What is chronic inflammatory demyelinating polyneuropathy (CIDP)?
function like electrical wires that carry commands in the form of impulses from the brain and spinal cord (central nervous system) to the muscles and organs of the body. The nerves form the peripheral nervous system. Most nerves are surrounded by a protective fatty coat that helps move the impulses quickly, called the myelin sheath.
CIDP is thought to result from an autoimmune response in which the body's immune system mistakenly targets the myelin sheath (protective layer surrounding nerve fibers). Although the exact cause is often unclear, it is believed that the immune system mistakenly identifies myelin as a threat. This triggers an attack that damages nerves and disrupts their normal function. Signals travelling through the nerves slow down. This causes symptoms that present as muscle weakness, and sometimes sensory changes such as numbness and tingling. The weakness can happen gradually and get worse over time or it can come and go, with partial or complete recovery between episodes.
Signs and symptoms of CIDP
- Weak muscles in arms and legs
- Difficulty walking and climbing stairs
- Sudden changes in the ability to perform daily activities
- Numbness and tingling
- Loss of coordination and balance
Diagnosis of CIDP
Early diagnosis and treatment of CIDP is important to prevent loss of nerve fibers. When nerve fibers are lost, their function is also lost, and it is very difficult to regain that function.
However, due to the rarity of CIDP, it is a challenging condition to diagnose and is often mistaken for Guillain-Barré Syndrome (GBS), as they can present similarly. Health-care providers may suspect CIDP if symptoms last for more than 8 weeks.
The health-care team may recommend several tests to help diagnose CIDP, including:
- Blood and urine tests
- MRI to detect nerve root involvement in the spinal cord
- Electromyography (EMG) and nerve conduction studies to assess muscle function and nerve signal transmission
- Lumbar puncture (spinal tap) to check for specific proteins in the spinal fluid
Treatment of CIDP
CIDP is a progressive condition that can lead to significant disability if untreated. Treatment options include:
- Intravenous immunoglobulin (IVIG): This is the first-line treatment; It helps reduce disability and improve function. IVIG is given through a vein in the hospital and takes several hours.
- Plasma exchange (Plasmapheresis): This procedure involves removing and replacing blood plasma, which may help remove harmful antibodies causing the disease. It is typically used when IVIG or other treatments are not effective.
- Corticosteroids: These medications can help reduce inflammation and improve strength. While they are inexpensive and easy to take by mouth, long-term use can lead to side effects that may limit their use.
Temporary relapses are common but can be treated easily, and long-term remission is a possibility for many patients with CIDP.
How to prevent CIDP?
It is important to remember that CIDP is not your fault, and your child’s condition is not something you caused. However, you can play a key role in helping to manage their health. By ensuring they receive their prescribed treatments and following up regularly with their health-care provider, you can help prevent relapses and support your child in maintaining the best possible health. Your involvement and commitment to their care can make a big difference in their progress and well-being.
Complications of CIDP
Patients with CIDP are often left with ongoing symptoms, such as mild weakness, some sensory loss or impaired sensation and increased fatigue.
What can I do for my child?
Physical therapy and psychological support play a crucial part in managing and recovering from CIDP. Physical therapy:
- Improves strength and mobility
- Prevents muscle contractures and respiratory issues
- Enhances quality of life by improving strength and mobility
- Slows disease progression
How else can you support your child?
- Communicate to your child and their siblings that life goes on, that this illness is a bump in the road and not a dead end.
- Do not excuse a child recovering from CIDP from responsibilities if they are capable. While it is important to adjust expectations, being part of a family means contributing in some way. A child in recovery can maintain a sense of belonging and purpose by playing an active role and offering help where they are able.
- Young children may create imaginative explanations for their illness, often connecting it to things from a magical realm. They might also believe their thoughts or actions somehow caused the disease. However, it is important to address the reality of the illness as it can bring up emotions and questions. All of a child's feelings should be recognized and openly discussed.
- Siblings can be a great support by helping with homework, assisting with physical therapy and making accommodations for the patient. However, it is important not to place too much responsibility on them, as their own experience of childhood is just as important. Balance is key to ensuring that both the child in recovery and their siblings feel supported and valued.
- If the child is capable, involve them in medical decisions. This can be very beneficial to their emotional development and help give them a sense of control in an otherwise powerless situation.
Other management options include:
- Occupational therapy
- Psychotherapy
- Counselling
- GBS/CIDP Foundation of Canada Support Groups (https://www.gbscidp.ca/)
Follow-up care with CIDP
Following up with your neurologist is crucial to ensure your child’s symptoms are well-managed and to prevent relapses or any further health complications. As your child’s condition improves, these follow-up visits may become less frequent but they are still important for ongoing monitoring and care.
It is important to inform your child’s primary care provider of the diagnosis. Children should continue to receive routine immunizations including an annual flu shot and may need adjustments to routine vaccination schedules as a result of treatment for CIDP. If your child requires live vaccines such as measles, mumps, rubella (MMR) vaccine or varicella (chicken pox), their vaccines may need to be delayed by a few months (7-11 months) following IVIG treatment. It is important to discuss with your health-care provider the timing and dose of IVIG in relation to live vaccinations.
At SickKids
SickKids has a specialized neuromuscular clinic that focuses on CIDP and similar conditions. Located within the neurology department, the clinic is staffed by a neurologist, nurse practitioner and experienced technicians who are dedicated to caring for children with CIDP. Our clinic visits range from simple follow-ups to more specialized procedures, such as nerve conduction studies and electromyography. Additionally, we offer access to a professional social worker who can provide support for both you and your child and connect you with relevant support groups if needed. You are not alone in this journey!
https://www.sickkids.ca/en/care-services/clinical-departments/neurology/neuromuscular-program/
Resources
GBS/CIDP Foundation of Canada
gbscidp.ca/
CIDP - GBS/CIDP Foundation International
gbs-cidp.org/cidp/
CIDP Progress
cidpprogress.ca/content/cidp-progress/ca/en.html
Shining Through CIDP
shiningthroughcidp.com
Reference
Querol, L., Crabtree, M., Herepath, M., Priedane, E., Viejo Viejo, I., Agush, S., & Sommerer, P. (2021). Systematic literature review of burden of illness in chronic inflammatory demyelinating polyneuropathy (CIDP). Journal of Neurology, 268(10), 3706-3716. https://doi.org/10.1007/s00415-020-09998-8.
