10 minute read
ADVOCATING FOR YOUR CHILD
This summer we received a copy of the following letter from a parent who explained that they had sent it to the administration and surgery clinic at the hospital where their daughter had been seen. They continued, “If it will save anyone from going through the same or a similar thing, we would like it to be published.” We are sharing it in the hope that you will find it helpful.
July 8, 2023
M. has asked us as her parents to write this letter not only to support her, but also in hopes that no other patient will have to endure what she has gone through the past couple of years following complications of her central vascular line (CVL) placement. M. has a rare form of intestinal failure called neuropathic chronic intestinal pseudo-obstruction, diagnosed at approximately four months of age. She requires parenteral nutrition (PN) to maintain nutrition and thus her life.
M.’s first central line was placed in her right internal jugular in September 2007, while she was a patient in the NICU. The external stay suture was removed while she was still in the NICU. This line lasted until April 2009 when it fractured over the clavicle due to her growth.
In April 2009, a new CVL was placed in the left subclavian. Again, the external stay suture was appropriately removed within the first two months after placement, at a clinic visit. Amazingly, despite several repairs, M. kept this line until February 2014, when it fractured near the exit site. It was replaced, again in the left subclavian. The right subclavian was attempted but could not be used at that time. And again, the external stay suture was removed during a subsequent clinic visit, within the first few weeks to months following placement.
M. was pre-pubescent, having no breast tissue, at the time of this surgery. It certainly could have been anticipated that she would continue to grow as she previously had and that she keeps a central line on average of three to five years. And we know, based on her history, that she will likely need a line the rest of her life. To our recollection no follow-up appointment was made following this line placement. For some reason, the external stay suture was left in.
As M. continued to grow, beginning around June 2020, granulation tissue began to grow at the exit site. The site was red and sore and M. had mild pain, which she dealt with on her own as she was told this was “normal” and there wasn’t anything that could be done. The suture could have easily been removed at this time. In October 2021, the pain, discharge, and redness became so bad that we went to the ER with concern of a tunnel infection after speaking with the GI team that managed M.’s care.
Blood cultures were drawn, and we were told there was nothing that could be done other than taking ibuprofen or Tylenol. M. is unable to take ibuprofen due to GI bleeding and we try to avoid Tylenol because she has some PN-associated liver issues. M. was told that she should not be experiencing any pain because sutures, central lines, and granulation tissue “do not cause pain.”
M. was able to keep this line for approximately five years, which we are told is unheard of and is a testament to the excellent home care she has received. Maintaining central line access is critical to M.’s survival. She has only had one line infection, and it was successfully treated while preserving her central line (it did not have to be pulled). This line began leaking internally in August 2019 and required replacement on her twelfth birthday. The new line was placed in the right subclavian.
We were scheduled to go to the surgery clinic in October. There, we asked that the suture be removed because it seemed to us to be the main cause of the granulation tissue. We were told that the suture could not be removed because it “was holding the line in.” We had never been told this before and assumed that something had changed or was different with this line than every other line she had previously had. In other words, we trusted and believed what we were told.
Again, we were told there was nothing that could be done and that she should not be in pain from her line, the sutures or the granulation tissue. The suture could have been removed at this time.
As M. continued to grow (thanks to several of her doctors) in height and began to develop breast tissue, her skin and the granulation tissue grew over these sutures and her discomfort and pain, the oozing, redness, etc. continued to worsen. Every time we would do a dressing change, it would cause her immense pain to move or even touch the line. It was difficult to keep a dressing on for more than a couple of days. This is a child who has dealt with intestinal and abdominal pain her entire life.
Every time M. rode her horse, she would complain that her right shoulder hurt. This got worse and worse until January of 2023, when we decided that we could no longer continue to “just deal with it.” We saw the same doctor in January that we had seen previously, and her comment NOW was that the suture “wasn’t doing anything but causing the granulation tissue to grow.” We asked ourselves, what?
At this point the suture was underneath skin and granulation tissue and was not accessible until the site had been treated with silver nitrate, which was done that day in clinic. This was very painful due to needing to move the line around to get underneath and all around it. M. was in tears and extreme pain, and was highly anxious. We continued to painfully treat the granulation tissue at home on January 11, 18, 23 and 29, and February 5, 13 and 19. Each treatment was painful and her chest, arm and shoulder were uncomfortable and painful. It reached the point where she missed school because she could not comfortably “move her arm.” It was torture. After treating it with silver nitrate, the granulation tissue would grow back within hours to days.
On February 22, 2023, they attempted to remove the suture in clinic. M. was in so much pain and crying that there was absolutely no way the removal could be done. On March 7, 2023, we were forced to visit the ER again because M. woke in so much pain that she could not move her arm. Again, she missed school and both of us missed work. Once again, we were told there was nothing that could be done to help her. So, we sought a second opinion. After a long consultation and the doctor saying, “I do not want to hurt you,” a decision was made to put M. under general anesthesia to remove this suture in an attempt to save the line. This procedure was done on April 13. This was the first opening in the OR. She was very sore and uncomfortable for about a week following and some of the pain had subsided, but it was not completely resolved.
Within ten days, we saw another piece of blue suture poking through the newly grown granulation tissue. After this we spoke with the doctor again. It was decided to attempt to remove the remaining suture by placing M. under anesthesia again on June 15. Again, we had to wait for an opening in the OR. The doctor’s comment in the post-op consultation room was something like, “I don’t know who put that line in, but I would have never used that much suture or left it in like that.” She commented that it was so tight that it was “strangling the line.” We can still see an indentation in the line.
The very next day M. could tell that she was better. The doctor was so concerned that M.’s pain would be much more intense following the second procedure because she had had to dig so much further up the line into M.’s skin to retrieve the suture. She had given M. oxycodone and had to put several dissolvable sutures in to hold the dug-out skin together. However, M. did not require any pain medication whatsoever. She was up and moving around and moving her arm more freely than she had in two years.
It is painfully obvious that the suture was causing the issue all along, just as we suspected. For two years this child has been gaslit by being told that granulation tissue does not hurt, and sutures do not hurt. We have the sutures in a specimen jar, and they are hard as a rock and stiff like barbed wire. There is no doubt in anyone’s mind that this was causing the problem. M. has had a central line her entire life and never had any that were painful until this one.
We were able to attend the Oley Foundation conference in St. Louis, Missouri, June 27–30, 2023. This is a conference for patients, caregivers, clinicians and suppliers of patients on enteral and parenteral nutrition. At this meeting, Dr. David Mercer spoke on central line access and complications. Dr. Mercer is a multivisceral transplant surgeon at Nebraska Medical Center. He has done many intestinal transplants. However, one of his main goals is to keep patients like M. from needing an intestinal transplant by maintaining CVL access and limiting complications from PN (liver failure and sepsis).
During the Q&A period, I asked Dr. Mercer how long the external “stay” suture should remain in following CVL placement. His response was that it could be removed once the cuff has grown in approximately three to six weeks to be safe, but one week would probably be ok. The internal cuff is what “holds the line in,” not the external suture.
If you would like to view the video of his presentation, it is available on the Oley Foundation YouTube channel (search “The Patient’s Voice in Vascular Access” or go to youtube.com/video/euKmAeWUHZ0). His answer to my question is at approximately 42 minutes. Consider watching the entire video as you will learn so much from the patient perspective and from Dr. Mercer’s experience and expertise. As the GI clinic ramps up, you are likely to save the lives of many more PN-dependent kids like M. in the years to come.
It is our plea and hope that in the future, when you are caring for a patient that requires long-term vascular access through a central line, you will change your post-op protocol such that the child has a post-op appointment within six weeks where the suture can be removed before the child grows skin and breast tissue over it or before it becomes so aggravating to the skin that granulation tissue takes over.
This has caused M. to miss school, miss social events, miss horse shows and practice; it has caused her a significant amount of pain and added anxiety to her already extremely difficult health situation. She has had to be placed under anesthesia twice, which is terrible for her GI motility, and has cost our insurance thousands of dollars. As her parents, we have missed numerous days of productivity at our jobs, costing us financially. More so, it has cost us countless sleepless nights and parental guilt for not advocating for her as much as we should have or could have.
Thank you for the care you have provided to our child and our family over the past fifteen years.
Most sincerely,
M.’s Parents
