IVIG (intravenous immunoglobulin therapy)

Question:

My husband was diagnosed w/ ALS 4 1/2 yrs ago (he is 43 yrs old now). He recently has a trach - after months of being on a bi-pap. he has been a ventilator for the last 2 1/2 months (since getting the trach) and has just gone for a week of being off the vent during the day. I am wondering if this is normal? Everything that I have learned, read and been taught about ALS is that Respiratory functions do NOT improve. What are your thoughts?
 

Answer:

Your question was forwarded to me by Mr. George Goodwin < alshelp@alsindependence.com >.  I was delighted to hear that (Name Withheld) is doing so much better now on tracheostomy ventilation.

I agree that usually when a person with ALS needs tracheostomy ventilation, they require at least as many hours of ventilation as they used when on noninvasive ventilation (such as BiPAP S/T, VPAP II ST-A, or the KnightStar 330).

Your question: Can independent ventilation improve dramatically for a person with ALS who has weak respiratory (and bulbar) muscle function after starting tracheostomy ventilation?. You are correct, the answer is generally "no". Well, if that is true, how could (Name Withheld) experience be possible? Two possibilities that I can think of are:

<> If the nighttime ventilation that (Name Withheld) receives with the tracheostomy and a portable volume ventilator is considerably better than he achieved with a noninvasive interface and BiPAP, it is possible that this now results in better muscle rest at night and better control of nighttime sleep-related respiratory failure; and that this explains why he is able to breathe independently now during the day. It is my experience that it is often much more likely to achieve optimal ventilation with tracheostomy ventilation than it is with BiPAP. The proper optimal settings initially, and during follow-up, for people with ALS using BiPAP of VPAP are often not achieved. (Name Withheld) may experience that he feels more rested after a night's sleep now, than he did when using BiPAP.

<> Unlikely, but another possibility: If the diagnosis of ALS is not correct and (Name Withheld) has a neurological condition that mimics ALS closely, but is actually a different condition which varies or improves over time.

The good news is that (Name Withheld) is able to breathe independently for more daytime hours at this time. On the other hand you both should expect that with ALS, that over the next 3-18 months the number of hours of ventilation needed will increase, so that at some point (Name Withheld) will need 20-24 hours ventilation using tracheostomy ventilation. Occasionally people with ALS "plateau"; this means that their motor neuron impairment either stops progressing or only progresses very slowly.

I shall send a copy of this email to some colleagues experienced in ALS respiratory care, to see if they have additional comments or suggestions.

In order to evaluate (name withheld) experience carefully, it would be important to examine him and review all his medical records (email has its limitations of course).

With all best wishes,

Edward Anthony Oppenheimer, MD, FCCP
Pulmonary Medicine
Los Angeles, California

Additional Answer:

I would agree with Dr. Oppenheimer's comments. (Name withheld) BiPAP support may have been limited, in part from his bulbar paralysis. Without knowing his BiPAP pressures, the amount of time he was using BiPAP per day before being started on volume ventilation via tracheostomy, and his most current breathing test results, it is difficult to say why he is doing better with volume ventilation. In retrospect, the timing of the tracheostomy procedure was good, both for the protection against aspiration and for the benefit of improved ventilation support. Take care.


Louie Boitano MSc, RRT
Pulmonary Clinic, Northwest Assistive Breathing Center University of Washington Medical Center Seattle, Wa

 

Question: 

I had mentioned my allergic reaction to Dr. Oppenheimer to aspirin as well as sulpha drugs and I think that his response was rather interesting.

Answer:

I too was surprised to hear that you had difficulty with an allergic reaction related to aspirin; this is unusual. Medications have various additives to help bind them together, increase shelf life, coat the outside, etc.; sometimes it is one of these that actually causes the problem, however after a bad reaction you certainly don't want to risk using aspirin again in any formulation. Allergy to sulfa medications is much more common and often includes sulfides which have been used as an additive to preserve color in salads, dried fruit, etc..

Question:

Hello, Let me start by saying that your site is a Godsend. My brother has ALS and I want to give him all the support I can. This leads me to searching the net for anything new in treatment or help aids. I was wondering if you have any information or data on stimulation therapy? My brother is having problems eating and was wondering if this treatment would help him? Thank-You again for this wonderful site, it has helped us greatly

Answer:

Unfortunately stimulation therapy requires intact nerves and muscles in order for an electrical stimulus to work. Another example of this is electrophrenic stimulation (pacing) of the diaphragm when assisted ventilation is needed -- it does not work for people with ALS.

Evaluation by a speech-swallowing specialist experienced with ALS would probably be very useful.

Edward Anthony Oppenheimer, MD, FCCP
Pulmonary Medicine
Los Angeles, California

Question:

I asked Dr. Oppenheimer about the effects of cold weather on PALS as it seems to bother them a lot more than others that do not have ALS and this is his reply.

Answer:

Dear Mr. Goodwin,

It is always a pleasure to hear from you.

Yes, the winter months result in a number of increased risks for people with disabilities and certainly for people with ALS. We all tend to be in closer contact with each other so respiratory viruses spread more easily and thus cold, flu and pneumonia are more common. The cold weather itself is more of a risk for people with motor neuron disease since we use our skeletal muscles not only to allow us to move but to generate heat -- to keep the body warm -- when the temperature around us drops. Thus "able" people, without disability, can maintain body warmth outside in cold weather longer, while people with ALS may not be able to maintain safe body temperature very long in the very cold weather of winter.

The good health practices that are usually advised include:
<> Influenza immunization annually, and Pneumovax immunization at least once and then once again five years later
<> Stay away from people with a cold or flu since the microscopic droplets sprayed out during cough, sneezing, laughing and even speech are quite infectious; crowds of people at stores and airports during the winter months are often places where viruses are passed from person to person
<> Wash your hands, and request other to do so, regularly since this decreases the spread of infectious particles
<> Maintain good nutrition (and be aware of problems with aspiration with weak cough that can lead to aspiration pneumonia)
<> When going outside in cold weather, be sure to dress warmly and limit the time you are outside if the temperature is low, since people with ALS limb impairment cannot shiver with as many muscles or use impaired muscles to maintain body temperature
<> Check your vital capacity and peak cough flow regularly so you know whether your respiratory muscle function for breathing and cough are impaired (if they are impaired then advice from your physician and careful more frequent follow-up is needed).

With warm best wishes,

Edward Anthony Oppenheimer, MD, FCCP
Pulmonary Medicine
Los Angeles, California
 

"The manager asks how and when; the leader asks what and why." Warren Bennis

Question: