Klaus Dragon | CaringBridge

Klaus Dragon’s Story
Plexiform Fibrohistiocytic Tumors (PFHT) Metastic: A very rare soft tissue sarcoma of intermediate grade malignancy-under 175 cases are on record since 1988, less than 4% becoming aggressively metastic-moving to regional lymph nodes and pulmonary system.Sarcoma is a cancer of the connective tissue--including muscle, bone, fat, nerve, cartilage, blood vessel and deep skin tissue.  This specific disease is considered an "orphan cancer" because very little is know about it, and how best to treat it besides surgical treatments. Many times, orphan sarcoma's such as PFHT are misdiagnosed. 



Our story began in January 2007 when we found a pea size lump in Klaus' mouth-now he lives with multiple bi-lateral lung mets. We live life, hope and pray for a cure.
   Klaus' metastatic sarcoma was misdiagnosed for a very long time before proper surgical treatment was taken. A confusing aspect of this rare sarcoma is the fact that in pathology, there is an overlap of PFHT (malignant) with Cellular Neurothekeoma (benign). This key point was an area, which caused our original medical team to make safe calls instead of hard calls, which are needed in sarcoma management.
  As parents, we did not know that PFHT was considered a sarcoma until August 2008. We did not know the #1 goal for treatment & cure for rare sarcoma's such as PFHT is to obtain clear and wide surgical margins in the 1st surgery. If upon review of the surgical pathology-clear margins are not obtained-you MUST go back and obtain clear margins.  Klaus unfortunately did not have clear margins on surgeries #1 and #2 with our original medical team.  
    The location of the first disease was challenging to surgically treat, but we as parents, were never informed of PFHT being a sarcoma. We never knew there could be two choices in his care: 1) be aggressive & obtain clear margin, or 2) watch & wait.  The only medical advise shared with us was "not to worry-watch & wait-this is not something to be concerned with-the disease was encapsulated".  In retrospect, we know this type of sarcoma management is incorrect in regards to the diagnosis of PFHT--even with the confusion of the benign Cellular Neurothekeoma.  We want other families to learn from our journey and make informed choices to keep this disease from spreading as it has in our son.
  Because of the opportunities of the Internet, we have connected with 3 families around the globe with children diagnosed with PFHT. All 3 medical teams knew enough about this sarcoma to go back after the original surgery & re-excise & obtain clear/wide margins.  Klaus' original team unfortunately did not have this expertise or knowledge.
  During our 18 months under care of the original team, Klaus' body was not monitored with an MRI or CT of his head/neck or lungs to look for metastic spread. After 17 months and 2 surgeries, we finally met our 1st oncologist for an evaluation. 
MEDICAL TIME LINE:
*January 2007-pea size lump found in gum of lower left jaw
*May 2007-1st surgery removing lump-margins not clear, confusing pathology
*January 2008-reoccurence noted but watchful waiting advised--not surgery
*May 2008-Parent's decide to push hospital to remove lump and suspicious lymph node against advise of medical team. Pathology confusing again.  Disease confirmed in lymph node. Medical team advised no further treatment--margins not clear.
****At this point, we began to search for experts in sarcoma for help. Consulting with 30 doctors and institutions across the USA & world. Making sure we are not missing anything to cure this. Discussing surgery, radiation and chemo.
*August 2008-Klaus' care is moved to Rocky Mountain Pediatric Hematology Oncology at Presbyterian/St. Luke's in Denver, CO--Dr. Jennifer Clark. Tracking lymph nodes, oral structure and lungs.
*January 2009-Enlarged lymph node & new lump in mouth noted so together we consulted with multiple sarcoma institutes in USA.
*March 2009--3rd surgery--found 2 more tumors wrapping around critical facial nerves. Because of complexities, surgeon was unable to remove all disease.**We decide to seek care from a specialist in sarcoma & pediatrics--Memorial Sloan Kettering Cancer Center in NYC.
*May 19th, 2009--4th surgery--12 1/2 hours. Disease had spread throughout his jaw, eroding his jaw bone, going thru nerve channels. Surgeons removed the lower left mandible, two nerves, 6 teeth, facial muscles. Replaced the jaw & the tissue lost with his fibula, a complete neck dissection. Disease was tumors, large & small microscopic disease, lymphovascular (disease in the walls of the blood vessels to the lymph system) Finally, clear margins were reported but one small spot.
*December 2009-5th surgery to remove infected gum tissue & tooth-jeopardizing his jaw. Clear pathology.
*March 2010-6th surgery-a lung thoracotomy-disease confirmed in his lungs. Multiple bilateral lung mets. 
*March 2011-7th surgery-dental reconstruction surgery--3 implants set with dental bridge on top.

   We constantly review the risks,benefits of chemo, radiation, & surgeries for a cure. Unfortunately, these options have more toxic side effects compared to living with this chronic condition.

Newest Update

Journal entry by Tracy Heiman

It's been a year since our last update:

Monday, June 18th, 2018 - Klaus did it again - his lung mets continue to be stable!  There is nothing to be found in his head or neck.
 

As we traditionally share - there was a lot of hugs shared at the oncology office today.  We are SO grateful.

 

Klaus is now 6" 1" tall! 150 lbs, 15 years old and going into 10th grade next year!  He's getting to the end of his growth spurt and therefore the highest concern for growth of the lung mets.

Last year was a big leap of faith - moving out to yearly reviews consider the mets in his lungs.  Both our oncologist Dr. Clark and our Naturopathic doctor - Dr. Rao - are working to slowly cut back on his use of supplements.  He's down to 5 supplements now and it would be great to see if we could reduce the list even more without doing any harm to his strong immune system.  

Next appointment will be in one year unless some lumps or bumps show up unexpectedly.  

We are working on making him a new prosthetic dental bridge with a team here in Boulder since he cracked it a bit ago.

Below is a picture taken at the hospital today.   A non-profit called "BITE ME CANCER" shared a care bag with some gifts specific for teenagers. it was a very nice surprise. 

Last week Klaus was at Camp Wapiyapi and was a "JC" Junior Consular in training and tomorrow he heads off to Camp Courage.  Both are camps in Colorado for kids and their siblings dealing with childhood cancer.  We have been so grateful for the support of these amazing communities of volunteers and families who just get it.  At these camps our kiddo's are so grateful to have their own community of others who really understand their journey.  Each year Klaus gets farther away from his beginnings - the brighter the light he shares with others for hope during their darkness.

Other special news:
Big brother Calvin graduated from Boulder High School this year and will be attending Columbia University in the fall.  Studying Biochemistry.  At this time he is thinking of a premed track. In order to attend Columbia he's needed to apply for many scholarships in order to make the financial responsibilities possible.  He's been working so hard on all his applications.  One of the scholarships he applied to was the North Western Mutual Siblings of Childhood Cancer Scholarship.  It made us all so proud when they provided him with a 2 year scholarship!  We are so grateful for the generosity of this special community to help him along and support his dreams.  As Calvin has filled out all his school and scholarship applications - I've had the opportunity to read his words, and get a deeper understanding of how this experience as a sibling has impacted him so profoundly.   He has taken the darkness and molded those experiences into something that motivates and inspires himself and others.  This year he also organized at Boulder High School the 1st Annual St. Baldrick's Head Shaving Event.  In less then 6 weeks - he motivated his close friends and over 100 kids to participate, volunteer and shave their heads for awareness.  Together this community raised over $45,000 for childhood cancer research.  Imagine what each of these kiddo's will do in the future for the cause of their beliefs?  

We could not be more proud or grateful for both Klaus and Calvin and how they use this experience in a positive way for others.  Each time I hear our news, I feel grateful.   But at the same time the darkness is present too.  With a heavy heart, today as Klaus and I were walking back to the office from his CT and MRI - in front of us was a police escort walking a man down the hallway with a tiny blue gingham coffin in his arms.  I can't but imagine the hardship others are feeling the same time we are feeling our joy and relief.  That is why we will never drift away from this community - it is always part of our lives - the good and the dark.  There is always a way we can contribute to make a difference for others,   our chosen path is helping others and helping to inspire funding for research and cures.  

THANK YOU ALL for all the support over the years for our family and also all the donations for all the walks, climbs, head shavings we have participated in.  Together we will continue to make a difference.

With a grateful hearts,

 
xoox Tracy, Timothy, Calvin and of course - Klaus Dragon Heiman

 

 





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