Living With Von Hippel-Lindau Disease

A diagnosis of von Hippel-Lindau disease (VHL) may come with anger, fear, and uncertainty. This genetic disease causes cysts and tumors to develop in the body—most often the brain, spinal cord, pancreas, retinas, kidneys, and adrenal gland

You may fear these tumors will become cancerous. Although that is possible, there are ways to watch for these tumors and treat them if they develop.

There are multiple emotional, physical, and social considerations when living with VHL. This article will discuss these and how someone living with VHL can feel empowered in their care and find ways to manage the uncertainty that comes with this diagnosis.

Emotional 

Studies have shown that those living with VHL carry a lot of emotional distress about their disease. These feelings are normal and completely valid.  Some commonly reported fears of people with VHL include:

• Fear of tumors growing
• Fear of losing a loved one (as this is a genetic condition that runs in families)
• Inability to feel they can share their fear and stress with others
• Fear of passing the gene on to their children

Many people living with VHL can also experience fear and anxiety when awaiting results of imaging tests, which must be done routinely. 

It is important to know that the feelings of anger, fear, and anxiety are normal, and it is acceptable to communicate these feelings with others. 

Continuing to stay engaged with family and friends—and even reaching out to them for your emotional needs—can help to relieve some of the stress and anxiety.

If your feelings have become more negative, or if you’re experiencing symptoms of depression, reach out to a healthcare provider immediately. 

Physical

Surveillance

One of the most important measures to take when living with VHL is to follow your healthcare provider's recommended schedule of surveillance imaging and follow-up exams. Surveillance imaging is done at predetermined intervals to evaluate for any tumor growth. Commonly ordered imaging includes:

• Magnetic resonance imaging (MRI) of the brain stem and spine
• Kidney imaging
• MRI of the abdomen

Other surveillance interventions include:

• Routine blood pressure evaluation
• Laboratory tests
• Eye exams
• Hearing tests

Lifestyle

Living a healthy lifestyle can help improve your overall health and can provide your body with the energy needed to function. Important lifestyle tips include:

• Regular physical exercise
• Not smoking
• Limiting alcohol intake
• Eating a healthy, well balanced diet

Consulting with experts such as a dietitian or physical therapist, along with your healthcare provider, can arm you with the best health plan possible.

Social

Spouses, loved ones, and friends of those living with VHL can be great sources of emotional and social support, especially if your loved one also has VHL.

You may have access to local support groups to connect with people with conditions like VHL, but if joining a support group isn’t something you’re comfortable with, there are other ways to seek support from others. The VHL Alliance offers a hotline to call and connect with others who have experience with VHL.

Feelings of being alienated or alone are common for those with VHL. Keeping in contact with others and maintaining relationships will help prevent feelings of isolation. 

Practical 

Financial security may be a concern for someone living with VHL, as insurance, missed work due to surgery, medical bills, or fear of unemployment may cause additional stress. Consider meeting with a financial planner to help sort out your finances.  

Another important tip is to find a healthcare provider you are comfortable with and who has familiarity with rare genetic diseases like VHL. Having trust in your healthcare team can be a big part of a successful journey with VHL. 

Summary 

Living with VHL can bring a lot of uncertainty and stress. Establishing supportive relationships, living a healthy lifestyle, and following recommendations by your healthcare provider can help. 

A Word From Verywell

It is important to know that whatever you’re feeling, it's OK. The emotions that come with living with VHL can vary from day to day but are all normal. Finding great support systems can be helpful. Remember that you’re not alone. If you’re feeling depressed, reach out to your healthcare team for help.