Elisabeth's Eye Exam

Well, we are home from Spokane with some new information about Elisabeth. She appears to have cortical blindness. This is visual impairment due to damage to the brain. Her eyes are actaully perfect, but the visual systems of the brain cannot interpret what the eye sees. As of right now her vision is 20/2000. The good news is that she is not completely blind, she can see colors, and her vision could improve some over time (especially if the stem cells help repair damage to the brain).
But for now she will continue having eye exams every 2 months to monitor how she is doing.


Here are some facts about cortical blindness or CVI as it is also called:
-Vision appears to be variable: sometimes on, sometimes off; changing minute by minute, day by day.
-Many children with CVI may be able to use their peripheral vision more effectively than their central vision.
-One third of children with CVI are photophobic, others are compulsive light gazers.
-Color vision is generally preserved in children with CVI (color perception is represented bilaterally in the brain, and is less susceptible to complete elimination).
-The vision of children with CVI has been described much like looking through a piece of Swiss cheese.
-Children may exhibit poor depth perception, influencing their ability to reach for a target.
-Vision may be better when either the visual target or the child is moving.

-Spatial confusion is common; for example being unable to locate their chair even though they can see it.
-Seeing with CVI can be compared with trying to listen to one voice in a noisy room or to speaking a foreign language.



So that's a little on her condition. Elisabeth will have her challenges, but we feel so blessed to be able to take care of her and love her. I am so thankful for my musical background so that even if Elisabeth has trouble with vision I have something to offer her that will enrich her life (Her right ear is failing hearing tests, but her left ear is perfect!).

Elisabeth and her stem cell transfer in North Carolina

We flew out of the Spokane airport on Sunday to fly to North Carolina where Elisabeth was scheduled to receive an infusion of her cord blood stem cells. Elisabeth was a very good little traveler and slept through both legs of the flight just waking long enough to drink a bottle and then doze back off. We arrived in Raleigh a little after 10:00 p.m. and made our way to the Millennium hotel to get a little sleep before meeting the medical team the next day.

On Monday we woke early and left to find our way to the Duke University Childrens hospital where we were supposed to check in at 8:30 a.m. Once we got there they took her to an exam room where they proceeded to check her vitals, weigh her, measure her, and check her blood pressure. The nurse then left and we remained in the room while we awaited the doctors visit. It was while we were waiting that we noticed Elisabeth's foot twitching. It continued for a small while and soon both feet were twitching. At first we just wrote it off as a muscle spasm, but after a few minutes the 'twitch' had moved up her body and she was convulsing steadily from her shoulders down to her feet. Then we began to worry. We hurried out to the hall and found a nurse who then found the nurse practitioner. After examining Elisabeth it was determined that she was in fact having a seizure. Luckily it calmed on its own.....but I was still very upset. I did decide however that if there is a place to be when your child starts having a seizure it is a hospital :) The nurse contacted the doctor who ordered a CT scan and an EEG. The CT scan was first. Elisabeth remained very still and calm during it. The results determined several things....first that her shunt seemed to be in the correct place.....so we could presume that the seizure was not from shunt malfunction. It did give us some new news however. They discovered that Elisabeth was missing parts of her brain. There is absence of the septum pellucidum, meaning that it isn't there all together. There is also a partial absence of the corpus callosal which is the part of the brain that connects the left side of the brain to the right. What this means for Elisabeth....that is still to be deteremined...we'll keep you all posted.

Elisabeth then went on to the EEG. She was a trooper! It took forever to set up for the test. They carefully had to measure out precise points all over her head and mark them with a wax pencil. They then had to go through and clean each spot with a special sollution and attatch electrodes. When the test began Elisabeth had to remain still and quiet for about 1/2 hour . The results came back showing a minimally abnormal EEG due to presence of focal sharp waves and they said it may be suggestive of neuronal dysfunction in the bilateral occipital regions.

OK, so then a morning full of tests was done. We next went to meet baby Owen and his family. Now Owen is the baby who inspired us to do the cord blood infusion. He was born one year prior to Elisabeth and was the first hydrocephalic baby to have the stem cell procedure. His family started a website to help eduacate and lend support to families going through the same thing. Infact, until I found their website I was convinced by doctors that Elisabeth wouldn't live. Owen and his parents have meant so much to our family and it was an honor to get to meet them. It was fun to see the two babies together and take their pictures...little friends :)

After having lunch with them in the hospital cafeteria we were just getting ready to leave and spend the rest of the day exploring the town...but then it happened again....the foot started to twitch. Yes, it was another seizure coming on. Luckily we were still in the hospital. So we hurried back upstairs in the childrens hospital where they took her back to a room and went to call the doctor. Dr Kurtzberg (the dr for the infusion) then called Elisabeth's neurosurgeon to give him a heads up on what was happening. He gave her the go ahead to do whatever was needed. They got her on a medication and warned us that she would get extremely drowsy.

So we finally left the hospital at around 7:00 p.m. A long day considering that we were originally only supposed to be there for around one hour in the morning.

The next day we checked back into the hospital at 11:30 for the stem cell infusion. It took them about 1/2 hour just to get the IV set up. Poor Elisabeth....it's not easy to find a vein on a newborn. She was so tolerant though. They had to try 3 locations before they were successfully able to get the IV in. Then came the moment that we had been anticipating for months - the actual infusion!!! It took only about 15 minutes to get it all in her. Before the infusion they mentioned that the solution that the cells are preserved in had a funny odor to it - similar to creamed corn. Boy were they right. So for the next 24 hours Elisabeth's breath smelled of creamed corn, not a terrible thing...just strange. Donald and I have started calling her our little corn on the cob. Halloween costume next year??? Anyways, Elisabeth then stayed on IV fluids for 4 hours. At one point Donald and I ran down to the cafeteria to grab a bite to eat. When we came back to her room she had an entire medical team around her. Luckily it wasn't anything too serious, Apparently she had received a double dose of drowsiness....from the seizure medicine and then from the meds that they gave her during the infusion. She had fallen into such a deep sleep that her oxygen levels had dropped really low. But they snapped her out of it and got her levels back up. At 7:00 they let us go. The nurses there were so kind and sent us off with campus maps and suggestions for what we should do and see the next day before our evening flight.

So now we are home and doing well. We still have lots of follow up to do in regards to the seizures. But it's good to be back. We feel so blessed to have been able to do this for Elisabeth. There will never be any way for us to know just how much difference the stem cells will make in her life. As her Doctor put it, we would have to have two of Elisabeth, one that received the cells and one that didn't to see how much good the cells do for her. But the important thing is that we are trying everything in our power to give her the best in life which is what she deserves.

Elisabeth is 1 month old today! 10-24-07

Well, we have had Elisabeth in our family for a month now. What a joy she has been. It's crazy...in a way it seems like it's gone by so fast and I can't believe that she's already a month old, but on the other hand it seems like we have known her forever and soooo much has happened in just one month.
Elisabeth ended up staying in the NICU just one week. She amazed everyone with a quick recovery. They thought it might take her a while to learn to suck, but they only had to leave the feeding tubes in for a few days. She was born with rolls of chub and I don't think that she wanted to lose them...she loves to eat!
After a few days at home Elisabeth started spitting up - tons!!! It was to the point that we thought her entire bottle was coming up after every feeding. The worst part about it for her was that it came out her nose too!!! Ouch! It got so bad that she was getting cold like symptoms that actually ended up being caused from all the spit up. She is now on medication that helps some....but we still have a fair amount of spit up. She also came down with thrush.....which I think bothered me more than her. Unfortunately it meant the end of nursing for her....now she is an Enfamil kid. But she is on medication for the thrush as well and it seems to be clearing up.
Elisabeth is a good baby! She rarely cries. To express herself she likes to grunt and starts acting anxious. Alexandra and Lorelai sometimes ask why she fusses so much and I remind them that she doesn't cry nearly as much as they do....and they are 3 and 5! At night Elisabeth is waking up once to eat, usually sometime between 2 and 3 and then she is good till morning!
At 10 days old we took her back to her neurosurgeon to get the stitches out of her head. It all healed up very well and very quickly. We also saw the scans of her head and were shocked to see how much fluid really was in there when she was born. Dr. Gruber said there was about 10 times the normal amount. He has her shunt on a low setting right now and it is slowly draining the CSF. He plans to turn it to a higher setting when she is 3 months old. Luckily her shunt seems to be working well, but we are constantly on the lookout for signs of it malfunctioning...as it is very common during the first year.
Elisabeth has now outgrown all of her newborn size clothes and comfortably fits into her 0-3 months outfits! I have been photographing her in each outfit so that I can put together an album titled 'Elisabeth's First Year Fashions'. It will be fun to see all her clothes and to see her grow. Elisabeth is definately our most photographed girl. She has had her portrait taken professionally 4 times already and I have taken hundreds upon hundreds of digital pictures. I just sit and snap the camera at her all day long. I love digital cameras and rechargable batteries.....no waste...just keep taking pictures and save the best of the best.
It has been a joyous month. Looking back over the past 1/2 a year it's just amazing that she is here and that we (the whole family) made it through the roller coaster ride that the pregnancy was. Elisabeth has proven to be a miracle and has completed our family in a beautiful way!

Elisabeth is Here! 9-24-07

Well, I have to say that this has been one of the best weeks of my life. After months of worry, ultrasounds, doctors appts, and uncertainty, Elisabeth was born. She was delivered by c-section at 10:27 am on Monday September 24, 2007. It was amazing. The operating room was on the 3rd floor of the hospital and one whole wall was windows - letting the sunshine pour in. They asked what kind of music I wanted to listen to and so Elisabeth was born to Vivaldi's 4 seasons. The music was heavenly, the sunshine was heavenly, and then there was our little gift from heaven...alive and crying.....weighing in at a whopping 9 lbs 5.5 oz. The joy I felt that moment and in the hours immediately following was unlike anything I have ever experienced. A few months ago they told me to expect her to die shortly after birth, but there she was - fat and pink and crying. The most beautiful sight that I have ever seen!!
Her surgery was at 4:30 the next after noon. They put a camera in her skull to get a better idea of what was happening in there. Oddly enough her two lateral ventricles had formed as one, with nothing separating them. They also confirmed that her condition was caused by something called aquaductal stenosis, which means that one of the passages between the 3rd and 4th ventricles was blocked, not allowing the cerebral spinal fluid to drain properly. But she now has her shunt in place and it seems to be working just as it should. She was in somewhat of a daze for a day or so after the surgery, but as of this afternoon she really seems to have perked up. I was visiting her this afternoon and she drank 2 ounces, so it looks like they will be removing her feeding tube soon!!
Elisabeth is my miracle. I am so in love with her and I feel so blessed that she is alive and recovering. Thank you to everyone who has showed us support and love through all of this. It has been one of the most emotional, educational, and memorable experiences of my life so far. I can't wait for all of you to meet her.