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!

Christmas 2012

This year we stayed home for Christmas instead of traveling to PA. It was nice, but I missed riding the quads in the snow with the dogs. That would have been fun. I got some pretty cool stuff and even some gifts wrapped in Bone Collector wrapping paper... It was relaxing to have Christmas day at our house and not have to travel. Everything went pretty smoothly. The ladies did their thing in the kitchen and Amanda successfully had dinner ready at exactly 5:30. Dinner was delicious and dessert was amazing! We had some friends and neighbors stop by. It was great watching the niece and nephew and neighbors kids open their gifts. We just want to thank everyone for coming here and making Christmas at our house possible this year.

December 2012

Things started out slow, but got pretty busy after Novermber. In the beginning of December I was starting to feel a little better with the help of the Humira. The doctors decided to go ahead and lower my prednisone again. So I was moved down to 7mg. Then as long as things were going okay I could lower .5mg every two weeks and return to the doctors in the middle of January when I reach 5mg to discuss the next step. I can't wait to be off this stuff!Rehab medicine said the strength is increasing in my foot that had barely any movement when I left the hospital. Still can't lift up my big toe though. Christmas is only a few days away. Hope everyone gets what they want.

Holiday's with the Fischel's

As many of you know- but for those who don't-- Our recent publication in the Philadelphia Inquire has led us to connect with the Fischel Family from Moorestown, NJ. (See publication here)

Sean is an amazingly strong 7 year old who is battling HLH and so much more than most do in a lifetime in the past few months and those to come. As we go through this Holiday season we ask you to continue to keep Sean, his parents, siblings, family and friends in your prayers. If this is the first time you are reading about Sean's journey, please read our first blog entry here.

Over the holidays we will update you on Sean's progress below. Please feel free to leave words of encouragement for his family below.

--Amanda & Eric

December 2,2012
Sean is going into a hyperbaric chamber tomorrow to help him prepare for his surgery on Friday. This unconventional treatment is supposed to infuse his body with 100% oxygen which helps in the healing process and fighting infection. I will update you with surgery info once family has been informed. Prayerfully in God's Hands.

December 6,2012
My dear friends, we are taking a big step tomorrow on Sean's road to recovery. He is undergoing bilateral below the knee amputations as well of amputations of all of his finger tips (except his left ring finger!). He sustained this damage due to being in septic shock for so long with very poor circulation. We are sooo truly blessed and thankful that Sean is alive and that he is mentally intact. Our Sean can do this!! He is so laid back, adaptable and happy. Please pray for the surgery to go well and for Sean's spirits to remain high.

All my love!-Kim(Sean's Mom)

December 7 (Post Surgery)
Sean is out of surgery. It was about 6 hours total. All the procedures went well with few complications. He was extubated in his room and spoke a few words. He was given more pain med rescues and is now sleeping. The reality of it all is becoming more concrete. We have a long journey ahead of us. Thank you so much for all your notes and messages of encouragement and faith. I can't tell you how much they mean to me. The Lord is my strength.-Kim(Sean's Mom)

December 21
Sean is our Christmas miracle! He got his wish this afternoon...he has been cleared to drink and eat soft foods! His first request was sushi!! He can't eat that yet but his next desire was mashed potatoes and icy cold water!!

Although we may not have all our festive trimmings around us, we have the true Christmas Spirit within us. We are so blessed!! Merry Christmas to all our amazing and supportive friends!

January 1, 2013

Update: It seems we have hit a plateau. Sean continues to fight the fungal infection in his lungs while we pray the HLH is in remission. His breathing settings have not been weaned in weeks and his chest tube is still producing fluid. He continues to present with intermittent fevers, tachycardia, and vomiting. We are entering our fourth month in the intensive care unit. The weeks are long, but the Days are even longer. The duration and severity of Sean's problems are really taking a toll on him emotionally. It is so hard to watch sometimes. Please pray that he has the emotional and physical strength to make it through this tough course ahead. I pray that each and every one of us can count our blessings big and small in this New Year.

Update: January 6 Update: Sean has been declining medically. A full body CT Scan revealed an abscess on his chest wall between his aorta and pulmonary artery. He went to IR to get it drained under a "live X-ray" for precision. His chest tube was also moved into a lower part of his lung for better drainage. Because of this recent development he will most likely need invasive surgical intervention. The extent of this surgery is being discussed by his PICU team, general surgery, and infectious disease. Keep praying please!!

Next Blog on Sean

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HLH Check-up/Thanksgiving

So far my labs look pretty good. My white and red blood cells have normalized into healthy range. I guess good labs make for a boring update. But it is nice not going nuts trying to figure out why they're not normal. I have a follow up with rheumatology in a few weeks. We'll probably be lowering the prednisone again. I can't wait to be off it.

Hope everyone had a nice Thanksgiving. Ours was good. We visited my family and Amanda's. Christmas is at our house this year so were going to the tree farm this weekend. Amanda already has the lights up.

Just wanted to say thanks to everyone for helping share our fellow HLH family members stories. It means a lot to us an them. Keep an eye out for more ways you can help. Amanda will be posting info on volunteering on the website soon.

-Eric

Lung check-up

Today was really long and i'm pretty tired. First I had a CT scan on my chest and abdomen to check my lungs, liver and spleen. Then I had to do a test to see if I breathe ok during and after exercising. 4 hours later I finally had my actual check-up with results.

Overall my lungs are where the doctors expect them to be. The medicines i'm on are limiting the amount of 'good inflammation' that increase healing, so everything will take much longer than it would if i wasn't on them.

The CT scan showed scarring from the spots that started to harden when i was on the ventilator and ECMO. Hopefully they'll continue to heal and soften. It's still hard to breathe big deep breaths sometimes and i get sharp pains too. They were still a little fuzzy in some spots. For those of you just following this is an improvement because i was told when i was on ECMO my lung xray was completely white and its supposed to be black to show air in them not fluid. My breathing test was good. They took my blood and measured the oxygen and co2 and then i exercised and they measured my breathing and heart rate continuously and then they took my blood again. I never needed oxygen help once-so that's good. My spleen is getting smaller, but is still enlarged and my liver is still enlarged. last time labs showed my liver function was okay though so we'll see next week after my labs come back.

The doctor said as long as I continue to do healthy things and don't get sick I shouldn't have to come back for a few months. So I wont have a lung doctor appointment until March of next year.

I also saw some familiar faces and met a new one. Maureen, who's having a baby next month, was one of my ICU nurses that took care of me when I was sleeping (medically sedated). She even donated blood directly to me with the program Amanda set up with the Red Cross when I needed several transfusions a day.It's pretty cool knowing these people worked so hard just to save me.

I'll have some bloodwork done Thursday to test my cell counts and see where I stand with the HLH. I won't have those results until next week. The Humira is helping my arthritis and I feel much less pain and almost normal, but weaker and slower.

New HLH Support Website

We are pleased to annouce the newly published HLH Support website --created by Kelly Marsh, mom of HLH and Bone Marrow Transplant Survivor Hannah Marsh. Kelly started a support group on Facebook a few years ago so families could unite, share their stories and support one another through this trecherous journey. With limited resources available for histio diseases, this website is a one-stop-shop for those privy to the HLH world who are looking to recieve a more intimate inside to what they are about to endure. Where there is Hope and Love - there is strength. --And there is plenty of that to around in this group from stories of loss that still empower others to continue to fight and stories of success, like Hanna's, that give us all the will to continue on this journey. This website will connect you to resources such as the Histiocytosis Association and other non-profits with specific goals in funding a cure to HLH.  It will guide you through the bone marrow transplant process for HLH patients. There you will find a link to the private Facebook group page. We hope you will find comfort in this site. Without Kelly's efforts to organize the support group the world would be a much colder place for HLH patients and their families. I think we can speak for the group when we say her mother's touch has touched us all.

Thank you Kelly! Congratulations!

-Amanda & Eric Majusiak

www.HLHSupport.org 

Sean's Journey with HLH

Our recent publication in the Philadelphia Inquire has led us to connect with the Fischel Family from Moorestown, NJ. (See publication here)

This is Sean's story from his write to unite campaign that supported the Histiocytosis Associations campaign to raise awareness and funds for histio research. Below are some updates on him. Please keep him in your prayers. He is an ECMO warrior just like me! He has developed an infection and is battling that and HLH while on chemo. Please pray!

This September, Sean was a normal, happy, healthy boy beginning his first grade year and celebrating two new front teeth that came in over the summer (just in time for this school photo). A soccer and street hockey player, novice wrestler, and even a student of sailing, Sean tackles everything with a sparkle in his eye and a caring heart. When he developed a high fever, it was monitored by family and doctors; but wasn’t of great concern, nothing out of the ordinary for a child this age. What we didn’t know was that the fever was the beginning of a very frightening journey in which Sean and his family are now living. 

Sean’s rapid decline included multiple organ failure, several cardiac arrests and finally a diagnosis of HLH, an extremely rare disorder of the immune system effecting only 1.2 children per million. Sean is now fighting for his life at CHOP hospital in Philadelphia, one of the few hospitals in the country equipped to treat this disease. His team of doctors and support staff are remarkable and Sean’s family is facing each day with guarded optimism as they watch Sean’s slow progression. The physical and mental anguish is overwhelming; but, every day that Sean is with us is a blessing and sign of hope for the future of this beautiful boy.

We still have a long way to go with understanding this disease. Every new day presents another difficult challenge for Sean; but he is a warrior and is fighting hard as he confronts this disease head-on.

Leave an encouraging note for Sean!

Update 10/27:Please, fb friends and family, keep praying for Sean to recover from this unexpected illness:

4PM marks the time exactly 28 days ago, that we arrived to CHOP via helicopter. I didn't think we'd make it this far and at the same time I didn't think we'd here this long. This journey has been so painful, tumultuous, and unexpected. We have so far to go. Sean is a warrior! He is like David in the face of Goliath. In a world where many give up, he is persevering! He is truly fighting for his life daily! He amazes me everyday!

Sean's body CT scan did not reveal anything new! There were no new areas of infection. His fever has broken today and his vitals look good. This kind of infection however, is very slow and difficult to cure in the setting of being immunosupressed. It has also set back the treatment of HLH as his chemotherapy and steroids have be significantly reduced. There is such a fine balance that changes daily with HLH on one side and Infection on the other. Both are ravaging his body.

Thank you so much for your love, prayers and support.

Update 10/28:Sean had a stable quiet day. He opened his eyes this morning in response to me talking. It's the simple things that make me so happy.

Update 10/29: Seany has a lung thoracoscopy tomorrow to see the extent of the fungal infection and to wash it out. Praying it is not invasive (in his lung tissue) or he is going to need surgery to remove part of his lung! He is running a fever again and has elevated blood pressure and heart rate. Pray again!

Update 10/30: Sean came out of surgery well. Results however were pretty bad. He has invasive fungus in his chest wall, on his entire right lung and on his diaphragm. Not sure how we are going to proceed...we have a meeting soon with Oncology, rheumatology, infectious disease and surgery to discuss the best of the worst options. Again a fungal infection cannot be eradicated unless you have your own immune system...however his own immune system has been causing all this nearly fatal damage.
Update 11/1(From Sean's Mom): "Days in the PICU are filled with so many ups and downs that I feel like a schizophrenic! I find myself watching Sean's monitor (Heart rate, BP, Temp, Venilator etc) like TV. Still love doing all his dressing changes and washing his now bald head.--- Fungal infection is the most imminent threat. Without an immune system it is fatal. His immune system however poses the same threat. Doctors decided to stop suppressing his immune system with chemo. They are also going to trigger it by transfusing donor granulocytes (WBC). Their hope is that it attacks the fungus and not Sean. Love my prayer warriors!"

Update 11/5 from Sean's mom: As I head to bed tonight at the hospital, I wanted to let you know that Sean had a good day!! His white blood cell count is so robust that the transfusions have been halted. He's been fever free for 24 hours. His blood pressure hasn't been fluctuating out of control. His skin is healing really well. And his ventilator settings are being weaned aggressively. The Lord is answering prayers. I can feel it and I believe it. It seems that Sean's fight has a bigger purpose. The Lord is inspiring so many people to come together. Thank you so much and keep praying!

Update 11/7: Big day...Another MRI of his brain to be followed by a spinal tap to deliver his chemotherapy directly into his cerebrospinal fluid. He was extremely agitated this morning but then calmed down and seemed to enjoy his ride to the MRI floor. There is a chance they may extubate (take him off ventilator) tomorrow if his weaning goes well. His fungal infection vs the HLH are in a precarious balance.

Update 11/10 from Sean's mom: Sean was extubated (This means his breathing tube was taken out) on Thursday and spoke his first words in 44 days!! He said "My belly" in response to what hurts?!!

Update 11/15: Sean has gone from "being the sickest kid alive in CHOP" to trying pull a "jail break"! He somehow managed to shimmy out of his bed with his respirator, feeding tube and med lines being pulled out in the process. The nurses and Brian found him on the floor unhurt. He told Brian in his tiny soft voice, "I want to go home, Daddy take me with you." My tiny warrior continues to fight! We have at least two big procedures coming up at the end of the month to fix some of the damage to his body. He will probably be fighting the fungal infection for months (hopefully without surgical intervention). Then we hope we are stable enough to move out of the Intensive Care Unit to the Oncology floor to treat his HLH with Chemo before Christmas. Faith hath no Fear! We continue to see miraculous improvements. Please keep praying!

Holiday's With the Fischels (Sean's Journey Cont.)