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Coping With An HLH Diagnosis

Friends Holding HandsAll families dealing with an HLH diagnosis will experience a range of emotions as they begin to process the full spectrum of what their young patient will face.  On an individual basis, it should be expected that each family member will have their own personal reaction, as well as an individualized set of ways in which they will process and cope during this time.  No one way of dealing with the situation is right or wrong and it should be stressed that everyone’s emotions are perfectly normal, expected, and should be treated in a respectful manner.

To begin to understand what this process will look like, it’s usually best to reference what is called the grief-cycle.  This cycle of emotions includes – denial, anger, depression, and fear.  These emotions are all perfectly normal for those facing a rare-disease diagnosis due to the tremendous amount of stress involved.  It is also important to remember that all individuals will experience “good days and bad days.” Encourage those going through these ups and downs by reminding them that they will find relief with the passing of time.

As a part of the coping process, each member of your family will begin to understand how they best deal with the situation and will thus find their preferred coping method.  Some of these methods of coping may include: finding social support; partaking in individual counseling or therapy; becoming educated on the disease; and/or acting as an advocate for the patient.

For those who are looking to social support, it is important to remember that asking for help is okay and that graciously taking any help offered to you is okay also.  You will additionally want to connect to your significant other or to another important person in your life by communicating daily.  Even if you only have a few minutes to talk, those daily moments will keep help you to stay connected and provide a sense of normalcy to your life.

Individual counseling or therapy is another effective way to communicate your feelings with someone who can support you in working through them.  Ask your child’s heath care provider, or other parents, for a recommendation.

Educating yourself about the disease your loved one is fighting will help you to understand the situation they are facing in a whole new way.  Education is often a way for individuals to feel like they have more control over a situation.  This is because it allows them the chance to know more about what the patient will experience as they undergo treatment for their disease.

Finally, becoming someone’s advocate is a great way to get involved and make a difference.  As a part of this process you’ll need to do some research, ask questions, and develop relationships with the medical professionals around you.  This type of support is crucial and everyone around you will benefit from your belief in the patient’s full recovery.

If you are a family facing an HLH diagnosis, don’t feel like you are alone.  The volunteers at the Matthew and Andrew Akin Foundation want to connect you with the support you need to make informed decisions while fighting this disease.  We encourage you to explore the services provided at www.matthewandandrew.org, as well as those provided by the HLH Center of Excellence at Cincinnati Children’s Hospital.

How Cincinnati Children’s Is Diagnosing HLH

Writing Diagnosis With Chalk On A Blackboard.

At Cincinnati Children’s Hospital, the HLH Center of Excellence is committed to getting children diagnosed with hemophagocytic lymphohistiocytosis or HLH as quickly as possible. Unfortunately, this task is often harder than expected due to the fact that HLH can look a lot like a number of other conditions.  For this reason, HLH often goes overlooked for some time causing the treatment of an HLH patient to become even more urgent.

The symptoms all doctors and parents should be looking for when evaluating someone for 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, seizures, and altered mental functions.  Having any number of these conditions does not mean you are dealing with HLH, but understanding that they may point toward HLH is imperative.

To properly diagnose HLH, Cincinnati Children’s Hospital recommends proper blood tests that indicate blood cell counts, liver function numbers, and immune system activation markers including ferritin and soluble IL-2 receptor levels.  It is indicated that doctors may also perform additional testing like X-rays, CT scans, ultrasounds, and MRIs so that they can look for infections that can be related to HLH.  A patient may even undergo specialized blood tests designed to rapidly screen for the genetic causes of HLH.

Due to the discouraging fact that HLH often gets misdiagnosed or overlooked, it’s so important to continue educating pediatricians and parents to look for this life-threatening disorder.  By focusing its efforts on a mission centered on education, the HLH Center of Excellence at Cincinnati Children’s Hospital is getting the word out.  Thanks to educational efforts like professional lectures, published articles in professional journals,  and the annual HLH conference held at the hospital, Cincinnati Children’s has been able to increase HLH awareness.

Cincinnati Children’s is also focused on increasing HLH awareness via recruiting and training new physicians in this specific area of study.  As the next generation of HLH specialists enters the field, the center intends to build a team by encouraging fellows to focus on HLH research, by competitively recruiting, and by supplying educational sessions at professional society meetings.  All of these efforts are important to ensuring the continued research and development of the most-effective diagnosis and treatment solutions for those suffering from HLH.

The HLH Center of Excellence also partakes in efforts to raise awareness outside of the medical profession by partnering with advocacy groups like the Matthew and Andrew Akin Foundation. At the Akin Foundation, Matthew and Andrew Akin’s parents work with other volunteers to raise awareness and funds that are used to support HLH research, HLH education, and to support families dealing with an HLH diagnosis.  These efforts are backed by a number of fund-raising events including the 700 Miles to Hope bike ride.  During this bike ride, participants pledge to travel 700 miles over the course of seven days, all while raising money and awareness for HLH.  All donations are then given to the HLH Center of Excellence at Cincinnati Children’s where it is used to support their on-going, HLH-centered mission.

If you are a physician, parent, or friend of someone facing an HLH diagnosis, the Matthew and Andrew Akin Foundation wants you to get in touch with the amazing team of HLH doctors at Cincinnati Children’s.  The Akin Family understands how important it is to receive top-notch care when dealing with HLH and they encourage you to do your research.  Please feel free to contact the Foundation with your questions or concerns, or to additionally reach out to Cincinnati Children’s directly.  Children suffering with HLH are truly one in a million.  Get them the love and care they rightly deserve by contacting the specialists at Cincinnati Children’s.

Exploring New Possibilities In Treating HLH

Research. Wordcloud Concept.At Cincinnati Children’s Hospital, the HLH Center of Excellence team is committed to continually making strides in improving the diagnosis, treatment, and outcome for those suffering from the immune disorder HLH.  Through studies and trials, physician-scientists get closer to finding treatments that offer a more effective, and less toxic alternative to what is used currently.  HLH Center of Excellence physician, Dr. Michael Jordan, is counting on his Hybrid Immunotherapy for HLH trial to provide just that for more and more patients with HLH.

The Hybrid Immunotherapy for HLH or HIT-HLH trial is designed around a combination therapy approach.  This hybrid immunotherapy utilizes two treatment approaches in an effort to calm the inflammatory response that happens during the first stages of HLH.  Dr. Jordan’s HIT-HLH trial is the first-ever U.S.-based trail for HLH.  It is also the only trial funded by the National Institutes of Health or NIH.  According to Dr. Jordan, “This study focuses on controlling the disease process, not fixing the underlying problem.  But we believe it may give more rapid and complete control of inflammation. We hope it will be a real improvement.” 

When utilizing this treatment, doctors use a unique combination of three drugs.  These three drugs include: anti-thymocyte globulin (ATG), etoposide, and dexamethasone.  Ten centers throughout the U.S. and Canada are currently conducting HIT-HLH trials with an initial enrollment totaling 12 children.  Dr. Jordan and his team have reported pleasing results with the early stages of this trial and plan to enroll up to 40 more patients in the future.

Dr. Jordan and his colleague, HLH expert, Dr. Lisa Fillipovich have additionally paired up with other internationally-based researchers and a Swiss pharmaceutical company to test the effects of an antibody named, NI-0501 on the protein gamma interferon (IFNg).  It has been determined that this protein is greatly elevated in HLH patients and that the NI-0501 antibody has the ability to target and neutralize the protein.  The theory surrounding these findings is that elevated IFNg causes HLH. According to Dr. Jordan, if this promising theory proves true, doctors could potentially target the protein and shut-down the HLH disease process.

Thanks to the efforts of Cincinnati Children’s physician-scientists, both of these exciting trials offer the hope and promise needed by so many suffering with HLH.  Along with these efforts, the HLH Center of Excellence is also focusing on developing an HLH gene chip, preclinical studies of gene therapy in PRF1 deficiency, and preclinical trials of small molecule target therapy.  It is additionally the intent of Cincinnati Children’s to build their collaborative relationships to spread across Europe, Central American, South America, and Asia.  The center is currently working with the National Institutes of Health, along with HLH centers in China, Japan, Vietnam, Canada, and Sweden.  As these efforts expand, it is no doubt that more children suffering with HLH will be diagnosed, receive treatment, and have the chance to beat this horrible disease.

The research taking place at the HLH Center of Excellence at Cincinnati Children’s Hospital is supported partially by the efforts of the Matthew and Andrew Akin Foundation.  At the Akin Foundation, dedicated supporters raise awareness and monetary support to help those with HLH immune disorder via research, education, clinical care, and family support.  Thanks to these efforts, strides are being made in the fight against HLH.  If you are interested in joining this fight or know someone who may be suffering from HLH, the Akin Foundation can help!  Please feel free to explore this site or to make conduct with the foundation via phone or email.