September is Hydrocephalus Awareness Month!

           September is hydrocephalus awareness month. Some may have seen posts on Facebook or Twitter, or perhaps it was mentioned in your newspaper. Many of you don’t know what hydrocephalus is, and why would you? I had no idea- had never even heard of it- until the day the Dr. in the NICU came to tell my husband and myself that our seven day old son had it. So, in honor of my amazing son Cole, I will share the story that many parents share: how we made it through Cole’s diagnosis and how we have dealt with issues regarding his condition.

            Almost from the word “go”, my pregnancy with Cole and his twin sister Ava was anything but normal. I won’t go into all the gory details (we’ll save that one for another day!), but the bottom line is I was diagnosed with having a placenta previa at around twelve weeks. A previa is when the placenta covers the cervix- the exit for the babies- instead of being in the back of the uterus like it’s supposed to be.

            On one of my many trips to L&D (labor and delivery) for bleeding, it was discovered that I was beginning to dilate, so those “braxton hicks” contractions I thought I had been having were actually the real deal, and my back pain was in fact back labor. This is when they made me stay in bed 24hrs, and we all crossed our fingers that I would make it to 26 weeks. This was the goal. 26 weeks would drastically reduce many of the complications from a preemie birth, and with them being twins, it was especially important.

            At 26 weeks and five days I was sent to the hospital for the last time. The contractions were stronger, I was still dilating, and now it appeared there were blood clots behind the placenta, which is a clue that it is not fully attached anymore. In an effort to slow down or stop my labor, I was given magnesium sulfate. This is a drug given to people with severe hyper-tension. Women who have pre-eclampsia are given mag sulfate to keep them from having a stroke. A side effect is that it relaxes the uterus, so they give it to women in pre-term labor as well. The little contractions went away, but the “I mean business” ones stayed. Thus, at 27 weeks and 3 days, my twins were born via cesarean section. Baby A, Ava, came out completely fine, just a little small. Cole however, refused to budge. After a tug of war, he emerged and they were both carted off to the NICU to begin their long stay.

            If you are fortunate to have never needed the use of a NICU, I will briefly describe the experience. Try building a sand castle at the shoreline. Every time you think you are almost finished, a huge wave comes and knocks down a wall. You rebuild, a little bit farther way, and another wave comes and knocks down a turret. Such is the life of a NICU parent. Three steps forward and two steps back. Things change so quickly, that in the time it takes you to go to lunch, or pump breast milk, the condition your child was in when you left, may not be the same when you get back.

            When the twins were first admitted to the NICU, they told us right away the hurdles we had to get over, and what to pray they DON’T get. Most were just set-backs, but two were very serious: necrotizing enterocolitis (or NEC), and a brain hemorrhage of 3 or 4. NEC is the death of intestinal tissue, and preemies are at risk because their little bowels just aren’t ready to work yet. Now to the brain hemorrhage; I know what you're thinking: anything that has hemorrhage in it has to be really bad, so why only 3 or 4? Well, it has to do with where the bleeding is, and a 1 can be found in any baby-preemie or not- because it is basically like a little bruise. A three is bleeding in the ventricles, the things that move the spinal fluid from your spine, to your brain, and back down. A four is bleeding in the brain tissue, and this usually leads to CP. They test every newborn the Monday after they are born, as long as they are at least 3 days old. Babies born before 28 weeks are at greater risk because the blood vessels or so fragile, and with the trauma of delivery and being on a ventilator, it has unfortunately become common enough that all preemies are checked.

            Both Ava and Cole were doing great at 7 days. They were both off of the ventilator, and on a nasal canula. They were getting breast milk by gs tube, and were not showing any symptoms of anything wrong. Everyone was pleasantly surprised at how well they were doing, and no one expected anything abnormal to be seen on the tests. So it was a complete shock when I went for my daily visit and kangaroo care and they took me into a little room and gave me the news: Ava had a grade 1, and Cole had a sizable grade 3. We could expect head swelling, possible need for intervention (lumbar puncture), and transfer to another hospital for extended care. Time would only tell how severely his brain would affected. He had an active bleed, and it was getting worse. My husband and I went home and cried in bed together. Four years of trying to have kids, a nightmare pregnancy, and now our son was probably going to be severely brain damaged. What was his quality of life going to be?

            They measured Cole’s head several times a day, and after a couple weeks, they started preparing us for the inevitable transfer. We had a meeting with the doctor and she compared the films for us. They scanned him every day- probably more.  And then, a miracle happened. Right as they were prepping him for transfer, the last scan came back: the bleeding had stopped; All on its own. They assured us that since it had stopped on its own, the nightmare was over. He would be fine. His head was still a normal size and was no longer growing faster than his sister’s. We breathed a sigh of relief.

            Ava came home after eight weeks, and Cole followed a week later. We took them for their check-ups, and everything seemed perfect. Because I am OCD by nature, I continued to take temperatures, chart feedings, and measure Cole’s head, even though they had told us it wasn’t necessary. After about three weeks, I started to notice Cole’s head was getting a little larger. Now, it would be odd if it didn’t grow at all, so at first I wasn’t concerned, but after five days, it had grown three centimeters. This was not normal. Cole was not that interested in eating all that much, and was sleepy all the time- as most newborns are. It was difficult to figure out what was normal and what was not. After discussing it at length with my husband, he decided to look up the symptoms of hydrocephalus, a condition caused by the type of bleeding he had. Because he was a baby, most of the symptoms were difficult to see, either because he couldn’t tell us, or because newborns are not generally active. There was one: sun setting of the eyes. Sure enough, it appeared that he could not look up.

             It was midnight. We called his doctor.  His wonderful pediatrician insisted that it was impossible for it to come back once it resolved on its own. Then he called back an hour later and told us to bring him then next morning to be sure. It worried me that he had been up in the middle of the night thinking about it. The nurse at his office verified my measurements, and Dr. Mannan and I talked about what to do. He suggested taking him to the ER for a new scan, just to be sure. Maybe we’ll get lucky, and he’s just a big headed kid.

            The CT scan confirmed what we’d feared: he had hydrocephalus and needed a shunt placed immediately. He was transferred to Children’s National Medical Center and put under the care of amazing Dr. Yaun. He was first on the schedule to have a fixed valve ventriculo peritoneal shunt placed. I know, it sounds like something from a science fiction movie. Basically, he has a valve that drains the spinal fluid to relieve the pressure. It has a tube that runs (internally) from his head to his belly and the fluid is absorbed there. There is several feet of tubing in there so that as he grows it lengthens on its own. It is permanent. He will always have it. After a few days, we brought him home again. The shunt was working, and it seemed we caught it in time to prevent any serious brain damage.

            Things were going well until about two months later, when I started to notice his head was damp when I would hold him sometimes. At first, I thought it was sweat, but Cole had had a stitch that never dissolved and eventually fell out. It had left a tiny pinhole in the skin, and I thought the fluid was coming from there. Due to staff issues at the Children’s hospital, I was not inclined to go back, so I made an appointment at John’s Hopkins. I met the dr., he looked Cole over, and said it was probably sweat and not to worry about it. And the nightmare begins.

            In early December, about five months after bringing the twins home, Cole got an unusual symptom: his scrotum was getting large and turning purple. Now, I am not a man, but I am pretty sure this was not normal. It would go away, then come back. We went to a “specialist” who ok’d us to go on our family trip to California for Christmas. He said Cole had what was called a hydrocele- an opening between his scrotum and abdominal cavity. This usually closes in utero at around 28 weeks (sometimes later), but Cole was born before that. It is not a big deal, and usually self resolves by the time they turn one. So we hopped on the plane and left.

            Cole had been having some other strange symptoms: diarrhea and not a lot of interest in eating. We thought maybe he had some kind of allergy, and tried every type of formula (my milk had dried up and the Lactation Consultants thought it was from all the stress, no sleep and poor nutrition I had during this whole ordeal). He just wouldn’t eat. It seemed like he was afraid to. Two nights before we were to return home, he began vomiting and his scrotum was the worst is had been up to this point. We took him to the emergency room, where we were told he needed emergency hernia surgery and were transferred to Loma Linda hospital.

            We were talking with his doctor the whole time, who was begging the hospital to call him, and they refused. A whole day passed, and we waited for them to do something. Our suspicions aroused, we asked them to do a shunt series, which they refused to do. Finally, when it was clear that they were in no hurry, we called his doctor and he said bring him home. Our flight left in six hours, and we would be home with doctors who knew him and his history. Loma Linda was furious, but what could they do? They weren’t acting, and they weren’t even considering his shunt.

            We got home, and he seemed better. We took him to the “specialist”, who once again said he was fine, and we tried yet another formula. After a few weeks, he was vomiting again, and the scrotum was back to looking like a rubber bouncy ball. We took him to Hopkins, where we waited 18 hours for a neuro consult. At two in the morning, he finally deigned to gift us with his presence. He glanced down at Cole, told us to find a pediatric urologist, and sent us home. During this time he had been on IV antibiotics and the vomiting and fever went down. We were beginning to form an idea of what was happening. Our theory was that he had a shunt infection, and the fluid in his belly was infected. As the pressure mounted, the fluid was being pressed through that hole in the abdomen, causing the scrotum to get red and large. The doctor of course dismissed us as crazy parents.

            A few weeks later, we had seen the new urologist, and he wanted to hold off until the shunt situation was taken care of. By now, Cole was not just missing milestones, but losing those he had previously mastered. He had to be held all the time, would not bear weight on his legs, and still vomited after most feedings. He looked miserable. When the fever came back, we took him to a new ER in hopes of getting a different result. The first thing they did was a shunt series. The head of neurosurgery came down and told us Cole had a shunt infection. It had to be externalized while he received IV antibiotics for ten days, at which point, a new, clean shunt could be placed. Unfortunately, they did not have a pediatric neurosurgeon anymore, and were transferring us to Hopkins, where a doctor had agreed to take his case.

            When we arrived at Hopkins by ambulance, said doctor had decided to go home. After many hours, my husband and I switched- I came home and he went to Hopkins. A resident finally came down, did his own shunt series, and put him on the surgical schedule for the next morning. About the time I was expecting an update on how the surgery went, hubby called to tell me that they took him off the schedule. The neurosurgeon was not convinced. They tested him for everything. We waited all week for consults from other departments that never came. After three days, only the pediatrician came to see him. The doctors at the other hospitals called repeatedly to convince Hopkins to externalize his shunt, but no dice. The fever was gone, and his symptoms must be caused by five other things. I always thought they were to try to see how they fit together, not diagnose several different conditions. We left the hospital with several referrals to other specialists, and the feeling that they were relieved to see us go.

            After a few weeks- you guessed it- the symptoms were back. This time with a fever of 105. I lost it. I couldn’t go back. His pediatrician felt bad, but said he had to go. I knew that, of course, but just wasn’t sure it would ever end. (I can't stress enough how helpful Dr. Mannan was during this time. He called me every day to check on Cole's progress when he was at a hospital he did not work in. He always trusted my instincts when I told him something wasn't right.) This time, we went directly to the doctor who had helped us the first time: Dr. Yaun. We had avoided the hospital because of mistakes made by the staff during our first stay there, but we were getting nowhere with Hopkins, and Dr. Yaun promised to oversee his care herself. He was seen right away, and surgery was first thing the next morning. The infection was so bad that she could visibly see the bacteria. Tests further confirmed that it was a staph infection.

Cole at Children's while the shunt was externalized.

            How had that caused the scrotum to swell? Well, it turns out, we are pretty smart parents. Exactly what we had thought- that the belly could not absorb the infected fluid and was pushing the fluid through the hole. The infection was irritating his bowels, causing the vomiting, diarrhea and constipation. The reason he would get better was because of the antibiotics. It would clear the infection enough to reduce the fever, but because the bacteria was living on his shunt, it would spread as soon as the antibiotics were out of his system. Because of the damage to his abdominal tissue, Cole had to have a VA (ventriculo atria) shunt placed. This meant the tube went to his heart. Since you can’t have extra tubing in the heart, he would require surgeries to lengthen the tube as he grew. The goal was that when it was time to lengthen the tube, to check his abdominal cavity and see if the tubing could be placed there again. This would reduce the amount of surgeries and risk of infection. Think about it: the infection went from his brain to his abdomen, but what if the tubing had been in his heart?

Cole with Dr. Yaun at his his check up

            At his post op appointment, the doctor told us how worried she had been about Cole. She said his recovery was a miracle, and that his brain appeared to have fully recovered from what she was sure was going to result in permanent, severe brain damage. When Cole was three, the tube was placed successfully in his abdomen. Since then, the only issues he has had are gastrointestinal, which we believe are a combination of genetics and the tubing irritating his belly. He goes to Kindergarten with his sister where he excels at everything. He loves to play, “read”, and ride his bike. The only restriction he has is that he cannot play football. Cole knows about his shunt, and does not feel weird about it or shy. It is just something he has- like his glasses.

            Many parents of children with hydrocephalus have similar stories, and some are even worse. This taught me that I have to fight for my kids’ rights, and just because a person has “Dr.” at the beginning of their name, does not mean they always know the answer. If you have a child with hydrocephalus, and are concerned about the quality of care your child is getting, don’t hesitate. Get a second opinion. There are misconceptions that doctors will not touch a shunt they didn’t place, and that is ridiculous. What if you moved out of state? Or to a different country? If it doesn’t make sense to you, then get a second opinion. Do your own research. Empower yourself so you can make informed decisions instead of assuming the doctor knows everything.

            If you are a friend or family member of someone who has hydrocephalus, or has a child with hydrocephalus, give them a hug next time you see them. Many people with hydrocephalus have long term effects: severe headaches, pain at the shunt sight, abdominal trouble, eye problems, depression, vertigo, and multiple (as in many over 50) surgeries. There is no cure, and the only treatment is shunt placement- which has many risks.

            Show your support by wearing a light blue ribbon this month- the official hydrocephalus color!