Headache

[Janice]

Hi I was wondering if any of you who have neuropathic deposits would describe your symptoms as a severe constant debilitating headache? My husband has not been diagnosed yet but along with unexplained profuse uncontrolled diarrhoea this would be his biggest symptom. Testing soon.

[Mhelenx]

Hi Janice, sorry to hear about the severe symptoms your husband is currently experiencing. Like your husband, I also get daily severe, constant debilitating headaches, which is one of the many symptoms I experience. I was diagnosed in 2005 with localised AL Amyloidosis Paranasal Sinuses. After several Endoscopic sinus surgeries, regular monitoring by my local hospitals and NAC, my symptoms continues to worsen especially the headaches. I don't think I have neuropathic deposit as my Neurologist said that it is possible I am having Vascular headaches caused by chronic inflammation and infection. I was prescribed Topiramate 25mg which is an anti-seizure drug but had to discontinue it after a week because of severe side effect.

On a typical day, I wake up with a dull headache and can do my home and office work until just after midday. I know the headache will worsen when I start yawning persistently. It feels like I am struggling to get oxygen when in a smokey atmosphere. I start getting toothache, pains in my jaw, then throbbing pain in my face, ears, behind my eyes and forehead. Within minutes of these symptoms starting, I start experiencing throbbing headache, top of my head in the fontanelle region. I get 25 seconds of throbbing in a minute then it dies off and starts again, minute by minute. It feels like my heart is beating in my head. My entire head becomes sensitive to touch. The nausea and vomiting then starts with stiff neck, sensitivity to light and sound, throbbing in my neck and swelling around my eyes. My only option is to go to bed in a dark, quiet room and lay very still, unable to read, listening to music or speak. I can only tolerate sips of water until the symptoms abates. These symptoms can persist for between 8 to 12 hours. I fall asleep for about a couple of hours at a time and take a long time to go back to sleep. On a few occasions, the symptoms lasted  for 2 days constantly with very little to eat or drink. Unless someone experiences these types of headaches it is very difficult to describe. It feels like the worst toothache or stubbing your big toe in a constant loop. My energy is sapped after each episode and my brain feels exhausted.

I have been trying desperately to get a grip with my symptoms. I do not tolerate analgesia well as I have GERD and IBS so I can only take Paracetamol or Paramax. If I take pain relief before the headaches starts in the morning, sometimes it works and I can manage the headaches and other symptoms without having to go to bed. I also find the soluble tablets work more effectively. Other times I can as many tablets as I can and nothing works. I also use an ice pack on my head, it doesn't really ease the symptom but it cools the 'hot head' feeling. I cannot plan a day knowing that once the headache and symptoms starts, my day has ended. Maybe your husband can describe his symptoms in more details so I can understand a bit more what he is experiencing. I hope this insight has helped.

[Janice]

Hi Helen
(Janice's Husband Colum writing)

Firstly thanks for such a detailed reply i really appreciate it.

I started the whole headache thing in my late twenties - i am fifty now - i had many sinus surgeries up to and including 5 years ago when i had a complete frontal sinus drill out. And then several month later adhesion decision. In parallel to this was gradually worsening bowel issues. I'll come back to this.

After seeing and been referred both privately and then on NHS i am now under the care of Prof Gadsby and his team at Kings. They are great. I have tried all the standard meds like you including Gabapentin, Pregabalin, Topirimate etc. You name it i was on it. Some of it helped the headache but i steadily started loosing my faculities due to the medication. My headache is in many ways just like yours - quite simply its throbbing and stabbing constantly on the move side to side, front to back, top, behind the eyes, frontal forehead. It can be minutes or seconds before it moves or changes in intensity.  Currently i rarely get any reasonable days. Reasonable is around 6/10 mostly its 8/9 out of 10 and i cannot function normally.

My mum is currently having chemo having just been diagnosed with some AL variant yet to be formally diagnosed she is 73 and her AL is mainly in her heart which is in a pretty bad way. She has being treated for heart disease which she didn't have (not in the normal sense) for the last few years. Her AL is pretty advanced and it will be weeks before the benefit if any is known. The higher risk for her is treatment mortality.

Prof Goadsby's team have had little success in getting my condition to respond - even to some curating edge stuff - like a vagal nerve stimulator which has to date only moderately had any impact. I too find tablets ineffective so i also go for soluble. I take cocodamol and try to regulate this as i understand the view (although not held by all neurologists) of rebound headaches. Otherwise the treatment for the headache is much the same as yours. 

Recently the Professor's team (whom i cannot praise enough) referred me to a gastroenterologist Prof Epstein who immediately sorted out a sequence of scans and tests and put me on liquid Imodium to try to control what in essence very bad IBS. Scans of the Lower and then Upper bowel - we're not really revealing other than to note some diverticula although not severe. At the second appointment he asked me to try to some colestipol hydrochloride T/A Colestid which is a binding agent for Bile Acid which he felt could be getting into the lower bowel causing my issues. His conclusion (not by testing) was BAM Bile Acid Malabsorption. Between this and the Imodium things are a little better but i still get the chronic stomach pain on attacks sometimes with or without the bowel issues.

I get night sweats / day sweats during head even though it might be cold to touch or sometimes i do have a temperature. I can be just sitting or lying down and for no reason will feel really hot and uncomfortable. Currently i am taking antibiotics for a sinus infection.

The Professor (Goadsby) isn't happy that we really know whats going on and he wants to rule Amyloidosis out before he tries to resume treatment. The guys at the NAC are reticent about the highly unlikely scenario of AL (not hereditary) being present in both my mum and me. It may well be that they are right but something isn't right and i have to live in my own body day to day despite statistics. The current plan is that i will have the tests next week at the NAC which i want to pursue.

I don't sleep much - and certainly not any 'good sleep' and usually woken by headache or sometimes stomach pain and the mad dash to the toilet.

The headache despite best efforts the headache has been getting worse over time and i keep regular diaries and produce stats for the professors team and the trend is continually upward pain-wise. I have not worked for over 1 1/2 years simply over time the headache the bowel issues made travel impossible and decision making even more problematic and i ceased to be able to function at the level i was. I don't see any immediate change on fortune on the horizon so i need to pursue as many avenues as i have and i can even if some of them are blind ones.

Thanks very much for letting me know i just might not be as mad as a hatter - sometimes it feels like that. I'd be very interested to know how you got diagnosed if you don't might me prying?

Best
Colum

[Mhelenx]

 
 
Hi Colum
Thank you for detailing your symptoms so precisely. Since my diagnosis, I have always wanted to find someone who was experiencing similar symptoms to mine so I didn’t feel so isolated and alone, although, I wouldn’t wish my symptoms on my worst enemy.
 
I will give a brief history before I was finally diagnosed. I have a family history of sinus related problems and although I had frequent sinus infections, bronchitis and asthma, my symptoms were well managed and never suffered from headaches with an acute sense of smell.
 
In 2000, I started a new job and had an accident at work (carbon toner spillage) whilst changing the carbon toner cartridge on the photocopier. I was not wearing a mask at the time and used a brush and pan to get rid of all the carbon dust on the floor.  After the incident, I noticed I was losing my smell and thought it was due to a cold. I then started to have nose bleeds, tearing and headaches. I was referred to ENT and Ophthalmology in 2003 and after several months I had surgery, diathermy to the inner turbinates in both nose and DCR (dacrocystorhinostomy), eye operation for a new tear duct to left eye. The headaches and infections got more persistent and after convincing my medical team that I can no longer cope with the symptoms, I was sent for a CT scan which showed complete blockage of the sinuses. In 2005, I had FESS to my left nose and because of the severe infection I had a long course of antibiotics before surgery to the right nose. A biopsy and swabs were taken and sent for histology which came back Negative. My ENT surgeon was unhappy with the amount of spores seen during surgery so had the samples retested by the lab and the result was positive to congo-red staining, a sign of Amyloidosis. I was referred to the National Amyloidosis Centre to confirm the diagnosis of Localised AL Amyloidosis Paranasal Sinuses, diffused in nature. Since then, I have had several sinus surgeries but it resulted in heavy scarring and formation of new bone with more amyloid deposits. I also had additional surgery to have a Lester Jones (glass) tube to my left eye as a tear duct. The treatment over the last few years continues to be palliative care. After my visit to NAC this year, and consultation among many medical teams, I am due to start Chemotherapy with Velcade and Dexamethasone to help reduce the inflammatory/cellular infiltrate, although speculative, prior to further surgery. Not sure if this will work but I have to give it a go as my condition is worsening. I hope this information helps.
 
You have my consent to use my name and the details I have provided as a reference at your clinic appointment with the NAC because a lot of your symptoms are so similar to mine. From experience with so many different antibiotics, the most beneficial was Ciprofloxacin 750mg, twice a day, for 28 days. I wish you the very best and hope you get a valid outcome with some answers to help you get the correct treatment to relieve your distress. You are not alone, be persistent until someone listens. I will keep you and Janice in my thoughts and prayers. I will also update you with any further progress on my condition.
Good luck and God bless
Helen xxx
 
 
 
 
 
 

[rsskga]

I also have a history of atypical chronic sinusitis, secondary to a primary immunodeficiency and deviated septum. My amyloidosis diagnosis is still pending, but is quite likely since I'm presenting with macroglossia, slight periorbital purpura and an erratic heart rate.

I've been experiencing very strange, insoluble sinus discharge for some time. I also feel that tissue invasion may have occurred because the bones/cartilage/etc. around my sinuses have begun to feel structurally unstable.

I initially thought the discharge was due to a biofilm infection. I did benefit from intense use of macrolides, fluoroquinolones (i.e. ciprofloxacin) and fluconazole for a time. I now think there was an infection, but that amyloid might have been the underlying problem all along.