Remission Five Years and Counting

I had my annual check-up earlier this month at M.D. Anderson Cancer Center. Five years ago today I was in the operating room at UCLA Medical Center having my tumor removed. Right now my health is excellent. The residual tumor on my carotid artery is still stable and not actively excreting any excess hormones. Also, my pituitary is functioning as it should. I am relieved by the good news!

While I was in Houston I met a fellow acromegalic for the first time. Marisol is a member of the Acromegaly Community and she had surgery in September 2009 to remove her tumor which was followed by radiation. She contacted me through my blog last year and we have kept in touch ever since. It was so neat to meet her in person and I really enjoyed our visit. The most wonderful news is that she is pregnant again and her baby is due in December!

The only thing pending at the moment is I am scheduled for a repeat CT scan in September to observe the condition of my sphenoid sinus which has given me trouble off and on these past few years. However, I can hardly complain because it's a drop in the bucket compared to the health problems I could have incurred from this whole ordeal. Everything looks so good that I have been told by my endocrinologist that I do not have to return for an MRI or labwork for 18 months. I am still waitng to hear from my neurosurgeon, but I suspect he will agree will the findings.

It's (not so) Complicated

When I tell someone that I've had a brain tumor removed they automatically think I've had a craniotomy. I know this because their eyes usually begin to skim the top of my head as if they're wondering where the entry point was when the tumor was removed. I mention the tumor was taken out my nose and they're surprised that can even be done. Somehow, it never seems like I've said enough about my experience, but any detailed discussion about it would be lengthy and probably more information than they want to know...so I hold back. I usually clarify the tumor was benign and removed from my pituitary, but that's it. When I told my brother about my tumor and how it would be removed he asked, "Oh, so...is it kinda like that scene in the movie Total Recall when Arnold Schwarzenegger pulls something out of his nose?" Well...almost, but not quite.

I received the news about my pituitary tumor in the spring of 2005. The phone conversation with my doctor was brief. I listened intently as he explained my MRI results and when I heard the words "referral to a neurosugeon" I realized I was in a serious situation. It was as if I had received a death sentence even though the doctor assured me I would be fine. Shortly after this I met with a local neurosurgeon who attempted to educate me in 20 minutes on the structure of my brain and what a tumor on my pituitary meant. The appointment eased my fears, but I still had so many questions. Unfortunately, many of these questions would have to wait until it was revealed what kind of tumor I had and whether or not my pituitary was functioning properly.

For days after my visit, I scoured the Net and searched for every piece of information I could get my hands on. At the time, personal stories were scarce but I read about the brain anatomy, pituitary function, different kinds of tumors, lab work, surgeries, hormone replacement, what to look for in an experienced surgeon, and the list goes on. Even with all the information I was getting, it seemed so complicated...I had information overload. It was difficult to sift through and absorb everything I was reading, especially since I knew little about the pituitary function to begin with. I realize that this is the case with many people. I've tried to explain my situation to those that ask about the tumor. Most of the them are not aware of the entire brain anatomy and that there are different kinds of pituitary tumors that affect it. I think it's in the "need to know when it happens to me or someone I love" categories of life, or at least it was for me before my diagnosis.

The pituitary is the master endocrine gland and it affects (either directly or indirectly) all the major organs in your body. Having a tumor wrapped around it is a big deal. There are many support groups being formed online to help people who are struggling. One group in particular that is gaining attention is http://www.acromegalycommunity.com/. If you have recently been diagnosed with acromegaly I would encourage you to sign up. There are many people sharing their stories on the message boards, and they may be able to help you with any struggles you may have in understanding complications that come about with having a tumor on your pituitary.

Sinus Update - Three Years Later

As many of you have read already, immediately after my surgery I developed sphenoid sinusitis. This went on for a several months after my surgery and it was discovered that the titanium mesh put in my head dislodged from its original placement. It was suspected that this was attributing to my problem. In 2007 it was decided that I would undergo a second surgery and have the mesh removed. However, I found out I was pregnant while I was on the operating table and the surgery was canceled.

Early last summer I began having headache, ear pain, and pressure behind the eyes. I was treated by my general doctor for sinusitis and returned to my ENT in January to discuss symptoms that resurfaced again. After treating me with another round of antibiotics for sinus infection, he ordered a CT scan (because symptoms did not go entirely away) which revealed mucosal thickening >8mm in the sphenoid sinus. My ENT thinks my symptoms and the scan results may suggest sphenoid sinusitis though the CT scan is not overwhelmingly convincing. What is seen could be infection, but it is hard to make out if the post operative changes are scarring or inflammation. There is a slight change in the right sphenoid from prior studies that is suspicious and so in this situation the doctor said it is prudent to prescribe an extended course of antibiotics and see if the symptoms go away (as they have in the past). I will be rescanned in a few months to see if there is any change in my sinus. If there is evidence of more thickening, then removing the mesh might be the right course of action to take.

For now, I have put off pursuing surgery. Someone might ask, "Why wait? You were going to have the surgery before...why not just go get it over with?" Well, here's my reasons: 1) According to my ENT there is not preponderant evidence that the surgery is needed. Menningitis is a risk with sphenoid sinustis, but the doctor does not see an imminent risk on my CT scan and this is not likely to happen if I'm on medication. 2) It's a risky surgery. My ENT said it would be like having pituitary surgery all over again. Staying close to home would be most convenient, but not possible. If I stayed in Dallas the operation would involve a team of doctors who are not experienced in removing titanium mesh from the sphenoid/sella. I would be better off returning to my neurosurgeon in California. 3) A family of six traveling from Texas to California is costly and not easy. 3) Lack of time (I know - I could make time...) 4) Honestly, I'm scared to go back in surgery.

For more information on the history of my pituitary tumor: Click here.

It's Just in Your Head

Here's a recap from a previous post on what some of my symptoms were before my brain (pituitary) tumor was discovered and the difficulty I had getting a doctor to look for root cause of my problem(s):

"...I began having severe headaches shortly after we moved from California to Texas in 1999. I had no history of headaches, so a CT scan was ordered by my general physician. It came back 'normal.' At this point, my doctor assumed, and so did we, that the headaches were allergy related because this all started after our move (the area is awful for allergies). I never vigorously pursued another answer for the headaches after that, although I mentioned it whenever I went to the doctor. I frequently took naproxen sodium whenever I had a headache, which helped me relax and drift off to sleep.

In addition to my other symptoms, I developed chronic (left) ear pain. For almost two years, I visited several doctors, including two ENTs and a TMJ physician, to find the cause. The TMJ physician said I didn't have TMJ (took x-rays, etc. to rule it out), but the ENT (who referred me to the TMJ doc) insisted I had the problem. I asked if he could order an x-ray or something to look deeper in my ear, but he felt there was no justification because I had a "classic case" of TMJ. He didn't think it was necessary to look any further until I went back to the TMJ doctor for more testing. Finally, I decided to get yet another opinion from a third ENT..."

What I didn't expound upon in this earlier blog post is what transpired with the second ENT, Dr. B, before getting a third opinion. After being told by Dr. B that there was "no justification" for an x-ray or MRI, he told me not to go to another doctor because he would only tell me what I wanted to hear. Dr. B knew I had gone to several doctors before him, and I had been diagnosed with all sorts of things, including an arthritic jaw, ear travelers syndrome, otitis media, and TMJ. It was becoming obvious I was annoying the doctor with my ongoing concerns and acting a little crazy because I had seen so many doctors. He mentioned his TMJ patients were his most difficult because they never think their problem is what he tells them. He insisted I go back to the TMJ doctor and have him take another look before anything else would be done. What was frustrating was I went to the TMJ doctor on the top of the list Dr. B gave me because his opinion was held in high regard. When the TMJ doctor said I didn't have TMJ, Dr. B insisted I return for the diagnosis. There was no other possible explanation for my symptoms.

I was discouraged and embarrassed by the ENT's remarks so I waited about seven months before I dared to make another appointment with the doctor. The headaches and ear pain persisted, so I made the return appointment with the TMJ doctor. Fortunate for me, the day I had my appointment, the doctor had a stroke. I was walking out the door to the doctor's office when the phone rang, and his receptionist told me my appointment was canceled until further notice. At this point, if I were to return to Dr. B, it would mean I had to seek another TMJ's opinion before returning. Reluctantly, I decided to get a third ENT opinion instead. He differed in thought about the TMJ diagnosis because the specialist had ruled it out. He didn't see my need to go back for a second work-up. His conclusion was we had to think worst a case scenario - that I might have a tumor causing the symptoms. He ordered the MRI that revealed a 2.5cm tumor under my brain, wrapped around my pituitary and growing into the left cavernous sinus.

The doctor called me less than 24 hours after the MRI was done and told me about the brain tumor. After our discussion, I instant messaged my husband at work with the news. He was joking with me at first because he had been teasing me that it was all in my head. How could so many doctors not know the cause of my problem? Our conversation went something like this:

Me: Just got off the phone with the doctor about my MRI

Dave: Oh, what did he say? You have a brain tumor? LOL

Me: Yes...

Dave: LOL

Me (crying while typing): I have a brain tumor.

Dave: No, seriously - what did he say?

Me: I'm not kidding...he says I have a brain tumor.

Dave: ??

At this point Dave picked up the phone and called me. His boss was very understanding and sent him home to be with me the rest of the day.

Dr. B's remark about my numerous doctor visits, "...they'll just tell you what you want to hear," was not valid. I wasn't given definitive reasons for the persistent headaches and ear pain - just theories. For example, I was told by the first ENT that "chewing gum causes headaches." He diagnosed me with an arthritic jaw and then handed me a powerful prescription (I never filled) for pain medicine (later taken off the shelf by the FDA) to cope. If it worked, we knew that was the problem. Explanations like this made me pause and think about getting different opinions. I'm sure there are hypochondriacs doctors encounter, but my common symptoms were related to an uncommon disease. I happened to be that freak rare statistic patient that no one expected. Seriously, I would've preferred a better explanation for my symptoms, but everything made sense the day I learned about the tumor. I felt vindicated because it was revealed I wasn't crazy after all.

Endo Update

The drive was easy, the weather was pleasant, and I visited with some friendly folks while I was at M.D. Anderson Cancer Center. It is a nice hospital, and the staff is helpful and accommodating. It was, however, rather somber because it was evident that people were fighting for their lives everywhere I looked. Other patients I met traveled from out of state to see a doctor about their problems. I didn't feel like I belonged there and kept thinking I was not that sick. Even still, I had a good experience, and I am glad I moved my follow-up care there.

First, here's some pictures from my trip to Houston:

It was icing over in Dallas when I left...

but it looked like spring in South Texas!

Here were some of my questions for the endocrinologist and the answers I received:

Q. Is there any sign of pituitary failure or hypothyroidism? No, my thyroid is functioning well - all the labs came back normal.

Q. I was not able to nurse my daughter beyond 7 months postpartum because my milk production was so low. Why did this happen? He really didn't know - there is no evidence that suggests an existing pituitary condition caused this. Sometimes it just happens.

One of the reasons I went to M.D. Anderson looking for a new doctor is because my endocrinologist in Dallas said that a normal IGF-1 means there is no tumor. Therefore a patient doesn't need to have the Growth Hormone Stimulation Test. He believes a normal IGF-1 is sufficient to determine whether a patient is in remission from acromegaly. While I understood what he was saying it was a red flag to me because I had a normal IGF-1 with an abnormal GH test before surgery. My new endo says I have residual tumor, and he spent a lot of time explaining what he thought was going on. When I saw him last month, I didn't have my past MRI scans or records. I mailed them after my appointment and have been waiting for his response to my other questions:

Q. During my appointment it was mentioned that you think I have residual tumor. Now that you have reviewed my medical history and MRI scans do you still have this opinion? The doctor suspects there is residual tumor in the left cavernous sinus and that it is non-functioning or making GH to such a small degree that it's clinically irrelevant.

Q. Is a nonfunctioning/functioning tumor "better" than a full blown GH or prolactin tumor? Is it less agressive because it is only partly functioning? Nonfunctioning tumors can be aggressive in terms of growth (hard to predict) but my tumor is not clearly overproducing GH to warrant medical therapy. The plan will be to carefully monitor the residual tumor's growth, and if that happens, we can consider radiation.

The doctor believes there is evidence of residual on the MRI scan even though it is not seen in the blood work. I am over the shock of hearing that I have tumor, so I was not upset when he told me this. He says my tumor is partially functioning, which means it was not consistently producing excess GH or prolactin presurgery. He believes there is evidence of this in the tumor pathology and that I had a normal IGF-1 lab before the macroadenoma was removed. He thinks the residual left is not functioning right now, which is why my blood work is normal. The tumor could start functioning again, but right now it isn't. It's unusual for a person to have a partially functioning tumor. Most people have one or the other. What he said makes sense, and it explains why my IGF-1 levels were mild (barely elevated). He mentioned residual tumors could cause insulin resistance, but there's no evidence in the blood work to support this. Diet is the only way to control the episodes - eat small meals throughout the day and watch my carbs intake.

Since the remaining tumor in the left cavernous sinus is inoperable, the only options are to treat it with medicine or proceed with radiation. There is no excess GH or prolactin hormone to suppress, and radiation treatment could damage my pituitary gland so the doctor recommends we watch it. There is no need to do anything right now. As crazy as it seems that I can safely live with tumor on my carotid artery, he assured me it is okay. It's growing slowly, and he does not think it will erode the artery or invade my brain. At this time, it looks like I'll be going to Houston for any future check-ups. I'll return to M.D. Anderson in 6 months for a repeat MRI and bloodwork.

Peace of Mind Granted

My recent hormone levels indicate the acromegaly is still in remission and I can relax. I don't have the details right now but I've been assured by neurosurgeon that everything is good and my GH suppressed. I'll post the hormone numbers later. My IGF-1 level is still pending but I am not worried about that since the growth hormone suppressed below 1.0.

Dr. Kelly really went out of his way to help me and for this I am so grateful. The endocrinologist explained his stance on why he refused to repeat blood work. He feels that when IGF-1 and prolactin levels are normal this is sufficiently indicative of remission; therefore, the glucose suppression test is not needed. He said he thought I was the only one that asked to have the suppression test done and did not realize the suggestion came from the neurosurgeon. He felt the MRI report was "vague" in description of the mass(es) and it did not warrant further investigation. He made it clear that he disagrees with having the suppression test regularly as definitive testing even when there is questionable residual on the scan.

As you can imagine I am VERY relieved that the blood work is normal, especially when I consider my options concerning growth of any residual tumor next to my carotid artery. The location deems it inoperable. Dr. Kelly mentioned this shortly after my surgery when the macroadenoma was removed three years ago:

- August 31, 2005

“...should this [2mm] area in the cavernous sinus prove to be residual tumor, it would be an ideal target for radiosurgery and would likely not be amenable to further surgical removal given its location within the cavernous sinus.”

Given my endocrinologist's reluctance to put my mind at ease by ordering simple blood tests, I am considering moving any future appointments to a different doctor. I prefer to go to an endocrinologist that has an active interest in researching acromegaly, and so I am looking into MD Anderson Cancer Center in Houston as a possibility. Although it is evident I am still in remission, I shouldn't feel that I am annoying my doctor when I voice concerns about symptoms I am having or fear returning tumor. This is especially true when it is explicitly stated on MRI results that there is an area that is suspicious for residual tumor and it is slightly larger. There is no pathological evidence to support recurrence of tumor, but this area previously measured 2mm after surgery and it is now aprox 8mm. The neurosurgeon recommends that blood work be assessed every 6 months to be sure I remain in remission - this is just standard protocol.

Then & Now

Click to Enlarge

I am posting the most recent scan for comparison to previous years. The area in question has always been there and not changed in size. The scan I had done last year looked like the one I had in August of 2006 (seen above) so I am not posting it. The films have been sent to my doctor and I am awaiting his interpretation of my recent MRI.

**Note: the red arrow is pointing to the macroadenoma I had removed. The other arrow is pointing to tumor that had grown into the cavernous sinus on the carotid artery. This is the suspected residual. My labs have been in the normal range, and so the area in question has been considered to be scar tissue.

Prolactin Level While Nursing

Visit Source: kellymom.com Breastfeeding & Parenting

(Normal value ranges may vary slightly among different laboratories)

I got a call from my endo this morning. My prolactin level is 4.9. I guess this explains the lactation problem. My (pre-pregnancy) prolactin was 5.2 last year. I am amazed that I am producing any milk right now. I am so glad I had this checked out. At least now I know what my daughter's low weight is caused by; there is no doubt that she is not getting enough breastmilk. The doctor said this is "almost surely" due to my pituitary condition, and there is nothing I can do to increase my prolactin level. It is interesting to note that I did not have a pituitary condition (that I knew of) before I had Danielle. I asked if I needed to come in and discuss this further with him, but his nurse said he had no appointment available until November.

I feel like it's futile to try and continue nursing Danielle. I will feed her what little I can, but since she is weaning naturally, I expect I will mostly likely stop altogether soon. The bottle is certainly doing a better job than I can right now. I plan on going in for my annual MRI some time this month.

Feeding Baby Post Surgery

A couple weeks ago I started getting concerned about my daughter's weight. She seemed awfully small compared to what my other three children weighed at her age because she's not as chubby as I remember my other kids being. I began weighing her on the scale at home and noticed she wasn't gaining weight since her 4 month check up in June. I went digging through my children's baby books to see what their weight was at 6 months. All of them had more than doubled their weight and were above 16 pounds. Danielle was barely 13 pounds (she weighed 8 1bs 4 oz in February). I began to suspect my milk production was diminishing. At 4 months old Danielle began nursing constantly and her hunger wasn't satisfied so I started feeding her solids also to accomodate for her appetite. She wasn't showing signs of dehydration and all seemed normal except for her feeding frenzies. I was still nursing her around the clock, but I wasn't sure I was keeping up with my daughter's needs.

I took Danielle in for her 6 month check up last week and although she is doing well, her weight is in the 5% range (13 pounds 11 ounces). My suspicions were confirmed - she is under weight. Her little growth curve has taken a downward turn. I asked the pediatrician if he thought maybe she wasn't getting enough breastmilk and he said it was possible given my history of pituitary surgery; however, he was not too concerned because my daughter's length and head circumference is growing nicely. He recommended I try to up my milk production using Fenugreek, but he suggested I ask my doctor first because of my medical history. Fenugreek spurs on prolactin and the pituitary tumor I had was both prolactin/growth hormone producing so he wasn't sure it was safe for me. I could supplement (optional) and give her some Poly Vi-Sol vitamins to help.

When I got home I tried expressing milk during feeding time to see how much milk I was producing and I could barely get an ounce! This is very abnormal for me. I called my doctor and told him what was going on and wanted to see if there was any way to know whether something was wrong with my pituitary function and he said no. He said I cannot take Fenugreek or anything that would help with milk production because it might spur on tumor growth. He advised I supplement since there is suspicion of milk depletion.

This is strange - something's wrong. I fed all three of my other kids with ease for at least a year. Although I wondered if there would be an issue breastfeeding Danielle post-pituitary surgery I had no problems right after I delivered her. Her first few months she was thriving so there was no question as to whether I could breastfeed successfully. Now it appears I am not producing enough milk. I don't know what changed from May to now. I have drank more fluids and done everything humanly possible to change this. In light of the situation I am going to have to supplement and see if it helps with Danielle's weight gain.

Planning Ahead

I have recently received a flier from my neurosurgeon, Dr. Kelly, about two websites that have been created. They are very patient-friendly and offer an abundance of support and information regarding treatment for pituitary tumors:

Neuro-Endocrine Tumor Center at Saint John's Medical: "NETC's new comprehensive website is filled with outstanding current and accurate information. The website is designed for the patient. Dr. Kelly spent many hours writing medical content and meeting with the web-technicians to ensure that patients can use the site for education as well as a tool to help themselves and others...take time to watch a couple videos of our past support group meetings with friends and family!" - Sharmyn McGraw, Neuro-Endocrine Tumor Center's Pituitary Support Group Facilitator.

*Please note: NETC is now known as Pacific Neuroscience Institute.

Meet Sharmyn Mc Graw: A patient of Dr. Kelly's and former Cushing's sufferer. She has been in remission from the disease for several years. She is head of the Neuro-Endocrine Tumor Center's Pituitary Support Group in Southern California.

I would encourage you to take the time to sift through both sites. Treatment for pituitary tumors can be a life-altering experience; therefore, if you are thinking about having surgery or have been told it is necessary, it is important to be as educated as you can and plan. Dr. Kelly has put together an informative video where he addresses many questions people have when preparing for surgery. You can click here to watch the video or follow the link on the right side of my blog.

**Warning: the video does show a graphic video of the surgery being performed.