Not A Head Cold?
SS awareness bracelets
My name is Julianne. I am 64 years old and live in Devonport, a city in northern Tasmania. Tasmania is a large island state located 240 km (150 mi) south of the Australian mainland. I was diagnosed with Superficial Siderosis in September 2011, so nearly ten years! In April 2011, I went to my doctor after losing my sense of smell and having trouble with my hearing I thought was part of a heavy head cold. I was also struggling a bit as my legs felt like lead at the same stage when I tried to run, but I had no idea that the two were related!
The Specialists
My GP referred me to an ENT specialist, who sent me for an MRI scan. My MRI showed what looked like a pituitary adenoma (Pituitary adenomas are benign tumours of the pituitary gland), so I was sent first to an endocrinologist and then to a neurosurgeon. The report also mentioned a past sub-arachnoid bleed. Although concerned about the black spots on my brain, we decided to tackle one thing at a time, so I had surgery to remove the tumour on my pituitary gland in September. Turned out to be no tumour. I had Pituitary Hyperplasia. My pituitary gland was the size of a tumour (walnut). I had the part of the pituitary gland removed that was pressing on my right optical nerve and had a biopsy done, which returned a benign result.
Dr. Google Makes The Diagnosis
Before the surgery, my neurosurgeon diagnosed the black spots as SS – as had I. (I reckon like me, he had asked Dr Google as he had never seen it before.) After the pituitary gland surgery, he sent me to a neurologist who organised for me to have a CT myelogram which identified a pseudomeningocele between C1/T4. Because of its size, I was sent for a more in-depth CT myelogram a few months later to determine the actual bleed site so the surgeon knew where to operate. It was found at the T3/T4 level.
Locating The Trouble
The German professor who performed the second CT myelogram told me the dura is like a seam in a jumper. If you pull too hard in one spot, you will get a hole up further. I had spinal surgery in May 1992 – 19 years before diagnosis– at T11/12, and the pseudomeningocele was at T3/4. It was a textbook case!
I already had neurological issues from a disc crushing my spinal cord – hence my first 1992 surgery – resulting in me having Brown Sequard syndrome, which magnified my SS. I had an MRI scan in 2007 after having chronic sciaticaOverview Peripheral neuropathy sciatica is a pseudo-sciatica.... Unfortunately, the SS was not picked up at this scan; otherwise, I wouldn’t have any real issues now.
Fixing The Source
In July 2012, I had a hemilaminectomy to remove the pseudomeningocele, which had set up its own blood supply (and contained a large disintegrating old blood clot). They hoped it was the source of the bleed. I had 10 days in hospital, six of which were spent flat on my back. A drain from my spine was taking out 5ml of fluid every hour to give the graft from my leg (which was glued and stitched), used to seal off the dura, giving it a better chance of healing. My spinal fluid started off the colour of stale urine (with red stripes) and, after six days, was the colour of pale straw.
The Situation Worsens
I recovered well and was almost back to where I had been. On 21st December 2012, I was struck by closing automatic doors in a store while shopping. I fell back onto the pavement, knocked unconscious, fracturing my skull – the occipital bone on the right side.
I have never been the same since.
In 2018, as part of my legal claim, I saw a neuropsychiatrist who diagnosed me with Functional Neurological Disorder, which is like post-traumatic stress of the brain. Unfortunately, this exacerbates all my neurological symptoms. I lost more hearing on the right side, my balance and gait are much worse, and I have chronic tiredness. After eight and a half years of money, frustration, and tears, we have finally settled with the store. Still, unfortunately, no amount of money will ever make me better.
Controlling My symptoms
Since the accident in 2012, I have done everything I can – from changing my diet, regular massage and chiropractor appointments, supplements, physio, PT, Pilates, and walking indoors with my hand weights. I recently found out about and started vestibular rehabilitation. It is a long, hard road, but I am determined to be the best version of myself that I can be.
I hadn’t tried chelation therapy as my neurologist wouldn’t hear of it when I was first diagnosed, as Deferiprone was still in its trial phase. He thought I didn’t need it as I had my bleed ablated, believing there should be no more progression. I haven’t seen my neurologist since September 2016. He felt he didn’t need to see me unless I had any worsening of my symptoms. I am seeing him again in September, so I will raise the issue of the Deferiprone again.
Going Forward
Luckily, my hearing hasn’t changed since my fall. My other symptoms seem stable, although I have developed insomnia and double visionOverview Diplopia, commonly known as double vision, happens ... in my right eye in the last couple of years. Apart from these, I have a loss of hearing in both ears (I need hearing aids), gait ataxiaOverview Ataxia is considered a symptom of your superficial ..., balance issues and chronic tiredness. I am fortunate that I don’t have any pain, and I lead a reasonably normal life. I have a wonderful husband, family and friends who support me as well. Like all of us, I am a work in progress
**Just a footnote: I think follow up MRI scans should be mandatory after spinal surgery, which would avoid a lot of heartaches.
Original responce to Jullianne dated Jun 21 20121 with corrections update & additional explanations/details
Very interesting. Thanks for publishing. The Pituitary gland as you know sits just below the brain within the cerebrospinal fluid which supports and protects the brain. The pituitary gland is therefore subject to attack by corrosive iron deposits. The pituitary gland is the master gland of the Endocrine nervous system parts of the Autonomic nervous system Para and Parasympathetic nervous systems. Systems which control the bowel, digestive system, heart rate and blood pressure. My SS was claimed to be mild when confirmed in 2016 and as my bleed was stopped in 2012 I was told Ferriprox was not appropriate. However, when UCLH checked my claims of increasing neurological symptoms since 2009 in 2018/19 problems with my bowels oesophagus and anus were confirmed. So I have been taking Ferriprox since Nov 2019. In 2016 neurophysiology tests located lesions on various cranial nerves (iron deposits). In 2020 despite being very fit and active problems with my heart rate and BP occurred more and more frequently when exercising. UCLH Haematologists referred to Neoplasms of Uncertain Behaviour on my Endocrine glands & nervous system. Note the pituitary gland is the master gland of the Endocrine nervous system which is controlled by the brain via the Hypothalamus. The Endocrine glands control heart rate and blood pressure by injecting hormones into the blood supply. And as you will be aware treatment with Ferriprox requires regular blood samples and tests. The Enteric Nervous System is located in the side walls of the digestive system in the lower half of the Oesophagus and extends throughout the digestive system and bowels. The Enteric Nervous System is known as the second brain as it works independently of the brain. Unlike the Endocrine nervous system which is controlled by the Hypothalamus and Pituitary gland. The Enteric Nervous System also controls the heart by injecting hormones into the blood supply. Slight swallowing problems were confirmed by gastroenterologists in 2018. Due to muscle immotility in my oesophagus. Plus the upper and lower sphincter muscles do not open and close automatically as they should when I initiate swallowing when food passes over the rear of my tongue. Also, a normal-sized endoscopic camera cannot pass through the sphincter muscles so a paediatric camera half the diameter had to be used. Speech & Language Therapists then proved muscle immotility also affects my lips all muscles in my mouth and the complex muscles my throat. In 2019 Gastroenterologists/Neurophyisiologists confirmed the muscle immotility effect all muscles in my bowels and rectum. My brain does not receive signals to indicate when my bowels are full. So my brain cannot signal the inner anal sphincter muscle to open. Leaving me control over the only one of 6 sphincter muscles from just behind the tongue to the annus over which we have voluntary control. So in Dec 2020, I was prescribed the Anal Rectal Irrigation System Piristreen. Which I have been instructed to use every day since Dec 2020. Necessary to keep my bowels moving, avoid blockages and the need for surgery to divert waste into an external bag. Note the Vagus Nerves innervates muscles involved in swallowing, digestion and bowels and is subject to damage by iron deposits. The Endocrine nervous system starts in the sidewalls of the lower portion of the Oesophagus and extends through the digestive system and Rectum. In Aug 2019 following the Anal Rectal Neurophysiology tests I was advised investigations into my Enteric Nervous system had been authorised and a date awaited. Those investigations have not started. In Dec 2020 the Gastroenterologist/Neurophysiologist involved in the Anal Rectal Neurophysiology tests in 2019. Who prescribed the Peristreen System asked me to participate in investigations to check my bowel Microbiome and analyse what Microbiome exist so alterations to my diet could be advised and monitored. The objective being to hopefully reduce the need to use the Peristreen System EVERY day! That would be done by a test kit similar to a bowel cancer stool test kit sent to my home. The first test kit has not arrived 8 months later. The latest research concludes the Enteric Nervous System (the second brain) communicates with the brain via the Bowel Microbiome. Hence the problem of my brain not receiving signals to indicate when my bowels are full? For further information see the ZOE-Health Study. https://health-study.joinzoe.com
Original responce to Jullianne with corrections update & additional explanations/details
Very interesting. Thanks for publishing. The Pituitary gland as you know sits just below the brain within the cerebrospinal fluid which supports and protects the brain. The pituitary gland is therefore subject to attack by corrosive iron deposits. The pituitary gland is the master gland of the Endocrine nervous system parts of the Autonomic nervous system Para and Parasympathetic nervous systems. Systems which control the bowel, digestive system, heart rate and blood pressure. My SS was claimed to be mild when confirmed in 2016 and as my bleed was stopped in 2012 I was told Ferriprox was not appropriate. However, when UCLH checked my claims of increasing neurological symptoms since 2009 in 2018/19 problems with my bowels oesophagus and anus were confirmed. So I have been taking Ferriprox since Nov 2019. In 2016 neurophysiology tests located lesions on various cranial nerves (iron deposits). In 2020 despite being very fit and active problems with my heart rate and BP occurred more and more frequently when exercising. UCLH Haematologists referred to Neoplasms of Uncertain Behaviour on my Endocrine glands & nervous system. Note the pituitary gland is the master gland of the Endocrine nervous system which is controlled by the brain via the Hypothalamus. The Endocrine glands control heart rate and blood pressure by injecting hormones into the blood supply. And as you will be aware treatment with Ferriprox requires regular blood samples and tests. The Enteric Nervous System is located in the side walls of the digestive system in the lower half of the Oesophagus and extends throughout the digestive system and bowels. The Enteric Nervous System is known as the second brain as it works independently of the brain. Unlike the Endocrine nervous system which is controlled by the Hypothalamus and Pituitary gland. The Enteric Nervous System also controls the heart by injecting hormones into the blood supply. Slight swallowing problems were confirmed by gastroenterologists in 2018. Due to muscle immotility in my oesophagus. Plus the upper and lower sphincter muscles do not open and close automatically as they should when I initiate swallowing when food passes over the rear of my tongue. Also, a normal-sized endoscopic camera cannot pass through the sphincter muscles so a paediatric camera half the diameter had to be used. Speech & Language Therapists then proved muscle immotility also affects my lips all muscles in my mouth and the complex muscles my throat. In 2019 Gastroenterologists/Neurophyisiologists confirmed the muscle immotility effect all muscles in my bowels and rectum. My brain does not receive signals to indicate when my bowels are full. So my brain cannot signal the inner anal sphincter muscle to open. Leaving me control over the only one of 6 sphincter muscles from just behind the tongue to the annus over which we have voluntary control. So in Dec 2020, I was prescribed the Anal Rectal Irrigation System Piristreen. Which I have been instructed to use every day since Dec 2020. Necessary to keep my bowels moving, avoid blockages and the need for surgery to divert waste into an external bag. Note the Vagus Nerves innervates muscles involved in swallowing, digestion and bowels and is subject to damage by iron deposits. The Endocrine nervous system starts in the sidewalls of the lower portion of the Oesophagus and extends through the digestive system and Rectum. In Aug 2019 following the Anal Rectal Neurophysiology tests I was advised investigations into my Enteric Nervous system had been authorised and a date awaited. Those investigations have not started. In Dec 2020 the Gastroenterologist/Neurophysiologist involved in the Anal Rectal Neurophysiology tests in 2019. Who prescribed the Peristreen System asked me to participate in investigations to check my bowel Microbiome and analyse what Microbiome exist so alterations to my diet could be advised and monitored. The objective being to hopefully reduce the need to use the Peristreen System EVERY day! That would be done by a test kit similar to a bowel cancer stool test kit sent to my home. The first test kit has not arrived 8 months later. The latest research concludes the Enteric Nervous System (the second brain) communicates with the brain via the Bowel Microbiome. Hence the problem of my brain not receiving signals to indicate when my bowels are full? For further information see the ZOE-Health Study. https://health-study.joinzoe.com
Very interesting. Thanks for publishing. The Pituitiry gland as you know sits just below the brain within the cerebrospinal fluid which supports and protects the brain. The pituitary gland is therefore subject to attack by corrosive iron deposits. The pituitary gland is the master gland of the Endocrine nervous system which controls the Autonomic nervous system para and parasympathetic nervous systems. Systems which control the bowels digestive system heart rate and blood pressure. My SS was claimed to be mild when confirmed in 2016 and as my bleed had been stopped in 2012 I was told Ferriorox was not appropriate. However when my claims of increasing neurological symptoms since 2009 were checked problems with my bowels oesophagus and anus we’re confirmed. So I have been taking Ferriprox since Nov 2019. In 2016 neurophysiology tests located lesions on various crainial nerves (iron deposits). In 2020 despite being very fit and active problems with my heart rate and BP occurred more and more frequently when exercising. Dermatologists referred to neoplasms if uncertain behaviour on my Endocrine glands. Note the pituitary glsndcus the master endocrine gland. The endicrine glands control heart rate and blood pressure by injecting hormones into the blood supply. Note treatment with Ferriprox requires regular blood samples and tests. Slight sealliwing problems were confirmed due to problems with the muscles in my oesophagus in 2018. Those problems were confirmed to also affect the misled in my boweks and an us. So in 2020 I was prescribed the anal irtigation system Piristreen to keep my boweks moving. Note the Vagus Nerves inerveate muscles involved in swallowing digestion bowels and the endicrine nervous system.
Wow Julianne, you have been through the wars. Love your spirit to keep fighting and being the best you can. Wishing you luck with the specialist re Defriprone (Ferriprox). Maybe you could print off some reports for him explaining the importance of iron chelation to reduce further onset of symptoms ? Xx