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Josiah (2007) |
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Jo Jo and Baby (Mr. Socks) |
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Josiah (2002) |
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Josiah with Mom (2002) |
My pregnancy was pretty uneventful. We went to the hospital at 7:00 am on June 27, 1991. The doctor broke my water to induce my labor, but after eight hours it had not progressed. So reluctantly I allowed them to induce using Pitocin, and had an epidural done. After that my labor progressed fairly quickly. We were encouraged that the nurse kept commenting that the baby's heart seemed very strong, and that its heartbeat was very regular.
(Incidentally, that night our church was meeting as usual for kinship, a kind of bible study/worship time. While I was in labor, one of the men in the group who had a prophetic gift spoke this word, before Josiah was born. He said, "This is a child obtained at a high price." I believe he spoke of the great sacrifices that we and other families make when their child is born with a serious disability. We would have to pour our lives into him to keep him alive. I had also, early in my pregnancy, felt that there was something wrong with the baby. I prayed, "Lord, I don't care what is wrong with this baby, I will take care of it, no matter what it takes. Just let it live.")
I only pushed for about 15 minute when the OB said to stop pushing. I was not aware at the time, but apparently the baby's umbilical cord was wrapped around his neck. Very smoothly and confidently he unwrapped the cord, and within a few minutes Josiah was born. We were excited to find out that he was a boy. I kept telling his father, "It's a boy! It's a boy!", but he only stared in disbelief. Then the OB asked Dean if he wanted to cut the umbilical cord. As he had done with our daughters, he cut Josiah's cord. Again, everything seemed perfect, and unbelievable. Within a few seconds of cutting the cord our lives changed forever..
The doctor said, "The baby is not breathing", though he was obviously alive, moving and thrashing about. He called the nurses to take the baby to the warming table to bag him (assist in breathing). I said to Dean, "Call Dick and Martha!", our pastor and his wife. They began to pray for our son's life. It was four minutes before he would breathe his first spontaneous respirations. They wrapped him in a blanket and brought him over and said to me, "Kiss him." So I did (We made eye contact. His eyes were so sad and he looked very frightened, and seemed to understand the moment) The nurses whisked him off to NICU. Again the nurses said, "At least his heart seems very strong and regular." I would not see him again for six hours.
Those hours were some of the longest and loneliest hours of my life. Dean had gone to get coffee and had stayed away with a friend who ministered to him. I was alone with my fears and unable to get out of bed to find out if my baby was okay. I was so afraid, and grieved as though the baby were dead. Doctors and nurses came in and out, but with little information as to what was wrong with my baby. They were performing test after test. Finally, after six lonely, frightening hours the neonatologist came in to speak to me. He told me that Josiah had somehow developed pneumonia, and that he should be able to come home in about five days. I was then taken in to see him. He was lying on the warmer with IV's and wires, and a plastic hood that served as an oxygen tent. He was a big baby and the hood barely fit over his head. He was sucking on the side of it for comfort. I could not pick him up and comfort him. I could not nurse him. All I could do was sit by his bed and touch and talk to him.
After about 36 hours he came off the oxygen and was saturating at about 99% (that's the amount of oxygen that is getting into his blood). The doctors still maintained that he had pneumonia. On Monday, July 1 we were told he had a heart murmur, but not to worry, it was probably innocent. They wanted to check it to make sure, so they ordered an echocardiogram. The results were that he had a very large VSD (hole in the ventricular septum), and that they could not visualize the pulmonary artery. When the cardiologist came in he asked me what I had been told. I said to him that I was told that he might have a minor heart defect. Well, to my surprise he said, "Yes, in fact it is a very serious heart defect." As he described to me the type of defect that he thought Josiah had, the tears ran down my face and I was overwhelmed with fear and grief. He further explained that he wanted to do a heart cath (angiogram) to make sure of the diagnosis. Of course I agreed to the procedure. He warned me of the risks, but there was no question whether or not we would have it had done.
I called Dean, his father, and he came in immediately. After the cath they led Dean and I to the dreaded parent conference room. There the cardiologist, Dr. I. Hunter Crittenden, explained that the results of the cath confirmed his earlier suspicions. He explained that Josiah was born without a pulmonary artery, and that he had an enlarged aorta that had several collateral veins leading to his lungs, providing pulmonary blood flow. He also said that Josiah had a very large VSD (ventricular septal defect) and a right aortic arch. Our first question was, "Can this be fixed, or will he require a transplant?" To our great relief, we were told it could be repaired, and that it would be achieved in a series of surgeries. The plan at the time was to manage it using heart medications, Lasix, Aldactone, and digoxin. They hoped he would stabilize and that he would be able to wait unit he was older to have the surgery so that his collaterals could grow, and he would have a better chance of surviving the surgery.. They would observe him in the hospital for a time.
After observing him for a week (his second week) it was decided on a Saturday morning that he was in need of emergency surgery. The collateral vein feeding his left lung was too large, it was flooding his lung with high volume and high pressure blood. This was causing damage to his lungs and his heart. That also happened to be the day of a full solar eclipse in Southern California, which occurred while Josiah was in surgery. I felt it was a sign: This darkness will pass. Everything will be all right. And it was. So that morning Dean and I waited for four hours as they opened our son's chest and placed a "rubber band" around the large left collateral vein. The original size was 5 mm. They reduced it to about 3 mm. It was easy to see after the surgery that he was doing much better. He looked like a new baby. Soon after they let us in to see him , the right side of his face turned beet red. The left side was white. The surgeon could not explain this, but we found later on that it was probably "sympathetic nerve" damage.
After another week of observation, 21 days total, we brought him home. He was on Lasix, Aldactone, and Digoxin. When he was diagnosed we were told that Josiah had "Persistent Truncus Arteriosus Type IV". Later we were told that this was the "antiquated term" for the disease. It is now referred to as Tetralogy of Fallot with Pulmonary Atresia and a Large VSD, and Multiple Aorticopulmonary Collateral Arteries (MAPCAs). He is, after four surgeries at UCLA Medical Center (by Dr. Hillel Laks), corrected.
Josiah has now developed pulmonary stenosis at the base of his RV to PA conduit, aortic insufficiency, pulmonary insufficiency, and mitral valve, and tricuspid valve regurgitation. He also has right hemidiaphragm paralysis and right bundle branch block. He is now 10 years old and a happy guy. He faces more surgery in the future, but hopefully we will be able to put it off for a few years. Thanks for reading our story, and God's richest blessings to your and your family!
July 2007 Update
Josiah is now 16 years old and is doing well, heart-wise. He had a redo of his correction in August 2003 at the age of 12. They did do more repair at that time as well. First Dr. Laks of UCLA dissected his calcified homograft (PA) and patched it with Gore-Tex. He then added a Mosaic porcine valve to the PA, the first time Josiah ever had a pulmonary valve. Then Dr. Laks resected Josiah's aortic aneurism and "pulled it down" and reattached it to his heart (scary), and then repaired his aortic valve. The repair went very well, and he was out of the hospital helping a friend water their flowers after five days! That was his shortest recovery ever. He is only on aspirin at this time for heart meds.
In November of 2005 Josiah was put on Concerta for ADD (supposed ADD, I say) and within the first week started complaining of numbness, tingling, and weakness in his right hand/arm. The ped, who is a very good doctor but just missed this one, said it was nothing and sent us home. I discontinued the Concerta just in case—I didn't like the way it changed his personality anyway. The symptoms did not go away and persisted through the year. As usual, Josiah continued to have difficulties staying awake at school, and the pc's always said there was no medical reason for it.
In August of 2006, Josiah woke up one day with sudden vision loss. He had been suffering headaches all week, but hadn't told me. I took him to the ER concerned that he had thrown a clot into his retinal artery, and the peds ophthalmologist at the hospital agreed. Strangely, though she said time was of the essence, the ER docs decided to send him home. I then said, "Don't you think we should admit him, in case it is a clot? Maybe he could throw another one in a more dangerous place, or maybe we can treat this to recover some of his vision". Again, strangely, the agreed and did what I said. It was really weird, like it hadn't occurred to them to do anything about it. He spent that night in a hyperbaric chamber (it scared him, but as with all things, he handled it well). So, Josiah was admitted into the PICU and started on heavy-duty blood thinners. Injections of a heparin type drug (can't remember its name), and high dose coumadin went on for about six days. An MRI was done the second day there. Josiah was afraid of the MRI because he has so much metal in his chest, and though the docs and radiologist said it was not a problem, he was pretty freaked out, so they sedated him with IV sedation. The Radiologist did not find a clot, but found seven lesions on his brain, one or two of which looked like old strokes. It was nice to have proof of what I already knew. After surgery number two I knew he stroked, though everyone denied it and would not do any neuro exams. Anyway, the radiologist looked at the other lesions and said, "If I didn't know better, I'd say this is Multiple Sclerosis."
He said that because MS in children and teens is extremely rare. In fact, the only peds MS doctors are at six peds MS centers in the US. I was informed of this and the neuros felt it was not likely MS, but they repeated the MRI to do a much more in-depth exam. The exam gave the same results, with another lesion on his spine. They did a spinal tap to check for oligoclonal bands, which is a sign of MS, but for kids its not unusual for them to be absent. Josiah had a few borderline Og bands. It was decided that the mostly DX was MS, and the peds retinal specialist agreed. There was no clot, but there was optic neuritis, one of the most common symptoms of MS. Josiah's vision in his left eye had gone from 20/15 overnight to 20/400. He was treated with high dose IV Solu-Medrol for five days (1000mg) and sent home. He had a severe headache from the tap, and was readmitted overnight for treatment and went home again. When we were sent home we were not given any instructions other than the neuro would contact us. After a few weeks I finally called them because of the uncertainty and because Josiah was starting to have pain and blurring in his other eye. He could go completely blind and it would be uncorrectable. The peds neuro we were assigned was annoyed and said that pain in his eye was not a symptom, and even if it were there was nothing they could do to stop or slow down the MS. I knew better because I read everything I can get my hands on when it comes to Josiah's health issues. Early treatment is imperative for slowing down the disease and lessening the amount of disability and the newer drugs (Rebif, an interferon) can even cause old lesions to shrink or disappear. The neuro lied to me and said I was not correct. So, I insisted on an appointment. We came in and they did a test that detects possible MS symptoms of optic neuritis, and the adult MS specialist was called in. He said, "This really looks like MS. I want to start treatment right away." The peds neuro that had lied to me and dismissed my concerns came in with his tail between his legs (so to speak) and was very kind to Josiah from that moment on. So another course of IV Solu-Medrol (at home with a PICC line), and soon he began his Rebif treatment—injections three days a week sub-Q.
He is stabilized now regarding the MS, and his vision has come back to 20/40, a remarkable change. It is not correctable, but he doesn't seem to notice it much. He had a PC appointment about 5 months ago that showed some thickening of the left ventricle, which they were concerned about. I feel that it might have been the extra work his heart had to do in order to cope with all of the steroids. Also, the interferons can damage the heart muscle. We are due for another check now, so hopefully there is an improvement and maybe we can say it was the Solu-Medrol and not the interferon or some other problem. Otherwise, his heart seems good. The PC did ask, "Are they sure its MS? These kids often have those types of lesions on their brain" (CHD Kids). I agreed, but the fact that he had optic neuritis tells me its more than the typical CHD kid's lesions.
Josiah has such a positive outlook on life, and though many people with MS struggle with depression, Josiah seems to push right through. I have to ask him periodically how he's doing, is he depressed, does he feel sad. Recently he answered, "I don't understand why people get depressed. I just get over it." He is the happiest and most positive person I know, and is extremely caring. At 16 years old, he is a freshman and on homebound tutoring due to his extreme fatigue (MS). But he is still my baby, and often still crawls up in my bed at night for a snuggle because the shots he takes give him flu-like symptoms. The snuggle helps him get through the chills and aches. I worry almost every day, fearing the MS and his heart. I feel kind of mad at times because I feel he has been cheated the quality of life that he worked so hard for—I fear he will worsen. I really get scared sometimes, like I never did before. What keeps me going is God's promise to me when he was first born from Psalm 91 that says, "Because he loves me", says the Lord, "I will rescue him. I will protect him for he acknowledges my name. He will call upon me, and I will answer him; I will be with him in trouble, I will deliver him ] and honor him. With LONG LIFE will I satisfy him and show him my Salvation."
I am so happy to have him in my life. Before he was born I had the feeling he was sick. I said to God, "I don't care what is wrong with him. I will take care of him no matter what is wrong—just let him live." I am committed to that promise and to Josiah and will care for him my whole life if that is what he needs.
Blessings,
