Eric Majusiak's Journey with HLH - Hemophagocytic Lymphohistiocytosis, a rare and potentially fatal blood disorder of the immune system. Visit www.EricsJourney.org for more information
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.
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.
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.
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
