What To Know About Managing Plexiform Neurofibroma Symptoms

Plexiform neurofibromas are one of the many kinds of tumors that people living with neurofibromatosis type 1 (NF1) can develop. They form on your nerves’ protective coverings and can grow to be quite large. Most plexiform neurofibromas are noncancerous. But about 15% develop into malignant peripheral nerve sheath tumors (MPNST).

In some cases, plexiform neurofibromas are a minor inconvenience or cosmetic concern. In others, these tumors can be disabling or even life-threatening. It depends where in your body they’re located, how large they get and if they become cancerous.

It’s hard not knowing what the future holds. And you can’t prevent neurofibromas from forming. But there are things you can do to ensure you get the best possible care. Neurosurgeon Ajit Krishnaney, MD, explains.

Tips for managing plexiform neurofibroma symptoms

Have you ever heard the phrase “The best offense is a good defense”? It’s especially true when comes to managing plexiform neurofibroma symptoms.

“NF1 is a complicated disease to manage because most people who have it have more than one neurofibroma at a time. They often have more than one type of neurofibroma, too,” Dr. Krishnaney says. “Plexiform neurofibromas, specifically, are very slow-growing tumors. Many pop up and stay the same size for years.”

Nobody can predict how a plexiform neurofibroma will develop or how it will impact you. Because every case is unique, there’s no one way to manage plexiform neurofibroma side effects. But if you want to protect yourself against complications, Dr. Krishnaney recommends you:

• Build a solid care team
• Monitor your symptoms
• Take a proactive approach to your health

Build (and communicate with) your neurofibromatosis care team

Most NF1 care teams start with a neurologist or a neuro-oncologist. (Children with NF1 will see a pediatric specialist, if one’s available.) They’re the hub: The person in charge of coordinating your care with all the other specialists. That’s because all neurofibromas involve your nervous system. And while plexiform neurofibromas don’t affect your brain or spinal cord, other tumors can.

Additional members of your multidisciplinary team may include:

• A mental health professional. They’ll teach you the coping skills you need to stay resilient in the face of a chronic illness.
• A plastic surgeon. Some neurofibromas can impact your appearance. A plastic surgeon may be able to reduce the tumor’s impact on your physical features.
• A physical or occupational therapist. Physical therapy and occupational therapy can help you address any challenges that your tumors create. And if you have tumors removed, they can improve your chances of a successful recovery.
• A pain management specialist. As plexiform neurofibromas grow, they can press on other tissues, causing pain. Pain management specialists help you address those challenges in a way that’s safe and sustainable.

Other specialists will join your team if you develop a new tumor. For example, let’s say you develop a neurofibroma on your esophagus. Your team might grow to include a gastroenterologist or gastrointestinal surgeon. If you have trouble swallowing, you’ll work with a speech-language pathologist. A dietitian might enter the picture too, to help you get the nutrition you need.

“It’s very, very important not to be afraid of your doctors,” Dr. Krishnaney urges. The members of your care team will be with you for a long time. Get to know them. And don’t hesitate to ask them tough or embarrassing questions. We promise: They’ve heard it all before.

One more thing: It can be frustrating going to regular screenings when you don’t feel like anything’s wrong. But a boring appointment is a good thing. And skipping them could be dangerous.

“A lot of what we do is trying to catch tumors early, evaluate them and — if they’re starting to be bad actors — treat them,” he explains. The longer you go between check-ups, the higher the risk of a neurofibroma causing permanent damage.

Advocate for yourself

If you’re living with a rare condition like NF1, it can be easy (and comforting) to hand control over to your providers. But educating yourself, asking questions and playing an active role in your care helps ensure you get what you need.

“Catching these things early and being up to date on the most cutting-edge research is probably the best available medicine out there,” he notes. That’s why Dr. Krishnaney urges you to get care at a medical facility that specializes in the condition.

“If you can get plugged in to those resources earlier, when your tumors are smaller and more manageable, there’s more we can offer you — and more we can do to help you manage your symptoms,” he adds.

One of the most important things you get from working with neurofibromatosis specialists is access.

“There are new therapies being tested to try to help slow or stop the growth of different neurofibromas,” Dr. Krishnaney shares. “NF1 specialists are aware of these therapies and can help you enroll in clinical trials.”

Keep a symptom journal

Tracking your symptoms is a daily habit that helps your providers manage your care. The easiest way to monitor your health is to create a symptom journal. You can do this with a regular notebook or through an app. You can even download symptom tracker templates online.

Symptom journaling helps you see patterns and changes in your health over time. Dr. Krishnaney says it’s usually best to leave a plexiform neurofibroma alone until it grows, changes or starts impacting your quality of life. So, it’s important to be aware of what’s happening in your body.

“People with plexiform neurofibromas often know that one of their tumors is changing before we do because they can feel the difference,” Dr. Krishnaney recognizes. “In some cases, they start experiencing discomfort before we can even see the changes through imaging studies.”

Talk to the members of your care team to determine what kinds of information they’ll find most helpful. And alert them if you notice a change or trend in symptoms:

• Type
• Severity
• Frequency
• Location
• Duration

“Symptom tracking is really helpful because we can catch things earlier,” he continues. “It’s especially helpful in cases that involve organ systems. It means we can try to preserve the organ’s function and address your symptoms. If those tumors get too far along, they can sometimes cause permanent damage.”

Stay as active as possible

Depending on the size and location of your plexiform neurofibromas — or any other neurofibromas you’re dealing with — may prevent you from exercising the way you want. But movement still matters.

Dr. Krishnaney emphasizes the importance of staying active in whatever way is safe for you.

“It’s the use it or lose it principle,” he explains. Flexibility, balance, strength and more are crucial to our overall health. Find physical activities that you enjoy doing and do them consistently. And remember: It’s OK to start slow. 10 minutes of chair exercises may not seem like much. But it’s more than nothing!

Not sure what kinds of exercise are safe for you? Ask your physical or occupational therapist.

An active lifestyle is an important part of plexiform neurofibroma symptom management. So is rehabilitation. If you have surgery to remove a tumor, keeping up with your physical or occupational therapy program is critical.

“Being aggressive about doing physical therapy [PT] when you need it can help you maintain or even regain function,” Dr. Krishnaney states.

PT should be challenging but not painful. Your physical therapist can adapt your program to fit your needs. So, be clear about how each activity affects you.

Don’t shy away from assistive devices

Another way to preserve your independence is to use assistive devices. Assistive devices are tools that help you perform activities of daily living (ADLs). Glasses, hearing aids, grab bars and wheelchairs are just a few examples. People with NF1 often need to use assistive devices — sometimes temporarily, sometimes permanently — to address the impairments their tumors cause.

“A physical or occupational therapist can help you figure out how to modify your environment and activity patterns to create a new normal,” Dr. Krishnaney encourages.

Losing function, even for a short time, can be a bitter pill to swallow. And internalized ableism sometimes makes it feel like having disabilities or needing extra help is weak.

It’s not true. Assistive devices help us preserve our independence and quality of life. And, according to Dr. Krishnaney, that makes them important for your physical and mental health.

When to see your doctor

Neurofibromatosis is an unpredictable, systemic condition that causes a wide range of symptoms. So, how do you know when you need to see your provider?

Dr. Krishnaney takes a practical approach.

“If you give it a few days and it gets better, it’s probably fine,” he says. “We all get aches and pains sometimes. But if it’s not getting better after a few days or a week, that’s when you should call your provider.” (That symptom journal comes in handy in these moments.)

The better your care team knows you, the easier it will be to assess your symptoms.

“Having an experienced neuro-oncologist or neurologist follow you long term is important,” he emphasizes. “They’ll know your usual symptoms and where your tumors are. They’ll also have a sense of your personality. That insight will help them respond appropriately to new or changing symptoms.”