Leigh, Kym, William, Jorryn, and Macyn … and our adventures!

We met with Dr. Kim in the Ear, Nose, and Throat clinic this morning. He says that it’s possible that, because of it’s shape, the tumor in Leigh’s right ear may actually be two tumors.¬† He checked his vocal cords, and once again, they were clear.


He does not think that radiation would benefit Leigh, and that after the tumors in his chest and neck are addressed, surgery would be the best option. He agreed with what Dr. Strome had told us last month regarding radiation; if Leigh were much older, it would be a better option, but because of his age and overall health condition, the side effects and risks of the radiation far outweigh the potential benefits.


He feels, as did Dr. Strome, that the biggest risk with the removal of the tumor in the ear is damage to the nerves controlling the voice box and swallowing reflex. He feels that it is quite likely that, to some degree, there will be damage to these two nerves, which will leave Leigh with a raspy voice and the inability to swallow. He is, however, more optimistic regarding the longevity of these conditions. He says that there is a small implant they can put in the voice box, and with that and physical and/or occupational therapy, his voice should be relatively normal within a few weeks. As far as the swallowing reflex, basically the same thing; he may have a feeding tube for a few weeks, but that normal function should be able to be restored. (Dr. Strome had told us that, more than likely, the feeding tube would be permanent.)


We are still planning on meeting with the radiation oncologist (hopefully this week sometime) to get his/her opinion on the radiation vs. surgery debate.


This is totally random, and you’ll only get it if you’re familiar with central PA!

Going to NIH from my brother and sister-in-law’s house, we travel on Cabin John Parkway. Parts of this road totally remind me of Nyes Road!

That is all! LOL ūüôā

Yesterday was a “light” day at NIH! We went in in the early afternoon, and Leigh had another PET scan, and that was about it!

We spoke with Karen, the Clinical Coordinator, and we have our ENT consult scheduled for sometime next Wednesday morning; we’re still waiting to hear when our thoracic surgeon consult will be.

While they know that Leigh has this genetic mutation, there are two that he could have, and they have not received a definitive answer on which one it is. That being said, I spoke to Karen again yesterday regarding the genetic testing for the girls. Because they don’t know which mutation to test for, they can’t do the testing quite yet. But, we are bringing Jory in tomorrow to have her blood drawn. They will preserve her blood until they have Leigh’s results back and then they will test hers as well. They will not test Macyn until she is 18 months old. If the girls’ results come back positive for the genetic mutation, then they will also undergo scans to see whether or not they have these masses. They do not, however, do the scanning until age 5, so should Jory have the mutation, she will be able to have them whenever we are able to get them schedule; Macyn, regardless of the results of her tests 10 months from now, will not be able to have the scanning done for another 3.5 years after that.

Today’s schedule consists of another CT scan (this time a specialized one of his right ear) and a series of MRIs on his chest, abdomen, and pelvis. Tomorrow is another MRI (not sure what that one is of off the top of my head!) and Jory’s blood tests.

Another Long Day

Yesterday, we arrived at NIH early in the morning once again. First thing, we met with Dr. Chong, the Endocrine Fellow to discuss Leigh’s case. Then, we met with Karen Adams, the clinical coordinator, and Dr. Pacak, the chief of the Pheochromocytoma/Paraganglioma Protocol.


Leigh had his IV put back in, and he had another PET scan in the afternoon. During this scan, they found another tumor, this one in his chest. This tumor is small, and it is “active.” Dr. Pacak informed us that he is confident that this tumor is the one producing the hormones causing the Pheo as well as the other tumors.


We now need to consult with a thoracic surgeon to address the tumor in the chest (the thoracic surgeons only see patients in clinic on Friday, and we are hopeful that we will be seeing them this week). The tumor in the chest will have to be removed first, and then they will address the two tumors in his neck. After those three have been removed, Leigh will most likely undergo radiation treatment to treat the tumor in his ear. We will also be consulting with an ENT here at NIH regarding the ear tumor.


We have an added scan this week, and they have added two more studies that Leigh will undergo next week and the week after.


Dr. Pacak is positive that this is caused by the genetic mutation, and has offered us genetic testing for Jory and Macyn; we are hoping to have this testing for the girls done either this week or next.

We met with Dr. Strome again on January 24th, and that day, we also went through the pre-op physical at University Medical Center. After another long day in Baltimore (this one broken up with a yummy field trip to the Cheesecake Factory overlooking the Inner Harbor!), we came home and began what was our last week of normalcy before the surgery and subsequent recovery.


On Friday, I was to call the hospital to get our check-in time for the following Monday. It was just before this that I received the call from Dr. Wright. The surgery was postponed. If everything went as planned, he would be put on some medication, and the surgery would be rescheduled for Friday, February 11th and the embolization would be the day before.


An¬†endocrinologist had reviewed Leigh’s lab work¬†¬†and was not happy with the results that he saw. The carotid body (paraganglioma) tumors (the ones in his neck) have the potential for secreting enzymes that will raise the blood pressure. This, when combined with the stress on the body caused by the surgery, can cause a hypertensive situation, or basically, his blood pressure could skyrocket, putting him in even more danger than the surgery itself. So, know we’d have to go back for more lab work. I drove to Baltimore to pick up the orders and supplies that afternoon, and Leigh went back the following Monday to have the labs repeated.


On Thursday, February 3rd, I received a call from Dr. Strome and Dr. Wright. The levels they were testing for in his lab work were there and rising, and Dr. Strome did not feel comfortable going forward with this surgery. He had contacted the “world expert” in this condition, who luckily was at NIH (National Institutes of Health) in Bethesda, Maryland.


Now, the second surgery was postponed as well.

On January 10th, we made our first trip to University Medical Center in Baltimore. That day, we were meeting with two surgeons: Dr. Eisenman (read his bio: http://www.umm.edu/doctors/david__eisenman.html) for the skull base tumor (the one behind his right ear) and Dr. Scott Strome (read his bio: http://www.umm.edu/doctors/scott_e_strome.html) for the carotid body (paraganglioma tumors).


After a three-hour-plus wait in the office, we saw Dr. Eisenman, and after our consult with him, we left the exam room confident that he would be successfully able to remove the tumor with little difficulty and that, provided everything went well, the hearing in Leigh’s right ear would be restored.


After meeting with Dr. Eisenman, we went to a neighboring room to meet with Dr. Strome. Yemi, the nurse practitioner came in and set everything up for Leigh’s vocal cords to be scoped, and a little while later, we met with Dr. Strome. All I can say about him is that we absolutely LOVE this doctor!!! He’s got a great bedside manner, and was able to answer all our questions and explain things in terms that we can understand.


This is what he told us: first, these tumors are rare, and it’s even more rare to have them bilaterally (on both sides). They’re typically not cancerous, but they will be biopsied after surgery to confirm that fact. The surgeries are full of dangers, particularly stroke and nerve damage to nearly every moving part from the shoulders up. But, they HAVE to come out; they’re huge as far as these types of tumors are concerned. Dr. Strome said that if Leigh were a 70-year-old man, he would not hesitate to offer radiation therapy to stop the growth, but, unfortunately, that is simply not an option in our case.


We left the office knowing that there would be two surgeries: one to address the carotid body tumor and the skull base tumor on the right side, and then the left side would be addressed about six months after his recovery from the first surgery.


Dr. Strome referred us to Dr. Lal, a vascular surgeon that would be assisting with the surgery. Dr. Lal ordered an MRI series of the abdomen since these tumors typically present themselves in the adrenal glands.


We met with Dr. Lal that Friday, and we were very relieved to find that Leigh did not have any tumors in his abdomen. Aside from the risks that Dr. Strome had noted, Dr. Lal was also confident that the surgeries would be effective. Each surgery would consist of two surgeries: an embolization the day prior to the surgery and the surgery itself. The embolization is a procedure where they will thread a small catheter through a vein in his groin, up to the tumor and place tiny coils in the blood vessels feeding the tumors. These coils will block off the blood vessels and reduce the amount of bleeding during the surgery.


Following our appointment with Dr. Lal, we made our way back to Dr. Strome’s office, and met with Dr. Wright, his resident. The embolization was scheduled for Monday, January 31st, and the surgery would be the following day, February 1st.

High Blood Pressure

In August of last year, Leigh had an outpatient procedure, and after it was over, the doctor called me in to the back and explained that he was concerned about Leigh’s blood pressure. It was REALLY high at this point, but it went down, and the doctor didn’t seem overly alarmed, and let us go, just telling us to keep an eye on it. Neither of us really gave it much thought, and quite honestly, forgot about that episode all together until a few weeks ago.


In September, Leigh had to go to see the State doctor for his DOT physical, which he failed. For high blood pressure. Now, it wasn’t nearly as high as it was the prior month, but high enough to warrant a temporary DOT card and a follow-up visit with our primary care doctor.



From BP to an ENT

In October, actually, it was the day that we were leaving for vacation, Leigh finally went to see our primary care doctor as a follow-up to the DOT physical. Dr. Ukens didn’t find anything of concern with Leigh’s blood pressure at all, told him to monitor it weekly for the next few months, and if there was not problem, he would send him back to the State doctor to have his DOT card reinstated.

It was at this appointment that Leigh brought up the issues he had begun to experience with his right ear. Dr. Ukens looked at it and said that he had a red spot, but that it wasn’t infected, or anything else that would warrant the typical antibiotic and ear drops. He told him to follow-up with an Ear, Nose, and Throat doctor if the symptoms didn’t go away or get worse.


We went on vacation, and had a great time visiting with family in North Carolina and Georgia! When we came back, life as usual continued. Two or three days after our return, Leigh called me about two o’clock in the afternoon to tell me that he was going to the doctors. Now, this is alarming because 1. he N.E.V.E.R. makes his own appointments, and 2. he went straight to the ENT and didn’t bother going back to Dr. Ukens. He said that the ringing and pulsating in his ear was just too much to bear.


The ENT took a look at everything and said that he had a sinus infection and an ear infection. He sent him on his way with three weeks worth of antibiotics, and an appointment a month away. Dr. Patterson said that his ear was red, and that he had a lot of fluid in there, but that he also saw “something” behind his ear drum, something that he believed was a “mass of veins.” This day, they also preformed a hearing test, and Leigh had moderate hearing loss in the right ear.


A month came and went, and Leigh was still experiencing the pressure, ringing, and pulsations, so it was back to the office to see Dr. Patterson. The infections were completely cleared up, but he still had quite a bit of fluid. They “poked” his ear to relieve the fluid build-up. During this visit, he still saw something behind the ear drum, so Leigh was sent for a CT scan of his head.


After the CT scan, we went back to see Dr. Peterson a third time, results of the CT in hand, and we were informed that “there is definitely something there.” We were given a referral to surgeon in the same practice for a consult to have whatever this was removed.


After this appointment, we were contacted by the ENT office to have a series of MRIs completed prior to our appointment with Dr. Marcus. This proved to be more difficult than it sounds, and after a series of events that occurred as a result of these scans, I’m pretty sure we will never see Dr. Patterson again.


Long story short, Leigh had the scans done, and we went to see Dr. Marcus. Because his appointment was not long (hours) after his scans, the doctor only had the images to look at and not the radiology reports. He confirmed that there was something there, and left us with two scenarios: 1. it was a simple mass that could be easily removed in an operating room at Anne Arundel Medical Center, or 2. it was a complex mass, one that would require a referral to a surgeon that specializes in these more complex tumors.


A few days later, we received the call. Not only did Leigh have the tumor behind his ear (which is responsible for the hearing loss, ringing, pulsating, and pressure), but he also had two tumors in his neck, one on either side, and we were being referred to two surgeons at University Medical Center.

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