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.  

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.  

Leah

It's all about the Bones

If you read my last blog I explained our current dilemma on whether Brandon should have scoliosis surgery or not. I feel like the options are not good and have decided to go to meet with other physicians around the state to get more information. This blog is all about the bone research.  

Since I last updated you, Brandon visited with Sharon Hausman- Cohen MD our integrative medicine physician.  I am telling you her name now because she has just opened her practice to help other children and adults with intellectual disabilities among others with disabilities such as Alzheimer's.  I will say that Brandon has had significant cognitive improvement since we have started seeing her.   For Brandon she has guided us on building bone and is looking at some genetic causes of Brandon's disabilities.  She previously had a great deal of experience with patients with osteoporosis.  So she was a perfect choice for Brandon.  She also is a strong supporter of using supplements, diet and some drugs on the market to help with osteoporosis. At our last visit she rechecked some additional blood tests which were normal and we discussed the current plan to have surgery in October.  I explained to her my concern that it appears Brandon is at high risk of a break during surgery or after surgery on his spine and hips if we have surgery in October.  I asked her if she recommends moving forward with the surgery knowing Brandon's risk.  I also told her our two options presented - surgery with risks or no surgery with future deterioration of the spine.   She suggested there is a third option to continue to build bone, follow the scoliosis and hope our treatment will allow Brandon to maintain his current curve and no further decline of the spine.  We went over everything we have done to rebuild bone.  The supplements (see earlier blog).  She added vitamin K.  The diet.  We will continue.  The exercise - Brandon goes to the Y 3 times a week and does weight bearing exercises and his jobs require a lot of walking.  She suggested we buy a whole body vibration machine.  Brandon now had a vibration chair (thanks to my friend Steve and Cathy Beard) and we have ordered a standing vibration machine.  Our goal is to obtain up to 40 Hz for 30 minutes a day.   Whole Body Vibration

Lastly, we discussed continuing for another year with approval from the manufacturer on forteo.  She recommended to wait on having the surgery.  She ran a series of genetic tests focused on Mitochondrial conditions.  I am going to list the services she is currently offering in her practice below.  We are scheduled to update the bone density tests in Sept. to see what progress has been made.

I still felt like I needed more information on the causes of Brandon's osteoporosis.  To move forward with surgery I would want to know if there are any underlying causes besides the seizure medications. Any underlying conditions may change whether to proceed with surgery or better inform us of anticipated outcomes.  Thanks to Dr Peacock at the Baylor Transition Clinic asking me the question of why and finding a great resource to discuss Brandon's "bones".  We were able to secure a visit with Dr Gagel at MD Anderson.  Thanks to Marian Cabanillas for helping us get an appointment.  This week we visited with Dr Gagel in Houston.

My question to Dr Gagel is why does Brandon have osteoporosis at age 25.  I also wanted to confirm the severity and the need for surgery.  I spent a few weeks getting all of Brandon's medical records together.  A little frustration - we have been scanning the records and putting them on an app called CareSync.  Really great concept and it saved me from having to drag around 10 pounds of medical records.  But a few weeks ago they went out of business.  We stopped scanning the records and I brought all hard copies and the actual scans, xrays, etc on disc.

Dr Gagel reviewed the relevant records, we discussed Brandon's foot and information from Scottish Rite (we had all the records and scans) and he reviewed the actual bone density scan.   Dr Gagel confirmed that Brandon has severe osteoporosis and at this point is at high risk of a fracture during surgery.  He reviewed the scan and the crucial areas where Brandon has severe bone loss.  He does not recommend we move forward with surgery at this time.  Instead, his recommendation is that we continue with forteo for another year.  Then we move to another drug Romosozumab.  This is a new drug just approved by the FDA to increase bone loss and has double the results of the other drugs.  All of these drugs come with potential major side effects but the likelihood of getting the side effect is very small. Here is some information on the drug  Romosozumab.  Dr Gagel wanted us to know that all of these drugs will improve bone health but once Brandon has achieved the maximum bone improvement he is able to achieve, he will then need to start taking a drug to hold the improvement level.  All of these drugs do not maintain the level of bone health once the drug is stopped.  New news to us.  I also asked Dr Gagel what level of improvement we can expect.  He said with one year of forteo we can expect a 5% overall improvement and with 2 years a 10%.  We will expect more from romosozumab.  Even with these drugs Brandon's bone health will unlikely recover from the diagnosis of osteoporosis.  What it will do is improve the risk of fracture by over 50%.  All much better news.  I also asked Dr Gagel if he believed Brandon needs surgery for his scoliosis. He reviewed the actual x-rays and scans, pointed out where Brandon's back has severe scoliosis and bone structure issues and he said that yes, Brandon has severe scoliosis and will need surgery to repair.  However, we will have the weigh the risks of his bone health and any underlying conditions when making these decisions.  Can Brandon wait 3 years.  He didn't know but said we will just have to continue to monitor.  All the above was exactly what I wanted to know.

Next he said most patients he sees similar to Brandon have some underlying genetic condition contributing to bone health.  I showed him Brandon's genetic tests.  Brandon has several genetic markers for osteogenesis imperfecta (the brittle bone disease Osteogenesis Imperfecta) and other related conditions. Not one marker but several.  We have never claimed this diagnosis because we have had a few physicians review the reports and indicated it is unlikely Brandon has the condition. But he was not convinced and immediately called the Baylor Genetic Clinic and asked that Brandon be seen and hopefully during our next visit to Houston in Sept.  They agreed.  He called them while we were in the room and he sent over Brandon's records.  He is concerned and wants Brandon seen because Forteo is ineffective in treating osteoporosis if Brandon has OI.  There is a different treatment option.  He would like Brandon to be followed by Baylor's clinic to rule out any of the conditions and if found he does have this or other conditions, they would do the recommended treatment.  I know kind of a shock, but I have know for many years Brandon could potentially have this condition.  I have sent his information to experts in this diagnosis and they have said it is unlikely.  But, these reports are old and I think there is newer testing.  So I don't want to blow this diagnosis off for Brandon.  It can be significant if he was diagnosed with this condition and we would need to completely change his treatment.  So once again another curve.

Why haven't I cancelled the surgery?  This surgeon recommended Brandon have surgery in 2012.  I declined to move forward.  But I didn't see the whole process through where they do the complete testing, determine exactly the surgery they recommend and then take those recommendations for second opinions.  We have very little baseline information. I need to continue to move forward to find out what Brandon will be dealing with whether it is today, next year or in a few years.  I also need for them to finalize all the tests so we can continue to monitor and see if Brandon may have a decline in the future.  I also want to get the second opinions to get their opinions on treatment and determine the best surgeon who has treated a lot of "Brandons" with great outcomes.

Thank you to all of you who have helped finding physicians for Brandon, alternative treatment suggestions, connections to other families, helping with my insurance questions and coverage, all my nurse friends for your dumbing down the medical information for me, helping us obtain the appointments for physicians that are very hard to access and all of our friends for your support.  It takes a village.  You know who you are.  Although the news of Brandon's severity of his scoliosis and osteoporosis has not changed, I feeling much better about having a plan for treatment with the best possible outcome.

More to come.  Next appointments are the surgery recommendations for scoliosis and second opinions then the genetic appointment.  AND most importantly Brandon will definitely be able to see Paul McCartney at ACL.  That is his number 1 priority.

More later
Leah

Information from Resilient Health 

(Dr Sharon Hausman-Cohen)

Genomics Consults Available to Friends & Family!

Want to share our unique genomics reports and consults to help your loved ones? Resilient Health is excited to now offer Genomics Consults for the family and friends of its members. Have them contact our office to schedule an appointment.

Many of our Resilient Health members have had their genomics reports interpreted in our office where they have been used to customize treatment and personalized prevention plans. Several of you have been asking if we can provide genomics consults for your relatives and friends. And we are happy to finally say, Yes, we can! We are now offering full genomics consults to your family and friends. They need not be a member of Resilient Health to enjoy the results of our genomics consults.

For those of you who have already "given us your spit" - results are continuing to come in, and we will review them with you at your regular Resilient Health appointments. 

What is Genomics?

For the past two years, Dr. Sharon Hausman-Cohen has been utilizing genomics research for her patients as part of their care at Resilient Health. Due to popular demand, and with the use of our own IntellxxDNA reports, Dr. Anne Stefani is now offering genomics consults to the family and friends of Resilient Health members and to our extended community.

For those of you who are not familiar with genomics, it is the field of understanding how subtle changes in your DNA help make you unique. These small changes in your DNA, also known as Single Nucleotide Polymorphisms (SNPs), can then often be addressed

with the proper use of diet and lifestyle recommendations, supplements, and sometimes medications. These SNPs provide information that contribute to mood, brain health/Alzheimer's risk and cognition, risk of macular degeneration, cardiac risk factors,

diabetes related issues, hormone related issues and much more. All of them are highly modifiable. For example, some people benefit from aspirin for reducing cardiac risk with a 54% reduction in risk, while others would be anticipated to get only a 7% reduction in risk. The knowledge that can be obtained from our genomics consults is extensive and can often be helpful in solving medical mysteries.

For individuals interested in consults who don't live in Austin or surrounding areas, we will have telemedicine genomics consults available soon!

You can read more about the use of our genomics tool at www.IntellxxDNA.com. It is getting rave reviews! One doctor who has been featured on Dr. Oz referred to the IntellxxDNA report as "game changing and mind blowing."

Read about our package options below!

Genomics Reports & Packages

We have 2 genomics packages available:

Package #1

1 Report + Review $1500 

(Either Alzheimer's/Cognition OR Medical Overview Report) 

• One 90-minute initial consult
• One 30-minute subsequent follow-up appointment
• DNA test kit and processing
• 1 report 

Package #2

2 Reports + Review $2000

(Both Alzheimer's/Cognition AND Medical Overview Reports)

• One 90-minute initial consult
• One 60-minute subsequent follow-up appointment
• DNA test kit and processing
• 2 reports

We know that you will have questions! 
To schedule your saliva collection and/or to have your questions answered, please call us at 512.717.9775 and ask to speak with our Genomics Coordinators. They will walk you through the steps and answer any questions you may have. 

Resilient Health

3410 Far West Blvd, Suite 100

Austin, TX 78731

512.717.9775 phone

512.599.5034 fax

ResilientHealthAustin.com