Daphne's Moyamoya Surgery Journal

2-27-05   Flew to Stanford, CA

2-28-05

10:00 am – Met Dr. Olsen in Neurology to go over seizure precautions before surgery.   Very nice.  Mentioned concern about g-tube irritation area, and he called to get us an appointment with a pediatrician. 

Daphne did not like the bright sun, and had worked herself into a frenzy before the appointment.  She was very irritable

12:00 Met with pre-op anesthesia.  Nurse was very impressed with our records :)

1:00 Met pediatrician.  Very nice lady.  Said Daphne had g-tube granuloma, and prescribed steroid cream.  Daphne also had a chapped pinky finger from chewing on it, and she prescribed Eucerine.

1:45 Met Teresa, Dr. Steinberg and team.  Nice, comforting meeting.  Dr. Steinberg examined Daphne and said that she was moving around better than he’d expected.  Teresa explained visit with Neurologist in am was for sanity check, as neurologists tend to be much more pessimistic than neurosurgeons.  Dr. Olsen had called them and told them that, without a doubt, Daphne should have the Moyamoya surgery.

The team also felt that the Moyamoya was a likely cause of Daphne’s infantile spasms, and may also have been the cause of her developmental delays.  The senior resident talked to us about Daphne’s left hemisphere brain atrophy, and said that having Moyamoya and the resultant lack of blood flow could have prevented Daphne’s brain from fully developing and growing.  

I asked Dr. Steinberg if there was an advantage to having two separate surgeries for each side of the brain.   He answered “yes” and explained that it reduced the risk of prolonged anesthesia, and allowed him to really concentrate on one side.   His answer made sense, and

We also met a reporter from the New York Times who was here covering the story of another lady who was here for Moyamoya surgery. 

Very busy and exhausting day.  We will be meeting back with Teresa on Wednesday to go over all details of surgery.   I really like Stanford, so far.  Everyone has been nice, accommodating and, even, comforting.   I hope that we’ve really found our answer, here.   I know that the surgery is not a cure for the injuries to Daphne’s brain, but maybe if it can prevent future injury, and also improve her blood flow, maybe Daphne can finally start to grow and develop – and maybe even has the potential to have only mild or moderate disabilities.  

3-01-05  Uneventful day.  We had no appointments, so we had a leisurely day.   Daphne had lots of fussy periods again, but happy times, too.   We did take the opportunity to go to downtown Palo Alto to the shopping district.   Mostly we just walked and enjoyed the fresh air.   We were a little jealous when we saw all the nice restaurants, knowing that we wouldn’t get the chance to use them.   We are already getting sick of the hotel's food. 

3-02-05

Daphne had her MRI today under general anesthesia.   I was worried about them using gas to put her out, and the risk of stroke, but all went well.   She woke up pretty angry, but calmed down after an hour or so.

We had lunch and then went back to Stanford Neurology clinic to meet with Teresa for questions.  We had a great conversation, and Teresa confirmed that there is a pretty good chance that this surgery could help Daphne’s development.   She explained a little more about the surgery, although I still don't feel like I've fully grasped how everything works.   I am very interested in what Daphne's SPECT scan will show.  

Teresa also said that this surgery is not nearly as painful as heart surgery, and that Daphne will only stay in the hospital for three days, if all goes well, for each surgery.  She will also only stay in the hospital for six hours after her angiogram.   Not sure, right now, if I’ll feel comfortable taking her back to the hotel after the angiogram, but we’ll see how it goes.

After our meeting, we went downtown again for a short time.  The weather, besides the night that we came in, has been beautiful!

3-03-05

 Daphne had a SPECT today.  The procedure seemed long.   Daphne was gone for well over two hours, and I even asked one of the recovery room nurses about her once, as I was very nervous.

She came out fine, though.   Her eyes were only slightly open when we came in, but she woke up without fussing, which was nice.   The anesthesiologist told us that Daphne was really difficult to get an IV into, and suggested they do a central line for the angiogram.   They obviously did have a tough time, as we saw about six bruises on Daphne’s arms and legs where they’d tried to put an IV.  Good thing they put her to sleep before they started trying!!  The anesthesiologist said she hadn’t even intubated Daphne after all that… she just held the oxygen mask over her face the whole time.   That was nice.

Hopefully they got everything they need, because they cancelled her Friday SPEC – thank goodness.   I am so worried every time that Daphne has anesthesia that she will have a stroke.   Plus she doesn’t get food all day… not that Daphne is complaining about that.

3-04-05 

A really rough start today.   Daphne didn’t have any procedures scheduled, but from about 8:30 to about Noon, Daphne cried inconsolably.   We stopped her feed early, we vented her g-tube, we gave her Motrin, Magnesium, a suppository, and then finally – in exasperation, we gave her a little Benadryl.  About 15 minutes later Daphne was resting comfortably.   I hated to give her Benadryl, but with that intense crying I always worry about more strokes, or causing damage to her brain.

Daphne only ended up sleeping about an hour, though, and woke up pretty content (even if still pretty sleepy).  We got the chance to go to a little place for lunch, and went to the shopping center and I bought some clothes.  Fun for me – I’m not so sure about Ray.  Daphne didn’t mind and alternated between little naps and looking around, right up until we were ready to leave.  I guess the Benadryl – even though I sometimes feel guilty about giving it to her – really was worth it.  Daphne felt much better, and Ray and I got to get out for a bit.

When we got home, Daphne fired off a huge poo, and then took a nice nap.   Poor baby.

3-05-05 

Ray and I did some exploring.  We went up 82, into some mountains and out to RT 100.  We spent some time strolling along a beach.  Everything was beautiful!  The trees were huge, and the shore was carved with rocks and sandy cliffs.  And Daphne, for the first time, got to see the ocean.   She did watch it for some time at one stop-off point, as the waves crashed against some rocks.  When we took our stroll at another spot, though, Daphne kept her eyes closed.   She didn’t enjoy her day, overall, and slept most of the time.   I am glad we got out, but it’s hard to really enjoy yourself when you feel like your baby is uncomfortable.  At the hotel, she was very fussy in the evening.

3-06-05

Ray decided to try giving Daphne Nuerontin (which had been prescribed to her earlier, but we had mixed feelings about it - as it's also a seizure med, as well as a pain med).   She did seem to fuss less in the morning, and we decided to take her to Muir woods, outside of San Francisco.   We thought that she would like taking a walk in a nice shady area, and Daphne has always loved trees.

We are learning that Daphne is extremely photosensitive.  Even indirect sunlight forces her eyes clamped shut and is painful to her.  Daphne slept the car ride up, and continued to sleep, with some bursts of fussing, through our walk.   We were so disappointed that she was in this state, because we knew she’d love looking at all the huge redwoods.

Towards the end of our walk, we finally rustled her out of her stroller for a picture, and she finally opened her eyes.  For awhile after that she was alert and looking at all the trees.   We were so glad that she got to be a part of  the experience, even if for only a short time.

The ride home was tough, and we ended up giving Daphne some Morphine.   After about 30 minutes, this seemed to help her, and Daphne rested easily.   We are so much hoping that the surgery Daphne will be having will help stop whatever pain Daphne keeps having.  We are not sure what to expect – we are just hoping our baby can be more comfortable.

3-07-05

 Daphne’s Angiogram.   It lasted a couple of hours, and they decided to do a central line, as well as an IV, which meant we ended up staying the night.

Daphne woke up fine from her anesthesia.  Oddly, since her left hand had an IV in it and couldn’t easily move, Daphne started having improved movement of her right arm.   Ray spent the first night with Daphne, so that I would stay the night after her surgery.

Daphne was much better behaved today.   She fussed in the early am, but was conked out by the time we were in the hospital.   She did wake Ray up quite a few times in the night, but was easily settled.

3-08-05

Daphne’s surgery.   What a big day --- unfortunately, I didn’t get a chance to write during that day, but I’m writing two days later all I remember.   First, even though I rushed to get to the hospital by 7:30, by the time I got to Daphne’s room, they’d already taken her to surgery.   I hopped on the elevator and ran into Ray on the second floor, and he said he’d just seen her off.   I was sad to have missed her, but Ray said things happened really fast.

Ray told me that he’d talked to the neurosurgical fellow that morning, and that he’d told Ray that the angiogram showed that the blood supply to Daphne’s left brain was supplied mostly by the collateral vessels that had formed on her right brain.   Hard to imagine!

After grabbing a bite to eat, Ray went back to the hotel to take a shower and do a few things, and Jill and Tara (from the Moyamoya.com support group) kept me company from about 10am on.   They were so nice!   There was a group of people next to us whose child was in surgery, and a CNN television crew was covering it. 

The wait seemed to go pretty quickly.  Around Noon, I got notice that Daphne was 45 minutes away from coming out of surgery (it was really nice… they gave the parents pagers, so they’d be notified if they’d chose to wait somewhere else or grab lunch).   A little while later, I saw Dr. Steinberg coming down the hall toward us… I thought I detected a smile on his face, and I just knew things must have gone well.   He told us that Daphne had “behaved” herself during surgery, and that there was only very minimal blood loss.  He said that they’d found a good artery in her scalp, and had sutured that to her left brain, after making a small opening in the sylvian fissure area (Daphne's were already underdeveloped and open, so he didn't need to make much of an opening).  They’d then folded the dura in, so that the vascularly dense side was against the brain.   He said he felt that good revascularization should be achieved.

By about 1:30, we were let in to see Daphne, who was crying and who had an oxygen mask taped to her face.   They gave her a second dose of morphine, and that calmed her quickly so that she went right to sleep.    Once she did wake up, her eyes were glassy, but she definitely saw us and turned to our voices, she was very calm.   There was some concern that she was moving her right arm, now, more than her left, but I told them that I felt it was because the IV was in her left arm, and they cut the board off to allow more movement (it did take awhile, but by the next day she was moving it around just like before).

Daphne slept most of the time, when she did wake up after the morphine and anesthesia had worn off, her eyes were a lot brighter.   She was still very mellow, though, and hardly complained all day and night.

3-09-05

Daphne continued to do well.  She was moving around more, and still stable.  We were told that she could go upstairs, and get out of intensive care, but we never ended up getting a bed.   Another night without sleep for one of us (Ray, this time)

Ray also felt Daphne’s first tooth!!   The little sharp edge was poking out of her gums on the bottom, front row.  It was so funny (and relieving!), because the previous night Daphne had a visit from a couple of geneticists.   During their examination one of the doctors asked me if Daphne had any teeth, and I replied "no".   The doctor then proceeded to tell me that she thought the cut-off age for getting teeth was fifteen months.   Apparently, Daphne had other ideas.

3-10-05

Daphne was pretty much the same as yesterday.  Very mellow, still acting tired, but moving, listening to us, watching us and looking at her toys.  Rene, her nurse, and I took Daphne for a wagon ride around the second floor, and she seemed to enjoy getting out of the room.

She had one, really cranky period just before being (finally) taken up to the third floor to her new room.   She got a dose of morphine, but that didn’t work.  We think it was gas – she did eventually settle down once she was in her new room and not being bothered by nurses (or her mom).   After that, she was calm for the rest of the night.

They started her feeding at 40cc an hour through her g-tube continuously, which is too much for her.   Why, oh why, do they always mess with her feeding schedule at the hospital???   Why don’t they just let the parents decide how to do it (if it were a child without a feeding tube whose parents stayed around, I’m sure they would have.   We know what she can handle and how much is a good feeding for her better than they do!)

Everyone concedes that Daphne is recovering well.   She isn’t herself, though.  She is too mellow… too calm.  Not to complain, and she did just have major surgery, but I wonder what’s going on.

3-11-05

Saturday – Daphne was discharged this afternoon.   She seemed to be more alert, and a little more herself (still no smiling.)   After coming home, Daphne and Ray bathed (thank goodness!  Whew!)  Then, we went to the noodle bar at the Stanford Shopping center. 

Daphne did get fussy toward the evening.  We have rigged up a contraption that keeps her g-tube continuously vented, so she has less gas.   You wouldn’t believe the air that comes out of this poor baby’s tummy.

My Mom came in the evening (flew in from Philadelphia), and brought Daphne’s Ferrell bag from home, but it just didn’t work as well as our contraption (the bolus feeding tube is attached to Daphne’s button, and tied up over her and left with an open bolus syringe for venting.  When feeding, we set her feeding pump kit inside the bolus syringe and just let it drip).  All said, while Daphne is fussy again, she seems much more like herself.

She even seemed to know when we were getting ready to be discharged from the hospital, and had a burst of kicking and grunting.  Funny how she does so much better outside the hospital.

3-12-05

Daphne was fussy at night and in the am.   We drove to Half Moon Bay, through 82 West, and Daphne loved the car ride and looked at the trees.   We stopped by the ocean on Rt 1, again.   Daphne didn’t like stopping.  She wanted to keep going.

We went for a walk in Downtown Palo Alto, which Daphne truly enjoyed.  We took Daphne for a walk around the hotel in the evening.  She loved looking at the lights in the trees!

3-13-05

We drove to San Francisco.  Daphne seemed less light sensitive… still sensitive, but not as bad.  It’s definitely not our imagination.  Her eyes aren’t tearing as much, and she is able to open her eyes in cloudy weather or under shade, which she simply couldn’t do last weekend.

3-14-05

We Drove to Sonoma County and toured a couple wineries.   Daphne was very well behaved, alert and bright.   Not smiling, but taking everything in.  She slept well through the night.

3-15-05

Daphne’s second Moyamoya surgery.  We went to the hospital around 6:00am.   Daphne had slept in bed with us for much of the night, and continued sleeping away while we showered, etc.   She wasn’t happy when we finally woke her up, but after the initial fussing was bright and alert again.   In hospital admissions, I put a rattle in her left hand and she held it close to her face and really scrutinized it.  She began moving it slowly, just a little, while looking at the little beads inside, then finally really shook it.

She seemed to be studying everything, especially people.   As soon as we took her into the PACU, though, she started screaming (I think she knew the routine by now!).   She was happy to be held, but didn’t want to be put down.  When I held her hand, she didn’t want to let go.

The anesthesiologist gave her IM versed.   It didn’t work right away, but I held Daphne in my arms as we were leaving for the OR.   On the way she was looking deep into my eyes… I’ll never forget that look.     It was heartbreaking to hand her over to the anesthesiologist.

The wait was longer than the last surgery.  Ray even asked them to check on Daphne at one point.  The waiting room was lots busier, too, and there was one extended family that was taking over most of the seats.  They had an adorable little nine-month –old baby girl who was rolling all around the floor on a blanket.  I wondered whether Daphne would ever be as active and happy as that baby girl.

Finally, around 1:00, we were told that Daphne was 45 minutes away from coming out of surgery.   Dr. Steinberg later came out to see us, just as before.   He told us that Daphne had done well.  He said she had a really good artery in her scalp, and he’d even considered doing a direct bypass, but that he ended up doing the same surgery that Daphne had on her left side.  He then added that Daphne had “behaved” herself, and that her EEG looked good throughout surgery.

Finally, around 2:30, we got to see Daphne.   She had a nasal cannula, and was in between sleep when we came in.   She opened her eyes a little (slits), she was having a little labored breathing, but got better.   Mom was worried, but I told her I thought Daphne tended to have some labored breathing after anesthesia, which improved after all the medicine wore off.

Three surgeries in about a month and a half.   Two strokes in the last six weeks.   I still can’t fathom all that my poor baby has been through.   I only can hope that this will be her last surgery for a very long time, and that this surgery will be a significant benefit to Daphne.   We have real hope that this may cure her seizure disorder, prevent her from future strokes, and possibly even help her development.   I truly pray that everything we have all gone through, as a family, is worth it.

3-16-05

Daphne had a tough day.  She definitely seems to have more pain after this surgery than her last one.   She was fussing much of the day, or sleeping.   At one point, I felt panicked because she was crying and hyperventilating – something I’ve never seen her do, and I was petrified that she’d damage her brain.   They gave her morphine, which didn’t calm her down, then versed, which finally did help.

Ray stayed to watch her while mom and I went to dinner, and he reported that she’d smiled at her butterfly toy while we were out.   Isn’t that something?  Smiling after all she’d been through, and all the pain she’d had all day. Daphne also got moved up to the third floor, and got her own private room.

3-17-05

Happy St. Patrick’s Day.   Daphne was still fussy.  She was somewhat better than yesterday, and – at least – was getting feedings, and was tolerating them OK. 

Daphne did get a visit from OT and PT.   They noticed that Daphne now had a purposeful grasp with her left hand, when given a rattle.

3-18-05

Fussy again.  Waaahhh!

Ray and I talked to the PR guy at Lucille Packard’s about Daphne, so that he knew about her story.   They said they’d like to pitch Daphne as a success story to some local papers after she has her follow up MRIs and SPECTs this summer.   Well, that’s positive thinking.   Hopefully, she will be a success story.

Teresa is so nice.   She even cried a little when we got to the part about Daphne going home on hospice care.   She later said she’d never seen another case like Daphne’s, and that as far as she knows, Daphne is the first reported case of Moyamoya and IS.   Daphne has a will to survive, that’s for sure.

3-19-05

Daphne’s discharge orders were written this morning.  Yey!!   She’s still in pain, but it’s nothing we can’t manage at home at least as well as they can manage at the hospital.

We got Daphne unhooked from her monitor and got her into her stroller, and she was instantly looking a little better.  She was so happy to get out of bed.   She insisted we stroll her around the hospital floor.

We had to wait until a doctor was available to take out Daphne’s central line, so even though we had discharge orders by 9:00am, we didn’t end up leaving until well after 2:00pm.   We did get to meet Laurel, though, from the Infantile Spasms board, and her daughter Isabel.

It was nice to meet them in person, and Isabel was absolutely adorable!   She is 18 months, and Daphne is 17 months, but the difference between their appearances was astonishing.   Isabel looked like a toddler, while Daphne still looks like a big baby.   Both are cuties, though, and sweet.  Poor Laurel, Isabel had been up since 4:00am, and was very sleepy during the visit.   We got a couple pictures and what a pair.  Daphne with her bruised eyes rolled up, and little Isabel snoozing away.

3-23-05

We are going home tomorrow!  And by that I mean back to Pennsylvania, not to the hotel we've been staying in at Stanford :)

Daphne went to her follow-up appointment today and was given the clearance to go home. Dr. Steinberg also showed us Daphne's MRI and SPECT that she had out
here, and we were surprised to learn that her strokes did not look that bad on the latest MRI. There were some small areas in the brain that showed injury, but Dr. Steinberg said most of the imaging showed healthy brain tissue. Just for comparison, he showed us an MRI of a man who'd had a major stroke, and it was quite a difference ... Daphne's MRI showed a few "spots" of injury, whereas the major stroke showed a much larger area on just one hemisphere. We also learned that the left side of Daphne's brain (the one that was most severely affected by Moyamoya) was 20% smaller than her right side. The SPECT showed significantly reduced oxygen supply on that side of her brain. It just makes so much sense, since her EEG always showed such chaotic activity on the left side of her brain.

We still have a ways to go, since it will take awhile for the graft that Dr. Steinberg attached to her brain to grow new vessels into the brain, but it is just so amazing that after her doctor at CHOP had expected that she'd die, that Daphne made it through two brain surgeries, and we've then learned that her strokes weren't even severe.

Incidentally, in the past two days, Daphne has started smiling again (a little) for the first time since her surgeries. She is also moving her right arm lots more. I just pray that she can continue to make progress, little by little, and I am always so fearful of another setback. 

3-24-05

We made it home.  Daphne was thrilled to be home.  She was full of smiles, and very interactive.   When I strolled her around the house, she looked around and just giggled…. I haven’t heard her laugh in so long!

There is no question that Daphne knows where home is.  We were all glad to be back.   The dogs were happy to see us all, too.

3-29-05

Daphne is napping, so I thought I’d take the time to wrap up the journal.   I may add some updates later, but probably won’t keep it up as regularly.

Daphne has been doing well.   She still has irritable periods, but her mood is generally much, much better.   She has started babbling again, interacts with us, smiles and laughs with us.   It really has been a pleasant surprise, after all she’d been through over the past month.

She still demands lots of stroller rides, and she still strongly resists any physical therapy work – even just simple things like sitting on your lap.    Working with her, I do realize how far we still have to go.   Her head and trunk control need some major work.   When in supported sitting, she leans way to the left.   If we hold her on our shoulder she arches  back.   She refuses to do anything on her tummy.   She also isn’t grabbing things, but she is moving both arms, and will shake a rattle if you put it in her hand.

It is going to be a long road to recovery.   I am at the same time both very happy that she has made it through so much, and now, hopefully, will have the chance to develop and live a meaningful life; but I am also frustrated by the fact that, after it took so long for her to roll, sit supported, and develop fine motor skills (I always told Ray that we appreciate all milestones achieved much more than parents of “normal” children possibly could), that she has lost so many skills and we have to go back, again, and pick up on physical therapy where we had been months and months ago.

Still, just a smile from Daphne and my mood is so much uplifted.   We just have to work through it and have patience.   God knows that Daphne is a fighter, and we have to fight to give her every chance we can.

3-31-05

I just had to add that I had the most wonderful time with Daphne last night.   She was a gem all day.  Very interactive and playful with me.   In fact, I know she loves me because earlier in the day she was in her crib and we were “talking”.   She was smiling and laughing, and having a wonderful time (she even said “mama”, or something that sounded like it, two times while she was babbling).   Then, there was a knock at the door, and UPS was delivering several packages.   I went downstairs to take them inside, and suddenly Daphne was crying.   When I came back into the room, I asked her, “Aww… did you miss me,” and she instantly saw me and stopped crying, and was attentive to me.   It was great.

In the evening,  I put on our “Bend it Like Beckham” CD.   I mostly strolled her around “dancing”, but a few times I took her out of the stroller and danced with her on my shoulder.

Daphne would lean her head back a little to look at me, and just laughed and laughed, and kicked her feet.   She was truly having a good time, and I swear it was the most fun I’ve ever had with her.

I know she’s not supposed to have cognitive improvements from the surgery yet, and I don’t know what to make of it, but I sure have enjoyed Daphne’s attentiveness and desire to interact with me.

4-28-05 to 4-30-05

We have had a completely exhausting last few days. Daphne started inconsolably crying on Wednesday evening, and on Thursday continued to cry. We had an appointment for her in Baltimore for feeding clinic, but, once there, decided to take her to the ER at John's Hopkins.

To make a long story short, we eventually learned that Daphne has an Epidural Hematoma on the right side. Thankfully, it is nothing life-threatening, and surgery was not needed because her brain has plenty of room to swell (she has brain atrophy as a result of her moyamoya disease and strokes). She is home now, and feeling better with some oxycodone.

The experience, though, was made so much more stressful because we ended up taking Daphne out of John's Hopkins, against medical advice, and driving her to Children's Hospital of Philadelphia (which is now her usual hospital). Hopkins was absolutely horrible! First, the doctors told us that Daphne had an epidural
hematoma, and that she would need surgery (which scared the heck out of us b/c the hematoma was right over the area where she recently had her bypass surgery for moyamoya, and we were terrified that they'd screw something up). Then the surgical team decided that Daphne did not have a hematoma, and that they were just seeing the
muscle overlay from her moyamoya surgery. This didn't make sense to us for a number of reasons that we explained to them, but they did not listen to us (turns out that we were right!). Plus, we had a parade of residents coming in to examine "the moyamoya patient", all prying open her eyes, poking her ears and otherwise aggravating her
while she was already in pain. Also, she was there over 8 hours with no food, no IV, no pain medicine. And the list goes on and on!

I now truly appreciate Children's Hospital!! I know Hopkins has a great reputation, but there was just no comparison in the level of care.

Teresa and Dr. Steinberg, by the way, were wonderful throughout the whole ordeal.  We called them from the ER at Hopkins, and were able to talk to Teresa and get a call from Dr. Steinberg within about 10 minutes.  He spoke to us, as well as the attending and the neurosurgical resident for quite awhile.  We were truly surprised by how quickly Dr. Steinberg responded, and it was so reassuring in such a scary situation.  We know he has lots of moyamoya patients, but he and Teresa made us really feel important, and that we could count on them for support in Daphne's care.