Saturday, 7 December 2013

Interview with multiple surgery patient and Nexavar with family background

Today I have an interview with a very experienced plantar fibroma patient. She has had numerous surgeries and it a perfect example of why I personally do not think that it is a great option for treating the condition. Read on for her story. 
1. Do you have Ledderhose disease, Dupuytren's or both and at what age were you first diagnosed? 
I have both Ledderhose and Dupuytren’s, in both feet and both hands, and I have knuckle pads on 6 knuckles.  I was first diagnosed with it (Ledderhose) when I was fifteen and I had a 'bump' on my instep that hurt.  I was a gymnast and I just couldn't compete with the 'bump' so the doctor recommended it be removed so he could find out what it was.  He removed it in his office under local anaesthesia, threw in a couple of stitches and sent me on my way with orders to stay off it for a week.  Within a couple of months it grew back, bigger.  My hands started a couple of years later, in my twenties.  I have cords, tumours and knuckle-pads.

2. Do you have a family history of the condition, if so how prevalent is it? 
My father had Dupuytren’s but didn't know what it was at the time, and complained about bumps in his feet but again didn't do anything about it.  He passed when he was 40, I was 15,  he never had anything done.  I feel that had he lived longer he would have had a doctor work on both his hands and feet, and we would have learned more about this disease sooner.  At the time no one else in the family had the disease that we know of. I have five children, 4 boys and a daughter.  My second oldest son has Dupuytren’s in one hand and looks like he is growing a tumor in one foot.  He is 24.  My 4th son also has Ledderhose in one foot.  He noticed a small bump and questioned me about it.  It has since grown and now measures about 1 inch by 1 inch.  He is 22.   One of my sisters(I have two and no brothers) has Ledderhose in one foot.  She was diagnosed when she was 45 and had her surgery that same year.  Her tumor grew back bigger, but she is still able to get around with minimal discomfort. She has not had any more work done on her foot....yet.  Her daughter has it in one foot also, diagnosed when she was 2 but the tumor never grew so she never had anything done.  It remains the size of a large pea in her instep.  My other sister who doesn't have either disease has three kids, one who has Ledderhose in one foot.  She did two rounds of cryo surgery when she was 17, then at 18 had surgery to remove the tumor that had grown quite large.  It's been 4 years since her surgery and the tumor hasn't grown back, not that she has noticed.  

3. Please describe your progression with the condition up to the point that you required treatment. 
Like I said, I had my first surgery at the age of 15 and since then I have had 30 surgeries on my feet and 5 surgeries on my hands.  I have had both fascia bands removed from the bottom of both feet in hopes that it would stop/slow the growth of the tumors.  I have had fibromas grow on the outsides of my heals, on the balls of my feet, on the insteps growing up into my ankle, and towards the outsides of my feet.  At the most I had 5 tumors removed from one foot at one time, and the largest was 9cm x4.5cm by 2.5 cm deep.  And, this was growth in only a year.  The form of Ledderhose I have is very aggressive.

4. What treatments have you had other than surgery? 
In the fall of 2011 the tumors had grown so large and my left foot was swelling to the point I couldn't even get a flip flop on, and the doctors deemed my feet couldn't withstand another surgery so I set out looking for an expert in the field of Ledderhose disease.  After a long search and many visits I found Dr Lackman and Dr Staddon at UPenn Hospital.  Dr Staddon is an Oncologist Hematologist and he knew everything about this disease.  Dr Lackman is an Orthopaedic who also is very familiar with this disease.  Dr Staddon said they were having promising results with Methotrexate and Valban given intravenously once a week for 8 weeks.  I did that course of treatment but had no good results.  Then he said there is an experimental drug that is used for the treatment of non-operable liver cancer, that has proven to reduce the size of fibromas.  The drug is Nexavar,  I took one tab in the morning and one at night.  The drug is powerful with side affects, but it did shrink the tumors to less than half their size and reduced all the swelling.  I was on Nexavar for 10 months, then taken off it to see how the disease reacted.  I have been off it exactly one year and the tumors are growing, but they are growing at a much slower pace but are causing considerable pain.  I return to the doctor on Dec 10 and will find out what course of action he plans to take.

5. I understand you have had numerous surgeries, please could you describe the progress and the recovery times, recurrence times?
  
I had 30 surgeries from the age of 15 to 47.  There was a couple of years where I was having two surgeries a year, one on each foot.  The surgeries lasted anywhere from 2 hours to 4 1/2 hours, I was off the foot totally for six weeks, then started slowly putting weight back on it.  Within months, roughly 6-8 months, the tumors regrew large enough to cause pain and make wearing shoes almost impossible.  Sometimes the tumors grew in the same spots where I had just had them removed, and sometimes they grew in new spots.  The bottom of my feet, and my hands, look like some kind of terrible road map.


6. What information were you given about surgery and other treatment options for this condition? 
The first few surgeries I was told this condition is rare, and rarer in a child.  I was also told that the chance of reoccurrence was very slim.  Fast forward a couple of years and a few surgeries later, the information changed.  I was told the reoccurrence rate was very high and to put off surgery until the pain was unbearable.  In the beginning I couldn't find any information about this disease, but with the growth of the internet I have learned so much, have met so many different kinds of people from all over the world who suffer from this disease, and am a member of a couple different Ledderhose/Dupuytren’s groups.  Other treatment options that I was told about was a cream that is used to reduce the size of the tumors.  My doctor tried to contact the pharmacy but had no luck getting in touch with anyone over a two year span.  I have also been given cortisone shots to see if it reduced the size of the tumors.  The shots did nothing but cause further pain.  I was also told about Cryo where they insert a probe into the tumor and try to freeze the tumor to kill it, but as I said earlier my niece had this procedure done twice and did not have promising results.  My doctor at the time didn't feel this was an option for be because of all my prior surgeries.

7. What treatment options are you seeking now? 
Right now I am waiting to see what my next step is going to be with the cancer drug Nexavar.  Because there is no documentation for this drug and Ledderhose my doctor isn't quite sure of what step we should take next.  I might have to go back on the drug because of the pain and because the tumors are growing again.  Hopefully the time frame will be shorter than my first go around.  I will see the doctor next week, so after that visit I will let you know what path we will be taking.  I am also getting MRI's every three months to check the growth of the tumors and to make sure they aren't affecting nerves and tendons.

Finally do you have any other advice you would like to give to patients?
Read as much as you can on this disease. I have found every case is different so what works for one won't necessarily work for someone else.  If your tumors aren't causing pain and are slow growing don't rush to have any procedures done.  And, make sure the doctor you do pick knows about this disease inside and out.  Don't fall for any quick fix promises, for there aren't any in regards to this disease.  And, lastly, know you are not alone, there are many of us out here that are suffering from this same disease. 

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