NF Community: Questions and Answers
Welcome to the Tumour Foundation of BC's vibrant community question platform
Welcome to the Tumour Foundation of BC's vibrant community question platform.
This page is a hub of valuable insights generated from questions posed by individuals living with NF through social media and our dedicated form.
Do you have a health-related query related to NF?
We encourage you to share your questions using the button below. Our experienced nurse is committed to providing thoughtful responses to your NF-specific inquiries.
Please note that while our nurse offers valuable general advice, it's essential to consult your healthcare professional for personalized medical concerns. Your health and well-being are important to us.
DISCLAIMER: The information provided on the website is for informational purposes only and should not be considered as medical advice. Please consult a healthcare professional for personalized medical guidance.
Factors Contributing to
Worsening Symptoms with Age
Research on Mushroom
Benefits for Tumour
Management in NF
Question: Why does NF get worse with age?
Answer:
NF affects individuals differently, with some experiencing mild symptoms, while others have more complex medical issues because of the disease.
There are several factors that can worsen NF symptoms with age:
- NF tumours tend to grow over time and this growth can increase the pressure on nerves, tissues, causing increased pain, weakness, and other complications.
- As people with NF age, the growing tumours can cause hearing or vision loss, mobility issues, and other neurological problems.
- Over time, NF can lead to conditions such as scoliosis of the spine, high blood pressure, and learning disabilities.
- As individuals with NF age, the effects of tumour growths and the complications caused by these tumours add up leading to worsening symptoms and a decline in overall health
- Common health conditions associated with ageing include hearing loss, vision loss and cataracts, back and neck pain and osteoarthritis, diabetes, depression, dementia and respiratory disease, these combined with the challenges of NF can further lead to decreased functioning.
It is important for individuals living with NF to strive to achieve optimal health and wellness at any age. Seeking regular medical care, health screening and monitoring tumours and symptoms of the condition are vital. In addition, patients may need to access specialists to address specific NF challenges as they emerge. These actions will help promote optimal health outcomes. Regular mammograms for female patients, geneticist referrals, eye and hearing exams, bone density tests, neurology assessments, dermatology assessments, are some examples of medical care that some NF patients may need. Maintaining a holistic approach to health including self-care, elements of nutrition, exercise, socialization, spirituality, and a positive outlook are beneficial to healthy ageing.
Question: Have there been any research studies investigating the potential benefits of mushrooms in managing tumour growth for individuals with NF?
Answer:
While medicinal mushrooms have shown promising health benefits, there is limited research addressing their use or potential benefits in managing Neurofibromatosis. According to the NIH-National Cancer Institute, medicinal mushrooms have been used to treat infection, lung diseases and cancer. These mushrooms have been approved as an enhancement to cancer treatments in Japan and China, sometimes on their own or in addition to radiation or chemotherapy. In Asia, there are over 100 types of mushrooms used in the treatment of cancer. Studies are progressing to learn more about how mushrooms affect the immune system and how they could potentially slow the growth of tumours or eradicate them altogether. Compounds in turkey tail mushrooms are believed to strengthen the immune system to fight cancer. To learn more about the use of medicinal mushrooms, please visit the link below:
It is important to note that medicinal mushrooms may interact with medications and other treatments. Patients need to consult with their health care provider prior to starting any new treatment regimen and prior to incorporating alternative health care therapies.
We look forward to ongoing research into the use of medicinal mushrooms and their potential benefits for conditions such as Neurofibromatosis.
MEK Inhibitors for Plexiform
Tumour Treatment
Fibromas: Effective Alleviation
Strategies
Question: Q: Can MEK inhibitors be used preventively for treating plexiform tumours?
Answer:
MEK inhibitors such as Koselugo (Solumetinib) have shown promise in treating various types of tumours, including plexiform tumours, which are a characteristic of neurofibromatosis type 1 (NF1). In this genetic disorder tumours form along nerves throughout the body.
While MEK inhibitors have demonstrated efficacy in reducing tumour size and improving symptoms in some patients with NF1-associated plexiform tumours, their use as a preventive measure has not been established. Preventive treatments aim to stop the formation of tumours before they occur, while MEK inhibitors are typically used to shrink existing tumours. “KOSELUGO® (selumetinib) is a prescription medicine that is used to treat children 2 years of age and older with neurofibromatosis type 1 (NF1) who have plexiform neurofibromas that cannot be completely removed by surgery. It is not known if Koselugo is safe and effective in children under 2 years of age.”
Presently, MEK inhibitors are primarily used as a therapeutic option for symptomatic patients with existing inoperable plexiform tumours rather than as a preventive measure. Future clinical trials may provide more insight into their potential preventive effects.
Question: Q: What strategies are effective for alleviating the itchiness associated with cutaneous fibromas?
Answer:
Cutaneous neurofibromas are benign tumours that affect the skin of many individuals living with Neurofibromatosis type 1. These tumours can form on or just under the skin and vary in size and number. The cutaneous neurofibromas grow on the nerves in the skin, are typically non-cancerous and do not normally cause any pain or health problems. In some individuals cutaneous neurofibromas cause itching, discomfort, or cosmetic concerns.
Strategies for alleviating itchiness caused by cutaneous neurofibromas include protecting and enhancing the natural skin barrier through skin moisturizers and by avoiding irritants such as fragranced and drying cosmetic products. Topical treatments and medications such as antihistamines may be prescribed by a physician and a referral to a Dermatologist is often beneficial. Surgery, laser, or electrodessication may be considered for fibromas that are increasingly bothersome.
Here are important tips on protecting your skin with cutaneous neurofibromas:
It is important to keep the skin as healthy as possible, this will decrease inflammation, itching and pain.
1. Avoid using wash cloths or irritating skin care products, such as exfoliators.
2. Use fragrance-free gentle soap.
3. Moisturize the skin daily with thick ointments or creams that are fragrance free. Examples: Aveeno, Dove sensitive skin, CeraVe.
4. Use sun protection daily, such as broad-spectrum SPF sunscreen and longer clothing.
5. For itchiness not relieved by gentle skin care alone, please consult with your doctor for possible topical or oral medications.
Please refer to free downloadable resource provided by The Tumour Foundation of BC for tips, strategies, and self-monitoring of your cutaneous neurofibromas. Check out this resource for more information.
Neuroma Risk
Question: Is there a specific age range where the likelihood of developing acoustic neuromas decreases if not yet diagnosed?
Answer:
Not all patients diagnosed with Neurofibromatosis Type 2 (NF2) develop acoustic neuromas (vestibular schwannomas), but it is one of the hallmark features of the condition. NF2-SWN is a genetic disorder characterized by the development of tumours on the nerves that control hearing and balance, primarily affecting the eighth cranial nerve (vestibulocochlear nerve).
These tumours can lead to hearing impairment as well as balance and coordination issues. NF2-SWN typically manifests its symptoms in late teens or early adulthood, though approximately 10% of affected individuals may experience symptoms during late childhood.
While the majority of patients with NF2-SWN do develop acoustic neuromas at some point in their lives, the severity and progression of the disease can vary significantly among individuals. Some patients may have multiple tumours, while others may have fewer or smaller tumours. Additionally, not all NF2-related tumours are limited to the acoustic nerves; they can also affect other nerves in the brain and spinal cord.
Regular monitoring and management by healthcare professionals specializing in NF2 are crucial for early detection and appropriate treatment of associated tumours and symptoms.
Pain Management Treatments
and Medications
for Schwannomatosis
Notable Specialists in the Field
Question: Are there any recent advancements in treatments or medications for managing Schwannomatosis pain?
Answer:
Ongoing research in the study of all types of Neurofibromatosis including Schwannomatosis is in progress, leading to increasing understanding about the condition. Currently, there is no cure for schwannomatosis, and no medications have been shown to effectively treat schwannomas. The focus of management is to address the specific symptoms that individuals may encounter. Pain, often the most common and bothersome symptom, necessitates priority in management, as it can be chronic and result from schwannomas exerting pressure on nerves.
Treatment options for pain management include surgical removal of the problematic tumour that is causing the pain, and pharmaceutical interventions. Various medications such as anti-inflammatory drugs, opioids, anticonvulsants, and antidepressants can be considered for pain relief. Additionally, non-pharmacological approaches like physical therapy, acupuncture, and massage therapy may provide relief. However, not all schwannomas are amenable to surgical removal, emphasizing the importance of seeking assessment from an experienced surgeon to explore this option. Radiation therapy may be another consideration to shrink tumours or alleviate symptoms, particularly for inoperable tumours.
Patients may find consultation with a pain clinic specialist beneficial, which can be arranged through referral by their General Practitioner.
Individuals with schwannomatosis are best cared for by a specialized NF clinic where specialists experienced in the condition can prescribe protocols that are most effective for people living with the condition.
Ongoing research into schwannomatosis includes emerging clinical trials. One such trial is the Phase 2 Study of Tanezumab in the United States. This trial aims to evaluate the effectiveness of Tanezumab, an anti-nerve growth factor antibody, in managing moderate to severe pain associated with Schwannomatosis. For further details about this trial and any other emerging clinical trials related to Schwannomatosis, refer to:
Question: What current research initiatives are underway for Schwannomatosis?
Answer:
Ongoing research aimed at understanding the causes, progression, and potential treatments for Schwannomatosis is underway. Research initiatives include genetic studies to identify the specific gene mutations associated with the condition. Understanding genetic factors paves the way to potential targeted therapies.
International Schwannomatosis Database is an exciting initiative aimed at accelerating research by connecting patients, families and scientists. The goal for this study is to collect information from patients related to symptoms, management, medications, treatments, and outcomes to better understand the condition and to help make treatments as effective as possible. To learn more about this database and get involved, visit the link below:
Clinical trials help find potential new treatments to benefit people living with rare diseases such as Schwannomatosis. One such trial is the Phase 2 Study of Tanezumab in the United States. This trial aims to evaluate the effectiveness of Tanezumab, an anti-nerve growth factor antibody, in managing moderate to severe pain associated with Schwannomatosis. You can learn more about this trial and all the new schwannomatosis related clinical trials below:
Individuals living with all types of neurofibromatosis and Schwannomatosis are invited to sign up to the NF Registry, a website that was created by the Children's Tumor Foundation to keep patients informed about NF news and current research including new treatments, and to help NF.
researchers learn about NF from the patients’ point of view. To join the NF Registry, visit the link below:
Question: Expertise in Schwannomatosis: Notable Specialists in the Field
Answer:
Specialized Neurofibromatosis Clinics offer consultations and management for patients with Neurofibromatosis and Schwannomatosis. Specialists from various disciplines such as Geneticists, Neurologists, Neuro-oncologists, Neurosurgeons, Ophthalmologists, Dermatologists, and Pain Clinic Physicians, have a specialized body of knowledge and experience related to the unique challenges of Schwannomatosis.
The Elisabeth Raab Neurofibromatosis clinic located in Toronto, Ontario is the first multidisciplinary clinic in Canada that focuses on the care of adults living with NF. This clinic adopts a holistic approach to the management of NF.
In British Columbia, The Tumour Foundation of BC is actively pursuing resources, funding and support for the permanent re-establishment of a specialized Neurofibromatosis Clinic. We ask British Columbians that are living with Neurofibromatosis/Schwannomatosis to join the clinic waitlist and partner with us in raising awareness for the need of specialized NF care in our province. To join the NF clinic waitlist please visit the link below:
It is difficult to identify a select few specialists highly regarded in the field of Schwannomatosis since there are countless physicians, researchers, and scientists that are making positive contributions in understanding the condition and striving to discover and create effective treatments. Additionally, NF specialized clinics comprise highly knowledgeable clinicians in the field of Neurofibromatosis, including Schwannomatosis. It is a collaborative effort across various institutions and disciplines.