Living with Hereditary Spherocytosis
By Sinead Borgersen, Coordinator for Nimsoft Blood Drives
My two-year-old son Faelan has a hereditary red blood cell fragility disorder called hereditary spherocytosis. He inherited it from me and I inherited from my mother with another of my siblings. His red blood cells are fragile and spherical in shape instead of the normal donut shape due to a defective gene that causes the shell to be misshapen, like a pole missing in a tent. His red blood cells live a shorter life and the spleen becomes enlarged as it attacks the red blood cells, causing them to live a very shortened lifespan of 3-10 days. So he is anemic and fatigues easily.
Although rare, it’s one of the most common blood disorders for children. We were the first to be diagnosed ever at our hospital when he was born and ended up educating the doctors/nurses on what the disease was. We got them in contact with Dr. Glader, a pediatric hematologist at Lucile Packard Children’s Hospital (LPCH), as I knew my children may get the disease and so was prepared beforehand. Thanks to our education of the staff where he was born, they referred another three families in less than a year to the hematology clinic at LPCH where the families didn’t know their child had the blood disorder.
When he was born we spent 32 days in the hospital with pathological jaundice and anemic crisis. Although his anemia is continually monitored with blood tests, much lower red blood cell production (aplastic crisis) can be easily caused by many viral infections, which can make anemia worse.
I also have this disorder and it is treated by removal of the spleen and gallbladder, normally at age five (gallstones are a complication of the early breakdown of the red cells). However, until he reaches age five, he is at risk for normal viruses putting him into anemic crisis. Faelan is lucky as he has stabilized somewhat but his 14-month-old cousin also has the disorder and has just received his tenth blood transfusion.
Faelan’s Blood Use
Common childhood viruses have caused Faelan’s hemoglobin to dip to five and below (should be 13-17 in children). So he has needed immediate emergency transfusions. To date Faelan has received three full blood transfusions that literally saved his life. He had gone into anemic crisis where he becomes like a rag doll, pale as a ghost, you can see right through his ears, he can’t lift his head, can’t feed, can’t even cry for what he needs. One of the signs is that he is hard to wake, very sleepy and not responsive. Most parents love that their kids nap or are good sleepers we have to be careful of him napping too long or being hard to wake as it’s often before he gets sick. Sometimes his hemoglobin dips to eight and he is borderline for a transfusion at which point we wait to see how he responds to medicine or steroids.
The bag of blood though, is like a battery just charges him right up! I see him pink right up, get more alert, start feeding and getting ready to explore like any normal toddler. When you see him running around and joking and sitting on his brother wrestling, it’s hard to believe he has gotten so sick. We are very blessed as we know it could be a lot worse and it’s very manageable. It’s only manageable, though, because strangers donated blood to give to children like Faelan. Without those magic batteries he wouldn’t be alive. When he gets his spleen out he will also need a lot of extra blood in the OR and at any time that he gets too sick. I can’t donate as I have the same disorder plus my time in Ireland disqualifies me due to mad cow scare. My husband is in a different blood group than Faelan so we literally depend on the generous hearts and blood of strangers.
How I got involved as a blood drive coordinator
I’m the Senior HR Business Partner for Nimsoft, a CA technologies company in Campbell. One of my staff mentioned that there was a bloodmobile at one of our Redwood City offices, so I thought it would be great to organize a local drive at work. Many people want to give they just don’t have time to go off during the day so bringing it to them seemed the right way to go.
I contacted Stanford Blood Center and my real estate management team at Pruneyard Equity and asked them to all come to a meeting. We planned a date for May 2012, I got set up as a coordinator, and got the ball rolling. I asked my husband to design a T-shirt we could give away as I knew that would be a good draw. (Below, Faelan wears the shirt proudly.)
I advertised on Facebook setting up an event, put it on LinkedIn, Twitter, and G+, sent notice at work, and sent details of Faelan’s story to local newspapers for Campbell. I also emailed everyone I knew and asked them to spread the word even if they couldn’t donate. Pruneyard – Equity put up posters at work and sent out notice to tenants. Before we had the posters up I had all appointments full and had to ask they extend the drive and bring more staff. We had a great first drive and had to turn away folks. The t-shirts and giveaways were a big hit and I’m hoping we do as well for our next drive on August 14. I know August is harder but we’re at 30 appointments now for the goal of 31. Be great if we could fill all 48 slots.
I can’t donate myself but like I said that doesn’t stop me from getting others out to do this. My sister, dad and the others in our family who can donate donate every eight weeks like clockwork. My dad was awarded a special pelican statue in Ireland years ago at a special award dinner with our Irish president for donating the gallons he has donated over the years. So it’s in our blood to donate for sure ☺