Finally got to the UC Irvine Neuromuscular Center on Monday. Was examined by two doctors and they are doubting my Neuro’s CIDP diafnosis, but I am not sure why. They are going to redo the EMG and NCV tests, but I have to wait until November for that to happen. They seemed to be more concerned about the numbness and muscle wasting along the ulnar nerve in my left hand and arm than the fact I cannot walk.

New blood tests being ordered, including TSH, B12, autoimmune and neuropathy. Because I started having issues after all of my old mercury fillings removed, I am also being checked for heavy metals. I am now waiting to find out if insurance will pay for the blood tests. They didn’t like paying for the last batch, that included Lyme Disease and a bunch of other things.

It has been a long battle trying to get a diagnosis and treatment. I first saw my neurologist August 18, 2016 after losing the ability to walk September 2015. My money is running low after having to pay $7500 per month to stay in a nursing home. This current delay is driving me more nuts. Coming off of two months of Prednisone, which did little for me and now I have to lose 50 pounds.

Since I am in a nursing facility that refuses to,let me have IVig on site and the incursion center won’t take me if I can’t walk, I am really hoping this can be approved soon. I see my neuro on August 8 and the UC Irvine Neuromuscular Center August 14, I would like this started. I am tapering off Prednisone right now as it hasn’t worked as hoped.

This was posted on Twitter July19th:

FDA Accepts CSL Behring’s Supplemental Biologics License Application for Hizentra® Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) Indication
KING OF PRUSSIA, Pa. — 19 July 2017
Global biotherapeutics leader CSL Behring today announced that the U.S. Food and Drug Administration (FDA) has accepted for review the company’s supplemental Biologics License Application (BLA) for Hizentra® [Immune globulin subcutaneous (Human) 20% liquid] for the treatment for Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) as maintenance therapy to prevent relapse of neuromuscular disability and impairment. CIDP is a rare immune-mediated disorder of the peripheral nerves and the effects can worsen over time.
“We remain committed to CIDP patients and their families and the review of this application is another step towards delivering on our promise to them.” said Dr. Andrew Cuthbertson, Chief Scientific Officer and R&D Director, CSL Limited. “We’re also excited about the possibility of adding a CIDP indication for our industry-leading portfolio of immunoglobulin therapies.”
The application was based on data from the largest-ever randomized CIDP trial, PATH (Polyneuropathy And Treatment with Hizentra®). The clinical trial was completed in March and was designed to demonstrate the efficacy, safety and tolerability of two different doses of CSL Behring’s subcutaneous immunoglobulin (SCIG), Hizentra®, compared with placebo, in the maintenance treatment of CIDP patients previously treated with intravenous immunoglobulin (IVIG).
Hizentra®, the no. 1 prescribed immunoglobulin therapy in treating primary immunodeficiencies (PI), the most prescribed SCIG worldwide, and the only 20 percent SCIG designed with the natural stabilizer L-proline, was self-administered by patients and care givers throughout the study. Subjects were allowed to use dose volumes up to 50 mL/site and infusion rates of up to 35 mL/hour, to provide them with greater flexibility and autonomy to infuse when and where they choose. A long-term open label extension study is ongoing and is expected to be completed later this year.
About Hizentra®
Hizentra® (Immune Globulin Subcutaneous [Human]), the first 20 percent SCIG developed for subcutaneous use, is registered in over 46 countries and approved in North America, Europe and Japan. Hizentra®, the world’s most prescribed SCIG, has a proven track record of safety, efficacy and tolerability and has over 3.6 million exposures worldwide since 2010. In the United States, Hizentra® is indicated for the treatment of patients with primary immunodeficiency. In all 29 European/European Economic Area member states and Japan, Hizentra® is authorized for treating patients diagnosed with PI as well as secondary immunodeficiencies.
For country specific indication information, visit:
Switzerland: https://compendium.ch/mpro/mnr/22484/html/de (German) or https://compendium.ch/mpro/mnr/22484/html/fr (French)
Europe: http://www.ema.europa.eu/ema/index.jsp?curl=pages/medicines/human/medicines/002127/human_med_001440.jsp&mid=WC0b01ac058001d124
United States: http://www.hizentra.com/Professional/Prescribing-Information.aspx
Canada: http://labeling.cslbehring.ca/PM/CA/Hizentra/EN/Hizentra-Product-Monograph.pdf
Australia: http://www.csl.com.au/productfinder/hizentraau
Important Safety Information
Immune Globulin Subcutaneous (Human), Hizentra®, is indicated as replacement therapy for patients with primary humoral immunodeficiency (PI), age 2 and older. This includes but is not limited to the humoral immune defect in congenital agammaglobulinemia, common variable immunodeficiency, X-linked agammaglobulinemia, Wiskott-Aldrich syndrome, and severe combined immunodeficiencies.
WARNING: THROMBOSIS – Thrombosis may occur with immune globulin products, including Hizentra®. Risk factors may include: advanced age, prolonged immobilization, hypercoagulable conditions, history of venous or arterial thrombosis, use of estrogens, indwelling vascular catheters, hyperviscosity, and cardiovascular risk factors.
For patients at risk of thrombosis, administer Hizentra® at the minimum dose and infusion rate practicable. Ensure adequate hydration in patients before administration. Monitor for signs and symptoms of thrombosis and assess blood viscosity in patients at risk for hyperviscosity. See full prescribing information for complete boxed warning.
Hizentra® is contraindicated in patients with a history of anaphylactic or severe systemic reaction to human immune globulin preparations or components of Hizentra®, such as polysorbate 80. Because it contains the stabilizer L-proline, Hizentra® is contraindicated in patients with hyperprolinemia. Hizentra® is also contraindicated in patients with immunoglobulin A deficiency who have antibodies against IgA and a history of hypersensitivity.
Hizentra® should be administered subcutaneously only. Do not administer intravenously.
IgA-deficient patients with anti-IgA antibodies may be at greater risk of developing potentially severe hypersensitivity and anaphylactic reactions with administration of Hizentra®. If hypersensitivity occurs or anaphylactic reactions are suspected, discontinue administration immediately and treat as medically appropriate.
Monitor patients for aseptic meningitis syndrome (AMS), which has been reported with SCIg. In patients at risk of acute renal failure, monitor renal function, including blood urea nitrogen, serum creatinine and urine output. Also monitor patients for clinical signs of hemolysis or transfusion-related acute lung injury (TRALI).
Hizentra® is derived from human plasma. The risk of transmission of infectious agents, including viruses and, theoretically, the Creutzfeldt-Jakob disease (CJD) agent, cannot be completely eliminated.
The most common adverse reactions (observed in 5% or more of study subjects receiving Hizentra®) were local reactions (ie, swelling, redness, heat, pain, and itching at the injection site), headache, diarrhea, fatigue, back pain, nausea, extremity pain, cough, rash, pruritus, vomiting, upper abdominal pain, migraine and pain.
Ig administration can transiently impair the efficacy of live attenuated virus vaccines, such as measles, mumps and rubella. It can also lead to misinterpretation of serologic testing.
Please see full prescribing information for Hizentra® including boxed warning.
About CIDP
CIDP is a rare disorder of the peripheral nerves (those outside the brain and spinal cord). The condition is immune-mediated and the effects can worsen over time. The protective covering of the nerves is damaged, which may cause numbness or tingling, muscle weakness, fatigue and other symptoms. CIDP can occur at any age and is more common in men than in women. If left untreated, approximately 30 percent of CIDP patients will progress to wheelchair dependence.
For more information about the PATH study, visit http://www.clinicaltrials.gov and search with identifier: NCT01545076.
About CSL Behring
CSL Behring is a global biotherapeutics leader driven by its promise to save lives. Focused on serving patients’ needs by using the latest technologies, we develop and deliver innovative therapies that are used to treat coagulation disorders, primary immune deficiencies, hereditary angioedema, inherited respiratory disease, and neurological disorders. The company’s products are also used in cardiac surgery, organ transplantation, burn treatment and to prevent hemolytic disease of the newborn.
CSL Behring operates one of the world’s largest plasma collection networks, CSL Plasma. The parent company, CSL Limited (ASX:CSL), headquartered in Melbourne, Australia, employs nearly 20,000 people, delivering its life-saving therapies to people in more than 60 countries. For more information visit http://www.cslbehring.com and follow us on http://www.Twitter.com/CSLBehring.
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Media Contact
Jennifer Purdue
Office: +1 610 878 4802
Mobile: +1 610 306 9355
Email: jennifer.purdue@cslbehring.com

I have found that getting doctor’s to just pay attention to be the hardest part. My journey has been a long one. I was diagnosed with Multiple Sclerosus in 1993, but my problems go back to 1962 when I developed pneumonia at the age of 8 and my only symptom was severe leg pain and not being able to walk very well. After seeing the top ortho doc – who was later President of the AMA – my mom took me to the GP who delivered me, and he came up,with the pneumonia diagnosis.

In 1969, I had another bout of extreme weakness that kept me out of school for a month. In 1974, I saw my first neurologist because of weakness, numbness and pain in my left leg. Checked for Diabetes, but again, blown off.

So in 1991, I,saw a neuro who tested for Myesthenia Gravis, but the insurance start balking at paying for further tests. The neuro suggested a change of insurance to an HMO that would pat better but less options. That led to the MS diagnosis in 1993.

I had been doing fairly well until around 2001, when I started having balance issues. I went to using a cane, to using a walker.

I finally had to retire from work as a computer technician July 2015, as not only was my mobility impaired, but my eyesights was rapidly disappearing. My last outing was to a retina specialist to see if I could be cleared for cataract surgery with having severe retinal bleeding. Got home from tha appointment, and was horribly sick with intestinal distress. By a week later, I wound up in the hospital Emergency Room with what I thought was a build on my rear end. Turned out to be necrotising cellulitis and I was admitted to the wound care unt.

The day after I was admitted, they got me out of bed and I was able to walk around the hospital with a walker. But they started filling me with drugs, including four IV’s at once, high blood pressure drugs which ,add me so dizzy, I totally lost the ability to even sit up, but much less walk.

I had two surgeries for the skin infection, and they removed the worse of my cataracts over the next five weeks. But I could not get them to do a single test as to why I could not walk anymore.

So about a week after my skin graft, I was shipped of the a nursing home to recover, me screaming all the way out about the walking issue.

After four weeks in the nursing home, my skin graft was declared healed and my insurance demanded I leave, but the facility doctor said it would be dangerous for me to leave. The insurance said they didn’t care, they were not going to pay for me to stay and theywouldn’t help in getting any further treatment, as they assumed it was the pre-existing MS causing the issue.

So I paid out of my pocket for an extra 3 weeks – about $5000 – to get some extra help in walking, and I finally headed home December 12, 2015. That lasted for about five hours when I couldn’t get up to got to the bathroom, my housemate called the nursing facility and they suggested I go to the ER for a new evaluation.

We called an ambulance, and they called the paramedics, who insisted that I prove I couldn’t walk. I agreed ro stand and try warning them not to let go but they did, shattering my right ankle and breaking my tibia. The hospital they took me to asked a neuro to see me. MRIs were ordered and he said I didn’t have MS, but I had something.

Again, he was not allowed by the insurance to find out what was going on, so I was shipped off to another nursing home with a suggested neuro consult when the leg was healed. I didn’t get to see an neuro until August 2016. Delays in approving tests got me the CIDP diagnosis the Tuesday after Thanksgiving.

The battle continues with getting treatment. Got five days on IVig at the end of Marc, and I am on my third round of steroids, having put on over 50 pounds. Still in nursing home on my own dime, which is $7500 per month, since I still can’t walk. Money is running out fast.

On August 14, I got to the UC Irvine Neuromuscular Center for a second opinion requested by my neuro. That will determine if I can afford to keep alive.

I started a 6 week run on 60 mg of Prednisone back on June 17, and will do a taper after that. I have been bombarded with steroids for both retinal bleeding (implants in eyes for six months) and after one IVig treat me at the end of March, I had five days of Prednisone in April and six days of Solumedrol pills.

Since starting the steroids, I have put on almost 60 pounds, 21 in the last month. This is all in an attempt to get me walking because the nursing home I am in will not allow the infusion service in for IVig, and the infusion center will not take non walking patients without a full time attendant. This has been very frustrating, because the nursing home is saying it is a liability issue, even though I am willing to sign off on it, as well as the infusion service. The insurance allowed me five day hospital stay for IVig, but it cost them almost $300000 and $1400 for me, plus having to pay the nursing home to hold my room, and when I got back, they had given it away to someone who was admitted to receive IV treatment!

The Prednisone has given me back some strength and a little numbness is gone, but all of the extra weight isn’t helping my ability to walk. I can stand, but haven’t taken any steps yet, as waiting on new ankle brace because to swollen for the old one.

I have been referred to the UC Irvine Neuromuscular Center on August 14, sainting since February for the appointment. My neurologist wants to confirm the CIDP diagnosis and my insurance doesn’t want to pay for any more tests or treatment without confirmation.

All I want is to be able to go home and walk enough to get to the bathroom.