Reprinted are some of the questions from patients that we forwarded to members of our Global Medical Advisory Board. We have included the responses that we thought would be of interest to you!
QUESTION: The question is asked about life expectancy in CIDP.
REPLY: In general, life expectancy for CIDP patients is good, comparable to the general population who don’t have this disorder. CIDP can follow various courses, sometimes with recurrences or relapses over years, sometimes with a chronic progressive course. Complete remissions can occur, where the disorder seems to burn out. Rarely, respiratory failure may occur, which is relatively much more common in the acute acquired demyelinating neuropathy, Guillain-Barré syndrome (GBS), placing the patient at risk for cardiopulmonary collapse and death. Other potential causes of death in GBS, such as cardiac arrhythmias, are also quite rare in CIDP.
QUESTION: My daughter is diagnosed with CIDP and for 4 years is receiving monthly transfusions of IVIg which has limited the progression of the disease. She still suffers from pain in her hands and fatigue especially the week prior to her IVIg treatment. I have been reading about the use of Low Dose Naltrexone for patients with autoimmune disease and am interested if there is any evidence of use/success with CIDP. Any information would be appreciated. I am a donor to your organization.
REPLY: There are few if any reports on the use of naltrexone with inflammatory neuropathies such as ClDP and Guillain-Barré syndrome. Studies have been
performed using this drug in hereditary neuropathies such as Charcot-Marie-Tooth disease and metabolic neuropathies such as in diabetes and alcohol use as
well as multiple sclerosis. But it Iikely would not be easy to extrapolate from those experiences to predicting naltrexone’s potential benefit in ClDP. The patient is
described as having pain in her hands and fatigue during the week before her monthly IVIg treatment. Rather than entertaining a trial of naltrexone, it might be more fruitful to consider moving the IVIg treatments up, to every 3 weeks, to see if that approach will help relieve symptoms. For pain, such agents as pregabalin (Lyrica) and gabapentin (Neurontin) have sometimes been beneficial and are likely worth a try. The key is to start these drugs at a low dose and slowly build up the dose over weeks till there is benefit or intolerance. There is just not enough information available to offer guidance on trying naltrexone. It seems unlikely that a trial of low dose naltrexone would be harmful if its use is guided by the drug’s literature. All of these suggestions likely should be discussed with your daughter’s neurologist and/or family physician who are familiar with the particulars of her medical status and thus in an optimal situation to offer guidance.
QUESTION: I’m a physiotherapist working in rural Australia and have been asked to provide some assistance to a young boy suffering from this condition. I haven’t reviewed with him yet. But understand it took a few weeks to diagnosis and he has some postural issues, along with fatigue and lots of global weakness. If you have any protocols for physio rehab they would be greatly appreciated.
1. Ideally getting a PT and OT evaluation to work on strength,safety and activities of daily living.
2. Patients with GBS tend to have increased fatigue, for this reason the sessions should focus mainly on balance, stretching, endurance and functional adaptations. We don’t want them to get exhausted.
3. Equipment and brace evaluation is important. Patients may need AFO’s during initial recovery due to distal weakness and sometimes foot drop. We recommend the use of manual wheelchair for long distances if the patient is too fatigued, with the goal of energy conservation.
3. Ideally the session should be performed in a rehab/PT gym to take advantage of the equipment.
4. Be aware if there is any sensory loss, to educate the patient and he family about daily foot inspection and making sure the temperature of the water is appropriate.
5. Educate family about transfers and position in bed.
6. Educate the family about stretching and exercises they can do safely at home.
QUESTION: Part of my job as a Home Infusion pharmacist is initial assessments on potential IVIg patients, so I speak with many CIDP and GBS patients. I was wondering if you have any information on diet and ClDP-specifically ketogenic diets.
REPLY: That has not been formally studied for ClDP that we know of. Some recommend an anti-inflammatory diet, which is avoiding foods high in saturated fats. Eat more fruits and vegetables. Foods thought to reduce inflammation include olive oil, dark leafy vegetables like kale, spinach and broccoli, oily fish like salmon, tuna, mackerel, and sardines. Almonds, ginger root, low-fat yogurt, tomatoes, beets, sweet potatoes. This again has not really been studied but is a reasonable diet anyway.
QUESTION: I have a damaged peroneus and I can’t push my feet up, on the hands I do not have fine motor skills. They told me that this damage is from a vaccine named POLIO. That happened to me when I was 3 years old, now I’m 20. I’m interested if there’s a solution for this and can
you help me? Can I renew my nerves?
REPLY: Damage to peroneal nerves and loss of fine motor skills can be due to several disorders. It is unlikely that these current problems reflect the effects of a vaccine received so many years ago. Rather, it would likely be prudent to consider other causes, such as hereditary (e.g., Charcot-Marie-Tooth disease) or acquired peripheral neuropathies. More information than is provided in the query would likely be helpful to hone in on the underlying diagnosis. It will likely be best to obtain a hands-on evaluation by a neurologist who specializes in neuromuscular disorders and/or peripheral neuropathies. Your local medical school’s university teaching faculty likely has such specialists in their neurology department. The neurologists of the Center of Excellence of the GBS|CIDP Foundation are also suggested as a reliable resource to obtain an evaluation. Best of luck. A timely evaluation is encouraged since earlier treatment of some disorders
may lead to less risk of more nerve damage.
QUESTION: My question for medical expert is: I only receive 55 grams IVIg (has been every 3 weeks since end of April, will be every 2 weeks after this plasma exchange). The other man I know about (different doctor, my age-74, who weighs 1 1/2 times what I do) has been getting treatment (after loading), 5 days straight x 3 times, now 2 days x 2 times, way more grams, is improving happily, was first seen by hematologist to target his IVIg. I have no health issues in my life (till the back surgery last November which threw me into CIDP) except right breast cancer mastectomy 11 years ago, and I do take RX for high blood pressure and for hypothyroid. How many more grams may I take?
REPLY: Different CIDP specialists have different approaches to how they dose IVIg. Having said that, the most common strategy is to give 2 grams for every kg
of body weight as an initial dose and then give 1 gram of IVIg per kg every 3 or 4 weeks. The number of days of treatment is not the important factor; the total amount of IVIg given is the important factor. A person’s age and other medical issues determine how quickly IVIg can be given. A relatively young and healthy person can get 1 gram per kg of IVIg in a single day. In an older patient we may divide that dose over 2 or 3 days (some physicians are cautious and give IVIg over several days in everyone). Some CIDP specialists give less than 1 gram per kg per month. Surprisingly, there is not much research to determine the optimal IVIg dose. However, if someone is not doing well it makes sense to give a higher dose. The bottom line is that it would be reasonable for you to receive at least 1 gram per kg of IVIg every 3 weeks. Some physicians will give as much as 2 grams per kg of IVIg every 3 weeks. That would generally be the maximum.
Hope this answers your question satisfactorily. (to get your weight in kg divide your weight in pounds by 2.2).
QUESTION: Is there a standard protocol that they follow to detect any cardio respiratory regression?
REPLY: For GBS in the acute setting spirometry should be measured frequently, between twice daily and 4 times daily depending on the clinical concern for respiratory compromise. When expiratory FVC decreases below 15cc/kg/body weight or NIF declines to less than 20 cm H20, urgent intubation and mechanical ventilation should be strongly considered. If these respiratory parameters are precipitously declining but have not reached these thresholds intubation should also be performed on a semi-elective basis to avoid urgent intubation at untimely hours or uncontrolled settings.
QUESTION: How hard should one push the patient (especially patients who are say, gym instructors and already attuned to muscle fatigue and/or a certain amount of pain-tolerance)…are there any guidelines in this respect that cater for specific patients like these? That is, if physiotherapy pushes the selected few GBS patients, who ultimately want to be pushed, in a gym style workout rotating limbs, etc. are we causing deleterious effects?
REPLY: There are no formal guidelines to counsel patients on activity following GBS but we know that ability appropriate escalation of exercise is good. It has
been shown that post-GBS patients have the capacity to improve fitness and that exercise improves fatigue (a common residual in GBS). Caution is advised with isometric exercises in muscles that are weak, as this may induce deleterious damage to muscles that are already impaired. I discourage patients from exercising weak muscles until failure. I encourage regular exercise and activity, even in weak muscles, that is appropriate to the patients ability level, safe, focuses on flexibility, and is aerobic.