About CIDP and Guillain-Barre' Syndrome

Since this is the first time I’ve ever “blogged,” I hope that you’ll be patient, as this may seems like indiscriminate rambling.

My intent with this is twofold:

 1. To get information out about a condition known as CIDP. I want people to understand the effects it can have on the patient, their families, their work and just their overall well-being.

 2. I need to vent. Having dealt with this disorder for a little over a month, now, I can tell you that this disorder sucks on gigantic proportions and there are times that I just want to rail against feeling so darned bad.

What I want to do, first, is tell you a little bit about CIDP (Chronic Inflammatory Demyelinating Polyneuritis). Many people have heard of Guillain-Barre’ Syndrome, but not many have heard of its cousin, CIDP. Guillain-Barre’ Barre’ (which usually sets in after the patient has had an immunization or the flu) is an acute (short lived) disorder of the autoimmune variety. Meaning, the patient’s own immune system attacks them. In the case of Guillain-Barre’, the immune system attacks the patient’s nerves, removing a signal-conducting coating known as myelin. With the myelin removed, nerve signals cannot travel to the muscles, thereby rendering the patient immobile. This is very dangerous, as ALL muscles may be affected (including the diaphragm), and the patient may need to be placed on life-support. Guillain-Barre’ comes on very quickly, usually reaching its peak in 3-4 days (sometimes a week). It can take several years for a patient to regain their strength, and most (85-90%) make a complete recovery. To date, it is unknown how or why Guillain-Barre’ occurs. It has been speculated, however, to have a genetic component.

CIDP is known as the chronic form of Guillain-Barre’. CIDP can come on over a period of weeks, but progresses, usually, much more slowly. Weakness, tingling and lack of coordination in the lower extremities are usually the first signs that there is a problem. Pain in the lower back may make the patient believe that they have a back issue, and delay getting checked out (I am guilty of that). The disease mechanism for CIDP is very similar to Guillain-Barre’. Myelin is removed from the nerve endings, but also affects sensory nerves (touch, taste, smell) as well as motor nerves. Weakness/stamina are the biggest issues with CIDP, with patients reporting that they have markedly decreased endurance. Imagine trying to run a 400hp electric motor using stereo wires to conduct the power. That’s just not going to happen. That is what it is like trying to get your body to run without myelin on the nerves. The signal won’t conduct, so the muscles won’t work. So, while CIDP is not as aggressive, it is more persistent. CIDP can last anywhere from a couple of months to the rest of the patient’s life. It has been shown that the longer the patient has the active condition, the less likely it is for them to make a full and complete recovery.

Treatment for both disorders is very similar in practice, but varies in scope. With Guillain-Barre’, the treatment is aimed at getting the acute phase of the disorder handled, and making sure that there are no life-threatening implications, such as the loss of the patient’s ability to breathe. Plasmapheresis, IVIG infusions, steroids and ventilator support may all be used (although plasmapheresis and IVIG are the two most accepted treatments). Once the acute phase is under control, therapy for GBS patients is essential. Patients need to re-learn how to walk, eat, bathe and other daily tasks that their muscles/nerves have forgotten how to do. This portion of the recovery was often overlooked in the early treatment of GBS, but has since been recognized as one of the most important aspects of treatment.

CIDP is treated with plasmapheresis or IVIG, along with high dose steroids, gabapentin, baclofen and other medications to deal with the various symptoms. IVIG (intravenous immunoglobulin) is given at a dose/frequency determined by the physician. The dose is based on patient weight (which needs to be monitored while the patient is on steroids) and the frequency is based on how well the treatment is working and how long the effects last. These infusions may be given at home by a nurse, or the patient may go to an outpatient center at a hospital, as the patient has to be monitored for any signs of a medication reaction. The medication is given at a very slow rate to minimize the chance of a reaction, and may take up to 4-5 hours. Some patients receive these infusions so regularly, that they need to have an implanted port surgically put in so as to “save” the veins in their arms.

To speak, personally, I am currently undergoing bi-weekly treatments of IVIG, and I am on gabapentin and steroids (although I am tapering off the steroids). To this point, the IVIG has helped, but for very short periods of time. The doctor says I may need to go to treatment every week, but we’ll see. I am very fortunate that I have a neurologist who understands this disorder, and is working diligently to help me get it under control. If this is something that I’m stuck with for the rest of my life, so be it. I would love to see it go away, but the evidence has shown that probably won’t be the case.

One thing I do believe in, however, are miracles. Sometimes those miracles don’t come in the form of healing, but in the trials and tribulations that come your way. They redirect your path and show you where you’re going wrong. Sometimes, they show you what’s important. I will accept whatever it is that Jesus has in store for my life, as He is the one who made me, and He understands where my meager mind fails.