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HLH: When a Fever Is a Deadly Sign

Henry Bley. Picture taken by his parents in April 2015.

Henry Bley. Picture taken by his parents in April 2015.

Lynnette Satterfield, Editor in Chief

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The Bleys’ joy could not be contained when Henry Bley was born on Jan. 16, 2015. He was a chubby cheeked boy with an abundance of brown hair and beautiful blue eyes. After a three weeks in the neonatal intensive care unit, due to being born eight weeks premature, it seemed like Henry’s health was good despite his rough start.

Henry was a happy baby. He found funny faces and pretty much anything amusing. He started smiling around his third month, and he was described by his parents as a bubbly baby with a great personality, until he died on July 15, 2015.

Everything seemed fine until May, when Henry started having very high fevers. His mother took him to his pediatrician, who told her, as she recalls, “It was just a virus and to push the fluids and make sure he kept having wet diapers.”
Molly did just that, but Henry got sicker. Molly recalls that they were on the phone with the pediatrician two to three times a week because he couldn’t shake the fever, and nothing they did worked. After weeks of suffering from fever, the doctor diagnosed Henry with a double ear infection and croup, even though there was no evidence of either, according to Henry’s mother.
After weeks of Henry showing no improvement, an acquaintance of the Bleys, who was also a pediatrician, told them to take Henry to the hospital and have his blood drawn. This is when the Bleys’ nightmare started. It was hard to draw Henry’s blood because he was dehydrated, even though Henry’s mother Molly had pushed the fluids, and he had continued to have wet diapers. His mother recalls that Henry was pale, but it had happened so slowly, no one had noticed. His red and white blood cells were dangerously low, and the hospital told the Bleys that Henry could have suffered a seizure or some other side effect from this. Molly Bley said that this was the first time Henry had had any blood taken because his pediatrician had never ordered tests in spite of the fact that he had been ill for so long.”

Molly Bley says that night, Henry Bley was admitted to the children’s intensive care unit at Mercy Hospital in St. Louis. An oncologist, a doctor who specializes in cancer, told Henry’s parents that he thought Henry had leukemia. An infectious disease specialist told Henry’s parents that she wanted to run a test for HLH which stands for Hemophagocytic Lymphohistiocytosis, a rare blood disease, but the Bleys were told it was unlikely he had it since HLH is so rare. The Bleys were in shock when 24 hours after being admitted to the Intensive Care Unit on June 5, Henry was diagnosed with from MUNC 13-4, a genetic form of HLH. ”

Henry’s mother tells the story leading up to her son’s death by saying that after a week at Mercy Hospital, Henry was moved to St. Louis Children’s Hospital, where steroids and Cyclosporine and an etoposide, a type of chemotherapy, were started, as was the search for a bone marrow donor. His ferritin, a type of iron marker, started to decline, but then his liver, kidneys and spleen became inflamed. He developed Tens disease from his blood pressure medicine.

Tens Disease “caused big blisters all over his body. There was liquid seeping out of him. They put him in an incubator to protect him from infections because they couldn’t put anything on his skin, like bandages or sterilize the ports because of his skin being so sensitive. If they put anything on it would just rip his skin right off or it wouldn’t stick at all,” Molly Bley said.
Henry’s mother said with emotion in her voice, “[if your child is sick], take it very seriously. Any illness that you are unsure of or if you have any uneasy feeling, immediately go to the emergency room. If they tell you it’s a virus but they aren’t sure what it is go to the emergency room because the emergency room can test for viruses as well as that it could be something more serious. You would think that everyone would think worst case scenario, but no one does. Go with your gut instinct and do it sooner than later. Go to the hospital and if you have to pay just to have that peace of mind that your child has a virus, pay it any day of the week because it could be something like our case.”

Since Henry died, his parents really want other parents to know what HLH is, and his mother is intent on raising awareness. “The sooner a patient who has HLH is diagnosed, the higher the chances are for survival” said Molly Bley.

What Is HLH?

According to the Cincinnati Children’s Hospital’s HLH Center Of Excellence website “HLH is a condition of the immune system in which the T cells and NK cells don’t work properly in order to destroy infected or damaged cells as they should. Because of this, the immune system becomes overstimulated and over activated.”

According to Cincinnati Children’s Hospital HLH Center for Excellence website”[The symptoms of HLH include] persistent fevers, rash, enlarged liver, enlarged spleen, enlarged lymph nodes, anemia, low platelets, low white blood cells, jaundice, hepatitis, liver failure, respiratory issues (coughing, respiratory distress), seizures, and altered mental functions.”

But in Henry’s case and also in many other cases of HLH, the only noticeable thing without substantial labs was his fever, which was mistaken for a virus and infection.

Amanda Majusiak a board member for the non-profit Liams Lighthouse Foundation, said “What has been coined as Secondary HLH is those who are diagnosed where there is no known genetic mutation. Most common secondary causes are mono, autoimmune arthritis and other autoimmune diseases. Treatment is generally the same and may include steroids, chemotherapy or bone marrow transplant. Only a physician can determine what treatment path is right for each patient.”

Liam’s Lighthouse Foundation is non-profit that raises awareness and money for HLH. Liam’s Lighthouse Foundation was founded by Michelle Schulze after her son Liam died from HLH in 2009.

Eric Majusiak “was treated with steroids and chemotherapy. Since his body responded well to the treatment, his attending physician felt it was best not to do a bone marrow transplant at that time. Should his HLH relapse in the future, he will be required to have a bone marrow transplant,” Majusiak said in reference to her husband who has secondary HLH that he acquired because he has form of Rheumatoid Arthritis called Stills disease.

“He didn’t make it that far” Molly Bley said about her son receiving a bone marrow transplant, which is what the Bleys were told was his best chance at survival. A bone marrow match for Henry was not found before he died.

“HLH is a disorder of immune regulation where certain kinds of immune responses become dangerously strong and cause damage to the body. Physicians recognize it by recognizing a specific pattern of damage … Now with genetic testing we find that most infants with HLH do have a definitive genetic cause, in other words they are born with a certain set of genetic abnormalities that predispose them to develop HLH in a very serious sort of way. But we are finding increasingly that older children and even adults also have genetic problems that predispose them to develop HLH. The only real difference for many of these patients is that a lot of these mutations happen to be milder in the individuals that don’t get sick until they are older,” said Dr. Michael Jordan, a researcher at Cincinnati Children’s Hospital in his YouTube video “Overview of HLH” posted by the Histiocytosis Association.

Regardless of whether a patient has primary or secondary HLH, this disease shows how serious a fever can be and how important it is to fight for testing if something feels off. If someone has a fever with no known cause it is important to have blood tests done, and including a complete blood count (CBC) to have the immune system tested, because for the children that lost their lives to HLH, many of them only had showed signs fever for the first few months that they had HLH.

“Liam’s Lighthouse Foundation supports education, awareness and research. Our current initiatives involve awareness, campaigns and large group donations to key hospitals involved in HLH and other Histiocytic disorders in the US (including) HLH center of Excellence at Cincinnati Children’s Hospital and Texas Children’s Hospital Donations help drive key research programs to develop new genes and better treatments. There is a way to make a financial contribution to help the HLH cause by visiting LiamsLighthouseFoundation.org,” Majusiak said.

More Information

Cincinnati Children’s Hospital HLH Center of Excellence: http://www.cincinnatichildrens.org/service/h/hlh/default/
Liam’s Lighthouse Foundation: http://www.liamslighthousefoundation.org/
Histocytosis Association: https://www.histio.org/

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Lynnette Satterfield, Assistant Editor

Lynnette is in her senior year at National University, pursuing her Bachelor of Arts degree in Digital Journalism. She enjoys writing about education,...

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HLH: When a Fever Is a Deadly Sign