Thursday, December 6, 2018

The Mayo - A week of learning

In my last post I told you that we have decided to go to the Mayo Clinic in Rochester Minnesota to get help making a decision on the best treatment for Brandon's scoliosis and osteoporosis.  We have two very different recommendations for surgery, both of which are major surgeries for Brandon to repair his scoliosis.  

Since my last post the Mayo has been incredibly helpful.  They called and gave me options for dates to come to Rochester Minnesota and I was a little shocked that the dates offered were so soon.  I was also surprised that we will be in Minnesota for at least 1 week for assessments.  The Mayo has called almost daily since the United medical director called and made the referral.  The best call was the Friday before we left when a patient relations representative checked to see when we are arriving, what hotel we are staying in and then told us she would meet us in the lobby and take us to our first appointment.  I have to say that was a big relief and one less thing I have to deal with - finding the office for the first appointment at 6:45 am in this mass of building called the Mayo Clinic.  Two weeks ago I drove all over Austin getting medical records and Cd's with radiology films.  I finished sending medical records to the Mayo.  But I had another conversation with the Mayo before we left about records I didn't include.  They asked for me to bring them.  I am not packing another 15 pounds of records so I scanned more documents.  There really isn't anything left that is not now in a PDF file.  When we got to the Mayo they still asked me to walk around with some of the records in hard copy just to make sure everyone had what they needed.  We are a long way to go in this country to have electronic medical records.  It makes it much harder for people like me and Brandon trying to have as much info available for better health decisions and better outcomes.   

Brandon and I left Sunday evening for the direct flight to Minneapolis.  Minneapolis is about 1 1/2 hours from the Mayo which is located in Rochester.  Luckily my friend Carrie Coleman just happened to be on the same flight and we booked our seats together.  She helped us make it to our shuttle to Rochester.  We didn't get to our hotel (Grand Kahler) until 1:00 am and then to bed by 1:30 am.  This was good because Brandon was only supposed to get 4 hours of sleep for the EEG they wanted to perform the next morning.  That also meant I only had 4 hours of sleep.  Rochester just had is first major snow and we came in while it was snowing.  Really it hasn't stopped since we have been here and it hasn't been above 32 so the snow is staying.  The Grand Kahler has an underground walkway so we don't have to walk out in the cold and all the buildings connect.   The hotel is a great location but probably could use a little upgrading.  There are new hotels being build all around the Mayo.  

Monday we met Brandon's new best friend Christel (Mayo assigned us a patient representative from the UHG Spine Program) in the lobby of our hotel at 6:45 am.  The first day was a day of meeting with our lead internal medicine physician who will put an entire plan together for Brandon concerning his spine surgery, scoliosis and genetics.  Then Brandon had a bone density test, spine imaging and an EEG. Brandon has developed an infection at his gtube site so there was a little extra time figuring out treatment.  It was a long day but ended around 3:00.  Brandon and I went to eat and then to bed early. 

Brandon getting ready for the EEG and with his new BFF Christel our patient representative 
who took us to all the visits.   

Tuesday was all about the bones.  But first we had blood tests and Brandon was on a strict diet for the blood tests the night before.  After the blood tests we had breakfast and then met with the endocrinologist to go over the results of the done density tests.  The good news is the highest number for Brandon's bone density went down to -2.8 (the high previously began was -3.7).  Still osteoporosis, but much better results.  The other good news is the lumbar spine overall score is 1.8.  Major progress.  The doctor would like Brandon's highest score to be below 2 before having surgery.  The other news is that there is no treatment approved for men, especially men below age 50 for bone growth beyond 2 years total.  That means once we have completed forteo we will have to move to  different stabilizing treatment. The doctor recommends prolia 60 mg subcutaneous injection twice a year after forteo.  Prolia will help him gain further bone density slowly over time more than Reclast.  If Brandon doesn't have surgery within the period of Brandon using forteo (which is preferred), then surgery cannot be performed until after 6 month from an injection of Prolia.  They are also watching new drugs soon to be approved, but have not seen a change in allowing more than 2 years of treatment.  

Our second visit was with an integral medicine doctor specializing in eastern medicine.  We both really liked her.  She wanted to check for drug interactions with some of the medications and supplements Brandon is using.  The main recommendation for Brandon is to stop using Vega his supplement we use as a base and change to active probiotics.  Here are her recommendations:

I recommend switching to whole foods, whenever possible.
In 2018 the Clean Label Project, a nonprofit organization that examines labeling safety issues, tested 134 protein powders from 52 top-selling brands. They found that virtually all of the 134 products tested contained detectable levels of at least one heavy metal and 55 percent tested positive for BPA. Overall, plant based products fared worse than whey based products. This included the Vega brand, which was ranked poorly and was listed amongst the 5 worst brands. A 2010 study by Consumer Reports of 15 products found arsenic, cadmium, lead and/or mercury in samples of all the 15 powders tested, a few in excess of the maximum USP acceptable intake.  
Regarding Multivitamin Supplements - The worst brands and products  in the Clean Label study: Garden of Life, Organic Shake & Meal Replacement Chocolate Cacao Raw Organic Meal; Natures Best, Isopure Creamy Vanilla Zero Carb; Quest, Chocolate Milkshake Protein Powder;
360Cut, Performance Supplements 360PRO Whey Chocolate Silk Premium Whey Protein; Vega, Sport Plant-Based Vanilla Performance Protein.
Best overall (but still tested positive for contaminants):
Pure Protein Vanilla Cream 100% WheyPerformix Pro Whey Sabor Vanilla Protein with Amino BeadsBodyFortress Super Advanced Vanilla 100% Whey Protein BioChem Vanilla 100% Whey ProteinPuori PW1 Vanilla Pure Whey Protein
Regarding probiotics: Overall, I highly recommend probiotics obtained form whole foods, such as kefir (Nancy's kefir is one example), pickles, sauerkraut, kimchi, kombucha tea, or miso.  
There can be substantial variability between probiotic supplement products based on manufacturing methods, storage, and species-related or strain-related differences. Generally, look for active or live cultures on a label, with colonies in the 10-20 billions. The best supporting evidence, generally speaking, is for Saccharomyces boulardii or Lactobacillus GG. For functional constipation Lactobacillus reuteri DSM 17938 () - 200 million colony-forming units daily for adults can be considered. For irritable bowel syndrome, Bifidobacterium infantis 100 million live bacteria daily can be considered.
I hope that helps anyone on supplements or probiotics.  

Brandon in the spine clinic
Wednesday was all about the spine.  We saw the spine surgeon first.  Some new information.  The surgeon compared spine xrays from 2007, 2014 and to the current xray done Monday.  When you look at these comparisons it appears that the 2007 and 2018 xrays are similar (49 and 51%).  The 2014 xray may have been in a different position than the others.  Basically it is very hard to confirm that Brandon has had significant decline.  In addition, the surgeon here believes it is possible Brandon has had a fracture at the upper portion of the spine and probably due to osteoporosis.  It is hard to see on the film, but two physicians here believe it is possible.  They are recommending that an MRI be performed on the entire spine so we can clearly see if there is a fracture in this area or any other fractures.  If there is a fracture Brandon's surgery will need to have a rod placed in Brandon's spine above the fracture and all the way to the pelvis if or when we move forward with surgery.  The surgeon here believes that the plan from Dr Viere in Dallas is the better or the two options.  He did not agree with the surgeon in Houston's recommendation.  Here is what I gathered from the conversations:
  1. Dr Viere's (the Dallas surgeon) approach for surgery more closely aligns with the Mayo's recommendation. He was the one with the more extensive surgery. However, if Brandon does have a fracture at the upper part of the spine a rod may need to go down almost the entire spine and fused to the pelvis. Even more extensive than the one recommended by Dr Viere.
  2. They do not believe we should move forward with surgery unless we can confirm that Brandon's scoliosis is declining. Based on various x-rays it is unclear. They recommend following his back using the same machines for x-rays and the same approach to take the x-rays (i.e. hands to the side, hands above, standing straight, etc).
  3. Surgery is not imminent unless we see a decline (that would include any fractures).
  4. They recommend that Brandon get an MRI of the spine (also Dr Vere's recommendation) but different from Dr. Viere they want the MRI for the complete spine and determine if there is fracture at the upper part of the spine or any other part of the spine. They are suggesting the MRI be done within 1 month of this appointment. I will  ask Dr. Viere to do these in Dallas.  
  5. Brandon has scattered degenerative arthritis throughout the spine and abnormalities in the hips. This appears to be normal for Brandon due to his osteoporosis, but not normal for a healthy young man of his age. I will ask them to continue to monitor the hips.
  6. Brandon then saw an OT and a physical medicine doctor who recommended Brandon have physical therapy to work with Brandon's lower extremities which will support the spine after surgery. Brandon's legs, feet, etc are spastic and need more flexibility. They have recommended no more than 10 pounds for lifting, fall prevention since it appears Brandon has had multiple fractures, and a few other precautions for him to remember.  In  addition, they recommend light weight lifting and walking 30 to 40 minutes 3 days a week.  
That evening we had to have some better food - gluten free, almond milk etc.  So we ventured out thanks to Uber and went to the local People's Food Co-op.  Loved it.  It is similar to a small Whole Foods.  When we left it was snowing again.  

Thursday was all about neurology and genetics.  Brandon was fortunate enough to meet with neurology for this visit and go over his medicines and diagnosis.  The neurologist agreed to Brandon's medicine but also made a suggestion of a new medicine to consider in the event we decide to move.  In addition, he ordered a new more defined MRI of the brain.  We will do this Friday morning before we leave here.  

This was the longest appointment because we have so many tests already done for review.  I think it is the consensus from all the doctors that Brandon has an underlying genetic condition impacting the bones and vitamin D absorption.  But none of the obvious ones identified in his chart can explain his condition.  They are running 1 additional genetic test, but they are also going to contact Baylor and download Brandon's complete genome report.  In addition, Brandon signed up for a research study for patients like Brandon who have not found a diagnosis through any other method (genetic, metabolic, etc).  The study is looking for more ways to diagnose patients through whole genome sequencing.  They will continue to work on Brandon's case until they find a diagnosis.  This is paid through the Mayo research funding and could last for years.  

Ready to Come Home and Brandon's Birthday
Brandon is a little homesick and ready to come home, but the only thing keeping him from really complaining loudly is that we have scheduled to meet with another Beatles fan we connected with who has met 3 of the Beatles.  He is looking forward to that tomorrow night in Minneapolis.  We come back to Texas on Saturday staying with Carrie tomorrow night.  Then Brandon turns 26 on Sunday.  Happy Birthday Buddy.  

This says it all for the week.  

The Mayo
Lastly, I cant thank the Mayo team here enough for all the help they have provided this week.  Squeezing appointments in for the week, meeting us and taking us to each appointment, finding the right physicians to meet with during our time here so we can make better decisions.  Everyone has been very helpful, know their stuff, made sure we don't have any holdups and going that extra mile.  I feel like we have a good plan. I am much less stressed than I was before I came.  I also feel like I have a team here to help in case we get stuck again.  All round great customer experience.  

It was a memorable this week as the nation celebrates the life of George Bush.  George Bush and Barbara Bush were major supporters of the Mayo Clinic.  In fact, Barbara Bush was on their board.  Many great stories about them this week here and a small memorial on one of the floors where Brandon had many visits.  This added to the fact that George Bush signed the bill allowing people with disabilities equal rights so people with disabilities could have the same education and work at jobs equal to their peers.  He was a man who devoted his life to public service and Brandon has benefited from his efforts.  

Related image

That is all for now.  Thanks everyone for supporting us as we stayed here at the Mayo.  Looking forward to being back in warm Texas. 

Friday, November 16, 2018

Back to the Back - the quest for the best outcome

For the last few months I have been quietly taking in more information in an attempt to make a decision concerning Brandon's scoliosis surgery.  We were lucky to have a nice break with Brandon meeting Paul McCartney and a few musical performances Brandon and his band had the last few months.  But I haven't stopped trying to gather the best information I can find concerning the recommendations Brandon has received from several very qualified physicians.  I have come to some conclusions.
  • Everyone agrees Brandon must have surgery or his condition will continue to decline.  
  • Brandon's multiple conditions make a decision difficult for me and the physicians treating Brandon, perhaps intimidating. 
  • All the physicians are confident in their recommendations, but appear to recommend treatment based on expertise they have gained treating patients like Brandon.  There does not seems to be a clinical norm for Brandon's treatment.  
  • Everyone would like an explanation of why Brandon has such severe osteoporosis.  Me too. 
  • I will be hesitant to move forward on anything until I feel comfortable with the outcome.  I am not a clinician and need help working through how to make me feel more comfortable making a decision.  
  • Whomever treats Brandon better like the Beatles.  
I have reviewed all three recommendations for surgery.  I decided not to move forward with the surgeon in Austin mainly because he has less experience operating on people with complex medical conditions.  The other two surgeons have drastically different recommendations.  Neither of the recommendations are wrong, just a different philosophy on how to repair the spine for this specific population.  

In the last month I reviewed one of the recommendations put in writing, I called the other surgeon and clarified his recommendations.  I then compared their recommendations.  I don't often talk about working at UnitedHealthcare but they have been incredibly helpful even though perhaps by accident.  United has a mandatory "spine" program for employees.  I was hesitant to sign up for the program because I often find these programs are for someone without complex conditions and can just add more work for me.   That is the case for the "spine" program, but the staff still wanted to help me find the right solution.  They guided me on questions to ask the surgeons.  I set up two more calls and received more clarification on exactly what they are recommending.  After getting those additional details I worked with our assigned nurse and went over the two plans.  She took the information and talked to the medical director of the program.  He recommended that Brandon go to one of three centers of excellence to help us work through what surgery options will lead to the best outcomes.  Today I had a 40 minute call with the medical director and we agreed that the best place for Brandon to go next before deciding which surgery is the Mayo Clinic.  We decided on the Mayo clinic because of the conclusions above.  The Mayo clinic will develop a team approach for looking at Brandon's options including determining any underlying cause of the osteoporosis and if this may have an impact on the overall results from surgery.  They also have a spine program that is number 1 and 2 rated in the United States.  I placed a call to the Mayo and started the application process which took 40 minutes. My new BFF medical director will call the Mayo clinic to determine the best path with them and try to help us through the process.  For those of you that don't know, the Mayo Clinic is in Minnesota.  It will probably be cold there when we go.  I don't really love the cold but hey - thinking of it as an adventure.    

So this all sounds like good news but it is pretty depressing for me and brings up how medically complex Brandon truly is.  I have been a little sad this week.  Trying to think of the positive but any change with Brandon always results in the grieving process starting over for me.  I am telling you this because as a mom I am not the only one.  The next time you hear a mom with a child with autism talk about a new struggle with her child, or a mom trying to work through her child having new seizures that were previously under control, or a child's behavior changing, know that that mom will probably go through the grieving process at some point.  I hope you support that mom as she, her family and her child work through the change.  

I think I talked about this previously but I have to point out that next week I am taking the week off.  It is my birthday. Taking a Leah and Tony day in San Antonio. Brandon has a dental procedure on Monday under sedation. The rest of the week I will be copying and faxing Brandon's complete medical records to the Mayo.  This includes ordering and picking up copies of xrays on CDs from various facilities all over Austin.  The reason why? - the Mayo as with Baylor and all the other facilities we have been to so far do not accept electronic medical records.  They must either be faxed or mailed.  This is kind of shocking to me, but I will take a day for collecting all the records and getting them to the Mayo.  

Some really good news - Brandon was accepted by UnitedHealthcare to continue as a dependent under my coverage indefinitely due to his disability after his birthday in December where he will turn 26.  I will blog on this separately for those parents looking to understand this process.  

Brandon also had an appointment with Sharon Hausman-Cohen (his integrative medicine doctor who I love) a few weeks ago and we were able to get extensive results back from her new genetics program.  I will also blog on this separately as it deserves its own post.  As a result we have modified Brandon's supplements.  

AND - It looks like Brandon will start the new CBD oil drug Epidiolex approved by he FDA.  He should start this in December.  The hope is to start lowering Briviact (his seizure medication).  

Too much going on.  That's all for now,

Have a great Thanksgiving Holiday.  Remember to hug your loved ones and I hope you are able to spend time with your family.  

And as Brandon says - ALL YOU NEED IS LOVE.  


Saturday, October 20, 2018

The All Good News Post

Brandon and I had a nice reprieve of not thinking about or seeing doctors for  few weeks thanks to Sir Paul McCartney.  I am just giving a short overview of our day to feel like rock stars.  I wrote an email to ask whomever would listen to give Brandon the opportunity to meet Paul McCartney.  Many people gave me suggestions as I sent this email out on who I should contact and I have to give thanks to each of you who lobbied for Brandon, giving me suggestions and pointing me in the right direction.  The email ended up being sent to Paul's company with a "not promising anything but at least getting it in the right place".  The news that Brandon was finally going to meet Paul came when we probably needed the most - at the airport coming back from our last of all the specialist's appointments.  I was so tired I just wanted to get on the plane so I could take a nap.  Brandon had already taken a nap in the airport and the doctor's office.  Thanks to Peggy for driving us a around that day.  At the airport I decided to look over my email when I saw an email from Paul's charity offering Brandon a private meet and greet with Sir Paul at ACL.  When I told Brandon he started squealing and saying he could not believe he was going to meet Paul McCartney.  That woke us up to get on the plane and home.  

Brandon at the airport

We received an email the next week explaining the location of the tickets and packets for backstage passes.  The team offered back stage passes for Rush Evans (Off the Beatle Path) as well who is the biggest Beatles fan as Brandon. Tony who was with is for the evening also received backstage passes.  It was good to have both of them there because backstage can be a little challenging for person who cant see down very well and is not great walking on uneven surfaces.  But those challenges did not slow down Brandon.  The day before the event Michele from Paul's team (who has been with him 28 years) contacted us about how we were getting to the concert, what time etc.  After talking to her and explaining we may have to use the wheelchair to get Brandon around ACL (if you have gone to ACL you know that is a little tricky).  She offered to have a car sent for us and to drive us right to the Paul McCartney backstage area.  It was a relief for me.  The day of the concert the car showed up to pick us up with Albert our driver (sweet guy) who had our passes and extra tickets we gave to Brandon's aides.  He drove us right to the area where Paul was set up.  

Brandon and Rush

We had shirts made here in Dripping Springs for Brandon by Emily Heckmann Konkle .  Love them.  

 Michele greeted us and showed us to the backstage AC lounge where there were drinks and snacks.  We wanted to go out front to see David Byrne.  The staff helped us through the maze and Brandon was able to sit in the disability section right up front.  Great show.  We went back to the lounge after his set.  Rush was able to meet many of the musicians hanging out in the back, but Brandon, Tony and I hung in the lounge in the AC.  It was pretty hot and I wanted to preserve some of Brandon's energy.  We met the whole horn section who were so nice to Brandon asking him about his love of Paul and the Beatles.  During the show they all turned to him and gave him the thumbs up and one of them came up to check to see if Brandon was loving the show - he was.  

The horn section 

About 15 minutes before Paul was about to perform they took everyone back stage to a balcony on stage right above the band.  It was the most amazing view of the show and looking out at 80,000 Austin fans cheering on Paul.  They took Brandon and me to a private area to meeting Paul.  Brandon had a ukulele that our friend Scott Fredrick put the Egypt Station art on it and Brandon handed it to Paul.  Paul immediately started playing the song he wrote "Dance Tonight" on the ukulele and singing it.  Brandon and I danced to it.

When he was done Brandon told him that he "has been listening to his music all his life".  He also told him that he listens to the Beatles radio show on Sirius XM everyday.  Paul looked at me and I said he likes to hear the stories that Paul and the others talk about on the show.  Paul said to Brandon well I have a lot of stories I am going to tell tonight.  Brandon smiled.  Brandon then told Paul that his music makes him happy.  Lots of hugs.  Brandon asked Paul why he added the horn section and Paul said - I thought it would be nice to have something different on this tour.  Brandon was excited.  Then we took pictures.  Lots of hugs.  Someone handed Brandon a set list and we headed to the balcony.  It took both Michele and me helping to get Brandon all the way up the stairs but Brandon wasn't going to let that make him miss the concert.  On the balcony there was one chair for Brandon so he could sit when he needed to.  He sat a few times that night but he tried to stand for almost the whole concert over 2 hours.  Brandon has been showing signs that his back is hurting and standing is one that we can tell put pressure on the back.  But he didn't want to miss a thing.

The show was fabulous with Paul singing a mix of Beatles, wings and his new album.  When the show ended Brandon almost fell over the side trying to waive at Paul as he walked under the balcony.  Then the band came out for an encore and as they were leaving for the final time Paul stopped and pointed at Brandon then blew him a big kiss.  Brandon was beside himself and almost frozen with excitement.

A rock star kind of night.  Paul's staff couldn't have been nicer. Paul McCartney talked directly to Brandon and interacted with him and Brandon was very engaged.  I think we will have some great pictures from Paul's photographer.  We should be receiving soon (if we ever get mail in he mailbox - been flooded out here so we haven't received mail for at least  week).

Brandon is still on the Paul high and walking around saying Paul McCartney about every 10 minutes.  What a great guy Sir Paul is.  Our night to live like a rock star is something Brandon and I will never forgot for many reasons.  I will leave you with one pretty magical moment of the night from our view.  Paul singing Hey Jude with all of Austin from Rush's video with over 95,000 views.  It is pretty awesome.  Thank you Paul McCartney for being so kind to my son and making his dream of meeting you come true.  

I am still trying to be on that Paul high, but I also have been working very hard to better understand the recommendations for Brandon as we receive more information concerning his treatment.  AND there is good news as this is only a good news post.
  • Brandon cleared all the heart tests.  His heart can take a very long surgery
  • We found out this week that he is not predisposed to blood clots via some genetic tests that we have received and that is important for this surgery.   
  • We also received good news on Brandon's foot.  The structure is still good, he does have arthritis and osteoporosis in the foot but that is to be expected given his history and the fact that the foot has been operated on.  We also found an Austin doctor to see in the event he has any other feet issues. 
  • In the last two weeks I have talked to the two scoliosis surgeons we are considering for Brandon's surgery.  I haven't made a final decision but I have ruled out the doctor in Austin solely because the other two surgeons have done many more surgeries on this complex population and seem to really understand what will be needed moving forward.  Based on my extensive conversations with the surgeons in Dallas and Houston I better understand why they both have different plans.  I am leaning towards one surgeon but am still reviewing their recommendations.  I am really relived that we are closer to making a decision for treatment.  
  • We have received a new set of genomic information (133 pages) that I am reviewing now that has a great deal of information both positive and some explain some of Brandon's bone and spine issues.  More on this in the next post.  Trying to keep it positive here.   
  • We are changing Brandon's medications slightly based on our visit with Dr. Sharen Hausman- Cohen (Brandon's integral medicine doctor) and his neurologist.  Brandon seems to be doing very well on the supplements and the CBD oil.  
That is enough for now.  I will go over the options for surgery and hoping that if any of you are trying to choose options for you or your loved one that it helps a little.  

Lastly - as Brandon says - All you Need is Love.  

Thanks to all of you that followed us as we were meeting Paul and the positive thoughts.  I read each message to Brandon and he loved them.  More later.  

Tuesday, September 11, 2018

Three opinions, Genetics and the Heart

For some reason I was hoping that when we went to three doctors to see what treatment is recommended for Brandon's spine, I would have three opinions all the same and it would just be a decision on whether to do surgery or where to do surgery.   But instead I now have 3 very different recommendations.  And if you saw my original blog I hate making these very important health decisions for Brandon.  Brandon has been actively involved in the discussions and I have been talking to him about options but he just doesn't understand truly what is involved. He does understand if the surgeon likes the Beatles and that is the most important thing to him.  I also thought this post would be about genetics.  But we have much more than genetics to report.  

Lets start with genetics
  • Our goal with genetics is to find out if there is an underlying genetic issue with Brandon contributing to his osteoporosis.  
  • I also want Baylor to update Brandon's complete genome profile which is over 5 years old.  Six years ago we didn't know as much as today about what genes are associated with diseases.  We have become much more informed and in 10 years this will be the new norm.  I have mixed opinions on doing genetic testing and resisted it for many years until Brandon started having uncontrolled seizures at 16.  While in the seizure monitoring unit at Dell we decided to run tests focused on seizures and Brandon's other conditions.  However, we didn't run the tests focused on bone health.  At some point we had the entire genome report run.  What did we find out?  Brandon has a ton of possible serious conditions.  Pages of them.  Most of them he is a carrier of the specific gene related to a condition.  We have confirmed that is the case for the three markers for osteogenesis imperfecta (brittle bone disease) and that Brandon is unlikely to have this condition.  
  • The last thing I would like if possible, is a diagnosis for Brandon.  We don't have a real diagnosis.  We have used cerebral palsy since he was 9 month's old.  Cerebral palsy is associated with damage to the brain.  All the tests run on Brandon (many) do not show damage.  But we needed a diagnosis and we are sticking with this one for now.   Very common for the time period of Brandon's birth. 
  • The genetic clinic decided we will run a series of genetic tests focused on bone health and they believe there is enough data in Brandon's complete genome file that they can run the tests from the data rather than drawing more blood. So we are waiting on the clinic for next steps.   

3rd Opinion - We met with Dr. Hanson at Baylor who was highly recommended by Dr. Peacock from the Baylor Transition Clinic where she treats hundreds of Brandon's.  I am always amazed at her work and her knowledge of the population.  I really liked Dr. Hanson.  His whole team met with us.  Reviewed the medical records, took x-rays (everyone wants their own), walked with Brandon and had him move in many positions.  Dr. Hanson said that Brandon does have severe scoliosis and that if I asked 100 orthopedic physicians if Brandon needs surgery, 98% of them would say yes.  I think the number 1 question - does he need surgery is Yes.  
The second question - what kind of surgery is different. Dr. Hanson said he has the most success with the least invasion on the body and with this specific curve doing surgery from the side and only repairing the curve (T9-L3 or 4).  He has operated of many patients with neurological conditions and this has been successful.  He said that in the operation part of it will be to build bone in the effected area (a little unclear on this).  Brandon would be in the hospital 5-7 days and would be able to go home afterword's with no rehab needed.  He strongly recommended we do not fuse the spine to the pelvis because Brandon would lose his ability to bend, he would be stiff, and he believes it is harder for these neurological patients to adapt.  I did ask about the fact that Brandon is not aligned in the spine and if Brandon was still out of alignment after the surgery or the alignment gets worse can we add that to the spine at a later date.  His answer was yes.  I know it sounds great.  BUT Dr. Viere was very concerned about the spine being out of alignment and the Austin surgeon said he may have to fuse the spine as well.  So I really need to review this carefully.  Dr Viere gave us a detailed plan of care with reasons why we need to do each step.  I need the same from the Austin surgeon and Dr Hanson if I am really going to compare.  Then I need some medical help reviewing the comparison.  
Dr Hanson also felt we could go a little longer on forteo.  Based on our visit it sounds like he is recommending doing surgery in 6 months to a year with the next check in 6 months.  I made one serious mistake while talking with Dr Hanson.  I forgot to ask if he loves the Beatles.  Brandon was not too keen on Dr Hanson as a result.  I am going to take the next step of asking all of the surgeons to put their plan for Brandon in writing.  Good news we have more time to make a very serious decision.  

Heart - The last blog told you that Brandon flunked the EKG and the hospitalist wanted Brandon to be seen by a cardiologist.  After I posted I received a great recommendation for a heart physician and we met with him last week.  As I suspected the EKG appears to be normal.  But Brandon does have PDA (Patent Ductus Arteriosis) which is an unclosed hole in the aorta. (Before a baby is born, the fetus's blood does not need to go to the lungs to get oxygenated. The ductus arteriosis is a hole that allows the blood to skip the circulation to the lungs. However, when the baby is born, the blood must receive oxygen in the lungs and this hole is supposed to close. If the ductus arteriosis is still open (or patent) the blood may skip this necessary step of circulation. The open hole is called the patent ductus arteriosis.)  This is very common.  Many of you had this and never knew it.  Usually the hole will close up after birth, but in some cases it does not.  If you read on this, it is normally not an issue until possibly later in life when there is a slight risk of stroke if the hole does not close.  It also can be a risk in a very long surgery such as Brandon's especially if the hole has grown.  We are testing to see if the hole has grown so we have a better idea on possible heart risks for Brandon during this long surgery.  Brandon has no signs of any heart issues at this point and Brandon had previous testing on this 8 years ago.  At that time there was still a hole in the heart and it had not grown.  So I am expecting the same with the next test.    

A little surprise, the foot -  The reason we know Brandon has severe osteoporosis is because Brandon's foot swelled last year and he was limping on it.  We had an x-ray at the PCP's office and he said that Brandon's bone health did not look good.  Two weeks ago Brandon's foot swelled again.  This time the radiologist also said Brandon's bone health did not look good.  Brandon has problems with his feet for 20 years.  Breaking them, spasticity, one foot curving in like a C.  Not unlike this decision we had many recommendations on how to repair the feet ranging from do nothing to do surgery on both feet and hamstrings.  But the feet have always been the worst and the scoliosis was secondary.  I have been ignoring the feet.  After this last swollen foot, I consulted with Scottish Rite on what they recommend we do (they operated on one of he feet).  They would like the foot checked as well, but with a physician who specializes in feet and has done surgery on many complex patients.  We haven't checked the foot since we left Scottish Rite 7 years ago.  We are seeing another doctor on the feet in Dallas.  

One last point.  How does Brandon get to see all these doctors all over Texas?  He has private insurance, regular Medicare and regular Medicaid.  But Brandon is about to turn 25.  There is a federal law that allows parents to continue coverage for their adult children on their insurance beyond 25, but they must be totally disabled.  Brandon turns 26 in December.  I will be applying for Brandon to continue with my commercial insurance policy to allow coverage beyond 25.  I will also blog about the process in the future for any family wondering how to do this process.  

It has been an exhausting couple of weeks but glad we are getting some great expert advise.  Only a few other appointments and then some down time while we look at all options and go to see Paul McCartney.  Did I mention he released his new CD and we listened to it on our drive to Houston.  Preparing for the big gig.  Also Brandon will be doing another radio show this Monday Sept 17th on KOOP radio with Rush Evans.  He will be interviewing Van Wilks (Texas guitar god) and Stephen Doster and Andy Salmon (before their upcoming John Lennon Tribute).  More later.      

Sunday, August 26, 2018

The plan for the Spine and Paul McCartney

I wanted to start off the blog today by letting you know why I do this blog.  The blog is both therapeutic for me and helps me keep up with Brandon's progress.  But I started the blog with the intention that when someone asks me how is Brandon doing, I can say fine and then refer back to the blog for how he is really doing.  Repeating the same story over and over sometimes is difficult for me and it is just easier to say he is fine.  And let me say - Brandon is really doing great.  He has a great life despite the challenges he must deal with everyday.  He doesn't even think about those challenges and instead focuses on those things he loves.  It is about music and in particular the Beatles.  He is very happy.  I want him to always be happy. 

I also do the blog because I hope it helps at least 1 more family searching for that little bit of information they have been seeking.  Maybe it is the name of a doctor, a drug Brandon is using, diet, supplements, employment, housing and accessing services.  I feel lucky that we have been able to find great resources for Brandon because of my past work, work on advisory committees or where I currently work.  But not all parents have the same access and don't really know where to start to find resources.  I hope some of this is for parents searching for answers.  

Probably more importantly I do the blog for all of you who have looked at a child with special needs and don't know what to say or don't understand what it is like to be a parent of a child with special needs.  Many of my friends are lobbyists/lawmakers/policy developers making policy for people like Brandon.  But they don't know any Brandon's.  This is my attempt at helping educate, even if only one person takes an interest and then uses the examples for change.  I have found over the years Brandon has been referenced in many conversations and I am fine with that when the end result helps many Brandon's in the future.  I will continue to be the voice for change for Brandon and so will Brandon.  

Many of you have commented that you are so glad Brandon is doing better since my last post on facebook giving an update.  I have mixed response to this.  Brandon still has a serious medical condition he is facing.  He still must have surgery to correct this condition and it will be major surgery to rebuild his spine.  But what we have learned for the last few months is there is a treatment that can slowly improve Brandon's osteoporosis.  Brandon's bones are too fragile to handle a major surgery today.  Continuing with treatment of the osteoporosis must be a priority for Brandon currently.  Monitoring Brandon's spine will be a priority.  At some point probably within the next 3 years Brandon will have the surgery.  Because Brandon's surgery is so complicated we really need to have a surgeon who has done other Brandon's.  And it is a plus if the surgeon loves the Beatles.  Our goal is that after a successful surgery Brandon does not have any more pain than he currently has today.  So here is the latest.

Update on Brandon's Osteoporosis - Before we went to meet with surgeons this week Brandon had a repeat bone density performed.  We received the results right before our meetings with the surgeons.  The results are similar to what we were told to expect by the MD Anderson doctor.  Brandon's overall improvement after 1 year on forteo, changes in supplements, exercises and vibration improved by 5.4%.  However the hip and femoral neck declined. Brandon is at risk of a major osteoporotic fracture at 13%.  Brandon's scores in the spine which is the area we need more increase of bone, range from -2.5 (T and Z scores) to -3.4.  Normal is 1 and low bone mass (osteopenia is between -1 and -2.5).  Our goal is to get to these scores to the osteopenia range.  This was important news before our visits.  

The Austin surgeon's surgery plan - Brandon met with the Austin surgeon who was scheduled to perform surgery on him in October.  This surgeon reviewed all the records and is suggesting that Brandon have a rod inserted from T9 through L4 (see below).  The surgeon was ready to do surgery in Oct but agreed that more bone improvement is always ideal.  He said that Brandon could wait while completing the osteoporosis treatment.  He said he wasn't sure, but he may need to insert the rod from T9 all the way to the pelvis because Brandon's spine is not aligned.  He would open Brandon up in the OR and decide if he would need to go all the way to the spine based on the alignment.  He said that balance is key to the success of the surgery.  He also said that Brandon's curve has changed from 52% to 56%. He went on talking about how bad Brandon's curve is which is in between T 9 and L4 and specifically serious decline between L1 and L4.  He really didn't have much more detail on what he is recommending for surgery.  We actually left this appointment feeling fairly good with the recommendation except the part where he would not decide whether to go to the pelvis until in the OR.  

Image result for picture of the spine with numbers

The second opinion - We left Austin and headed to Dallas for our first second opinion.  It was actually nice that they were close to each other in time.  Dr Robert Viere in Dallas was our second opinion physician.  We have another one scheduled in Houston with Dr Hanson in a few weeks.  When everyone said Brandon needed surgery on the spine I outreached to Brandon's foot surgeon at Scottish Rite.  He followed Brandon's scoliosis for several years while treating the foot.  I sent over a few reports to him and he looked up Brandon's history from Scottish Rite.  He was concerned and strongly insisted that we see Dr Viere.  He said they refer their patients who age out of Scottish Rite to Dr Viere and all the physicians practicing at Scottish Rite use him personally.  I brought all of Brandon's records and films with me.  Dr Viere reviewed all the records and reports.  When he came in he immediately started checking all of Brandon's limbs, moving them around and pressing on them.  That actually felt great because no one seems to be interested in Brandon's overall musculoskeletal health and I have always thought that is important even in regards to his spine.  We did not move forward with his foot surgery until we were able to determine the impact on Brandon's body from the deformity of the foot and the impact surgery will have to improve his gait.  I thought this was lacking for prep of this spine surgery.  The first thing he said was you cant do surgery on a person with cerebral palsy or a neurological condition the same as other adults.  Spasticity plays a large role.  A person with tight spasticity can pull bones through the implants.  Based on his exam Brandon could have the surgery as his muscle tone in the truck is low and therefore is a candidate for surgery.  Brandon's x-ray in his office is showing the curve is around 52% overall in the spine.  He is also concerned with the curve between L1 and L4 and the severity of the decline.  

But his plan is different.  He says that Brandon's spine is leaning to the left and this is why Brandon walks leaning to one side.  The only way to repair this is to align Brandon's spine to the pelvis.  Brandon's spine will need supports when doing the repair.  They will insert these supports through the front.  This will cushion the spine as they align.  Besides tilting to one side, Brandon is tilting forward which will also need to be corrected.  Once the rod is inserted all the way to the pelvis Brandon will not be able to bend over or move from side to side.  Bending over is really not an issue because Brandon rarely does this now.  But he does not want Brandon to have this surgery until Brandon is in the osteopenia range as I described above.  He is recommending that we continue on forteo and then the new drug until we have better bone health.  He wants to check Brandon next year to see progress and anticipates in a couple of years he will be ready.  He also believes Brandon must be checked for any genetic condition because this will factor into the surgery.  He definitely wants more information on osteogenesis imperfecta or any other genetic information.  We will come back to his office for more tests and review in 1 year.  As we were walking out we met Dr Viere heading to the hospital.  We commented about Brandon's love of the Beatles and Dr Viere said he has every original Beatles album released and that he is a major Beatles fan.  That really made Brandon's day.  

Recovery - For both surgeons Brandon will be in the hospital for 5-7 days.  Dr Viere's plan is for Brandon to go to inpatient rehab to learn to walk again without leaning and improving his balance.  He will need to be in a hard plastic brace after surgery and will be in significant pain for around 2 months which they will manage. At 3 months he will begin to feel better.  When recovery is complete Brandon will be feeling better than he is currently.  

The heart - The Austin surgeon wanted Brandon to go through a complete adult assessment to make sure he is healthy enough to go through surgery.  At our hospital visit to check his ability to undergo surgery Brandon flunked the EKG.  This is not unusual and normally associated with a lead from the test not correctly placed, but he looked through previous EKGs ( he has had one every quarter for the last 15 years) and requested Brandon go to an adult cardiologist to get checked and to review his genome results.  We did have a check previously with the pediatric cardiologists but it has been at least 8 years.  We will go meet with the adult cardiologist to make sure we have checked all the possible conditions.  As Brandon said - mom we will check this one off the list.  Hmm wonder where he heard that.  

Relief - I can only tell you that I have a major sigh of relief, even if for a short time.  Relief is that we are not doing surgery in 6 weeks.  Relief is that we have a good plan and a good team (with the exception of genetics -still working on this with Baylor).  Relief that Brandon is not in significant pain and will not be in significant pain for a few more years unless his condition worsens. Relief is that we have time to work through any other major issues that may come as a result of the genetic workup or the cardio visit.  AND relief is that Brandon gets to see Paul McCartney in October without worrying about a surgery around the corner.  

And to put it in perspective as Brandon says - All you need is love (and Paul McCartney).  

More on the genetics as we move through this process and the heart with includes some genetics.  But all is good for now.