Post and Meta- title: Chronic Inflammatory Demyelinating

Polyradiculoneuropathy and IVIG (Intravenous immune globulin): Symptoms to

costs/ co-pay assistance

Meta-description:

Chronic Inflammatory Demyelinating Polyradiculoneuropathy (CIDP) is an

uncommon autoimmune disease that impairs nerve transmission. IVIG
(Intravenous immune globulin) can successfully treat CIDP.

H1: Chronic Inflammatory Demyelinating Polyradiculoneuropathy and IVIG:

From symptoms to costs/ co-pay assistance

Chronic Inflammatory Demyelinating Polyradiculoneuropathy (CIDP) is an

uncommon autoimmune disease (i.e., the body attacks itself) where the body’s
immune response targets the protective covering around nerves known as
myelin sheaths. Myelin sheaths are made up of a fatty substance that enables
the rapid transmission of signals in the nervous system. Once damage to the
myeline sheaths occur, nerve transmission becomes impaired.

H2: What are symptoms of Chronic Inflammatory Demyelinating

Polyradiculoneuropathy?

Nerve damage can lead to weakness, fatigue, numbness, tingling, prickling,

cramping, impairment in motor movement (causing uncoordinated
movements) and difficulty walking. CIDP patients may also experience
challenges with breathing, difficulty chewing food, and, in some cases, patients
may even develop respiratory arrest.

Although similar in symptoms, it is important to note that CIDP differs from

Guillain-Barre syndrome in that it is chronic and the symptoms progress over
time (8 weeks or longer). CIDP sufferers may experience symmetric weakness
of muscles in their hips and shoulders. Other symptoms are burning, pain,
clumsy movement, vision impairment and in some instances, muscle atrophy.
Some patients may only experience sensory symptoms which is considered as
a variant. Other variants include:

 Multifocal – asymmetric and involving individual nerves (Lewis-Sumner

syndrome or MADSAM: multifocal acquired demyelinating sensory and
motor)
 Sensory variants – imbalance and uncoordinated movements through
demonstrated muscle weakness
 Distal variant involving the extremities
 Pure motor variant in which no sensory abnormality is detected

H3: What are the causes of Chronic Inflammatory Demyelinating

Polyradiculoneuropathy?

Causes of CIDP are still being researched and to date there is no conclusive
answer. On account of it being an autoimmune disorder, it is difficult to
pinpoint the cause but there are multiple triggers of CIDP. CIDP is rare and can
occur in any age group, however, it has been more prevalent in males over the
age of 50. It can be found in 5-7 patients per 100,000 people. There is no
evidence that CIDP is hereditary on account of there being no clear genetic
predisposition or any predisposing factors. CIDP is therefore considered as an
acquired disease.

H4: Diagnosis and treatment of Chronic Inflammatory Demyelinating

Polyradiculoneuropathy

CIDP is difficult to diagnose and symptoms must be present for at least 2

months. Patients must also present with symmetric proximal and distal
weakness and display reduced or absent tendon reflexes. Testing that can
assist in diagnosis are nerve conduction testing, electromyography to detect
slow nerve conduction velocities, a lumbar puncture to detect an elevated
spinal fluid protein content with the absence of inflammatory cells and MRIs of
the nerve roots to observe nerve roots for enlargement and signs of
inflammation. A biopsy of nerves may also be used to detect CIDP.
Furthermore, a positive response to immunotherapy can provide supporting
evidence for a diagnosis.

There is no standard treatment protocol, and it will depend on the patient’s

choice, side effects, the cost of treatment options, duration/course of
treatment and the availability thereof. There are also the pros and cons
associated with each option that must be discussed in depth before deciding
on the course of action. Three main options and their pros and cons are listed
below:

1. The administration of intravenous Ig, which adds antibodies to blood

plasma to reduce effect of antibodies causing the problem. This option
 leads to rapid improvement in CIDP but may not produce a remission.
This is also a costly procedure and may have supply constraints
2. Glucocorticoids which will help to reduce inflammation and reduce any
symptoms. This option is inexpensive and will be more likely to produce
a remission of the condition. However, chronic use of this drug leads to
side effects which may restrict its long-term use.
3. Plasma exchange to remove the harmful antibodies. This option is costly,
invasive, and limited to being offered by specialized centers which may
be inaccessible to many patients

As a last resort, other drugs may be used in place of those listed above.
Physical therapy may also be used to focus on muscle strength, function, and
motor function.

H5: Dosing

Patients are given a loading dose of 2g /kg for 2-5 days, which is followed by a
lower dose of 1g /kg in 3 week intervals are used as a starting point for IVIG
treatment.

H6: Side Effects

In clinical trials of IVIG for CIDP, headache was the most common side effect.
Other common side effects for CIDP treatment include fatigue, nausea, fever,
and high blood pressure. Serious adverse reactions include exacerbation of
CIDP, hemolysis, increased blood pressure, hypersensitivity, acute rash,
respiratory failure, pulmonary embolism, and migraine. Notify your medical
doctor to effects that you experience.
H7: Uses

IVIG is currently being utilized in haematology, neurology, immunology,

dermatology, and rheumatology. It is currently being studied in the treatment
of Alzheimer's disease, heart failure, epilepsy , connective tissue disease,
adult respiratory distress syndrome, fibrosis, organ transplantation,
encephalitis and mycobacterial infection. 

H8: Cost/ Price

A study found that IVIG treatment can cost approximately $60, 000 USD a year
or just over $65, 000 USD per year if additional therapy is required by a
patient.

H9: Co-pay assistance

CIDP medication is confusing, complicated, and costly. There are many

resources and programs that can help with the burden.

- Obtain the name of your IVIg medication and the manufacturer

- Call the company to find out more about their “medical assistance
program.”

 Gammagard, HYQVIA
888-229-8379
Shire’s Patient Assistance Program
 Who? 1. Have a current prescription for a Shire hematology product 2.
Do not currently have insurance 3. Meet financial eligibility guidelines.

 Gammaked
1-855-353-7466 Ext. 4
Who? Assistance given to families, healthcare professionals and patients
with verifying insurance coverage and benefits for GAMMAKED, helping
to obtain prior authorizations and with claim denials.

 Gamunex-C
888-694-2686
Grifols Gamunex-C Connexions Program
Who? The uninsured/ underinsured. (CIDP only)

 Hizentra
877-355-4447
CSL Behring Patient assistance program:
Who? The uninsured/underinsured are eligible for medication for one
year.
 Privigen
877-355-4447
No copay assistance if fully insured.
CSL Behring Patient assistance program
Who? The uninsured/underinsured are eligible for medication for one
year.
Contact your infusion company (in-home or in-suite infusion)

Speak with your infusion nurse or clinical case manager about financial
assistance programs. You may be assisted if you speak to them directly.

Other Assistance:

 Good Days: Assists in paying costly insurance co-pays of lifesaving and

life-extending treatment.
(877) 968-7233
 HealthWell Foundation: Assists patients living with chronic diseases
with copays, deductibles, premiums, and out-of-pocket expenses.
(800) 675-8416
 Hill-Burton Free and Reduced Cost Health Care:  About 140 health care
facilities nationwide provide free or reduced-cost care for those in need.
(800) 638-0742
 Kaiser Foundation Consumer Guide: Helps people handle disputes with
employers or private health plans. 
 MedMonk: Medmonk helps to provide financial assistance to patients
who are unable to afford their medications.
 National Organization of Social Security Claimants (NOSSCR): The
National Organization of Social Security Claimants’ Representatives is to
advocate for improvements in Social Security disability programs and to
ensure that individuals with disabilities applying for Social Security
Disability and SSI benefits have access to highly qualified representation
 and receive fair decisions.
(201) 567-4228
 Needy Meds:  Provides a free drug discount card that offers a discount
of up to 80% at more than 63,000 pharmacies nationwide. They also
maintain a website of free information on programs that help people
who cannot afford medications and healthcare costs.
(800) 503-6897
 Partnership for Prescription Assistance (US only):  Helps patients who
lack prescription coverage get the medicines they need at little to no
cost.
 Patient Advocate Foundation: Helps patients with access to care,
maintenance of employment and preservation of their finances   
(800) 532-5274
 PSI ACCESS Program: Pro-Bono Attorneys to help you navigate Insurance
issues and medication denials.
Toll free at 1 (877) 851-9065 to speak with an attorney.
 RxAssist: Offers a comprehensive database of pharmaceutical patient
assistance programs, as well as practical tools, news, and articles

H 10: Prognosis

The long-term prognosis of CIDP patients is generally favourable, but less than
50% of patients still need immune treatment and just over 10% have severe
disabilities. Mode of onset, distribution of symptoms, and electrophysiological
characteristics may be prognostic factors for predicting a favourable outcome.

H11: Life Expectancy

Prognosis for CIDP patients varies but data on the prognosis itself is limited on
account of the rarity of the disease. Due to the slow progression and onset of
CIDP, it may go undetected and undiagnosed for a long period of time and
therefore cause extensive damage. Patients have reported complete and
partial remission, as well as disability. Patients may experience occasional
bursts of CIDP coupled with recovery. Others go through CIDP with partial
recovery but then relapse. Others still can experience numbness or weakness
and will require long term care. In extreme cases, paralysis, respiratory arrest
and death have been reported.