Sunday, October 28, 2012

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!

Sunday, October 21, 2012

Fall Changes

So my sleep study got canceled because of insurance reasons. Hopefully they'll reschedule it sooner than later. I was hoping to get it over with. The Humira seems to be working so i'm crossing my fingers Rheumatology lowers my prednisone tomorrow. This week MTG should be finishing the kitchen floor up. Justin, my dad, and Jode and the neighbor helped put the fire stove back together too this weekend. It sure did warm up the house nice. We started getting things cleared up outside since it's getting colder. The leaves are all yellow and orange and starting to fall. I hope we can keep up with them.

Update: Rheumatology was pleased with how the Humira is helping me and decided to start lowering my prednisone. It was 10 now we're moving down to 7.5! Hopefully it will go well and I can move down to 5 in December.

Tuesday, October 16, 2012

Fall 2012 Events Update

Together with Rowan engineers and the Geography department at Rowan and our efforts at the Blackwood Pumpkin Festival this past weekend we have raised about $1,000 for Histio research and our fellow HLH family members in need. 
Our table was sponsored in part by the Wickstrum family in memory of HLH Warrior 
Neil Wickstrum,69 who lost his battle with HLH in August 2012.

We can't thank everyone enough for all of their

 support this year!

Over the winter we will be working on event ideas for next year.

If you would like to help volunteer or be a sponsor at future events please email  EricsHLHJourney@GMail.Com  with the Subject 'Event Help 2013'. Please include your name, location, and areas you would like to help with or services you can provide. (ie: design flyers, reach out to sponsors, host your own event, volunteer at our event, donate items/materials, ect.) We will compile a list for our reference and send out an email along with events and services needed to make that day possible.
You can also make donations via

Thanks for being a part of Eric's Journey to fund the cure for HLH. 
-Eric and Amanda

Wednesday, October 10, 2012

Hats off to Rowan Engineers!

We can't thank everyone enough for all that they have done. A team of Rowan engineer student presidents were presented with my story and asked if they'd like to help us raise awareness and lend a hand to some repairs at my home to help make it safer for me. They did not hesitate for one moment. Over the last few months they discussed ideas and met weekly for a month before the Histio Kick off event they held on October 5,2012. It included Rowan Physician, Dr. Peggy Hurley, the President of the Histiocytosis Association, Jeff Toughill and myself. That week they also held wristband and food sales in addition to the two Dine for A Cause night at the Landmark Americana in Glassboro. 25% of each check that presented a coupon was donated. This week MTG construction has begun the repairs on our home. We are still in awe over all of this and are amazed and filled with joy and gratitude for all of the hard work that has been put forth by Rowan students & faculty and MTG construction. We are truly blessed.

A special thanks to Dr. Beena Sukumaran and Dr. Chin for coordinating & hosting the histio awareness events. It means a lot to us that we could share this information and help someone in the future by doing so. We are eternally grateful to all Rowan has done for us and the histio community.

Check out the Rowan PR release on this here:

Tuesday, October 2, 2012

Love for Lana

Our friend Lana is an adult with HLH just like me. Please share her story and help if you can as she goes through a bone marrow transplant in the upcoming weeks. If you can't help, please pray. Prayers are strong and she will need them.

My name is Lana Leake (Johnson). I am 43 years old. This past May, 2012, I was diagnosed with Hemophagocytic Lymphohistiocytosis (HLH). I was close to dying. I was almost in kidney failure, my liver was elevated, my spleen was enlarged, my heart was out of rythym, I had bilateral pneumonia and my body was full of sepsis. Hemophagocytic lymphohistiocytosis (HLH) is a rare but potentially fatal disease of normal but overactive histiocytes and lymphocytes .Fever, hepatosplenomegaly, pancytopenia, lymphadenopathy, and rash often comprise the initial presentation.. Secondary hemophagocytic lymphohistiocytosis occurs after strong immunologic activation, such as that which can occur with systemic infection, immunodeficiency, or underlying malignancy. HLH is characterized by the overwhelming activation of normal T lymphocytes and macrophages, invariably leading to clinical and hematologic alterations and death in the absence of treatment. The spleen, lymph nodes, bone marrow, liver, skin, and membranes that surround the brain and spinal cord are preferential sites of involvement. Incidence is reported to be 1.2 cases per million persons per year. Familial hemophagocytic lymphohistiocytosis is uniformly fatal if not treated; the median survival time reported in various studies is 2-6 months after diagnosis.

The historical series collected by theInternational Hemophagocytic Lymphohistiocytosis Registry reports a less than 10% probability that the patient survives for 3 years. Even with treatment, only 21-26% can be expected to survive 5 years. Remission is always temporary, as the disease inevitably returns. Bone marrow transplant is the only hope for cure. One study found that 50% of deaths were due to invasive fungal infections, which are probably underdiagnosed. The outcomes of secondary hemophagocytic lymphohistiocytosis vary.

I am to have a bone marrow transplant at WV University Hospital in October or November, as soon as I find a donor. I am still waiting on a match. I have approximately 20 appointments in Morgantown prior to the bone marrow transplant. After the transplant, I am going to have to stay in Morgantown for approximately 4 months. I am asking for donations to help pay for my medical expenses, and lodging in the Rosenbaum House, food and travel expenses. My husband is on disability and I have been off work since May, 2012, and any donation that you can give would be greatly appreciated.

Update: Since this was written, Lana's brother Gene will be her bone marrow donor.
Update: I will be admitted to the WVU Bone Marrow Transplant Unit on Sunday, October 21, 2012, and will have the transplant on November 2, 2012. Please keep me in your prayers.

Update 10/23: I just wanted to let everyone know that my bone marrow transplant has been postponed once again. This is the second time, and I am so bummed out, but I guess that's the way God meant for it to be. My donor was unable to give enough stem cells after 2 days of collecting (10 hours total). I have to go back to the docotor on November 1st and they will let me know what the plan is. Said they may want remobilize my brother and get more cells once he has had time to all them to replenish. Thanks everyone for the thoughts and prayers. Will keep you updated.

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