A long overdue update....

We have progress appointments with Dr Paley or one of his team every two weeks.  I keep meaning to update this blog but keep delaying it because we have an appointment coming up and decide to wait until after that appointment to be completely up to date.  We had our progress appointment and x-ray today so no more delaying, I am updating the blog!

Daniel's x-ray from today

Daniel's x-ray looked all good today and we have achieved about 5.5cm in length so we are getting closer to our goal of 8cm!   We are behind about a month because we had some issues with Daniel's fixator not distracting properly.  After a few weeks of Dr Paley's staff having a go at fixing it, Dr Paley had to look at it and it took him about 5 seconds of looking at it to solve the problem.  So, we got back on track.  Today, because Daniel has lost some range of motion in his knee, down from 80 degrees to 70 degrees, they have decided we should slow down lengthening from 1mm a day to 0.75mm a day.  This is pretty normal as we are now in the really difficult stage of lengthening.  Hopefully we can stay at this rate and we should be done lengthening in about a month's time.  

Fixing the Fixator

Daniel's muscles (quadriceps and hamstrings) are getting really tight, you can see how tight they are by looking at them.  The knee bends (or belly bends as the kids call it) are still the toughest.  His muscles are so tight now that it takes all my strength to be able to bend the fixator to get a good range of motion in his knee.  Daniel really loves his therapist, Don.  I asked Daniel if he likes going to PT and he says he does which is a huge advantage.  Some kids throw up before they go to PT because they are so anxious about those belly bends.   The pain these kids go through with their belly bends certainly inspires all the adult patients who witness how brave and strong they are.

Soccer in the hallways for PT with Don, John and Tony

Daniel has to wear a knee bar at night to keep his leg fully extended and this is quite painful.  Although, Shane came up with a genius idea a couple of nights ago which has helped a lot.  For Paley patients - we use a cable tie and tighten it very slowly over about an hour until it is tight enough to put the knee bar in.  It means he gets to sleep with no more screaming and over an hour of massage. He takes some pretty serious pain medication which also helps a lot.

Daniel has started attending an after school program at Max's preschool three times a week.  He loves this as he gets to play with other kids his age and get some time away from all this lengthening business.  It also gives me a couple of hours free time to get some shopping done or grab a coffee.  He is also continuing tutoring three times a week but is begging me to go back to school.

Christmas concert for the After School care kids (second from right)

To give you an example of a day in the life of a Paley patient and his family:

7am - Breakfast and get Max ready for school.  First turn on Daniel's fixator.  Give Daniel his supplement** and his pain meds ready for home therapy.

8am - Take Max to school

8:30am - 9:30am - First session of home therapy - includes warm up with heat pack, electrical stimulation and massage.  Muscle strengthening exercises.  Stretches - foot stretch, abductor stretch, hamstring stretch, hip stretch, two quadricep warm up stretches and ending with the belly bend.  We have to three reptitions of each stretch for 30 seconds each.

10:30am - 11:30am - Pin site wound cleaning - includes disenfecting the bath, epsom salt bath, removing old bandages, cleaning wounds with antibacterial soap, removing debris and gunk from each pin site.  Allowing to dry before re-wrapping with new bandages.

11:30am - 12:00pm - Lunch time, second pin turn, supplements and pain meds for PT.

12:15pm - 1:00pm - Travel to PT at the hospital

1:00pm - 2:00pm - PT with Daniel's physical therapist

3:00pm - 5:00pm - School work with tutor or after school care (pick up Max)

5:30pm - 6:30pm - Third pin turn, dinner, take pain meds for next home therapy session

7:00pm - Get Max to bed.

7:00pm - 8:00pm - Second session of home therapy, final pin turn, supplement, valium for muscle spasms caused by knee bar, story, brush teeth.

8:00pm - 9:00pm - Slowly insert knee bar until fully in, hoping he does not wake in pain.

Weekends are pretty much the same except we don't have PT at the hospital but we have to do three home sessions which is actually harder because we feel like we spend all our time on PT and pin care and not much time for normal weekend activities.

Lengthening is really tough but we have had some pretty wonderful experiences over the last couple of months that make it much more bearable.

Dr Paley presented Daniel's case to a room full of doctors.  I was so proud of Daniel standing up the front showing these doctors his fixator and his x-rays up on the big screen.  If Daniel can teach other doctors to help other children with orthopaedic conditions, that is a pretty amazing feat for a 6 year old.

Daniel and I raised over $1,000 for the Paley Foundation in the annual Palm Beaches marathon.  We were invited to a party at Dr Paley's house to celebrate our achievement.

Collecting coconuts from Dr Paley's beachside home

The next day, Shane, Max, Daniel and I walked in the marathon.  It was an amazing experience walking with such a big group of Paley patients.  We were so proud of Max who walked 5km and didn't once complain.  We pushed Daniel in his wheelchair but he walked for the last part which was lined with people cheering him on.  It was very emotional for both of us.  After the race, there was a breakfast for the Paley team and we got to meet other families who we have known for several years over Facebook and email.  We really felt part of a wonderful and strong community of families who are going on the same journey as us. 

We have experienced first hand how charitable people are in Florida.  Although we are not staying there, we have been able to attend events at Quantum House (similar to Ronald McDonald House).  We had Thanksgiving and Christmas dinner there.  Breakers Resort cooked Thanksgiving dinner for all the families and a family cooked Christmas dinner for us.  We were invited to attend the Help-Portrait event where photographers give us a free family photo shoot.  This is a global event that occurs on one day all around the world.  The Boys and Girls Brigade invited all the Paley patients to the Hilton and gave all the kids presents and cooked dinner for us.  The hospital held a Christmas event for all the kids and they got to fill their sacks with presents and have a photo with Santa.

Santa visiting the Childrens' Hospital

Christmas Day at Quantum House - Generous people donated presents including a handmade blanket

Dusty from Disney Planes visited the kids for Thanksgiving

Once a month, the Psychology PhD students at the local university host a session for all the patients and their families.  They provide us with dinner first and then we break into a parents group and patients group and talk about a different topic each month, for example, pain management, stress, being away from home over the holidays.  It is great to talk to other parents who really understand this process and the kids really enjoy it, including Max who comes along too.

We have had some visitors to Palm Beach since we have been here.  Our friend Steph and our nephew Mark have stayed with us.  We also had a visit from my parent's friends, Jenny and Neville.  We haven't had a lot of time for socialising but we spend time with other Paley patients which is great as we can talk Paley patient lingo, share what we have learnt along the way and they understand how time limited we are and that we need to get home or we won't get our last home therapy session in before bed time! I think we will really miss this when we go home (although we can't wait to see our family and friends again!).

Pass the parcel with other PFFD Paley patients

So there you go, a very lengthy and long-overdue update.  Thanks everyone as always for your support, we couldn't do it without it.

Posted by Kristen (Daniel's Mum)

** FYI Paley patients - The supplements Daniel is taking were recommended by Dr Paley - www.bonehealthnow.com

On our way...Daniel's first lengthening begins

It's been a while since I updated the blog and a lot has happened so its not going to be a short one!  It has been so busy and continues to be so I am finding a little time to give you all an update. 

We left Singapore at the end of September, packing up everything into storage and moving to West Palm Beach for the duration of Daniel's lengthening.  It was very difficult leaving, the kids love their life in Singapore.  Daniel was loving his school and had a great group of friends at our condo.  He was very settled and very happy.  I remember reading at some point how lengthening robs children of their childhood and I really felt anxious about this leading up to Daniel's surgery.  During the previous surgeries, Daniel's life was centered around his family but at age 6 he is really independent and has his own little life.  But, we have thought about this a lot, and still feel that it is the right thing to do and it is something that Daniel wants.

Goodbye and see you soon to our Singapore friends!

My parents met us in Palm Beach to help us settle in.  They were incredible help and I don't think we could have done it without them.  From settling Max into a new school, to learning to drive again after 10 years (on the other side of the road too!) and Daniel's big surgery, there was a lot of anxiety so have my parents there was a huge help.  Thanks Mum and Dad!!

We enrolled Max in a preschool in the local area.  After a week of separation anxiety and tears, he settled in and enjoys going to his new school.  We will see what 8 months in an American school will do to his accent!  I asked Max the other day whether he is English, Australian, Singaporean or American and he replied, "I am Spanish!"  He is learning Spanish at his school so its not a crazy response. 

Daniel is not attending school during the lengthening but we have found him some tutors which come in the afternoons.  He absolutely loves it and tells me it is the best part of his day and wishes he could do tutoring every day.  He would love to go to school though, he really misses it.  He cried the first day Max went off the school because he wasn't able to go.

So, now on to the big event....the surgery.

Daniel had his pre-op appointments on the 7th October.  That was not without drama, it took 3 attempts to get blood out of his arm.  It was awful watching the nurses dig around trying to get the blood to flow.  We ended up going to see the vampires (what we call the laboratory staff who visit us every morning at 5am) and they were able to get the blood to flow.  He was told he couldn't take of his red blood band or they would need to draw blood again.  Three weeks post surgery and he still hasn't taken it off!

The biggest drama was on the day of the operation.  We arrived at the hospital at 9am on 8th October.  It was a long wait and Daniel wasn't given the pre-med until 2pm so obviously by this time he was very hungry.  Shane took him in to the operating theatre to be put under.  As the staff were about the put him under, Dr Paley rushed in and asked to speak to Shane.  Dr Paley had a family emergency and would have to postpone the surgery.  Daniel was taken into recovery until the pre-med wore off.  Meanwhile, one of Dr Paley's PAs came in to speak to me.  The look in his face completely freaked me out, I thought how could something have already gone wrong during surgery!  It felt like we were in an episode of Grey's Anatomy!

We were back on again a couple of days later.  Dr Paley was on a morning flight and called when he landed to get Daniel prepped for surgery.  The surgery went for about 6 hours and went really well.  We were excited to hear that he wouldn't need pins in his hip (these really bothered him last time).  It also meant 3 less pins to clean (he has 11 pins in total, 8 in his femur and 3 in his tibia).

When we went into recovery to see Daniel, he was sucking on a popsicle.  He was in quite a bit of pain but was slipping in and out of sleep.  Daniel had a epidural to help with the pain and once this started working well, his pain was well managed during his time in hospital.  We had one instance where his pain went up to a ten but the anesthetist was able to give him something extra.  Daniel was pretty good at telling the nurses his level of pain (from 1 to 10).  The physiotherapists would get him out of bed each day and into a wheelchair but most of the time he was zoned out watching the cartoon network.  We were allowed to go home after 4 nights in hospital. 

In hospital, post surgery

Out of bed, into his wheelchair

Ready for a walker race

There was no rest as the next day we had our first PT (physical therapy) session and pin turning/pin site cleaning session with Tony.  Four times a day we have to use a small wrench to turn a dial on Daniel's external fixator a 1/4 of a millimeter, totalling 1 mm a day.  Daniel has PT five times a week and we have to do 2 sessions at home as well as pin site cleaning.  PT involves some strengthening exercises but primarily a series of stretches.  The worst one is left to last and all the kids hate it - the dreaded "belly bend".   This involves laying on your stomach and pulling your foot into your butt to bend your knee.  Over the first couple of weeks we worked up to Daniel being able to bend 90 degrees which is the maximum required.  This was painful because there was a lot of swelling and pain around the pin sites.  Daniel works really hard to get his knee up to 90 by himself with us just helping with the last few degrees.  This is really painful for him and we have been working on him breathing during the stretch, kind of like what they tell you to do during pregnancy contractions.  We need to try and maintain this angle, if we lose too much motion in his knee we have to slow down the lengthening process or stop which we don't want to do.  The external fixator grows the bone but the only way to grow the soft tissues is by stretching them. 

The fixator

At first, Daniel would yell at me a lot telling me he doesn't like me and I always try to hurt him.  But the last week, he has been coping a lot better.  He understands what he needs to do and he wants to achieve it.  Last week after we finished his painful belly bends, he wiped away his tears and took off with his walker.  He came back with a sweet and said, "This is for you Mama, for doing my stretches".  The next day he got out 2 plastic medals he got from the treasure box at PT and gave one to me.  He said, "These are for us Mama, because we are a good team".  He is really proud of himself and is very determined to grow his leg longer.  Sometimes he even tells me I am not stretching enough because its not hurting enough.  We are very proud of him!

We had our first x-ray a week ago and it all looks good.  We have already grown his leg 2 cm longer, well on our way to achieving our 8 cm goal.  It will get a lot tougher though as his muscle starts tightening up.  Dr Paley is going to present Daniel's case to a group of doctors as part of a training session so our next x-ray will be taken then.  After that, we will have an x-ray taken every 2 weeks during the lengthening phase.

First post op x-ray

Both the boys enjoyed celebrating Halloween in the USA this year.  Max's school put on a "Trunk or Treat" after school which we were all able to go to for trick or treating and Halloween themed games.  Daniel went to the celebrations put on my the Children's Hospital.  It was pretty amazing, all the staff were dressed as minions from Despicable Me.  We were made to feel very special!  Our next celebration will be Thanksgiving.  We are looking forward to spending our first Thanksgiving with Quantum House, where families stay during their stay at the hospital (similar to Ronald McDonald House). 

Trick or Treating at the Children's Hospital

Max's Halloween costume - Wolverine

Before I sign off for now, Daniel and I are entering the Palm Beaches 5K on the 8 December to raise money for the Paley Foundation, Dr Paley's foundation which strives to improve the lives of children and adults who suffer from rare and challenging congenital, developmental and post-traumatic orthopedic conditions throughout world.  We would love it if you could sponsor us, no amount is too small.  Thanks to everyone who has donated so far, we really appreciate your support. Just click on this link - http://www.paleyfoundation.org/2013-Palm-Beaches-Marathon/DanielRigby

Thanks to everyone who has sent us messages of support via email and Facebook.    It means a lot to us!!





Posted by Kristen (Daniel's Mum)

Looks GREAT!

"Look's GREAT!"  Dr Paley's verdict on Daniel's latest x-ray.  Yay!!!

So preparations for Daniel's first limb lengthening have begun.  We have booked in a surgery date for October 8th this year. We will need to stay in West Palm Beach for about 6 to 8 months.  We have quite a lot of work to do before then.  We need to:

• Find somewhere to live (thinking of a house in Palm Beach Gardens)
• Find a preschool for Max 
• Find a tutor for Daniel to continue his school work
• Buy or lease a car
• Apply for visas to the US which in it self requires quite a list of paperwork (we need one because we will be there for longer than 90 days)
• Organise our insurance company to pay the hefty bill (got the cost estimate yesterday and let's just say we could buy a house for the cost of extending Daniel's leg by 8cms so everything crossed that the insurance company pay up!)
• Move out of our condo in Singapore and put all our things in storage
• Find somewhere for Shane to stay when he is working in Singapore (Shane is going to stay in Singapore and continue working but come to the US whenever he can to visit)
• Book flights (can't fly much further than Singapore to Miami!) 
• Bring our dog Tommy to Florida or find a kind family to care for him while we are gone

Have I forgot anything fellow international Paley patients?  Oh, and I have just started back at work so this will be my after hours project.  But it keeps my mind busy and distracted from what I know is ahead of us.

Daniel is excited about the trip to Florida and keeps asking when we are going.  We have talked about the fixator, pin site cleaning and physiotherapy but he does not appear to concerned about it. Daniel is very gift/reward driven so he is talking a lot about what presents he will get in hospital and treats he can eat, probably a product of the countless number of reward charts we had to use for his last surgery.  Daniel has quarterly blood tests and tells me he wishes he had a blood test every week so he can visit Toys R Us.  He just puts his hand out and has the blood test with no fuss and then usually follows up with a critique on how the doctor did and then its off with his $5 budget to Toys R Us. I think we will leave all our toys in Singapore because I think we will be buying out the store for this next surgery. 

I have asked Daniel whether he wants to have his little leg longer.  He said he does because he will be able to do more things so I think he is becoming aware of his limitations.  He also is getting fed up with people asking him about his leg.  He doesn't get upset, he just gets annoyed and frustrated.  Probably like when I got engaged or pregnant and I was sick to death by the end of people asking the same questions over and over! His best friend, Maya, thought it would be great if they had a special chrystal which stopped people always asking so I think she is getting sick of it too.

So, I think we are almost at the right age to start lengthening his leg.  He wants it now but before he couldn't have cared less whether he had a short leg or not.

Daniel's recent x-ray showed a difference of 15cm which after plugging into Dr Paley's "Paley Growth" iPhone app predicts a difference of 26 cm at maturity.  This has improved from the 30cm initially predicted when Daniel was a baby so hopefully it improves even more.

Each lengthening we are aiming for 8cm of additional length in Daniel's short leg (each of the lines on the x-ray above is 4cm). This will take it from 15cm difference to 7cm which will make a huge difference even though we won't get to even legs this time around.  Daniel should be able to get around without a prosthetic, just standing up on his toes and will be able to have a shoe lift instead of a prosthesis.   He will also find it easier to sit on chairs.  When your knee is in the wrong place, standard chairs were not really built for you.

So, how do they lengthen?  The first four months is the lengthening period.  Basically, Daniel's femur bone will be broken in surgery and an external fixator applied.  Each day we will turn a screw on the external fixator which will pull the ends of the bone apart about a millimeter a day.  As bone is a living substance, new bone grows at each end.  Growing bone is the easy bit, the hard bit is stretching the muscles and nerves.  Dr Paley requires all his patients to stay in West Palm Beach to attend twice daily physiotherapy, land and pool therapy.  We will see Dr Paley every two weeks for a follow up and we will need to do exercises at home. 

The second four months is the consolidation period where we stop lengthening but the external fixator stays on and daily physical therapy continues.  A lot of patients go home for this period but because we live so far away, we have decided to stay in West Palm Beach where we have access to Dr Paley and the best physiotherapists.   Once the fixator is removed we can return to Singapore where Daniel will continue physiotherapy but be able to return to school and all his activities.  We will need to go through this process two more times at age 10 and 14 and then hopefully, Daniel will have two equal legs.   We will take each lengthening as it comes though to see how Daniel copes with it both physically and emotionally.  

Here is a good link to the process on Dr Paley's website - limb lengthening.  Also, here is a video that takes you through the Paley Institute.

On non-limb related news, Daniel started big school in January and Max started at preschool.  They enjoy getting the school bus together every morning and afternoon.  They are both really enjoying school life.

Little Bro Max will start at a new preschool in a new country (again!) when we go to Florida.  He is a pretty adaptable and easy going kid which makes it easier.  He will see his brother go through something pretty tough though so we will need to keep a close eye on him.  He was really just a baby when Daniel had his hip surgeries.

Daniel is still doing Taekwondo and was awarded his yellow belt recently.  He is now aiming for green stripe.  He is still swimming and can swim 200m freestyle.  He still loves his mixed sports program every weekend and has just taken up piano.  He really enjoys piano lessons and we are hoping to continue them in Florida. With Daniel's Dad being a keen musician, a wall full of guitars, a piano and a drumkit in our living room, we hope music can become a passion for him that he can keep up even during his lengthenings.

This will probably be my last post before we depart for Florida so until then we are going to live a fixator-life to the fullest!

Posted By Kristen (Daniel's Mum)