I think it's taken this long for me to process all that I learned and all the feels that came with this new knowledge. I have been feeling terrible about not getting back to you all sooner since so many of you supported me in getting there. Please know I felt so very loved that you would help me on this journey.
So here's my story...
My trip to 'OZ' ... I'm off to see the Wizard?
Myself and Dr. Karen Herbst
First, let me say I always knew this experience would be unique, meeting a doctor that knows all about my rare disease. In addition Dr Herbst is one of the most compassionate and friendly doctors I have ever had the pleasure to consult with. I instantly felt comfortable and knew It was most definitely worth the trip.
So what did Dr Herbst have to say about this odd bod?
A LOT! There is a lot to get through so I'm going to lay it out briefly with helpful links if you want to learn more about the conditions mentioned.
DISCLAIMER - To all my fellow DD and Lippy peeps who may be reading this. Below is a set of recommendations prescribed specifically for me by Dr Herbst. As we all are aware, as similar as we are, we are also very different. There are many factors not talked about here which maybe very different for you (including but not limited too, blood test results, other medications I may be taking etc.) So please do not take anything talked about here as a diagnosis, possible prognosis or the right course of treatment for yourself.
Dercum's Disease - Confirmed that I do indeed have Dercum's Disease. Not that this was in query but it's always good to have one of the world experts back up your diagnosis. Especially when there are still doctors out there that don't believe it is a real thing or possible for lipoma to hurt.
Lipedema Stage 2-3 of legs - This is a condition that I have suspected I have for quite sometime but was unable to get a diagnoses in NZ thus far.
Also Possible Mast Cell Activation Disorder - This rare condition would account for a lot of my allergy like symptoms. I will need to be diagnosed by a specialist in this field.
A little more information on these diagnoses
Rather than go into detail about DD here is a couple of links to Video's in which Dr Herbst talks about Dercum's Disease and a short white paper that outlines Dercum's Disease.
- Please follow the link and scroll down to the video titled Dercum's Disease Overview.
- In this video Dr Herbst answers Questions and talks about treatment.
- The White Paper.
Again rather than go into details please follow the links below to learn more.
- Dr Herbst's description of Lipedema on her site.
- Preview for a documentary Film by Catherine Seo about lipedema: You Mean It's Not My Fault: Lipoedema, a Fat Disorder
- There are also many longer videos where Dr Herbst talks in depth about Lipedema and all RADs (Rare Adipose Disorders) on youtube.
1. Evaluate for Mast Cell Activation Disorder. While I can look at a list of the symptoms of mast cell activation disorder and tick of most of them, it shares many symptoms with Dercum's Disease. It's really important that I find out if I have it as there are known treatment options and it also may in pact on how other medical procedures are carried out for me.
2. PTEN mutation genetic testing. A test to see if I carry the genetic markers for Lipomatosis as I was born with a lipoma. It seems there is some connection between Lipomatosis and full blown Dercum's disease, my understanding is that this is not a hard fact but a line of inquiry still. Whether or not I have this mutation would be important if I was wanting to have children of my own. As Dercum's appears to be autosomal dominant (though again this is not confirmed and they have not identified the genetic marker) I have made the decision that I will not be having my own biological children. This genetic evaluation however maybe helpful for family members should (and touch wood it never does) one of them starts to develop lipoma. It also could be helpful from a research perspective.
3. Liposuction Surgery. Dr Herbst was very clear that given the level to which I have Lipedema combined with my Dercum's diagnosis that should I require future surgery there is a very high chance that it will spark full blown Lymphadema that will be hard to control. I already know I will most likely require a hysterectomy thanks to the way my endometriosis presents. One way to potentially avoid this is Liposuction. In addition Liposuction is the only way to debulk Lipedema limbs/body areas as it is impossible to loose lipedema fat through exercise and diet. There are two types of Liposuction that have been successfully used in Lipedema by experienced doctors, water jet assisted and tumescent technique. With luck It will all so help save me knees from needing to be replaced and increase my mobility.
4. A whole lot of Supplements. Dr Herbst has 'prescribed' me a list of supplements to try. 'Supplements' is probably a bit of a misnomer as none of them are really supplementing anything. For those of you that feel deep concern when they see the word supplement, please know that there is some solid science behind each recomendation. Also when you have something rare (as in the case of DD) or something not well studied and under diagnosed (like lipedema) most treatments recommend are going to be on the edge of medical knowledge and/or being used 'off script'. They include: N-acetyl-cycstine (NAC), L-arginine, Diosmin, Butchers Broom (Ruscus Aculeatus), Yohimbine and Selenium (ok Selenium is a supplement ;) ). Also I'm to consider taking a digestive enzymes. As the months go by I will be trying these one at a time so I will go into each in more detail as I try them.
5. Whole Body Vibration (WBV). There is a lymphatic component to both DD and Lipedema. WBV has been shown to be "A unique means of increasing muscle, decreasing fat and improving lymph flow."
6. Get and use a Lymph Pump. A lymphatic pumps are sequential compresion devices that are used to move lymphatic fluid through a body. They are normally used for those with full blown lymphadema, But have shown to benefit those with DD and Lipedema. They come in many shapes and sizes from one limb to most of the body. For me I will need one that does my whole body (most likely in sections). It's a little hard to imagine so take a look at the Lympha Press website to get a better visual.
7. Compression Garments. Dr Herbst took at look at the compression I have been prescribed and suggested I move to two pieces, a long sleeved vest and full stocking up to under bust. And if possible in a lighter weight material.
8. Swimming. Dr Herbst felt the best exercise for me would be swimming. I love being in the pool so this pleases me. On the other hand with Endo, IBS type symptoms, and dystonia (outside of the fatigue) I'm not sure how viable this is as my regular exercise. But maybe once the other things are in place they will have a positive impact on the things that keep me out of the pool.
Where to from here?
Over the year ahead I will be attempting to undertake the recommendations with the hope that they have a positive effect on my health. It's important to keep in mind that though there are a lot of possible positive outcomes that can come from these recommendations that how my body reacts to them may not be inline with the desired outcome. For example, as Dr Herbst expressed, the supplements can be very effective but as I am someone who reacts to medications/supplements in odd ways I may not tolerate them well or at all. The other complication is the cost of each treatment. At the moment it looks as if many of these treatments are not available through my DHB nor can be paid for through WINZ.
It's also important to keep in mind that none of these treatments is a cure. With luck they will have the effect of upping my quality of life for now and into the future.
I will be documenting my journey here in the blog. It feels important to share how I'm finding this journey. In part because I think it will keep me focused. And perhaps it will also promote awareness of DD and Lipedema and give some insight into what we (myself and my fellow chronically sick peeps) go through.
But what about the Conference?
As many of you know there was also a patient conference. It was amazing to be around a group of lovely friendly people who happen to have the same diseases as myself. Who knew exactly what I was going through on many levels. The talks were very informative and I hope to post links to them as soon as they are published. There is some exciting research now being done in this area and with luck, money and effort it will hopefully yield some great results and possibly effective treatments!. So watch this space...
Well that's it for the moment.
Thank you all again for your generosity and compassion in helping me get to Australia to meet with Dr Herbst. It really has been life changing.