Hi, all. Haven't been on the site for a year or two as I get over my procedures. Both adrenal glands removed in their entirety because of pheos. Laser on tumours in both eyes. Waiting for an appointment to see the hearing bloke as it looks as if my ears are limbering up for trouble. Cysts on both kidneys, not yet cancerous, but one is 12cms across and quite uncomfortable. Otherwise bearing up.
My 3 siblings have all tested negative and 2 of my 3 children are negative. The third has his head in the sand!
The main purpose of my post is to ask if anyone on the group is interested in family history? I have traced my VHL so far back through 5 generations and it is fascinating to see the ACTUAL causes of death given before VHL as a disease had been identified.
Richard
Already a member? Sign in
Welcome to Inspire!
What - Inspire is a place where you can connect with people who share your health concerns and find information and advice in groups sponsored by organizations you know and trust.
Why - As a member you can use Inspire to let friends and family know how you're doing, contact others who share your health concerns, receive personalized updates and information about participating in surveys and clinical trials, and more.
How - Joining Inspire is completely free and usually takes less than a minute. Join now!
Genealogy - Family History
- By Richard
- Posted March 14, 2009 at 7:43 am · 8 replies
- In Getting a diagnosis
- Shared with the public
Explore topics in this discussion:
8 replies
- Oldest first
- Newest first
- By TinaDiane
- Posted March 14, 2009 at 9:59 am
- Report post
Hi Richard,
I am very interested in family history of VHL. My Mom died at age 36 of a brain "astrocytoma", but had several tumors removed prior death. That was 41 years ago. Who knows what she really had??????????
I am the first to be diagnosed in my family. And all 4 sibs tested negative. My family is German originally, from the Black Forest area, where VHL is very
"common". My Mom has 2 remaining sibs that are in their 80's and they are fine but have not been tested (why would we want to do that?? duh). Others died along the way. My Grandma died in her 60s, (long time ago) but they opened her up on the operating table and closed her right back up due to cancer spread so bad, forget it. So, sometimes I sit and wonder...........
I am so grateful for the scans/MRIs we have now, to stop this in its tracks, IF we can.
I also have had a total bilateraladrenalectomy!! I think life is fantastic without adrenals... No more rushes, no more physical panic, no worries about extra steroids causing all kinds of symptoms. There are a few down sides, but not too many. Eating every 4-5 hours with a little salt, helps us not dehydrate. Staying hydrated with water, also helps. Taking pills on time is the MOST important. Taking more cortisone when sick, and getting to the hospital if vomiting for more than 12 hours.
Anyway Richard, I seem to be on a roll today!!
Best of health to us all........
Tina
- By Richard
- Posted March 14, 2009 at 10:26 am
- Report post
Hello Tina,
I love your description of life without adrenalin; no wonder people describe me as laid back. But there are down sides, as you say. For me - sleep disturbance. How the night will go is a complete lottery. I find that by 7pm the batteries are empty. Sleep until 11pm, and that's my lot. Plus - I had to run across a road recently to avoid a rather large lorry and nearly collapsed on the other side. No reserves.
My father died at 43 of a brain haemangioblastoma. His mother at 36 of a 'cerebral osteoma'. I have read somewhere recently that research may be associating Arnold-Chiari Malformations with VHL. Was this the real cause of death? Her mother was symptomless.
Glad you're on a roll today. They are few and far between!
- By TinaDiane
- Posted March 15, 2009 at 9:50 am
- Report post
Hi Richard,
Yes, I totally get it about the running out of steam and the sleep problems. I take my hydorcortisone (10 mg in am) and (5 mg about 3 pm). I take extra when I feel totally out of it or start crying for no reason (or of course when sick). I also go to bed whenever I am tired (if a bed is available!!) and I take naps whenever possible (even if for 10 minutes). I do drink coffee, which seems to help. I also take prescription Ambien to help me stay asleep at night. My doc is great about it.
I, too, have had a few instances when I would like some adrenaline. I have been in a few fender benders because someone stomped on their brakes in front of me and my brain went "Tina, put on your brakes, put on your brakes, PUT ON YOUR BRAKES!!!" and then, I smashed into the back of the car............. Thank God, I was only going about 30 miles per hour.
Take care,
Tina
- By katmine
- Posted March 15, 2009 at 7:06 pm
- Report post
Hi Richard,
Geneology really fascinates me, especialIy the link to vhl. I'm on quest to find ancestors with this disease. Recently, I found out that the cause of death that's listed on my mother's death certificate (she died in 1970) was hemangioendothelioma, brain stem. She lived in a little farming town in the San Joaquin Valley, California in the 1940s and 1950s where they sprayed the crops with pesticides. Apparently you can get this disease from inhaling pesticides. Since her only brother also died of a brain tumor at 24, (engaged to be married), We don't have any first cousins on my mother's side. She was 41 when she died. This doesn't explain how her grandmother from New Brunswick, Canada also died of a brain tumor in her mid thirties. My great-grandmother lived most of her life in Presque Isle, Maine. Her name was Clara Watson. That's as far back as we've been able to research. So far, I'm the only one in our family who has vhl. I wasn't diagnosed until I was 51. So, If there's anyone out there who had ancestors from Presque Isle, Maine with the last name of Watson or Sparks at the end of the 19th century to about 1940, who has vhl, I might be related.
All my best,
Kathy
- By Richard
- Posted March 16, 2009 at 12:58 am
- Report post
Hello Kathy,
Thanks for your reply. We all seem to take a different route. In my case I can claim that genealogy saved my life; I had noticed a large number of younger deaths in a quite distant line of the family, contacted one of those still alive and he said they had VHL. Started working back round to my line and found similar patterns, although nowhere was VHL mentioned. Convinced my doc that a DNA test would be appropriate, and the rest is history.
If I have a problem now I go straight to the consultant as the GP just seems fixated on treating a symptom rather than the underlying cause.
Good luck. Richard.
- By MaryP
- Posted March 16, 2009 at 11:21 am
- Report post
Living in Salt Lake City, we are born and bred for genealogy. The largest genealogical library in the world is located here. The library actually has satellites known as Family History Centers throughout the world. Through these centers, anyone can access the resources held here in the library. The centers are owned by the Church of Jesus Christ of Latter Day Saints, but they are open and available for public use.
If you are interested in taking your genealogy to the next level, you can google "Family History Center" and it will give you a link to their website. You put in your country and zip code and it will give you the contact information for the family history center nearest you. They have volunteers trained to help you research your family lines. You may be surprised at how much of the work and research has already been done.
Having VHL is a reminder that we are connected to our family and ancestors. There is a part of them in us (and, obviously, it's not just VHL.)
Best Wishes to you, your health, and your family.
- By Richard
- Posted March 17, 2009 at 1:34 am
- Report post
Hello Mary. Thanks for your advice. I have used FHCs and the IGI frequently, as well as attending our County record offices, Good, old fashioned, page turning! But I have to say that it's a lot easier using a computer from the comfort of your home.
I say to everyone who scoffs at any sort of history and says 'forget it, it's dead and gone', that our history is what makes us who we are, and who we are today contributes to the future. Good luck. Richard.
- By joyceg98
- Posted March 24, 2009 at 1:29 am
- Report post
I've done quite a bit of family history, hunting VHL in my late husband's family. And I have to say that through the LDS library I actually saw the recording of my husband's great-grandfather's birth in a tiny town in Poland which has since been wiped off the map by the Nazis. But before that happened, the LDS church went through the town and microfiched the church book, and I read it in the New Hampshire branch of the LDS library. Amazing! So thank you for the stewardship of these records.
Don't forget too that the listed "cause of death" can be misleading. My father-in-law's death record was very informative, named VHL and the hemangioblastoma of the brain stem that did him in in 1953.
My husband's death record, from 1977, says that he died of "pulmonary edema" which is about as informative as saying "his heart stopped". Duh. Not a word about his 15-year battle with VHL.
Terms like "hemangioendothelioma" could well be a misdiagnosis of a hemangioblastoma, especially in 1970. According to Wikipedia, "They have been described as masses that fall between a hemangioma and angiosarcoma. They are vascular tumors that commonly present with an enlarging mass and have been reported in the head and neck, intestines, lungs, lymph nodes, pleura, retroperitoneum, stomach, and many other body sites."
In other words, it's a hair's breadth from a hemangioblastoma, and it's in the brain stem where hB's tend to hang out, so draw your own conclusions. There will never be a totally reliable answer, but the assumption is reasonable.
Great sleuth work there, Richard!
Best wishes,
Joyce
Add to the discussion