This is a guest blog from a mother of a miracle child, Bennett, who passed away. Please read and see why we are all FTK and why we will all always #RememberBennett.
I’m Aimee Hanneman. I’m honored to share with you about my Miracle Kid, Bennett.
In August of 2012, when Bennett was just 5 months old, he wasn’t himself one Thursday morning. He was lethargic and didn’t want to eat. A trip to the pediatrician’s office ended with drawing blood. A few hours later the pediatrician called to tell us he was SEVERELY anemic and we needed to get to Children’s Hospital in OKC as soon as we could. They’d be waiting for us in the ER. After a flurry of activity (and the realization that something was REALLY wrong), he immediately received a blood transfusion to stabilize his hemoglobin level, but during his hospital stay, they also discovered his white blood cells and platelets were deficient, too. During our 6-day stay, the doctors ruled out leukemia and some other ‘yucky stuff” but were unable to make a diagnosis.
It didn’t take us long to understand how lucky we were to have even made it to the hospital that day. A normal hemoglobin level (which quite simply is a measure of how much oxygen in in your blood) range is 9-12 micrograms/kilo. For ease, it’s just talked about in numbers from 1-12, with normal being 11.5-14. Bennett’s hemoglobin that day was 1.7. Around the hospital the lowest they’d seen was about 3. He was at risk for cardiac arrest or stroke and we didn’t know it. Thankfully he’d been rather healthy and we hadn’t had to find out that he had very little in his body that could have fought an infection.
On December 19, 2012, after four long months of tests, we finally learned his diagnosis. (How can it be three years ago?) Bennett was fighting an EXTREMELY RARE disease called Pearson(‘s) Syndrome. When I say EXTREMELY RARE, I mean there have been less than 100 diagnosed cases in the WORLD!! He’s the only patient in Oklahoma EVER with this diagnosis and it was a surprise even to his doctors.
Pearson Syndrome is a rare multisystem disorder caused by mitochondrial deletions/mutations in cells throughout the body. (Mitochondria are small units responsible for energy production – the battery packs, you could say, in the cells throughout the whole body) Mitochondrial disease causes cells to not be able to carry out their function and result in various symptoms/organ difficulty. Two of the most recognizable symptoms in Pearson Syndrome are bone marrow failure and pancreatic insufficiency, which is what he fought the most. Pearson’s is a waiting game to find out what part of the body will fail next.
Most cases of Pearson Syndrome appear to be random occurrences instead of an inherited condition and Bennett’s is just that. Unfortunately there is no specific treatment for Pearson Syndrome other than to support the symptoms, which is exactly what we did from day one. Bennett’s greatest need to survive was to manage the bone marrow failure. Until a year ago, (and I’ll tell you more about that in a minute), he was dependent on red blood transfusions (about every two weeks) to treat his anemia. For over two years, he received a daily shot to stimulate white cell production and he needed platelet transfusions at various points. In all, Bennett needed over 30 liters of blood products!
For Pearson kiddos who’ve lived past the age of 2, their bone marrow has recovered on its own, eliminating the need for transfusions. We thought that was happening for Bennett in the spring of 2014, but by June, things were starting to drift the other direction again. The bone marrow failure of Pearson Syndrome created an environment for other yucky cells to set up shop. In August of 2014 we learned Bennett had developed Myelodysplastic Syndrome, a precursor to leukemia, as well as monosomy 7. Long story short, the duplication of these unwelcome cells was taking up space in his bone marrow and not allowing his body to make the good kinds of cells he needed. Our best chance for survival was a bone marrow transplant. (Without it, doctors anticipated the cancerous cells would take over his body in a matter of months.)
An anonymous donor was a REALLY GREAT match for him and we were set for transplant mid-October. Much to our dismay, he acquired a nasty fungal infection in his left foot which doctors told us a healthy person, not to mention an immune-compromised kiddo like Bennett, would have had difficulty clearing with medication. We attempted it for nearly six weeks to no avail. To rid his body of infection and get him to transplant, we made the difficult decision to amputate his left foot. The amputation site healed beautifully and finally, on December 11, 2014, he received the gift of new life in the form of healthy bone marrow from the original donor who’d waited for him to be well enough to proceed.
Our hearts wrenched at every turn as we worked with his medical team to make decisions for his care. It was a catch-22 knowing that all of the medications – antibiotics, antifungals, chemo, immunosuppressants, steroids, anti-rejection – made his body work overtime! As he battled against that infection and as his body worked to accept the new marrow, every day was a juggling act by the doctors to keep his electrolytes in balance and keep him stable! Even the smallest fluctuations had the potential to send his body into a state of metabolic crisis. We hoped and prayed for the best but our hearts and minds questioned what the long-term effect would be on his vital organs like his kidneys and his heart.
Bennett was in the hospital for 108 days from the beginning of October to mid-January of this year. His bone marrow transplant had gone better than anyone expected! Now we needed to wait and see. The next few months were crucial. He ended up being in and out of the hospital as his body worked hard to accept that new marrow. The anti-rejection meds and steroids kept his system suppressed as he fought graft vs. host disease and other things. At the end of March, dehydration from a stomach bug turned into a three-week stay to get his electrolytes back to (his) normal. (This was supposed to be a one-night stay for some fluids!)
A fever and cold symptoms sent us back to the hospital at the end of May. I didn’t get my hopes us for a quick discharge but in no way were we prepared for what was coming. We spent six days fighting that, went home for a day and returned to the hospital. The wheezing in his lungs had worsened and pneumonia was apparent. A day later he was transferred to PICU when his body reached a point of metabolic crisis.
On June 17, 2015, after twelve days on life support, Bennett ended his fight. I can’t say he lost his fight because he won so many battles! Bennett fought everything thrown at him like a champion!! Through it all, he displayed the brightest smile you’ve ever seen, along with resilience, perseverance, bravery, determination, stubbornness, and grace. He loved and was loved like crazy!
Our family will forever be grateful for the incredible team of doctors and nurses at The Children’s Hospital and the support of Children’s Hospital Foundation. Since we live in Oklahoma City, we’ve had only a short drive across town for all of his care! We had everything we needed to help Bennett live life to the fullest despite battling such a rare disease.
As an OSU alum, I am especially thrilled about the efforts of Cowboython that will significantly impact children all across Oklahoma. Personally, I can’t help but think how amazing it would be for The Children’s Hospital and Children’s Hospital Foundation to play a role in finding a cure for rare mitochondrial disease like Pearson Syndrome! FTK!
Our family is looking forward to joining you on February 13th! See you then! Until then…
Love like crazy.