Matthew Venses was born on April 2, 1992.  He was my second child, but my first boy.  He was a healthy baby with normal milestones reached throughout his infancy.  He started Kindergarten and was one of the first students to learn to read.  In first grade his teacher said that he was always the first one to raise his hand and wanted to contribute to whatever they were discussing at the time.

    Then, on May 10, 1999, we were studying for Matthew’s weekly spelling test.  My little boy, who had been in the 1st grade spelling bee the week before, couldn’t spell any of the words. We also noticed that he was having trouble adding.  Matthew’s teacher had also reported that he was one of the fastest with his addition facts.  I remember vividly looking at my husband over Matthew’s head.  We were both scared and wondering what was wrong.  Finally I sent Matthew to bed, hoping he was just tired.

    The next morning, Matthew’s teacher called at about 10:00 in the morning.  He asked what was wrong with Matthew.  I told him that I was hoping Matthew was having a bad day.  If he didn’t feel better the next, I would take him to the doctor.  That turned out to be exactly what happened.  However, Matthew’s doctor looked in his throat, had him say ah, and sent him out of the room.  She told us that Matthew was seeking attention after the recent addition to the family (our fourth child, and second daughter, was two weeks old).

    I tried to think Matthew was only attempting to get attention, but several days later he gave up asking for ice cream because he could not get the words out.  It was then that I decided to pull a few strings.  I am a teacher at Matthew’s school.  I went to the speech therapist and asked her to talk to Matthew.  I wanted to know - was this my imagination?  The answer . . . NO, it was NOT!  We then went for a second opinion.  God smiled on us that day, and for many days to come.  The pediatrician that I had picked out of the yellow pages was married to a neurologist.  She listened to what I was saying and not what she saw.  She pulled some strings; we were seen the next day by her husband.  He also listened to what I was saying and not what he saw.  He ordered a MRI.

    Matthew was unable to stay still for the entire session.  After one hour, he had to get up.  The technician said he thought it would be okay.  He said, "I think we can see what is going on."  I knew then, just from the sound of his voice, that they had found something to be wrong.  It was a Monday, and our return appointment was on Friday.  By Tuesday morning, the neurologist had called.  Matthew had had a stroke!  Testing began with pediatric neurologist to see why he had had the stroke.  The very last test (and most invasive) would tell us why.  Matthew had Moyamoya.

    Moyamoya is a Japanese term to describe what the blood vessels in the brain look like on an angiogram x-ray.  The arteries to the brain begin closing off.  Because this happens slowly, tiny vessels grow in an attempt to provide blood to the brain (the frontal lobes are affected the most).  Eventually hundreds of tiny vessels will be present.  When the dye used to help the vessels show up on the x-ray enters the Moyamoya vessels they will look like a "puff of smoke".  That is what Moyamoya means in Japanese.  It is considered extremely rare, but some days, after talking to all the parents who belong to this network, my husband and I really wonder.

    After Matthew’s angiogram a second stroke occurred, most likely caused by the anesthesia used for the test.  This stroke affected the left side of his body.  He had trouble using his hand and foot.  Again, God smiled on us.  Matthew regained most of the usage of his foot and hand even while still in the hospital.  During his hospital stay, it was decided that he should be placed on coumadin, a powerful blood thinner.  In order to achieve a low clotting rate quickly, he received heprin shots twice a day while the coumadin took affect.  Then, to check drug levels, he had blood drawn twice a day.  My son was a human pin cushion!

    Once home I began looking on the Internet for information on Moyamoya.  Everywhere I turned I found a Dr. Scott, from Boston,  as the expert on Moyamoya surgery.  However, I kept thinking, "I can’t go all the way to Boston!"  Finally I broke down and called my insurance.  Again, God smiled on us.  Our insurance had the same contract with Dr. Scott that they had with the doctor in Albuquerque (the nearest large city to us).  This meant they would pay for the surgery regardless of which doctor we used!  We began making our plans for a trip to Boston.  Still, God smiled on us.  Our church was able to get us FREE plane tickets.  The hotel had an agreement with the hospital and gave us a 50% discount (though still expensive!)  Another friend arranged a rental car at a 50% discount.

On September 7, 1999, Matthew had the surgery that Dr. Scott had perfected for Moyamoya.  There is nothing the doctors can do about the arteries closing off.  Instead, they allow the brain a way to compensate, literally.  Dr. Scott lifted arteries from Matthew’s scalp without disturbing their blood flow.  He "detoured" a section of the arteries through the skull, onto the surface of Matthew’s brain.  Over the course of a few months, the brain would realize that another blood supply was present and grow new blood vessels branching off the detoured artery sections.

    Matthew did well during the surgery and was released in record time.  He was also readmitted to the hospital in record time.  His first night out of the hospital we woke to find him having another stroke, and a seizure with it.  We immediately went to the emergency room.  This stroke affected the left side of his face and made speech VERY difficult.  His father and I seemed to be the only two who could understand him.  Swallowing was also impossible and a dreaded IV had to be started.  Matthew, like all children who have been poked and prodded, had to be tied down.  When he became upset, his speech was unintelligible, even to us.  We managed to be released from the hospital the next day.  We flew home and have monitored Matthew constantly.  Today, he can again be understood by all.  He has regained his motor skills to the point that not even a trained professional can see deficits.  He takes one baby aspirin a day, rather than the coumadin that required weekly blood level checks.

    In July of 2000 we returned to Boston (we went at 10 months rather than a year because I am a teacher and it is very difficult to take time off from work in September).  Matthew underwent another MRI and another angiogram.  God smiled on us again.  Dr. Scott said that the results of his angiogram were very good.  He had good circulation to both side of his head, although the right side had grown more blood vessels than the left.  I find this curious.  The left side had the Moyamoya vessels in the original angiogram while the right was just beginning.  However, Matthew has had two strokes on the right side and only one on the left.  I am sure that God knows what he is doing.

    While we were in Boston the doctors also tried to correct Matthew’s high blood pressure.  This had been discovered in the process to find why Matthew had had the first stroke. During the original angiogram they had found he had a narrow artery leading to his right kidney. The kidney perceives this lower blood flow as dehydration.  So, it sends out a chemical that raises the blood pressure.  Long periods of this can cause damage to the heart. Although I have to say here that I believe the high blood pressure was another smile from God.  I truly believe that it protected Matthew during all three strokes.  The high blood pressure prevented the strokes from causing massive damage and kept them as light as possible.

    During the follow-up angiogram to see if the surgery had been a success, the doctors widened Matthew’s kidney artery with a balloon (much as they do for heart patients who have too much plaque on the arteries leading to their hearts).  Here I must go into further explanation.  At the time we thought the surgery was a success.  However – several months later we realized that it would have serious repercussions. 

   Matthew was able to come off one blood pressure medication.  We began cutting back on the second, but had to immediately put it back up.  When Matthew had a 24 hour study of his blood pressure (a portable machine attached for 24 hours), we discovered that his high blood pressure had returned.  A renogram showed that his left kidney was no longer functioning.  I don’t think the technician realized that I would be able to see that – or she would not have let me in the room.

   We immediately followed that up with another angiogram – which showed the kidney to be very small, but still receiving blood.  All I will say, it was the angle of the picture . . .  Again, I began doing research.  It was at this point that I discovered that angioplasty does not tend to work with children under the age of 12.  The doctors had decided to recommend the least invasive and go from there.  I immediately requested that we go with the most effective.  We contacted Dr. James Stanley at the University of Michigan, who has perfected a renal bypass and worked with other moyamoya patients.  Matthew and I flew to Ann Arbor and he underwent the surgery in March of 2001.  However, once inside, Dr. Stanley discovered that during the angiogram, small – tiny – portions of the renal artery had broken off and clogged the even tinier blood vessels in the kidney.  One third of Matthew’s kidney had died.  It was impossible to repair in a way that his blood pressure would be normal.  Dr. Stanley had to remove Matthew’s right kidney. 

   Despite all this, Matthew does continue to do well.  He is currently on grade-level in all areas.  He is monitored by his nephrologist simply to ensure that the remaining kidney is growing and healthy.  It has overgrown in order to compensate for doing the job of two.  Matthew’s prognosis continues to be good.  Issues I once had with his behavior, that I attributed to the strokes, are gone – but have also turned up in his healthy little brother (who is now that age).  So maybe he has always been exactly as he should be (and, for once, I was just a paranoid mother J).