My 10 year old daughter has Phaechromocytoma and VHL Syndrome which caused it.

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Hi just came on because my 10 year old daughter has Phaechromocytoma in her left adrenal gland which was caused by secondary inheritance gene VHL (Von Hippe Linda disease). She inherited VHL syndrome from her dad who also had Phaechromocytoma in both his adrenal glands and his adrenal glands and he had to have both of them removed in 2009, because he had the Phaechromocytoma for such a long time and was not diagnosed he unfortunately had other organs which git damaged, he had his spleen removed due to the complexity of his surgery, he has had to undergo a heart bi pass and has lots of health issues but he survives by taking life long steroid tablets and extra med. I mention this because I wanted to give a background to my daughter's circumstance. My daughter was tested after her father was diagnosed with VHL Syndrome by the genetics and endocrine team doctors as they say that once a parent has been diagnosed there is a 50% chance they will pass on this gene to their child/children, well my daughter was tested positive for the VHL gene. My daughter started having severe headaches at the age of 7, the headaches then started to increase more over the years with nausea, vomiting, problems with her vision in 2013 to 2014, I kept bringing her to the doctors, A&E and they referred me to see a paediatric, they diagnosed her with migraines and put her on pitzofen medication which made her worse, at each apoint and hospital attendance I ask could her symptoms be a contributing factor because of the genetic syndrome she has VHL? When I ask this question the doctors and specialist look at me as if I made up this name or syndrome, they have no clue what VHL is, so the turn the subject to something else or ask me what's that, do they not know to check a patients medical notes, or communicate with the specialist team at Guy's, no they prefer to dismiss you with medication and not follow why this poor child is suffering. The last A&E attendance where she was so I'll, she had severe abdominal pain ,they had to do a ultrasound scan, blood test, urine specimen, they said that she showed swollen glands in her tummy on the left side, and that's what was causing her to be ill and also she had urine infection, at this point I noted before they gave me these results they had to get three extra specialist doctors to have a look at the test results, I mentioned the VHL syndrome my daughter had but they hadn't a clue what I was on about, the had to get another doctor above them to come and speak to who again said the same results, told him about the VHL gene inheritance and he dismissed it I ask him to speak to the gene clinic on Guy''s to double check and he dismissed this and refer me back to my GP. My daughter had to have a screening test done each year because VHL makes you more vulnerable to tumors of the adrenal glands, the back of the eye, the kidneys, you spine and other parts of the body. Due to my daughter being under 10 before she only had the eye test done, because children only get offered full screening test at the age of 10, my daughter turned 10 in April this year and was now readd for her first full screening, my daughter went to the A&E on May 05, 2015 when the doctors told me all the rubbish I mentioned previously. My daughter had her fist full screening done on the 10th of June, 2015, before leaving the clinic I remember the ultrasound specialist and the receptionist telling me if there were any problems the doctors would ring me as soon as possible. What do you know, was happy at home with my children when two days later 12th of June I had a devastating phone call my daughter has a 3.6 cm mass showing on her ultrasound scan on her left side, I had no idea what they meant, the doctor started explaining that it looks like the sign of a Phaechromocytoma. The following week I was like a zombie going back and forth to the hospital for testing, Mris, Mibg scans, blood test, echo test, they couldn't tell me if it was a benon tumor or a malignant tumour, all I can remember is this is very rare for a child of your daughter's age to have this at such a young age, it's just unfortunate. Cut the story short, my family and myself have had a stressful time, the test came back as being, she was put on phenoxybemzamime med ASAP to sat treatment and she is due to have surgery to remove the Phaechromocytoma by removal of the adrenal glands, I am praying it all goes well, as I have not been able to find any support groups to find out about their surgery for children with Phaeo and what their recovery was like. My daughter has had bad attendance at school over the years and the doctors ha's told me that it was because he blood prseeure was too high, hence the migraines, vomiting, anxiety she had over the years, heart palpitations, dizzyness, nausea, feeling weak and tired. Just wanted to know others experience of VHL and Phaechromocytoma, if there are any support groups for children with this please.

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7 Replies

  • Posted

    Hi Shelly, So sorry to hear about your daughter and what she is going through. I am waiting to see a consultant (appointment on 19 th August) because I had a CT scan about 6 weeks ago for something else and they found a mass on my adrenal gland. I am getting the same symptoms as your daughter although she has got the VHL gene.  Looking back I have had symptoms for a long time. The weak feeling is awful. I am not sure what the mass is at the moment until I see the consultant. I was told my adrenal gland is lumpy whatever that means. Your daughter is so young to have this condition. Pheo's are supposed to be very rare. Sorry I cant be of any help but hope the operation goes well.        
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    • Posted

      Hi Merryl, sorry to hear about your problem too, I hope all goes well for you too, just praying she will have a smooth surgery and get well quickly to start school as she missed school this last term.
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  • Posted

    Hi Shelly, my daughter had a 'phaeochromocytoma' removed 3 years ago aged 12, her story is much like your daughters except my daughter has no genetic disease that cause this type of rare tumour.

    If you have a Facebook account there are a couple of good support groups.

    ''phaeochromocytoma and paraganglioma support for UK and ireland'

    Also an international group ' pheochromocytoma and paraganglioma support group'

    Very good information on these,

    Hope your daughter feels much better soon.

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    • Posted

      Hi Julia, thank you so much for the information. Please can I ask if your daughter had laparoscopy done or other and how many hours was her operation, how soon was she discharged after surgery, how soon was she able to get back to school? Sorry for asking so many question I am quite anxious and don't know what to do.
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  • Posted

    Hi shelly, she had laproscopic, her tumour was 7.5cm and wrapped around her vena cava, her surgery was 5.5 hours, she was on the high dependency unit for 4 days and was allowed home on the 9th day, she recovered well and is monitored 6 monthly.

    I will reply properly later, just on my way out.

    By the way these tumours are so rare I only know of one more child with pheo in the UK at the moment.

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    • Posted

      Hi Julia, thank you so much again, this is really use ful for me to know, wow I hope she never has to go through that again, well done it must have been really stressful for you at that time. You are the first person who I am able to relate with this problem in regards to a child, it feels so good to finally communicate with someone who has a child who experienced this, this has made me feel more positive, especially because she has recovered so quickly. Thank you again.
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  • Posted

    Shelly if there is anything I çan help with please just ask on here although these comments are not private and I am reluctant to put too much info, not sure if giving out email addresses is allowed on here but if you like you could give me your email address and hopefully help to put your mind at rest a little.
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