Friday, 21 March 2014

A patients perspective: A brief overview of all things Ledderhose

This condition, which for simplicities sake I will just call Ledderhose, is an often painful condition which presents as a lump or knot in the arch of the foot or feet. The purpose of this introduction is to try and make a concise overview of the significant amount of information I have learnt over the last 3 years. 

This information has come from books, the internet, and conversations with patients, conversations with doctors and conversations with scientists. I hope to cover a little bit about what the disease is, how it can present itself and how this can be very different patient to patient. I am going to look at the risk factors and the different treatment options and will try my best to offer an unbiased opinion on the treatment options. 

If you are new to the condition hopefully you will find this helpful, if you are familiar with the condition then perhaps you might still learn something. I hope to be able to tell it as a patient, first developing the condition (risk factors), seeking information (the basics of the condition), disease progression and then treatment followed by anything else I see fit. 


Much of the information I have taken from conversations on the related condition Dupuytren's, here I will basically treat the conditions as the same thing from a risk factors and science point of view, the treatment options are Ledderhose specific. 

Developing the condition

Ledderhose is not something you can catch from another person, well not like a cold, but it often will have genetic links. It is thought that Ledderhose requires several changes to a pathway as well as perhaps a triggering traumatic event. The genetic changes that are required can be inherited from your parents, this will greatly increase the odds of you developing the conditions. 

A family history is by no means required for this condition to develop and indeed a strong family history does not mean you will 100% develop the condition. See the family trees shown below: 


Figure 1: 
a) Family tree of a patient with no family history.             






b) Family Tree from a family with a lot of family history of Ledderhose and related conditions.








As you can see from the 1st family tree there is no genetic predisposition for the condition, there is no family history of the condition, this either means that there was no underlying genetic element in this patient or the individual patient likely developed many themselves. The 2nd family tree shows a strong family history of the condition, this likely meaning that they have many of the predisposing genetic elements in place from birth and are therefore highly susceptible to developing the condition. 

What does this genetic side mean? That those who have a genetic background are more likely to develop the condition. 

There are many other factors that have also been linked to the progression of this condition. Nothing has really been proven but it appears that doing things that give you a chance of having underlying health issues will likely results in an increase in your odds of developing Ledderhose.

Other factors that have been linked to these conditions includes over drinking. More specifically it seems to be linked to the possible liver damage that can come from over drinking. Diabetes is another proposed risk factor. The conditions have been linked to various insulin related pathways so it is not surprising that a defect in this pathway can increase the odds of developing these conditions. Another possible risk factor is smoking this causes underlying health issues and in many cases could be linked to excessive drinking.

The final risk factor that I shall mention is trauma. Trauma to the foot could be picked up on many ways e.g. I may have stepped on a stone when running and I have heard a child developing the condition after damaging their foot (personal communication). I shall not go into detail here but Ledderhose seems to share pathways and processes with the healing and scar process and it could be an over-activation of these pathways that ultimately causes the condition. So if you think you are at risk there is no harm in being careful with your feet. 

Disease Basics

So maybe the disease basics should have come first by personally I had no clue what this conditions was before I started developing it and therefore felt that its rightful place was after the factors that can increase your chances of developing the condition. 

Ledderhose lumps are basically a ball of cells that are growing too quickly, therefore they form a tumour. This is much the same process as cancer except they cannot spread so please do not confuse the 2, in fact see this post on why this condition is not cancer. The lumps that are formed are very hard, this is because the nodules that form are high in collagen. Collagen is a protein that is often found in repairing tissue and this is why the condition is thought to start with a trauma and develop as an overreaction to the repair that is necessary. 

Diagnosis and Progression

Diagnosis is normally initially achieved through palpation of the lump and will often then be confirmed with an ultra-sound or ultimately an MRI. It is not until an MRI is performed that the tissue can truly be differentiated between basic scar tissue and a fibroma [Personal Communication].

Disease progression is highly variable, not just in terms of the size of the lump(s) but also in terms of pain. As an example one patient could have a large lump and no pain, another patient a small lump but a lot of pain, the patient with the large lump could have only developed the lump in the last few months whilst the smaller lump may have taken years to develop. I for example had a gradual increase in pain over the course of a couple years until it reached the point where I could not walk. Despite me being unable to walk my lump was not that big. It is not possible to tell whether a lump will become painful or not and therefore it is not possible to guess what the best treatment option will be.

Treatments and considerations:

There are many different treatment options but there is no perfect treatment option. Below I am going to try and cover some of the more common treatment options and discuss the pros and cons:

Surgery:

Surgery has long been the go to treatment for Ledderhose patients. However surgery has also had the problem that it suffers from high recurrence rates. This means that for a lot of patients that have surgery the lumps with grow back. I have spoken to some patients who have had surgery work and are happy with the results many years after treatment. I have also spoken to patients who are unhappy with the results of surgery just months after they had the treatment. I have spoken to one person has had 20 plus surgeries and is still battling the condition. The success rates have increased with the use of greater margins or complete removal of the plantar fascia.

The Pros for surgery are that it is the standard treatment used and is readily accessible.

The cons are numerous. It is invasive, requires a long time off of the foot, it requires physiotherapy and the chances of the condition coming back are high.


Radiotherapy

Me getting Radiotherapy
Radiotherapy has emerged in the last 20 years as a real treatment option for many benign conditions including Dupuytren’s and Ledderhose. When performed correctly the success rate is in the region of 80% of patients seeing an improvement. I personally have had radiotherapy and for me it has worked really well, the process is 5 treatments on consecutive days followed by a repeat of that around 8 weeks later, each treatment takes 5-10 minutes. I went from using a walking stick and being in a lot of pain to being in less pain, then I could stop using the stick and then I started running and completed a 10km run. Overall I couldn’t be happier with how it went for me. I have heard of it not working for some patients and there are risks. Any radiotherapy, even the lose doses used in this treatment, will increase the risk of the patient developing cancer. I suffered from dry and itchy skin on my foot for several months which was irritating but E45 helped.   

The pros are the treatment has a good success rate and is not invasive and doesn't require time off of your feet.
The cons are that in the UK at least you will have to go private to get the treatment and the increased risk of cancer. Limited locations offer radiotherapy for Ledderhose and related conditions.

Other options:

On the NHS in the UK the final option provided is surgery, as mentioned you can get radiotherapy and there are other treatments that will get tried first or are just becoming available.

The first of these is steroid injections. In some cases it has been found that steroid injections can ease the pain and shrink the lumps that are caused by Ledderhose. These injections can be very painful and one specialist I spoke to advised against them because "There is no specific evidence that I am aware of that steroid injection helps or improves this condition" [Link]. There is no overwhelming evidence that it will help and it can cause problems with the tendon. I did have a steroid injection, it did hurt but it did seem to ease symptoms for around 6 months to a year.

The second option is very likely to just be a stop gap solution. This option is orthotics. Orthotics are shoe inserts that are supposed to help distribute weight around your foot and take weight off of the lump. They can help but will need changing if the lump is growing or your walking bio-mechanics are changing as the pain increases. I still use orthotics now, for me they are more of a corrective tool to try and help me walk normally now that the pain has gone.

The third option is just becoming available in the UK, cryotherapy / cryosurgery. This option involves freezing the area with a very cold needle, this numbs the pain indefinitely and has been shown to have good results. This treatment does not however have any publications proving it is a good and viable treatment option. There are also very few locations worldwide that offer this treatment for Ledderhose.

The fourth and final option is a gel called Verapamil. I am not aware of this being widely available in the UK but it is available in the USA. The gel is supposed to block a pathway required for nodule formation and this will stop growth and ideally cause the lump to shrink. I guess the best way to sum this treatment up is as a quick summary of most patient responses; 
"Verapamil may have helped, it may have stopped it from getting worse or have slowed it down but I am not sure". The gel seems to help some, in most it helps but not enough.  

I have not mentioned Xiapex, this is an enzyme injection. In theory it and similar injections can be used in the foot but this is currently mainly a Dupuytren's treatment. 

After treatment:

What happens after you have had treatment is highly variable not only from treatment to treatment but also from person to person. With surgery there will be time on crutches and a high chance of it coming back. With radiotherapy there will be dry skin and the increased risk of cancer. 

Something that all patients will have in common is the fear it will come back or indeed the fear it will spread. The hands and the feet are all potential targets as is the shoulder and other areas I would rather not talk about.  

Summary

I guess one of the biggest things is that the ultimate go to option still seems to be surgery, yet here is a quote I got from a UK surgeon about this condition: 

"Finally the text book, which is Surgery of the Foot and Ankle, is probably the most authoritative text we have on foot and ankle surgery and it quotes “attempted surgical resection is best avoided”."

This shows that the go to treatment option is one that is best avoided. I am not saying don't have surgery I just think that you should always discuss all the options with your doctor before having surgery for this condition as there is no guarantee it will help, in fact it can even make things worse. 

Now patients and professionals need to work together to improve patient care, to improve both professional and patient awareness of all the treatment options and make sure that everyone gets the treatment that gives them the best chance to heal.  

References

You might want to check out the following link: Plantar Fibroma - A real pain in the foot

For more information on different section and to see where I obtained the original information from please use the following links: 

Risk Factors:

Genetics

Dupuytren's Disease and Related Hyperproliferative disorders, Chapter 11 - The Genetic Basis of Dupuytren's: An introduction

Is Dupuytren's in your DNA?  - A blog post previously done by me

Diabetes: 

Elhadd et al, 2007, Plantar Fibromatosis and Dupuytren’s disease: an association to remember in patients with diabetes, Diabetic Medicine, 24, p1305:

Stradner et al, 1987, Dupuytren's contracture as a concomitant disease in diabetes mellitus, Wien Med Wochenschr, Vol 137, p89-92.  



Alcohol and smoking: 

Harrt and Hooper, Postgrad Med J 81:425-428 doi:10.1136/pgmj.2004.027425, http://pmj.bmj.com/content/81/957/425.long


Burge et al, 1997, also find the same results as the above paper in that alcohol is related to Dupuytren's and this is further backed up by (5) Bradlow et al 1986

Gudmundsson et al, 2001, Scand J Health care, vol 19.

An HS, SouthworthSR, JacksonWT, RussB. Cigarette smoking and Dupuytren's contracture of the hand. J Hand Surg Am. 1988Nov;13(6):872-4.

Treatments: 


Cryosurgery post by me with more links contained

Steroid injections: 

Steroid injection - a post by me 



Meek et al, 2002,The effect of steroids on Dupuytren's disease: role of programmed cell death, J Hand Surg Br. 2002 Jun;27(3):270-3.accessed 21//01/2012

Plantar Fibroma Support Forum - Post by peekaboo64 http://plantarfibroma.freeforums.org/injections-t9.html

Dupuytren Online, Steroid Injections - http://www.dupuytren-online.info/dupuytren_steroids.html accessed 21/01/2012

Othotics: 

Orthotics: a post by me


Radiotherapy: 

Personal experience and personal communication with Dr Shaffer and other medical professionals

Interview with Dr Richard Shaffer - He covers a lot of the basics and there is some good information.

Radiotherapy post by me

Book: Radiation Therapy for Early Stages of Morbus Ledderhose: Dupuytren’s Disease and related hyperproliferative disorders, chapter 50 – Long-Term Outcome of Radiotherapy for Primary and Recurrent Ledderhose Disease:

Surgery: 

Personal communication with multiple patients 

Surgery a post by me 

Dupuytren's Disease and Related Hyperproliferative Disorders: Principles, Research, and Clinical Perspectives -

Charles Eaton M. Heinrich Seegenschmiedt Ardeshir Bayat Giulio Gabbiani Paul Werker Wolfgang Wach