ECD Global Alliance Gathering 2015 - Patients Speak

I'm the first Patient speaker on the panel. Check out the video at the link below.



https://youtu.be/YVEGXpIhcU4

Lingchi

Someone asked me recently through the Blog how I was doing, so here's the answer.

Over the last year I have gained back much of the weight I lost when I first got sick. Which is good from a health system perspective but not good news for my pants. I am still limiting my fat intake and not eating pork or beef (ever). I've also not had soda since I first got sick. Who knows if these things are making a difference or not, but I haven't had pancreatitis again so I think I'll stick with whats working.

In the last year the ECD has spread from my abdomen to my testicles (in a related form of the disease called Rosai Dorfman), completely destroying all natural testosterone production. To address this issue I am injecting testosterone every six days with the nastiest looking 2 inch needle I have every seen. The thing is if I don't take the shot I'm tired all the time and insane/moody.

In the last few months I have had to have a growth of ECD removed from my tongue and the most recent MRI showed that the disease is now present in my nasal cavity.

What does it mean? That's the scary part. Who knows. Its like every new piece of information is another cut. It's like Lingchi, the concept that a thousand cuts is a much more horrific way to die then by a single catastrophic event.

Every day I wake up I wonder when the next cut is coming. Now I know I'm supposed to stay positive and if you ask I'll tell you I'm doing just fine...but the truth is the cuts...they hurt.

I Have Cancer - Thank YOU Lord!

One of the things I have struggled with the most over the last two years since being diagnosed with ECD is what to call this horrible disease. The rarity of the disease and the general lack of consensus on what exactly the disease is has left me trying to explain to people what I have while having no generally agreed upon term to use.

Then this morning I read this...

Please visits the ECD Global Alliance for more information.

Did you read that!!!, "to classify ECD as a cancer".  Tears stream down my face as I write this...I now know more about my tormentor then I did just 24 hours ago. I also realize how desperately I needed a name for this disease that I will live with...until the day that it takes me from this earth.

Survivabilty Numbers Soar!

Last year in San Diego patients, families, doctors, and other health care providers gathered for the first ever ECD Medical Symposium. One result of this gathering was a consensus paper (written by experts in the field) on ECD. I have included the first and last page of the paper here. Send me a message if you want the whole thing.

 While there is no "cure" in sight the good news is that the five year survival numbers have improved. Plus with a standard now set for treatment and diagnosis other will be able to receive an effective treatment from inexperienced clinicians (inexperienced with ECD) in a more timely manner.

Remember, every day is a gift. So live your life to the fullest.

Super Random Update

The field of medicine for all its advances sure hasn’t made it any easier for us patients to understand what doctors and the labs they use are talking about.

For example I have started to get some test results back from a series of labs I did while at Winthrop Hospital's Hematology and Immunology practice. I don't understand them...but I've gotten them back. We are looking at how my body responds to attacks on the immune system. It clear that I will need to schedule an appointment with the doctor to get the results explained. Sure they send me the results but it’s all written as high and lows with no explanation to what those highs and lows mean.

One advantage to having a rare disease is that until now I could always count one getting quick access to medical professionals. I used to be able to call up and pop in the next day. Unfortunately, the novelty seems to be wearing off and I am going to have to spend some more time waiting in doctor’s offices in the future.

Oh. I had my appointment with Dr. Diamond at MSKCC last week and Dr. Diamond told me I was probably the healthiest ECD patient he had ever seen! I feel like there should be a prize for this fleeting distinction. We did some tests and special stains that I will get the results from as they come in.

After the appointment I had to get blood drawn and I had to wait a long time....almost had a yelling melt down. Luckily DawnMarie was there...she calms me.

Dr. Diamond sent me this email today.

Brad, I meant to write you about your PET scan, this is important. It’s much improved from your prior one. Your initial PET from 2013 showed avid lesions in the abdomen (pancreas and around the aorta), as well as the right tibia. These are all essentially resolved on the current PET.

Now I think this means that what were once active histocyte cells (caused by Erdheim Chester Disease) are now just dead "scar tissue" which is why they showed no uptake on the PET Scan. This is good news. I think.

I continue to work on my nerves/anger/emotional outbursts. I'm not sure what causes me to flip out but I suspect it’s related to the disease process, or perhaps I'm just being a petulant child.

I am in Pittsburgh for the next two weeks by myself (I am starting graduate classes). I haven't been alone for more then a few hours since I first started to get sick....so wish me the best. 

I Am Alive!

I haven't been posting recently about my ECD journey because I am looking for a new teaching job (don't worry I still have my current position I'm just looking for a new challenge). I have been worried that a prospective employer will read my Blog and decide I have way to many issues to take a risk on.

The thing is...I miss talking to you all and the truth is that I don't want to work for someone who would refuse to hire an excellent (though often grammatically challenged) math teacher because they have a rare disease.

So here is my health update. I have been prescribed a drug called Creon (a pancreatic enzyme) to help address my weight loss. I am not taking it! I know. I know. What do I think I am a doctor? The thing is I don't really want to be on a bunch of different medications because who knows what the long-term consequences of taking them for the rest of my life will be. I am hoping that with the right balance of diet and herbal supplements I will be able to hold my current weight. [My weight been stable for 6 weeks.]

I am still taking my daily shot of Kineret. It is annoying to have to tote the shots around with me on vacation and even more annoying to speak with express scripts every 30 days to reorder my prescription, but other then that the shots have become a regular part of my life. One weird side effect is excessive bleeding from the injection site; this seems to occur more often if I leave the shot out at room temperature for more then thirty minutes. I would ask if anyone else was experiencing this but the fact is there are probably only a few dozen of us on the drug…so what’s the point.

When I was at The National Institute of Health Dr. Estrada-Veras suggested I see a hematologist and an immunologist. I got that done yesterday. They suggested I get a blood test to look at my T cells (T cells or T lymphocytes are a type of lymphocyte (itself a type of white blood cell) that play a central role in cell-mediated immunity). Thank God for Wikipedia right! This blood sample is taken and then flown to California for testing, apparently it has to arrive within 24 hours or the test cannot be preformed. So…it is actually my fault that health care costs will be increasing next year.

One last thing. I am going to see Dr. Diamond at Memorial Sloan Kettering next week (He is reviewing all my records and testing to see if I qualify for a drug trial he is running). I’m nervous. You see…I desperately desire to find peace with my diagnosis. Yet so much about my future with ECD feels unresolved and if Dr. Diamond doesn’t figure it out next week it will stay that way.

Not feeling very brave right now.

Teaching and Stress

I rarely write about my work as a teacher in much detail because I know that many of my co-workers and perhaps my boss read this Blog. My dad (a expert on old sayings) once told me you don't s**t where you eat and so with that in mind I have generally avoided the subject of teaching. However, as the year comes to an end I want to recap a few things and share some stress with the Blog so that I can let it go.

In June of last year I went to NYU for surgery, my boss and co-workers were incredibly supportive. I came back to teaching in September with my own room (my mobility was very limited at the time) and teaching a new subject (Geometry).

I would say it took me a good four months to really get going again. I have found the stress related to teaching this year to be incredibly high. First, I was teaching a new subject. Second, the adjustments needed to meet Danielson requirements took lots of time. Third, I found my self caught up in a cycle of negativity among the staff that really quashed my normally happy spirits.

As the year comes to an end I am still trying to figure out how to deal with the moodiness caused by my low testosterone levels. The thing is it has never been a problem in my classroom but the energy needed to keep it at bay all day has left me with little reserves to deal with workplace drama. This is an issue I haven't really brought up at work but nonetheless they have experienced it.

The funny thing is I feel bad that I have let my boss down and that I haven't been the leader I wanted to be this year, but I'm not sure (given the circumstances) I was capable of anything more this year.


Healing from an injury takes time. Healing from a broken life takes longer. My life was broken in 2013 and its only now that I'm beginning to see what the new Brad will look like.
 


 

So Much

I have been feeling stuck recently when it comes to my health. You see for all intents and purposes I have arrived at a stalemate in my battle with Erdheim Chester Disease.

The thing is it may not really be a permanent stalemate so I have to go get a mri or ct scan or fb scan once every 6 months.  And so now I survive from one 6th month period to the next each time praying that I'll be granted another 6 months until my next exam. 

Meanwhile I am still trying to finish seeing all the doctors that the NIH suggested. 
1. I saw the gastroenterologist who grave me a drug called Creon that was supposed to cure my Pancreatic problems but instead has just caused unnecessary side effects. It was like 8 pills a day (for the rest of my life ) and I just decided I wasn't willing to go down that road. So I am trying to maintain my weight more naturally but I just don't have the appetite except for when it comes to frozen yogurt. 
2. I have an appointment to see an immunologist in June, but I don't trust he'll see my health issues as anything more then a long term consequence of ECD that I am just gong to have to learn to accept. 
3. After that I just have to schedule an appointment with a hematologist.
4. I have an appointment at MSKCC in July (testing me for the B-raf mutation while reviewing my biopsy slides and then having a whole set of scans done) for tests that if confirmed negative will mean I am not eligible to participate in a study at MSKCC that is "curing" people with ECD and the B-raf mutation. Thus ending my hope for a cure (at least currently).

I feel Guilty.
I feel like I should be happy with how well I'm doing but I feel like I'm really just pretending like everything is okay while we all wait for my next health disaster to occur.

This along with all the elements of my real life (as it existed prior to ECD) that draw my time, attention, and energy have left me unable to express my thoughts clearly about how I feel. Tired, Energetic, Blessed, Scared, Tense, Angry, Gracious, and Burnt Out.

My future seems great, it just doesn't feel that way yet. 

Name

A baby Alpaca is called a Cria, A baby Badger is called a Kit, A baby Grasshopper is a Nymph, and a Baby Ox is called a Stot. What does one call these ridiculously cute guys? Platypus. Yup, that’s is. There is no name for the baby version of the Platypus.

 

They do exist though. They certainly are capable of causing one to feel happy (heck I want to adopt one). If we saw one being abused or killed we would respond with alarm and disgust. Just because something doesn’t have an officially designated name does not mean it is not important, that it does not exist, or that you are crazy for thinking about it.

Every day people all over this world are diagnosed with diseases that have no category in which they can be placed. Erdheim Chester Disease for instance is not defined as cancer or as an autoimmune disorder (even though it carries characteristics of both). So when people ask us what we have we say ECD…but its hard to describe after that.

Names help us categorize things. Names help us group things. Names help us figure out where we belong. I pray every day that soon ECD and all other un slated rare diseases will be given the power of a name. Not just the name of the disease but the name of the family in which their disease belongs, because when one has a family one has understanding, direction, and support.

Well Enough

I am alive and if being alive is the standard by which we judge our life then I am doing well. 

The thing is I meet people every day who are alive but their lives are not going well. Many of them are isolated and rarely receive visitors while others spend large portions of their day creating drama and fomenting chaos. If that were not bad enough far to many people I know spend all of their time running from one appointment to the next never really enjoying their lives at all. 

They are alive but they are not doing well.   They survive each day but they never really live! 

I want to live boldly!!!

I don't know about you but I have grand dreams for my life. I want to excel at everything I do. I want to be the worlds best husband. I want to get my doctoral degree. I want to be the type of father that raises children who will inspire others. I want to lead my congregation in faith and follow the Lord where He leads me. 

If I could do all these things...it would be amazing. 

But to tell you the truth I would be happy to have just one more day...

...to hug my kids...

...to hold my wife...

...to enjoy the flowers and breath deeply. 

You see one thing I have learned this year is that none of us know how long we are for this earth, so with that in mind I will do my best to squeeze every drop of life out of the time I have and I think that will end up being well enough. 

Spring

Spring brings with it pure sunshine and nourishing rain that will soon turn our neighborhood into a urban oasis. I look forward to the end of dull gray days that end as cold as they started. 

I am also looking forward to my last visit with a new doctor (at least new to me), in June. 

I still have an appointment with Dr. Eli Diamond at Memorial Sloan-Kettering (which will take 2-3 days) and Dr. Carsons at Winthrop University Hospital in June, but there seems to be an end in sight to the constant list of appointments. It's not so much that I mind going to the doctor it's just a lot of work to see a new doctor.

I look forward to someday soon settling into a predictable pattern of check ups with doctors who know me well. Where the hardest question to answer will be, "have there been any changes to your insurance?", or "how have you been feeling?"

Instead of, "we need you to get us the slides that were made of your ECD biopsies", or "could you bring a copy of all the labs you've had done in the last year?", or "we need your families medical history for the last five generations".

These last few visits will hopefully end my time of discovery and help me transition into an era of stability and stagnation (the best I believe I can hope for with ECD at this point). 

We shall see. 

"If God is good and all powerful...how come there is so much evil in the world?"

Ever since I was first diagnosed with ECD I have struggled with how to respond when people tell me that God is in control and it will all work out, because i know they think this means I will get better. I know God is in control, but I don't take that to mean that I will ever be cured of this disease (or that there will even be a tomorrow to wake up to). 

I am okay with this uncertainty, yet i understand for many the notion that God would allow disease, suffering, and death to happen (particularly to those that serve him) is a hard thing to understand. Personally this uncertainty has always given me comfort because it proves to me that God is much mightier and more complex then I can ever, then I should ever hope to understand. For others though this uncertainty causes confusion and pain.

Today I read a wonderful blog by a Pastor I know that shares some thoughts on the question, "If God is good and all powerful...how come there is so much evil in the world?". I suggest you check it out at the link below.
 
Blog of Pastor Rhyan Smith

Who Am I

I just got done yelling at the cashier at Stop and Shop. His crime?!?! 

This guy just stood there staring at me as I bagged all the groceries. He also ran other food up on my eggs and bread, poor form for sure but hardly worth causing a scene over. Feeling very embarrassed plus apologizing ten seconds after yelling makes you look even more crazy.

I have got to find a doctor who can help me with my low Testosterone! I find myself saying things, responding to others, and generally acting like someone who is not me. I have noticed that exhaustion and stress make it worse so....good night. I have to get my sanity sleep.