Just when you thought the appointments were letting up.

Sorry for the late update. Thank you all so much for your love and support!

Friday we went to see the pulmonologist and he showed us Eric's X-ray we took Tuesday in comparison to the one he had in February during his last Still's Disease flare. You could see a small hazy white patch in the bottom of his right lung - it appears to be fluid-like. We could most likely attribute this to allergies or something he picked up. The symptoms started a few days before we left for NY (which was awesome and we have an update coming this week for that at well).

One theory causing the mild chest pains during large breaths is that Eric may have neuropathy up in his chest as well. We know he has some scarring on his lungs from the extended time he was on ventilator support and has a chest tube when his left lung collapsed, as well as the ECMO lines being stitched to his body. We're going to follow up on this at a later date and Eric will begin the recommended dose of his Neurontin again to see if this helps with his chest.

We follow up with rheumatology next week to talk more about the deterioration in his shoulders and what type of shots they will give him. We're hopeful we can reduce the Medrol/Prednisone at that time. We got his labs back from last week and they were ok for the most part, but his CRP went from 2 to 16! And the Ferritin went from the high 600s to 1300+! We'll have labs done again soon for comparison and hope this was a lab error:/ 

In September, Eric is scheduled to get a sleep study again if we can get the insurance to cover it- a CT scan of his lungs and abdomen which will show us his liver and spleen and we can compare from his last CT scan to get an idea how things are healing. He'll also do some exercises and have a blood has exchange draw to see how well he breaths while doing physical activities. For now he is on a 5lb weight lift restriction until his lungs heal up. We also have a follow up with Dr.Essex from hematology in September. Actually the last 3- CT scan, blood gas exchange and hematology are all on the SAME day... Unfortunately, it's what must be done when you're short on leave and you travel 40 minutes to see good doctors :)

Just when we thought the doctors appointments were letting up...

1 week till Eric's 29th Birthday! 

Impromptu X-Ray

Eric has been feeling a little better or about the same over the last 6 weeks since our last rheumatology appointment. The days are very tiring for him and nights are often uncomfortable. Shoulder and elbow pains are persistent on a regular basis. He has taken an injectable form of methotrexate over the past 6 weeks as well. It's a higher dose than allowed by pill and he takes it once a week.

Today we have a new fellow rheumatologist and Dr.Shookory who spacialises in Adult MAS as well. Dr.F has left Temple and went to north jersey. He will be missed. The new fellow doctor seems nice and knowledgeable about Still's and how it presents. Lately, Eric has had some headaches and minor chest pains. The headaches appear to be attributed to his sinuses and most likely allergies . He'll continue to be on allergy meds and to try using some Flonase again to see if that helps reduce the headache. As far as the chest pains, the docs advise a chest X-ray just to be safe given the recent past. We have a follow up with Dr.Desai Friday late afternoon and we will be attempting to get a sleep study scheduled again. We had a failed attempt due to the run around the insurance face us last fall.. We follow up with rheumatology in 2 weeks and hope to reduce his Medrol to 2mg at that time. He will also have an injection in his shoulder as well.

Busy day! Waiting for labs then going for our X-ray. 

Histio awareness weekend update coming soon!

Update July 24, 2013 12PM:

The doctor called and said eric has some slight cloudiness in his right lung. Although with the pains at deep breaths and crackling they heard, I suppose it's what we expected. Thankful it's nothing to be admitted over! Antibotics have been ordered and we will pick them up after work today. Hopefully when we visit the pulmonologist (lung dr) on Friday he will hear clear air exchange! -or be less concerned at the least.

Team Eric to Travel to NYC

We are just two weeks away from joining non-profit Liam's Lighthouse Foundation(LLF) and many others who continue to fight for a cure for Histiocytosis!

The weekend event will take Place in the heart of New York City! We will be participating in 3 of the 4 days of events to take place July 20-23. Our itinerary will include a Meet-and-Greet event at the Marriott Marquis in times Square on Saturday night to kick off the weekend. Sunday morning we'll gather with family to walk in the 1st Annual Liam's Lighthouse Foundation 5k to Fight Histio which will take place along the Hudson River. Team Eric and all the other teams that will join us there have raised over $65,000 this year since the fundraising started in early 2013. July 22nd  we will be standing outside the Today Show which broadcasts on LIVE TV! We along with other warriors and families who have lost a loved one will hold posters of the faces of Histiocytosis! This will be the end of our NYC trek, but LLF and others will continue awareness efforts outside of Good Morning America in July 23. 

It is truly amazing how just a few years ago Michelle Schultz and two others started out on this journey and now dozens will flock the streets of New York wearing blue with pride; to support, to educate, to FIGHT BACK!

To learn more about Team Eric's fundraiser or Join Team Eric visit:


Liam's Lighthouse Foundation 5k to Fight Histio Fundraising Page
http://www.firstgiving.com/LiamsLighthouseFoundation/5KTOFIGHTHISTIO/fundraisers

Life outside of Blogging

It had been a crazy month and a half since we wrote our last blog. It is now June, but I'm going to backdate the posts  so the updates can all stay in order. Eric had been slowly on the mend and many days he still has 'trigger fingers' so I will likely be posting many of the blog updates. We have had our Clay Shoot for HLH and are getting ready to take part in the 1st Annual Liam's LighthouseFoundation 5K to Fight Histio in NYC which will be held on July 21, 2013. If you'd like to run/walk with us visit www.EricsJourney.org and click on the events tab for step-by-step instructions in how to register for this event. 

Stay tuned - we have lots of exciting things happening!!

  As always, feel free to comment on any if the posts with questions or email us if you'd like to know more about something. 

Amanda

This day in 2012 - April 6th

Today in 2012 I was finally starting to have some memories. I didn't really know what day it was and everything seemed so confusing. People just kept telling me how lucky I am to be alive and how i'm a miracle at Temple [Hospital]. I was told it was April 6 and knew it was my older brother Jay's birthday. A couple days before my pulmonary doc, Dr. Desai told me I should start a video journal. It really wasn't something I ever thought of doing and at that time I never thought i'd even share them with anyone. As time went on, I began making more more. Often times forgetting until late at night or the next morning. They wrote the day on the board, but I couldn't really see it from the bed. I never wore my glasses because of the stupid feeding tube. I had been ok'd to talk after being taken off ventilator support, but I wasn't allowed to eat food or drink anything until i passed the swallow tests to make sure everything was going into my stomach and not into my air way.

I hope to help people and their families not experience some of the things I did and I hope that sharing these will help raise awareness for this disease and help others who become diagnosed.

Here's my first video.

Erics Journey With HLH  - Journal 001 [you tube link]

March 2 - This Day in 2012

This day in 2012 was one of the most profound. I'll take you back to yesterday in 2012 first. Doctors had spent the past 2 weeks tsting Eric for every possible infectious disease there was from Avian flu to just about the entire alphabet and then they said it might just very well be a Zebra... What's a Zebra you ask ? A Zebra is a 'rare' disease. One that may present some common symptoms of other more know illnesses, but in actuality it becomes overlooked because most people have what is common.

The day was different, Karen spent part of the day at the hospital or came up in the afternoon and left with me. We came home Early that evening and I remember how Sunny it was still when I received a phone call from Eric's hematologists Dr.Essex and Dr.Fasan. They both got on the phone and reinforced how they thought we should go ahead and do a bone marrow biopsy again. They briefly mentioned their hunch a few days before... Upon running some additional lab work earlier in the week, results showed that if the bone marrow biopsy did indeed show this macro something or another they think they might have a diagnosis. Now in all honesty I can't speak for anyone else, but I sure wasn't convinced after hearing we had over 50 pages of pathology reports and everything was negative! So0o0o after about 15 minutes or so I said Okay - let's do it! 

So on March 2nd 2012 Eric was prepped to do a bone marrow biopsy on his front left groin. Usually they go through the spine, kind of like an epidural, but Eric was on ECMO and was unable to be turned over due to the fragile nature of it. So about mid morning one of Eric's buddies, a close family friend and co-worker was on his way back to the office after being out in the field and decided to stop in on his lunch break. Of course he too was a bit skeptical about this magic moment. At this time the hospital had been letting us use one of the grieving rooms since we had been there so long. I was talking to Eric's buddy Erik when Dr.Essex and Dr. Fasan came in almost bursting with information. They were so astounded by the findings they rushed to have me sign the paperwork for treatment. So Dr. Essex begins to explain this really long named disease hemo...fag-o what ?? red cells, white cells, eating each other... chemotherapy macro ? huh ? What in the hell is this guy saying - Is this cancer?  He assured me I did not need to know what 'macrophages' were at that time and we said okay. Erik had written down all the symptoms Eric had possessed for this differential diagnosis on a napkin, which I still have if I might add. Dr. Fasan handed me the papers and gave me all the warnings of chemotherapy! So I agreed and signed away once again. At that time pharmacy had already been notified of our need for the chemo and to rush it down immediately on receiving the authorization from me to treat.The doctors left. Erik and I just looked at each other in awe. WAS THIS REALLY THE DIAGNOSIS ? One month after Eric was admitted to the hospital we could finally say he has - well HLH at that point! It took me about a week to say Hemophagocytic Lymphohistiocytosis! So Erik called up the colonel and said he couldn't make it to the afternoon meeting and stayed with me until Karen and Tom trekked back up to temple a little early that night. 

Chemotherapy was started with caution as no one was sure as to how Eric's body would react to it. His previous 10% chance of living seemed to dwindle to a 5..especially after google-ing this disease! The literature just wasn't there. Eric was the first or one of the first to be on ECMO and recieve chemotherapy while on over 4,000 units of heparin  a blood thinner used to keep his blood from coagulating and clotting the ECMO machine.

So here we are 1 year later about to celebrate - odd way to put it, but celebrate Eric's diagnosis and 1 year survival since. This journey has been unimaginable to most, but just another day to us. Who knew was Histiocytosis was? I sure didn't..

We can't thank all of our Temple doctor's enough for working so hard!

So much to do before everyone gets here! Time to go HLH-Awareness-ify everything! Go BLUE!

-Amanda 

This weekend in 2012 - ECMO

 Eric remained medically sedated and paralyzed due to the fragile nature of ECMO. He had been on ECMO for about two weeks- its a machine that filters your blood by directing a tube directly to your heart and to machine that adds oxygen and removes CO2 before sending it back into your body because your lungs cannot help circulate it. For Eric they used a line that went in his neck and out his groin but they had to insert larger tubes so they could increase the blood flow since he's so tall! So he ended up with two in his neck. Because he 'wore out the life' of the machine and to reduce the risk of blood clot in the machine they even switched it out to a new one! He was on full ventilator support which basically opened and close his lungs because there was so much fluid around them and they were so weak. We had no diagnosis at this time.

-Amanda

This day in 2012-Valentine's Day

As a culture we honor milestones throughout our lives: birthdays, holidays, graduations, memorials, and even death. The first year of marriage holds a new start for each day that passes on that journey. And on our journey as a married couple we lost many days of milestones to celebrate, often times close to memorials and death.

You cannot put a price on love, but the government has put a price on how long we have to love those with rare diseases. We are fortunate to have been blessed with love from the physicians who treated Eric at Temple. Just 13 days after going to the ER and 3 days after transferring into Temple I said my first 'Happy Valentine's Day' to someone I spent nearly 11 years of my life with and yet they barely knew how loved they really were and how much live would be given to them in the upcoming weeks. Eric was on almost full ventilator support. His lungs were on the verge of failing if it weren't for the machines. He had begun sedation that would last virtually 2 months and responses were weak with just the notion of muscle movement, but not a word or eye-contact. An occasional squeeze of the hand ever so faintly dwindled in the days to come.

Milestones are merely a way to make marks on life when were too busy to notice the rest of the days of the year. For us, Valentine's Day has never been important. It's just another day to be forced to buy something to show your love. We've always celebrated life and how lucky we are to have each other to love. We could never repay the unconditional love we received and still receive from the physicians, nurses, CNAs and supporting staff at Temple University Hospital in Philadelphia.

So many have lost their LOVEs and we can never replace that, but we can help keep others here with us if we love one another enough to help a stranger. We have been so humbled by the overwhelming support of the other Histio families. We have supported other families and they have supported us even after they've lost their LOVE.

Please help us LOVE another HLH/Histio family with opportunity for cures to these awful diseases and memorialize those who gave their life to HLH with a $10 donation to #TeamEric. You will find the donation box on the right column .

We are thankful for organizations like Liam's Lighthouse Foundation, the Histio Association and the Akin family, for the quote and book they wrote "Love Triumphs Grief" will be a testament to families we have grown to he a part of- for this will be their first valentines day without their child, mother, father, husband, wife, sibling, grandparents extended family and friends.

May you be able to find love every day, not just on this holiday. Let us open our hearts to love all who need love.

Amanda

Cold? Flu? Still's Disease Flare?

If you are out of the loop see this post first: Angels and Prayer Warriors

Tuesday night we only got about 2 hours of sleep total the entire night, so needless to say yesterday was exhausting! We spent the entire day at the ER as recommended by Eric's Rheumatologist due to the fact that meciations like Humira, methotrexate, and Humira can allow for Eric's body to become ill at a much faster rate. Fears of Sinusitus or even meningitis were on the table of possibities. Early labs taken at the family doctor trended away from an HLH Relapse

We had full labs, 2 X-rays and a CT scan on his head.

They showed:
• white cells were trending down, but are still above normal range.
• lung X-ray is clear
• CT scan was clear

Eric trying to rest off this pain gave me the dead fish face and stuck out his tongue for laughs...

That being said it has been proven to be a less intense sinus infection or a Still's flare up, possibly both. We were discharged since its safer to be home that the hospital and no imminent danger showed.

This morning we went to the Rheumatologist to go over everything and map out a plan. For the time being we stopped prednisone and replaced it with Medrol which is another similar type steriod which sometimes works better for patients than the prednisone. Also started a stronger antibiotic to fight infection. Hopefully we'll see a difference in a few days to a week and if so we can monitor and begin to taper the steriods again to a better level.

 Sorry for the delay - I didn't get much to eat and was super tired so when we got home last night..I ate and fell asleep on the couch and my phone wasn't charged all night.

- Amanda

Calling All Angels

Just like the song by Train, I'm Calling all Angels. It's been a long week and a half for us. Excited with anticipation of dropping prednisone levels again, Eric got his wish. We went down to 5mg! That's pretty close to what your body naturally produces so were almost ready to see if he can begin to start making it on his own and get off prednisone all together.

Just a few days after lowering the prednisone Eric began having severe headaches/migraine and became a little more tired and exhausted easier than usual. So we drank healthier drinks, bundled him up more and hope for the best.

The work week started and the headaches continued. They'd come on strong in the late afternoon and peak highest just about bedtime and would linger all night long off and on, waking Eric every 30-45 minutes. I haven't had nights like that in over 6 months. It was pretty rough.

Eric went to work Tuesday and by the afternoon he was feeling as crappy as the days before. So as we normally do we contacted all his doctors and decided to increase his prednisone by .5 and go back to the usual drop of .5mg every two weeks. Wednesday came and went and the headaches did too. So Thursday I called te family Scott and we were able to squeeze in an afternoon appointment.

Typically most people wouldn't fret over he aches and tiredness, I mean seriously, who can't use a little extra sleep every now and then?

While we were there we took some blood work again to check Eric's inflammatory markers and his cell counts as well as some vitamin levels. He also got a B12 shot which might be a regular regiment after we consult his 4 other main doctors. She put him on Bactrim and Flonase in hopes that this might just be the start of a 'simple' ear infection or sinus infection and nothing worse. The thing is- the simple anything to the average healthy person can become easily more complicated and detrimental to someone like Eric because of all the immunosuppressant drugs and life threatening diseases he has.

So as Eric finally drifts off for who knows how long, I'm calling all ANGELS and PRAYER WARRIORS to surround him with their love and pray for this to be nothing more than a simple cold that passes soon and that the Stills Dragan lay dormant. For more information on Still's Disease visit: www.StillsDisease.org

-Amanda

http://youtu.be/TaG9SDxwPBg

Sean Fischel, HLH Warrior

Last fall we had the oppotunity to interview with the Philadelphia inquire on our recent experience with HLH. It connected us with the Fischel family of Moorestown, NJ. The Fischel's youngest son Sean had been transferred into Philadelphia Children's Hospital for treatment for HLH. He put up one amazing battle that surpases even my own. He is truly one amazing kid I wish we could have met and gone fishing when he was better. After everything he had been through he managed to get a smile for camera on his moms updates. It was so exciting seeing him travel outside of his room in a wheelchair for the very first time a few weeks back. I remember how much freedom it gave me.
Rest easy little Sean. We will miss you.

In memory of Sean Fischel, age 7, HLH Warrior

October 2005 - January 6, 2013

Here is a small clip from Sean's Campaign that took place in September during Histiocytosis Awareness month where he surpassed his goal of $7,000!

"Our warrior Sean lost his battle with HLH on the afternoon of Sunday, January 6, 2013. He fought courageously for three months and made remarkable strides towards recovery. Many in the medical field used the word miraculous when speaking about Sean. Indeed, he was a miracle child to those who knew and loved him. He will be greatly missed by so many but he will remain in our hearts and souls forever. We have no doubt that this brave boy is now shining in GOD's light."

The Fischel Family is asking that donations made in memory of Sean be sent to 

 Children's Hospital of Philadelphia.

The CHOP Foundation

Lock Box 1352

PO Box 8500

Philadelphia, PA 19178

Donate online here.

Directions to include a note to be sent to the Fischel Family: Check off  'This is an honor or memorial gift'.

Then choose in Memory of from the drop down menu under Tribute Gift Type and type in Sean Fischel below

and the address you wish to send the note to.

Service Information

Friday, January 11th, 2013

12:00 PM

Funeral Mass at Our Lady Of Good Counsel

42 West Main Street

Moorestown, NJ 08057

Gathering immediately following mass at the Moorestown Community House

Older Blogs About Sean

Sean's Journey With HLH

Holiday's With the Fischels



Love For Lana Part2

We posted about Lana's journey with HLH a few months ago and were so excited her brother was a match, but do to complications the bone marrow transplant could not take place, leaving Lana back at square 1- looking for a match. Here is her update. View 1st blog here.







Lana Leake
**UPDATE** My brother was an identical donor match for me. He had a stem cell collection on October 23, 2012, but didn't give enough. They let him go home and rest and rejuvenate for about a month, and brought him back for stem collection. This time, he gave even less. They said it was because of his prior radiation due to prostate cancer and possibly his age. Now they have decided to find another adult donor. If you haven't already done so, please get tested. It is a simple swab test and the collection is given by IV, just like giving blood. Just think, if you are lucky enough to be a donor, the life you save may be a family member, a friend, a neighbor, a stranger, or me.

January 11th: **UPDATE** I went to Morgantown yesterday to hear "the plan". Well, there is not one as far as he is concerned. God has a plan, but did not tell the doctor yet. I have such a rare HL type, they are unable to find a donor. Said they will keep searching, but have already ran a worldwide search. He said the only other alternative would be to have my son be the donor. We will have to go to John Hopkins Hospital in Baltimore, Maryland for a consult. Not sure that it will be successful. I am now more than scared, I am terrified. I have so much to live for and will not go down without a fight.I want to thank everyone for their prayers and concerns. I am asking that you please keep me in your prayers and askiing anyone out there who hasn't already done so, to please, please get tested to be a donor. If not for me, for others out there who are in the same position I am in. Sending love, hugs and prayers to all of you. ♥ ♥ ♥

Donate
Lana Leake at PO Box 241, Cannelton, WV 25036.

December Labs

The doctors say to take the 'numbers' with a grain of salt. If I'm feeling 'good' or better and not worse --that's most important. These were taken in December but with the holiday Amanda didn't get them updated on our Quest App which is really helpful for any of my fellow HLH family reading this. It's called Gazelle by quest diagnostics and all you do is type in the date you had blood drawn and the doctors name and info and you'll have them on your phone app in just a few days. Then you can view them or fax or email them to another doctor with just one tap!

So my ferritin dropped back down and so did the CRP. They look at ferritin for several things. Ferritin is a measure of iron in your blood and is followed for HLH diagnosis, but my red and white cell counts don't indicate any crazy HLH stuff going on so it's likely attributed to my Still's Disease. CRP is a non-specific inflammatory marker they look at. Last time it was 4 something so this is a step in the right direction.

Just moved down to 6.5mg of prednisone last Thursday an I'm feeling it a little but I'm hopeful it will subside just like it has before so I can move down to 6 next week!