Ken and I met at an Air Force hospital where we were both working, near the town in England where our families have lived for generations. When I was pregnant with our first child, Ken began having all the symptoms of sympathetic pregnancy. We teased about his "morning sickness", until it became much too intense to be a joke. He had a brain tumor removed about the time our daughter Anne was born. Because of the stories of other tumors in his family, I asked the doctors if it could be hereditary, but was assured it was not.

With a sick husband and a tiny baby at home, I worried that I wouldn’t be able to cope, but Ken quickly bounced back from the surgery and we managed just fine. We settled into a happy and comfortable life. We welcomed our daughter Jayne two years later.

A few years later Ken began experiencing pain in his back. Doctors told him he had a slipped disk, and he was undergoing treatments at the hospital clinic to stretch his spine and heal the slipped disk. He went for treatments regularly for some six years, and was not getting better. It was our general practitioner who finally suggested that perhaps it was not a disk after all, but some kind of tumor. Tests revealed a spinal tumor which needed surgery. The stretching treatments had aggravated the tumor, which was now so involved with the spinal cord that they feared it would rupture. Surgery was very involved. While it saved his life, it left him with paralysis from the tumor down.

"Laughter has been the most important ingredient in our marriage."

With this tumor, we received a diagnosis of VHL. We were told it was hereditary, and the doctors began to look elsewhere in his family to see if there were other affected relatives. Five of Ken’s seven brothers and several of their children and grandchildren have VHL. His father and grandfather also had tumors. After at least four generations, the condition was finally given a name and we can now see the connections among the symptoms.

Ken worked hard with his physical therapy and began to walk again. One time when I went to pick him up from the rehabilitation treatments, seeing him, silver-haired and walking with only one crutch, I laughed that he reminded me of Long John Silver. The next time I came I brought him a stuffed parrot to perch on his shoulder. Laughter has been the most important ingredient in our marriage.

Ken began having symptoms which felt to him like a urinary tract infection. The doctor who was treating him was convinced it was a spinal tumor and declined to do tests for an infection. By the time they did the tests, the infection was well established, and the medication he had been taking had caused a stomach ulcer. The surgery for the ulcer reduced his mobility, and it was back to rehab to learn to walk again. Our daughter Jayne, then age 12, who was always closest to her father, became very depressed. We believed that she was missing her special games and outings with her father.

Ken was very discouraged, and would often ask me "Why do you stay with me?" Our daughters were seeing the marriages of many of their friends’ parents breaking up, and were getting worried, both about their father’s health and about the prospect of our home breaking up.

We went to family therapy for a time, to help us all deal with our fears and emotions about Ken’s health and his disability. Through that counseling we learned that Jayne was blaming herself for her father’s paralysis! Some years before she had left a roller skate in the back garden, and Ken had slipped and fallen on it. Jayne was sure that it was this accident which had caused Ken’s disability. As much as we tried to reassure her that it had nothing to do with her, she had convinced herself that it was so. Little by little we are working to help her let go of this self-punishment.

I decided to take action to reassure my husband and daughters. I arranged with the vicar of our local parish church for a renewal of our wedding vows. This is not normally done in the Anglican Church, but the vicar was very sweet, helped to write a special service, and arranged for coffee and a little celebration to follow. On 30 August 1986, with a group of our special friends, we renewed our vows — for better or for worse, in sickness and in health. Our daughters put tin cans and a "Just Married" banner on our car, and saved their own money to buy us a lovely gift.

Knowing now that Ken’s was dealing with von Hippel-Lindau disease, and that our daughters were at 50% risk of having it, we became very watchful for signs of the disease. We took them for eye appointments every six months. Each time one of them had a headache, I would play games with them, based upon the neurological tests I had seen the doctors do for Ken in the clinic — walk a straight line, heel to toe; touch your finger to your nose. While it may not have been very scientific, it helped me to calm myself down when I saw that they could pass these tests, and that the headaches went away like normal headaches.

Jayne is very like me, Anne is very like Ken. I guess I had convinced myself that Jayne couldn’t possibly have VHL. After all, Jayne had inherited my asthsma, which I felt terribly guilty about. So when they found a VHL lesion in Jayne’s retina we were doubly shocked. Fortunately it was easily treated, and seems to be her only involvement. But for Ken and me, and of course for our girls, this began a second voyage with VHL.

At age 18, Anne had a brain tumor removed, and only months later surgery to remove a large tumor from her pancreas. Ken had a particularly hard time seeing Anne in the hospital after surgery — all the emotion any father would feel seeing his child in pain, along with a certain guilt because she got this disease from him. Just as I didn’t want to give Jayne my asthsma, so Ken certainly did not want to give them VHL. Neither of us had any choice in the matter. But here we are, and we’re going to make the best of it.

It is hard work coping with Ken’s disability — hard for him, and hard for me. But I have learned to do his exercises and care at home. I am a member of Carers National,¹ an association of people who care for handicapped people at home. We receive a magazine with lots of information about services and legislation of interest to handicapped people.

Ken and I laugh a lot. We have a wierd sense of humor. Sometimes people look at us as though we are not taking life seriously enough. But if you can’t laugh at yourself, who can you laugh at? Everybody has a different way of coping, ours is laughter.

I watched my family go through each of their operations and come to terms with their illness in their own way. At times I felt as though I was going out of my mind with worry and that my heart would break. It was hard to be cheerful all the time! I just had to be there for them.

Ken is working hard to make sure that the doctors learn as much as possible through his own case, in order to make our daughters’ medical care that much better in the future. We only want them to be happy and to live life to the full.

Our family is very active now in publicizing VHL in England, through Action Research,² a charitable organization which has helped to fund the genetics research of Dr. Eamonn Maher at Cambridge University. Anne recently participated in a bike-a-thon for cancer and VHL. She rode 27 1/2 miles and collected over £200. People are very moved that, having had brain surgery last year, Anne is working, riding in the bike-a-thon, and living a normal life thanks to the medical advances that have been made since her father’s time. We are hopeful that through continued research and information sharing our children will never experience the kinds of serious problems we have seen in our own lives and in older generations.

This poster³ hangs in our kitchen, where I read it at least once a day:

Think Positive!
Greet the morning with a smile,
Ponder each new day awhile.
Don’t let your mood be sad or grey,
Be sunny and enjoy the day.
The world is waiting just for you
To come and show what you can do.
So don’t sit back or shy away.
This day is yours in every way.
Be Optimistic!

1. Carers National, 29 Chilworth Mews, Longon W2 3RG. In the US and Canada contact the Well Spouse Foundation, http://www.wellspouse.org, wellspouse@aol.com.

2. Action Research, Vincent's House, North Parade, Horsham, W. Sussex, RH12 2DA England U.K.; in US: American Cancer Society, 1599 Clifton Rd, NE, Atlanta, GA 30329-4251; in Canada: Canadian Cancer Society, 77 Cloor St, W, Ste 1702, Toronto, Ontario, Canada.

as published in September 1993, VHLFF 1:3