First off, when I had a chest x-ray before one of my surgeries they noted a “sudden change in course of the port tubing possibly as it enters the right internal jugular vein, raising the possibility of kinking". Now my port is still working just fine, but since the port leads directly into my heart any irregularities or concerns need to be treated with urgency. We don't think anything is wrong and it probably just a benign cyst, but we will still check it out. I met with a new general surgeon here in Cincinnati since the doctor that put it in is back in Illinois where we used to live. She again indicated that since the port was working well that she did not see any reason to replace the port, which would be the only course of action to remedy the kinking. If it stops working because the kinking gets worse only then would we need to replace the port. She did take some x-rays because she wanted a different view than the ones that were done before my surgery, but unfortunately my collar bone was in the way of the cyst and kinking in the tube that she wanted to see, so we were not able to get the view that she wanted to see. For now we are just going to leave everything be and hope and pray that my port continues to keep working!
Next, I had had a biopsy of my stomach taken when I had a Botox injection done at the beginning of May. This biopsy was to check for something called mast cells. Mast cells are the cells in your body that respond to allergens. Newer research is showing that mast cells play a role in wound healing and regulating the immune system, but these functions are still not well understood. There is a disorder that is a common comorbid condition with EDS called Mast Cell Activation Syndrome (MCAS). The mast cells in people who have this disorder are not too numerous, rather they are overactive. They respond to things they shouldn’t and respond too severely to things that they should respond too. It is a very complicated disorder that is not well understood. MCAS can cause GI symptoms, flushing, headaches, rashes, unexplained anaphylaxis (severe, life threatening allergic reaction), and breathing related issues, among other symptoms. I also had a blood test and did a 24-hour urine connection to check for markers of MCAS. The blood test almost always comes back normal unless a person is having an MCAS reaction at that exact moment the blood is drawn, so we were not surprised that this came back normal for me as well! The 24-hour urine collection also came back as normal, which was a surprise! The biopsies came back with mild-moderate mast cell reaction in my stomach and small intestine. At this point we are not treating the mast cells in any way and they think I have a very mild case of MCAS. The reactions could worsen and I get older and I have had some clinical signs of MCAS in the past such as unexplained anaphylaxis and unexplained rashes/reactions, but for now I am just happy that I do not have one more thing to add to my list!
Now both of those things were very positive updates, but unfortunate I also have a few not so good updates. I have been doing physical therapy for my hip after my hip surgery back in May. My physical therapist is quite pleased with the strength of my muscles and the range of motion of the joint, but unfortunately I am still having pain within the joint. I have pain walking and doing stairs. The physical therapist has tested some different exercises and movements to see if she can pinpoint why the hip is hurting and based on those results she really believes that it is coming from deep within the joint. The surgery I had was very minor and was to repair the torn cartilage in the joint. We did not address the anteversion (turning inward of the hip) because that is a much more invasive surgery and we were not sure if it was necessary, so I am really hoping that is not the problem now :( I was not set to see the orthopedic surgeon until August 24th, but my physical therapist wanted me to try to get in with him sooner. I called today and was able to get an appointment with him next Wednesday, August 3rd. I hope he is able to give some insight into why my hip is still hurting and help us to figure out what we can do about it!
The other issue I have been having is severe headaches for a
few months now. I have not had any
headaches since my craniocervical fusion 2.5 years ago. The headaches from my Chiari
and craniocervical instability were always in the back of my head and down into
my neck, but these headaches are on the top of my head. It feels like a ton of
pressure and my head is just going to explode! I brought this up to my primary
care doctor who also happens to be a headaches specialist. He was concerned
that my intracranial pressures are high. Since these headaches are a new occurrence
he thinks that my increased pressures are due to scar tissue build up. I have
quite a bit of scar tissue in that area from two spinal/brain surgeries that
went through the same incision. He ordered a MRI that looks specifically at the
CSF (Cerebrospinal fluid), which is the fluid that flows constantly around your
brain and spinal cord. When its flow is blocked, for any reason, it can raise
the pressure within your skull called the intracranial pressure. The MRI was
called a CINE MRI. The results came back and it shows that my CSF flow is
blocked in both the front and back of my craniocervical junction (where the
head and neck meet). I sent the disk to my neurosurgeon in New York and he is having
some technical issues trying to view the disk, but hopefully he will be able to
get back to me soon with his thoughts on the images and how he thinks we should
proceed.
I think that is all for now! Please pray for my hip and my
headaches and that the doctors know how best to help me! Sorry for the long delay
between updates! I am almost done with my summer classes and I get about 2
weeks off, so I am looking forward to a much needed break!!!
