Interview with Seph, dual Dupuytren's and Ledderhose surgery patient

Today I have an interview with Seph from the IDS forum who kindly agreed to share her experience with these conditions. One of the few Ledderhose surgery patients who has not had a horrific experience. 

1) Do you have Dupuytren's, Ledderhose or both? How long have you had there conditions and what was your age when diagnosed? 

I have Dupuytren's and Ledderhose. Both bilateral. I first developed LD in my left foot in my early teens. I may have been as young as 10 as I'm sure when it first started but I know I was at junior school. Right foot developed LD in my late teens followed by my left hand and then my right hand in my early 30's. I am now 59. It may be worth noting that I did not know what the issue was or that the feet and hand issues were related until it was properly diagnosed when I was 33

2) Where do you live and do you have a family history of the disease?

 

I was born and grew up in New Zealand but have lived in Australia for the past 25 years. My family heritage is a mixture of Welsh and Scottish with a few Englishmen thrown in. Father had what I now know was DD in one hand, one of two brothers has LD in one foot and one of two sisters DD in one hand. I am not aware of any other relatives with the disease but I haven't seen any of my cousins in years so I wouldn't know if they have developed the disease

3) Would you consider yourself to be at risk based on any of the other risk factors commonly associated with these conditions?

 

I drink wine with dinner nearly every day but that doesn't account for the disease starting in junior school. I have some liver damage caused by a bout of Hepatitis A when I was 6/7. I sometimes wonder if this mimicked the effects of alcoholism and acted as the trigger for me. A few years back I developed bilateral frozen shoulder. My father who was skinny developed diabetes in his 60's and my mother who was obese developed it in her 70's. I am a candidate but don't have it yet and am trying to fend it off through an aggressive Gym and sport program (Gym 3-4 times a week with heavy weights plus tennis 3 times per week)

4) What treatments have you received for

a) Dupuytren's

I had surgery on my left hand when I was 35. It should be noted that the DD in my right hand appeared a couple of years after the surgery on my left hand. No signs off DD in my right hand prior to then. I had NA from Dr Badois in Paris on both hands April 2009 and again on my right hand December 2009. Last year I had NA done by Dr Manet-Chopin in Paris on both hand and she will treat my right hand again next month.

b) Ledderhose

Surgery when I was 13/14 since then nothing.

5) How successful were these treatments?

 

Surgery on my foot did not cause me any problems but the disease returned within months with a broader mass and I wonder if it was the trigger for the disease popping up on my left foot. Surgery on my left hand was moderately successful but I wonder if it was the trigger for the disease then popping up on my right hand. Each time I get NA done I seem to get an immediate burst of DD activity then it settles down again so I get it done again where the action starts and I am right then for a couple of years. I am troubled that of late I am developing a lot more nodules on the palms of my hands and I am wondering if this is triggered by the NA

6) You were treated a while ago, if you were to get treated now would you take the same treatment option? And why?

 

If I could return I would not have the surgery and I would not have waited so long before starting NA. The surgery achieved nothing other than a temporary fix with the scar tissue limiting my future options. Because I waited too long NA was not able to straighten my little finger on my right hand and surgery did not straighten it on my left hand

I do not agree with the people that advocate protecting hands and wearing soft shoes etc. I have found that by pushing through the skin toughens and the nodules settle down. On my feet I went through years where blisters would form in the arches of my feet where the lumps rubbed but the skin did toughen and the pain went away - now no problems. With my hand no special treatment but I have had to thicken the grip on my tennis racquet to avoid cramp caused by DD and in the gym I do now where gloves a lot of the time to reduce the rubbing on new nodules.

Interview with DD and LD patient

This is an interview that I have had for a while and I am waiting for an update from the patient, however they have become silently harder to get hold of so I thought I would post the interview and then I can always add an update. This patient has Dupuytren’s, Garrod’s pads and suspected Ledderhose with some family history. They were early post-surgery which is why we were going to wait.

Read on for the interview:

1) Do you have Ledderhose Disease, Dupuytren’s disease or both? 

I have Dupuytrens Disease on both hands with Garrods Pads to all my PIP joints. Effectively my palm and ring finger to the PIP on my left hand and my right hand is contracted on the palm and to the little finger PIP. Whilst I haven't yet sought medical advice I have a pea sized lump on each of my feet in the arch area which I suspect will be diagnosed as Ledderhose Disease (hopefully I'm wrong)

2) Do you have a family history of the disease? If yes how prevalent is it? If no are you at risk from anywhere else such as diabetes, excessive alcohol, smoking etc?

My Grandfather had Dupuytrens to one hand. The disease has not passed over to (his son) my father or my siblings.

I do not smoke, drink excessively or have Diabetes.

3) What is your age and how long have you been symptomatic? (Can talk about disease progression etc such as how long it took to reach a certain point etc)

I am fifty years old. I think I first noticed the creases in the palms of my hands and the lumps on my knuckles approximately fifteen years ago. I was told by my GP regarding my palms I had Dupuytrens Disease and to stretch my affected fingers. I was x-rayed for my knuckle condition and told everything was fine.

Over time and very slowly the creases in my palm became deeper and imperceptibly the fingers contracted towards my palms. There was no pain or real inconvenience until recently I noticed clapping (the Olympians parade in London) and putting on gloves was becoming awkward.

4) What treatments have you received? 

Apart from my GP's advise to stretch the fingers I received no treatment. Recently I was referred to an Orthopeadic Surgeon who advised partial fasciectomy to my left hand, once that has healed he recommends the same surgery to my right hand.

5) How successful were these treatments? 

I can not really say as I am still wearing the dressing, however I can see that the contracture to the PIP joint has not be resolved, hopefully once the bandages are removed I'll see that the overall angle is less, it feels as if that will be the case (stretching my fingers out within the dressing). I'm also hoping that post operative physiotherapy will improve the situation.

6) You said you had surgery, what was this experience like? 

The surgery thus far has been fine, very little discomfort although I wouldn't recommend knocking the hand! Obviously I'm not sure as to the position after the dressings are removed but at this point I'm feeling optimistic even though I'm prepared for a time of scar healing which I expecting to be sore for some time.

7) Are you currently satisfied with the treatment that you have received?

I am very satisfied in as much that the route I've taken seems to be the most common scenario. I would say the development and use of Xiapex seems to be very promising and given a choice I would of opted for that course of action before surgery.

Is Dupuytren's in your DNA?

As many of you will know I am in a science based job and I did my undergraduate degree in Molecular Genetics. Today I want to cover whether there is a genetic element to Ledderhose and Dupuytren's. As always the information available on Ledderhose is not there and so I am looking at Dupuytren's as if this is a predisposition at the genetic level then it seems likely that this is the same for both.


I felt that a good place to start here is the book - Dupuytren's Disease and Related Hyperproliferative disorders where: 


Chapter 11 - The Genetic Basis of Dupuytren's: An introduction: 

First of all they start by saying that virtually all diseases are a combination of Hereditary and environmental factors and from this I am concluding that this is why it is capable of skipping generations or like in my Ledderhose popping up from nowhere.

The familial incidence of this disease does vary and in Scotland, Sweden, Iceland and Norway for example it is high and several papers have put it as high as 44% and 74% (Skoog 1948 and Gudmundsson et al 2000) They also look in a variety of ways at 1000 patients in a study themselves where they observe that 40% of these have a family history of the disease although I cannot see any note of how recent this family history needs to be they do note that in these cases onset seems to be earlier and develop quicker. Interestingly, bearing in mind other posts that I have done on susceptibility, they do see 11% of cases with diabetes but there is NO link to alcohol or smoking.

From my point of view it makes some sense that if you have a family history you are more likely to suffer from this earlier. I think this because if you do have a genetic background that means then the balance between hereditary and environment is already heavily shifted towards you getting it so that only a slight environmental factor such as trauma, that in most cases would not result in the development of these diseases does for you because of the increased chance you have.

In their concluding comments of this section they do state that there are on-going studies where many countries are collaborating to try and tease out some of the mutations that might lead to increased odds of Dupuytren's and Ledderhose.

Interestingly research has already been done to look into some of the obvious candidates such as TGF-beta (see my picture at the bottom of this post as to why looking at this makes sense) and actually they have not seen a link between the two. This means that the problem lies elsewhere. In Chapter 12 this is discussed further to show that not only have they look at TGF-Beta but other members of that pathway and again no link is seen. This is somewhat surprising as on the one hand you would think that an increased level of TGF-B would result in an increased likelihood of Dupuytren's but all that the above results mean is that this cannot initiate the disease and but I reckon a higher level of TGF would result at least in faster progression of the disease.

It is obvious that there is some genetic element and that it some cases it plays a huge role, in some cases entire countries seem to be at risk once they have reached 50 and I guess these countries are where this research needs to be continued.

Genetics is not the be all and end all of getting these diseases, I on the one hand have no history of either Ledderhose or Dupuytren's but I have spoken to several people with extensive backgrounds of the disease. Yet in all the people I have spoken to only 1 has had a younger age of onset and they had a clear case of massive trauma to the site shortly beforehand. So although there clearly is an increased risk of getting a bent finger or a lump in your foot if you Mum and Dad did, if they didn't it doesn't mean you're safe.

Interview with Ledderhose surgery patient with family history of Ledderhose

So today I have another interview with a person who has had surgery for their Ledderhose, not only that but this person also had a family history of this disease, this makes research very important to them as they do not want their 2 daughters to get the disease. Look below to see her answers to some of my questions as well as a family tree showing the disease. Thank you Ohmyfeet from the plantar fibroma support forum.

1) How have you had Ledderhose and long had you had Ledderhose before considering surgery? and what other treatments had you received? Did any of these treatments work at all?

I have had the lumps for several years without pain. But have been having trouble with them for more than a year. I had previously had injections in several nodules, which was more painful than surgery!! My Dr did not numb it first but used both in the same syringe. Now I am not a wieney when it comes to pain, I had two children without any pain meds, I broke my foot on my way to have a stress test and went ahead and ran on the treadmill and passed with flying colours, only to hobble down to the ER after X rays the Ortho Dr came in and said you are tough!! I spent the next 6 weeks in a cast.

2) Were you at the stage where you couldn't walk before you had surgery / what sort of pain were you in?

I was at the point that sleep was interrupted and I work in a factory and stood all day (recently my Dr. had written a note and was able to sit as needed).

3) What kind of surgery did you have? Did you just have the lump removed or the entire fascia?

 My surgery was a cross between the two, as there were several nodules and each required that the healthy tissue accompanying the nodule be removed, I had several nodules, one of which was about the size of a walnut. Only the affected band of fascia was removed.

4) How did surgery go? How long did the surgery take and what was recovery time like / expected to be like and did / will you have to spend long on crutches?

My surgery took about 2 1/2 hours and I was told that the tissue they removed was about 5 x 3 x 1....I cannot image how much they would have had to take in order to remove all the bands of fascia. I had a nerve block at the knee which kept the foot numb for 2 full days, which was followed by a couple of days of pain meds (which I do not tolerate well). Crutches sucked so after two days I got a knee scooter which was wonderful. Everyone has their own pain tolerance and when you reach your breaking point you too will decide to have surgery, unless of course there are new treatments available.

5) How long ago was the surgery? How was recovery?

I had the surgery four weeks ago today and there is still some numbness in my foot. Each day I feel better. I have been somewhat mobile since the stitches were removed but incisions are hard and painful. I am scheduled to return to work in four weeks, I cannot imagine at this point standing all day. At four weeks post surgery after being out for an hour I must elevate and ice in order to assist in pain management.

6) Would you say that the surgery was worth it? and would you recommend it to people that have Ledderhose? Would you consider having it again?

At this time I don't know if I will have the other foot done.

7) Did you know about the chances of the disease returning after surgery?

Yes, I was very aware of the reoccurrence rate, but given the fact that my podiatrist had to remove so much tissue I (we) felt like with the removal of 90% of the medial band of fascia, that my chances were lower. Hopefully this calculation was correct

I also had one nodule that was in the distal band (which was directly below my fifth metatarsal) which was removed at the same time, although through a separate incision, this lump was not visible but could be palpated under the skin, this came back as not a fibroma but more in the category of a cyst.

I hope we can be of help to people who have this disease.