaitlyn couldn't be cuter, and we absolutely love having her with us all the time.  Now we just need to pick up Damon and Audrey and we'll be together as a family again.  Life is good.  Thanks for all the prayers.  Kaitlyn is doing great, and we are so grateful.  So, get ready for us, we're on our way!!

Wednesday, October 7, 2009

Good MRI News

We were up bright and early this morning to take Kaitlyn to have an MRI.  They had to put in an IV to sedate her throughout.  She did well, but when they woke her up, they had to give her oxygen for awhile, and she was quite drowsy all morning.  So, before I explain what the MRI showed, you should know that when I had the two MRI's during the pregnancy, they showed that Kaitlyn's Chiari II malformation went down to the C3 vertebra.  That means that her brain had been pulled down by the suction that the opening on her back had caused.  Kaitlyn's Chiari was slightly worse than the average baby with spina bifida (about C2).  My theory is that she had an open lesion (not covered by a sac), and thus there was a greater suction.  That, I believe, is also the reason why her ventricles were so small before the lesion was closed during fetal surgery.  Once closed, the suction stopped, and her ventricles began to slowly fill with fluid.  The last week of my pregnancy, her vents were measuring 14 and 11, which is slight hydrocephalus.  Since birth, those numbers have held steady and not increased.  We'll keep watching.

So the great news from the MRI today is that her Chiari has completely reversed.  Her brain is now in a normal position, which is awesome.  We are so grateful that we were able to do the surgery.  It was definitely not an easy journey, but we choose to believe that all of the positive developements that we've seen in our daughter are largely due to the early intervention.  We're thrilled at each bit of good news.

It's looking like she'll be released by Tuesday of next week.  That would put me back in Texas in a week!  Hooray.  Then Kendall will drive to Utah, pick up the kids, and continue the 24-hour drive down to Houston.  I can't wait to have all of us together again.  There are 2 little ones that are just waiting to be tickled and smooshy kissed.   

Monday, October 5, 2009

Baby steps

Kaitlyn is doing very well.  It seems like every morning when we get to the hospital she has learned something new.  She is no longer in the incubator, because she is able to hold her temperature.  Once in a while she'll have a day where she is quite drowsy, but most days she seems more and more alert.  After losing a little weight, yesterday she finally started gaining, and is now slightly up from her birth weight.  She now weighs a hefty 5 lb 3 oz, and counting.  They now have her eating 45 ml (1 1/2 oz) at each feeding.  She tolerates it well, but occasionally has trouble staying awake for the whole feeding.  They put an NG tube from her nose to her stomach to finish off all of the feedings that she is not able to complete on her own (which at this point is all of them).  Yesterday she did drink 35 ml from the bottle, which is the most she has done on her own.  Her back is healing amazingly; you can see in the last two pictures below that her skin has almost healed entirely.  The second-to-last picture is the day after she was born; the white areas are the gaps where the skin had not quite closed yet over the skin patch, and as you can see in the last picture taken yesterday, our little Wolverine has incredible healing powers. Cathing is going well.  It is now very easy for us to insert, and we're glad that it has not been difficult to learn.  Her leg movement is still great for how high her lesion level is.  When we place her on her tummy, she is almost able to push up onto her knees.  Her head size is at the 50th percentile for her age, and no one has noticed anything that would make them think she might need a shunt soon.  She will have an MRI tomorrow, and that should tell us quite a bit about what is going on in that cute little head of hers.  Quick warning: I'm going to go into a little bit of diaper talk, so feel free to skip over this next part.  One thing the nurses told us is that most myelomeningocele babies tend to constantly dribble their bowel movements out, because there is not much sphincter control.  So, they typically have a terrible diaper rash.  But Kaitlyn seems to have some control, because her diapers are not always dirty, and she has not developed a diaper rash at all yet.  This may not seem terribly exciting to most of you, but we are thrilled that her little bum is not sore yet.  So, there you go.  The latest on our little cutie pie.  We love holding and cuddling her, and can't wait until she's well enough to come home with us.

Kendall's parents were able to come up last week, and spent a lot of time in the NICU with Kaitlyn.  It was great to have them, and we're so glad they got to meet her before we took her halfway across the country.  Damon and Audrey are still in Hurricane with Grandma and Grandpa Ferrin, enjoying all the great family and cousin time.  Damon told me that I "better gotta watch the mouse movie (Stuart Little) when we get back to our house."  I can't even begin to tell you how happy it will be to be together as a family back in our own home.  I am so excited to see how the kids do with Kaitlyn, and to be able to care for and pick up my children.  That was really tough when they were staying here, because I could not lift them, or hold them like I wanted to. 

I was looking back at the posts we had written after the prenatal surgery, and I cannot believe the difference between that one and the c-section.  Night and day.  I guess not having had a caesarian before, I thought that the fetal surgery was not too different, but hindsight certainly tells me otherwise.  I am really fortunate to have healed wonderfully from the c-section, and out of necessity, am already walking about 1 1/2 miles a day to and from the hospital on no pain meds.  I am absolutely astounded that I feel so good.  It was a long and hard pregnancy, and it is wonderful to have her out and not in.

So all in all, things are going great.  Kaitlyn is on a good track to be released at the end of the week, if nothing goes wrong, and we are getting closer and closer to the end of this road and the beginning of a new one.  We're so grateful for all of the prayers and service we've recieved and we can't wait to see all of our friends in Houston again.