My husband Kent, not only has Myotonic Dystrophy (DM) but he also has Multiple Myeloma (cancer of the bone marrow). Kent was originally diagnosed in 2008 with his Multiple Myeloma and it has been a long and arduous journey for him to keep his cancer managed. He has had many relapses which is typical for Multiple Myeloma. Last summer they had to change Kent’s chemotherapy due to acquired resistance for the medications that have worked in the past to keep his cancer under control. Basically that means that the tried and true treatments are now exhausted for Kent and he is having to be treated with newer medications that the doctors don’t have as long of a history with working out the details.  Kent has been experiencing more side effects from his newer chemotherapies.

Due to the reduced immunity that chemotherapy causes, Kent ended up in ICU at Huntsman early this year. They immediately called a code on him. It wasn’t the typical code that you see on TV where they have to give CPR and resuscitate the patient, it was a code for loss of ability to stay oxygenated. It was unreal how many individuals came to Kent’s rescue. One of the head nurses came and sat by me and told me what was going on. I’m sure that they need to keep the family member of the patient updated so that they don’t panic and get in the way of the medical team. It was incredibly helpful for me.

Pretty soon a social worker changed places with the head nurse and stayed by my side. She asked me if Kent needed to be intubated would he want to be. I already knew the answer to this and I told her that if he needed it, he definitely wanted any life support that they could give him. At this point they moved Kent from the main floor to the ICU where they were going to intubate him. The social worker invited me to stay or leave while they intubated him. I asked if it was hard to watch and she said that it was. So she and I left the room and waited in the lobby until they had finished. It took 2-3 attempts to intubate Kent so I was glad that I didn’t stay.

For the next 5 days Kent was intubated and sedated. The sedation is to keep him from pulling the tube out. They also restrained his hands so that he wouldn’t pull it out. These were very intense and scary days.

As you may already know, sedation is very risky for a DM individual. It is hard for those with DM to come out of sedation. It stays in their system longer than the average individual. They were using propofol, which is one of the safer sedations but I was still concerned. When you are under the care of the ICU team, you have excellent doctors that specialize in ICU medicine but it’s also likely that they don’t know about DM and its many complications. I didn’t want to interfere with the excellent team but I also wanted to inform them about DM.

I always keep the Anesthesia Guidelines in my purse just for such an occasion. When Andrea had her heart ablation last December we had a pre-surgery appointment about a week before the procedure and I shared these guidelines with the anesthesia team. They were very appropriate and responsive with the information and it seemed like they would address all of the anesthesia issues for DM.

Of course, we didn’t have a pre-hospital visit for Kent since this was an emergency hospitalization. I didn’t get to personally talk to the head of the ICU team so I wasn’t aware if they were taking my information seriously. It’s a very delicate situation when you want to educate a professional. They have lots of experience and tend to know how to handle these critical situations. But, they also haven’t had experience with DM. So, how was I to inform them without making them think that I thought they weren’t doing their job? About 2 times a day I would question the nurse or the medical team and share with them my Anesthesia documentation. They were always good with me but I never quite felt that they understood the perspective of a DM individual.

At the same time, were the anesthesia issues as great a concern when they had Kent intubated and his breathing was supported. The anesthesia was critical in order to keep Kent intubated. Kent’s fight to stay alive had so many complications. He was diagnosed with pneumonia and RSV (Respiratory Syncytial Virus) and he also has DM.

After 5 days of being intubated Kent finally started to improve and they were able to remove the tube. He was still very sick and he needed to stay in the hospital for 5 more days. For the last 2 days they were able to transfer him out of the ICU and onto the main floor. When it came time to release Kent, we were both surprised that they allowed him to return home instead of to a Rehabilitation Facility. Kent came home on oxygen but it only took about a week before he was able to do without supplemental oxygen in the daytime. For a few weeks Kent had home health visit him and address his Physical Therapy and medical needs. Eventually we were able to get Kent’s care transferred to an out-patient rehab clinic. Kent is still receiving therapy but he is back to work full time and functioning quite well.

After we got home I went to the Myotonic Foundation’s website to look at the anesthesia guidelines. To my surprise, the document that I had been carrying around wasn’t the most recent. Come to find out, sometime in 2018 the guidelines were updated. The old paperwork that I was carrying around was still valid but the new guidelines had information about sedation and intubation included in it. It would have been more helpful if I had the newest guidelines in my purse.

I didn’t know about the update and it’s possible that you don’t either. You might want to check out the current anesthesia guidelines at myotonic.org.