Rare Disease Day

Today was Rare Disease Day (a day in which the world wide community recognizes all those living with rare diseases). I have been living with Erdheim Chester Disease for 8 months now and honestly I wish I could just forget it all and be "normal". No shots, No Pills, No dietary issues, Just normal. 

I feel like ECD has infiltrated every moment of my life. Tonight we went out to eat and I ordered a sandwich, dry (just chicken breast and bread). The guy got all confused, so DawnMarie said, "he can't eat pesto or dairy, Allergies". This led to the waiter warning me about cross contamination! Honestly...I don't even want to eat a lame dry chicken sandwich let alone have drama while ordering it. 

I think the reason I am feeling so aggravated is because I know there are a lot of doctors visits and tests ahead (to nail down the fat wasting issues) and I just don't feel like doing any of it. I need a break!

Don't worry about me. I am sure this funk will pass.

If you want to help just pray for sunshine, we could all use a little more light in out lives right about now.

Drained

I have over the last few months found the natural balance of work, family, and stress that I can handle on any given day. If I am respectful of that balance I  feel well, maintain my weight, and stay mentally grounded. 

Occasionally there will be a day or two that spikes above the norm, but I seem to have enough reserves to handle it. 

The last week though...it has been tough. 

One of the problems with looking healthy (which I do) is that people forget that I have serious health problems that cause me to have significant limitations. 

For the past few days I have dealt with appointments, phone calls, and personal stressors that are completely draining me. 

Feeling tired, nauseous, emotionally wiped out, and my weight is decreasing again (now below 146). 

ARB

I just got done meeting with the architectural review board tonight and they approved our addition (extended kitchen, family room, bathroom, 2 bedrooms). Now we just need final approval from the building department and we can get started. 

Last year (November) I asked a contractor that we found on Angie's List to come by and take a look at the house before we purchased it. After some calls to the building department and some input from their favorite architect (Bernie Rodgers) we decided to risk the purchase and see if we couldn't turn this 1913 fixer upper with charm into our dream home. 

It took a few months to hammer out our final design....then I got sick.

At which point having a "dream home" stopped being important. 

Yet things kept creeping along. Interviewing Contractors and Architects. Initial Arcitectural Plans. Revised Plans. Integrating building department suggestions. Zoning Waivers. Etc. 

Now here we are a year later and it's about time to turn it all over to the contractor. 

Through this process I have learned to let go and patiently wait for things to happen in their own time. Now I just need to have that same patience with my medical ticks (non life threatening issues that need to be resolved).

You would think that it would be easy to turn it all over to God, but it's not. 

Funk

My parents were at my house for the last few weeks helping out and visiting their grandchildren, but have recently returned home. It is amazing how helpful it is to have a few more adults around the house lending a helping hand. They really helped keep things calm and relaxed. 

I have noticed though that since they left my stress level is building. There are a lot of things going on right now and I am finding it difficult to manage them all. From problems with tax forms to attitude from some of the adults I interact with on a daily basis it's starting to get to me. 

Meanwhile I am hoping to hear back from a local immunologist and my gastroenterologist soon. Then perhaps we will be able to plan out the next few steps. Funny thing is the timetable is always much more urgent for me then it is for anyone working with me. So much for patient centered healthcare. 

For know ill just keep on smiling and hope the funk passes. 

Wisdom

The more I learn, the less I know. 

Processing it All

On Friday I wrote...

"The samples taken at the NIH show malabsorption of fat. This is very big news because it proves that I am not crazy. Up until this point I have gotten the distinct impression that every doctor thought I was just not eating enough and that was why I am not gaining weight. This test shows that is am wasting fat and indicates that either I have a problem with my pancreas or with my bowels." 

This has been a gigantic revelation and the funny thing is I have had literally no time to process it, no time to talk with my wife about it, and little time to plan for next steps.

I believe that Dr. Scheel from John Hopkins will be coordinating with my Primary Care Physician (who just happens to be a gastroenterologist as well) to determine next steps.  This condition is called Streatorrhea and can be caused by malabsorption, pancreatic insufficiency, pancreatitis, etc. Here are the symptoms: pale stools, weight loss, fatigue, flatulence, anemia, B-12 deficiency, bone pain, vitamin malabsorption, protein malabsorption, general motor weakness.

WOW! It is amazing how many of these symptoms I have been dealing with.

I have had a theory for the last 6 months that my wife has had to hear like a thousand times. It goes like this..

"The only reason I am not experiencing the pain I felt when this all started is because of my diet. In fact I believe that be eating super healthy I have masked the problems I have been experiencing from the doctors. Essentially by not taxing my system with fatty foods I am treating myself. The problem is it makes it difficult for them to spot whats wrong."

The perfect example of this theory is the fecal fat test. It is supposed to be done on someone eating 100 grams of fat per day over three days (I rarely eat more then 20 grams of fat on any given day). Yet my result is still 3 times normal rage.

I am feeling confident that while this new test will cause me to undergo many more tests (DawnMarie has been trying to get them to do a colonoscopy. That's mean right!) it will also help solve the most persistent and annoying problem related with my disease so far, my weight and digestive pain issues.

Keeping my fingers crossed and praying that the coming months provide treatable answers.

"Vacation"

I have been on "vacation" this week, by this I mean I have had the week off from teaching. However, it has not been a vacation in any sense of the word.

I have traveled from Baltimore, MD to Monroe, CT to visit doctors. I have officiated at a in home prayer service, two funerals, and a cremation. I was one of the first people to welcome a new infant member of our church family. It has been exhausting. All the driving and the time it took to prepare four sermons has been draining.

The miles were made easier because DawnMarie was always with me. The time spent on sermon preparation is balanced some by the joy of serving this lovely group of people. Although I must admit that I do often feel guilty about not spending more time with my family.

Today as I traveled from the cremation service of a wonderful man who had exceeded 80 years on this earth to visit a newborn baby I was struck by just how quickly life passes.

I can still remember thinking as a child how quickly each summer seemed to fly past as compared to the one before. Now I am 37 and the relentless passing of time seems to just continue to accelerate. 

Life is short, Life is a gift, Life must be lived at every moment to the fullest. I know I am. Are you?

Call Worthy

I got a call tonight from both my primary care physician and from my doctor at JHU.

Primary Care Physicial - Turns out that I have IGA deficiency (since birth) and that my platelet count  is very low. He suggested that I see a immunologist, so we are setting that up now. By the way...if you are related to me IGA deficiency is hereditary and is often associated with having other autoimmune diseases (so you may want to have the blood test run the next time your at the doctors office).

John Hopkins Physician - The samples taken at the NIH show malabsorption of fat. This is very big news because it proves that I am not crazy. Up until this point I have gotten the distinct impression that every doctor thought I was just not eating enough and that was why I am not gaining weight. This test shows that is am wasting fat and indicates that either I have a problem with my pancreas or with my bowels.

For most people this would be bad news, but I am thankful for the little we have learned and I hope with my doctors help we can build on this new found knowledge.

Good Night

Went to the third night of the wake being held for a parishioner from my church tonight. Every person who cam into the house would go around greeting everyone with, "good night". Generally it is customary to greet someone with "good evening" and then say "good night" when you are departing. This was a well educated group, which leads me to believe this grammatical switch was being done on purpose.

It is probably just tradition. However, last night at the wake it seemed sublimely appropriate. It was almost as if each visitor was inviting the next to enjoy the celebration of life we were all present to participate in. Each person was giving the next person to arrive permission to enjoy their grief, enjoy the company, and enjoy the gathering of God's people.

I am sure I am over thinking this, but that's okay because it helped me have a good evening.  Good Night.

Knowledge and Power

Rarely in my life have I been the smartest person in the room and I am fine with this. I have come to see in the past decade that there is much to be gained by surrounding oneself with intelligent folk.Which makes me think that there is power of some sort to be found in knowledge.

Foucault (a french philosopher that I have never understood but is fun to quote) says that power and knowledge are not independent entities but are related—knowledge is always an exercise of power and power always a function of knowledge.

How then does knowledge give one power in the management of ones health? I have surrounded myself with some very smart doctors (A great team at the NIH. A world class doctor experienced with ECD at JHU. A general practitioner that loves exploration and enjoys a challenge. A Natural Doctor that has filled in the gaps). I feel better knowing I have these people on my team.

There is one problem though. At some point (particularly when dealing with our health) we reach the edge of human understanding and knowledge. That edge is often far closer to us then the destination we desire. I desperately want all my questions answered and yet the truth seems to be that much of what I desire to know has no answer.

Perhaps just perhaps there exists something even more powerful then knowledge, the ability to accept that which we cannot change.

Guyanese Mourning

I spent most of today with a lovely Guyanese family as they prepare for the funeral of a devoted husband and father. Their tradition of mourning is different from what I am used to. One thing I have learned in the past few years is that it is best to explain one's ignorance instead of trying to, "fake it til you make it"

So after explaining this morning that I didn't know what to expect here is what I have learned. The family gathers every night from the time of death until the funeral for a informal service of remembrance and music. In this case that means a weeks worth of gathering (it reminds me a sitting shiva).

When I went tonight people who had been prepared to speak immediately deferred to me and asked when I would like to start the service (I explained I was just there to watch and learn tonight). A very traditional group of people with a level of respect for the position of Pastor that is rare now a days.

Here is what is expected of me. I will be leading the gathering on Thursday at the home, Funeral on Friday, 2nd Funeral on Saturday. Then I will travel to the crematorium where the family will be present for the cremation. This is a first for me. Then we will all gather together for food at a local church.

I love this family very much and I am sad that they have lost their father/husband. As a Pastor I am incredibly proud of this families faithfulness and adherence to a tradition that puts their faith at the center of this life event. I only hope that I can properly pay homage to this wonderful man and his big faith in the coming days.

My Day

Good, off to Baltimore by 3 pm on Sunday. Three and a half hour drive to the LaQuinta outside Baltimore proper. Enjoying the silence. Holding my wife. Quiet nights sleep. Wake up 6 am. Normal breakfast (2 eggs, Toast, Applesauce). Nervous waiting. Coffee and window shopping to kill time. Buy a book to keep my mind busy. Nice lunch with DawnMarie. Drive to appointment but get there to early. Wait. Talking, Questions, Waiting, no answers. Drive home (5 hours). Long day. Tired. Sleep.

I Live In NY

I live in New York, but I am certainly not from here. New York has all four seasons, with Summers that are hot and sweaty and Winters that are packed with snow. I come from a wonderful place with only three seasons (Fall, Spring, and Warm Spring). Having all four seasons is overrated!

I am only 37 years old but I can't imagine shoveling and stacking the snow like we have been doing the last few weeks. This morning I went out and shoveled the snow so that people could get into church, half way through I started just cutting a small path to walk on. I have been making a real effort to not over tax my system because it seems to wipe me out for a few days when I do.

To all my family in Arizona...smart, very smart!

Tonight I head down to John Hopkins for my 6 month followup with Dr. Scheel. I am praying that the Kineret has been working well and that I won't have to switch my medication to the much more invasive Interferon Alpha. I'm sure it will all go well. God Bless you all and stay warm.