Klaus Dragon’s Story

Site created on March 8, 2009

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. (as of June 2020 - mets are gone)  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. 
*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. 21 Multiple bilateral lung mets. 
*March 2011-7th surgery-dental reconstruction surgery--3 implants set with dental bridge on top.
*February 2020 - removal of lump on top of left foot.  Benign.  (regrew as of Dec. 2020)
*June 10th, 2020 - Chest CT - Amazing news -  No evidence of pulmonary metastatic disease.  N.E. D.  No evidence of Disease.  First time since 2010.  
*December 21st, 2020 - Final post on Caringbridge.  Unless there is some news to share in the future.  Grateful to share this final update.   

   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

Tonight is the winter Solstice.  Each new day will only bring  more light.

The year of 2020 will be the year we all remember because of Covid-19 and the global pandemic.  Who would have ever imagined 2020 would have unfolded the way it has?  So many surprises, ups and downs.  Instead of sharing the disappointments, I feel so blessed because we've had many silver linings this year.  So many times we all have felt numb because at the same time of our joy,  we all know so many around us have not been so fortunate.  Let's face it, it's been a very hard year.

Which takes me to the reason for this sharing this post.  On June 10th, 2020 - Klaus for the first time in over a decade heard the news "N.E.D." .  

On the CT report it states "NORMAL CT CHEST: No Evidence of Pulmonary Metastatic Disease". 

WOW,,,   The picture on the right was when Klaus was 3 1/2 years old and his first surgery.  Now Klaus is 17 years old and Plexiform Fibrohistiocytic Tumors cannot be found.  He used to have over 21 small nodules on his lungs.  They looked like popcorn in all 4 lung quadrants.  But now they are gone.  It's just amazing and I share this update in closing since many new families are just starting this journey in care for their loved ones and I know it can be so scary. May our sons silver lining share light with you to help navigate the care you need for your child.   In this year of the pandemic, it has been so busy and crazy,  that I had not taken a moment to share this important post.   But being the solstice, I feel inspired to share the light and close out this chapter. 

Klaus and our entire family has lived with a weighted burden on our shoulders for so many years.  I can't even imagine what it's felt like for Klaus?   So even in with midst of a global pandemic, how amazing is it that there is light to be found?  Since hearing the news in June, Klaus leads to show us how to adjust - working to shake off the past.  He's so inspiring.  It feels pretty incredible! WOW?  So unexpected - it's gone?!  We are grateful.

This year Klaus is a senior at Boulder High School and with this too,  he continues to adjust and find his way.  With only experiencing on-line learning for his senior year and the end of his junior year, this entire school year has been challenging to say the least.  The list of what has been "cancelled" in his senior year is long.  Blah.  Grandpa Ted also contacted Covid but thankfully only had a mild case.   There really has been so many bummers on so many fronts.. but none of this really matters in the big scheme of things because being NED tops all the cancelled parts and all the heart aches over the years.   Grandpa being well too tops so many parts!  It's all really good and we are grateful.  Klaus is moving on, figuring this crazy year out and taking small steps forward, step by step.  He applied to 4 colleges,   let's enjoy what comes next right?

So with that, it's time to close this out.  Caring bridge and my journaling has been a lifeline to process, to connect, to share strength and to find strength.  To share information for others to learn from in support of their loved ones.   Life is good, what are the most important things in life?  Your health, your community and your family.  If you have that, you have everything!  Our entire family is grateful.  Thank you all for being our soulful community in this journey.  Grateful to be on the other side.

  xooxxo  Tracy Heiman and Family

My social media post on June 12th, 2020:

"Introducing Mr. “N.E.D.” No evidence of disease! What began in 2007 is now officially in the past. So so so grateful. This is only the second time in over a decade +, that Klaus has heard this incredible news. A new chapter begins today! Thank you to all those who have supported Klaus and our family over all these years. What an incredible gift to receive today? We do not take any part of this for granted. #letthegoodtimesroll #sounexpected #blessings #childhoodcancerawareness #plexiformfibriohistiocyctictumors #pediatricsarcomaawareness #dragonstrong #community #gratefulheart #thisis17 #stbaldricksfoundation #therewithcare #shiningstarsfoundations #rockymountainpediatrichematologyoncology #lessthan4 #curesforkids #mskccpediatrics #rememberingallourdearangels #hope #rememberingthoseinthefight #foreverchanged #makeawishcolorado #bagsoffun #curesearch #Campwapiyapi #campskyhighhope #nighthawkranch #bravehoods #corporateangelnetwork #rockymountainhospitalforchildren

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