Patient Stories: I Have A Giant Living In My House
I am the proud mother of three wonderful sons and a wonderful daughter. My third child, Mark, was diagnosed with a pituitary macroadenoma (not microadenoma, it was too big, approximately 5 cm.) in June 2000. He was 16.
He had been growing, and went through an awkward stage which I described as bursting into the room when he entered. I noticed he was tired and when he lay down on the floor, well he took up a lot of space. I am guilty of calling him lazy, but on several occasions I thought maybe the heat or some other change in the weather was affecting him. He started not feeling well on Sunday and not going to church. A lot of people would not be alarmed, but I knew it was not because he didn’t want to go. Later, he enjoyed reminding the pastor that the pastor had told Mark, “It’s all in your head.” Finally, one Sunday, he complained of pain in his eye and I noticed it was drooping. My mother had had Bell’s Palsy, so I thought maybe that could be happening. Monday morning I made an appointment for Wednesday.
His eye seemed to temporarily improve.
On Tuesday, he was sitting on the grass with his brother and they moved away so I could have a private conversation with their sister. Then, when they came back, my youngest son said, “Oh Mark, I’m sorry! I’m sorry!” I looked over and Mark appeared to be unconscious in the grass. I asked what happened and Stephen said that he had accidentally kicked him in the head. It was actually more of a nudge.
Mark has been involved in drama, so I thought he was playing around. Later, he said to me, “Mom, I know it was an accident, but Stephen kicked me in the head and I think I was out. He should at least say he’s sorry.” Well, obviously he was out, because Stephen had apologized. The next day, I related all this to the doctor. Mark had a CT scan and we found out about the mass. When the doctor came in to tell us, Mark and I knew something was up. We exchanged glances. It must have been very difficult for the doctor. I was in the process of a rather nasty divorce, and my father had died within the last two weeks. My daughter was in the hospital for depression. On Thursday, Mark had an MRI. We didn’t know what it was. Mark never cried and never seemed depressed. He said two things to me over the weekend while waiting for the appointment. The first was, “Well, if I die, I know where I am going,” and the second was, “If I am going to die, I’d like to know about how much time I have left.” As a Mom, I didn’t want to deal with the “d” word. He was light years ahead of where I was.
Monday, Mark, his father, and I went to a neurosurgeon. At this point Mark was about 6’2″, 180 lbs. His father is 5’8″. I am 5’4″. However, I had a grandfather who was 6′, 200 lbs, and Mark was kind of a chunky kid unlike his leaner brothers. He wasn’t heavy. He carried his weight well, but he has broad shoulders. We were told that he needed surgery, radiation (which would probably be pretty simple but that ended up not being the case) and we could probably expect him to live to 50. So, although the thought of brain surgery is pretty scary, we knew Mark would be in expert hands, and that he would most likely come out okay.
Technically, Mark had gigantism, because the tumor had squashed the pituitary and puberty was delayed. Now, he has passed that and is currently getting an injection of testosterone every 2 weeks. He was using gel, but really didn’t like it, and stopped using it, so the injection is better for him. So, now he has acromegaly. He had two surgeries in July and August of 2000 with Dr. Edward Laws at the University of Virginia Medical Center in Charlottesville, VA. Dr. Laws is respected world-wide. The first was removal through the nose (transphenoidal) and the second was through the left side of the skull–craniotomy. Dr. Laws said that he couldn’t get as much of the tumor as he would have liked without doing permanent damage.
It was also a harder consistancy than he expected. Before surgery, Mark had lost 75% field of vision in his right eye. This was the eye that was not drooping. After surgery, it was almost 100% restored, but he was still complaining about his vision. It was only by examination before radiation that we knew how well he had recovered.
When he was recovering from his craniotomy, Mark was put in intensive care for awhile. They didn’t have a room for him on the wing, so they kept him there a little longer than he would have liked. They did not want him to room with one patient who was noisy. Mark understood this. He told me once how someone had to escort him to the bathroom, but they enjoyed it because the other patients in intensive care complained so much. My next visit, which was probably later the same day, Mark was extremely negative. This was uncharacteristic. I know I am a very proud Mom, but I was a little concerned. I told myself, “Give him a break, he just went through brain surgery.” When he came back from going to the bathroom, he was complaining about not having a room and still being in Intensive Care. He knew why and they had a good reason for this. I mentioned gently to him that he was being extremely negative. He said, “Am I? Maybe I’m reacting to the medicine.” I went out to dinner and came back to check on him. He was asleep, so the lights were very low, but his face was so red, I could see it in the dark! Indeed, he had reacted to the medicine, but they were able to straighten that out. After this second surgery he told me, “Mom, I know that whatever I face in life, the Lord will be with me through it.”
By looking at photos, we figure the tumor was about 4 years old before we discovered it. Mark had not complained of a lot of headaches. After surgery, I had Mark speak at a school where I worked. He had to close one eye or he would see double. He looked funny. He looked like the actor who played Eyegore in Young Frankenstein. You could see where all the staples had been because his hair had not grown back yet. He had several groups of inner city kids hanging on his every word. He spoke to kindergarten to eighth graders. If they had made fun of him, he would have been okay. But they didn’t, and some of their questions were amazing. It was great for them and for Mark. Another time, teachers signed up for a visit from him.
We brought a tape from the Discovery channel about giants. Mark did a question and answer with each group. This was before his radiation. I brought some of his MRIs to show students the tumor.
When it was time to schedule the radiation, it was not the walk in the park I expected. Because the tumor was so large, and not much was able to be removed, conventional radiation was recommended. However, I had heard so often,”You don’t want that because of blindness and brain damage,” that I was very upset. Once again Mark, his father and I went to an appointment to discuss options with a radiologist. I was so upset even to be there that
I wasn’t saying a word, but I did listen. The doctor said that conventional was not that dangerous and we could have him in school after treatment which would take five weeks locally. Mark went to school part time at this point, and I was supervising some of his studies at home. I worked as a substitute teacher. No one was commenting. So the doctor continued and said what would have the least risk to Mark was Proton radiation at Massachusetts
General Hospital. I was still unhappy with conventional radiation, so we went for a consultation with Dr. Jay Loeffler at Mass Gen. He basically said they couldn’t turn us down and we scheduled to go during the summer. At the time, it was still based in Harvard, but now the cyclotron is across from Mass General’s main entrance.
In the literature I did find about proton radiation, most acromegaly patients get one day. Mark got 30 or 31 days. That was because his tumor was so large. He didn’t have a very large bald spot, and his hair grew in curly. It’s not so curly now, and his hair is as thick as ever. He has
seen Dr. Loeffler twice for follow up visits and everyone is pleased that the tumor is shrinking. While we were at Mass Gen, I found out through the internet that there was an open study on acromegaly at the National Institutes of Health (the largest research hospital in the world). So
within a week of returning home, we went to the NIH where Mark was an outpatient for 4 days. He was invited to talk to a group of military doctors on rotation at the NIH.
Several months after radiation, Mark started not feeling well. He was often sent home from school and I ended up withdrawing him in May 2002. He started passing out. At first we thought this was a fluke, but before we went for our annual visit to the NIH in August, he was passing out sometimes 7 or 8 times a day. But when he was asked to speak to the military doctors, he wasn’t told that it would be about 15 minutes. He thought it would be
about two hours! He loves talking to people.
I remember the passing out had barely started when we had the first check up with Dr. Loeffler in February 2002. Dr. Loeffler was very happy about the tumor shrinking, but Mark had been feeling so poorly, that I was preparing myself for some kind of bad news. By the second check up in February 2003, it was suggested that Mark might have sleep apnea or narcolepsy. This is in the process of being investigated. At the moment, he is doing really well, but there has never been an explanation. At one point, the radiation was suggested, but Dr. Loeffler said the radiation would not have this kind of effect.
Last August, Mark was an inpatient from Sunday to Friday at the NIH. They did the 24hr. EEG, had a heart monitor and did a lot of other tests. He passed out during his MRI. He would pass out standing up, laying down or sitting down. It was not seizures. Outside the NIH, I was told that the radiation would take up to 10 years to bring his growth hormone down to 0. At the NIH, I was told based on his IGF-1, he probably had ten years to live. This was quite a shock to me, since my expectation had been that he would live to 50, and that is still quite a way in the future. This doctor did not know my whole context of what other doctors had said. I did appreciate knowing the information. All the doctors seemed to agree that Mark would be a good candidate for the medicine in the clinical study coming up pegvisomat. Mark’s blood work showed his IGF-1 was not high enough to get into the clinical study. I couldn’t understand if he supposedly had 10 years to live based on his IGF-1, how he possibly could not have a high enough number. Our endocrinologist, Jonathan Anolik, was listed as a “Top Doc” in Philadelphia magazine. He deserves that. All of the doctors we have worked with have been superb. Dr. Anolik got special permission for Mark to enter the clinical study. Then they closed the study. Finally, they let him in because they had given him special permission before they closed the study. Recently, Dr. Anolik called about other matters and I asked when Mark was going to take pegvisomat. Dr. Anolik said that his IGF-1 was in the norma
l range and he didn’t need it. Mark and I believe this is the result of answered prayer. When he started the process for pegvisomat, he was considered refractory, which meant his other medicines were not effective in lowering his IGF-1. No one has really attempted to explain otherwise to us. Even if they do, we know what we believe.
Being a Christian means not just that you believe Jesus Christ died for your sins and rose again and some day you will go to heaven, it means trusting Him day by day in your life. He died for our sins, and trusting Him means eternal life starts now and continues into the next life. Mark shows me this every day. He is not perfect, and our family is far from perfect.
His brothers have grown in compassion because of this time of having to look out for Mark. One doctor at the NIH questioned if Mark had control of the passing out because he wasn’t bruised and didn’t break any bones. I was working a lot at that time, so Mark’s brothers took care of him. I said that his brothers were very diligent. He didn’t accept that, so I said, well the angels are watching over him. He REALLY didn’t like that. As a Mom, although this hasn’t been real easy, Mark and the rest of us consider his tumor a blessing because we have learned so much from it. When I get discouraged, I look at him and think I really don’t have anything to complain about. He is an example to me. I think it has also affected the way we look at life in the sense that we want to enjoy simple pleasures of being together. We kid around a lot. We laugh. We tell each other that we love each other. Sometimes we have a family hug “sandwich” initiated by Mark.
The issues for Mark are at the moment, his bilirubin is high. Not long ago, his pupils looked yellow to me and even his skin. We’ve had a lot of rain, but we’ve managed to get him out and walking, which was a real difficulty when he was passing out more frequently. The other effect of radiation and probably the tumor as well, are his teeth are terrible. He has impacted wisdom teeth, his other teeth are losing root canals and soft. Since he was passing out, no one would agree to dental surgery, but now the doctors have given me a go to get it for him. His gallbladder is “sludgy”. He does not have gallstones. Dr. Anolik told me that sandostatin (which the NIH said helps shrink the tumor as well) could have affected this, but Mark hasn’t had sandostatin since January due to the study. He has purple lines from prednisone, which he takes in a different form now. He is presently 6’7″, almost 300lbs. He is starting to lose weight. He thinks that since his IGF-1 is down, that he is not as hungry as he was. He is a very picky eater, and does not eat hardly any fruit or vegetables. However, he takes supplements and drinks fruit juice. We are looking forward to dealing with these issues and going on a family vacation before going to the NIH again in August. Mark will turn 20 in September.