Dercum’s Disease, a Painful Mystery


Glenorah Helfrich prefers the Latin name for the rare disease that clutches her with pain from globs of fat called lipomas that form beneath her skin, robbing her of energy and strength.

Adiposis dolorosa is “much prettier,” Helfrich said, than “Dercum’s disease,” a syndrome discovered 125 years ago that continues to stump specialists and defy accurate diagnosis.

Dercum’s has neither a known cause nor cure and by some accounts is among the 10 rarest diseases on Earth. Some suspect Dercum’s is more common than perceived but is often misdiagnosed or not diagnosed at all.

Helfrich, 67, doesn’t really know how long she has been afflicted. In 2008, she began having trouble lifting her arms. Within six months after moving from Afton to Columbia to place her husband, Tom, in a local nursing home, rapid weight gain caused her to go from a size-14 to a size-24 dress.

“You have no idea what that does to a woman’s ego,” she said. Forty pounds in six months. “Even Tom noticed that I was gaining weight. And he didn’t notice anything.” She noted the irony in light of her husband’s worsening dementia and his lifelong indifference to hairstyle and makeup.

And there was pain, always the pain, the most universally debilitating symptom of the disease because the lipomas either rest on or press against nerve fibers. In one type of Dercum’s, the lipomas themselves are painful.

Noted Dercum’s researcher and physician Karen Herbst, whom Helfrich visited in May in San Diego, said the best way to understand the pain is to imagine what it’s like when a joint or a muscle hurts. Similarly, with Dercum’s, the fat tissue — and there’s a lot because the disease creates more of it — can be painful.

“Most normal people don’t feel their fat,” Herbst said in a phone interview. “I just don’t think that people understand that in Dercum’s, there’s actually pain in the fat tissue itself.”

A Facebook network of people with Dercum’s from around the world tells the story again and again about the frustrating path to diagnosis and perfunctory medical exams that lead to a common conclusion: “You’re fat. You need to eat better and exercise more.”

Helfrich had the same unhappy experience — a carousel of doctors and specialists, misdiagnoses and misunderstanding, until finally in the fall of 2012, a Columbia dermatologist mentioned Dercum’s.

Looking back, evidence that she had the disorder had been present far earlier than 2008. Those nodules behind her ears, especially that one that pressed on her ear canal, when did they first show up?

Other, more pressing matters at the time had demanded her energy.

In 1999, Tom began having difficulty walking, his handwriting changed and a series of small strokes exacerbated the onset of dementia and erased any recollection of regular falls and missteps that left bruises and broken bones. Helfrich began a 4½-year mission of becoming a thorn in the side of the Department of Veterans Affairs, insisting her husband’s condition was caused by something he encountered during his military service in Vietnam.

Also, before her own piercing pain finally won her attention, Helfrich watched her mother battle cancer. Twice.

Eventually, she made the hard decision to place Tom in a nursing home, where he could get qualified, professional care. She found a way to pay the $4,000 monthly bill until Veterans Affairs stepped in — after bankruptcy — and acknowledged a connection between Tom’s dementia and Agent Orange.

Tom Helfrich was 56 in 2003 when she took him to the nursing home in Festus, one week after spending Thanksgiving with him and other family members. She told him about her decision just before the holiday. “I didn’t know how to tell him. I knew he would be angry,” she recalled. “I knew he would be hurt.”

At that point, a silent scream began to grow within her. It forced Helfrich to mumble when she spoke and to take small bites when she ate, careful to chew ever so slowly, afraid that if she opened her mouth wider the scream might escape.

And then it did.

It was her own anguished, soul-deep reaction to that awful day when she moved Tom into the nursing home.

“It felt like leaving a little puppy at the door,” she said. “Nobody should ever have to go through that with their spouse.”

With tears flowing, Helfrich went to a movie — “I couldn’t go home” — then decided she was out of tears.

“I haven’t cried since,” she said.

Suppressing the guilt she felt for turning Tom over to caregivers and trudging through her own loneliness seemed unbearable.

 

Read her full story here. 

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Global Genes Comments

  1. Korry Robinson says:

    Anyone have any suggestions to help younger women with dercum’s disease, many smaller lumps on the abdomen, and all of the issues that come with periods? I got my first symptom at the age of 21, I’m now 30 and still offically undiagnosed. My family doctor has one of the largest and longest practises in my area, and if I am ever diagnosed, I will be his first patient. Visiting my cousins I was pointed to dercum’s disease (3 have been diagnosed,) after 2-3years of confusion,silence,and frustration for both the doctors and I. Just IBS was diagnosed(even though I can handle dairy) and comments like “those lumps don’t hurt right.” I’m sure everyone can relate to that. My doctors have suggested an IUD which has helped lessen the pain and symptoms a bit for a period of time. Just hoping for any tips or suggestions that have helped fellow dercum’s patients. I’ve never reached out to anyone besides doctors as the only similarly affected people I know are men or have the lumps that are large and form on the spine and not the hundreds that cover the whole torso.

    • Angela L says:

      Dear Korry,
      Most important is to go to see the “right” doctor to get the “proper” diagnosis . Since Dercum’s disease is a rare condition ,most doctors are not familiar and dont even know how to treat it as the medical literature is scarce on this disease. As you have read from all of us who have Dercum’s , it’s not only frustrating , but also humiliating as well as time and money wasting to see doctors who are clueless about the condition and even thinks that you fake out the symptoms. The best expert to see is Dr Karen L Herbst in Tuscon , Arizona. ( You can visit her website or the website of the Herbst clinic)
      I actually diagnosed myself of Dercum’s from the information on the internet and had learnt much from other patients’ blogs more than anywhere else. I was lucky that I was probably a bit earlier in the disease and now with Dr Herbst’s prescribed treatment of meds , the special diet together with the hydrotherapy and lymphatic drainge massage, I would say my symptoms are well controlled ( I have lost 40 pounds and the pain is now more bearable) It would be too soon and optimistic to say disease “cured” as newer smaller granular lesions are still coming out. The newer ones hurts more than the bigger lumps.
      I am hopeful that the cure to Dercum’s is on the way as more research and studies have been done. Let’s hang in there and better days shall be ahead.

  2. PatriciaPalmer says:

    Iwas diagnosed with Dercums Disease 3 years ago b
    firstly by my GP then confirmed at Truro Hospital Rhumatology dept. Quite frankly I have been on my own since, as they informed me that there was no cure, and it wasn’t life threatening unless the lumps attached themselves to vital organs. I had rapid weight gain and the doctors would not believe me that I wasn’t gorging myself on chocolate and donuts!! I have lost all confidence with the medical profession as I am fed up with trying to explain that I do not stuff myself with food. One surgeon informed me that Dercums did not cause weight gain. So I have given up. I try to manage it by pacing myself but there are days when the fatigue get me and I have to rest. the Fibromyalgia that has come with it makes it very hard to do much at times. I have resolved myself to having to live with it for the rest of my life, as none of the medical profession seem interested.

    • Dear Pat,
      Im sure you are not alone on this blog page. You will find many others sharing the same nuisance with those painful lumps. I did gain 30 pounds too with those lymphoedema swelling.

      To add to my misery, I have a 14 page drug allergy list , which includes all pain killers. The only thing I can take is tylenol(panadol). So the only option for pain relief in my case is faithfully doing physiotherapy ,hydrotherapy n lymphatic massage.

      I find the lymphatic massage most helpful in both pain relief n losing those excessive fluid swelling. Now my legs dont look like the elephant’s legs that they used to about a year ago.
      Hydrotherapy is a fun activity for pain relief n to get our muscles moving . It is very important to keep exercising, despite how painful, to prevent our muscles from non-use atrophy. It is very soothing in the warm water n I do shed some pounds peeing out the water after each session. I always arrange a treatment physiotherapy session the day after my hydrotherapy session as my muscles n joints +those lumps will hurt like hell.
      But no pain, no gain. In this way, I am able to exercise n lose my 30 extra pound Also, surprsingly,some of the lumps seem a bit smaller too.

      Yes, it is frustrating n there are days that seem like we are still standing in square one. But dont lose hope. I am sure you can always find a means to make your Dercum situation better n more tolerable.

  3. Elizabeth Wilson says:

    With all the lipomas I have had removed and they continue to come back, I can almost guarantee this is what I have. They also just recently diagnosed me with sarcoidosis. As a sufferer who takes pain meds daily, the one thing I found that works for the pain specifically from lipomas is the icy hot with lidocaine. They also have aspercreme with lidocaine. Most of my pain is in my lower extremities from the lipomas, but have had them in my neck and abdomen as well. For all of you suffering with the pain, I suggest you try the icy hot with lidocaine. For the people that aren’t sure if they are lipomas, you will want to have at least one removed to make sure it isn’t sarcoidosis or liposarcoma( fatty tissue cancer). Sarcoidosis can leave granulomas clumped together which will make it look like lipomas. Here’s to fighting and finding cures! If you’d like to talk, or need insight, you can add me on fb at Elizabeth Littlefield-Wilson

  4. Hi I am Kathy I am sure I have Decrums Disease and I am really scared.

  5. Art Collins says:

    I am a 67 year old man. Last September I started noticing pain in my left side. I found what was a small lump. It hurt and then if it got pressed by a finger then the pain really intensified. I woke up on 10/12 and my wife discovered a black and blue spot the size of a quarter. The bruising kept getting bigger and bigger. My PCP sent me to a surgeon. He had no idea what it was and in fact told us that he was not sure that he could help. The biopsy showed that I had a neuroma. More spots of pain kept showing up on my left side. I had a pain management doctor that was providing meds to deal with the pain of arthritis in my back. Soon the pains of the many spots took over my being. Most of the time I did not even think of my back. What was weird to me is how could it be possible for a “neuromas” keep showing up in different parts of my torso. My surgeon said that he had never seen anything like this in his 25 years. He suggested a new pain doctor and an appointment was made for 1/14/16. The surgery was in November so I still needed pain management for the pain. My pain doctor said that he wanted to do epidurals and other types of shots. I said that will not help. I told that doctor that I would not be coming back and that my drug contract was over. So this past Thursday Dr. Bowden say me and as I was describing my symptoms he said that he had some thoughts come to his mind. He excused himself for about 4 minutes and he came back and told me about Dercums disease. We went over the internet results about the disease and wow it fits me to a tea. We then found the name of Dr. Herbst here in Arizona. So on Thursday PM I made a call to set up an appointment. The lady that was doing the scheduling told me that her computer would not go out any further than 5/18 and nothing was available. The air was just sucked out of me. What are we supposed to do with this rare disease? Also last night and tonight I had very small amounts of food for dinner. And both nights my insides hurt like they are on fire. Is this a symptom of the disease? Is there anyone that can correspond with me that is in the same physical boat that I am?

    • I have not been officially diagnosed as yet, but know this is what I suffer from. Will recommend my Doc look into it tomorrow. Have spent years researching various possibilities for several developing ailments associated with Agent Orange exposure (Thailand AfB), and this new (starting 3+yrs) one was baffeling to me. I know there is a connection with this and the Lymph system, as it follows closely that system. I am not a doctor though, and you should run anything I say, through him/her. I have been experimenting with self “lymph massage” , but am 2nd thinking that. I think it is good, but not sure if it might enhance the spread. Please consult with your Doctor about this. And use your computer to check the Lymph system and it route through the body. Please keep posted any treatment or information that might help others with this condition. I will do the same. God Bless and good luck.

    • Mrs Kathryn Eddy says:

      Hi, I’m 62, from the UK and I’m quite sure I’ve got Dercum’s! I noticed lumps in my breaststroke, referred to Breast clinic and had excision of fibroma! Then more became evident! My hips are full of these large painful lumps, non smooth, in my back, tummy and arms! They’re very painful and the pain comes and goes! Analgesics do not help! I’ve been referred to an endocrinologist but this was six months ago and the lumps are getting more prevalent and painful!

  6. Its genetic hyperhomocysteinemia in CBS “partially deficient” heterozygotes, triggered by thyroid dysfunction, probably causing Sheehan syndrome as well. You need to treat the thyroid disorder, correct the hyperhomocysteinemia, and maybe HGH to correct any other pituitary issues.

    • You speak as if you know something none of us do. Are you a doctor? Are you a patient that has been successfully treated?

    • Chris A, I have Dercum’s disease n seems to have some of the high sounding condtions you mentioned. I have thyroid dysfunction with a low TSH, 3 thyroid nodules. I also suffered from severe postpartum hemorrhage after delivery of my daughter causing a partial Sheehan syndrome. My prolactin has been high at times. At one time, my aldosterone from adrenal gland is also high.

      You seems pretty knowledeble (even more than my doctors) Would like to hear more comments on you.

      How can they diagnose about the hyperchronatinemia n will there be any treatment to correct this?

  7. all the stories are all so similar to mine, the pain, the humiliation, doctors thinking your making things up, friends and family turning against you and wondering why you just don’t exercise or eat less, or do this or that. it’s not even easy finding the right meds or therapy, and every doctor you meet has no idea what dercums disease is and cares little about finding out. one of my doctors had the nerve to say in my chart that I had munchausen’s syndrome because I was a nurse and could easily make up illneses. do you know how that effects your credibility and makes you fee? of course you do. the pain is bad enough but the way friends, family, and medical staff treat you is heart wrenching!!!!!

  8. Many of the stories from people with Dercums could be my story, too. I remember having pain in my bones from my earliest childhood – and when I asked my dad about it, he just always said it was “growing pains” (but they never stopped, they only got worse over time) & as a teen I had eating disorders and was overactive – working hard labor on the farm, an intense athlete, dancer, swimmer, gymnast, etc. – all my life I did so much as far as physical activity and many of the sports I played in high school I did just to be thin. I gained a lot of weight with my 2nd pregnancy, in my 20’s & worked hard to lose it, then gained it again really fast and I’d be up or down 30 pounds a month – depending – til I had doubled my weight at many points. I was in nearly unbearable pain by the time I was 30 – when I went to the Dr in horrible pain from a migraine. Two doctors, & 2 rheumatologists diagnosed me with fibromyalgia & said to exercise more, which I did, as the tumors got worse & I got in worse pain, for 10 years, til they couldnt ignore the many tumors growing & finally a dermatologist & one of the female Drs said it was Dercums, which of course no one had heard of – & at that point the family Dr Id seen for like 15 years in CA dropped me, saying he wasnt able to deal with such a complicated thing – & that I needed an internal medicine specialist as a Dr. Meanwhile, ever since then I have been passed from Dr to Dr & not one has cared or tried to do anything to help. In fact at this point I havent even met the new Dr I was given, & none have even been internal specialists, tje 1st place was just a clinic owned by one – which was a whole horror story of its own. And since the last one left, Ive been left on my own basically. All they do is send me to pain management & give the least amount of meds they are comfortable giving in their ignorancd, with no changes in my rx for the last 6+ years! I had some small lumps in my teens that I noticed in my forearms, near my wrist, but later they kept getting worse, spreading to my trunk, upper arms, legs, on organs, and now my thighs have so many that are growing so big – it is painful all the time – but if I am touched – it’s a shocking pain. It is hard to believe! I am literally falling apart – with torn knees, torn shoulders, severe arthritis & dental issues (which I’ve read can also be a problem w Dercums – dry mouth – tho my Dentist thinks its from the meds, that’s another HUGE nightmare!). All sorts of other stuff besides the Dercums, all sit on the back burner as this has overtaken my ability to be active or do anything normal, much less things I love to do or even the ability to feel any joy. Idk if it’s from the disease, the meds or the combo, but I havent felt any kind of joy since it got really bad these last years, tho I should have, as I do remember what it felt like. It’s hard to have hope. Before this I was in school to be a Dr. I was designing a healing center and a better healthcare delivery system, with a 501(C)3 nonprofit I founded & I was working with another friend who also had a nonprofit. After he died, I was by that time so sick I have since let it all sort of go (but now I really need this place!) I can barely stand the pain, even with meds Im on. I can’t stand being on meds & going through torture every month when my insurance doesnt want to pay or there is some other problem in their system so I have to go days or weeks without my meds & so much stress that I can never get to a point of any normalcy. Who wants to have to go to the Dr this much – go through this much stuff – just to get meds that barely scratch the surface of the pain? It is so horrible, I dread every day, rather than looking forward to it, like I used to – before this got so bad. I wish more people could understand how really bad it is so that something more would be done. It’s a horrible thing to have to live with. It takes everything I have to keep going, as I am so tired…it’s so sad knowing its been around so long but since it is rare there is likely not going to be a cure or any good treatments discovered in my lifetime. I dont even get enough meds to be out of pain enough to be able to go out to dinner, – or even have an appetite – and that is the worst! To not eat much cuz you feel so sick, yet still be gaining weight! It’s hard to believe it could happen! It really feels like a nighttmare I havent woken from, tho I can rarely sleep from the pain, which only makes things even worse. I’ve had the worst care, even though I went to Stanford and UCSF pain clinics & specialists – no one cares – no one can imagine how bad it is – & I am not good at complaining, besides to my bf (who is really just like a friend by now as things are so bad!) I wish I could figure out a way to live with this better as I just dont have much hope left. Im running on empty batteries. I need to be recharged. I have had drugs that helped me but it’s difficult to get the right smount of the correct combinations & expensive, possibly w bad side effects, & thats all a band aid anyeay as it grows worse & worse. It needs to be fixed somehow. I do know the care I get is not even close to being adequate for the amount of suffering involved. Not sure how to change that. Every specialist I went to passed me along to the next. Or I didnt “look” sick, I didnt have “watermelon-sized” tumors – which one specialist in SF said that’s all he works on, as if I was just being a whiny baby with my lemon sized tumors…I bawled my head off after that appt as I’d waited many months to get in with hoped he would do SOMETHING to help me! It is humiliating & no one understands it who doesnt have it. I miss my normal self, my old life. I know it will never be that way again – no more carefree,
    no handsprings – or even cartwheels. I would do anything to be off the meds, if I could take that much pain, but even on meds I can hardly tolerate it – and I am tough – growing up working hard labor on my dad’s farms, riding horses & motorcycles, I always had a high tolerance for pain & didn’t take medications, believed in homeopathy & eating right. Well, I got things twisted up on me for sure! It is hard to imagine what really could be done to even really help things at this point, its gotten so bad. I know lymphatic massage is good & warm water myofacial pain release, if u can find it, but getting there became too difficult anyway. I wish I lived in the healing center I designed, so that I would have the right food, the right care – all that would be needed to make life more tolerable – hopefully – enjoyable, again. It seems like just when my mind overcame the emotional problems I had stemming from childhood & my parents/family, then my physical body went haywire. I peaked at 30. Its been downhill fast these last 18 years. I raised 2 daughters, alone, w this illness, and they both graduated college. They are beautiful & smart, but also lacking having had a father or even a father figure, they lack that confidence that daddys girls have. Eldest got her degree in cell & molecular biology, minor in Spanish, & is working as a nanny, in lots of debt (due to lack of confidence – which I feel is because I was too ill to be supportive enough to make up for no father.) The youngest has a BA degree in Kinesiology (after coming with me to a physical therapy appt once) & getting in to grad school is making her scared. At 1st they both thought they’d help me, I think, but later I think its hard to deal with when you dont understand how much pain someone is in. I am still strong because I was stronger than most men I knew & that hasnt all went away, in my mind I am still strong and thin. But in reality I just keep breaking myself more and causing more injuries every time I try to do anything that takes strength! Anyway, I never make comments but I guess I had to get it off my chest. As I am really losing my hope, & there’s not much further down I could be…

    • It felt good reading your letter. Sounds like you did a great job with you kids. Pretty fancy educational leaps. I just started having these lumps about 3+ years ago, and am wondering if the pain all of you are talking about came on at once with the first signs, or was gradual. I have no real pain of yet (aside from a sudden unset of some nasty arthritis), and am wondering if I should begin seeking solution for that now. What do you think? Glad I got to read your spot. Good to feel like I was getting the nonsense my VA docs give me, off my chest too. ha. Thank you for your post. God Bless and good luck.

  9. Why no reply for anyone that can help me in California young active but hurt so bad don’t want all the drugs just an option or at least a try from a doc

    • found a lot of information, people and other helps (info) on pinterest. Try it out. Hope you find someone in your area. I am a little unsure I want to. 🙂

  10. Hi Cynthia, I know your story, unbelievable.
    Laat je iets horen!

  11. Mike butney says:

    I had 291 lipomas and angios removed so far. I have about 225 still in me.. It sucks. I am male, 52 and I live on meds just like most.

  12. Hi we are a newly registered charity fighting to find researchers in dercums and to make doctors more aware of dercums disease! Here is our web site if anyone has any info that could help or you just want to talk to others or look your more than welcome please join for free so we can show how many people are out there that needs help.
    http://www.ddrdfoundation.org

  13. Cynthia Prentice says:

    I have had a life long battle with Adiposis Dolorosa or Dercum’s Disease as it is commonly called. Aged eight, I was a skinny little girl when pain began to eat away at me, by age ten I had pads of fat on my inner thighs which were incoherent with my own body shape. From age 16 onwards I have had a constant battle with trying to keep weight off by doing excessive sports and gymnastics, which I did not realise at that time, is precisely what you should not do. This disease appears to be a fault in the venous and lymphatic systems which are not efficient at flushing out toxins and the resulting excess lyphatic fluid feeds that fat even more, causing massive weight gain. From the age of thirty the pain was becoming rapidly unbearable with the many doctors visited telling me there ws no such thing as a disease that “hurts everywhere all the time”. By thirty-five I was covered in not only lipomas attached deep to connective tissue but angiolipomas with their own blood and nerve supply therefore causing even more pain. I suffered in silence because it is demeaning to be told hundreds of times “it is all in your head”! But when computers became accessible, it became easier to research medical articles and find the cause. Of course, the doctor was not happy to see the print out of a tentative diagnosis but had to agree that I might be right. But, no speciality covers this disease. No doctors want to take on something they can do nothing about. Not until Dr. Karen Herbst found us on the Lipoma Forum and decided to do her first study. Since then, I came from England to see her and to listen to her advice on how to look after my body and although the pain is still there, the lipomas and angiolipomas become more and more painful, I live with the hope that a cure might be found in my life-time. I hope that I have not passed this disease on to my children, but now aged sixty, I live with this disease in pain daily and handicapped severely for any activity at all. It hurts to pick up my grandchildren, it hurts to have a kitten on my knees…… it hurts when doctors dismiss you as a drug seeker or a fat, lazy, post-menopausal woman who chooses to live as a handicapped person in daily pain. I would not wish on anybody the last two operations I have had to remove nearly fourteen pounds of diseased tissue in order to restore the use of my arms so that I can at least use them. It hurts to be an ex-athlete and not to be able to walk on the beach with my grandchildren. It hurts – all the time.

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