631 days

Braeden and I were at Duke today for what was to be his 6th & final IVIG infusion.   Braeden and I had "placed our bets" that we would be there till at least 5pm making it an 7 hour day.  We were wrong. We also wouldn't have guess that the Neuro-oncology team would end up singing Happy Birthday to Braeden a few days early.  They definitely don't need to quit their day jobs, LOL.

After speaking with the team it was decided that Braeden would skip the last infusion.  We all had hoped that the IVIG would have had a positive impact on his hand strength.  Typically, a patient will see results within weeks of the first treatment.  We definitely gave it the old college try and it proved not to be our "miracle".  

So what's next?   

• Appointment with Nephrology in January to try wean and discontinue blood pressure meds.
• Repeat MRI in 3 months. Yesterday's, MRI showed no new changes.
• Because of Braeden's inability to write legibly, he is going to have a  consult with Neuro-Psych. They will do an in depth evaluation of his capabilities and will be able to make the school aware of any new and updated accommodations that he will need. 
• Braeden will learn to navigate his new normal. The child has lost a lot because of this damn spinal cord tumor. (Lord knows, he has already lost a lot from his brain tumor.  He has managed those loses more gracefully than most.)  
• BUT, he is going to be ok! He will NOT lose his smile. He will NOT lose his sense of humor that never fails to make me laugh.
• It's also my hope that this will be the last post of this blog.  I wrote this blog to keep family and friends updated on "everything medical"  Braeden.  (No news is good news or in our case, no new blog is good news.)     
  
• Most importantly, he will ALWAYS have his family and friends that will there for him no matter what.

I use an app to keep up with important dates.  Hard to believe that Braeden has only had 631 short days of his life that have been "Duke Free".

Prayers that the days, months and years going forward will be kind to my sweet HERO.   

#BraedenBrave

#MrUnlucky

Just what the doctor ordered

I  have post it notes everywhere to remind me of things that I need to get done. 

For weeks, I have had a post it note with the word "Blog" on it.   I am finally getting around to blogging tonight.   I will blame our new addition, time flies when you have a new puppy!

I can only describe Braeden's health and symptoms as plateauing. They have not gotten exponentially better, but thankfully they haven't gotten worse.  

• tremors and twitches are the same
• hand weakness on both sides are the same. We had a meeting last week with the school to discuss accommodations he may need next semester.  He is unable to write legibly because of his hand weakness.  This is definitely going to present a problem in some of his upcoming classes.  Currently, he is "pecking" by with his laptop.

On November 16th, I heard from Mayo Clinic, and they decided against taking on his case.  They felt that Duke and CHOP had done  a complete work up and they wouldn't have anything to add to his clinical picture. 

On November 18th, we were at Duke and Braeden had IVIG #5.  One of our doctors at Duke suggested that we "give" Braeden a break before pursuing a 3rd opinion, considering that Mayo declined his case and they are no new symptoms.  

Braeden has had quite the two years of surgeries, appointments, radiation treatments, OT, PT, lab work, MRIs, infusions, steroids for MONTHS and feeling just plain crappy.  

Taking a break may be just what he needs, until we figure out our next steps.

Thank you all for the continued love and prayers. Merry December to you all!

#BraedenBrave

#MrUnlucky

Next up:

Dec 15th-MRI
Dec 16th-IVIG#6

Jan 6th-Appt with nephrology, to hopefully start weaning his BP meds since he is off of the Avastin.

Enigma

Enigma - this is the word Dr. Smith used to describe Braeden's case today.   I don't have a lot of new news or any real new answers.  

• Biopsy of his bruising was done on 9/24.  Results came back negative.  This rules out the vasculitis. This is a definite relief.
• On 9/28 he began having leg tremors. Anytime he lifts his heel off the floor, his legs shake uncontrollably.  They are saying that this is because of the myoclonus.  It's hard to see him have this new symptom, and in the future, if it gets worse we can do medications to ease the symptoms.
• We saw Rheumatology today and she thinks that we need to revisit the Asialo-GM1 results. (If you remember, this was the result that came back "equivocal".  His immunoglobin G levels were low and it doesn't make since that his GM1 results were 49.  She thinks it is likely that his GM1 value is actually higher.  This means we are still on the path trying to figure out which autoimmune neuropathy disease he might have.  Both neurology and rheumatology saw him together today and they just don't know.   None of the "puzzle pieces" are fitting together and they don't give us a clear picture of his diagnosis.   I am sure is just as confusing for you as it for me to read all of this medical jargon. They suggested that once Mayo Clinic reaches out to me, that I let them know that his IgG-IgM was 49 and that his total IgG was in the 400s.  And, maybe Mayo can shed some light.  I plan on reaching out to Mayo this week to follow up.
• IVIG should have worked by now.  No improvement is noted yet.  We will continue the IVIG for 6 months, as CHOP recommended, and hope that it starts working.
• We saw nephrology today, and they were pleased with his blood pressure and his weight loss. Another positive for the day.
• He had an echo today to ensure things are ok with his heart.   His two blood pressure meds are controlling his blood pressure, but his heart rate is still high.

Braeden has felt crappy for the last few weeks. They blame the IVIG and being off steroids.

In other news, most will agree that puppies are the best medicine.    Especially cute ones like Ms. Daisy Penelope who will be joining our family around the 20th of this month.  (Finally another girl in the house!!)

Thanks for all the prayers and love as we continue to chug along.

#BraedenBrave

#MrUnlucky

How many doctors does it take?

It's been 13 days since my last post, and things as always are ever changing.  I mostly write this blog to keep up with the enormous amount of information and appointments and everything else that is involved with his case.  Back in May, when Duke said they had exhausted all testing and didn't know what else they could do for Braeden, I began a paper journal because I knew that it would be redundant to "blog" every new thing.  

Yesterday was a long day at Duke.  We were there for almost 12 hours, and we had a lot of new things thrown at us.  I am going to refer to my journal and give you a timeline of things that have happened the past two weeks.

09/14/2020- The results of Braeden's 24 hour urine reflected that his kidneys were potentially being damaged by the Avastin, and we needed to make a decision whether to proceed or stop the Avastin. It's been decided to stop the Avastin.  

On this day, I noticed his hand tremors were worse. He had a lot of new bruising that he had no idea how he got them.   I also noticed for the first time that his left arm was twitching.

09/15/2020- I texted Dr. Landi a video of the twitching.  Bridget his NP emailed back and asks that we keep an eye out for any bleeding, because his platelets were low last week on his labs.   We also need to continue to update them with any new symptoms.  

09/16/2020- I contacted Mayo Clinic and requested an appointment for Braeden.  For the past five years Mayo Clinic has been named #1 hospital in the nation.  Maybe they can solve the mystery of Braeden.

09/17/2020-Braeden officially dropped band.  The hope is that he will be able to rejoin the band when he regains hand strength.  The word hate is not strong enough to describe my feelings about this DAMN thing taking away another thing he loves.

09/19/2020-15th year anniversary of Craniopharyngioma diagnosis, 22 months since Astrocytoma Grade II diagnosis.  My hero.

09/20/2020- Braeden felt his first jolts on this day. He described them as a "violent" shooting pain.  This is also the first time I noticed his right arm twitching.

He looked exhausted and slept a lot over the weekend.  

09/21/2020- Mayo called and got basic information insurance etc

09/22/2020-Braeden had a routine appointment with his endocrinologist.  His blood pressure has been better since we started the 2nd blood pressure medicine, but his heart rate is averaging between 120 and 150.  His usual heartrate is around 60.  She was concerned and ordered a 24 hour holter, She has referred us to a Cardiologist. 

I contacted our navigator  Duke and made them aware that I had reached out to Mayo and that they would need his medical records.

09/23/2020- The team spent a lot of time with us yesterday. Below are the highlights.

• C-Reactive Protein was high. This indicates he  has some sort of inflammatory disorder,  or autoimmune disorder.  Lupus was mentioned. We are being referred to Rheumatology to try to figure out this bit of news.  
• His platelet labs are all low and it's possible he has something called Von Willebrand disease.  Simple definition is there is not enough of a certain protein that is needed for platelet adhesion.  We are being referred to Hematology to figure this other bit of news.
• The twitching and his tremors have gotten much worse over the past week.  The team diagnosed it as Spinal Myoclonus.  And guess what????  It's RARE and can't be cured.  The treatment for it is focused on easing the symptoms. We are also doing a MRI to ensure that his tumor is stable. We are being referred back to our Neurologist to manage this part of his treatment.
• We should have seen improvement with his hand strength from the IVIG infusions.  Unfortunately, that has not improved.
• The bruising that keeps "appearing" from no apparent cause is something they are calling purpura.  This happens when there is bleeding under the skin and there hasn't been a trauma.  Braeden has several that fit this criteria.  He has one in particular on his face that could be in the textbook of Purpura.  They feel pretty sure he has also has something called vasculitis.  Another RARE condition that can't be cured.  This is an inflammation of the blood vessels. It can cause them to become scarred, thickened, weakened, or become narrow.   The plan is for him to be referred to Dermatology. Dermatology will need to get biopsies of his purpuras as soon as possible.  The vasculitis can range from mild to life threatening.  Getting this done ASAP is our first priority.  (They called me today, appointment is tomorrow morning.)
• IVIG #3 infusion done.  The infusion takes about 3 hours and with the help of IV Benadryl he slept the whole time. IVIG can also be used in treating vasculitis.  
• 127 pages of medical records were sent to Mayo clinic.  They will review his case, and if they accept our case, we should be able to be seen sometime in November or December.
• MRI done last night, and today they called to tell me that the "the MRI shows no radiologic evidence that the tumor is causing the progression of his symptoms.

His diet is going well. (We are working on the steroid weight gain, and happy to say he has lost 9 pounds over the past two weeks.)  Calorie counting, and no processed foods, and lower carbs, and lots and lots of vegetables.   Side note: cauliflower mashed potatoes are YUM!

Braeden-ism of the day yesterday.  Braeden and I listen to podcasts together.  Last night, he chose the episode about Woodrow Wilson from the show Very Presidential.  With in the first moments of the show, they talked about President Wilson's failing health.  One of the first things mentioned was his right hand weakness.  (Umm, sound familiar?)   They went on to say that he ended up being blind in his left eye. (Seriously? Braeden is blind in his left eye.)

Braeden looks at me and deadpans, "I am so Presidential and I didn't even know it."

My head and emotions are in a tailspin.  The year  2020 has not been kind to him.

Much love to all.

#BraedenBrave

#MrUnlucky

1 out of 7.8 billion people

WARNING:  Skip to the end if you only want the CHOP  update from today.   It's been a busy few days since last Monday.

Braeden's blood pressure kept acting up and it was decided he needed to see a Nephrologist to help manage his BP.   The Nephrology team will see kids like Braeden because they are confident that the increased pressures are from the steroids and the Avastin; both of which have a negative effect on the kidneys.   His pressures kept rising and even reaching the scary numbers of over 220 etc.   

We saw the new doctor this past Wednesday and he was truly one of the nicest doctors I have ever met.  Dr. Delbert Wigfall - if your child needs a kidney specialist, my vote is for him.  He took all the time in the world and was awesome with Braeden.

He started Braeden on an additional BP medicine, and, after just 2 days his BP is back to the 130s/80s.  He also ordered a kidney ultrasound to ensure that his kidneys were not damaged from the aforementioned medicines.  I got an email yesterday that those results were normal.  Definitely a positive for the week.  We get an echo next month to make sure everything is okay with his heart.

Braeden was unable to get Avastin # 12 on Wednesday.  His protein/creatine ratio was 497.  It needed to be below 300 for them to do the infusion. (This is usually a sign of kidney damage.) Plans were made to do a 24 hour urine sample. Those results will determine our Avastin path.  

Dr. Landi also made me aware that some of the Mayo testing had come back.  Braeden ruled out for a lot of the immune testing. Another positive...

But, there is always a but.... Dr. Landi, asked me to reach out to CHOP and get them to go over the abnormal results that Mayo had sent.  He wasn't able to tell me much about the abnormal results. 

I got a copy of the results and knew I would be busy googling until I heard from one of the other doctors who would be able to tell me something definitive.  I emailed both CHOP and our Neurologist at Duke on Wednesday once I got home to call me.  

After those emails, the googling and searching began. 

His testing for the Asialo GM1 antibodies, IgG-IgM was 49.   What does this mean?  According to the reference ranges as seen below this result was EQUIVOCAL.   I can promise you after all this kid has been through, the last thing I wanted to read was the word Equivocal.  This word basically means it the result is  "open for interpretation" and could be either positive or negative.  Great, even more of the no answer thing.   But you are "almost positive".   I don't want him to have any of this,  but something is wrong and it would be nice to know how to FIGHT this thing from getting worse.

29 or less      NEGATIVE

30-50            EQUIVOCAL   

51-100          Positive

101 and Up    Strong Positive

I won't bore you with all the information I found, along with my sister-in-law Sara, and Teresa, my BFF were able to find about the GM1 antibody and what it causes.    Multifocal Motor Neuropathy (MMN) and Chronic Inflammatory Dymyelinating Polyneuropathy (CIDP) continue to come up in our searches.   

If you read about the MMN, Braeden could seriously be the poster child for MMN.   MMN  presents clinically like ALS.  The difference is that MMN is a chronic condition and some patients improve with the IVIG path.  At least, we have already started on that path. 

Sara, Teresa, and I figured that MMN was our best guess.  I am just an internet Doctor; those two play two in real life.  LOL 

Below in Red is part of an article describing MMN.  He literally could be the poster child for MMN. His physical symptoms are spot on.  

But,  that is always a but....The week is not over.  After the article, I will write about my call today with CHOP and their interpretation of the results from Mayo.

Multifocal motor neuropathy is a rare disorder characterized by slowly progressive muscle weakness, primarily of the arms and legs. The disorder is considered to be immune-mediated, which means there is inflammation resulting from abnormal functioning of the immune system and the presence of specific autoantibodies that target a specific protein in the body. Other symptoms including muscles cramps and wasting (atrophy) of muscles can also occur.  Multifocal motor neuropathy usually responds to treatment with intravenous immunoglobulin.

Multifocal motor neuropathy is a progressive disorder, this means that the signs and symptoms tend to worsen slowly over time. The main symptom is progressive muscle weakness of the arms and legs. Unlike other neurological disorders affecting the arms and legs, there usually is not any sensory deficits. This means that feelings of tingling or numbness or pain are not associated with the disorder. Muscle weakness often begins in the hands, causing hand weakness and affected individuals may drop objects frequently or have difficulty performing tasks that require fine motor skills such as turning a key in a lock. Fine motor skills are those that require small movements involving the hands and wrists. Some individuals have trouble extending or bending their wrist upward (wrist drop). Reduced grip strength of the hands and reduced dexterity of the hands is seen. Sometimes, muscle weakness starts in the legs. 

As muscle weakness progresses, severe fatigue in the affected muscles may be seen and the disorder will cause functional disability. Affected individuals may have limited endurance. The muscles on one side of the body that are affected may be different from the muscle affected on the other side of the body (asymmetrical muscle weakness).

Additional symptoms associated with multifocal motor neuropathy include cramping, involuntary muscle contractions or “twitches” (fasciculations), decreased muscle tone, and, sometimes absent deep tendon reflexes. Later in the progression of the disease, wasting (atrophy) of the affected muscles may occur.

Other muscles such as those affecting breathing are unaffected and the disorder usually does not affect life expectancy.

Affected individuals have autoantibodies. Antibodies are part of the immune system; they are specialized proteins that target foreign or invading organisms. Autoantibodies are ones that mistakenly attack healthy tissue. Many individuals (30-60%) with multifocal motor neuropathy have autoantibodies that target GM 1 ganglioside, 

Dr. Brandsema from CHOP called me today and we had a long conversation about the results.

The two things that Braeden's symptoms symbolize the most are the CIDP and MMN.

Dr. B says that he doesn't have the CIDP. There was no protein pattern in his spinal fluid and  his EMG does not reflect CIDP.

It's not MMN; his EMG doesn't reflect MMN and also MMN isn't a pediatric disease.

What now????? (I mean seriously, I would have bet money on the MMN.) 

Wait for it....

He is PERPLEXED and Braeden's case is very UNIQUE. And, he doesn't have an answer for us.  

He suggested to wait and see if the IVIG improves anything over the next 6 months and plan a treatment regimen at that time.  (Steroids, etc)

If there isn't any improvement with the IVIG, his thoughts were that Braeden has a very unique disorder and one not previously described.  

(7.8 billion people in the world, and this kid keeps showing all these doctors how UNIQUE he is?)  You are telling me that my child is only person in the world that has this issue. I can't wrap my head around that.  He can't be the only one.

His other thought was that Braeden has a known disorder, but it is presenting itself in an unusual way and with any new symptoms make them aware.  Sometimes, when someone has neurology issues, new symptoms happen and can lead the doctors down another path that may include a diagnosis.  

Six months is way too long for me to wait and see, considering everything.  I appreciate what Duke and CHOP have done, but there has to be another hospital that can help us.   He suggested that we go to Mayo Clinic. He thinks they are the Pinnacle of the Neurology field.  He did suggest we wait until the IVIG has been in his system for a while and if there is any improvement.  

That's a good point, but not happening.   A call has been put in to Mayo Clinic.  Once, I talk to them, and get an appointment, I will let them decide how long we need to wait for the IVIG to be seen by them.

 

Braeden-ism for the week.   His new BP medicine is actually the same as one of the ones I take.  I was telling him how much it had helped my pressure, etc.  He said, "Mom, I am not sure its a good thing that we share the same kind of prescription drug bottles."    

You all are so appreciated.  Thank you all for being with us on this emotional, exhausting, never ending ride.

#BraedenBrave

#MrUnlucky

Good news Monday

Shortest post ever....

Blood pressure is better.  It is still high but manageable.   He needs a few more days for the medicine to work.  The decrease in steroids should help too.

Cytology came back and although there were "limited cells" with his sample but there isn't 

ANY EVIDENCE OF LEUKEMIA OR LYMPHOMA.  

Now we wait for the other testing to come back from Mayo Clinic and go from there.  Those tests will be ruling in or ruling out any autoimmune diseases.

Until next time.

#BraedenBrave

#MrUnlucky

Dreaming, Decadron, Diuretics, Diastolic, and hopefully (no) Duke visit

I really try hard not to over post, but sometimes I have  to keep up with all the ACTION that surrounds my oldest fellow. If I didn't keep this blog and my written journal, there is no way in HECK, I could keep up with it all. 

Yesterday, was another long day at Duke. Braeden had his 2nd IVIG and his 11th Avastin infusion yesterday. Thankfully, he slept through most of the treatments.  His blood pressure was misbehaving and hanging around 160s/110s.  Steroids are likely the biggest culprit causing it rise. Those little pills are the biggest troublemakers.  I am so glad he is being weaned off of them within the next few weeks. Avastin can cause issues with his kidneys which in turn causes BP issues, but Braeden's kidney lab work from Tuesday looked good.

They were able to finally bring it down with IV blood pressure medicine and, finally, diuretics.  Once the diuretics started working, there was no sleeping for him, lol.  We were told we could go home as long as we had a way of checking his blood pressure and to call in today with his numbers.  If his BP was still up today, we would begin blood pressure medicine.  Once again, thank you steroids for being such a PITA. 

This morning before school his blood pressure was 156/96 &140/90.  We made a plan for him to continue school at the kitchen counter and not to do anything else.   (Duke wants to see his bottom number (diastolic) below 90)

Around 11:45, I rechecked his pressure and rechecked and rechecked....His pressures were 187/125, 185/118, & 181/118.  

I called Duke and they immediately called in a blood pressure medicine for him.  He began the medicine today at 1:30.  Realistically, the medicine will take a day or two to start working.  

I gave them another call at 3:30 because he was having a nose bleed and his pressure was still high at 

187/112.   

He is pretty asymptomatic even with his blood pressure being so high.  If he ends up having a terrible headache, a more significant nose bleed, or if his pressure climbs anymore we will definitely head to Duke.   

For now, he is being waited on hand and foot, drinking lots of fluid, taking his Tylenol for his headache and says he is feeling  "pretty good".  

I just asked him what the worse thing about his day has been so far.  He responded pretty quickly and said having to get up for school.  I think the kid is going to be ok.

Prayers that his BP doesn't get any higher, and that we can all have a nice quiet weekend at HOME.

UPDATE just checked his BP at 5:45 after I posted this blog and his pressure is 160/90 so we are heading in the right direction. 

#BraedenBrave

#MrUnlucky

Shopping List

On Monday we saw Dr. Landi  and got the pre-op stuff  done for the lumbar puncture that was scheduled for today. His lumbar puncture was planned to be done under anesthesia in the OR. 

The team felt that in general his spine and case were too complex to do the tap with out putting him out.   (I totally agreed with this, the kid is fidgety, and the last thing he needs is to be fidgety with a needle in his back.)   Plus, I figured he would get a good nap, since the steroids keep him up most nights.  

Dr. Landi said he had quite the shopping list from CHOP of testing that they suggested  to be done with his CSF.   Shop away, shop away.  Answers are needed.

It was also decided that day, to start weaning his steroids, and a plan was made to be completely off of them by September 14th.  While they were a necessary evil and they did some good, they also did their share of bad.

The side effects, are just a big UGGGGHHHHH for him.  The constant hunger, the weight gain, the acne, no sleep and the list goes on.  He is ready to feel better, and it goes without saying that we all want that for him.  Because of the steroids he now has a condition called Cushings syndrome.  Most of the side effects that associated with the Cushings should resolve slowly after he is weaned off of them.  He unfortunately, has developed the deep purple stretch marks that are common with Cushings, and he will always have those as a reminder of his latest "gig" at Duke. 

Being 16, he may not get that scars tell a story, and being able to tell the story is way more important than how they look.  

Braeden also had Covid testing done on Monday.  I am happy to report that his test was negative.  He had to have a negative result for his "procedure".   He also reports that the actual "swabbing" wasn't that bad, and that NO, it didn't go up to his brain, lol.

Today was a fairly long day for Braeden.  We left home this morning at 8:30 and got home this evening around 8:15.  I think they need to put us on the payroll.  

In true #MrUnlucky fashion, the LP should have only taken 10 minutes or so, after he was prepped and "knocked out".  Of course, with his luck this didn't happen, he was under for almost two hours.   It took five different doctors to try to get his sample.  The doctors started with a 3.5 inch needle, and ended up having to use a 5 inch needle to obtain the sample. (Blame the steroids for making this so difficult) OUCH, is all I can say.  They were able to finally get about 16 ccs of CSF from him. Most patients only have 2 to 3 ccs  of CSF drawn from their spines.  His sample was larger than most, because of his "shopping list" and all the different testing that is ordered.  

We will not get the results of most of the autoimmune disease markers for weeks.  We will get the cytology results in about 72 hours, that will rule in or rule out Lymphoma or Leukemia.

After his LP adventure, we headed upstairs for his first IVIG infusion.   We go back tomorrow for another IVIG infusion and an Avastin infusion.  In the future, the IVIG can be given in one day.  Today and tomorrow are considered the "loading" doses and they want to ensure he tolerates it well.  The IVIG took about 3 and 1/2 hours to infuse.  Braeden was tired, and hurting during the infusion.  When we got home, he went to bed after he ate and has been sleeping ever since.   

I sure do hope that his full day of "work" will provide the answers that are needed to get Braeden back to being Braeden.

Thank you all for the love and prayers.

#BraedenBrave

#MrUnlucky

Is the mystery solved? NOPE! Is there a Venn diagram to lay out our plan? YEP!

Home from Philly...

The past 60 hours have been busy.  Those hours were filled with doctor appointments, more testing, a lot of yummy food, some heavy traffic, and a little sight seeing. 

I will try to condense everything and keep this post shorter rather than longer.  

A lot of those 60 hours, were spent questioning the doctors, and asking what more we could do?  Where else could we go, if you can't solve the mystery of Braeden?

I had written about Braeden "stumping" the Duke doctors a few blogs back.  WELLLLL, it seems he is stumping the doctors of CHOP as well.  Is this what we wanted to hear?  No, it's not.  Is there a plan? Yeah, kinda.  

I will start with the appointments we had yesterday.

Dr. Brandsema the Neurologist

His best guesses were as follows:

        

    The testing shows that Braeden's problem is mostly motor and not sensory.  (This is RARE.  Typically, it is a mix of motor and sensory)   Of course, this kid is going to keep these doctors guessing.  

    

    Braeden's issues are nerve root related. This could be caused by a autoimmune process.  Most of the the things that cause nerve root issues are not treatable.  

    His suggestion is to try a "Hail Mary" and begin IVIG infusions for six months.   He said it was a long shot, but if there is a response to the drug then great. (IVIG is a blood product used to "wash away the bad antibodies" in the blood that are attacking the "good". )

    He also questioned, why a sudden auto-immune disorder if he has already had a brain tumor and the spinal cancer? (Kind sir, that's why we are here.)

    

     He thinks that his symptoms are tied in to his tumor, or possibly something called Chronic inflammatory demyelinating polyneuropathy (CIPD).  He said it was a long shot, but CIPD can be motor related.   (His EMG does not support this diagnosis.)

So our plan after leaving his office yesterday was:

    Plan a lumbar puncture at Duke. (I texted Dr. Landi this morning and he is going to take care of this getting this ordered for next week)

        

        

The results will rule in or rule out the CIPD, and or a Lymphoma or Leukemia process.  Both Lymphoma and Leukemia can cause multiple nerve root issues, and his roots are are all being affected.

If his cerebrospinal fluid shows an abnormal cell pattern then it is likely CIPD.   If the cerobrospinal fluid show abnormal cells it may be a Lymphoma or Leukemia process.

Are you dizzy yet?  Because, I am.

Our next appointment was with Dr. Chen yesterday.  She is one of the Neurosurgeons at CHOP.  I will just let a picture of my notes below sum up our appointment. 

Today, we met with Dr Phillips who is the neuro-oncologist.  He literally spent hours with us today.  He has no clear answers.  He only has BEST GUESSES as to what is going on. His best guess is that it is a paraneoplastic neurologic syndrome. HIS BEST GUESS, does that suck? Yes it does, and I 

couldn't help to ask, what next?  

If you don't know, WHO WILL?  Dr. Phillips looked at me and said he would not leave us behind.  Once, we get the lumbar puncture next week at Duke, and if we find out we have one of the  paraneoplastic neurologic syndromes,  he will help us navigate and find the expert we need to deal with "his PNS".

If it is not a PNS issue, we will begin the IVIG infusions, and hope for the best, that his symptoms will get better, and we can stop the steroids.  I certainly hope it is this simple of a fix.  But as Dr. Philips stated today, "Braeden's case is CONFOUNDING and doesn't make sense."

Our appointment is summed up below, Braeden read it and said it's my VENN diagram, lol.

This road is not over for my boy.  

I appreciate all the love and prayers.  He deserves them.

#BraedenBrave

#MrUnlucky

    

Got Brace?

Got Brace?  

I know Braeden wishes he could get rid of one or two. 

He has been wearing his C-Collar and loathes it. That's probably the nicest thing I can say on his behalf about that subject.

Two weeks ago, the Occupational Therapist at Duke added 4 additional braces.  Two of which, he is using during the daytime for his left and right hand.  She wants the extra stability for his wrists.  His left wrist is extremely weak and "floppy" and if he were to dislocate that wrist it could cause another set of problems. 

Another set of problems is the last thing he needs. Can I get an AMEN!?!

The other two braces will be the  "two fancy custom-made for both of his hands that cost a small fortune" that he is to wear at night to try to keep his fingers from contracting any further. 

He has also been working his "OT program."  Think hand putty exercises, and wall push ups to try to increase upper body strength, etc.  

In the two weeks, since his initial OT with the Duke therapist, it seems he is doing something that is helping.   His hand strength was averaging around 10 pounds the week before he saw the new OT.  The 1st week of him doing "his program" his average was between 11 and 12 pounds.  This week his average is between 13 and 14.  It is definitely nice to see the numbers rising.

Yesterday, he had Avastin #10.  

Braeden-ism of the day yesterday.  

We take a picture of Braeden during every infusion.  Yesterday, he stated the obvious that he was running out of fingers to hold up.  I suggested we hold up toes for treatment #11.  That idea was nixed very quickly.  The kid wears shoes and socks ALL the time, and is not a fan of bare feet.  

I suggested a sign for his mask.  He thought about it, and then he said that he thought sharpie knuckle tattoos would be better.  OK, kid as long as you stick with the sharpies.  

#11 pictures to come...

We met a new intern, who had recently worked with Oncology Neurology team at Mayo.  He spoke highly of the ONC-Neuro  team at Mayo.

 

I told him that in two weeks if we hit a dead end with CHOP that he could expect a call from me, asking him to help with getting a referral at Mayo. (I think he took me so seriously, because I looked old enough to be his Mama, lol.)  

The disappointment of the UDN still hurts, but  I know it wasn't the path we needed to take. Braeden showed me videos on his phone yesterday from his bowling days.  I know the weight of all this is a lot for him to bear.  I know he misses his normal.  Answers to get him back to his normal is all we want for him.  As a family, we had a pretty good handle on the brain tumor side effects.  It is time to get this spinal cord tumor figured out.  

We are running out of options, and I will not stop trying to find the right place for Braeden if CHOP does not work out.  

We don't have a lot going on for the next week or so.  This Saturday he has another MRI.  Dr. Landi wants another spinal MRI on the books before CHOP.   

Keep the prayers coming.

Love to all.

#BraedenBrave

#MrUnlucky

One Door Closes

As the cliche goes, when one door closes another door opens.   I will let the letter below speak as to how one door has closed.

Disappointment, saddened, discouraged, crestfallen are just a few emotions my Mama heart is going through.   I am so ready for Braeden's miracle to happen.  August the 18th cannot get here soon enough.  Hopefully, behind the doors at CHOP will be Braeden's miracle we so desperately need.

Love to all.

Keep praying for my hero.

#BraedenBrave

#MrUnlucky

Another day, another Avastin (#9)

Hi to all,

Braeden had another Avastin infusion today.  This was done because his steroids were increased to 8 mg a day last week, and his numbers have not changed or improved from the 10 pounds.  This number continues to concern, and scare the crap out of us.  His left hand is at 2 to 3 pounds and if you have followed this blog, you know how little he is able to do with that hand.  (For reference, Braxton his twin, is able get numbers in the 90 pound range.)

Below are pictures of him with each treatment he has had thus far.

Ideally, we would love to see his hand strength numbers rise, and keep rising.  Unfortunately, we need have to have realistic expectations.  It is likely, he will never have full use of his hands again.  Braeden, is not sure if he will be able to write or type to meet the expectations of being a high school junior.  I can't even begin to think how that is going to work out.  

I am planning on contacting "my school people" who have been fantastic throughout this past year and  a half at the high school and plan a meeting,

Chop Update

So far, we have a full day of appointments on 8/18, and an appointment on the 19th.   Plane tickets, hotel, and car have been reserved.  8/18 cannot get here soon enough.

National Health Institute/Undiagnosed Disease Network

Dr Landi told us today, that UDN has contacted Dr. Smith.  Below is the email to Dr. Smith. Big prayers are needed that NIH & UDN will get in involved with his case.  

This tumor has taken away quite a bit from my boy.   Right now, I would be happy if it just returned a few things.  

Continued prayers are appreciated.  Love to all.

#BraedenBrave

#MrUnlucky

Delays, tears and a lot of disappointment

Braeden’s appointments at CHOP  have been delayed until 8/18/2020.  The reasoning is the whole team would not be able to see him next week while we are there.  Dr. Minter, who is coordinating all of the team was not able to get us neuromuscular appointment till 8/18.   I will keep you posted as I know.   I know that things happen for a reason, but this stings a lot.  

#BraedenBrave

#MrUnLucky

Shot in the Dusk...

"No, I wouldn't say it's a shot in the DARK, but rather a shot in the DUSK."   This was Dr. Fuchs response when I questioned, if Braeden having a spinal fusion would be a shot in the dark.  

Comforting, I know...

He had just finished telling me that out of a team of 4 spine physicians, that only one of the physicians would consider doing the surgery.  Believe me, we don't want him to have the surgery.  

But there is something very wrong, there is no doubt.  

Just in the past six weeks, Braeden's right hand has deteriorated even further.  

His hand strength in May averaged 18 to 20 pounds.  

In the month of June, his average fell to 13 to 17 pounds. 

In these first few weeks of July, his strength is averaging 10 to 11 pounds.   

Braeden told Dr Fuchs today at this rate, he wouldn't have use of either hand in 6 weeks.  His left hand strength has been at 3 pounds for months.  He has been "one handed" for months using his right.   

It is alarming how quickly his right hand is deteriorating, and the amount of muscle wasting and atrophy the right hand is now showing.  

The simplest of things are becoming even more impossible for him.

Last night, we were playing a game, that required him to write his answers.  He was only able to write by holding the pen with a closed fist.   Besides, all of the other things that is going on with him.  How in the heck is he suppose to get through what will already be a challenging 11th grade school year?

Plans were made today for Braeden to skip Avastin tomorrow.  (If CHOP deems surgery necessary, they don't want it to be delayed by 4 weeks.  This is the amount of time he has to be off of the Avastin if he requires surgery.)   Clearly, we don't have 4 weeks to spare.  

Could it be that because he has been off of the Avastin that this is causing the quick decline of his right hand?  If you know the answer, just put MD behind your name and say you work at Duke.  Your guess is just as good as anyone else's.   

Our band aid solution to stabilize his hand strength is to begin the  massive doses of steroids again.....I can't even say how much this sucks for him.  The side effects are terrible.  

He is to continue to wear the C-collar.   (I can't tell you how much fun this kid is having.)

CHOP update:

We plan to fly out on Monday.   He has an EMG first thing Tuesday morning at University of Pennsylvania.   (EMG=PAIN.)

We have a MRI scheduled on Tuesday at CHOP. We will see the neuro-oncologist, and the neurologist that day as well. 

Everyone's love and prayers and thoughts are greatly appreciated.

#BraedenBrave

#MrUnlucky

Countdown to CHOP 11 days and counting

I am so very excited to let everyone know, that we have been scheduled to be seen at CHOP on July 21st at  10:15 am.
Eva, the scheduler, with whom I spoke to make this appointment is planning on calling me back today at 4 pm call her back today at 4 pm to let how me know how many days we need to plan to stay. 

This news couldn't have come at a better time.  This past Wednesday, Braeden recorded his lowest right hand strength of 10.2.  We restarted his steroids in hopes that this would improve his numbers.  Steroids are a last resort, and I know his doctor was hesitant to restart them.  It was definitely a "between a rock and a hard place" decision. 

Next week, Braeden has a follow up with Dr. Fuchs on Tuesday.  This will be the day we discuss if he thinks Braeden needs a spinal fusion.

On Wednesday, we have a tentative Avastin infusion scheduled.  If we plan a surgery on Tuesday, this appointment will be canceled. 

Until next week!

#BraedenBrave
#MrUnlucky
#B.A.S.S.

#B.A.S.S.

Hi to all, I wanted to update you all about Braeden's appointment this morning.  I am very appreciative of Dr. Fuchs coming in during his vacation week to see us.  What better place to be, than work when you are on vacation, right?   He started out the conversation saying how surprised he was by what his x-ray showed this morning. I believe the next word he used to describe the x-rays was perplexing. But honestly, are any of us surprised?   NOPE, this thing from the beginning has been the biggest mystery for all involved.  Very little about this whole thing makes sense.
I will attach a picture of both his MRI from last week and his x-rays today.  On the MRI, you can definitely see that when Braeden bends his head, that his spine becomes compressed.  On the x-ray, you can see that when he is sitting straight up, bending his head forward and backward that there is one part of his cord that is not moving with the rest of his cord.  This is one of the things that is  perplexing to Dr. Fuchs.  (Never mind, the fact that he has stenosis along with having the tumor.  As Dr. Fuchs said this morning, none of the puzzle pieces are fitting together, and Braeden issues are quite a mystery.) 

I will just let the pictures do the talking. (The X-Ray pictures are first and the MRI images are second.)

Braeden was fitted for a hard C-collar today, and he is to wear it while awake.   On a scale of 1 to 10 this rates around a negative 1000 for him.  He quite simply hates everything about it.  Hopefully, he will get the hang of it after a few days, and find a way to acclimate to wearing it.  One of the issues, is he tends to look down when walking, and with the collar he is unable to do that.  Rest assured, he says he is going to figure out a life hack to make it work. His first attempt is below, and as you can see he still has some brainstorming to do.   The thought is that if he wears this for two weeks and he shows some improvement with his hand strength, that he will be a good candidate for a spinal fusion surgery.  We continue to wait for CHOP.  We are holding the Avastin, in case he is scheduled for surgery on July 14th when we return to see Dr. Fuchs and Dr. Karikari.

Braedenism for the day

The doctors keep telling him that he needs to name this thing of his.  Because it is likely he is the only person in the world that has what he has.  Braeden decided he needed to come up with an acronym. 

On the way home today, he decided on B.A.S.S. (Pronounced like the guitar, not the fish.) 

Braeden's
A-hole
Spinal
Syndrome

We are open to any other suggestions. 
Thank you all for the constant love and care you show us.

#BraedenBrave
#MrUnlucky
#B.A.S.S.

Fickle and bizarre

If I could sum up Braeden's tumor and medical stuff in one word, I would chose fickle.  This thing is always full of surprises and seems to be ever changing.

I don't know if I can keep this short, but I am going to go day by day since Friday to update you all.

Friday (June 26th) 11:00 am

That morning from CHOP, I got a very encouraging email from Amy.  The team at CHOP are reviewing his case and are trying to coordinate all of the appropriate providers so they can begin to schedule consultations accordingly.  They also want to be sure that once we are there we have a productive visit, and  that we meet with as many provider's as possible during our time there.

This is awesome news!  I am elated that CHOP is willing to see him, and hopefully give us some answers.  

Friday (June 26th) 4:30 pm

Dr Landi, called me with the results of the Flexion MRI.  I believe if he could chose a word for Braeden's issues he would say "bizarre".  He used this word at least 5 times during our conversation.
The long and short of it is....

The flexion MRI shows moderate to severe stenosis at C5, C6, & C7.  Its bizarre because it's located where his laminectomy was done and he is missing the lamina.  The MRI definitely shows impediment on the spine.  It is also bizarre, because apparently you never get stenosis with a spinal cord tumor.   Stenosis is also more common in the lower back not the cervical area. Dr. Landi's best guess that this is likely due to an anatomical defect?  Again, bizarre... He said that the MRI was pissed when Braeden flexed during the images.  As of Friday, the plan was for Dr Fuchs to speak with a spine neurologist and give me a call on Monday.  Dr. Landi and Dr Fuchs had already spoken before he called me.  They both think that a spinal fusion surgery will be suggested.

UGGGGGGHHHHHHHHHHHHHHHHHHHHHHHHHHHHHHHHHHHHHHHHHHHHHHHH

I asked for the new MRI results to be emailed to CHOP.   

Saturday & Sunday (June 27th & 28th) 

I didn't give Braeden an update over the weekend about his results.  I didn't want him to have it on his mind, and I wanted to speak with Dr. Fuchs office on Monday and get their thoughts.

Monday (June 29th)
Dr. Fuchs office called, they described his MRI as showing instability.  They want to see us first thing in the morning at 8:00.   (That's going to hurt a teenager I know :)   He didn't get up today till 1pm, lol.)

They have scheduled Braeden for a flexion and extension X-ray at 8:00 am.  Dr Fuchs hopes this will show which is doing the more pinching, the bone or the nerves?  We are also suppose to meet with an adult spine neurosurgeon tomorrow. 

I talked with Braeden today about all of the above.  I think the most important question he wants an answer to, is the same one that most of want to know.  "Mom, if I have that surgery, will my hands get back to normal?"   I sure hope so, my sweet boy. 

#BraedenBrave
#MrUnlucky

Prayers, good vibes, well wishes and positive thoughts. Pretty Please

Twenty days since my last post here.  I have started a paper journal documenting all this "Braeden stuff."  I don't want to blog every little thing that happens day to day.  Since May 13, I have written 14 pages in my journal.  This kid definitely keeps me on my toes trying to keep up with everything.  (Thank goodness, he is my favorite full time job.) 

I will give a quick update about the past 3 weeks.  Braeden was on vacation with his dad for two weeks, and got a much needed break from all this "Duke" stuff.  He  was on standby to return back home if CHOP accepted his case.   During vacation, Braeden recorded his two lowest hand strength numbers.  June 11 his strength was 14.9, and on June 16th his numbers went to 13.9.  The cause is likely due to him weaning his steroids and probably all of the beach activity he was not use to doing.

As Duke time goes, the pathology that CHOP requested did not arrive until last Friday the 19th.  Amy, the navigator from CHOP called me and told me that on that very same day she presented his case to the team of doctors.  She said I may get a call from Eva who is the scheduler to try and coordinate appointments sometime this week.  So far, I have not heard from Eva.  Amy said I should expect a call by the end of this week if the team decided to take his case.  We appreciate any prayers, good vibes, well wishes and positive thoughts that this will happen.  

Children's hospital of Atlanta, contacted Dr Fuchs, and suggested Braeden have a spinal tap done, to rule out MS.  Dr. Fuchs thinks this is highly unlikely the cause, and does not want to plan for him to have that test performed.

Monday he finished his second round of steroids.  The Neuro oncologist we saw today does not want to continue the steroids because of the overall side effects from taking them long term.  Even though his hand weakness is not better.  

Yesterday, he had the flexion MRI to rule out the Hirayama disease.  No results yet.

He had Avastin #8 today. 

Braeden reached out to his band director yesterday, and asked if he thought the trombone would be a good fit for him.  For now, he is not going to be able to play the trumpet.  He is unable to press the valves down.  Hopefully, the trombone will be a good fit for him.  Being able to rest the trombone on his shoulder and having to "slide" the trombone rather than press the valves should allow him to continue in the marching band.  (This is so important to us all.  He loves his band family.) His band director emailed him back and is allowing him to switch instruments.  Now Braeden has the fun part of learning a new instrument.  Braeden was joking yesterday that he was going to have to figure out his new spatial awareness with the trombone. He has been a trumpet player since the 6th grade. 

I ask again for all prayers, good vibes, well wishes and positive thoughts.   Mama really wants to take her baby boy (man-child) on a jet plane to Philadelphia.

Just ONE page???

I was sent an email this past Wednesday from the Undiagnosed Disease Network.  One of the things they requested was that I write a one page narrative of Braeden's journey.  Whoa!!! Just one page?  They obviously have no idea how wordy I can be once I start typing. I felt a lot of pressure to make the narrative perfect.  (Braeden being accepted into the NIH could be the life changing miracle he needs.)   I started with several pages of notes and an outline.  Once, I felt I had my train of thought together, I began to type.  I finished the paper last night, but I was well over the one page limit.   I enlisted the help of my brilliant brother, Eric, who happens to have a Master's degree in English to help me edit my first draft.   He did a fantastic job putting my semi colons where they were needed.  He was also able to condense the paper to meet the requirements.

Below is the final narrative I sent to UDN.

How do I begin to summarize Braeden’s, my courageous and battle-worn son’s, medical journey in one page? A journey that has lasted for more than fourteen of his sixteen-year life. Anyone who knows me understands how difficult of a task this is for me to complete. I tend to get lost in my words as I advocate for my son who has been dealt a cruel hand since birth. I will try to keep this as succinct as possible, and please know that I appreciate every opportunity to tell about my personal Hero who has been treated at Duke for two different tumors.
I will start with his Craniopharyngioma brain tumor with which he was diagnosed when he was 21 months old. This tumor was quite a shock, and it changed everything for him. The tumor left him legally blind while also taking a wealth of medications daily to supplement not having a pituitary gland. Let’s not even mention the dreaded words, “vomit, fever, or stomach bug.” For most children, these words are not a big deal, but for him it usually means an ED visit and an overnight stay at the hospital. For the past 13 years, we thought he would be “safe’ from anything else medically going wrong for him. My son has a full plate already with all the things that come along with being a “Craniopharyngioma” kid. We were, no doubt, surprised when he was diagnosed with a Diffused Astrocytoma Grade Two spinal cord tumor. It began when Braeden started having left hand weakness. He couldn’t hold a pool stick; he wasn’t able to type on his laptop; and he couldn’t even tie his shoes. After three months of countless appointments and diagnostic testing, the team at Duke decided that the spinal cord tumor needed to be removed. He had a Laminectomy on November 28, 2018, and Dr. Fuchs was not able to remove all the tumor for fear of causing life-changing damage. Surgery and recovery went well. He was motivated to get better and to return to school as soon as possible. His life did, for the most part, return to his normal during 2019. His weakness in his left hand was really his main obstacle. The fact that something else could go wrong never crossed my mind as this kid had now had two different tumors and surgeries that would scare most of us and bring us to a halt. However, life once again slammed its brakes, and his normal day to day routines stopped. Braeden began experiencing weakness in his right hand in late 2019. Braeden knew; I knew; his father knew; his stepparents knew; his brothers knew that Braeden could not risk losing the use of his right hand like he had with his left. The loss of his right hand would devastate him as life is already difficult enough for him to navigate with his challenges. During January 2020, it was imperative to us and the doctors that we urgently treat Braeden as quickly as possible to slow down any further right hand atrophy and weakness. Any further weakness in that hand would be detrimental to his daily life. Braeden underwent 30 radiation treatments, Avastin infusions, and massive doses of dreaded steroids. After he finished his radiation, Avastin infusions, and the steroid regimen in early April, we continued to test his hand strength at home to ensure he was maintaining adequate numbers. For reference, in October 2019 his right-hand strength was 69 pounds; during his radiation, his strength averaged 23 pounds. By the beginning of May 2020, his strength was at 15 pounds. This caused us great alarm, and the decision was made to begin Avastin infusions and steroids once again. The toll this is taking on my son’s physical, mental, and emotional well-being is indescribable, yet though it all, he remains hopeful. We know he is fearful, but he maintains dialogue with us and asks us and the doctors thoughtful, focused questions as he seeks understanding in his journey. It breaks our hearts that he has had to do so his entire life. Alas, in mid-May we were informed by doctors at Duke that they had “exhausted all ideas and avenues” in trying to figure out what was causing the atrophy in Braeden’s extremities. I am writing in hopes that your facility and team of doctors will give us the much-needed answers we so desperately desire for our son. Braeden has weakness and numbness in all four of his extremities. His hands presently are causing the most issues. Unfortunately, Braeden is unable to do two of his favorite pastimes. He is unable to bowl in his bowling league, and he is unable to play his trumpet. For a young boy, who is already blinded by a tumor as an infant, not to be able to do the two things he has worked so hard to accomplish is devastating. I have only seen him cry once during all of this, and it was when he realized he couldn’t hold on to his bowling ball any longer. The tumor finally won, but only for a short while. Braeden decided within moments that if he couldn’t hold the ball with his fingers, he would give a two-handed bowling technique a try once he finally finishes with treatments.
Braeden being Braeden has always found the positive. He often jokes with his twin and his younger brother about his “normal” vs their “normal.” I know it must be difficult for him to see these differences. Braeden is the kind of child you hope you have when you become a parent. Braeden is funny. Braeden is smart. Braeden is who you want your student to be. Braeden is who you want your best friend to be. Braeden is who you want to be at your side. Oh, and Braeden is sarcastic at times. (He is a teenager, after all.) Most importantly, Braeden has fought harder and overcome more with grace than most of us. He has done it all without complaint. Honestly, he has every reason to complain, but he simply doesn’t. This kid, my firstborn, deserves a chance to get back to his “normal.”
I will leave you with what I call a “Braeden-ism.” He recently asked me that when a doctor or a person asked him what kind of tumor he had, should he say brain or spinal. I told him he should say both because he has had them both. He then smiled and replied, “I just wanted to be sure that was ok. I don’t want anyone to think I am an overachiever.”
Thank you for your consideration,
Marsha & Braeden
For more Braeden’isms visit my blog which follows his journey from the beginning.
www.braedenbrave.blogspot.com

This week's update
Braeden had Avastin number 7 this week, and he is scheduled for his flexion MRI on June 23rd.  In speaking to Children's Hospital of Philadelphia this week, they are waiting on
pathology slides. Once they have these slides, we will be closer to an answer as to whether they will accept his case.

Till next time...

#BraedenBrave
#MrUnlucky

Not your Mama's Band-aid

Today, Braeden had another busy day at Duke.   He has a total of 6 appointments this week alone. He had the SSEP testing done today.  This test will be used to measure the electrical activity in his extremities. This test may pinpoint if the issues he is having are originating in the brain or in the spine.  He is also scheduled for another EMG on Friday morning.  It measures nerve response, and it is very painful.

Braeden is a tough kid, but he hates the EMGs.  Think of a 2 inch needle being buried deep in your arm and legs to try to get a nerve response. OUCH!

It was decided on Monday for Braeden to begin the Avastin infusions again.  He had Avastin #6 today and the plan as of now is for him to get them every 2 weeks. At this time, we have been told that he may be on them between 3 to 4 months.  I think the Avastin is a band aid to help get Braeden through the next months to prevent further weakness with his extremities, and we can be seen at a bigger facility.

I sent the application in to the NIH/Undiagnosed Disease Network last night.  In looking at their website, I found these stats. 

• Applications received 4467
• Applications under review 398
• Participants accepted 1663
• Participants evaluated 1392
• Participants diagnosed 408 

Dr Smith, the neurologist, called me last night and he plans on seeing Braeden next week, along with a rheumatologist. 

Since last week's post, I can't tell you how thankful I am for everyone reaching out to me with "leads" and contacts at facilities across the US.  There are doctors all over the country looking at his case.  

As soon as I posted this, Dr. Smith called me and we talked for 30 minutes.  The long and short of it is, the test results were normal, and this means that it is unlikely brachial plexus, or peripheral nerve damage.   The plan is to get the EMG on Friday and discuss those results.  If the EMG is normal, this suggests that the issue is arising from his cord.
Another test that may be done if the EMG is normal is called a Magnetic stimulation, aka Motor evoked potential.  This test basically records where the motor signal slows down when it is traveling from the brain. This would allow us to know if the slow down is happening in his cord, etc.  It also could rule in or out Hirayamo disease. That has been brought up to us a few times, but they don't think that is the cause of this.  They just don't know.

We are hoping that we get connected with a team/hospital that can solve this mystery.  

#BraedenBrave

#MrUnlucky

The kid that has STUMPED the entire DUMC team of doctors

Hi to all, I wanted to give a quick update about our past few days.

Wednesday

Braeden had a complete spinal MRI, and an appointment with his oncologist.  His MRI showed no new changes with his tumor even after radiation. (This really sucks that he went through those 30 treatments without a positive result.)
Braeden's right hand still continues to grow weaker.  During radiation his right hand strength averaged between 22 and 24 pounds.  In the six weeks since he ended radiation, the Avastin, and the steroids, his hand has continued to weakened and was at  a low of 15 this week.

Last Friday, Braeden was unable to press down the second valve on his trumpet.  I wish this thing would stop taking away the things that he loves. Between not being able to bowl, and now not being able to play his trumpet, I know his heart hurts.  I know that mine is hurting for him.  


Dr. Landi was very frank with us.  He used some of the words and phrases you don't want to hear at an appointment, "serious, rare, tragic, running out of time, have to keep an emergent pace"  Then he said something I never expected to hear. "Our team has done all they can do for Braeden.  We just don't know we need to do for him.  We can't figure out what is wrong. We need to get him to another team who may have an answer."    In my head, I was thinking WHATTTT ????? We are at Duke what do you mean you have done all you can do??????   

He emphasized that they would still be there to help us along the way until we find the right team to figure this thing out.  They are going to connect us with the neuromuscular team and autoimmune team and hopefully they can provide some answers.   The plan at that time was to possibly have Braeden admitted to the hospital this weekend, and have the teams consult while he was an inpatient.  Braeden began a whopping steroid dose again, and he hates them.   He doesn't sleep on them, and is a starving more so than usual teenager.   Avastin is not off the table, he may have to do more infusions if his right hand keeps getting worse.  Those two drugs are not a cure, but a bandaid that is not a long term answe

I brought up the possibility of  Braeden going to NIH (National Institute of Health) they have a rare undiagnosed disease clinic.   They may be able to come up with more answers.  He also suggested going to UNC for a consult.  I told him that we couldn't waste another six months to a year at UNC running the same tests.  I feel that both Duke and UNC are great and comparable hospitals, and  I wasn't really sure what they could do any differently. 

The next day everything changes.......

Thursday

Braeden fell down 12 stairs today.  I can't help to think it is because his legs and feet are getting more numb.  I texted Dr. Landi and asked him to call me.  My intent of the call was to ask him to start on the road for a  consult at NIH.  He said it is a difficult thing to get "in" NIH.  He said he was going to touch base with a patient's parent of his, whose son is seen at NIH and ask for her MD's information.  I told him my nephew was a patient at NIH for an autoimmune disorder.  He asked me to email my SIL and give her his number and info and send it to the doctor they see.  He hopes to be able to talk with one of them and see if this will fast track Braeden and get in sooner.

Then he once again said something I wasn't expecting to hear.

"The autoimmune and neuromuscular team contacted me today, and they said  that they don't think there would be any benefit to have them consult with Braeden. There will be no need to admit him this weekend."  You can't be serious!!!!!  WHY?????  WHY not neuromuscular at the very least?  He just fell and is now on the verge of losing use of another hand.    He said that they felt like any test they would have ordered has already been done.

So now what?  I wish I knew....

Friday
I spoke with his pediatrician today, she has been with my boys since birth.  I wanted her thoughts and her opinions.  She suggested that we pursue a consult at UNC while we are waiting for NIH to "accept us."  At this point, we have nothing to lose and it only takes just that one person that could solve this whole thing.  I am planning on asking Dr. Landi's office on Monday and seeing if they can refer us.

Dr Landi's PA also called me today, and she gave me the info I needed to get started on the path to NIH.  Apparently, it is a long process, and from reading their website today, it will be even longer because of Covid-19. (Ughhhhh, we don't have any time to waste.  He is struggling daily doing simple things and we keep added things to the list weekly that he is having problems doing.) 

I filled out my application. I need a document from their office which I will get on Monday.  And from there it is out our hands.  The hope is that they will accept Braeden into their program, and then the bigger hope is that we get our Braeden back in good healthy working condition.

I want to thank my sweet family and friends that have been keeping sane.  The texts and phone calls and messages are very appreciated. Everyone asks what they can do.  I have been telling them just to send tissues and pray.   I need the tissues and he needs the prayers.

#BraedenBrave
#MrUnLucky