Pheresis and IVIG?
AnonymousJune 12, 2007 at 11:40 pm
My daughter was diagnosed with GBS on May 11, 2007. Apparently she has a very aggressive case. She had 10 plasma pheresis treatments at an acute care facility and symptoms are still getting worse. New ones seemingly every day. She is reliant on a respirator and is totally paralyzed, toes to head. How long do symptons get worse? Has anyone had pheresis followed by IVIG? When and how was it decided to do one, then the other? What were your results? How is SIDP diagnosed? Does anyone know GBS “expert” doctors? Who are/how does one find the best neurologists? I want her to have the best chance for recovery. Despite the fact that I am not the primary decision-maker regarding her care, I have to do something. Can anyone help me help her?!?
AnonymousJune 13, 2007 at 12:18 am
SIDP, basically speaking, is when the attack lasts between 4 and 8 weeks.
Having plasmapheresis does not mean your daughter will ‘get better’ as such. Unfortunately it is not a medication that cures the illness. It is something to try and stop the attack and one does not necessarily see results straight away or even within days or weeks. Why is it they/you are wanting to do IVIg? Has it been determined that Plasmapheresis isnt working for her? Your daughter is on a respirator and totally paralyzed, how long has she been this way? Have you read the article called “Whats in a Name?” the differences between GBS, SIDP and CIDP. If not I will post it for you.
AnonymousJune 13, 2007 at 12:23 am
[I]Just in case[/I] you hadnt read it
[B][FONT=Arial][SIZE=5]What’s In a Name? Important Differences
Between GBS, CIDP and Related Disorders[/SIZE][/FONT][/B]
David S. Saperstein, M.D., Phoenix Neurological Associates, Phoenix, AZ[/I][/SIZE]
[FONT=arial][SIZE=2]This article will discuss the differences between Guillain Barre Syndrome (GBS) and related conditions. Recently I have seen cases where misunderstanding of these concepts led to less than ideal management. I have also frequently observed confusion about terminology among patients and physicians.
[/SIZE][/FONT][FONT=arial][SIZE=2]GBS may also be referred to as acute inflammatory demyelinating polyneuropathy (AIDP). This emphasizes the acute nature of this disorder: symptoms come on abruptly and progress rather quickly. Symptoms stop progressing, often within 2 weeks, and usually not more than 4 weeks. After a period of weeks to months, patients then begin to experience improvement. Although the majority of patients with GBS will do rather well, not all patients will recover fully and may experience chronic weakness, numbness, fatigue or pain. Once symptoms stabilize, there is rarely any further deterioration.
[/SIZE][/FONT][FONT=arial][SIZE=2]Chronic inflammatory demyelinating polyneuropathy (CIDP) produces manifestations similar to GBS, but there are important differences. Symptoms tend to come on more slowly and progress for a longer period of time. Patients may stabilize and recover, but then experience a return of symptoms in the future (this is referred to as the relapsing form of CIDP). Alternatively, patients may experience progressive CIDP wherein there is slow, continuous progression without a period of stabilization. By definition, if there is progression of symptoms beyond 8 weeks, the patient has CIDP. Patients with CIDP often need sustained treatment, but many experience complete remission or at least improve and stabilize on medication.
[/SIZE][/FONT][FONT=arial][SIZE=2]A less well-appreciated disorder is subacute demyelinating polyneuropathy (SIDP). SIDP is defined by a progression of symptoms for more than 4 weeks but less than 8 weeks. In other words, the time frame falls in between that of GBS and CIDP. This is an uncommon but interesting group of patients. It is necessary to identify these patients because there can be important considerations regarding their treatment (see below).
[/SIZE][/FONT][FONT=arial][SIZE=2]The most important reasons for distinguishing between GBS, SIDP and CIDP are to help anticipate outcome and to determine the optimal therapy. Patients with GBS are usually treated with a course of either of two therapies: intravenous immunoglobulin (IVIg) or plasma exchange (PE). IVIg and PE are equally effective (and there is not an advantage to using both treatments). Typically, a single course of treatment is given, usually as soon as possible after diagnosis. The goal of treatment is to hasten improvement. Patients with GBS will improve without treatment; IVIg or PE just accelerate recovery. As discussed above, the full extent of recovery will not occur for many months (or even years). This is an important point that is often not appreciated. Some GBS patients certainly do improve quickly and dramatically after being treated with IVIg or PE. However, most do not. Therefore, repeat courses of IVIg or PE or treatment with a different therapy are typically not indicated.
[/SIZE][/FONT][FONT=arial][SIZE=2]A number of GBS patients will have permanent symptoms. These symptoms are from nerve damage. IVIg and PE treat inflammation of the nerve, but do not help with nerve recovery. Nerve recovery can occur, but takes time. Persistent symptoms do not mean a person has CIDP. CIDP is diagnosed when there is continued [I]progression[/I] of symptoms (not continued [I]persistence[/I] of symptoms).
[/SIZE][/FONT][FONT=arial][SIZE=2]In contrast to GBS, CIDP patients are treated with repeated courses of IVIg or PE (or daily doses of other medications such as prednisone, azathioprine, cyclosporine or mycophenolate mofetil). Without sustained treatment, patients with CIDP will usually relapse and continue to worsen. Over time, the amount of medication can be decreased in many patients and, in some patients, treatment can be discontinued entirely.
[/SIZE][/FONT][FONT=arial][SIZE=2]Finally, we come to SIDP. Treatment is usually as for GBS: a single course of IVIg or PE. This will be sufficient for many of these patients. However, some SIDP patients are actually CIDP patients who got treated before they could declare themselves by progressing for 8 or more weeks. If they are not watched closely, patients with SIDP can quickly deteriorate. These patients will need more sustained treatment, as in the case for CIDP.
[/SIZE][/FONT][FONT=arial][SIZE=2]Now that I have defined the syndromes, I would like to give some examples of how incomplete appreciation of these disorders can lead to misunderstandings regarding therapy. I have seen several patients with SIDP diagnosed with GBS and treated with a single course of IVIg or PE. That is appropriate, but then when these patients subsequently worsened after a few weeks or months, they were either not re-treated or they were repeatedly treated with just a single course of therapy. They would improve and then worsen again and again. In such cases, continued treatment is needed to stabilize these patients (such as IVIg administered every month). A different error is to give a GBS patient IVIg or PE to treat chronic, stable, persistent symptoms. These treatments will not help. Recall that the persistent symptoms are due to damaged nerves. At the current time, we do not have therapies to restore the damaged nerves (but there are medications that can be used to help nerve pain).
[/SIZE][/FONT][FONT=arial][SIZE=2]Hopefully this review has helped clarify the distinctions between GBS, SIDP and CIDP and illustrate the differing outcomes and treatment approaches for these disorders. [/SIZE][/FONT][SIZE=2]
Article from the Summer 2006 GBS Newsletter[/B][/SIZE]
AnonymousJune 13, 2007 at 3:05 am
Hi Jane, so sorry to hear about your daughter. Could you tell us what major city she is near and/or what hospital she is in, once we know that someone will be able to share who their Doctor is. If she is near Ann Arbor then I have a great one. Also please contact the foundation, they will send you a packet of information which will be of great help to your daughter and your family. Call 610-667-0131 and you can request a patient packet, also they might be able to tell you of a Dr. with GBS experience near your daughter. Best of luck with everything, please keep coming back, we are here to help. Take care.
PS-If I can ever be of any help my email is jerimyschilz at hotmail dot com
AnonymousJune 13, 2007 at 11:58 am
I’m sorry you’re going through such a bad time right now. I can only imagine how helpless you feel.
If she was diagnosed on May 11 with a rapid symptom onset, that means it’s been probably around five weeks that she’s had GBS, correct? If so, then she should be about to the stage where she stops getting worse. GBS patients usually continue to worsen until the immune system stops its attack at between 4 and 6 weeks after symptom onset, at which time the condition stabilizes. And even after stabilization occurs, it may take some time before actual improvement can be seen, especially in severe cases.
It does sound like appropriate treatment has been attempted thus far, but like Gene said, not everyone responds to plasmapheresis or IVIg. I was lucky enough to respond well to IVIg and my condition stabilized within 48 hours of starting the treatments (I was about two and a half weeks into GBS symptoms at that point and was still declining–couldn’t walk, could barely breathe, etc.). If your daughter didn’t respond to PP treatments and is still declining, then certainly IVIg would be the next step to take; there’s always a chance that she would respond to that instead. PP or IVIg, if they work, speed up the progression of GBS so that stabilization can occur sooner than 4-6 weeks and also helps speed recovery once it begins, but if it doesn’t work, then it will just take that long to keep getting worse before getting better. It’s a helpless situation, to be sure.
I know it’s hard to be patient, but there’s really not much else you can do to speed recovery. Rather, all you can really do is focus on keeping your daughter comfortable and safe as much as possible. There’s a thread called “Things I Wish They Knew” elsewhere in the forum (do a search and you’ll easily be able to find it) with excellent tips for family members as they care for GBS patients who are still paralyzed.
Best wishes to you and your daughter. I will keep you both in my prayers.
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