Your Guide to Navigating HLH
Caring for someone with primary hemophagocytic lymphohistiocytosis (HLH) can involve ongoing and complex challenges. While this condition is rare and serious, trusted resources are available to help support families at different points along the way.
The sections below provide information about HLH so you can explore aspects of care that feel most relevant to you, your loved one and your family’s needs.
HLH Caregiver Bill of Rights
Caring for someone with HLH can feel challenging, but knowing your rights can help you stay hopeful and grounded.
Caregiver Discussion Guide
Conversations about care can feel daunting, but you don’t have to do it alone. This guide helps you find the words and the confidence to begin meaningful discussions about HLH caregiving.
The Healthcare Professionals You May Meet
Many types of healthcare professionals are involved in the diagnosis and treatment of primary HLH. Each specialist plays an important role in helping your loved one get the care they need, from diagnosis through follow-up.
Histiocytosis Foundation Physician Finder
Finding a doctor who understands HLH and related conditions can make a world of difference. Use this search tool to locate specialists experienced in diagnosing and treating histiocytic disorders.
Caring for Yourself at Home
Transitioning from hospital to home can be both a relief and a source of stress. This guide offers ways to maintain a safe environment, protect your loved one’s recovery and take care of your own health.
Caring for Caregivers
Caring for someone else can mean your own needs sometimes get pushed aside. This article offers practical tips to help you recharge, move more and protect your health as a caregiver.
Support for Rare Disease Caregivers
Emotional highs and lows can accompany caring for someone with a rare disease. Find caregiver resources focused on self-care, helping you manage stress and feel supported.
HLH Community Support and Advocacy Overview
Connecting with others who understand can make all the difference. This guide highlights advocacy groups, community resources and ways to find support throughout your HLH journey.
Connecting Caregivers to Respite
You deserve care, too. Explore opportunities for respite designed to help caregivers recharge, find balance and feel supported in the journey ahead.
Putting It All Together
Making sense of HLH
After an HLH diagnosis, even the name of the condition can feel overwhelming. Here’s a simple way to think about it. Hemophagocytic refers to the “eating” of red blood cells and also of platelets and white blood cells. Lymphohistiocytosis refers to a buildup and overactivity of certain types of white blood cells and immune cells. Putting it all together, “hemophagocytic lymphohistiocytosis” refers to a condition characterized by an abnormal activation of immune cells, particularly histiocytes. Let’s break it down further.
- An eater of particles
A macrophage is a cell whose job is to clean up the “mess” in our bodies. (It’s a type of phagocyte — an eater of particles.) - Working with T cells
Macrophages work with T cells. A T cell is a type of lymphocyte (white blood cell) that seeks out the mess caused by an infection in our bodies and alerts the macrophages to what it finds. - Releasing cytokines
The T cells do this by releasing messenger proteins called cytokines. In a healthy individual, the macrophage then cleans up the mess made by the viral infection by engulfing it. - Macrophages cause damage
In a person with HLH, something goes wrong and the macrophages react inappropriately. They become “angry,” they cause damage and they start to eat various other cells. They eat red blood cells and others, including the T cells that they should be working with. - Inflammation
The result is a devastating inflammatory response. Inflammation is sustained by the uncontrolled activation of macrophages and high levels of cytokines. - Hemophagocytic syndrome
This disorder is also known as hemophagocytic syndrome. It is most commonly known by the medical abbreviation HLH.
What are the signs and symptoms of HLH?
Understanding these signs and symptoms can be helpful as you navigate HLH. These can occur in both primary and secondary HLH. Their severity and order of appearance may vary among individuals and depending on the underlying causes.
- Fever: The first, and most common, symptom of HLH is raised temperature or fever
- Low blood cell counts: Anemia, low platelets, low white blood cells
- Skin rashes
- Swollen lymph nodes
- Neurological symptoms: Irritability, seizures or altered mental status
- Gastrointestinal symptoms: Nausea, vomiting, diarrhea
- Respiratory symptoms: Cough, shortness of breath
- Elevated liver enzymes
- Enlarged liver and spleen
Questions you may have during your care journey
During every stage of HLH caregiving, it’s normal to feel uncertain about what comes next. Consider asking your care team the following questions.
- How did you diagnose primary HLH in my loved one?
- Were there specific symptoms that made you think it was primary HLH?
- Did you do genetic testing? What were the results?
- Which types of treatments do you plan to use to treat primary HLH, and why?
- What are the side effects of each treatment you will prescribe?
- What is the goal of treatment?
- How long does treatment usually last?
- Are there any lifestyle changes we need to make?
To get the most out of further discussions with your care team, download Sobi’s full Caregiver Discussion Guide.
Affordability and Access
Sobi is committed to helping patients and their loved ones access the care they need. Our Sobi patient support programs are available to people prescribed a Sobi medication and can provide guidance on insurance and financial assistance to help make treatment easier to manage.
HLH Advocacy and Support Organizations
You are not alone. Connecting with advocacy organizations builds connection and support through shared experiences. These groups offer HLH resources for both caregivers and people living with HLH.
Liam’s Lighthouse Foundation
Raising awareness, education and support for families affected by HLH and other histiocytic disorders.
HLH Heroes Foundation
Providing emotional, financial and educational support to individuals and families facing a primary HLH diagnosis.
Histiocytosis Association
Offering education, community connection and research funding for histiocytic disorders.
Immune Deficiency Foundation
Advocating for, educating and supporting individuals with primary immunodeficiency diseases.