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. 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

Dear family, friends and community,

Just a quick update from our adventures yesterday:
(All good news!😃 )

  • 6:00 AM wake up and put Emla cream on both arms
  • 6:30 AM head to Denver in HOV lane
  • 7:20 AM check in - pay deductible
  • 7:30 AM head to the pediatric infusion center to connect with our favorite pediatric nurses who still remember Klaus and easily insert his IV for contrast.  1 poke!  YES!  This time Klaus even watched and was SO relaxed about it.  The growth emotionally for Klaus year after year is really something to appreciate.  So proud of this guy!
  • 7:45 AM head to adult radiology and check in.  Fill out sheets and sheets of registration forms and work hard to remember "all previous surgeries and procedures".  Klaus helps to fill out the paperwork.  (Learning the ropes.)
  • 8:00 AM Klaus heads back on his own for a low dose lung CT
  • 8:30 AM Technician advises Klaus' braces will be a problem and will cause image issues.  Advised to postpone the test.  Removed his IV.  
  • 8:45 AM  Tracy calls our orthodontist in Boulder and they agree to remove his braces so we can get a good test result with his head/neck MRI.  Lets' get this done!
  • 9:00 AM Head back in the FAST lane to Boulder
  • 9:40 AM Orthodontist team removes his braces - at the same time - Tim arrives with our tube of Emla cream, tape and saran wrap to apply Emla on other arm for a 2nd IV if we can get the MRI rescheduled for same day. Tim also helps and puts gas in the car so we can zoom back to Denver
  • 10:00 AM Head back to Denver in the HOV lane again
  • 10:45 AM Make it to our oncologists office --- fill out sheets of paper work again! Klaus helps again.
  • 11:00 AM Meet with Dr. Clark for a physical and to look for lumps, bumps and annual check u[. She shares the initial lung CT looks good but wants to check the images out herself and will call back.
  •  12:00 PM They are able to re-book the MRI.  We rush back to the adult side of the hospital -- no - wrong advise - head back to the kids side for the MRI.
  • 12:30 PM - check in for MRI at kids side of the hospital - need to get the  IV done again---choice for Klaus, go back to the infusion center or give the folks in MRI a chance at a good poke?  Best part  - is that the Pediatric MRI team has been part of Klaus' imaging team for YEARS and they all really love him.  So they can do the IV if he trusts them?  And showing his maturity again - Klaus agrees - goes back alone, gets the IV inserted, no issues and then has the MRI all by himself. 
  • Klaus comes out at 2:00 pm.  Rested, relaxed and DONE FOR THE DAY!
  • 3:00 PM head back to Boulder and eat out for lunch to celebrate at Panda Express!  
  • Our day is done!  WHEW!  

Today Dr. Clark called and shared test results:

LUNGS METS:  STABLE OR REDUCED - LOOKS LIKE A FEW OF THE LARGER LUNG METS MAY HAVE REDUCED IN SIZE!  WOW!!!

HEAD/NECK/THYROID:  NO NEW DISEASE!

Next appointment will be in a year unless any problems come up earlier!  SO SO SO GRATEFUL!

Updates:

2019 BHS St. Baldricks:

As you all know the St. Baldrick's Foundation is near and dear to our hearts - this year Boulder High School raised a total of $47,000 for research. WOW.  Check out the pic's of Klaus and his buddies shaving along side him.

Thian, Nik, Seb, Spencer. THANK YOU! These friends have been shaving and supporting Klaus for years during his cancer journey --we are so grateful for his amazing friends who support Klaus during the ups and downs of cancer - just being supportive friends who just "get it, and get Klaus".

BHS MTB Team:
The BHS MTB team together raised over $16,000 in honor of Klaus and his team mate Martha!  Pretty amazing right? 

I am also sharing with you the cb site of MTB teammate Martha.  She spoke and shared an inspiring message at our event- sharing the importance of the event, the benefits of research and explaining a bit of the life of a teen dealing with cancer and it's impact over the years.  Please send Martha and her family strength, love, prayers, and any support you can offer - it's just NOT okay that this soon to be senior in high school is now dealing with her 3rd relapse of Ewings Sarcoma.  https://www.caringbridge.org/visit/teammartha13

We honestly cannot thank you enough for your continued support of our fundraising efforts year after year...... more treatment options will become a reality....

Martha is one of the many reasons WHY we are SO grateful for the continued support.  For Klaus and all our friends and angels in the childhood cancer community.  So many children and their families appreciate your help. THANK YOU.

Remembering Jules and TEAM JULES:
Another very special team at the Boulder High Event was Team Jules.  Just over 16 weeks ago - dear Jules - sadly died from her disease. It's just not okay.  At the BHS event - members of her community gathered to share a sincere message of Jules journey, her struggles, her hopes, her aspirations, the complications and the impact her disease took on her body and mind.  3 compassionate team members chose to honor Jules and shave their head in her honor and memory. Together team Jules raised over $8,000.  https://www.caringbridge.org/visit/juleslaurita

How amazing are these kids with such incredible hearts? You will always in our hearts precious Jules and family - words just cannot properly express our sadness with your passing....


How strange to transition to this?
NEW PROSTHETIC TEETH:
Attached is a picture of Klaus and his 5th set of teeth?  I think that's right 5th set?  Looking good right?  These should last him a while.  Of course, we were very excited to have pre- authorization and confirmation insurance would cover the expenses. (these teeth cost more than our used cars! and took over 6 months of fittings and calibration)  But unfortunately, we have some kind of hangup and are going through insurance dispute proceedings... UGG... 

Not fun, but GRATEFUL his new teeth fit, look, work so much better than his older teeth.  We'll figure it out as we always do - but again this sheds light on so many other families with MULTIPLE insurance issues they are having challenges with... I feel for all these families...Thank you Dr. Gordon Gates for your work.

Whats on the net?  PFHT: 
Just yesterday as I was sitting and waiting during Klaus' tests- I googled "Plexiform Fibrohistiocyctic Tumors" and I was VERY happy to see a few more updated websites, research papers and clear information on this disease.  Grateful the sarcoma medical community has responded to the lack of information shared and has stepped up to be more collaborative with their information.  

Some of the new information found shares simple and clear advise on treatment options.

Imagine when we started this intensive journey back in 2009 - clear information WAS NOT available.  There was no mention of it being a sarcoma (cancer of the connective tissues). 
 
Yes, each case is a one of a kind, but in general - complete surgical removal with wide margins should do the trick and then diligent follow up is needed to track and respond to any spread as occured in Klaus' case. Klaus and his experience can help guide others so they can make better choices to prevent disease progress.  

I hope by telling Klaus' story - we can share some relief that even if disease progresses like Klaus' case - there is a positive light at the end of the tunnel.  We've had a LOT of worry  (the worst of worries) over the years and that never goes away. But it's the constant reality of the gift Klaus and our family has to celebrate.  We are gratefully invited to move forward. But why do others not have the same gift?  Why do they suffer, why are they lost?  It's just not okay.

We will always be members of childhood cancer community..and one day we look forward to the choice that further tests and monitoring will not be needed.   Klaus too will ring the symbolic bell in the waiting room that "treatment is over" and we are symbolically moving on from cancer. 

But for now and always, we will do our best to continue to support our friends and loved one in the trenches, our friends still fighting for their lives.  Saying prayers and always remembering our angels friends above,,,,

We are grateful,,,    

With Love, Klaus Dragon Heiman, Tracy, Timothy and Calvin

Remember - no news is good news.... xoxooxoxo
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