Long-term NIV success
Ed White knows a lot about assisted ventilation in ALS, a disease he’s had for 11 years. For eight years, he’s breathed with the help of a noninvasive ventilation (NIV) system.
White, 66, with help from his wife, Linda, and hired caregivers in his Fredericksburg, Texas, home, needs ventilation around the clock. But, so far, he’s avoided a tracheostomy (the surgery that makes a hole in the windpipe, or trachea, through which a tube is passed that can be attached to a ventilator).
That may be in part because he has "moderate" involvement of the muscles of the mouth and upper airway (bulbar dysfunction), which makes NIV strategies more feasible. But it’s also because of his preferences, persistence and proactive stance on the issue.
"Noninvasive ventilation is a long-term option for a certain percentage of patients," he writes in "Observations and Reflections," an essay on ALS that he recently e-mailed to friends and professionals. "And, if it’s not a long-term option, it’s an important intermediate step where the patient and caregivers can become familiar with all the aspects and variables of respiratory management."
White started with the advice of professionals at the MDA/ALS center in San Antonio. But he lives an hour away from the city, and, he says, "ALS is local." He and his local nurses and caregivers researched ventilation strategies independently and came up with a customized program.
He uses a Respironics PLV-100 and a Pulmonetic Systems LTV950, both volume-cycled ventilators, a more powerful type of device than the pressure vents usually used with NIV masks and mouthpieces. During the night he uses a Respironics BiPAP S/T-D 30, a less powerful, pressure-cycled ventilator.
"I’m convinced that the use of the volume ventilator has contributed significantly to the length of time I’ve been able to stay on NIV," White says. "In my opinion, too many neurologists are too quick to pull the trigger in favor of a trach. This is, after all, an invasive procedure. I’m willing to go there, but not before I have to."
White advises that anyone with ALS who’s told he or she can no longer use NIV should get a second opinion. Without his extensive research on the subject and his questioning of assumptions, he says, "I don’t think I would be here today."
Trach choice a ‘No-Brainer’
Rita Yorke and her dog, Huffy
When Rita Yorke of Tempe, Ariz., learned her vital capacity (the amount of air that can be exhaled after taking in a full breath) had fallen from 39 percent of normal to 21 percent over the first three months of this year, she knew something had to be done.
At that time, Yorke, 60, was using a noninvasive system with a bilevel pressure ventilator overnight and for several hours during the day. Her neurologist, Kumaraswamy Sivakumar, who directs the MDA/ALS center in Phoenix, and pulmonary consultants, recommended she move to a volume ventilator with a trach.
Changing to a more powerful ventilator while keeping an NIV interface wasn’t discussed, Yorke says, but she doesn’t really mind. The nasal pillows she’d used with her NIV system were "difficult to get adjusted just right," and her head itched from the strap.
Yorke views her switch to invasive ventilation as an active, rather than a passive, decision. As she puts it, "I’m not a passive person."
"If I had a bad heart and needed a pacemaker, I would get the pacemaker and go on with life," she says. She feels the same way about trach-delivered ventilation as she does about other accommodations to her ALS, such as her power wheelchair, her adapted computer software and widening the doorways in her home. The trach turned out to be a "no-brainer" for her, she says.
She now uses a Pulmonetic Systems LTV1000 with her trach and has ordered a 12-foot hose that will allow her to go in the swimming pool with it.
Narrow criteria for trach?
White’s NIV approach isn’t applicable to everyone with ALS. Even John Bach, co-director of the MDA clinic in Newark, N.J., admits that, and he’s among the world’s most ardent proponents of noninvasive ventilation in neuromuscular disease. (See www.doctorbach.com.)
For Bach, severe bulbar involvement is a reason — in fact, for him, the only reason — to perform a tracheostomy.
It’s the bulbar muscles that keep liquids and food going down the esophagus into the stomach, while keeping air going down the trachea (windpipe) into the lungs. Mouth muscles also help NIV users grasp certain types of vent interfaces.
When these muscles are too weak or spastic to function properly, bypassing the whole area by putting in a trach tube below the upper airway (see "Speaking of Speaking") can prolong life. Without it, anything in the mouth, including saliva, drips into the trachea and lungs, and air taken in through the nose or mouth may not end up in the lungs.
Broader trach criteria?
Other experts, however, are more willing to recommend tracheostomy to ALS patients, feeling that the trach connections are stronger and safer than those of NIV. Proponents believe the trach tube’s bypass of the upper airway provides a more reliable pathway to the lungs, and that patients may be more comfortable with a trach than with a mask or mouthpiece 24 hours a day.
Yorke uses a Pulmonetic Systems LTV 1000 laptop ventilator, which fits on the back of her power chair. A Passy-Muir speaking valve allows her to talk, and special software allows her to use her computer.
Edward Oppenheimer, a pulmonary specialist associated with the University of California at Los Angeles who’s had many years of experience treating ALS-related respiratory problems, thinks there’s a growing "tracheostomy phobia" and that the negative aspects of trach tubes may have been "overdone."
He says, "We’ve had wonderful experience over the years with tracheostomy. It’s effective, and although you can have problems, by and large, patients are quite stable."
Oppenheimer emphasizes that leaving the face uncovered allows a person to use facial expression for communication. He also says that people who work or go out in public may feel their appearance is better with a trach than with an apparatus on the face, and adds that masks can cause irritation and pressure injuries.
Family and finances
"At any one time," says Joshua Benditt, a pulmonary and critical care physician at the University of Washington Medical Center in Seattle, no more than 5 percent of ALS patients [in the United States] have trachs." That small percentage reflects the economic and social impacts of caring for a paralyzed, tracheostomized person at home, he says.
"The way that I present it to [patients] is very neutral," Benditt says of the trach option. "I sit down with them and we talk about it, and then we usually have a follow-up appointment with a social worker and a nurse practitioner, often with family members there.
"We really go through nuts and bolts, everything from what is [tracheostomy], the procedure for insertion, what it entails in terms of care, and also the issues of the impact on the family, the financial and caregiver issues. In most cases, I also have them meet with a patient who has [a trach tube] in now."
Rita Yorke’s son, Don Pollard, is the chief organizer of his mother’s care. When the trach was planned, he called a family meeting, at which he and his two sisters arranged for the round-the-clock care their mother would need.
Pollard and his significant other decided to live with Yorke and provide nighttime care, while they hired caregivers for some of the daytime hours. A sister who lives nearby in Phoenix comes in on her days off from her job.
Yorke’s retirement benefits from the state of Arizona have been helpful in covering costs, though not ideal.
Managing and adapting
Adapting to any kind of ventilation requires adjustment, especially when a trach is involved.
Yorke has had two scares — a serious but short respiratory infection and a mucus plug obstructing her upper airway that required a 911 call.
Pollard says they’re now revising her secretion clearance plan to incorporate more use of the CoughAssist device and less reliance on suctioning.
Ed White uses the acronym "MALS" — "manager of ALS" — for people with ALS and their caregivers. "I know of no other disease that involves so many dimensions and requires such extensive management skills to successfully cope," he writes.
"The road from neurology to pulmonology can be treacherous and difficult to navigate. It’s full of potholes and delays."
But he’s glad to be here, and to have avoided a trach.
Yorke feels similarly about her choice.
"It’s a matter of adjustment," she says. "That’s what this is. But when it hits you that you’re here and you really shouldn’t be — that’s pretty emotional."