Baroreflex Failure / Baroreceptor Failure

My husband, Dale, is 65 years old. He had 10 point radiation treatment 10 years ago when he had throat cancer in 2006/2007. About 1 1/2 years ago he began developing two medical problems. First it was Dysphagia and second was Baroreceptor failure - or Baroreflex failure. I also believe the surgery he had 1 1/2 years ago to have the spurs removed from his upper cervical vertebrae, (C2), may have set off the Baroreflex failure earlier than it would have occurred if he had not had the neck surgery. Surgery on the upper vertebrae can cause BRF.

This is pretty long and most people won't want to read it all so please, if you're not going to read through all of this, at least skim down to where I have made a note saying, "IMPORTANT INFORMATION" and read some obscure information I learned during my research on BRF!

We began dealing with Dale's dysphagia about a year ago. Dysphagia is: "having trouble swallowing. Radiation therapy can cause permanent tissue scarring, (in Dale's case the scarring is in the back of his esophagus). The muscles in your tongue and the back of your throat may not be able to move as well. This can also effect the epiglottis in that it will no longer close completely. When you’re having trouble swallowing, food or liquid can pool in the back of your throat. You can then aspirate your food, which means it goes into your airway instead of your esophagus." So when Dale eats or drinks anything he chokes/coughs A LOT! Rather than go through all that coughing, especially while he is in public or while he's at work - he chose to have a feeding tube put in. Also, before the doctors realized that he had "scar tissue" in the back of his throat and NOT a narrowing of the throat, he went through 4 procedures to stretch his throat out - which didn't help and probably did more harm than good. You see, if you have developed this problem with your throat because of radiation treatment, every time you go through intubation it causes more damage to your throat, etc. He has also gone through 30 days of Hypobaric Chamber treatments - 2 hours at a time. That didn't help either. Now he has just accepted the fact that he will have this problem the rest of his life and he will live with a feeding tube, eating solid foods now and then, and coughing a lot to keep healthy.

His second medical problem, Baroreflex Failure (BRF), is characterized by episodes of severe hypertension and tachycardia, alternating with episodes of normal or even very low blood pressure and bradycardia. It is often caused by interruption of the baroreflex arch as a result of bilateral damage to the glossopharyngeal and vagus nerves from trauma, radiation, or surgery. Less often, the lesion is in the brain stem, and in some cases, the primary cause is not found." Munchausen's Syndrome Presenting as Baroreflex Failure  (The New England Journel of Medicine)…Google search. http://www.nejm.org/doi/full/10.1056/NEJM200008243430814#t=article

IMPORTANT INFORMATION: There are videos on YouTube that you can watch that show the "workings" of the Baroreflex system. It will show you that the AORTA is DIRECTLY involved with the workings of the Baroreflex action!!!! It is very important that you mention this to your doctor and ask for tests to be done on your heart to make sure you have a regular heart beat!!!

Dale had already been working with a Cardiologist on controlling his extreme high BP by using Clonidine. The Cardiologist never did ANY test on his heart! This Cardiologist did not know ANYTHING about BRF so WE did not know anything about BRF. We learned by trial and error how to manage the extreme high and low BPs. Sometimes that just meant when Dale had the kind of high BP that made him vomit uncontrollably, sweat, dizzy (vertigo), etc.  we went to the emergency room. We averaged about 2 visits a month to the ER for high BP that we couldn't get down. We eventually reduced the Clonidine to 0.1mg and that helped his BP to "not" drop so low. He drank salt water to bring up the low BP. But what really helped was drinking A LOT of WATER!!  Stay hydrated!! That is hard for him because of his dysphagia!

Then about 8 months ago we began looking for answers as to why he had started to lose control of his right hand. It would all of a sudden start doing things all by itself. It would move in odd ways, become weak, and then useless for about 10 minutes or so. It quickly moved into his right leg doing the same thing as his right arm. Over about 4 months it became more extreme, more often, and lasted longer. He began having bouts of confusion, severe changes in his eyesight, and more extreme ups and downs of BP. He had become somewhat belligerent, lost some of his cognitive abilities as well as intellectual abilities such as spelling, and when I laid my head on his chest I could hear he had a very erratic heartbeat, etc., He "FINALLY" mentioned some of this to his PCP. His PCP sent him to a new cardiologist at Vanderbilt Med. Center in Nashville, TN. Vanderbilt sent him to the new Cardiologist, a Neurologist, and the Autonomic Center that specializes in BRF. The Neurologist was VERY concerned and told us Dale was probably having a type of TIA when he would lose the control of his right side for long periods of time accompanied with the confusion, etc. She told him to go to the ER immediately the next time it happened for more than 15 minutes and have them bring him to Vanderbilt. She ordered the following tests: MRI brain; MR angiography neck; MR angiography head. After the results of those test came back she ordered a CT HEAD, CTA HEAD AND NECK; and a special EKG - which we will have done on Jan. 12, 2017.

It took sometime but he finally got into the Autonomic Clinic. The doctor gave him instructions on the best way to control his BP in regards to the BRF such as to stay hydrated, to eat a little more salt to get his BP up (54/43), to wear pressure stockings when his BP went low, to lay with his head lower than his feet when BP was low. We had already raised the head of our bed 8 inches higher to keep his BP from dropping so Dale would just turn around during the night if his BP went low. The doctor gave him medication to lower his BP when high and raise it when low (the medication for high BP was too extreme in the drops so we went back to 0.1 Clonidine). The Autonomic Dr. ordered 2 Autonomic Function Tests (AFT). What I learned during this visit was that my research on his symptoms, "including loss of control of his right side", actually led me to the BRF information so we were already doing most of what this doctor told us to do! BUT I had done my homework and had the facts in hand so I was ready to "ask" if BRF might be the problem when we first started PUSHING to see the right doctors.

A lot of the results of the test results from the Neurologists tests were bad but some were good. In a nut shell Dale's test showed:

1. Dale's left cervical ICA (internal Carotid Artery) is completely occluded (completely blocked) throughout its course.

IMPORTANT INFORMATION: We were surprised to see he had blocked arteries because he had/has very low Cholesterol. My research on BRF showed that, over time, the ICA can become blocked with scar tissue - become stiffened - because of the radiation therapy. This means the arteries are NOT blocked with plaque!! Once an artery is 100% blocked there is nothing that can be done about the blockage - SO - stay on top of having your arteries checked!

2. HISTORY: G45.1 - Carotid artery syndrome (hemispheric)… (Hemispheric syndrome is otherwise known as anterior circulation syndrome. It is the result of a large vessel stroke, and occurs when there is proximal occlusion of the int carotid artery, middle and/or anterior cerebral arteries supplying the cerebral hemispheres.) This meant he had already had a stroke sometime in the past.

3. There is unchanged old left lacunar infarct. (Lacunar infarct is a type of stroke that occurs when one of the arteries supplying blood to the brain gets blocked. These arteries are quite small, which makes them vulnerable to damage. While most arteries in the body gradually become smaller, the arteries of the lacunar stroke branch off a large high-pressure artery.) (Symptoms of lacunar infarct. It’s important to recognize the symptoms of lacunar stroke so that you can receive medical attention right away. Symptoms of a lacunar stroke are similar to those accompanying other stroke types, namely, slurred speech, inability to raise arms, one side of the face appearing droopy, numbness on one side of the body, difficulty walking or moving arms, confusion, memory problems, struggling to speak or understand language, headache, and loss of consciousness.)

The CT HEAD, CTA HEAD AND NECK showed:

4. There is moderate atherosclerosis of the RIGHT proximal ICA (internal Carotid Artery) causing mild narrowing.

CT angiography head showed:

5. The intracranial left ICA is occluded from the petrous segment through the mid cavernous segment.

6. There is reconstitution of flow within the left cavernous, clinoid, terminal ICA, likely from retrograde flow from the contralateral side.

7. There is mild narrowing of the terminal ICA.

8. There is opacification of the left MCA and ACA secondary to retrograde flow from the contralateral side. No stenosis or occlusion of the ACAs, MCAs, or PCAs.

9.There is scattered atherosclerosis throughout the intracranial arterial vasculature.

What I am going to ask from Jim's doctors!

1. I can hear Jim's heart beat when I lay my head on his chest. It is noticeably abnormal - even more abnormal that it was a few years ago. It is especially abnormal after he has been active. I would like to request that he be given a stress test.

2. I would like him to have a Cortaid ultrasound: that would show the flow past the blockage as well as the pressure throughout.

3. I would like him to wear a 24 hour halter monitor: for irregular heart (a continuous EKG)? Does he need to be on blood thinners.

Repeat of IMPORTANT INFORMATION:

1. My research on BRF stated that, over time, the ICA can become blocked with scar tissue - become stiffened - because of the radiation therapy. This means the arteries are NOT blocked with plaque!! They are blocked with scar tissue. Once an artery is 100% blocked with plaque there is nothing that can be done about the blockage! I'm not sure how scar tissue blockage differs from plaque blockage. SO, to be safe, stay on top of having your arteries checked!

2. There are videos on youtube that you can watch that show the "workings" of the baroreflex system. It will show you that the AORTA is DIRECTLY involved with the workings of the baroreflex action!!!! It is very important that you mention this to your doctor and ask for tests to be done on your heart to make sure you have a regular heart beat!!!

Thank you for reading about our long journey of discovery about what the long term effects of radiation therapy can be. It's difficult to find detailed information about Baroreflex Failure without researching many articles, websites, forums, etc. I hope this helps to answer some unanswered questions.

Now that you are in the UK, and if you are able to get to London, my advice would be to contact Dr Mel Lobo at Barts Hospital and ask if he will see you. If he will then your GP would need to refer you. If you are not near London, he may know of a baroreflex specialist near you. Let me know how you get on.