Last Hope Saloon; Let's band together and fight

That feeling you get when the doctor's door closes in your face...

Apology

Firstly please let me start by apologising for the delay in writing this post, unfortunately I have yet again been battling an acute flare up of Melkersson Rosenthal Syndrome and as such have been completely unable to write anything remotely coherent.

I also yet again had to miss out on something that was very important to me; a good friend had a surprise baby shower and my very inconvenient flare up made sure that I could not attend! MRS is forever getting in the way of my attempts to live my life :-( 

I am now going to attempt a post, that I have had no choice but to start writing fresh, as I had almost finished it when the computer decided to delete the whole thing! Wonderful! It really isn't my week at all! lol

Doctors Visit - Help?

Painfully swollen lips and face

So... I did what any other self respecting British Citizen would do when they are very poorly, suffering and need help; I went to see my General Practitioner (family doctor) and asked for his expertise.

It would seem that this particular doctor, like most, had no expertise at all when it came to my condition. He advised that after my last visit, when he prescribed Prednisone Steroid tablets to take on top of my Hydrocortisone Steroid tablets, the practice had received a letter advising them against its use, with regards to me, from Dr Vyas (my chest physician who co-ordinates my care due to the fact there is NO specialist in MRS).

He told me that they had lost this letter, which let me tell you; does not inspire confidence! However I gave him the benefit of the doubt and looked at him across his desk hoping he had something up his sleeve or would contact someone for advice who did.

When a doctor says they can't help you; that leaves you feeling very alone

It turns out that I should learn a very important lesson as a patient with a rare disease; don't hope! He threw his hands up in the air rather dramatically and said that he didn't know what to do, he could think of nothing to help me and he didn't think anyone else could either.

So his plan... in all his great Doctor's wisdom he had no plan whatsoever, in fact he decided the best course of action was simply to do nothing and send me home in the exact same state of disrepair as when I hobbled into his office!

New symptom

I had also advised the GP that I had a new and disconcerting symptom, or believed that I did anyway, and asked him to see what he thought. Every time I swelled with my flare up my nose would feel blocked, like it was swollen inside.

The GP did actually use his torch and have a look, he advised that yes, I am correct, the inside of my nostrils is swollen and the tissue looks the same as my swollen mouth.

The pain my illness causes can be excruciating, the swelling unsightly, I am forever getting new symptoms but less help

Again he took no action with regards to this matter, not even informing Dr Vyas of my probable new symptom.

Now I will explain why this particular situation is far from ideal for me, I have Sleep Apnea and Type 2 Respiratory Failure due to which I use an NIV machine overnight to keep my airways open and to circulate the Carbon Dioxide correctly, as my body fails at both these simple tasks.

How the nasal mask is worn

(I think you can probably tell by looking at the above image that using an NIV machine is no fun and infringes on a good nights sleep as it is due to the uncomfortable nature of being attached to a breathing machine all night. Add to that the fact that I wear a nasal mask and I am certain you can appreciate the difficulties I face when my nose is blocked and/or swollen.)

The mask fits around my whole head, it is tightly fitted and constantly blows air into my airways

When you retain Carbon Dioxide it becomes a poison and if it builds up too much is deadly, I stop breathing again and again during the night as my airways close so using my NIV machine is vital. 

Wearing a nasal mask with a blocked/swollen nose feels like you are suffocating

However it is a nasal mask and as such I am unable to breathe as the inside of my nose is now swollen much like when you have a cold. So now it isn't just the pain that is keeping me awake all night, now I can't breathe either. I have not slept for more than a few hours a night for over two weeks now and that is making me feel ill in itself.

What the NIV machine looks like, it is also humidified meaning it contains water, which also blows into my airways to keep them moist

Medical History

When I told him my medical history, which he asked for rather than read through my mountain of medical notes it went like this:

• Swelling of my lips, face, eyelids, neck, epiglottis, larynx, thighs, legs, ankles, feet, arms, hands, fingers
• Swollen Subcutaneous Nodules in my stomach and back
• Swollen glands all over my body
• Extremely swollen parotid glands (saliva glands) causing me to have very little saliva in my mouth
• Swollen gums
• Mouth sores and ulcerations
• Split skin on my lips due to the swelling (often gets infected as low immune system)
• Pain all over my body due to MRS and the swelling pushing on my nerves
• Breathing difficulties because my airways swell
• Paralysed Right Hemi Diaphragm
• Sleep Apnea and Type 2 Respiratory Failure
• Chronic Asthma
• All of the above issues combined causes severe breathing difficulties
• Insufficient adrenal gland function, meaning I don't produce steroids or hormones anymore myself; I wear hormone patches and take a controlled dose of Hydrocortisone steroids daily
• Type 2 Steroid Induced Diabetes (Insulin Controlled)
• Peripheral Neuropathy (damage to the nerves outside the Central Nervous System) I get shooting and stabbing pains as well as numbness in my extremities
• Osteoarthritis (causes painful and stiff joints)
• Osteopenia (lower than normal bone density, basically thinning of my bones)
• Due to all of this is it surprising that the last thing on my list of ailments is; Clinical Depression? Well yes, I have that too and am on medication for it, 40mg of Citalopram every day.

So when the doctor looked at me with a blank expression on his face and ushered me out of his office, I think I almost heard his sigh of relief as the door closed behind me. 

I was left to go home like this; no treatment, no help, just physical pain and emotional despair

My double appointment was unnecessary as it happens, as no sooner than I had listed my medical repertoire and advised that he could add my nostrils to the growing list of my swollen body parts he told me he couldn't help with my current flare up and in panicked haste practically chased me out of the room.

In fact I am sure he called reception after I walked out of his office to make sure I had exited the building before he dared leave the room to call his next patient through. (You may laugh but I'm almost serious)

Utter despair

I left with Amy, got in our car, we drove over to the chemist across the road so that she could pick up my prescription of Insulin and the like. I couldn't bottle it up any longer; I cried, no I sobbed, really sobbed. I don't do that often but when I can take no more, when I am at my absolute limit the dam bursts and I can't control it any longer.

I can't begin to describe the feeling of utter despair that you get in the pit of your stomach and the panic that grips your chest when a doctor tells you they don't know how to help you, that no other doctor would be able to help and that what's worse; he wouldn't even attempt to try.

It made me feel lonely and isolated because there was no one left on the battlefield but me and my enemy; MRS. I was overwhelmed by the fact that MRS had all the weapons and artillery and I had none. 

I was facing my greatest enemy alone and unarmed without any backup at all. That is a truly frightening place to be, when a doctor tells you they don't know what to do about your illness your stomach and chest tighten, you feel sick and dive straight into the panic attack of all panic attacks. 

Your heart races and your stomach clenches, there is no help coming... You are alone on this dark, frightening battlefield and the enemy is already upon you.

So... I cried, a LOT, I was hurt and angry that he didn't even humour me, he made no attempt to get advice or to tell me that this wasn't the end of the road, that there was some hope, that he would email or write to Dr Vyas, for example, and ask what to do. He could have picked up his phone and made inquiries but instead it remained snug in its cradle, undisturbed.

For once I would love a doctor to say that they understand

Of course I have come across this situation before, I dare say every patient with a rare disease has had the same problem, they too will have taken a seat opposite a doctor who knew less about their illness than they did. This probably happens on most occasions and like now on some of those occasions the doctor would have been less than helpful.

It doesn't matter how many times this happens to you, each time it leaves you feeling crushed and alone.

Believe what you read on the Internet?

I want to cover this next topic because I have come across this issue many times as a person with a rare disease and I know many if not all of my fellow MRS sufferers have the same problem; Google-Dependent Doctors.

Hand swelling is yet another symptom I get time and time again that is not stipulated as an MRS symptom when a google search is undertaken

It seems to have become common practice for doctors and nurses who are unaware of my condition to use google as their research tool, not only that but they tell me that's what they have done too. Then they expect me to trust their methods of care?! What?! Seriously?! Did you get your medical degree by collecting Cereal packet coupons?!

This does not give you reason to trust their treatment decisions at all, I understand that doctors and nurses will not have heard of ALL illnesses however surely they could use a more reliable research tool than google, even asking me or another doctor who has already had experience dealing with me would be preferable.

The information that a google search throws up is at best outdated, it is also incorrect and under-informed making it dangerous as doctors who rely heavily on the information they glean from it could treat MRS incorrectly and not expect some of the dangers that they face treating a patient with it.

The top two search results give the following description of MRS:

Melkersson–Rosenthal syndrome (also termed "Miescher-Melkersson-Rosenthal syndrome"), is a rare neurological disorder characterized by recurring facial paralysis, swelling of the face and lips (usually the upper lip), and the development of folds and furrows in the tongue.

(Wikipedia)

"Melkersson-Rosenthal syndrome is a rare neurological disorder characterised by recurring facial paralysis or palsy, swelling of the face and lips (usually the upper lip), and the development of folds and furrows in the tongue."

(Facial Palsy UK)

There is so much more to MRS than the above descriptions of the illness and I have come across doctors and nurses who refuse to listen when I tell them that. They look at me like I am some crazed lunatic escaped from the nearest mental asylum when I tell them my other symptoms that don't neatly fit within the brackets set by a quick google search.

When I try to explain the pain I am in they appear to believe I am dependent on drugs and have a very vivid imagination, which I utilise in my efforts to feed my habit on the NHS budget.

The pain I suffer when I have an MRS flare up is very real and undeniable

I know for a fact that I am not the only MRS patient with this problem, as since Amy found the MRS Facebook support group I have read stories very similar to mine. People with the exact same problems that deviate from the google search descriptions of MRS.

We not only have this awful rare disease to battle but the small minds that refuse to expand further than a quick Internet search too.

I have been given permission to post the following two images of fellow MRS sufferers so that you can see other people with my illness.

Swollen right eyelid

Another lady with a very swollen eyelid, causing her eye to almost close involuntarily

The ladies in the above images suffer greatly just like I do, there are now 149 worldwide members of the Melkersson Rosenthal support group all of whom either have MRS themselves or know someone who does. That amounts to a lot of other people suffering along side me.

The lady in the first picture also suffers with another rare condition called Ramsay-Hunt Syndrome, if you would like to know more about her and the illnesses she struggles with you can visit her GoFundMe page, where you will also be able to make a donation, towards her ongoing medical bills etc, should you wish to.

I also have a GoFundMe page set up, which is there in an attempt to raise the funds for me to undergo a second round of Stem Cell Replacement Therapy so that I can solidify the results I have and gain new ones and of course to stop me backtracking and ending up pretty much bed-bound again. 

There is also another lady I know via the support group, whom I have been talking to for some time now, she is a lovely person with two young children who desperately need their mummy to get a bit better so she wont miss any milestones again like her son's first day of nursery due to awful flare ups of MRS. She is trying to raise funds for Stem Cell Replacement Therapy the same as what I had via GoFundMe and is almost at target, she just needs that final push.

Any help for one or all of us would be greatly appreciated and could very well prove life saving but will most definitely be life changing, so thank you to those who have donated and anyone who will donate in the future.

Stronger Together - Let's make a change

I am fed up of there not being correct, up-to-date, readily available information out there about MRS and what we have to deal with on a daily basis. It is something that needs to change so that we receive treatment based on what our condition is actually capable of doing to us. 

We need to be better understood and we need help but in order for us to be heard we need to make noise. We are so much louder when we all shout out together.

It would be great if any fellow MRS sufferers who read this could add a list of their symptoms to the comments section at the bottom of this post.  If you have any stories about your brushes with knowledgeable doctors and nurses etc please feel free to share. I want to try and get the information readily available on the Internet updated correctly and to do that sufficiently I need your help. Please post anything you feel comfortable with and anything you'd rather not post but would like me to know you can email to; chrissyjade18@icloud.com Please also feel free to email me any pictures that you wouldn't mind included in the blog so that others can see a collection of people with MRS and the affect that it has on us. I think it is vital to our ongoing care that we do something NOW to change the information out there, to get someone to sit up and take notice of us and our suffering. It is of paramount importance that someone medically trained to research MRS and find new and effective ways to treat it. We need a cure or at least an effective and available treatment. The only way I see this ever happening is if we collectively speak out and make ourselves heard! Make it so we cannot be ignored, not just MRS sufferers but all people with all rare diseases. We are by fate in minority groups, we are spread world-wide making us appear even more alone in our fight, as would have to cross oceans to put more than a handful of us in a room together. But our voices will be much louder if we all shout in unison; we need to demand help now not only for ourselves but for the children that will develop MRS and the people not yet born who will have to carry this dreadful burden too. Rare diseases are very lonely illnesses; there isn't an aunt, a cousin, a brother, a sister, a close family friend etc who has it too, who you can compare notes with, who can be an understanding shoulder to cry on when it gets too much.  We have our friends and family of course but having someone who knows exactly what you are going through without you having to breathe a word is not something most people with a rare illness have. In fact we pretty much face it all alone, without much medical help or expertise. You can cry for what you don't have or you can appreciate what you do have and fight for what you could have and if we undertake that fight together then I am certain we can and will achieve something wonderful.

It would be great if any fellow MRS sufferers who read this could add a list of their symptoms to the comments section at the bottom of this post. 

If you have any stories about your brushes with knowledgeable doctors and nurses etc please feel free to share. I want to try and get the information readily available on the Internet updated correctly and to do that sufficiently I need your help.

Please post anything you feel comfortable with and anything you'd rather not post but would like me to know you can email to; chrissyjade18@icloud.com

Please also feel free to email me any pictures that you wouldn't mind included in the blog so that others can see a collection of people with MRS and the affect that it has on us.

I think it is vital to our ongoing care that we do something NOW to change the information out there, to get someone to sit up and take notice of us and our suffering. It is of paramount importance that someone medically trained to research MRS and find new and effective ways to treat it. We need a cure or at least an effective and available treatment.

The only way I see this ever happening is if we collectively speak out and make ourselves heard! Make it so we cannot be ignored, not just MRS sufferers but all people with all rare diseases. We are by fate in minority groups, we are spread world-wide making us appear even more alone in our fight, as would have to cross oceans to put more than a handful of us in a room together.

But our voices will be much louder if we all shout in unison; we need to demand help now not only for ourselves but for the children that will develop MRS and the people not yet born who will have to carry this dreadful burden too.

Rare diseases are very lonely illnesses; there isn't an aunt, a cousin, a brother, a sister, a close family friend etc who has it too, who you can compare notes with, who can be an understanding shoulder to cry on when it gets too much. 

We have our friends and family of course but having someone who knows exactly what you are going through without you having to breathe a word is not something most people with a rare illness have. In fact we pretty much face it all alone, without much medical help or expertise.

You can cry for what you don't have or you can appreciate what you do have and fight for what you could have and if we undertake that fight together then I am certain we can and will achieve something wonderful.