My CIDP Record 2000 through 2024 - CIDP Experience Log
CIDP Symptoms + CIDP Diagnosis + CIDP Progression + CIDP IgG Treatment for Chronic Inflammatory Demyelinating Polyneuropathy - my cidp journey record

v Definition: CIDP stands for Chronic Inflammatory Demyelinating Polyneuropathy (or Polyradiculoneuropathy) and is considered an autoimmune disorder destroying myelin, the protective covering of the nerves. Typical early symptoms are extremities "tingling" (sort of electrified vibration or paresthesia), numbness, frequent nighttime leg cramps, loss of reflex in legs/feet, muscle fasciculations (muscle bubbling), "vibration" feeling, loss of balance (ataxia), general muscle cramping and nerve pain. These CIDP symptoms may worsen over time, can effect the whole body, and also potentially lead to paralysis (mostly in the limbs). The cause of CIDP is unknown and is thought to be very uncommon but significantly under-diagnosed. In 2013 my CIDP neurologist asked me to keep notes on the effects of a six month immunoglobulin "IgG" IVIG therapy campaign and taking this seriously I decided that it would be useful to post these "notes" for others to see. Thus the launch of my CIDPlog - a blog or journal about my CIDP affliction. I continue to log my CIDP experience through 2024.

My "Mild" CIDP Diagnosis?: It took many visits to six neurologists over four years before, thankfully and almost by luck, I was tested by one who knew about CIDP and its symptoms. Up to that point I had been told for example “to take a holiday” or don't worry "some people are just like that”. : A significant contributor to slow diagnosis is the difficulty I had communicating the symptoms - doctors many times seemed overwhelmed by my descriptions and I feel focused on symptoms that were not relevant in my case (such as strength loss and disability scores*). But it is quite difficult for me to explain my symptoms as the sensations and physical effects are so mysterious. I began to think I was a hypochondriac and accepted this overwhelming view from my doctors but I suspect my athletics and healthy appearance didn't help when it came to diagnosis as doctors found it hard to believe I could be ill. Importantly, I now realize that I have a highly sensory (versus motor) version of CIDP (sensory-dominant) which tends to be invisible to an observer (including my doctors). Unfortunately I wrongfully stopped seeking medical advice for about a year. Since I was diagnosed with CIDP in 2011 through 2015 I am told that my test results indicate only "mild demyelinating sensorimotor polyneuropathy" - and certainly many others are far worse off than I. My CIDP is apparently classified as multi-focal acquired demyelinating sensory and motor neuropathy MADSAM (in the past sometimes referred to as Lewis–Sumner Syndrome). Apparently, MADSAM is not uncommon as a class of CIDP but is considered difficult to treat. Early on (pre-IgG therapy) I experienced progressive very poor reflex in my knees/feet, very frequent muscle cramps, and continuous muscle fasciculations (muscle bubbling) especially in my legs.. In nerve conduction studies (NCS) and/or electro-diagnostic electromyography (EMG), I have slight prolonged F-waves and reduced amplitude latencies. I have slightly above normal protein in spinal fluid. By 2015 I have had progression overall since 2011: "The results indicate a reduced amplitude of the tibial and sensory responses. Conduction velocities in the peroneal and tibial studies are slightly reduced with universal prolongation of F-waves. The synapses are about half of what they used to be". I have had (only) some motor weakness including in my diaphragm.

 

My (70 - 80%) sensory-dominant CIDP is VERY unpleasant, with the continuous pain alone unmanageable and if it is so mild I would say it would be unbearable to have a severe case of CIDP. I have had every conceivable investigation done over the years, even looking for an alternative diagnosis, but all in vain. Bottom line is by 2018 I concluded that all of the symptoms I describe in this log have their roots in my CIDP. As the science surrounding CIDP has evolved, I too by 2023 have begun to appreciate the complexities, including that I may have had, or have developed over time, secondary neurological conditions. I now recognize the "poly" in CIDP (polyneuropathy meaning several) nerves are involved beyond the peripheral and also that CIDP is a spectrum of conditions - it is truly multifaceted and may involve the central nervous system CNS. Nowadays, some doctors actually admit to me that they actually "do not know".

 

My CIDP Development Overview: Started 2001 (aged 56) / Diagnosed 2011 (aged 65) / Progressed 2018 (age now 72 years). It was the strange bodily feelings that made me first think I had a developing health issue and ten years later medical assessments were indicating this too. Outward signs included loss of reflex in my knees & feet but my doctors remained perplexed. Soon the loss of reflex in my knees was total but I did not notice any weakness. About this time the previously weird invisible body-wide fasciculations (especially in eyeballs / eyes) began to show (on my calves), intensify and spread. Then aged 65 years the 2011 diagnosis of mild sensory-dominant CIDP. See "sensory vs motor CIDP" under my cidp symptoms. Now in 2018 aged 72 years I have had significant muscle atrophy (my muscles sag and skin hangs) but I have maintained my leg-strength through commitment to running. My body responds slowly and requires a mental push: I am deeply lethargic, but can still do most physical activities but with less control. Now, it takes me about 100 steps to feel steady walking. Both my feet are "predisposed" to foot-drop. My leg muscles (and feet) have become like rocks and I have a constant day-and-night-time battle against numbness, cramping and pain! The cramps can be single muscle almost anywhere in my body or multiple places (like both leg/s and foot at the same time). I have a whole body invisible minute vibration, or buzz as well as 24-hr fasciculations (muscle bubbling) everywhere (especially visible in the legs). I feel weak (at rest) but in fact am not weak (in motion). I am unable to stay in one position for long particularly sitting at a desk. And in mid-2013 I had developed new unsettling breathing problems (at rest and in exercise) to add to earlier issues that crept up on me over about five years: So again, I find it somewhat troubling that my doctors refer to my "mild" CIPD (referring to NCS results) as clearly to me my symptoms are far from mild!

My recent CIDP Timeline: March 11, 2015 I am told test results show no underlying improvement compared to two years ago and I have more motor involvement. By year 2016 I was having some of the worst days ever for torturous continual pain - in bold because my doctors do not recognize its significance or seemingly the nature of this pain - increasing upper-body involvement, sleep disruption and "sea-sickness" feeling. By early 2017 by breathing issue resolved: Nevertheless for the first time 2017 I feel my life is being threatened overall due to my CIDP, in particular by a feeling that my autonomic body control-system (for temperature, blood pressure and mood) is becoming increasingly unstable. By early 2019 by foot was disfigured and a CT scan showed "patchy sclerosis" in the setting of "inflammatory arthropathy": I have by the end of the year 2019 experience continued symptom spread through my shoulders, neck and head. I have worrisome episodes of dizziness, disorientation and balance problems. Despite NCS showing I am stable in the last few years, my subjective evaluation of my symptoms are that I have somewhat worsened (especially over the last two years). This is illustrated in my composite index chart of symptoms given shown at the bottom of this page. The condition is sometimes quite exhausting! Despite NCS showing I am stable, I the judge my overall health at 30% of the the pre-diagnosis level mid-2019. Entering 2020, new NCS results are encouraging and I do feel I have gained some stability if not shown some improvement in the last six months, perhaps as a result of the sustained Cuvitru IgG SGIG campaign. October 2020 I have experience massive scalp hair-loss caused by Cuvitru (adding to the continuing worrying rash). October 22, 2020: On the advice of my doctors I stopped my weekly CUVITRU scig infusion due to arm pain and potential adverse reaction. My doctor will review the situation November 3, 2020.

v» MAJOR EVENT: November 03, 2020: Doctors believe I am having an allergic reaction to CUVITRU or IgG, and as a result I am off all IgG and I temporarily transitioned to daily Prednisone. As of February 11, 2021, I am off ALL drugs of a month ago. i have tested negative for the Anti-Mag test: Had it been positive I would have alternative treatment options. So after a decade of CIDP, I am entering 2021 with really no treatment for my CIDP and escalating uncertainty. By August 2021, I am in a downward spiral especially with the involvement of my head - headache, weird happenings (I feel like I am having fits or mini-seizures), face pain (especially eyeball / eyes) with wild fasciculations and sudden involuntary snapping of my jaw.

 

My Life-threatening CIDP and again no-one listening after ten years: August 2021, after two visits to emergency for head, face, eyeball / eyes and weird brain issues I feel I have a life-threatening situation. I have even told my main Canadian doctors directly of my belief that my life is threatened, even though NCS mid-December 2021 showed my CIDP remained stable despite quitting IgG a year ago! For more see my CIDP Log after being forced to quit IgG infusions. I have had somewhat of a breakthrough after consulting doctors in Portugal, who in October 2021 had prescribed Oxcarbazepine for my eyeball/ face/ head issues. By January 2022 the drug greatly helped dissipate the severity of my head symptoms. Very surprisingly the Oxcarbazepine also strongly suppresses the wild fasciculations not just in my head but also my legs! I judge my overall health now in January 2022 at about 15% compared to October 2021 5% of the the pre-diagnosis level. March 2022 I faced mind-brain blank-outs and I feared could get into some form of locked-in syndrome: Doubling the Oxcarbazepine dose has fixed that - see my CIDP with life-threatening "mini-seizures" on Oxcarbazepine. Yet December 13, 2022, after twenty months off all CIDP drugs, I am officially discharged from the CIDP program as, by NCS yardsticks, by CIDP is stable! . My actual situation ending 2022 has got me increasingly thinking that I somehow have central nervous system involvement (with CIDP) although my doctors say not† Entering 2023 I had judged my overall health at less than 35% of pre-diagnosis level by my symptom yardstick. But mid-January I had a very disturbing turn for the worst when I had two critical breathing mini-seizures and developed racing pulse issue (over 180 beats per minute). The evidence is mounting that I do indeed have autonomic central nervous system CNS. So by march 2023 I am back at life-threatening 5%; After a diagnosis and treatment for supra-ventricular tachycardia SVT arrhythmia my pulse stabilized. First quarter 2024 I NOW take only the low-dose Metoprolol for arrhythmia.

 

v» ASTONISHING FINDING; Eagle Syndrome Diagnosis July 16, 2024: This year 2024 my number one "CIDP symptom" is facial fasciculations and facial pain, but I feel stable with overall health at 40% of pre-CIDP diagnosis. July 16, 2024 a potential breakthrough on facial pain as I have a diagnosis of "Eagle Syndrome", believe it or not after referral by my dentist!. Eagle Syndrome or Stylohoid Syndrome, caused by an elongated styloid process and calcified stylohyoid ligaments around the jaw, could explain some or all of my head/ face symptoms as the syndrome effects the cranial and vagus nerve (part of the CNS) and carotid arteries. Autonomic dysfunction is a feature of Eagle’s Syndrome. BIG QUESTION now is:How will we determine CIDP versus Eagle Syndrome causation of my symptoms especially since I am finding that expertise in Eagle Syndrome / elongated styloid is extremely limited?.

 

v My Last dated 2024 more detailed timeline Post is at "My CIDP log Going Solo without CIDP drugs" from February 18, 2021. this Link

 

My CIDP Management: Other than CIDP I have always had excellent health. As a 35 year veteran of 10k running I am very aware of changes happening to by body, and certainly did not expect to experience CIDP symptoms. But June 8, 2013 I tried and cannot run a 10k at my normal pace and indeed even a very slow 8.5k is problematic. My breathing prior to this issue was perfect - running a 10k with ease (NO elevated breathing necessary). This lasted about a year and by now in 2016 I am back to running 10km at a reasonable pace. Although movement is uncomfortable, exercise may be the best non-medical treatment for my CIDP. I also do daily stretching to alleviate cramping and I find frequent all muscle movement an essential treatment for my CIDP. Entering 2017 I find pain management a dominating need: movement, exercise, stretching as well as keeping "overheated" seem helpful in controlling my CIDP nerve & muscle pain. I have an excellent diet which is gluten free and this has reduced my CIDP-induced digestive discomfort. Previous to October 2017, I did not take drugs or vitamins (other than the IgG IVIG treatment, a blood product, commencing March 2011). However, this October 2017 I experimented with cannabidiol CBD oil extract from Cannabis Marijuana as a CIDP pain-killer but found it ineffective. In June 2018 I began I trial of very high "pulse-dose" dexamethasone corticosteroid aimed at better outcomes but this too was counterproductive. I avoid exposure to chemicals especially those at home including perfumes/lotion. More at "My CIDP Management of CIDP symptoms, CIDP pain, CIDP IVIG and subcutaneous infusions ".

Content: On this website CIDPlog I give you ONE person's version of how it is to have CIDP. According to doctors the symptoms of CIDP vary widely among patients and they say there are variants** of CIDP. Although I am a technical person, I am not a doctor so please do not think that I am offering medical advice on this site: All I suggest is that if your experience-and-symptoms are similar to mine tell your doctor about CIDP and consult one of the very few neuromuscular specialists in CIDP: Seek out a few opinions as even CIDP specialists do not always agree on CIDP symptoms or treatment! If caught early doctors say CIDP can be at least controlled (and remission can be achieved) with the blood plasma extracted product immunoglobulin or IgG usually administered intravenously, namely IVIG infusions. IVIG is a growing albeit very expensive first line treatment for CIDP and has proved fundamentally helpful in my case - especially in the early stages of my CIDP. I started IVIG March 2011 and the effectiveness by 2015 is much less and I am not cured (and indeed my doctors talk only of preventing further progression and never of a cure). For me CIDP as you will read is not pleasant: day-to-day living is quite a chore and the dependence on IVIG pretty tough going. In October 2015 I started infusing IgPro20 subcutaneously (Sub-Q Hizentra) weekly for an indeterminate time but this was stopped when it proved ineffective. As of March 2016 I was placed on a low maintenance dose IVIG and this continued through year 2017. Starting with my January 2018 infusion I began participating in a year-long (full dose every four weeks) clinical trial of Panzyga IVIG manufactured by Octapharma. Dec 2018 I went back to Sub-Q or SCIG IgG subcutaneous weekly infusions, this time using Cuvitru for 2019. My last Cuvitru scig was on October 16, 2020 as I had developed an allergic reaction to IgG. On this website CIDPlog.com I will -

1. Record my CIDP Timeline 2000-2020 until now (periodically updated)


2. Document and describe my CIDP Symptoms now in 2015 and beyond


3. Provide a summary log and findings on my CIDP infusions of IgG 2011-2020


4. My CIDP Log after forced to quit IgG infusions October 16, 2020 - January 2021


5. My CIDP Since Going-Solo without CIDP Drugs February 18, 2021 - Today
   
   AND LOGS OF RESULTS OF MY IgG CAMPAIGNS -
   
   
6. Daily log of response to 2nd eight-months round of IVIg July 2013 - April 2014
7. Monthly log of IVIG impacts March-October 2015
8. Log of Sub-Q trial SCIG (subcutaneous self-infused Hizentra) Oct. 2015
9. Low "Maintenance Dose" IVIg Therapy for CIDP March 2016 though 2018
10. Log of clinical trial of Panzyga IVIG Jan-Dec 2018
11. Log of Sub-Q Ig Cuvitru subcutaneous Dec 2018 through Oct 2020
12. Log of transition off IgG Infusions after Adverse Reaction to IgG November 2020

Unfortunately my IgG IVIG treatment was started too late due to the delay in diagnosis. I feel this was a result of the very low awareness of CIDP, patient-doctor symptom communications difficulties and the lack of expertise required to diagnose CIDP. Doctors kept telling me not to worry for eight years when I knew something was wrong! I have personally witnessed that this CIDP expertise is limited to less than a handful in Canada and there is only one CIDP Clinic in Canada that I could identify. However, for the ten years 2013-2023 I did have world class medical advice on CIDP from the very best in Canada - indeed a fully internationally recognized Centre of Excellence CIDP Clinic (Toronto General Hospital)! I visited the TGH CIDP Clinic every six months or shorter period when I have issues. I would usually have nerve conduction studies during those consultations.

Please write me with your CIDP experience: I would like to hear especially from CIDP patients who think I have missed something due to their own experience with CIDP.

Editor, CIDPlog.com email: editor@cidplog.com

† See reference in "My Message to Doctors" on September 2022 article "CNS Involvement in Chronic Inflammatory Demyelinating Polyneuropathy -Subtle Retinal Changes in Optical Coherence Tomography". This paper is very relevant to my CIDP situation as I have had eyeball issues since my CIDP diagnosis and in 2022 my retina damage is now significant.

GBS Guillain-Barré Syndrome relation to CIDP and the ZIKA virus: CIDP is the chronic version of GBS Guillain-Barre Syndrome meaning that it is more persistent with CIDP symptoms tending to develop over a much longer timeline - a diagnosis of Guillain-Barré Syndrome may be entirely reasonably later be revised to CIDP if the symptoms persists long term (over eight weeks). GBS Guillain-Barre Syndrome has in February 2016 been linked to the ZIKA virus (notably in Brazil & Columbia). If your symptoms are similar to mine, developed suddenly and you could have been exposed to ZIKA bearing mosquitoes you should seek medical advice as soon as possible!

* Rasch-built Overall Disability Scale (R-ODS)
** CIDP VARIATIONS: Chronic neuropathies are operationally classified as primarily demyelinating or axonal, on the basis of electrodiagnostic or pathological criteria. Demyelinating neuropathies are further classified as hereditary or acquired—this distinction is important, because the acquired neuropathies are immune-mediated and, thus, amenable to treatment. The acquired chronic demyelinating neuropathies include chronic inflammatory demyelinating polyneuropathy (CIDP), neuropathy associated with monoclonal IgM antibodies to myelin-associated glycoprotein (MAG; anti-MAG neuropathy), multifocal motor neuropathy (MMN), and POEMS syndrome. They have characteristic—though overlapping—clinical presentations, are mediated by distinct immune mechanisms, and respond to different therapies. CIDP is the default diagnosis if the neuropathy is demyelinating and no other cause is found. Anti-MAG neuropathy is diagnosed on the basis of the presence of anti-MAG antibodies, MMN is characterized by multifocal weakness and motor conduction blocks, and POEMS syndrome is associated with IgG or IgA λ-type monoclonal gammopathy and osteosclerotic myeloma. The correct diagnosis, however, can be difficult to make in patients with atypical or overlapping presentations, or nondefinitive laboratory studies. First-line treatments include intravenous immunoglobulin (IVIg), corticosteroids or plasmapheresis for CIDP; IVIg for MMN; rituximab for anti-MAG neuropathy; and irradiation or chemotherapy for POEMS syndrome. A correct diagnosis is required for choosing the appropriate treatment, with the aim of preventing progressive neuropathy.
* Diagnosis and treatment of chronic acquired demyelinating polyneuropathies Article · Literature Review in Nature Reviews Neurology 10(8) · July 2014 with 170 Reads DOI: 10.1038/nrneurol.2014.117 · Source: PubMed Cite this publication • Norman Latov

Kenneth C. Gorson 2014 Abstract: Chronic inflammatory demyelinating polyneuropathy (CIDP) is an immune mediated disorder of the peripheral nervous system with clinical features that include weakness, sensory loss, imbalance, pain and impaired ambulation which may lead to substantial disability. This review highlights current treatment strategies for CIDP, how best to utilize proven therapies such as intravenous immunoglobulin, oral prednisone, pulse dexamethasone, and plasma exchange, and when and how to use alternative immunosuppressive agents when first-line therapies are ineffective or poorly tolerated. >See PDF version: Gorson-Update-CIDP-Management-2014.pdf

CONCEPTS for My CIDP Log of CIDP Experience + CIDP Symptoms and treatment + CIDP Diagnosis + CIDP Progression Timeline + CIDP IVIG Treatment: CIDP, C.I.D.P., my cidp symptoms treatment, CIDP Log, my personal story patient CIDP as Chronic Inflammatory Demyelinating Polyneuropathy,my CIDP affliction sufferer of Demyelinating Neuropathies , Neuromuscular, living with CIDP as a patient, CIDP prognosis, IVIG treatment, CIDP Case Study, Chronic Inflammatory Demyelinating Polyradiculoneuropathy, CIDP Case Study, GBS, Guillain-Barre, CIDP symptoms, CIDP treatment, rare disease, my CIDP story, CIDP drugs, immunoglobulin Igg or IVIG imunoglobulin CIDP IVIG infusions, IVIG Therapy, CIDP ataxia, spontaneous, CIDP fasciculations, CIDP tremor, CIDP twitch, CIDP balance, CIDP and exercise, CIDP runner, worsening CIDP, how long IVIG, livingwithcidp.org, living with CIDP, my cidp personal blog as CIDP patient, getting worse with IVIG, getting better with IVIG, CIDP pain, GBS/CIDP Foundation, sensory, autonomic, motor, strength, ALS, neuron, neurological, neurologist, CIDP muscle weakness, assessing IVIG success, CIDP Canada, CIDP specialist, CIDP doctor, breathing, shortness of breath, Dyspepsia uncomfortable abnormal awareness of breathing, respiratory arrest, respiratory distress, digestion, cardiac, autonomic, neuropathy, peripheral, intolerance, gluten free, autoimmune, disease, syndrome, condition, medical, medicine, motor neuron, MND, blog, discussion, cidp blog, CIDP in Canada, Intravenous Immunoglobulin, worldwide IgPro20 drug trial, research, survey, side-effects, my CIDP, timeline, progress, progression, covid-19 coronavirus pandemic prognosis, F-wave, cidp worse with ivig, symptoms of cidp short of breath, life threatening, Autoimmune Polyendocrine Syndrome, conduction studies or electromyography, multifocal motor neuropathy (MMN). GBS related to ZIKA virus causes GBS CIDP. Updated August 14. 2023