Emily was diagnosed with subglottic stenosis - a rare disease with an unknown cause. Tissue builds up in her airway, causing it to close in on itself. She is currently treated at Toronto General Hospital by having balloon dilatation with laser surgeries. Unfortunately, these surgeries are a temporary fix and only last a few months before her airway begins to close in again. She has had 8 airway surgeries in the past 3 years.
The best way to describe it, is trying to breathe through a coffee stir stick, while plugging your nose. The surgery only opens her airway to about 60-70% and it starts slowly closing up again about 2-3 months post-surgery. This makes it difficult for Emily to do the things many of us take for granted every day, like walking up a flight of stairs.
On October 2, 2015, we welcomed our beautiful son, Bentley, into the world. Emily's pregnancy was high risk and complicated by 2 surgeries on her airway.
In September 2016, Emily noticed she was having more trouble than usual. She was waking up at night, coughing and couldn’t hold a conversation without feeling completely out of breath. She had already had 5 airway surgeries by that point, and was good at recognizing when the stenosis was returning. This time, she felt that it had returned aggressively over a few days. She knew that something was different. We decided to go to the Toronto General ER, where the on call ENT measured her airway at less than 2mm wide. She was immediately given a steroid through her IV and was admitted for surgery.The surgeon was concerned that he would not be able to gain access and had us sign a consent to have a trachonamy.
After this scare we began to explore other options. Doctor Lorenz is a surgeon in Cleveland Ohio, who specializes in subglottic stenosis. He is one of two doctors in the world who preform procedures called the Maddern and REACHER. Both procedures are similar in that they remove the diseased lining of the cricoid, while preserving the outer cartilage. (You can read more about this below). The removed tissue is replaced with a skin graft from the thigh with the use of a temporary stent. The stent stays in place for 2 weeks before being removed. To date, permanent results have been seen in all the patients (aprx 20) who have had this, over the 6 years it has been done.
We took a trip to see Dr. L , where he confirmed Emily is a candidate for this surgery. We have estimated the surgery to be between $80-100,000 and will expect to stay in Ohio with our family for the month that she will be in the hospital.
This surgery is not available in Canada. Emily's options in Canada are to have repeated dilatations every 5 months, until they can no longer do them. This could be years and dozens more surgeries. Each time she has surgery, scar tissue builds up and eventually, it becomes too thick for the surgeon to cut through. The only option after that would be to have a complete resection (taking the subglottic out and stitching it back up). Unlike the REACHER and Maddern, the diseased tissue is not replaced with a graft. This is a very high risk procedure and carries significant risks. It permanently damages many patients vocal cords, leaving them with no range of tone in their voice. It also does not always work and patients who have the stenosis return are left without options.
We have many fears about what could happen if she doesn't receive treatment from the US. Emily joined a world-wide facebook group filled with patients and their family members. Over the past year, she has connected with many of them and has faced the reality that complications do happen. A member recently passed away after having complications during a "routine airway surgery" very similar to Emily's. Another member, lost her daughter after she expectantly went downhill, received a tracheostomy, which got displaced. I have seen Emily remain positive about her condition, but also see the way it affects every aspect of her life. As she nears her next surgery, she becomes anxious and worries about the outcome and being around for our son. Last year, she applied to OHIP in hopes to have out of country coverage. It was denied because there is no research paper on the procedure. This is something that is expected to happen, but no date has been set and it could be a couple years and a then a lengthy appeal process to OHIP. It broke my heart to watch her read that rejection letter and the stress surrounding such a huge financial burden that paying out of pocket will be.
Before her diagnoses, Emily was otherwise healthy. She has several half marathon's under her belt and it was during training for one in 2013 when she noticed shortness of breath. Emily continues to do moderate exercise, when she feels good enough to do so. As you can imagine, to it takes a toll on her mental health, when she is not at the fitness level she was at prior to this. This disease comes with other symptoms too, coughing (mucus and irritated airway) and when her airway is very narrow, she lacks energy, wheezy, loud breathing.
She is optimistic that having the REACHER/Maddern procedure done will give her back her quality of life.
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**More on the procedure**
Essentially the differences are that the REACHER is done through an incision in the neck (transcervically - which is closed at the time of the surgery), vs. the Madden which is done through the mouth (transorally). The “R” of REACHER stands for “retrograde”, in which the trachea is opened and the subglottic area is actually approached from below, while an intra-operative breathing tube is used to ventilate the lungs but removed prior to the end of the operation. This allows patients who are not able to be successfully oxygenated with the “JET” ventilator for the length of the Maddern Procedure to still be candidates for these minimally-invasive procedures. Although the operation is technically “open” and not done endoscopically, since endoscopes are still utilized to see better and magnify the operative site, it is called “Endoscopically-Assisted”. Both the REACHER and Maddern Technique remove all the subglottic scarring, and replace it with skin graft from the thigh with the use of a temporary stent. Compared with a resection, the recovery of both surgeries is much faster, and risk of complications greatly reduced.