How to Live Well With Advanced Systemic Mastocytosis
A diagnosis of advanced systemic mastocytosis (advSM), a rare blood disorder in which too many mast cells accumulate in various parts of the body, can cause a range of strong emotions. AdvSM sometimes evolves into a form of cancer.
The prognosis for advanced systemic mastocytosis is daunting: It most often includes a shortened life expectancy and difficult-to-manage symptoms. But many people with the condition have lived with unexplained symptoms for years, and moving out of uncertainty and beginning the appropriate treatment can feel like moving forward.
The diagnosis presents a set of practical concerns: You’ll need to figure out how to find doctors who know how to treat it, manage your medications, tackle financial and insurance questions, and a lot more.
This guide will help you understand some of the challenges you’ll face, whether you are making decisions for yourself or are the loved one or caregiver of a person living with advanced systemic mastocytosis.
A healthy lifestyle is always a good idea, but it’s especially important when you have advanced systemic mastocytosis, says Aaron Gerds, MD, a hematologist and oncologist at Cleveland Clinic in Ohio.
“Following the basic ‘common sense’ recommendations on a balanced, healthy diet, and regular physical activity is incredibly important for a disease of this nature,” says Dr. Gerds.
Not only will this improve your overall health and quality of life, it may also make you eligible for more treatment options for advSM, he says.
“For example, if you had severe heart disease, and we thought that a certain treatment might help your condition but could potentially make the heart disease worse, it may preclude you from getting the treatment for the disease. So I think maintaining your health and wellness otherwise is one of the most important things that patients can do for themselves in the face of this disease,” says Gerds.
Current guidelines don’t suggest any special diet for people with advanced systemic mastocytosis, but some experts recommended a low-histamine diet, especially for people who have systemic mastocytosis that hasn’t developed into an advanced form of the disease.
For most people with advanced systemic mastocytosis, following a healthy and balanced diet that supports overall health is recommended, says Jason Gotlib, MD, a professor of hematology at Stanford University School of Medicine and a hematologist at Stanford Health Care in California.
Unless symptoms or medication side effects make it too painful or difficult, Gerds recommends that people continue to engage in physical activity.
It’s important to note that exercise can trigger symptoms, because it raises the body’s internal temperature and is a form of stress on the body. Strenuous exercise, especially in hot or humid conditions, usually isn’t a good idea.
Systemic mastocytosis can be unpredictable, in part because of triggers that can worsen symptoms. And having to be “on guard” 24/7 is psychologically trying.
When spending time outside, people with advanced systemic mastocytosis need to avoid insect bites (such as ant bites) and wasp and beestings, which can cause a potentially life-threatening allergic reaction. Sudden temperature changes are also triggers.
Traditional treatments for advanced systemic mastocytosis focus on managing symptoms and preventing complications, while newer medications are aimed at improving outcomes and survival.
Treatment plans for advanced systemic mastocytosis are highly individualized but may include pain medications, drugs to help with GI symptoms, and drugs to treat complications such as osteoporosis.
In the last few years, tyrosine kinase inhibitors (called KIT inhibitors) have really transformed treatment of advanced systemic mastocytosis, says Dr. Gotlib.
“These drugs are very effective in reducing and killing off the number of mast cells and reducing elevated tryptase levels, and sometimes reversing organ damage,” he says.
They also have the convenience of being an oral medication rather than an infusion, which makes treatment more accessible and convenient, says Gerds.
The symptoms of advanced systemic mastocytosis can be moderate to severe and depend on the number of mast cells and where they are located, says Gotlib.
Study authors concluded that the symptoms of advanced systemic mastocytosis are often more bothersome that many doctors realize. If you feel as though your medications aren’t managing your symptoms, make sure you discuss your options with your healthcare team.
To have the best possible outcomes and care, it’s important to have a doctor or caregiver who is knowledgeable in advanced systemic mastocytosis, says Tsewang Tashi, MD, a hematologist, oncologist, and the leader of the mastocytosis program at the Huntsman Cancer Institute at Utah Health in Salt Lake City.
“This is a rare disease, and not every cancer center may have someone who is able to manage this condition,” says Dr. Tashi.
If possible, that means finding a hospital that has been deemed a Center of Excellence by the American Initiative in Mast Cell Diseases, he says.
This is a center where you have a multidisciplinary group of physicians that may include:
It can take years of unexplained symptoms before a correct diagnosis of advanced systemic mastocytosis is made, says Gerds. “The period of going around and around before you ultimately get the diagnosis can be quite exhausting and emotionally draining,” he says.
Research suggests that depression and anxiety are more common in people with advanced systemic mastocytosis. A meta-analysis found that up to 75 percent of people with mastocytosis experienced depression and up to 27 percent reported anxiety.
“The reality of living with a cancer diagnosis is likely a major factor as well. It can weigh heavily on one's mood and be a psychological burden,” says Gerds.
It’s common for people with advanced systemic mastocytosis to think about how much time they have left to live, according to interviews with patients about what it was like to live with the disease.
Interestingly, many people say their doctor seldom addressed the question, and some people were told they had very limited expected survival but were still alive several years later.
“This is another aspect that makes the diagnosis of a rare disease difficult. It's not like you can go down the street and find four other people with systemic mastocytosis. If you have something like high blood pressure or diabetes, you may have friends and neighbors who are going through a similar and shared experience to connect with, but not with this, because it's just so rare,” says Gerds.
People with advanced systemic mastocytosis are often concerned that even their healthcare providers lack an understanding of the disorder. Some participants in the study referenced above about living with advanced systemic mastocytosis described how they were refused treatments for other conditions like operations or dental surgery because of unnecessary fears of systemic mastocytosis-related complications.
These knowledge gaps put many people with advanced systemic mastocytosis in the position of being an “involuntary expert” who has to educate friends, curious strangers, and even healthcare professionals.
Advanced systemic mastocytosis means feeling vulnerable, and the support of family and friends can be an important aspect of overall well-being. In interviews, patients express the value of having a friend or spouse to discuss their fears and symptoms.
“My husband doesn’t think it’s a big deal,” said one woman with SM. “I can say to him, ‘Look how awful my legs look!’ And he’ll say ‘What? That’s not a big deal.’”
Whether or not people with advanced systemic mastocytosis can continue to work depends on the affected organs and the symptoms they are experiencing, says Gerds.
“If a person feels well enough to work, there’s no reason they can’t continue to do so. But the symptoms of disease can be quite burdensome for some patients and may limit their ability to do their job,” he says.
Many people with advSM can do quite well on KIT inhibitors, and their symptoms improve dramatically, says Gerds.
“And because they are taken orally, they don’t have to go to an infusion clinic to get treatment. That gives people more flexibility in their day, so they are able to work if they feel able to do so,” he says.
Some people with advanced systemic mastocytosis may be able to get disability benefits through the Social Security’s Compassionate Allowances program, which enables people with certain types of cancer to qualify for Social Security Disability Insurance. There are different criteria for qualifying, including how long you’ve worked and your prognosis. You can get more information on the Social Security website.
Although there have been life-extending breakthroughs in the last few years, there are still many unanswered questions about treatments that need answered, says Tashi.
“That is exactly why we encourage people with advanced systemic mastocytosis to enroll in clinical trials — to help us better understand and treat this very complex disease,” he says.
Ask your provider or search ClinicalTrials.gov, a searchable database of studies around the United States and more than 200 countries to find out if there are any nearby clinical trials that are recruiting participants.
The people who tend to do the best with this very challenging diagnosis are those who strike a balance, says Gerds.
“They are mindful and attentive to their disease, they notice changes in their health and symptoms, but they don’t let it completely rule their life,” he says.
Even though the disease is rare, you can find online or in-person support groups, as well as other services to help you deal with the diagnosis and treatment, says Gerds.
“Many health systems offer programs such as art therapy, music therapy, and yoga,” he adds.
These organizations can also help connect you with social and practical support:
This nonprofit organization has an online tool to locate support groups in your community, as well as a peer-to-peer support program for those diagnosed with a blood cancer.
This national organization provides free support services and information for people living with cancer. They offer online support groups that are open to anyone in the United States, Puerto Rico, and U.S. Territories, and live support groups (in-person or via videoconferencing) for residents of New York and New Jersey.
The Mast Cell Disease Society offers a variety of patient support resources as well as an online tool to help you find a Center of Excellence in your area.
©2025 sitename.com All rights reserved