Port Placement?

[Ashley]

Hey Price,

I know we talked briefly on facebook about the possibility of you getting one!

The procedure itself wasn’t too bad for me. I work with the team who put mine in, so I had a little bit of a difference experience because I personally knew everyone including the surgeon. My advice to you is to make sure your anesthesia team is aware you have LEMS. They should be familiar with LEMS/ Myasthenias in general and know that you should not have any paralytics. They should be able to do this procedure easily without them, they did not use them for mine. If they do have to use them, it will take you a little longer to come off the ventilator but you may already know this.

I would also make sure to ask that they use local anesthesia at your port site, this will help with post-op pain a lot! I didn’t have to take narcotics except during surgery and the first day post-op so it shouldn’t be too painful. The only painful part for me was in my neck where they tunnel the tube in your jugular. Most surgeons I know use skin glue to close the incision, so you should be able to shower after if they do so, unless they access your port.

 

I did not have a problem with anesthesia, I was given a good amount of fluids. I also have a history of post-op nausea and vomiting, so I was given Zofran in the OR, but also asked for a scopolamine patch pre-operatively that I kept on for a couple of days after surgery to prevent any nausea.

 

I will be glad to answer any other questions you have! Hope this helps!

[Price Wooldridge]

Great information Ashley.  Thank you.  I’ve been put on the vent several times for procedures, but all prior to having the LEMS diagnosis (though it was present).  Waking back up in recovery has been a bit rugged, but not horrible.  I’ve taken notes on your comments and will have them to discuss with the anesthesiologist in advance.
I appreciate the comments on post op challenges too.  I have Zofran pushed into my IV prior to infusions due to nausea, so I’m familiar with that.  It works well for me.  Again, I’ve taken notes on the issues for the next day.
if I think of anything else! I’ll post.  Thanks again!

[Michael Morale]

Hi everyone, my name is Michael Morale and I’m the Senior Director of Multichannel Content for Lambert-Eaton News, and their parent company, BioNews Services. I’m a member of all of the rare disease forums, and the topic of subcutaneous ports is something that I’ve been looking into myself, and I wanted to get your opinions.

While I don’t have Lambert-Eaton, I do have spinal muscular atrophy, which is a neuromuscular disease that falls under the umbrella of the Muscular Dystrophy Association. In September, 2017, I started on the first FDA approved treatment for SMA, called Spinraza. All of us that are on this treatment, receive this drug via an intrathecal, or IT injection, which is basically similar to an epidural that a woman would get during childbirth. This lumbar puncture is the only way to deliver this treatment, because they have to target the central nervous system. While I receive my injections in my back, others who receive this treatment can get them through their sides, and some people get these injections through their necks.

The topic of subcutaneous ports was brought up about a year ago, because the majority of patients with SMA, have had spinal fusion, which can pose a problem when trying to find an opening between the vertebrae to insert the needle to get to the central nervous system. There have been numerous people that have not been able to take their treatments because of this problem. One of the members of the SMA forum said that he found a doctor that would implant a subcutaneous port, and after he had this procedure, he’s been able to receive his injections without any problems. When I started looking into this topic myself, I came across one major problem, which dealt with trying to find a cardiovascular surgeon or doctor who would be willing to implant a subcutaneous port on a patient. A lot of these doctors were telling SMA patients that they wouldn’t do this procedure, because their concern was the amount of time that this subcutaneous port would be in the body. Our injections are every 4 months for the rest of our life, and doctors are hesitant with regards to putting a subcutaneous port in, given that we would probably need to keep them for the duration of our treatment.

For those of you with Lambert-Eaton, are you finding the same kind of problems with regards to finding a doctor that’s willing to talk to you about the subject, and possibly put a subcutaneous port in?

[Dawn DeBois]

Hi Michael,

I’ve lived with the challenge of a spinal fusion and rod from scoliosis surgery when I was 13 complicating having an epidural and going through the lumbar puncture process leading to diagnosis. Put simply, I know the horror all too well, especially if you chance a provider who isn’t meticulous when accessing the spinal fluid. Ugh.

I had to have general anesthesia for my c-sections and then with one of my lumbar punctures, it was termed “traumatic” in the results due to the amount of blood in the spinal fluid sample. I was also traumatized by a 2 week spinal migraine.  The pain of your brain sitting on your skull instead of bring cushioned in spinal fluid is unmatched. So, I can’t imagine being an SMA patient, with a fusion, needing the treatment that works so well but that can only be administered via lumbar puncture? Ugh!

My conversations with my neuro in regards to the possibility of a port for my IVIG treatment were always coupled with concern expressed from her over the risk of infection. Many times an expression says more than the words; and her expression told be I want to explore all other options, first. She would have ordered a port, however, if I requested it. Thankfully I have the sub q route to turn to for my IVIG instead of getting a port at this time.

[Ashley]

Hi Price,

I’m glad I could help you!

 

Hi Michael,

My surgeon put my port in knowing that IVIG would be a lifelong treatment for me and told me I could keep it as long as I needed to unless it clotted or became infected. Luckily, I personally work with this surgeon at my job, so she knew me well and knew I was educated on the care that comes with having a port (I am an RN). I think most people may be concerned that having a port long-term could cause a higher risk than a short term one. With that being said, I think they can be worth the risks and the benefits of having one in this case outweigh the risk. I think you should be able to find a surgeon that would be able to put one in for you. The most important part of having a port is being sure that you are familiar with sterile technique and are educated in caring for your port and not being afraid to speak up if you feel someone has broken technique while accessing you since it is considered a “central line.” If you have any more questions, I will be happy to answer them! I have had my port for almost 3 years in Feb. and it is the best thing I’ve done for myself and makes my treatment much easier. In the past, I was an incredibly difficult stick and often was stuck up to 7-8 times just to get an IV for treatments.

[Price Wooldridge]

Just to update, my neurologist has approved my getting a port for IVIG.  I read this in my notes and last week was called by a nurse in neurology to inform me.  I’m waiting now for any contact on this from Interventional Radiology where the referral was sent.  Re-reading your comments, Ashley, I will be interested to see if a surgeon can be found for the procedure and what their experience may be.  Surely, in a large facility like the Dallas VA Medical System, the procedure must be performed with at least some regularity.  I have also reviewed your previous notes regarding the surgery and and prep to discuss with the anesthesiologist.

Tomorrow I have a meeting with my neuro and will also discuss the possibility of moving to sub-q since the port procedure is not scheduled yet, and I’m hoping sub-q is still on the table.  Honestly, it would be my preferred route.  We will see.  I have IVIG scheduled for this coming Wednesday/Thursday, so nothing will be in place for this round.

[Dawn DeBois]

Price, good luck with your appointment tomorrow. Using SubQ is new to neurologists as it was always thought that the only way a large enough dose could be given would be through IV. Hizentra gaining the Chronic Inflammatory demyelinating polyneuropathy (CIDP) indication proved that SUBQ could be just as successful with larger doses as with smaller ones for Primary Immunodeficiency (PI). Until now, SubQ was only used for smaller dosing requirements of Ig. Make sure to either bring the Hizentra Package insert or be prepared to send her to Hizentra.com to look at the details. My request for SubQ was the first my neuro has had.  That being said, I’m thrilled with how it’s going. The side effects are negligible and I feel an energy gain that I never had with IVIG- perhaps because my body isn’t using energy to recover?

I was going to need a port if I didn’t go to SubQ. By going to SubQ I alleviated that need, and, after looking at my insurance statements, SubQ is less than half the cost of IVIG. It’s win win for me!

[Price Wooldridge]

Dawn, so you’re saying Hizentra is the only game in town for sub-q?

[Dawn DeBois]

Price, there are other brands of subcutaneous immunoglobulin g; but Hizentra is the only one that had an autoimmune dosing indication. They’ve done the necessary studies to prove efficacy.

[Ashley]

Price,

I hope your appointment goes well! If you do end up getting the port, I would recommend asking for general anesthesia if you can. They do sometimes do the procedure under what they call a “mac” which is similar to anesthesia for a colonoscopy. If you are comfortable with that, then go for it, but personally I wanted general anesthesia and to go completely out. It’s not a long procedure at all, usually takes about 45 mins, sometimes it can be done quicker depending on the surgeon.

How did you appointment go about transitioning to sub q? I am kind of thinking about asking to do the same although I am pretty happy with my treatment although I wish I could do it at home instead of the infusion center. In the past, I did not have a co-pay at all with doing my infusion at the infusion center, but I would have one if I chose home health so I decided to keep it at the infusion center to save some money. This year my insurance has changed as they got rid of the “premium” plan I had, so I am waiting to see what my copays will look like still.

[Price Wooldridge]

Ashley, we’ll have the Sub-Q talk with my neurologist tomorrow when I can better judge what her thoughts are with the two alternatives.  The VA system my be the deciding factor.  I visited with Dawn today, so I’m up on her Sub-Q experience, and your thoughts have given me a perspective on the port.  I’ll report back after the appointment.
I’m with you 100% on full anesthesia.  I’ve had several procedures, and save the colonoscopy, all were intubation, taking me all the way under.  That’s the only way I want to do the port placement too.  As long as I’m on the vent they’ve got good control of me.  Stay tuned…

[Price Wooldridge]

Ashley, they scheduled my port placement for the 20th!  Whoop!  Talking to them, it doesn’t appear full anesthesia is on the menu.  Looks to be the colonoscopy-esque “mac”.  Picking your own surgeon is a fantasy in the VA healthcare system, where everything is much like an assembly line at Ford Motor Company.  No worries about an inexperienced team here.  They line ‘em up daily and send ‘um through.
Knowing all this, do you have any tips, or things to try and ask for.  I think you mentioned something to keep the pain down at the site???   Versed has always been my friend!  Sub Q now appears to not be an option, so port it is.  I’m excited to get it in!

[Ashley]

Price,

Happy that you have it scheduled! Your infusions will become so much easier with it! I would be sure to mention that you want a good amount of local anesthesia. This will help keep your post. op pain down and decrease the number of narcotics you need to be comfortable. I would also recommend that you become good friends with an ice pack, especially on the first few days. Ice helped me immensely with the swelling and keeping the pain down post. op.

Versed is everyone’s friend! LOL.

I hope it all goes well for you! You can feel free to message me if you have any questions!