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

Final poison

Braeden had his final Avastin infusion today.  I am happy that this part is for over him.  Avastin works differently than chemotherapy.  Chemotherapy attacks the cancer cells of the tumor.  Avastin blocks the growth of new blood vessels that feed his tumor.  The side effects of Avastin can be serious, and typically not recommended for use in children. (I know that Braeden, is a man-child, but until he turns 100 he will always be my baby, lol.) 

So what is next?

I asked this same question of the medical team today. 

• Following up with Dr. Smith about the SSEP. (The testing has not been ordered, as of yet)
• Following up with OT at Duke to try to get an appointment.  Duke OT is only seeing via video, and Dr. Smith would like him to be measured for a new brace.
• Spinal Cervical MRI on 5/13

Then what?

I don't know, but this "journey" is not over.  We appreciate all the prayers and can't wait for this chapter to be complete.

Love to you all!

#BraedenBrave

#MrUnlucky

I will end with the funniest Braeden-ism of my day.  We were navigating our way through Duke, and I missed a turn.  Braeden said, "Wow Mom." I told him to shush, that I have had cancer and my chemo brain is at work lol . He then replied, "Mom, you had cancer in your b***S, I have had cancer in my brain and my spine, and I still know where to go."  

He makes me laugh at the most unexpected moments,  Love him so much!

No alarms, no appointments, and apparently no answers...

Braeden finished his 6 weeks of radiation this past Thursday.

It has been nice not having to go to Duke everyday, and it's been even nicer not to have to set those early morning alarms.

He is having a pretty quiet week, and has been working on school work and trying to get caught up on all of his missed assignments.

His last Avistan infusion is next week. 

He has a new cold spot on his right ankle, that can't be explained. This means that now all 4 of his extremities are showing some sort of unusual symptom.

His steroid "hunger rages" have definitely calmed down. This makes the short order cooks,who live with him, jobs easier.

Dr. Smith, the Neurologist called us on Saturday. The call was long, and we still have no real answers. Plans were made on Saturday for Dr Smith to get in touch with Dr Hussain, and schedule a test called SSEP (Somatosensory evoked potential).This test will measure his electric signals of sensation between his brain and spine. They will be looking at 3 different waveforms to try to pinpoint where the new issues are originating.

1st waveform will measure the brain's response to the the electric pulses.

2nd waveform will measure the spine's response to the electric pulses.

If the 3rd waveform ends up being slow or abnormal, it is likely the issue lies within his spine.

If both the 1st and 2nd waveform are abnormal, this will mean that the testing is unable localize the issue. And we are back at square # who knows by this point.

I think that we all are frustrated with the lack of answers. As his Mama, I want this crap to be done. I want Braeden to get back to his normal routine. I just want him to be a kid without the weight of this unknown demon to bear. I know his medical team is working hard to try to get the answers to his case. I told Braeden, he didn't have to be SO unique. Like really kid, quit stumping all the brilliant doctors at Duke and across the world.

Blogging allows me to have a visual timeline and record of what is happening with Braeden.

Blogging allows me to vent my thoughts and feelings.

Blogging allows me to tell you all, that I love you and I appreciate your support and prayers.

Below is a small part of Dr. Smith's visit notes, after seeing Braeden. I know it's a confusing read. I included part of his notes for my records. Feel free to skip to the end of this post for a funny Braeden-ism.

"This is a perplexing, and very unfortunate, case. I reached out to a neuromusuclar colleague here at Duke to discuss this case further, and I still feel that central (cord) pathology could explain the symptoms. This is largely due to the intact ulnar and median SNAPs, normal median CMAPs and relatively preserved ulnar CMAP amplitudes (right) - all in very weak muscles. Furthermore, there are other weak muscles that demonstrate normal findings on EMG (ie, RIGHT EDC). The low-amplitude LEFT ulnar CMAPs could also be explained by the underlying severe muscle atrophy as opposed to true denervation. To some extent, there may be a combination of both peripheral and central etiologies.

I do not have an answer at this time to explain Braeden's clinical condition. This is terribly unfortunate given everything this young man has had to endure. Regarding further testing to possibly help with localization, media and tibial SSEPs may be helpful, especially given the low concern for a significant peripheral neuropathy. Lower extremity nerve conduction studies could be performed prior to SSEPs to rule out a tibial neuropathy (which would confound tibial SSEP interpretation), but given the lack of any concern for such on exam, I do not feel that this is necessary.

Finally - I suppose it is possible that a "Hirayama-disease-like" picture could be contributing as well. Given his known C6 tumor, he does not meet the definition of Hirayama disease. But, a dynamic (flex/ext) C-spine MRI might be informative if it demonstrated significant anterior displacement of the cervical cord upon neck flexion. This is a long shot, but it is the only other consideration that comes to mind. There is no clear cord atrophy in the C6-T1 region in my opinion.

I am sending several screening labs for "idiopathic" peripheral neuropathy, but Braeden's clinical picture really is not consistent with that diagnosis.

I will discuss my assessment with Dr. Landi, and if he agrees, then we will proceed with SSEPs and consider flex/ext C-spine MRI. "

I will leave you with a "Braeden-ism"

In the car, on the way to Duke the other day. I told him how brilliant his answer was to the question I had asked him. He replied, "Mom, it must be all of that brain enhancement I had when I was a baby. You know? Those 3 little brain surgeries."

This child has my whole heart.

#BraedenBrave

#MrUnlucky

A post from Uncle Eric

 I am lucky.

My children are lucky.

My nephews are lucky.

My siblings are lucky.

We are all so lucky to have Uncle Eric.

My precious boy will finish his 30 radiation treatments tomorrow. We are still facing so many unknowns about his condition.  There are still too many unanswered questions. This journey will not end tomorrow.  I am so thankful for our family, friends, and all the prayers that have gotten us this far. We are exceptionally blessed to have Uncle Eric in our corner.

Below are his words about Braeden.  

When I was younger, I always knew that I wanted to be a parent.  I felt that it would be one of my life’s greatest accomplishments.  To be able to raise a child, to teach them, and to help them develop as an individual of character who will one day go forth and make a difference in the world because of your influence was a challenge I eagerly looked forward to accepting.   Unfortunately, in my twenties, I learned that would not be possible for me.  It was a difficult truth to learn and to come to terms.  What I did do, however, was decide that I would be the BEST uncle that I could possibly be when and if given the opportunity.  I am now blessed with five amazing nephews who I love more than life itself.  Each one has a personality as unique and as special as the individuals they are, and all five are highly intelligent and talented with varied interests.  Among them, there is Braeden, my oldest nephew.

Braeden has always held a special place in my heart.  I have been there with Braeden as well as my sister for every significant surgery, treatment, and milestone in his life whenever humanly possible.  Since the birth of my three oldest nephews, my sister has done an extraordinary job keeping me informed and a part of everything that happens in their lives, almost weekly.  I am fortunate that those boys always want me to be included in the things they do.  Braeden, for one, texts me often to say hello, to invite me to an event, a trip, or activity, or to simply say “I love you.”

Braeden has always been an old soul who I have always admired for his courage, his perseverance, his positive attitude, and his gentle spirit.  I cannot imagine what he thinks about when he isn’t sharing his feelings with us.  What are his hopes, his fears, his dreams?  He feels deeply and is highly reflective.  He is inquisitive and seeks understanding.  With all that Braeden gives the world around him (and it is so much), the world owes him much more.  For those who do not personally know my nephew, he is one of the kindest young men one could meet.  He is loyal to his friends and family, and he has a heart as big as the moon. He is a man of integrity.  As Braeden ends his radiation treatments this week, I continue to pray that the doctors at Duke will find the answers they seek so this special young man, this loving boy, will once again find some “normalcy” in a life that was already rife with challenges for him, challenges that he was navigating well, but all challenges that I would take from him in an instant if I could.  He has struggled enough, but I know that if anyone can rise above them with an attitude that can teach us all a lesson in humility, kindness, and understanding, it is Braeden.

I felt compelled to share this perspective of Braeden.  Though I am in the arena with her, his mama is his BIGGEST advocate.  So, as we pray for Braeden, let’s make certain we include her, my beautiful sister.  She has the heart of a warrior, but inside I know she is scared.  Let’s pray that his strength, his feeling, his sensations, and his “normal” way of life returns.  Let’s pray for healing and a lifetime of happiness.  No one deserves it more than my nephew Braeden.  I love you, buddy.

We love you, Uncle Eric!  

#BraedenBrave

#MrUnlucky