Hard times

Hello Everyone. I see that thanks to Wessel and a caring offline friend, some of our latest news has made it online. I did not mean to leave this blog high and dry...but like a lot of first-time mothers, I had no idea how all-consuming it is to care for an infant. Now I know why my friends laughed at me when I talked about the "projects" I was going to get done around the house during my maternity leave!

Those first few weeks after we came home from the hospital were surreal. It seemed that no sooner had I finished feeding, catheterizing, diapering, and breast pumping...than it was time to start the whole thing over again. It was a labor of love for sure, yet labor it was. I got so sleep-deprived, I was hallucinating that the baby was in bed beside me one night and kept saying, "Here she is" to my husband and patting the covers. After that, he started taking one "night shift" so I could get some rest (grin).

Still, Madelyn seemed to be thriving and we had no idea what we were in for when we went on January 9th to Children's Hospital for our first myelomeningocele clinic. Spina bifida children have to see numerous doctors, and clinic day allows us to schedule all the visits the same day. The previous Thursday, we had noticed her making a sort of "whistling" sound when she was crying, but it went away when she calmed down and was not too loud, so we decided to wait until clinic to see a doctor about it. In fact, we'd nearly forgotten it and I was telling the pediatrician about Madelyn's severe diaper rash, when my husband remembered to mention the whistling sound.

To say that the professionals were concerned was an understatement -- they had Madelyn's neurosurgeon in the room in just a few minutes, and before we knew what was happening, Madelyn was being admitted to the hospital for a stay that ultimately lasted two weeks and three days.

It turns out that the whistling sound is called stridor, and we should have been watching for it all along as a symptom of Madelyn's Arnold Chiari II malformation.

The majority of spina bifida children have Chiari II, in which the brain stem extends past the base of the head, down into the spinal column. Of those children that have Chiari II, most are asymptomatic, and some may never know they have the condition. However, Chiari II is extremely scary for a spina bifida infant because, when the baby does have symptoms, these can be life-threatening. In infants, the symptoms can include vocal cord paralysis (which causes stridor) with blocked airway, swallowing difficulty, and respiratory problems. In older children, symptomatic Chiari II is not usually so serious, but it is the leading cause of death for spina bifida infants under the age of two years.

This was one of the main reasons why I wanted fetal surgery for Madelyn, since early data regarding the surgery suggested that closing a baby's lesion in utero might reverse the development of the Chiari II malformation in the fetus. This never happens in a full-term infant whose lesion is closed after birth, so if they prove this benefit of the surgery it will probably be routinely offered to women pregnant with spina bifida babies. But as you know, I failed to get the surgery for Madelyn and now she has symptomatic Chiari II. Have I mentioned the word "guilt" on this blog yet?

Anyway. Madelyn's first tests were to see whether her shunt was working properly. Scans did not show any significant enlargement of her ventricles, but our neurosurgeon is very thorough and chose to "tap" the shunt. This meant putting a needle through the skin of Madelyn's scalp and into the shunt, to see whether the fluid being drained by the shunt is flowing properly. In Madelyn's case, the fluid was flowing very slowly. The doctor theorized that fluid back-up was causing downward pressure on her brain stem, resulting in the stridor symptom.

So, on Tuesday night Madelyn underwent a "shunt revision" surgery, which is basically exploratory: The surgeon goes in to inspect the shunt and tubing to find and correct the blockage. Among those children who have shunts, this surgery is fairly common -- although unfortunately for Madelyn, kids who have one shunt malfunction tend to have more than one, so we can expect to repeat this surgery again; perhaps many times in her life. Our doctor found that the small tube leading from Madelyn's brain to the shunt had clogged, and she was able to clear it.

The next few hospital days were spent waiting to see if the shunt correction would resolve Madelyn's symptoms. She was given steroids to decrease swelling around her brainstem, in hopes that this plus the shunt surgery would reverse her symptoms. The other alternative was a cervical laminectomy, in which pieces of bone would be removed from Madelyn's cervical vertebrae (making them a "U" shape instead of an "O" shape) in order to "decompress" the brainstem. This is a serious surgery with numerous risks, especially in an infant, and we wanted to avoid it if at all possible.

Meanwhile additional tests were done, and other problems surfaced, all related to Chiari II compression of the brain stem. Madelyn had a swallow study that showed she was aspirating some of her food into her lungs; a chest x-ray showed an area of aspiration pneumonia. Swallowing difficulties are a common symptom of brain stem compression. She also has gastric reflux, where her food "backwashes" up into her throat, causing pain and regurgitation.

A scope of Madelyn's throat showed the partial paralysis of her vocal cords, which was of great concern to us. As it was, Madelyn had a sufficient airway though she made a racket while breathing; but if the symptom progressed and her vocal cords became paralyzed in the "closed" position, she might need a tracheotomy to breathe, or in the long-term a cordectomy, where they would remove one vocal cord to provide a better airway. Worst of all, one night a pediatric resident noticed long periods of sleep apnea, where Madelyn would stop breathing in her sleep for as long as eight seconds.

It was all very frightening, especially when I started reading about symptomatic Chiari II on the Internet and saw some very scary statistics. One source said that about 23% of infants with severe symptoms die before age 2, mainly from respiratory arrest or pneumonia. There was one day that I spent mostly in tears, as the bad news came in waves and I became desperately, if irrationally, afraid for Madelyn's life. I was much relieved when the neurosurgeon told us that in her view Madelyn was a "moderate" case for symptomatic Chiari II, and that "She's not one of those I'm afraid I'll lose in the first couple years." My father, a pediatrician, was worried enough that he drove up to see us from several states away...I was glad to have him with us, but scared that he was scared enough to make the drive.

After we'd been in the hospital for a week and four days, the neurosurgeon said we would not need the decompression surgery. There had been some improvement in Madelyn's stridor from the shunt surgery and steroids. One last test result remained to come back -- a sleep study done to measure her apnea. My father had gone home, and I was packed and waiting for my husband to return from the pharmacy where he was filling Madelyn's prescriptions, when a couple of nurses stopped me in the hall and said, "You're not going home tonight."

The sleep study had come back showing that Madelyn had over 120 apneic episodes during the night, and the pulmonary doctors were strongly urging the neurosurgeon to perform the decompression surgery. She consulted with us and after asking a lot of questions about the potential risks, benefits, and outcome, we consented to the surgery. I spent hours crying and cradling Madelyn. Could we really lose this child we'd struggled so hard for and loved so deeply? I knew that we could. Life truly can be that cruel, that pitiless.

The next day was fairly awful -- we were an "add on" to the surgery schedule, meaning they would take us whenever they got a free operating room -- and Madelyn had to be "NPO," or "nothing by mouth" all that time. She's a baby who likes her food; between hunger and the six "sticks" it took to start her IV, she was a very unhappy baby by the time they came for us in early evening. It was the hardest thing I have ever done, giving my baby into the arms of the nurse to be carried to the operating room. I was so afraid I would never see Madelyn again.

My wonderful father turned his car around and drove 600 miles again to be with us, arriving just as we settled down in the waiting room. My mother came as well -- she's only an hour or so from us -- so I had lots of support to get through the wait. They'd told us the surgery would last two to three hours, and between snacks and small talk and CNN on TV, I did all right for the first two.

Then I got out my worry stone, and sat down in a rocker where I could work off some energy. At the three-hour mark, I took my mother's worry stone so I could have one for each hand. And at three and a half hours, I was going bughouse. My husband looked into my eyes and said, "Want me to go check?" I just nodded. By then it was late evening and the volunteer manning the waiting room had gone home, so my husband patiently knocked on a connecting door until someone came, and then asked for a status. The word came back that Madelyn was doing fine, and they were "closing the dura."

We thought that we were home free at that point, but the surgery lasted another two and a half hours. By the last bit I was fighting back hysteria; I was sure something had gone wrong and we had lost Madelyn. My husband, stoic that he is, told me later that he had thought the same thing. Then the phone on the volunteer's desk rang. We all looked at each other...should we answer it? They'd told us a nurse would come out to us with news. It stopped ringing, then a few minutes later rang again. This time my husband grabbed it.

Have you ever had a moment when everything slows down; when your mind takes an indelible snapshot of all that you see and hear and even smell around you, and you know that your life will be forever divided into the time before that moment, and the time after? I'll never forget watching my husband's face as he listened, and knowing that Madelyn was safe.

The surgeon came out to talk to us and explained that the surgery had taken twice as long as normal because there was a great deal more compression of the brain stem than she had anticipated from the MRI images. She had had to remove bone from the first five cervical vertebrae, not just the first three. But she said, "That's actually a hopeful sign, because the more there is to decompress, the more hope there is that the surgery will relieve Madelyn's symptoms." One grim statistic she'd given us was that only 60% of children who have the surgery see any benefit from it.

Our baby looked so tiny and fragile in her bed in the pediatric intensive care unit (PICU). She still had a ventilator tube down her throat, and I've never seen anything so heart-wrenching as that little face scrunched up to cry, with no sound coming out. But her little arms were waving and her eyes were open. We were given a sleeping cubicle on the floor -- just large enough for a twin bed and a couch -- and in the PICU Madelyn had her own nurse, so we felt safe leaving her to get a few hours' sleep. By the next morning she still needed the ventilator and her heart rate had dropped a few times in the night, so they decided to keep her in the PICU that day. It seemed to be taking her some time to throw off the anesthetic, and she did seem to be in some pain -- she has a long incision down the back of her neck.

The next night my father volunteered to stay with Madelyn on the floor and my husband and I got to spend a night at a nearby hotel that offers a "caring rate" to families with children in the hospital. It was heaven to sleep in a "real" bed with a "real" shower! Later that day Madelyn had improved enough to move back to our room on the pediatric neurology floor, and her uncles came to see her. They were surprised at how good she looked for a baby who'd just had brain surgery!

We stayed in the hospital three more days for monitoring, and Madelyn was amazingly improved -- we heard stridor only very occasionally, and she was more awake and alert than she'd ever been. A follow-up swallow study showed that she was no longer aspirating her food, and the doctors felt completely safe sending her home with us.

Unfortunately, since we've had her home we've heard the stridor coming back a little bit, and we're a little worried. We have been told it could be from surgical swelling and we should just keep an eye on her...but last night I was so stressed out, I felt short of breath. It is a crushing load, knowing that if I miss the early symptoms of a shunt malfunction or of brainstem compression, Madelyn could be permanently harmed. Or worse. In early stages the symptoms are so subtle. Lethargy, irritability -- how much sleep is too much? How much crying is too much? And the stridor is hard to quantify because it comes and goes.

Somehow I'm going to have to find a way to mother this child and keep her safe without all the joy going out of it. And my return to work is only two weeks away. Please send us prayers if you can.