First, Saint Valentine. There were nearly a dozen people called Saint Valentine. Did this one have Dupuytren Contracture? He’s clearly in the high risk demographic – an old Caucasian man. Looks like he’s got it, but we’ll never know. It is important to know that there’s a lot of variability of Dupuytren disease – how much trouble it is and the fact that not everyone falls into the common demographic, as you’ll see.
Second, a sweetheart. Brandy Leigh Scott has dealt with Dupuytren contracture since childhood. Severe, crippling Dupuytren contracture which has resisted available treatment. Brandy was kind enough to participate in an online conversation to discuss her experience with Dupuytren disease. This interview is one of a series of Coffee Talk discussions featured in Facebook Dupuytren’s Disease Support Group (DDSG) https://www.facebook.com/groups/879351412113444/, a very active Dupuytren advocacy group. What follows is a transcript of the original interview from January 2017. In this transcript, <BLS> is Brandy Leigh Scott, <CE> is Dr. Charles Eaton, and <LWR> is Leslie Wieslander Rosenthal, the group founder and administrator. Because this is a private Facebook group, all other discussion participants are shown as <DDSG>.
January 7 COFFEE TALK – Brandy Leigh Scott and Dr. Charles Eaton Discuss Dupuytren Disease in Childhood.
<LWR> Brandy Leigh Scott was diagnosed as a child and has had many operations on her hands. She has graciously agreed to answer questions for us about her treatment throughout the years. Brandy’s first surgery was at age 7 for knuckle pads. She was not properly diagnosed until the age of 10 or 11. Brandy is now 41 years old and has had about 25 procedures over the years, from traditional surgery to NA and Xiaflex in both Switzerland and LA.
Link: Pasadena native’s hand disease inspires global 3-D printing challenge:
<LWR> Good morning Dr. Eaton – we are ready to start with your questions! Thank you for being here with us today.
<CE> Hi, Brandy – thanks so much for sharing your story with this group.
<BLS> Good morning Dr Eaton – let’s get this started!
<CE> Pediatric Dupuytren disease isn’t just uncommon – it’s pretty rare – only a few dozen reports. There’s not enough experience to know whether pediatric Dupuytren disease is a variation of adult Dupuytren disease or something completely different which looks like Dupuytren disease. Reports of pediatric Dupuytren disease have short follow up – months to years – which makes it harder to understand. There are other problems which can look like Dupuytren disease in children or infants – such as recurring digital fibroma of infancy, which isn’t Dupuytren, but looks like it. Until we have a biomarker test to tell Dupuytren from other problems which look similar. Doctors have to guess based on the person’s story. Here are a few “story” questions to follow which may help fill out your Dupuytren story and put it in context for other group members.
<CE> Your hands look just like you have aggressive Dupuytren disease. Many people with aggressive Dupuytren disease have other things going on. For example, does anyone else in your family (blood relative) have Dupuytren disease?
<BLS> According to my mom, her grandfather had it. But no one knew what is was called or it was even a condition back then. My Mom’s family was from the eastern shore of Maryland – crabbing & shucking oyster was a way of life. They actually though the curling of the finger was from all the years of shucking oysters.
<BLS> I know my Mom’s mother had a few small lumps in her palm that she – in her words – just kept pushing back in. My Dr in MD (Dr Michael McClinton) looked at everyones hands when I was first diagnosed.
<BLS> And just recently, my Dad thinks he might have a small lump forming in his hand.
<CE> Same theme as people thinking that Vikings got it from pulling on oars, or Dr. Dupuytren saying that his first patient, a coachman, got it from pulling on horse reins.
<CE> Heavy work may speed it up, but is a secondary factor compared to genes.
<CE> Many people with aggressive Dupuytren disease have foot involvement – Ledderhose, which makes lumps in the arch of the foot – the instep. Any issues of this nature?
<BLS> I don’t think so, but to be fair I have never had my feet checked. Just feeling my own foot, I could potentially have a cord but I’m not a doctor……….
<CE> There wouldn’t be much question if your feet were at all as bad as your hands. Sounds as though you dodged foot problems.
<CE> Many people with aggressive Dupuytren disease have other unexplained lumps – on the front of your wrist or your kneecaps. Anything like that?
<BLS> no, both of those seem to be fine.
<CE> That’s one less thing to worry about. So many different patterns of this problem. An example of how doctors can only guess at what’s going on without a blood test or some other way to quantify the biology.
<CE> You’ve had surgery on your hands. How long was it between your diagnosis and your first hand operation?
<BLS> I had my first surgery when I was 7 – my parents noticed in a school photo that there was a circle around my knuckles – so they took me to a hand doctor. He removed the knuckle pads and when they came back, maybe a year later – he admitted he had no idea what is was.
<CE> Lumps on the hands of children are not common. Dupuytren knuckle pads would be way down the list of diagnoses for a seven-year-old girl, even for a veteran hand surgeon.
<BLS> My mom was babysitting this kid who had just had brain/head surgery at Johns Hopkins by Dr Ben Carson. Dr Carson recommended we see Dr McClinton. At that point a small lump had formed near my pinkie. Dr McClinton diagnosed me immediately and my parents remember I had surgery pretty soon after he saw me, I was around 9 or 10. So 1984/1985.
<CE> Even in adults, Dupuytren is often misdiagnosed at first. The average adult with Dupuytren disease sees more than two doctors before they are properly diagnosed.
<CE> When was your last surgery?
<BLS> I haven’t had traditional surgery since I was about 19/20 – 1994/1995. My last Xiaflex shot was around 4 years.
<LWR> Do you know how many degrees of contracture you had when you had the xiaflex?
<BLS> I had Xiaflex on my left hand…. I should clarify that I have NEVER had traditional surgery on my left hand. That didn’t start progressing until 14/15 years ago. I know it was completely straight when I moved to LA in 2000. All of my surgeries were on my right hand.
<BLS> For my left – my pointer finger was bent about 90 degrees, and we got it completely straight. I was running around the office shooting people with my pointer finger gun.
<CE> What’s been going on with that finger since then?
<BLS> it’s back to completely bent.
<CE> A reminder of the work that has to be done to develop a medical treatment to slow or stop the biology.
<CE> The majority of pediatric Dupuytren reports are of no recurrence – but are short followup – a year or so. Patients with severe contractures may also feel that their disease has “burned out”, and stops getting worse, although sometimes after they have developed severe contractures. Do you feel as though your hands are “stable” or are they getting worse?
<BLS> My right hand is stable. Right now I feel like that is more scar tissue and Dupuytrens fighting one another.
<BLS> My left hand has gotten worse. I just realized the other day that my middle finger used to be able to raise out of my palm, and sadly it cannot anymore.
<CE> It may stay stable. Once someone has enough scar tissue or enough joint ligament changes, these may “protect” the Dupuytren tissue from being pulled on. That may calm down the biology of the contracting process.
<DDSG> That is interesting.
<BLS> Would there be scar tissue in my left hand if I’ve only had surgery on my right?
<CE> The end result of Dupuytren biology IS scar tissue, and it may be the final thing which stabilizes disease, sometimes by making the fingers too awkward to pull on. Just as now how you type with the backs of your knuckles rather than your fingertips.
<CE> Xiaflex doesn’t remove the Dupuytren scar tissue, but rearranges it.
<CE> People with severe Dupuytren disease and particularly early age of onset often have itching, burning, or tenderness in their palms. The literature is not clear about how often this is the case in pediatric Dupuytren disease. Have you had problems with itching, burning or tenderness in your palms?
<BLS> I’ve never had any of those issues.
<CE> People sometimes try nonsurgical treatments such as nutritional supplements, long term splinting, radiation therapy. What other treatments have you tried?
<BLS> I only did splints right after surgery. And radiation wasn’t an option back when I was having surgeries.
<BLS> I also think my body hates splints after all of the years of surgeries. Even 5 years ago with the splint after Xiaflex – I take them off in the middle of the night and throw them across the room – in my sleep. Every morning I play the game of “where’s my splint?”
<CE> Splints are traditional treatment. There’s controversy about when to use them and how well they work. Important that they be comfortable (loose) enough to wear overnight. If they’re too tight, eventually, everyone learns how to take them off in their sleep, and they wind up on the other side of the room all by themselves..
<CE> It’s a common problem, especially for people who are really motivated, who say to themselves “I can put up with this… I want my fingers straight!” Problem is, your sleep self has different motivations, like staying asleep!
<DDSG> My sleep self must be little Miss Goody Two Shoes.
<CE> If you were going to do things over with your hands, anything you would have done differently? What would you tell the doctors who have cared for you in the past?
<BLS> I wouldn’t do much differently – except get this disease in 2016 when there were other options than just surgery. 🙂
<BLS> The only thing I wish we could have done more of was went I went to Switzerland around 2002 for NA procedure. NA wasn’t approved here in the states so I flew there to try it out. Had 4 rounds in 5 days and my hand opened up by 20 degrees, if not more. I wish it had been available here or I had been able to go back and forth between Switzerland.
<CE> The trick will be to develop a treatment to keep the contractures from returning regardless of how they are straightened out.
<CE> I’m going to be in San Francisco this September at the annual conference of the American Society for Surgery of the Hand. Any chance that we could get together then?
<BLS> yes. I would love that. I’ll PM you my details.
<LWR> Thank you Dr. Eaton! I’ve got a few questions for Brandy, then we will open the thread up to our members for their questions.
<LWR> Good morning Brandy – thank you for being available to answer our questions! I think all of the parents on this that their children or grandchildren could get Dupuytren’s Disease and wonder how they would manage their care. We know so little about the appearance of the disease in childhood. Do you remember your first doctor appointment and how you learned that you had Dupuytren’s Disease and that it was rare in children? I’ll bet it was scary to learn that you would have to have a surgery.
<BLS> I honestly don’t remember being diagnosed. I just feel like I was always at the doctors, in surgery, or in therapy. So much therapy. I think my parents were just happy to know what it was and to have a plan once I was properly diagnosed.
<LWR> You were quite young at the onset, but do remember before it was visually apparent, if your hands felt suddenly different, with certain tasks like using scissors or coloring for example? Did you have aches and pains in your hands?
<BLS> No, unfortunately I don’t. (or maybe fortunately?) I asked my parents if they remember anything I couldn’t do – and my mother said I was “unstoppable” – it has never slowed me down. The only thing I remember is that I had to quit playing lacrosse – a request from my doctor. I had to choose between volleyball & lacrosse – because he was worried I would have done more damage to my hand if it got injured. I quit and refused his recommendation of playing soccer.
<LWR> Parents naturally want to do all that they can if their child has special needs, but often we make mistakes in helping too much or not in the right way. What would you most like to tell all the parents with a child or grandchild with this disease?
<BLS> I was (still am) pretty strong willed and wouldn’t ask for help. I made sure I did everything on my own, or at least tried. Whenever they would just do things for me, it honestly irritated me to death. I had to learn to live with this, which I figured out pretty quick I think, so I’d say to anyone who has a kid with this, let them figure it out. Not only do they have to do everything on their own when they are out of your sight, but if you are like me and get it early – you have the rest of your life to battle this – so you need to know how to adapt.
<LWR> What a great attitude!
<DDSG> At what age did you take over responsibility for the treatment of your hands?
<BLS> I’d say, high school? I remember at 18 telling my parents I was done for a while. We always made decisions as a family – mainly making sure surgeries happened when I didn’t have something going on at school. Even now at 41, I call them when something new happens with my hands to run it past them. My parents flew out for a few of my Xiaflex shots and they were both with me in Switzerland the first time I did NA.
<LWR> Were you able to learn how to drive at 16? Were there any concerns about your ability to manage the steering wheel or gears? Is your car specially fitted to make it easier to control?
<BLS> I could never drive a stick since I had surgery every year in high school – but I was fine with an automatic. Plus my left hand was still open then. Even now, my car is still the way it came off of the assembly line – no modification.
<LWR> When you were a child, were there any accommodations for you in school? Did the teachers give you class notes?
<BLS> I’m convinced my chemistry teacher just passed me since I missed 2 classes per week. And when my typing teacher tried to fail me – I reminded him that I had to look at the keyboard and I squeezed by with a B-.
<LWR> Do you have siblings? If so, how are they affected and were they helpful to you growing up?
<BLS> no siblings. which you would think would make my parents remember details better since it was just me. Alas, no. 🙂 Love you Mom & Dad! (if you ever read this)
<DDSG> Did your surgeries bring relief, for a period of time, or just aggravate your DD?
<BLS> I think they gave me temporary relief. But I’d be curious if there was a way to see scar tissues Vs DD which one was winning in my right hand.
<LWR> Hopefully when you see Dr. Eaton in California he can tell you that!
<LWR> I would now like to open the thread up to our members.
<DDSG> I looked into 3D printers for creating some devices for me last summer. Do you have any recommendations for printers and/or design software (I teach tech too, so having one would help my classes.)?
<BLS> Email Mara Hitner at Matterhackers – email@example.com You can also check out all of the designs from the contest we did over the summer at http://matterhackers.com/withinreach
<BLS> I’m not a 3D printer specialist. But the folks at Matterhackers are amazing and can help you out with whatever you need. They especially love talking to teachers about it. Mara is my BFF (she’s here now) so she’ll point you in the right direction.
<DDSG> Did you ever feel depressed or angry as a child, or was it just part of your life? How did you learn to accept your condition?
<BLS> It was all I ever knew. It was (and is) just a part of life and a huge chunk of who I am. My mom said I never let this stop me – I’ve changed how I do things, and I’ve swerved here and there to adapt, but I rarely pull – as I call it – the “hand card” – saying I can’t do something because of my hands. Unless it’s giving hi-5s. you guys are on your own there.
<LWR> Your can do spirit is amazing! It’s a good reminder not to let our attitudes get in our own way. One of the biggest impediments, I think, for those of us with less severe disease, or severe disease in early stage, is fear of doing things that will aggravate the disease. So if there’s the least chance of stress on the hands, we tend to avoid it and therefore, we say we can’t do it. Loss of grip strength in people with this disease is common and affects the ability to open bottles with tight lids, etc. We are learning to adapt with the disease onset in later life. Are you able to grip bottle tops and twist them off, or do you use an aid to help?
<BLS> I do remember being pretty bummed out when I had to quit guitar. After every surgery I had to relearn the same songs, so I just gave it up when I was a teenager.
<BLS> Bottle tops on soda and water bottles are tough but I can do it – or i just hand it to a friend. I have more issues with holding a water bottle than I do opening it. For cans & jars, I have 2 contraptions in my kitchen that are hands free openers.
<DDSG> I have LD. My son has had a bump on his finger appearing when he was a teenager and family physician looked at it back then and dismissed it. He is 20 now. Got to wondering if it is DD. So what is the trigger or sympton to tell him to watch for to indicate it is time to seek out a specialist? I know my LD wasn’t a problem for a decade and then I waited too long. Of course, I don’t want him to make the same mistake.
<BLS> I’d keep an eye on it. Look for dimpling, pulling in – anything like that. Take him to a hand surgeon now – it can’t hurt. Especially if it rules it out completely. I’m sure <LWR> has recommendations of people in your area.
<LWR> Have you ever had occupational therapy to help you learn how to better navigate your environment or use assistive devices.
<BLS> nope. I’ve been winging since 1982.
<DDSG> Did you have any other medical conditions or anything you thought was just strange happen in your body?
<BLS> Thankfully no. Unless puberty counts. 🙂
<DDSG> Have you come across any other really cool assisted devices you can share with us ?
<BLS> some really cool designs came out of the Matterhackers challenge. Feel free to check out the designs below. I especially loved the kid who made a design to assist with typing, the guys who made the door handle, and something just as simple as the assistance with sweeping – it’s all things I never would have thought of.
<DDSG> Thank You – Nothing quite as cool as an innovative mind !
<DDSG> How would you advise people to manipulate their medical care or after treatment ?
<BLS> Find a physical therapist that you love – you’ll be spending a ton of time with them – and listen to them and your doctor. But you also know your hands better than anyone else – so if something doesn’t feel right to you – speak up! I can’t tell you how many times I have had my therapist amend what they were doing because it wasn’t working for me.
<DDSG> Great advice !
<DDSG> With so many doctors not wanting to treat until surgery , what message would you give to the medical community when encountering Dupytrens?
<BLS> get a second opinion. The best thing about this forum is the ability to ask questions to a community whom are dealing with the same issues. I was diagnosed at 7 and didn’t meet anyone with DD until a friends wedding when I was 33. No one had every heard of this while I was growing up. Doctors need to be trained in every aspect of dealing with DD before surgery is performed. I think (and I am in NO way a doctor) having NA or Xiaflex before surgery is just an easier battle to start out with in this process.
<DDSG> Well – you are in good company with lots of us out here trying to get a hand on this disease !. Thanks for you input Brandy.
<LWR> Do we have just one more question for Brandy before I close the thread?
<DDSG> What is the best thing someone ever said to make you feel better about your hands?
<BLS> I’m always happy when people forget that I have a condition. And last night when my mom told me I was “unstoppable” when I was a kid – that made my night. Much like a yelp review I tend to only remember the crappy comments.
<DDSG> What would you advise people with DC that are in a lot of pain to try to help relieve it?
<BLS> Thankfully I have never been in pain with my DD. The only time it is somewhat painful for me is when it’s cold out – my joints ache.
<DDSG> It seems that with all the procedures and surgeries you have had over the course of this disease in your life to improve function have ultimately left your hands with limited function. Have you stopped having procedures and are now focused on adaptation?
<BLS> I stopped having my right hand worked on when I was about 20. I was just tired of always being in bandages and going to therapy. And it was starting to effect my job with all of the time I was missing.
<BLS> With my left hand – it hit so quick and aggressive, I did NA & Xiaflex but they both worked for a short time and then I was back to the drawing board. I need about 5 shots of Xiaflex just to get my left hand open, and then probably 1-2 every year to keep up the maintenance of keeping it straight. That’s time & money I just don’t feel like giving up. It sounds terrible, but this disease isn’t killing me – I’m ok. I just adapt and figure out how to do things my own way. If a miracle cure comes along, I’ll be the first in line. And I’m meeting with a Dr here in LA this month just to check my progress. But mainly, I’m just adapting at this point. One of these days, I’ll cut them both off and have amazing swiss army knife attachments and can do party tricks.
<DDSG> Edward scissorhands, lol
<LWR> Please no more questions – we have reached our max.
<DDSG> The Coffee Talk and this thread is now closed and the forum is open to our usual discussions. Everyone let’s thank Brandy and Dr. Eaton for a very informative Coffee Talk!
<LWR> Thank you Brandy Leigh Scott!
<DDSG> Thank you to both Brandy and Dr. Eaton. Brandy you are amazing and an inspiration to all of us.
<DDSG> Brandy truly is a remarkable woman, isn’t she?!
<LWR> Thanks, Dr. Eaton!
<CE> Thanks very much, Brandy.
<DDSG> Thank you, Brandy and Dr. Eaton for taking time out of your day to answer these valuable questions to help all of us.
<CE> It’s a team effort. Let’s keep this momentum going as we work for a better future for the global Dupuytren community! Together, we can make it happen.
<DDSG> High five to that! Or whatever number you manage to raise high.
<DDSG> Thanks for all your answers. Very helpful and appreciate it
<DDSG> Thank you so much <CE> and Brandy Leigh Scott, and thank you <LWR> for arranging this. And remember girls: keep wearing those night splints! lol.
<DDSG> Thanks to both of you !
<DDSG> Thank you!!
<DDSG> MacDermott Thank you, thank you! I love these coffee talks – always learn so much!
<DDSG> Thank you both.
<DDSG> Thank you both. I only have early case but am surprised how limiting it already is. I thought I had carpal or another trigger finger. This was such a surprise diagnosis to me.