Hello from Warwickshire!

[Sue A]

Hi,
We have recently discovered that the LFS syndrome runs in our family. My husband had a tumour 11 years ago, but it was successfully removed and has recently been diagnosed with Myelofibrosis and has just had his spleen removed in preparation for a Bone Marrow transplant which is scheduled for April 2012. We have only just had the test and the confirmation the he has a faulty P53 gene but we already knew that it was a very likely possibility. His dad has LFS but is 70 and has never really had a days illness in his life. His brother died of cancer when he was 36 and his uncle died when he was 44 and his cousins son died of cancer when he was 4. So far, only my husband, his dad and two of his female cousins have decided to have the test and its come back positive. The 2 cousins are 44 and 50 and havent had any cancers. They both get 6 monthly MRI breast screenings. We dont know if the gene comes from my husbands grandfather or grandmother as they both died at a young age, both of cancers. If it was his grandfathers, then he comes from a huge farming family in Warwickshire…..his surname is Alsop….is there anyone out there of the same family?
Our family is under the Birmingham Womens Hospital. When we last saw our doctor a couple of weeks ago, I asked about screening for the children. She told me that they dont test the children untill they were 16…..(I dont want them tested yet anyway)……and that My youngest who is 7 could have renal screening if I want him to, but it hasnt proved to be of any help and that my older two (who are 10 and 12) are past the risk of getting renal cancers. She also told me that once my daughter turns 20 she can get an MRI breast scan every 3 years. I was absolutely appalled at the lack of screening that they offer. I feel so helpless and so worried as to what the future holds for my husband and my children. I was thrilled when I came accross this website (purely by chance) and feel that shared knowledge can only prove to be a good thing for all of us who are affected by LFS. I also think that the medical profession needs to be enlighted about LFS aswell. When my husband had his spleen out a couple of weeks ago at the QE in B’ham, I was alarmed as to how little the medical staff knew about LFS. We had to explain it to them. They were about to give my husband an xray when he was in ITU and I had to stop them and explain about the risks involved from exposure of radiation to people with LFS. They had never heard of it.
Well, thats all for now. I hope I hear from some of you as it will be good to talk and hear of your experiences.
Sue.

[Verity]

Hi Sue

THank you for all the information you shared. My famil also has quite a chequred history of cancer. We found out about LFS about 4 yrs ago. I am still free of cancer, but unfortunately my 38 yr old brother died and my 6 yr old son has had cancer.

My immediate family is cared for at The John Radcliffe Hospital, Oxford. I have 4 children. My 6 yr old was tested for LF after his diagnosis with a Rhabdomyosarcom. He is possitive. However my other 3 children are purely treated “as if they have LF” Every year they recieve a full physical examination by the consultant oncologist and abdominal ultrasound every year to check for adrenocortical cancer. The age of my children are 11, 9, 6, 2. My eldest son is also being offered annual skin examination as of this July.

I have been talking to our oncologist and genetisist about increasing the survelliance to every 6 months.
If I remember rightly there is a Dr(Prof ?) Evans who is at Birmingham who spoke at the LF conference which took place a few months ago. His main speciality is breast Cancer, but he is also a leading expert on LFS in this country. It may be worth you trying to contact him to get his support and help in getting your children properly cared for. I am sure I have found his work email on line before.

Thank you for sharing your story. There are so few of us that each time I hear of another LF family, I feel like my family has grown. I hope your husband returns to good health again soon. Do keep us updated on his progress.

Verity

[Sue A]

Hi Verity,
I’m so sorry to hear about your brother and your son. I hope your son is doing well and will make a good recovery. I re-read my post and thought I sounded so pesimistic…..but we mustnt foget that my father in law who is positive has never been ill and he’s 70. Also, two female cousins are positive and they (44 +50) and they havent been ill.
I am going to ring our genetic consultant today and tell her that I would like abdominal ultrasounds for the kids and at least that will be one thing in action. The other tests you mention, did you get those arranged through your local GP or through your genetic Dr, or was it through the current Dr that is looking after your son? You also mention a LFS conference….where do I find out about these and can anyone go or is it just for the medical profession?
Thank you so much for replying, I felt real releif when I read your reply and not quite so alone to deal with this. Shared knowledge and experience is a wonderful thing.
Sue.

[PanP]

[quote=”Verity” post=40]
Thank you for sharing your story. There are so few of us that each time I hear of another LF family, I feel like my family has grown.
[/quote]

Verity, that’s so good to hear you say that. If that’s all we manage to acheive than it’s still positive – we know what it’s like to feel isolated and alone going through the system and so helping people break through that isolation is so important…

Pan

[PanP]

Sue, print off the information about the US/Canadian monitoring protocol that was recently trialled. I wrote about it here:

[url=https://www.tp53.co.uk/index.php/lfs-surveillance-protocol]https://www.tp53.co.uk/index.php/lfs-surveillance-protocol[/url]

It might help in persuading your doctors to take active surveillance more seriously.

And please do keep us posted on how things are going with your husband.

[Karen]

Hello Sue,

I also have only recently been diagnosed with LFS and to date have been lucky enough to remain cancer free.However I was shocked by the lack of screening offered currently. The only real screening currently available is for breast cancer. I feel that if you have made the choice to find out if you carry the gene then you should also be able to make the choice to under go screening as well. The experts are quick to point out that there is no proven benefit to other types of screening and there is large scope for false positives, however I feel it is up to the individual to make that choice. Going forward there does appear to be a research study to evaluate whole-body MRI about to be set up in the UK , this is similar to studies which are currently taking place in France and the US.
The other difficult decision for women to make is should they have surgery or carry forward with screening. The experts again seem quite pro bilateral mastectomies , and this is currently something I am considering especially being mid forties and having a high rate of breast cancer in my family. Have your husbands cousins ever thought about this option? To date I have received some good support from the breast team at my local hospital and have had several discussions with them regarding the operation. I am now going to take a couple of months to make up my mind on which way to go.
Lastly I think it is vital that GPs / medical staff are made aware of this condtion. When I went to see my GP after being diagnosed she had to do research to find out what the condition was and re book me in again after she had done her research. I think there is a long way to go and importantly there needs to be more awareness of LFS to the medical profession.

I wish your husband all the best with his surgery and importantly we all need to stick together going forward to get the best possible treatment and screening for our families.

Karen

[Sue A]

Hi,
It seems ages since I have updated everyone with our progress. My husband has just come out of hospital after having a Bone Marrow Transplant and although there is still a long way to go, he is doing very well….I will keep you updated on his progress.
The last time I posted a message it was to say that I had arranged a meeting with our local GP to talk to him about the LFS diagnosis and what this means to our family. We took the leaflet that you did Pan, (thank you for that), and just wanted to have a discussion about the importance of early detection and early referrals. We also needed to be sure that it was put on my husbands and the kids records so that it would flag up any time they went for an appointment. Very dissappointingly, our Dr was not very receptive and was very dismissive of the whole thing. I dont feel that we managed to stress about the importance of early detection at all. We could hardly get a word in edgeways as he kept going off on a bit of a tangent, talking about anything but LFS. He really didnt want to listen to anything we had to say. We came away feeling very despondant and feeling very let down. However, we decided that as there are some really nice doctors at our surgery, we would do our best to avoids appointments with him altogether.
Since then, we had an appointment come through for all the kids to see a Dr at Birmingham Childrens Hospital, with regard to screening them for adrenocortical cancer annually. This referral came from our genetic consultant. We couldnt have had a more different experience this time. I went in and spoke to the Dr alone before the kids did, and gave her the leaflet on LFS, and also gave her the information on the screening programme done in America, showing the protocol for surveillance. She was really pleased that I’d brought this information for her, admitting that she knew very little about LFS, and was completly understanding that I was trying to push for as much screening as possible. She is now going to go away and do her own research and we’ll know more when we have our follow up appointment. We came away feeling very positive.
Our next appointment is on 18th June with Professor Maher at Birmingham. He is a professor of medical genetics…..we havent seen him before, does anyone know anything about him?….. I am also going to take the information relating to the clinical trial done in America and see if there is any other screening we can get……or if there is none, then how do we go about getting it? I am also going to ask about any trials being done in this country? Basically, I am going to knock on as many doors as it takes to get some surveillance for my kids.
If anybody else is having any luck with any form of screening, then I would be really interested to hear.
Thats all for now, I will let you know how it goes with Prof Maher.
Sue.

[PanP]

Hi Sue,

Thanks for the update. Glad you’ve made some progress, though your GP doesn’t sound especially supportive. Please keep us updated on how things progress. It seems to me that different people are being told different things about screening depending on who they see. It’s ridiculous that something so important should be down to pot luck.

I hope other people on the forum who’ve had recent experience can offer some advice. Maybe we need to put together some kind of list of who’s managed to get what…

Pan

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