Dr. Matthew Kaufman, MD, FACS, plastic and reconstructive surgeon at the Hackensack Meridian Jersey Shore University Medical Center, talks about a treatment for phrenic nerve damage.
Interview conducted by Ivanhoe Broadcast News in January 2022.
What is the phrenic nerve and what is its function?
DR KAUFMAN: The phrenic nerve is a very important nerve. There’s one on both sides of the body, a right and left. The primary job of that nerve is to control the main breathing muscle called the diaphragm. The diaphragm muscle sits under our lungs and is responsible when we take a breath in, when we inspire, it does the work. The lung is just passively filling up with air, but that diaphragm muscle creates the space for our lung to inhale. The phrenic nerve’s job is to transmit the electrical impulse from our brain to cause the diaphragm to contract and to allow the lung to fill with air. The diaphragm moves down, closer to our abdomen to allow the lung to have more space.
Is that an automatic function?
DR KAUFMAN: Whether it’s voluntary or involuntary, we’re still breathing. We’re sitting here breathing and not thinking about it. Or, when you’re asked to take a deep breath, if you’re at the doctor’s office, you are voluntarily overriding that. When we’re sleeping, it’s involuntary. But you can also voluntarily do a deep breath if you’re doing yoga or other activities. So, there’s two control centers in our brain.
What causes the injury?
DR KAUFMAN: There are many things that can cause phrenic nerve injury. Starting from the top down, the top problem would be a spinal cord injury or even a brain injury. So, it could be a stroke. It could be an injury to the higher portion of the spinal cord, called the cervical spinal cord, where the electrical impulses are not getting out into the phrenic nerve, therefore, not getting to the diaphragm. Working our way down, it could be from a direct injury to the neck area, from trauma. It could be a chronic injury from years of wear and tear and football players or manual laborers that work with their arms over their head. We see a lot of electricians and painters. It could be people with poor posture we call tech neck where someone is crunched up in their neck. It’s the same reason why people see chiropractors. Then, it could be from an injury related to a surgical procedure, any procedure in the neck. It could be from a nerve block injury. It could be from chiropractic manipulation. And finally, we see injuries that occur from chest surgery or chest trauma. So, the nerve runs down through the chest area. After heart surgery, after certain types of surgery and what we call the upper chest or mediastinum for tumors, even lung surgery and, in rare cases, very severe trauma that occurs in the chest area when someone has a collapsed lung or bad rib fractures.
Can car accidents be a cause?
DR KAUFMAN: Yes. Car accidents, falls, whiplash, or neck injuries from being rear ended, things like that.
How serious is this injury? Can this be potentially life threatening?
DR KAUFMAN: It absolutely can. So, in the case of a spinal cord injury where there’s no function on either side, the patient immediately needs mechanical ventilation, or they’ll suffocate. So, those are the most severe cases. In cases where it affects one side, the problem is generally not life threatening but I would say significantly life altering. So, you take someone that’s 50 years old and goes to the gym and exercises, and now, one side is not working, they’re essentially living off one lung. Now, they have exertion, or shortness of breath, not when they’re sitting on the couch, but any time they exert themselves, they are extremely short of breath. Also sleep becomes very difficult. So, lying flat, they’re very short of breath. So, it’s life altering because they’re having trouble sleeping. They’re not able to do any exertional activities. And the prospect of living like that is not a good one to take a healthy 50-year-old and say, well, you’re going to breathe like you’re 80 for the rest of your life. And in that sense, it could be life shortening because they’re prone to weight gain because they can’t exercise. They’re prone to respiratory infections because that lung isn’t working as well on that side. In the current setting of the pandemic, they’re more prone to severe COVID because their lungs are not working at full capacity.
Prior to the surgery, what were the treatments for these patients? What were their options?
DR KAUFMAN: The traditional approach to managing these patients with what we call phrenic nerve injury or diaphragm paralysis would be conservative management, basically doctors saying just learn to live with it because there’s not much that can be done, which is not something that you want to hear if you’re symptomatic. Or a procedure called diaphragm plication, which is a traditional procedure where a surgeon goes in. Instead of trying to make the muscle work again, they’re just stitching the muscle down. They’re paralyzing it into a lower position, which can be effective, but it has its limitations. And certainly not everybody likes the thought of that. And if it fails, then they are sort of out of luck.
Can you describe what you and your colleagues are doing?
DR KAUFMAN: In 2007, we started a procedure called phrenic nerve reconstruction, which is based on the tenets of other types of peripheral nerve reconstruction that are performed in the body and have been performed for decades. For example, fixing a Bell’s Palsy, a droopy face or a paralyzed arm using advanced nerve microsurgery techniques. That means that we can sew nerves together. We can clear scar tissue from nerves. We can redirect nerves. We can do nerve transplanting and get nerves to restore their function. Basically, we can get nerves to work again, similar to doing electrical rewiring in your house. We can rewire the peripheral nerves. We can’t rewire the spinal cord yet. That’s hopefully on the horizon. But we can use the body’s ability for peripheral nerve regeneration, meaning that if we correct the damage, and in this case the damage being around the phrenic nerve, using advanced nerve microsurgery techniques, we can slowly restore the activity back down to the diaphragm and get that diaphragm to turn on. And then, either through rehab or through electrical stimulation or a variety of means, get that muscle to be strong again, which allows the resumption of normal or near normal breathing function.
Could you describe how tiny is an area that you’re working on in microsurgery?
DR KAUFMAN: The phrenic nerve is about a three-millimeter nerve. We always relate nerves to size of pasta. The phrenic nerve is roughly the size of a piece of regular spaghetti as opposed to thin spaghetti or linguine. When we’re sowing nerves or working on nerves, we’re using microscopes to magnify the view of these tiny structures. And when we’re sewing nerves together, we’re using suture material that’s finer than a hair because it’s a delicate structure that we’re sewing. And obviously, we don’t want to injure the nerve when we’re trying to put these nerves together so, we use microscopes. We use magnification and very delicate instruments to accomplish the task at hand.
Is this done through a keyhole surgery, or do you have to open a patient to get in there?
DR KAUFMAN: I would call it limited invasiveness, meaning we’re not doing keyholes. We’re not using large incisions. We’re using limited incisions to gain access. So, it has a limited degree of invasiveness, but a little bit more than keyhole surgery. Some of the techniques that we use, the technology is not adapted for keyhole surgery yet.
How large are the incisions that you’re going through?
DR KAUFMAN: Usually about two to three inches.
How many incisions? Does it depend on a patient and where you’re working?
DR KAUFMAN: It depends on the patient and where we’re working. But typically, it’s one incision to gain access to the phrenic nerve and another incision if we’re using a nerve transplant where we have to harvest a nerve graft. That’s usually taken from somewhere in the ankle area.
How long is the surgery?
DR KAUFMAN: Depending on the specifics of the surgery that we’re doing, it can range anywhere from about three hours to about five hours.
What’s recovery like for patients?
DR KAUFMAN: The recovery is typically about a two-week recovery if we’re working in the neck area and maybe four to six weeks if we’re working to repair the phrenic nerve in the chest region. Usually at about one to two months is when we engage the patient in the rehab phase, which involves some kind of exercise and physical therapy to improve breathing mechanics. In the case of a spinal cord injury, that’s different, obviously, where the patient can’t really exercise. But we do have them engage in other types of rehab using an electrical stimulator called the diaphragm pacemaker.
How long before most patients will be able to notice a difference in their breathing?
DR KAUFMAN: I’ll break it into two different categories of patients. The patients that have injuries that are not spinal cord injuries, where maybe one side isn’t working. And then after that, I’ll talk about spinal cord patients, which is its own separate category. But patients that come in that have one side that’s not working, one phrenic nerve that’s damaged, where they have exertional dyspnea, which means shortness of breath when they’re exercising or being active, we see patients where the recovery begins within the first few days, meaning that we can turn that ignition back on. We can turn on that nerve just a little bit. And every five or 10% of improvement, the patient will notice. So, the first thing they might notice is it’s easier to bend over and tie their shoes. And over the course of a year or two, they can get to maximal recovery. Typically, what we see, and this is based upon our evidence-based research where we’ve looked at hundreds of patients that we’ve operated on, that at a year after surgery, we can see about a 30% improvement in their function. At two years after surgery, they can be anywhere from 60 to 80% of normal. If we get that diaphragm working anywhere north of 50%, they will have very limited problems going forward. They’ll be almost back to normal.
How many procedures have you done? And is it correct to say you are the first in the country or first in the world to engineer the surgery?
DR KAUFMAN: We pioneered this. Prior to our work, and again, we started this in 2007, there were a few case reports of surgeons going in to fix phrenic nerves but usually in the acute setting, meaning if it was during the surgery where they had to take the phrenic nerve out or right after an injury or trauma. We were really the first to develop this and to repair this in a delayed fashion for patients that noticed this problem months later or sometimes even years later. In October 2020, we reached our 500 milestones. As of October 2020, we did 500 surgeries and we have done another 80 or 90 since then. So, we are very close to about 600 surgeries, 600 patients, and may even be there by now. At this point in time, there are a few other centers that are starting to look at this as a procedure or maybe doing some of these based upon our work at some of the larger medical centers across the country. We’ve even advised surgeons in other countries on what we’re doing. Obviously, we’re publishing our work, our outcomes, and we’re lecturing nationally and internationally to help other physicians to gain knowledge of this option.
I know you started in 2007, and there’s always a learning curve. Is there anything that is new or novel that you and your colleagues have added over the last year as techniques get better, as equipment gets better? Is there anything that you’ve changed with the surgery that really makes it even more novel?
DR KAUFMAN: Yes. Over time, we’ve gained knowledge about the problem that we’re dealing with or the variety of problems that we see. In the past, we would not operate on patients unless there was a clear-cut cause. In other words, if someone came in and said, I have a phrenic nerve injury, but I don’t know exactly what caused it, previously, we’d say, I don’t know if we can help. Now, we have a mechanism where we can put that patient through certain diagnostic criteria and testing. And in many cases, we can offer them treatment. That’s called idiopathic nerve injury, or “I don’t know what caused it nerve injury”. Idiopathic means we don’t know. But because of what we’ve seen over the years, we know that many patients have wear and tear or nerve damage without a clear-cut cause. And as long as they meet certain criteria, we can potentially help them. So, that’s one thing that we have advanced. The second thing would is that in performing the surgery, we do what’s called nerve decompression or neurolysis is the surgical term, which means we’re relieving scar tissue or relieving points of compression. And we realize that we have to be more thorough in doing that and basically leaving no stone unturned in the path of that phrenic nerve wherever we’re working if it’s in the neck or in the chest. So, just doing a more thorough surgery and that’s led to greater success. And finally, is to implement the use of electrical stimulation, so when we repair nerves, we find that we can get better recovery if we also subject that muscle and that nerve to the use of electrical stimulation. And that’s known in other areas of the body that that’s helpful. So, in many of the more severe cases, we’re using electrical stimulators, which we call diaphragm pacemakers, to improve our success. And it has improved our success. And we use that electrical stimulation only if it’s necessary. So, once the patient achieves full recovery, or near full recovery, we can remove that electrical stimulator, which is basically wiring that goes into the diaphragm.
Can you speak to what caused your patient, William’s, injury and why he sought you and your team out?
DR KAUFMAN: William was in a very tragic car accident shortly after completing the Naval Aviation Academy, and he sustained a high level cervical spinal cord injury. I believe this was in 2017. He was paralyzed from the neck down and he could not breathe on his own. He had bilateral phrenic nerve injury and diaphragm paralysis as a result of the spinal cord injury. So, he was completely dependent on mechanical ventilation. He was seeking out the top physicians in the country to help him to breathe independently. After a couple of other failed attempts, he was referred to our center. And our center is the only one in the U.S. that’s performing high level surgery for his condition, where we’re combining the use of diaphragm pacemakers, which are electrical stimulators. Those alone are performed at various centers around the country. But our center is unique in that we’re the center that’s performing combined nerve reconstruction, meaning repairing phrenic nerves, simultaneous with the use of these electrical stimulators all in one for the most severe cases, for the most severe types of spinal cord injury where pacemakers alone don’t achieve success.
So, William has had both the surgery to repair, and does he have that pacemaker implanted?
DR KAUFMAN: Yes.
Can you speak to how that procedure went and how he’s doing now?
DR KAUFMAN: When you have a high spinal cord injury, the brain is attempting to send the signal to breathe. But it’s not going anywhere. It’s not getting down to the diaphragm. So, it’s not getting to the diaphragm. In addition, because of the level of his injury, he had deterioration of the phrenic nerves themselves. So, it’s almost like he had two problems. He had a generator that wasn’t working, and he had bad wiring. So, we had to overcome both of those problems before he would achieve the ability to breathe on his own. So, just using the pacemaker is like replacing the generator. But if the wiring is bad, you’re still not going to achieve spontaneous respiratory activity. We did surgery to achieve both. We implanted a diaphragm pacemaker, and we reconstructed his phrenic nerves. He did require a second surgery about a year after the first. But after that, he was able to achieve long periods of spontaneous breathing without the use of the ventilator. And he’s now several years out from surgery and continuing to do well and maintain that success that he’s achieved. And that’s allowed him to live a much more independent life where he’s not tied to this big mechanical ventilator, which is large. It allows him to have more free access to mobility in his wheelchair. It allows him to have an easier time eating and speaking and clearing his secretions. So, there are many advantages to getting off the ventilator. And furthermore, from literature, we know that spinal cord patients that are on ventilators do not live as long as spinal cord patients that are not on ventilators by a large amount of time. So, this will hopefully prolong his life and in addition to giving him greater quality of life.
How gratifying is it as a surgeon to see the difference something you pioneered makes in someone’s life?
DR KAUFMAN: It’s the most rewarding thing you could ever ask for as a surgeon or physician to be able to give this function back to this young man who had such a bright future ahead. And when I see these spinal cord patients and their families and the devastating toll it takes on them and just to be able to give them something back, some sort of normalcy back, we still can’t achieve walking and we still can’t achieve full use of his arms, but breathing is one of the most amazing gifts of life. And when you lose that, it takes a devastating toll. And knowing the consequence of that, which is such a greater risk of infection and early loss of life, and to see now that he’s living a vibrant life, he’s doing some amazing things on behalf of the spinal cord community. And he’s giving back. And he’s such a determined young man with amazing spirit. It’s the most rewarding thing I could ever imagine.
Does the nerve regenerate in some cases without surgery?
DR KAUFMAN: Yeah. We sometimes will evaluate patients early after the injury or the onset of the problem and we follow them. We don’t always operate immediately and that’s because there are a subset of patients that will improve on their own spontaneously without surgery. So, in those patients, we put them on a course of breathing therapy and cardiovascular exercise and see if we can get things to improve on their own, because our bodies can sometimes work wonders. And the injury to the nerve can sometimes heal itself spontaneously.
Is there anything else that you would want to make sure people know?
DR KAUFMAN: Yes. I think this condition of diaphragm paralysis and phrenic nerve injury falls under the category of orphaned diseases or orphaned conditions. That means even many doctors don’t know that much about it or that there are treatments that exist. Every day, I speak to patients from around the country that are told by their doctors who have this diaphragm paralysis condition, learn to live with it. There’s nothing that can be done. And that’s a devastating thing to hear for someone that’s suffering and symptomatic. And we’re trying to fix problems where they’re being told there’s nothing that can be done. There is something that can be done. We can’t help everybody. But to raise awareness of this problem and maybe it only affects a few thousand people a year, it’s not like asthma or sleep apnea or other conditions that are much more prevalent. But for those few thousand patients a year, most of whom are younger and active, and certainly for the spinal cord community, spinal cord injury affects young people the most because it’s usually from a trauma. We want to make sure the word is out there that there’s potentially something that can be done.
END OF INTERVIEW
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Edna Arguello Eric Muench
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