My name is Sue Ann McKnight. I have had Classic MSUD all my life. I was diagnosed at eleven days old by Dr. Henry Waisman in Madison, WI. I am now thirty-two years old.
At the end of January 1994, I got the flu. I felt run down; I was sneezing, coughing - the works. Then my fingertips and calves went numb. They felt like they were asleep, only they never woke up. It was not that much of a problem until my fingers starting slipping off the keyboard of my computer at work. I went to see my internist, Dr. Frank Polyak on February 2. He didn't know what was causing the numbness. He sent me to a neurologist, Dr. Murphy. I was scared. I work at an insurance company. I know what a neurologist does.
Dr. Murphy did a variety of tests (made me squeeze a ball, push him as hard as I could, etc.). He tested my reflexes with a rubber hammer. My reflexes were gone. Nothing happened when the hammer struck various parts of my body (knee, ankle, etc.). Dr. Murphy diagnosed me with Guillain-Barré Syndrome (GBS). I had heard of GBS, but didn't know what it was.
GBS is a debilitating disease that attacks the nerves. It is caused by an antibody from an infection (such as the flu). Usually, the body produces antibodies to fight the infection. In the case of GBS, one of the antibodies turns against the system. Around every nerve in the human body is a cushion, so when you bump something, it doesn't hurt that much. With GBS, the bad antibody destroys the cushion around the nerves, making them highly sensitive and paralyzing them. The only cure is strong antibodies and time.
Dr. Murphy put me in the hospital to watch my condition. If GBS gets in the chest, it can kill by paralyzing the lungs. I was in the hospital four days. The GBS was in my arms and legs, but I could still walk, feed myself, etc. I felt fine, just numb. When I was discharged, Dr. Murphy said I could go home but not back to work. Another thing GBS does to a body is wear it out. The littlest thing can tire the body tremendously. He didn't want me to overexert myself.
So for two weeks, I stayed home, resting as the doctor ordered. Then one Monday, I woke up and was so dizzy that I couldn't walk down the hall without hitting the walls on either side. My husband, Lon, told me to call the doctor as soon as possible. I called Dr. Murphy's office at 8:30 when it opened. He told me to go to the Emergency Room as quickly as I could. I called Lon. It took him ten minutes to get home. I couldn't stand up. Lon helped me into the car and took me to the Meriter-Park Hospital Emergency Room.
The numbness was spreading fast. It went to my face. I had a hard time talking. My facial muscles were numb. I stayed in the ER for a few hours, and then I was put in my own hospital room. By then I couldn't move. My hands locked up into fists. I had IVs to prevent dehydration. They gave me a very powerful antibiotic called gamma globulin intravenously. Lon stayed with me until visiting hours were over. Then he picked up our then three year old daughter, Amanda, from my parents' house.
The only things I could move and control were my head and neck. So the nurse pinned my call button to the collar of my hospital gown in case I needed help. I remember lying in bed. Suddenly I started breathing real fast and I couldn't slow it down. I frantically hit my call button with my chin. The next thing I knew, it was the morning after. Lon and my mom were in my room. I had a tube down my throat and my glasses were missing. I didn't find out what happened that night until two months later. Lon waited to tell me until I was stronger and able to handle it.
He had gotten to bed about 10:30 p.m. and just fallen asleep when the phone rang. The doctor told him to get back to the hospital immediately. He took Amanda back to my parents, and he and my mother rushed to the hospital. Lon had been told to go to the ER because the main doors were locked. A security guard met them at the entrance and took them up to ICU. A priest met them in the waiting room. He told them I had gone into Code Blue and the doctors were working on me. GBS had gotten into my lungs.
I was on a respirator for a day and a half. I was in ICU for two days then moved to a private room. Then the pain set in. It was unbearable. The only way to describe the pain is - take the pain of when your leg wakes up after it falls asleep and multiply it one hundred times. My nerves were so sensitive. I couldn't stand to be touched, especially my hands and feet. GBS in the extremities (fingers and toes) takes longer to rehabilitate because they're farthest away from the body. For two weeks, I was miserable. I was paralyzed and in pain. No one knew what I was going through. No one could really comfort me because of the hypersensitive nerves and pain. I became very depressed.
I was transferred to the Rehab Floor where they help stroke victims, accident victims, anyone who needs to learn how to do things all over again because of a medical condition. I had four sessions of therapy, Monday through Friday, and two sessions on Saturday morning. I had physical therapy (PT) and occupational therapy (OT) - one session of each in the morning and one of each in the afternoon plus one of each Saturday morning. PT was for my lower body so I could learn how to walk again. OT was for my upper body so I could get back to my job and work with a computer again. In OT, I sorted beads, put puzzles together, and worked on the computer to help my hands get back to normal. In PT, I learned how to walk all over again with rigorous daily exercises and routines.
I went home the last week of April 1994. I went home with a rolling walker that had two wheels in front. I continued my physical therapy at home for a month. I could walk but I still had some numbness in my feet. I went back to work part-time in July and full time in August.
Two years later in September 1996, I took a home pregnancy test. It was positive. I had an appointment with my family practitioner, Dr. Russ Hermus, to check why my feet were hurting (I had a burning sensation when I walked). He confirmed the pregnancy and estimated my due date as April 4, 1997. I had an ultrasound later that day. Everything was normal. We told our families and I consulted with my dietician, Sandy van Calcar. She changed my formula recipe to regulate my diet. I had to keep close track of the daily amount of protein I consumed. I had to have a certain amount of protein every day and take Carnitor and MSUD-2 pills.
My due date turned out to be May 20, 1997. Dr. Hermus set us up with Dr. Susan Davidson, a perinatologist (high risk pregnancy doctor) at St. Mary's hospital. She took over my case.
In January 1997, I broke my left foot and had to be in a wheelchair for six weeks. The second week of February we had an appointment with Dr. Davidson for an ultrasound. She was worried about the results and wanted us to come back the next day to be monitored. They hooked me up to a monitor to check the baby's movement and the way the fluid moved through the umbilical cord. It also recorded breathing and heartbeat. There were times during the monitoring that it seemed as though the baby was crimping the cord and not enough fluids and/or oxygen were getting to the baby. Dr. Davidson didn't like the results so she admitted me to the hospital.
The baby wasn't growing as he should. So they monitored me for twenty-four hours straight, but since the problem didn't have any pattern to it, and was very sporadic, they decided to do it once in the morning and once at night. I had ultrasounds daily. They checked four different things on the ultrasound - the amount of amniotic fluid, two types of movements, and breathing. You rate two points for each. The best you can get is eight out of eight. Most of the time, I got a six out of eight. A couple of times, I did have eight out of eight. But still, for some reason, the baby wasn't growing the way it should.
Four days after I was admitted, I had slight contractions and the monitor showed a pattern to the cord problem. I was rushed to labor and delivery. An IV was put in. They were going to prep me for a cesarean section. Dr. Davidson discussed the options with Lon and me. My contractions had stopped and the cord problem lost its pattern. I was at twenty-eight weeks in my pregnancy. I could either have a C-section that night or hold off until my thirty-second week to have the C-section when the baby would be a little further along. We chose to wait until the thirty-second week. I began taking steroid shots. Because the baby would be born prematurely, his lungs would be underdeveloped and the shots would help the baby.
On March 6, 1997, I went for my daily ultrasound. The technician saw something wrong. He got Dr. Davidson right away. On the ultrasound, the baby was in trouble. One lung was filling up with fluid. I started to cry. I called Lon to get to the hospital. He was there in eight minutes. I was prepped for a C-section. An IV was put in. I was given a spinal epidural which made me numb from the abdomen on down. I was awake through it all. Lon sat next to me and held my hand. It took thirty to forty-five minutes (from prep time) to get the baby out and another forty-five minutes sewing me up. Blake Andrew McKnight was born at 2:44 p.m. He weighed one pound, four and one half ounces and at eleven and a half inches was a little bigger than his daddy's hand.
He was taken to the Neonatal Intensive Care Unit (NICU). He had an IV and was put on a ventilator. He was hooked up to different monitors - one checked his heart rate, one checked his breathing; his pulse, everything was watched. I got to see him about three hours after he was born. They wheeled me, bed and all, into the NICU. I never saw anything so small - I was amazed. He was perfectly formed, just small. They fed him through an IV. He had to have blood transfusions, because he didn't have a lot of blood in him, since he was premature.
He stayed on the ventilator until March 19. On March 26, his oxygen levels had fallen, so his doctor, Dr. Dorothy Ritter, decided to give him oxygen through a nasal tube. He showed what a fighter he is, because he pulled this tube out frequently. On March 27, a feeding tube was put in to replace the IV. We went to visit him every night. Amanda went with us. On April 7, he finally got rid of the nasal oxygen. The doctors discovered Blake had a double hernia, which is common in premature babies. This had to be operated on before he went home. I got to feed him through a syringe on April 13, 1997. He was given steroids to help his lungs. By April 23, he was being bottle fed. By May 7, he was a little over three pounds. On May 19, he had the hernia surgery. It went well. Blake finally got to come home on May 28, two and a half months after he was born.
He is now seven and a half months old and doing very well. He's cooing and making noises, drooling, rolling over, eating cereal and fruit, etc. Amanda, now seven years old, is in first grade and loves being a big sister.