Insider’s View

Noah Lechtzin, M.D.

Noah Lechtzin, M.D., is a Hopkins pulmonologist who sees ALS clinic patients regularly. He also does clinical research on respiratory problems.

I’m having some breathing trouble but I haven’t had ALS that long. I’m still active. Isn’t it too soon for this?
Some patients do experience weakness in muscles responsible for breathing early in the course of ALS, but it’s uncommon. Most patients are in wheelchairs before they have problems. Even then, breathing difficulties may not surface for some time because the quieter lifestyle places fewer demands on the respiratory system.

Sometimes patients become short of breath early on for other reasons, such as an excess of secretions in the nose, throat and bronchi. Or the upper airway muscles are spastic. We can reduce secretions with drugs. And we also have several medications that lessen spasticity.

Patients typically get referred to me because their pulmonary function readings have dropped. But that’s not necessarily because their diaphragm muscles are weak. To get readings, patients have to breathe into the testing device, a spirometer. But accurate results require a tight seal between mouth and machine. That’s hard when mouth muscles are somewhat weakened.

What about BiPAP?
Most patients know about bi-level positive airway pressure, a type of mechanical ventilator, but ALS clinicians still have questions about its effects. Some researchers thought that by doing a muscle’s work, BiPAP might hasten muscle weakening. Fortunately, that’s apparently not the case. Most studies show that patients on BiPAP have longer survival, better quality of life and improved thinking. And one or two studies suggest that it slows decline in breathing muscles. But others say that’s not true. So we still don’t know with certainty if BiPAP merely offers support or also brings helpful changes.

We’ve just started a patient study at Johns Hopkins, in fact, to answer that question. One group of patients will get BiPAP and one will have standard care for three months. Then they’ll switch places. We’ll measure pulmonary function regularly and compare both groups at the end.

Is it easy to tell who should be on BiPAP?
Not always. Some people need it but aren’t aware they do. If they have difficulty sleeping, have morning headaches and thinking seems a bit fuzzy early in the day, that might indicate a need. But to our surprise, others clearly show respiratory muscle weakness—pulmonary function tests reveal that—but they lack symptoms! For them, it’s hard to say, “Go on BiPAP.”

Is there anything new on the horizon to help breathing?
A current approach is to maximize the breathing you have. So, for example, you decrease built-up secretions by loosening them mechanically so they’re easy to cough up. We’ve used a high-frequency oscillatory vest on more than a dozen patients in the last year. It vibrates the chest and shakes phlegm free. There’s no formal study, but patients find it helpful.

Next > From the Clinic
Lora Clawson, M.S.N., C.R.N.P., manages Johns Hopkins’ ALS clinic, including its clinical trials. In this column she answers typical patients’ questions.