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

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!

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.

Foot Brace

Was fitted for my ankle brace today. Did the steps again and walked over 600 ft. Chemo tomorrow then more progress. See u all soon home

Day 89 in the hospital

Another first. And broke the walking record with 335 feet. They are now talking about what items I will need at home. And I think I will be able to get a camo foot brace for walking. Home is getting closer and closer. This is day 89 in the hospital. I Will Not Hit 100.

A lot of good news today.

A lot of good news today. All of my bad numbers are lowering. Still not where they need to be but getting there. The X-ray was good. Catscan was good no blood clots and clean strong lungs not perfect but a huge change from no lungs and a machine and a collapsed lung. Spinal tap returned with no problems in the brain which was a concern with my headaches and soreness. So it's just working out every day. Thanks I love reading the comments and the visitors it really helps me get better faster. Miss you all love you all and awaiting my homemade spaghetti ( Ty Lois). And my fresh philly pretzels (ty steve jason erik) and my piece of chiken steak (ty ruth and marty) thanks everyone for all the MUCH NEEDED support. You guys all had a huge part of me surviving and healing.

Another spinal tap-

Got a spinal tap some time this morning just to be safe, of headaches lately. Then an emg where the put needles in ur muscles and check for major nerve damage. My left arm and leg, mostly foot are still numbinsh and weak and the foot can't move up. Good sign I was able to move it the tiniest bit in pt yesterday but only once. I am looking forward to my homemade spaghetti meatball and sausage and homemade garlic bread dinner. That will brighten a day of tests mixed with hard workouts up. Love you all. Enjoy every day and don't take advantage of the little things you may miss them one day. Thanks for all the support.

7th chemo treatment..

 Finished the 7th chemo treatment.

 Feeling ok, not great but pt went well

 still pushing myself. Looking foreword

 to seeing everyone out of this place

Really hoping for 2 weeks or 2 and a 

half. Wish you all the best. Thanks for 

all the prayers. 

One more chemo?

Did 5 sit to stand today. Still need the help getting up but 

standing good on my own. One more chemotherapy

treatment tomorrow hopefully done with them. 

Miss everyone. Hopefully home in a couple weeks.

Hi Guys!

Hi Guys! I'm getting better slowly!

Why a big gap ?

There's a huge gap here because during this time Eric was medically sedated and paralyzed. He was placed on something called ECMO. ECMO is basically a process like dialysis, but for your lungs. It filters the blood out of your body and through a tube into an artificial lung machine that is outside of the body and adds oxygen and removes co2 before reentering back into the body.

Thanks
Amanda
(Erics Wife)