4/2/2016

Well the new titanium rod is in place!  Evan was operated on to remove the cadaver implant and place the new expandable rod on Friday 3/25/2016.  We needed to be at All Children's Hospital by 6:30 AM for admission and Pre-op with an expected surgery time of 8:30.  We left Fort Myers about 4:00 AM and arrived around 6:00 AM.  Evan was understandably nervous as he was well aware of what was about to happen to him.

The pre-op was going fine just as soon as we got the operating gurney out of the room.  Evan did not want to see that at all.  He was cooperative and eventually we gave him a nice cocktail of feel good drugs to get him further in the mood.  His Doctor and Anesthesiologist were both in and the good thing with pain management this time is the Anesthesiologist was able to place the nerve block for as long as necessary instead of just a few hours
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Prior to the commencement of surgery, Meghan was presented with a 7 page document from John Hopkins which All Children's is a part of.  It detailed how the new titanium rod was not FDA approved and the FDA had granted an allowance based on compassionate use.  This is a term the FDA uses when a non approved item would be of a better benefit to a patient even thought it is against the rules.  Evan qualified based on the fact that the other alternative would have been amputation. Stanmore the manufacturer of the device is based in the UK and I believe has been working with the FDA to obtain approval for  device use in the US.  Evan would be the third US approved implantation.  We know of one other patient with the device, her implant was done 3 years ago and involves not only the humerus but a full elbow.  I can tell you that she is doing very well with the device and had the procedure done at All children's with the Moffit team.  There are only 75 total implants done world wide prior to Evan's surgery.

The document went on to describe that the cost of the implant would be determined later and that there may or may not be a fee or co-pay.  The hospital may or may not bill insurance and that she by signing agreed that John Hopkins did not compensate the family in anyway for the device implantation.  The document contained an IRB number which I immediately knew that the device was part of an ongoing study.  The IRB (internal review board) is an independent  group that are advocates for patients and watch over physicians and hospitals when medications or devices or procedures are performed on patients when those items are still not approved by the FDA.  This board requires investigators (hospitals and physician) to regularly report on the device and the patient.   The FDA then reviews how well the device, etc. performed over a set period of time and will approve or disprove based on the performance.  In medications, if the medications caused severe reactions in a large percentage of patients it would not be approved.  In Evan's case, I believe he will be followed for many many years to see if the device performs as expected.  I also think that this is exciting as one of the youngest patients to have the implant will assist in future determination of acceptance by the FDA.  I have experience with the IRB as at my work, my physician has participated in several studies related to eye medications and surgical procedures.

The surgery was expected to take approximately 6 hours and ended up at around 7 hours.  the extra hour was needed to place the catheter for the nerve block so in reality the surgery did take 6 hours.  We were apprised of the progress of the procedure at about 1.5 hour levels.  Eventually we were called back to the conference room for a briefing with the Doctor.  He told us all went extremely well and that Evan had tolerated the surgery and anesthesia as expected.  We discussed how if he was doing well with pain management we could bring him home as early as Sunday with Monday being the most likely day for discharge.  He went over the pictures taken during the procedure and sent me three of them for my collection.  I am amazed at how complex this procedure was and the skill demonstrated by the Doctor.  I believe Evan has a great chance for a successful recovery and a range of motion beyond expectations.  He scar will be minimal and more of a straight line because the stitches are internal and he used Dermabond adhesive to seal the skin together.

Evan's recovery and pain management had its ups and downs but all in all he did very well.  As expected Saturday was very tough with pain management but by Sunday there was a different boy in the bed that was much better and tolerant.  Evan was able to move his hand and wrist almost immediately after surgery and noted a numb thumb sensation.  We expect the numbness to subside as this is a result of manipulation of nerves responsible for hand movement.  We were able to transition off the nerve block Sunday morning and by 6:30 PM we were on our way back home, a very nice Easter present.

Evan will have to have a shoulder immobilizer sling for 6 weeks to keep him from shoulder adduction which is to move the arm away from the body kind of like a chicken wing movement.  The reason for this immobilization is to allow for the muscles and tendons that were either stitched or placed around the new implant time to heal/scar in before movement.  The device was wrapped in a goretex like material to allow muscle to scar onto it.  He is to continue to move his elbow up and down and to move the wrist and flex the fingers.  This brace was designed and put on him early on Monday and he is tolerating the brace well.

Evan has a 2 week post-op appointment in Tampa with the Doctor on Friday 4/8/2016.  I will post on his progress after the visit.

02/14/2016

Since last post things have been almost boring with little to report on. Evan has been growing and going to school and doing well in school. He had a routine X-Ray scheduled here in Fort Myers on February 3 of his arm that was to be sent to the UK for final determination of his new expandable titanium rod.  The X-Ray was anything but normal. Meghan noticed immediately that the allograft was fractured about mid way up the bone. Of course all kinds of panic set in and it took a call from DR. B to alleviate any fears. He ordered a kind of brace for the arm to keep it protected until the rod arrived from the UK. When he left the place custom fitting the device he was in a bit of pain and the doctor had Meghan loosen the straps which helped. For additional support he had Evan wear the original sling he wore right after his first operation. The doctor wanted to see him so an appointment was made for Friday the 12th.
There were more X-rays taken and these were a little different than the Fort Myers pictures. All in all I believe the doctor wanted to see, feel and determine his comfort and motion. He was well satisfied as Evan was not in pain and had a very good range of motion. There was a new fellow there and I could tell by his expressions that Evan should not have the range of motion he has given the state of the allograft. 
There will be no further action for Evan until the rod arrives from the UK and the best estimate is 6 to 8 weeks. It could be longer as there could be production delays. The rod also must go through customs which was a surprise as I thought a medical device would be exempt; weird.
So unless something odd happens, the next blog will occur when we begin the scheduling process.

08/28/2015

The posts on Evan have become sporadic as there is nothing to report, a blessing for him and our family.  He continues to grow and remain healthy and happy.
 Last year we were unable to take a vacation (Sherie and I) as even though the Chemo treatments were winding down the thought of being somewhere other than within 25 minutes of him prevented us from taking a trip.  This year we took all 4 grandchildren on a car trip to visit the great grandparents in Boyertown Pennsylvania and I can tell you the kids had a blast (That is 1200 miles one way).  It was heart warming to see Ethan, Eli, Evan and Elaina having fun together and being able to spend time with their Ggrand parents!

Two weeks ago Evan had his 90 day appointment with his Chemo Oncologist and had X-rays and port flush.  His report card from the Oncologist was A+ and on September 17th he will have a sedated CT scan with contrast for his 1 year check up.  At that time a decision will be made to leave his port in place or remove it.  Hopefully his scans will be clean and we can get rid of 1 more symbol of this evil disease.

Today we had an appointment with the Orthopedic Oncologist in Tampa FL and the visit also went well.  The X-rays revealed that as expected the allograft is deteriorating at the upper end of the graft.  This is a normal happening with the graft as there isn't an area of bone growth medium  If you remember, the head of the humerus had to be almost entirely removed in order to obtain good margins and that wiped out the growth plate.  The screws are beginning to loosen and a phenomenon known as re-absorption is occurring.  When re-absorption occurs, the body absorbs/dissolves the allograft.  This would be worrisome because of the length of the natural bone attached to the elbow may not be long enough, but the Doctor was able to show us that this area of bone had grown at least 1CM since the surgery (that means that in about 1.5 years and through chemo his bone grew).  The Doctor is going to send the new X-rays to the engineers in the UK that custom make the expandable titanium rod to see if they can design one that will sufficiently fit in the lower portion of the natural humerus.  If they can arrive at a satisfactory model we will proceed with the procedure.  The Doctor feels confident they will be able to do so.  Once this procedure is done, it will be the last barring any accidents.  The titanium rod will be sufficient in length to carry him through adulthood. The engineering, design and production will take months so we are being proactive and have a tentative ETA for surgery in early to mid 2016.

We think that overall things are progressing very well and that hopefully the outcome will be a lasting one.  Our family wishes to thank everyone who has been following this amazing story of such a brave young man.  Your wishes, comments and prayers are read and heard.

 

04/25/2015

Yesterday we visited the outpatient clinic for All Children's Hospital in Tampa so the Orthopedic Surgeon could evaluate Evan's progress.  X-rays were taken and copies were given to me for archiving.  The staff at the clinic are very nice and the x-ray technician actually remembers us.

Evan's right arm is stable with the plates and screws firmly attached.  He has a better than expected range of motion and virtually no pain or tenderness.  He has full feeling in the arm demonstrating no resultant nerve damage.  He continues to impress the surgeon.

Evan had stated to Meghan that he feels his right arm is now shorter than his left but the surgeon was able to show us that the difference if any is miniscule and it must be the way Evan uses the arm as opposed to any anatomical differences.

The surgeon still has concern for the upper portion of the ball of the humerus as there is no growth plate there.  The screws that attach the donated cadaver bone are still firmly in place and the "halo" effect has not changed from four months ago.  The halo effect is caused by the loosing of the screws because there is no bone growth and as the ball deteriorates the screws will loosen resulting in the need for surgical intervention.

One very positive item in saving the upper portion of the ball is that it engages and stimulates the glenoid to grow with Evan.  The glenoid is the socket in which the ball is attached and allows Evan the movement he has which as stated earlier is better than expected.  The lower portion of the humerus by the elbow has totally healed with signification bone attachment to the cadaver bone.

Evan has had total regrowth of his hair and has had two haircuts since cessation of Chemo.  He has also gained a few pounds and looks really healthy.  His port is flushed every month and we hope to have that removed at the one year mark since end of Chemo.  He has had two CT scans with contrast that demonstrate that he is a NERD.  The Oncologist is so impressed with his progress that he is delaying further CT scans until there is a concern or year marks.  The CT scans have a fairly good dose of radiation and there must be a balance in exposure so there is no further encouragement of cancer cells.  Continued blood work will be the norm looking for a reason to do a CT scan.

It is totally amazing to me that his surgery was on 03/21/2014 and one year and one month later he is in a totally different position; one of a little boy finally returning to a normal childhood.  He is forever changed because of what has occurred but I feel that the end results will be positive for him and not negative due to family support.

So I know that I haven't updated the blog in awhile but as the saying goes, "no news is good news" certainly holds true here.  I will update when we have significant milestones to report.

One Year Tribute

December 09, 2013 is a day I will never forget.  It started out as a normal Monday morning.  I arrived at work around 7:30 AM as usual and began my routine for the day.  Shortly there after things went to hell in a hand basket as I received a panicked call from Meghan Hampel that Evan was complaining that his right arm hurt really bad.  The family was at  a church event Sunday night and during some normal rough housing that boys do, his brother had accidentally tripped Evan and he had fallen on his right arm.  I told her that if it was bothering him that badly they needed to take him to an emergency room for an evaluation to see if he had broken a bone or just sprained something.  I knew we had an excellent pediatric department at one of the local hospitals, Health Park here in Fort Myers.  After a few hours I received a call from Meghan at the hospital and she had some very bad news; not only was his upper arm broken, it appeared that there was a tumor in the bone that lead up to Evan breaking it so easily.  They thought it was a cancer called Osteosarcoma but they needed to do a biopsy of the bone to determine.
Anyone following this journey knows that Evan has been on a journey in 2014 fighting the beast known as Osteosarcoma and so far this journey has been successful.  It was hard fought and had moments of panic and fright as well as joyous occasions.  We have more to accomplish as Evan grows but as long as we can keep the beast from returning, the future operations necessary to maintain a right arm that is as normal as possible will seem somewhat unremarkable.

A trip on Friday to the Orthopedic Oncology surgeon went very well, the lower portion of the allograft is starting to bone over.  Evan is producing bone cells again since he ceased chemo.  One area of worry is the top portion of the allograft where it is attached to the upper ball portion.  This area does not contain a growth plate and there are no new cells growing there.  The screws that hold the allograft to the ball will eventually loosen to the point that surgical intervention will be necessary.  There is already some loosing happening and we will have to keep a close eye on it.  Other than that, he has better than normal movement in the arm and it is expected that he will continue to gain strength and mobility.  Our hope is a few more years of growth and we can replace the allograft with an expandable titanium rod which will be permanent.

I want to thank all the people who have helped Evan during this journey as well as all the people following his journey through this blog and on Facebook.  The outpouring of concern and love has helped the entire family to overcome this frighting disease.  We know that cancer can return at anytime so we must be vigilant and follow the scanning regiment closely.  Evan is scheduled in early January for full scans and I will report the findings when we receive them.

Everyone have a happy, healthy and merry Christmas!  Our hope is 2015 will be a booster year for us and everyone we know!

11/04/2014

It has been almost two months since my last post and most of you know by now that the testing needed to be done on Evan has been completed.  Evan has been declared NED and is currently disease free!  I waited for some time before posting to the blog to see if anything would arise to negate this great news and so far there hasn't been.  Evan is going to school, growing hair and returning to a life that is normal for a 5 year old.  I must say that all of this ended abruptly and the roller coaster just stopped and you can get off on the happy side  of life; it is a little numbing to the mind to say the least.

Evan will undergo testing periodically to make sure the beast remains at bay and I will report whenever he has the tests.  Happily, I can state that the posts to this blog will begin to diminish, something I never thought would happen.  I will post pictures of his life as normal when I can.

I leave you with a montage of his life since 2013 to current.  Thank you all for following this tremendous journey!

 

09/12/2014

The Chemo is OVER!  Evan had his second to last hospital stay over the labor day weekend and was discharged on Monday 09/01/2014.  His last round was started on 09/04/2014 and he was discharged on 09/08/2014.  He had blood testing yesterday 09/11/2014 and his ANC was excellent at 2067 so he is on his way to having normal white counts again.  He is currently scheduled to have a bone scan (dexa) and a series of x-rays on 09/24/2014 and will have a sedated MRI and Cat scan on 09/25/2014.  These tests will determine if the Osteo sarcoma has been chased away or has returned in another section of bone.  The tests will also determine if there has been any Mets to his lungs or other body parts.  This will put a milestone in his chart that he is NED (no evidence of disease) but he will need to be scanned on a regular basis for at least 5 years as the testing will not pick up very very small growths.  We are very optimistic that he will have clear scans but the waiting for the tests and results are very nerve wracking.

In August we traveled to Tampa to see the Ortho Doctor that did Evans' limb salvage.  He had x-rays of the arm to see if the screws, plates and bone graft were doing.  This was a happy report as the hardware is where it is supposed to be and there is even a very little bit of growth over the graft near the elbow.  This process is expected to accelerate once he stops the chemo completely and since the cessation of chemo has happened he should begin the process.  The Doctor also needed to determine if Evan needed PT so he asked Evan to move his arm up laterally away from  his body as well a up out in front of his body.  The expectation was for around 35 to 40 degrees and he achieved 75 degrees!  There will be no need for PT.  Evan then decided to show the Doctor he could do even better and grabbed his right hand with his left and lifted his right arm above his head.  The Doctor was impressed and Meghan was astounded.  We will see the Doctor again in December.

Evan started school on time and is doing really well.  He will miss time when he is hospitalized or in clinic for testing.  The school and hospital worked out a plan for him that makes sure he is not penalized for time out and that he receives tutoring when hospitalized.  He likes school and actually drags himself to school the very next day after hospitalization.  He is quite the tired puppy when he arrives home.

On a very sad note, a fine young lady that befriended Evan and Meghan in the hospital and had Osteo sarcoma has earned her angel wings.  Wendy was the type of person that would make Evan feel comfortable when in hospital and would even get in bed with him to comfort him.  They shared a special bond.  Wendy was 27 and fought the disease for at least 3 years.  Upon discovery she had Mets to the lungs, had leg salvage surgery and a few lung surgeries.  She was traveling to Moffit in Tampa for a study drug which was inhaled cisplatin but the drug didn't work as designed and she developed more areas in her lung.  She had more lung surgery in a specialty hospital in the Chicago area and returned to Fort Myers on 08/10/2014.  She was at all times determined to beat the beast.  I was at the hospital picking up Evan when her soul mate Shell was in Evans' room.  This was the first time I had met Shell (other than through social media) and Shell told me that Wendy really wanted to meet me as again we only knew each other through social media.  We were on our way down the hall to her room when the ICU team arrived and for privacy reasons I decided to not continue to her room.  I took Meghan and Evan home and found out that on Monday Wendy had passed away.  It was very sad that I didn't get to meet her but I will always remember her as the vibrant, tough young woman that took the time, even though she was hurting and battling her own disease, to nurture and ease the burden of a fellow "Osteoite".  She did this for many other people around the US that had Osteo sarcoma through blogs and Facebook Osteo pages.  There was a well attended memorial service for Wendy on 08/16/2014 that was touching.  On 09/02/2014 Chick-fila where Wendy worked held a fund raiser for the new wing being built at Health Park for ONCOLOGY PEDS department.  This was an event that Wendy had been working on putting together and the event went on as planned in her honor.  The event raised well over 5k for the hospital, a fine testimonial of all the people locally that knew of or about Wendy.

I will report on the findings of the testing that is about to take place.  I sincerely hope that we can all celebrate shortly.