Dr Clarks and Dr Metry's findings and recommendations

We received both Dr Clarks and Dr Metry's reports via email yesterday,whcih was good timing since we had Jayda's neurology appt too. Dr Clark told of the findings from the MRA which was that Jayda has several tortuousity of her arterial anatomy, has some anomalous origin of the posterior cerebral artery and some tortuousity of the basilar artery. He mentioned that Jayda does not show any Moya Moya patterns or any other immediate concerns. He said that the aspirin therapy or surgical intervention wasn't necessary based on these results and that future neuroimaging would be needed in the next 6 mths to monitor for any progression.

Dr Metry tells of how healthy and well-developed Jayda is showing no acute distress. She has segmental erythematous and telangiectatic plaques and patches which extend over both sides of her face in an S1 distribution on the right and in an S1 through S3 distribution on the left. She also has scalp involvement bilaterally. Dr Metry mentions that Jayda's bilateral segmental facial hemangioma in the setting of an aberrant subclavian artery,anomalous origin of the posterior cerebral artery and some tortuousity of the basliar artery, means that she definitely meets the diagnostic criteria for PHACE syndrome.

Dr Metry also mentions that there is no need to restart medical therapy for Jayda's hemangioma unless significant reproliferation occurs such that cosmesis becomes of concern. If the lesions remain stable then Dr Metry would expect the hemangiomas to involute well over time with good cosmetic prognosis. However if needed laser therapy may be done before school starts for residual erythema. Dr Metry has said that if we or our doctors have any questions or concerns that may arise in the future we are more than welcome to contact her. She would also like know the results of Jayda's MRA in 6 mths.

We have also emailed Dr Siegel asking for the consent forms to give blood as part of the PHACE registry. Which we will be recieving very soon in the mail aswell as the laboratory forms. It feels really good to be a part of such a good cause and knowing that it will all go to help find out more about this condition.

Neurology appointment at PMH

Yesterday Jayda had her appointment with her neurologist at Princess Margaret Hospital, it was the first time we'd seen her doctor since we got back from Texas. We had lots to give him, the reports from the MRI/MRA scans done at TCH, Dr Clarks and Dr Metry's reports and we also showed him the information booklets we had been given about PHACE. He asked if he could have a minute to read over the reports,which we gave him. He was happy to hear that Jayda doesn't need to be on aspirin,as this was one of the main questions he asked us to have answered while we were over there. Another thing he read was that surgical intervention was not needed at this stage but if there were any talk of this occuring Dr Clark would like to have us back at TCH. He tried to explain some of the results with us that we weren't 100 percent sure of and it made things alot clearer. He still puts Jayda at a 20% risk of having a stroke by the age of 18mths. He was extremely happy with Jayda's ability to walk along the furntiure and her newly aquired skills of clapping, playing peek a boo with herself and hi fiving us.He mearsured her head circumference and he was happy with the plan for the future scans. He is going to book Jayda in for MRI/MRA of the head and neck in August and then a review appointment with him afterwards.

Fun Things In Houston

Over the three weeks we were there we got to do many things in Houston.

SESAME STREET LIVE:

We had known about this concert for quite some time so it was decided before we left we were going.The concert was held at Reliant Park Stadium and was only 5mins from our hotel.We also went with another family whose daughter has PHACE.When the concert started Jayda started clapping and this was her first time clapping it was such a surprise.

HOUSTON ZOO:

The zoo was another thing on our agenda,as you always see something different.It was quite cold that day and it appeared that many animals felt the same way,but we still had a great day and saw lots of unusual animals that we had never seen back home.

HOUSTON SPACE CENTER:

The space center also encompasses the JOHNSON SPACE CENTER where all missions from the moon landing to current shuttle missions are run from.The complex is massive and they have a full size mock up of the shuttle to train astronauts.There is a lot of history here including full size rockets,space suits and the lecturn that President J.F.Kennedy made his speech to put a man on the moon "not because we can,but because it is hard" Of course we had a blast here.

DOWNTOWN AQUARIUM:

The Downtown Aquarium is located in downtown Houston.It has a variety of fish and animals including four white tigers and has a resturant that has an aquarium inside it that is huge.Jayda absolutely loved the fish and was facinated by it all.There is also a train ride,carousel and ferris wheel so there was heaps to do.

CHILDRENS MUSEUM OF HOUSTON:

Houston has many museums and they have one especially for kids.In some ways its like our Scietech but again this place is big and there is so much for kids of all ages.What we loved the best was that it has a part called KIDTROPOLIS and it is like a miniture city where kids can learn about banking,shopping,voting anything that an adult does it was amazing.

OUTSIDE PLAY:

The weather was quite cold the majority of our stay so when it was fine we tried to visit some parks and there are many in Houston.We saw turtles,squirrel's and fluffy headed ducks we took train rides,visited a japanese garden and saw many statues and water features.

MRI/MRA results with Dr Metry

The lovely Dr Denise Metry

Tuesday was our last appt at Texas Children's Hospital and to us our most important with Dr Metry.It was the most pleasant Dr's appt we have ever had and Dr Metry is just wonderful.The appt was about 2hrs and many things were discussed.So we will start with Jayda's Hemangioma.

Dr Metry said that her H is looking really good and that she does not need to go back on Propranolol for now.She said that our PHACE kids H tend to grow a bit longer and if we see a big change then put her back on it.She said Jayda is definitely a good candidate for laser at about 4yrs of age and that her skin type will do well with the laser.Now to her aterial anomalies.

FINDINGS:ANTERIOR CIRCULATION.Portions of her internal carotid arteries are tortuous.Her left ophthalmic artey is not visualized and her left carotid terminus is more posterior than is typically seen.The left A1 segment is hypoplastic but present.The right A1 segment does not originate from the internal carotid artery but rather an artery coursing around the temporal pole.No evidence of stenosis or aneurysm is seen.

POSTERIOR CIRCULATION:We will cut a fair bit out and give you the more important bits.The basilar artery is complex and tortuous predominately on the left.A linear defect due to fenestration and or loop is seen left of the midline slightly proximal to the left superior cerebellar artey origin.A right posterior communicating artery is not well visualized,the left P1 segment of the posterior cerebral artery and the left posterior communicating artery is not visualized.No evidence of stenosis or aneurysm is seen.Given the complexitity of the region of the basilar artery terminus and that the exact anatomy is difficult to deineate a follow up CT angiography can be performed if desired.

Texas Children's Hospital

We spoke about the CT angiogram and Dr Metry called Dr Clark to see if we could be scheduled in for one.He said that because of the radiation we can just wait and do follow up MRI/MRA every six months.He told Dr Metry if there were any big changes or any talk of surgery he wants us back in Texas.The MRA of her neck showed the dominant right vertabral artery is mildly tortuous.There is a small caliber vessel to the left carotid artery that may represent an anomalous ascending pharyngeal artey.There is early bifurcation of the left common carotid artery at the C6 level.The distal cervical internal carotid arteries are tortuous with loop formation left greater than right.No stenosis or aneurysm is seen.So on the whole things are looking good,but we are still not out of the woods.The biggest relief was no stenosis or aneurysm was seen anywhere.

Dr Metry gave us the big folder that was given out at the 2008 PHACE Conference along with the consesus statement on DIAGNOSTIC CRITERIA for PHACE Syndrome and the Clinical and Laboratory Investigations(we have a lot of reading)She was also going to take all our blood for the registry but needed a consent form from Dr Siegel,she has given us her email and she said we can get blood taken back in Australia and have it FEDEX to Dr Siegel.

She is writing up everything from the appt as well as Dr Clarks findings and emailing them to us so we can give them to our Dr's at PMH.She wants us to continue sending her every test that Jayda has, as she is now on the PHACE Registry.

The hospital was enormous!

CONCLUSION:Tuesday was just one of the best days we have had and to now know that the chance of stroke is less likely than what we knew a couple of months back is a huge weight lifted from our shoulders.The scans were extremely informative and without them we would never of known that what we had been told previously was incorrect.She still said the next 9mths are still critical for Jayda as this is usually the highest time for a stroke to occur if it is ever going to occur.

Texas Childrens Hospital:Jayda's MRI/MRA

Today Jayda had her second appt at Texas Childrens Hospital for her MRI/MRA.It was an early start for all of us as we had to be at the hospital by 8:00am.Before any testing or Dr's appts you first must pay.Matt went to pay and when they told him how much it was his jaw dropped and we are surprised he didn't faint.When we first got the estimate for the MRI Jayda's last scans had not been sent,but since the Dr's here have reviewed those scans they decided to do a head,neck and chest MRI/MRA.The original cost estimate we were given was $3500 U.S,so when we were told $5456 U.S. we were shocked and that was with a $1000 U.S. discount.

In the waiting room.

We were all taken to a preparation room,which had a T.V. and colouring for Amelia.The nurse then checked Jayda's pulse and blood pressure and then brought out Jayda's favourite (NOT) the stethoscope.The nurse ended up giving us a plastic stethoscope to take home so Jayda would eventually get used to it after having it to play with. Everthing was fine and next was the I.V. line.This is done a lot better than back home with the nurse putting heat packs on both feet so they can get the veins to show and the I.V. was in first time.

With mum waiting in the preparation room.

With dad watching whats on the tv.

Getting the IV line put in (not happy)

Wrapping the IV line with pink elastoplast.

We were then taken down to the imaging room to wait for the aneathetist to give Jayda her general aneasthetic.Tiff held Jayda as the G.A. was given and just like that Jayda was out of it.The scan took about an hour and a half and we made our way to the recovery area.

Waiting for the GA.

Getting the GA.

Going into the MRI/MRA machine.

The nurse was holding Jayda and she still had her eyes shut but was making some very pleasant sounds,it was unusual to here a child happy after being under a general.Jayda knew as soon as she heard our familiar voices that we were back.Jayda seems to come out the anesthetic quite quick and she recovers very well.We then put in a request for the scans to take home and right away they were given to us.This is very different again to back home as our hospital will NOT give you any imaging unless the Dr's requests it and that is why it took so long to get Jayda's scans sent to Texas Childrens Hospital.

In the recovery room.

So on a whole today was a pleasent experience and again so much different to back home.It has just reinforced us that by coming here Jayda is being seen by some of the best Dr's in the world

Texas Childrens Hospital:Jayda's first appt

Jayda had her first appt this week with Dr Gary Clark who is the chief of Pediatric Neurology at Texas Childrens Hospital.He was extremely nice and we felt very comfortable with him.He looked over the scans that PMH had sent but unfortunately they did not send complete scans so he was unable to get a good view of everything.What he could tell us is this.Jayda's internal carotid arteries are quite large and they are supplying ample blood flow to the brain for the time being.He said that the previous MRI/MRA that showed the high grade stenosis of her basilar artery could be a little deceiving as her ICA are suppling the blood from the front of her brain and that the MRI/MRA might think there is a blockage.He really didn't want to say to much until her MRI/MRA next week.We asked about surgery but he said this area of the brain is very difficult to go into and the risks are very high.We were also introduced to a pediatric stroke Dr and aspirin was dicussed and for now we will not put Jayda on it.Dr Clark was very happy with Jayda's development and surprised that she is walking her self around furniture.So we just have to wait until next week after MRI/MRA and things should be more clearer.

Houston we have landed!

Well we do we start?Our trip was going well until we hit San Francisco where our plane was delayed and by the time we reached Denver we had missed our connecting flight to Houston.The airline put us up in a hotel, gave us dinner vouchers and kept our luggage ready for the flight out in the morning.Now for those in Australia it is winter here and so we were confronted with freezing tempretures and snow and we weren't prepared(clothing wise).This was the first time any of us has seen the snow.When we finally made it to Houston and went to collect our luggage we find out it is still in Denver.Not happy since we had been in the same clothes for nearly two whole days.The airline got it on the first flight and Fedex dropped it off about 5pm at our hotel in Houston,Thank God!!!

Today we went out and looked around where we are staying and we are just amazed how big Houston is,there is nothing in Australia to compare this city to(it is HUGE)The road systems are so complex and the highway is a maze of concrete overpasses.We have come to the conclusion that everything in America is big,the cars,the roads,shops and products and even our microwave and oven in the hotel is abnormally big for what we are used to. We have decided to try the same fast food resturants here that we have back home to compare the taste and there is definately a difference,KFC tastes great here and there is a huge (again I use that word) selection on the menu.

We also went the Texas Childrens Hospital to meet Laura the International Patient Co-ordinator.The hospital is massive and again nothing to compare to back home.The set up is great there is more than one major building that all connect together with in door bridges,the interior is well suited for children beautifully presented.Both of the girls were given a gift from the hospital and we were taken on a tour.It is amazing and makes you think what is happening in Perth?

The people here have been so nice to us and they just love the girls.Also they don't stare at Jayda like they do back home and that has made us feel good especially as Jayda's Hemangioma is growing again.Jayda now see's the Neurologist this week and they are trying to push forward her MRI/MRA to this week,if not it will be next week as planned.