Three Things that Every Parent Should Know About HLH

The Akin Family at home.

Within the span of eight months, my husband Justin and I lost both of our boys to a condition called hemophagocytic lymphohistiocytosis or HLH.

I often say that we have lived through every parent’s worst nightmare. Twice.

Today, in July 2013, it’s been almost four years since we said goodbye to Andrew, our younger son, and three years since we said goodbye to Matthew, our first-born.

In the time that has passed, I’ve spent countless hours replaying the events of the time we spent fighting HLH. I’ve asked myself what I wish I had known and if there is anything I would have done differently. And, although it is painful to do the “would of, should of, could of” thing, the truth is that if we had it to do all over again, there are a few things that had I known them at the beginning, may have actually changed the outcome for our boys.

The points below are not at all meant to scare you or indicate that every child who has a fever should be tested for HLH, please don’t go there! Rather, I want to simply be a little voice that will perhaps speak up sometime down the road when you hear of a child who isn’t getting better after the flu. Or you hear that a family has received an HLH diagnosis and they’re considering next steps.

So, with that said, here are the things I wish I had known:

1. The initial symptoms of HLH mimic the flu. So many HLH parents’ stories begin the same way: our children are sick, not getting better and we are often dismissed with ‘it’s just a virus, they just need more antibiotics and time, they’ll be fine.’ It is important to keep in mind the most common symptoms of HLH are fevers, enlarged spleen, low blood counts and liver abnormalities. Many pediatricians and family doctors have never seen a case of HLH and they don’t know what to look for. If you think something more is wrong, speak up, push back and if the doctor hasn’t already done it, ask for a blood draw to do a complete blood count or CBC.

2. In my opinion, more than just knowing the list of red-flag symptoms, parents need to listen to their guts. During an appointment after Andrew wasn’t getting better, our pediatrician initially wrote orders for labs the next day but I demanded Andrew get them done immediately. I knew something was very wrong. Within eight hours of that doctor’s visit he was in the Pediatric Intensive Care Unit with liver failure and only a 50% chance of survival. If I hadn’t pushed the doctor for labs immediately, Andrew might not have made it through that night. Parents know their children better than anyone else and that knowledge is valuable beyond measure. Advocate for your child. Ask for further tests and demand they be done quickly. Time is critical with HLH.

3. Care is different from one hospital to the next. With a diagnosis as rare as HLH, the volume of patients treated in a center and thus the amount of experience a medical team has, matters immensely.

While I’ve definitely done plenty of reflecting in the last several years, I’ve been busy too. I wrote a book. We started a foundation and we became parents again via adoption.

Justin and I decided to start to The Matthew and Andrew Akin Foundation within two weeks of Matthew’s death. We had gone away to try and start to grasp everything that had just happened. We had lost both of our children within eight months of each other and we had just lived in and out of the hospital for thirty-three months. During the trip we talked a lot about helping the families who would unfortunately follow in our footsteps. We both knew we wanted to do something in their honor, something with their names attached, all the while letting everyone know what amazing children we had.

Our foundation is now a supporter of the HLH Center of Excellence at Cincinnati Children’s Hospital Medical Center. We want people to know how important it is to seek the care of Dr. Lisa Filipovich and the team of experts at Cincinnati Children’s. We are always grateful when families find matthewandandrew.org and reach out to us for help in getting their child to Cincinnati for care.

If you happen to be the parent of a child who has HLH, please know that you are not alone in this journey. HLH is an aggressive and nasty disease. It usually sneaks up quickly and attacks fast and hard. But it can be fought, it can be beaten and there is no reason to think that your child won’t be the next HLH survivor. Be insistent in your child’s care and seek the experts at Cincinnati Children’s. It is not a guarantee for survival but it surely will increase your odds and that is the best you can hope for. Our biggest regret is not starting our journey there.

I can’t help but smile when I think about our amazing children. They would have adored their little brother. The admiration I have for them and the strength they displayed while they were sick is something I will never fully be able to put into words. I am grateful every day that I had the opportunity to be their mom.

Justin and I hope that people who learn about our story become inspired to become marrow donors and go out in their corner of the world to help others. We have lived through every parent’s worst nightmare, twice, but we are not only surviving, we are thriving. By channeling our grief and sadness into something positive, our children will live on forever and nothing feels better than that.

In September 2013, Justin and Kristin Akin organized a 700 mile bike ride to raise money for the HLH Center of Excellence at Cincinnati Children’s. A documentary was created during the ride. 700 Miles to Hope – An HLH Journey captures the importance of the ride for everyone involved.

 

Kristin Akin

About the Author: Kristin Akin

Kristin Akin lives in St. Louis with her family. She stays busy working on their nonprofit foundation, The Matthew and Andrew Akin Foundation, traveling as a courier with the National Marrow Donor Program and volunteering with the Ronald McDonald House Charities of St. Louis. She loves cooking and entertaining, traveling and just laughing as much as life will allow. In her next life, she will finally be the pilot she always dreamed of being. She hopes her family’s story will inspire many people to join Marrow.org, become regular blood donors and realize that with love, anything and everything is possible.

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Comments

  1. Concerned Auntie August 02, 19:27
    Thank you for this blog and your story. Recently my niece was diagnosed with HLH at an Oregon hospital. The doctors diagnosed her within two weeks of being at the hospital. After reading about this disease I am concerned for her health because after antibiotics and some steriods they released her. Her WBC was 1,700 and she is to return for a follow-up in 5 days. They don't know yet if it is genetic or not. Should the doctors be releasing her so soon? Should they be starting chemo? I don't want them to wait until it's too late. Should I be concerned?
    • Rachel Camper
      Rachel Camper August 04, 15:25
      Hi Concerned Auntie, If you or your niece's parents contact our HLH Center of Excellence directly, they should be able to help answer your questions. Phone: 877-920-3590 or Email: HLH@cchmc.org.
  2. Heartbroken wife December 30, 11:29
    Thank you for your blog. Words cannot express how sorry I am for your loss. I too have lost a loved one to HLH. My beautiful husband of 46 years lost his battle with HLH two weeks ago and I still go over the last 4 months and wonder if we could have done things differently. My husband Allan was an extremely healthy, fit man of 66 in late July. He picked up,a virus which triggered HLH. It took almost 6 weeks to be diagnosed. In the course of treatments he required 9 blood transfusions and was hospitalized in September for two weeks. We were always positive that we would beat this terrible illness. In the middle of November Allan underwent the 2004 protocol. Aggressive chemotherapy.. after only 4 treatments he had to be hospitalized with febrile neutropenia. He passed away on December 14nth from complications. Allan was a wonderful husband, father and a very talented dentist who treated his patients like they were his family. Life will never be the same without him. I have experienced first-hand what HLH can do to a healthy grown man and it breaks my heart that small children and their loving families have to go through this. Hanne
  3. Jessie January 22, 13:48
    Hi Kristin My name is Jessica Wright, when I was eight I was diagnosed with HLH. In 1997 there was no information on this disease at all. I was blessed to have Dr. Filopovich on my team. And Ronald McDonald house for our family. I think that what you are doing is wonderful, and more people should be informed and taught about this. I now have kids of my own and worry every day about them when they get sick, I had to see a geneticist when I got pregnant with my first child, and they claimed I didn't have to worry but as a parent you can't do anything but that. I would love to help raise awareness and help fund a cure.
  4. May March 06, 23:49
    We recently lost our beloved daughter from inherited form of HLH, at the age of two months. She was perfectly fine by the second of January, and passed away 5 days later on the night of 8th.. We're still in shock, we deeply love our daughter and can not conceive what just happened. We're in Canada, it seems our medical center are not as specialized with this disease as Cincinnati hospital, still the medical team have been able to diagnose HLH in very few days. We were optimistic and thought she would have good chances of survival since we catched the disease so early, but her platelets went so low, blood infiltrate her lungs and the disease took her whitout allowing us the chance of even trying... I think the best thing to do to prevent this is to talk a lot about it and spread informations so that the medical professionals can react quickly. I've read your blog and web site, I think you really are helping the cause. If things didn't went so quickly in our case, maybe what you did could have helped save our beautiful angel. Thank you for your courrage
  5. Deb December 03, 19:10
    Thanks you for your story and I am so sadden to hear you lost both of your boys. Our granddaughter was just diagnosed with HLH Nov 3rd . We are pretty sure It is secondary but the genetic results are not back yet. She is in her 5th week of chemo and steroids. I would have never ever dreamed we would be fighting this rare disease. We are hoping to get to Cincinnati soon for more opinions on her treatment. She is still fighting to many white blood cells in her spinal fluid. But each week they are coming down.. The stress of wondering if the HLH will come back is terrifying. I’m just trying to learn all I can about this disease and it’s treatment. I have learned that every story is different, not just 1 protocol....