Coping with VHL

Gary L. Wood, Psy.D., Florida, from his talk at the Florida Regional Meeting in Tampa, January 2004

It’s always pleasing to be around people like those in this room, with a passion for their work, and a vision ... all of us, working together to address the challenges that affect our lives.

I am a licensed psychologist in the state of Florida. I own and operate a consulting firm here, and interestingly I do very little work dealing with patients who have physical health problems other than my own. I spend most of my time administering employee assistance programs for about 30 companies. They are based here, but they are all around the country. My primary work is in evaluating employees and family members who are faced with behavioral health conditions – primarily emotional problems or lifestyle problems. We do in our organization provide educational activities that focus on building resilience and reducing stress and so forth.

I am here to talk about the psychological aspects of this illness, and quite frankly I don’t know that we can say a whole lot about the science of the psychological aspects of VHL because we don’t have the data to offer. There was a talk by Dr. Joseph Locala at the VHL meeting at the Cleveland Clinic. But in the medical literature I could only find one article on the psychological issues of VHL, written in 1964, the year I was diagnosed with VHL, 40 years ago.

I have a lovely daughter who has this disorder also. And like you and your family members, we are trying to stay on top of VHL -- monitoring and doing good medical surveillance.

But first, please tell me what you would like to talk about on this topic.

1. At what age would you talk with a child who has been diagnosed with VHL.
2. How do you deal with the anxiety of waiting for test results?
3. What are the medical indicators of the progression of this disease in specific body parts. How do you deal with that?
4. How would you address an individual who won’t go for testing? My grandfather was the one who had VHL, and my father was from a very large family, but very few of his relatives have gone for testing.

You have to work out a way of going from putting your head in the sand to wearing it as a badge of courage. Good questions.

I think that the literature that you have to draw on to address some of your questions is the literature that we know best, and that is dealing with loss. We don’t necessarily know a lot about the particular behavioral manifestations that occur as a result of a diagnosis of VHL. But we do know something about how people react from a psychological standpoint in terms of facing loss. There have been some fairly good studies in cancer patients and how they react.

Now, I think it is important that we remember that VHL is primarily a physical disorder; it is not a psychological or a psychiatric disorder. And as people begin to try to gain awareness and insight into the nature of their condition, you have to be careful about the differential diagnosis in obtaining accurate and reliable information, or you can drift into speculation -- where every person has their own theory about what might work, but little real data. We could probably come up with some fascinating psycho-babble to try to help people a little bit. But I think we have to pay close attention to each case and obtain accurate and reliable information to help prepare the individual for what kind of loss, what level of loss, he or she will experience.

We do know from looking at the literature that some people fare better under loss than others. And we do not always know what are the prerequisites for doing better with loss. We know that certain individuals do better in adversity than others. And how do you identify those who do better than others? It’s just hard to say. I think that this is a line of research that would be useful in studying VHL patients.

But I think that when you are trying to face some of your questions — How to talk with children, how to face family members who may be in denial – it’s a good idea to remember for yourself and for them that they are facing a loss.

Now when you think about a loss, what comes to your mind? Death. Yes, that is the most stressful event that any of us can experience. What else? Loss of relationship, lack of interest, religion, faith, work, loss of independence. Yes. This study that was published in 1964 by a group of psychologists and psychiatrists is a study of a family of twenty-four people in San Francisco, California. The study focused on some of the behavioral characteristics of the family members. And some of the things that were noted are things that we see in loss – denial, anxiety, and avoidance.

Whenever someone experiences a loss, the first experience they have psychologically is shock. If you had a physical injury, your body would go into shock. When you learn that you have this diagnosis and you begin to get more information, you begin to experience a loss. And that is a normal reaction, it is not a pathological reaction. Loss is a normal psychological experience in human nature. And to experience shock and disbelief is a normal reaction. As a matter of fact, if you don’t experience shock, then we have a problem. Because then we would see you develop a lack of compliance with further medical evaluation and treatment, affecting the neurotransmitters chemicals. Your brain is a three-pound bag of juice. What you think and what you do have an impact on your chemistry. So people can negatively affect themselves if they don’t handle the stages of loss properly.

As parents, as employers, as caregivers, when someone experiences a loss, what do we do? Console. Get angry. Withdraw. Feel sorry for ourselves, be angry, fearful, have no motivation at all. And these are common reactions – not only on the part of patients, but these are reactions that occur on the part of caregivers, particularly if this is a loved one. It is important to understand that the initial stage of shock will subside with time. But you can accelerate the adjustment by offering support.

What is support? Listening. How easy is it to listen when you think that your daughter or your son or your wife has a life-threatening illness? Hard. Very hard. Unless you have some understanding yourself. I’m sure you are naturally compassionate, and you’re a caregiver type yourself, but even the best of us, if we are going to try listen, we should first try to educate ourselves. This is where the value of organizations like this Alliance comes in.

In psychology and in medicine we try to get people to become their own doctor, so to speak. You want get to the point where you are inquisitive and informed. If I am informed, I can listen better. I would be quite able to listen to any one of you about your VHL condition if you came to me as a patient. I think any of you would believe I would understand you, right? It’s because I have information about VHL.

And we as parents, we as caregivers, are much more able to help answer the questions of a child, to know what to do with a child, or to hear those family members who feel ashamed of their illness, or guilty that they passed it on to their grandchildren – all the shame and guilt that often comes with a genetic condition. You are more likely to intervene constructively with these people if you have an objective understanding of what’s happening.

There’s a difference among information, knowledge, and wisdom. Those are different things. And everyone has to address their questions at the level they are most comfortable addressing them, and with whom they are most comfortable. But I can tell you that loss, while it is a normal reaction, can also become malignant.

We are very fortunate to have people who have done research on loss. Dr. Elizabeth Kübler-Ross, who wrote the book On Death and Dying and has had a great influence on the grief literature and research on grief, has done a wonderful job of helping us understand the various stages of loss – beyond shock and disbelief and sadness and anger and guilt and — relief. It takes a long period of time to deal with these things.

I was diagnosed in 1964. I discovered that I was blind in my right eye at age 10. Coming from a blue-collar community where there was not much medical care available, I did not know why I had lost the sight of my right eye when I was 10 years of age. When I was 17-18 years old and a very good athlete with just one eye, I discovered for the first time that I would not be able to drive an automobile or shoot a basketball again or chase good-looking girls. It was pretty hard on me. But I was competitive. And I received good support from people I didn’t even know cared about me – school teachers, coaches, ministers, a loving family, and a counselor who understood something about grief. Somebody to just sit and listen. These things were very helpful to me at that time.

Out of my own faith – I’m one of those individuals who have a Protestant ethic that is non-dogmatic – I really believed that something positive could happen as a result of this experience. I was fortunate to learn about books you could read by way of audio tape. At that time they were disks – Talking Books, we called them. And the first book I read was the Introduction to Psychology – a textbook – they just sent it in the mail. And I said, “I think I want to be a psychologist.” I liked helping people, I was a team leader, and all that sort of thing.

And as I look back on my life and I have studied personalities and over the long haul, I do think that while having personal strength is important in being able to pursue goals, it’s critical when you are faced with a limitation – a loss. Having support around you, people who love you, people who can provide you with accurate and reliable information – like this morning, Joyce was providing us an update on some of the research. I’m going to leave here better equipped, aren’t you guys? And listening to the experiences of the people here who are participating in some of these Phase 2 clinical trials – this is very helpful, supportive information.

So I think before you start talking with someone, it’s good to get accurate and reliable information. If your child has been diagnosed with this disorder, you have to deal with your own feelings of loss first. And that means you having someone to turn to, who can support you with accurate and reliable information. Someone in this room, someone on the hotline or the online discussion.

But know that even with the best information, we are still going to feel a loss. We live with a life-threatening illness. Using the science of risk management in medicine, we can help ourselves. The Europeans have brought to us the knowledge of harm reduction technology. It helps us understand that there is no one absolute way to do things. You identify a disorder, and you identify the risk factors that accelerate that condition, and you intervene on those risk factors, whether they be physical risk factors, behavioral risk factors, or whatever. The objective is to reduce harm.

I think that grief counseling is a good idea for any of us who are looking for ways to communicate with our family members. It’s probably the most important if all the things that I looked at in preparation for this meeting. I hope you will take away from this talk an understanding that loss is a normal process. If you are trying to help yourself or a family member, then grief counseling is a good idea.

I have had to face this myself. I face it every day – we all do – especially when you get an ache or a pain or a sensation – hmmm, is this a tumor? I have a pain in my back – is this a tumor? Feeling a little unusual – do I have an adrenal gland tumor? What’s going on here? If you’re neurotic like I am, you start looking into it. I’m an information nut. I get a test as often as I can, and I make sure that my daughter gets them, and she’s probably neurotic by now too. Not really, she’s pretty easy about them.

At this point, his daughter teasingly interjected that Gary does still play basketball, and that good-looking girls now chase him!

I think that I would encourage all of you to learn as much as you can about grief and loss. Dr. Kübler-Ross has written a lot about it. I think it would help you in addressing ways that you would want to communicate with family members as well as deal with yourself. I also think that it will help you to look at your own grief process – your denial, your anger, your aggression, your desire to try to make things happen when you can’t make them happen, and the resulting feeling of stress and frustration. We do know that extraordinary anger can compromise one’s immune system.

We also know that positive psychology such as optimism, is good for your health. We know that looking at things realistically, finding meaning in adversity, and trying to make do with what you have, can cause secretion of hormones such that when you look at the brain with a functional MRI, you see the parts of the brain that are associated with resilience and coping light up! It’s beautiful!

So as you consider looking at your own grief, and those children you may have, or those children that you do have, keeping focused on ways that you can get your frustrations out, talking with someone. For me, usually it’s a friend. Although I have been through therapy myself and I found it very useful.

Now, grief is a process that takes time. Some of the information that I have collected suggests that it takes a period of several years after each loss. And in our situation there are many losses, aren’t there? And they can come regularly. There are periods of time between our losses. I lost my sight. That was a very specific loss related to VHL. Some of you have not lost anything physically at this point, but you may be concerned about losing something, and often the anticipation of loss can be as real as the loss itself.

There are specific psychiatric conditions that can develop independent of VHL and I’d like to touch on some of these. VHL does not cause these things, but if you have them you do want to know about them.

Major depression is a clinical disorder that is characterized by recurring feelings of sadness and loss of pleasure and enthusiasm. Individuals who have this disorder have had it for more than a month, and they also have trouble concentrating, they have trouble continuing to participate in activities that they normally participate in. Essentially they have a decrease in their energy. They often have sleep difficulties, particularly early morning awakenings. One’s thinking is negatively impacted – you are not able to get projects done as you used to get them done, your interpersonal relationships are challenged, you may lose intimate desire, you may become more irritable or agitated. In some instances people who suffer from this depression may act as thought they don’t have it. We call these smiling depressions.

The bad thing about major depression is that it feels really bad. The good news is that it’s very treatable. We have very effective treatments for major depression and we can clear it up quickly with proper diagnosis and proper treatment.

Another disorder that is quite common particularly in people with medical conditions like ours are anxiety disorders. Anxiety disorders can be characterized by what we call generalized anxiety which is a free-floating sense of anxiety throughout the day. You may have periodic panic feelings, but most of the time there is a generalized anxiety, a grand sense of doom, you’re a worry-wart and everybody knows it and you know it. And you can’t determine if you’re anxious because you were abused as a child, or your mother didn’t treat you right, or you’re dying, or what. You just feel overwhelmed, as if you can’t cope. But you keep going.

Usually people who have anxiety are what-if thinkers. They never answer the question, they just keep thinking what-if-this and what-if-that. Anxiety can also result in phobias, when you avoid people or places. You can develop anxious associations with doctors or hospitals or other places. You feel they are unpleasant, and want to get away from them. We have successful treatments for anxiety.

Panic attacks are another disorder that can occur. Panic attacks are physical sensations that you have that may last 10-30 minutes and then they subside. But you feel like you’re going to have a heart attack when you have one of these. You may even go to the Emergency Room and they don’t find anything and they tell you it’s all in your head. These kinds of conditions are very real and very treatable with the right diagnosis and appropriate treatment.

Some individuals when they lose something have a bereavement reaction. This goes beyond normal grief. The person develops symptoms of major depression. They lose interest in life. It’s all associated with a loss. You have not worked through the loss, and now you have developed an emotional health disorder as a result of the loss.

There are many other conditions that you can develop, but these are some of the more prominent ones. If you have persistent feelings of sadness or anxiety, or if you had a loss and you are unable to work through it, it is good to consult with a psychologist or a psychiatrist or a qualified mental health professional in your area so that you can determine if you have one of these disorders, so that it does not negatively impact your further follow-up with VHL.

With VHL losses can become so compounded that you don’t have time to breathe and mourn and instead you go into denial. A little denial is a good thing, and is sometimes a characteristic of resilience. But if you don’t grieve these losses you can develop smiling depression.

When you have had bad news, sometimes your normal support structure is also in shock and disbelief. It helps to get assistance from someone outside the family. Family therapy can be helpful.

When to speak with your children. Be sure to give age-appropriate information. What you would say to a 4-year-old is not the same thing you would say to a 14-year-old. My rule of thumb is to always give accurate, current, reliable information, that is age-appropriate. If you don’t know what that is, check with someone who understands developmental psychology.

What’s the best way to cope with fear? The first thing to do is to get all the information you can. Get educated. Look for the experts; get knowledge. Often that helps a great deal. If the anxiety and anger is daily, then you probably want to consult with a psychology professional.

We do not know that VHL causes these emotional problems. I think we all know that the chemistry in our bodies is very complex. There is a link between stress and physical illness, although it’s a weak link. There is growing information that what you think has a lot to do with your compliance with treatment, with your lifestyle, whether you choose to exercise or not, how you think about something does make a difference. And it’s a factor to consider. It’s not a cause of VHL. But it’s just as important to maintain our psychological health as our medical health. Be well.

As printed in the VHL Family Forum  12:1, March/April 2004.  For permission to reprint, please contact VHL Family Alliance, editor@vhl.org. Further information is available from the VHL Family Alliance, info@vhl.org.