PKU and Italian Hospitals

We’d been home with Adam for about a week when my husband got a call from a nurse at the base hospital. They said some of his test results came back abnormal and that they needed to see him right away and had scheduled for us to come in the next morning at 10 am. My hubby was still on paternity leave and informed them we’d be there. However, upon informing me of the appointment, I was slightly concerned. My hubby had forgotten to ask for any further details, so I naturally called them back so we could be better prepared for what to expect the nest morning. The nurse informed me that his newborn screening blood test had returned positive and that further testing was needed to verify that it wasn’t a false positive.

 I hopped onto the computer and began freaking out. I asked google what the newborn screening test tested for and it said, “serious developmental, genetic, and metabolic disorders”. Eek! It could test for up to 40 disorders and I scrolled through some possibilities. Cystic Fibrosis? Sickle cell disease? Autoimmune diseases? Which one was he positive for?

The next morning the doctor informed us that he had tested positive for PKU. My mind quickly ran through all the disorders I’d researched the night before. I recalled PKU. That was the one requiring a strict diet in order to avoid permanent mental handicap due to the fact that his body didn’t make a certain enzyme to digest protein and it would instead dump toxic levels into his bloodstream. Yikes. Mental retardation? It was hard to imagine looking down at Adam. He was so alert and he would thoughtfully follow us with his eyes. Granted, I’m sure every mother thinks her child is brilliant, and while I don’t think he’s the smartest child to ever walk the earth, looking at him I found it hard to believe he was on the track to mental retardation. I asked the doctor what was next. She said she didn’t know; she had never actually had anyone test positive for PKU. Awesome. But as I recalled the stats I had looked at, PKU positive people are one in every 15,000-20,000 births (depending on which stat you’re looking at), so no it wasn’t likely they had experienced one on this base before. She said there was a hospital nearby that specialized in genetic and metabolic disorders and they were going to contact them and ask what sort of blood tests they wanted in order to narrow down what sort of PKU it was and whether or not it was a false positive.

We went home, awaiting a phone call. They called that evening and my hubby answered. “Who was that and what did they say!?” I asked. Troy appeared in the doorway. “You’re not going to like this…” he said. Lol. Love it when conversations start that way. In short, there was a hospital two hours away in Padova that specialized in metabolic and genetic disorders and the specialist had requested we come in for testing and stay for the next three days. Three days?? Why did they want us to stay for three days?? We spent the night packing up our bags and looking up directions to the hospital. Our appointment was at 9 am and we left at 6:30 am to factor in getting lost and needing to pull over to feed Adam.

We did get slightly lost, and the toll on the road there was over 8 euro, but we finally arrived with plenty of time to spare and were soon on the proper floor of the hospital. The nurse at the front desk didn’t speak any English, but she had been told about us and admitted us to room 7-8.

The room was painted a sweet tart orange and had two colors of blue furniture. The woman indicated this was our room. Troy wheeled the stroller to the far side and the woman motioned to move it and said no. We were confused. She pointed to other places we could put it. Then I realized that there were two colors of furniture because the front half of the room was for one family, and the back half of the room was for another. We got the front half of the room. Not long after, a woman and her baby came back from somewhere and she pulled out all their things from the cupboards and set up her space again. Sharing that small room was difficult. She had the back of the room and thus had control of the windows. It was sooo hot and stuffy in there, it was a sickening feeling. Also, her baby was a nonstop crier, and she didn’t seem to care. She would put him on the bed and stick in the pacifier and then plug in her headphones. Her child would scream and wail, despite the pacifier in its mouth. When he spat the pacifier out, she would simply replace it into his screaming mouth and it would sit there, loose as his large mouth wailed around it. Troy noted that the whole day we were there she only fed him once.

Our roommate’s child’s screams were driving us crazy, but Adam slept through them in his stroller. The walls must have been thin because noise carried very well. One could hear the screams and cries of numerous other babies and children as well as the constant commotion in the hall. There was a loud speaker that would frequently summon nurses to the front desk, buzzers for the doors going off, carts being wheeled down the halls, and the general chatter and sound of people bustling down the crowded hall. Three days here? I feared I might go crazy.

Our room in the hospital. We had the front half. 

Although we’d been there since nine, we didn’t get seen until past eleven. In the meantime one of the nurses gave me a tour of the ward. It was a long hallway with many sections. The right side was entirely rooms with patients, and the left side of the hall had rooms for the doctors and nurses. There was a room where they provided diapers and recycled them. There was a room that worked as the milk kitchen were they made special formulas for the babies. She informed me when they figured out his diet specifications I would pick up his milk order here. The tour ended with the blood lab near the end of the hall. I looked inside. From the ceiling hung paper dolls by string. I glanced down the hall where brightly colored Venetian masks hung, and the large waiting room where Winnie the Pooh drawings hung. What kind of sick joke was this? They hung mini bodies in the room where they draw blood!? Why couldn’t they do snowflakes or something???

At half past eleven they escorted us to this room with the mini hanging bodies to draw blood from our little one. Our sweet happy camper had no qualms about being placed down on their table under the bright lights. He continued to be genial as they began to swaddle him. Wait, swaddle him?? I watched as they wrapped him up, leaving only his head exposed. Then I was freaking out. No heel stick?? From where were they going to draw the blood? One nurse held down and turned his head to expose his neck as the other nurse had a hand on his body and another hand preparing the needle. Oh my. Those vampires were going to feast off his neck. I watched him make his genial faces and flash his half smiles, completely unaware of what was about to happen. Shoot, I couldn’t be here for this. I gave Troy a look and he grimaced and nodded as I left. I walked clear to the other end of the hall, where I could be surrounded by the chorus of screams from other babies and would be unable to hear mine own.

All this and he's still smiling. What a guy. 

Some time later my DH came back to our room and handed me our tiny bundle, who still had a wad of gauze on his neck, and informed me that we could expect the lab results around 4:30. I prayed his results would come back negative, that the first test had been a false positive and that we’d be able to go home that night, cook dinner in our kitchen, and sleep in our beds in our peaceful house.

Troy took that opportunity to go and get some food, as we were both ravenous. He was gone for a very long time, because as I mentioned in a previous post, Italy doesn’t do “take-out”, thus making it very difficult to find food that he would be able to bring back to me. He was unable to find a grocery store in this part of the foreign city, but eventually he found a Panini stand that let him take the sandwiches to-go. However there was some miscommunication, and although the woman had many ingredients to offer, we ended up with two dry bread sandwiches with a few slices of meat. Troy said he had asked for veggies and cheese, but somehow they didn’t make it onto the final product… haha. We’re still working on our Italian. Regardless, we downed the food in no time, along with two apples he found in the car.

Adam, chillin in the boppy. 

The hospital eventually brought by a tray of food for me to eat, which was quite different than the food offered by the American hospital on base. The food was much healthier, but the portions were far smaller. I had a bottle of water instead of the two juice boxes, and there was no sugar or dessert sort of food to be found. Instead there was a small bowl of pasta with a bread roll and an orange. In another packet was a chicken patty. I knew I’d be starving with three meals like this every day, and my appetite had significantly increased ever since I started nursing Adam. However I was still holding onto the hope that it was all a mistake and that we would get to go home that evening. Adam was still sleeping in his stroller, ready to be wheeled out at a moment’s notice and our bags lay unpacked underneath the stroller.

Four thirty came and went, and thankfully so did our roommate. She and her screaming, starving child departed, giving us the room to ourselves. So, we slept. I pulled out the fold up bed for parents and the three of us fell fast asleep. Sometime after 6 the main doctor and his main nurses came in, rousing us from our slumber. I held my breath hoping for good news. We didn’t get any. Apparently his Phenylalanine levels were dangerously high and his diet was going to have to be adjusted. Also we may have to stay for the next week or more as they made adjustments to his diet and took his blood every four hours. My heart sank thinking about the effects this would have on Adam’s life, and the long, hungry, blood-drawing road ahead of us.

At this news, I finally consented to set up our gear and set Adam down in the crib. My DH wasn’t permitted to stay with us, so he left for home. It was also his last day of paternity leave and had to go to work the next morning. I was jealous he got to leave this crazy/noisy place and go home to our bed, house, and food. We made a list of things for him to bring back with him the next time he came. At the top of the list was food.

Found my snuggle buddy for the night! 
Looks like he already said his prayers too. Such a good kid <3

           That night wasn’t so bad as it was just Adam and I in the room. I had been informed that we’d likely get a new roommate the next day, so I relished the time and peace we had together. The hallway was bustling and didn’t start to die down until sometime after 10 when they dimmed the lights. I had had the window open for fresh cool air, but the sirens were wailing all night long, so I ended up closing it, leaving Adam and I holed up inside.

The next morning proved to be quite busy, and I realized we wouldn’t be getting much rest during our stay here. There were always things to be doing and nurses coming in to see us.

Breakfast finally came sometime after nine. I had been so hungry and excited, only to be rather disappointed when it arrived. A cup of coffee and two pieces of toast were all that was on the menu for breakfast today. And when I say toast I don’t mean two slices of American sized bread toasted, I mean Biscotti. These two pieces were smaller than my palm. There was a small pad of butter for them and a wee bit of jam. Darn, it was going to be a hungry day.

Just as I was opening the Biscotti packet, a nurse came in saying it was time to weigh the baby. So we undressed him and weighed him, which naturally woke him up from his peaceful deep sleep. After she left I worked to calm him down. He soon stopped crying and I went back to my toast. Just as I was opening the pad of butter, another nurse walked in, saying it was time to draw his blood. Bother. So I followed her to the room where they do such things and they undressed and positioned him, once again waking him from his sleep. I was rather distressed I had to be present, as my DH wasn’t there.

They stuck him and he screamed as they squeezed his ankle to get enough blood drops to fill the row of circles on their chart. This was dreadful, and I hated the idea we would have to do this every 4 hours for who knows how long until they got the results they desired. I stood out of view and didn’t speak. I didn’t want him to see or hear me until they were done and I could take him away. As they handed him back they informed me he’d probably be hungry and feeding him would be a good idea. He was hungry and feeding him soothed him back into sleep. Then we were free to head back to our room.

As I walked down the hallway I saw the food lady collecting the breakfast trays from the rooms. NO! I hoped she hadn’t already taken my toast. It was meager, but it was all I was getting for the next few hours. I weaved through the crowded hall and into our room to find that thankfully it was still there. I put down my sleeping baby and opened the jam. As you can imagine, I didn’t make it through this either. Another nurse walked in saying it was time to administer the medicine. She held a syringe with 4 ml of a gooey yellow fluid.

*sigh. No breakfast for me, no sleep for my little one. So once again we woke him and she had me hold him while she attempted to administer the medicine. I must say, she was rather terrible at it. I offered to do it instead, hoping perhaps I could alternate milk and medicine or perhaps fool him altogether to simply get it down in a few good gulps, but I don’t think she understood my intentions due to the language barrier. She seemed to think I wanted to consume it and kept smiling and saying, “no, for bambino!” ah well… She would administer the most miniscule portion right onto his lips or just barely inside. He would then scrunch up his face, purse his lips, and make all manner of disgusted faces. The process was taking forever and each time the medicine level seemed to stay at 4 ml. “Ah!” she said, pointing her finger toward the ceiling. She had an idea. “Acqua!” and she ran off. Water?? I thought one wasn’t supposed to give babies water… oh no, she was going to water it down wasn’t she? I could also hear the food lady wheeling closer. I quickly buttered and jammed my toast and set them aside so they were no longer on the tray. Just in time too because the lady came to grab them and the medicine nurse was back, this time with a larger syringe with a lighter yellow medicine. Yup, she’d watered it down. Great, now this would take even longer…

He's such a thoughtful little guy. 

It was a very long process to finally get all of the medicine in, and by the end he was very unhappy. The medicine nurse left, her job finally complete, and I was left to calm him down. I attempted to give him milk afterwards, in the hopes it would help mask the taste, but he refused to open his mouth. I had great difficulty and no success getting him to nurse after that. He refused to open his mouth, or eyes… or fists… for anything. Still, I hoped this medicine would do the trick. If this worked I’d still be able to nurse my baby, our stay in the hospital would be much shorter, he’d have to get less blood drawn, and in the long run he would still be able to eat meat and dairy with us because the medicine would help him digest it properly. However if this didn’t work, we had yet a long road ahead of us.

I rocked and hushed him back to sleep, placing him back on the bed in the hopes that he could finally rest and not be disturbed. I was then able to eat my pieces of biscotti.

Then I had to confront another problem. I had needed to use the restroom for so long now, but I wasn’t comfortable leaving him alone. There were no locks on the doors and it was so crowded and busy I feel anyone could easily slip in and out with a baby and no one would be the wiser. Eventually I was able to flag down a nurse who stood by his bedside while I ran to the restroom at the far end of the hall.

Around 11:30 am our new roommate arrived. A man dressed entirely in orange with reflector tape burst through our door, carrying a baby attached to an IV. Soon the room was filled with nurses and doctors as well as the baby’s mother, speaking in rapid Italian on the phone. I counted 9 nurses and doctors before it became too crowded to count. Nurses were wheeling in many beeping machines, and the medicine nurse was back with a handful of things as well. I heard the mother on the phone say the word ambulance a few times, which confirmed my suspicion that this babe was fresh off the ambulance. Though I was confused why he was in this section of the hospital. After the commotion died down the father arrived. I caught bits and pieces of their conversation, and at the end the nurse informed them they were rooming with an American who didn’t speak Italian. Haha.

Seeing their baby attached to all the tubes and machines reminded me how blessed we were and how it could be much worse. They knew what was wrong with Adam, it had been caught early, now they had only to experiment with diets and medicine dosages in order to get the proper levels. They were unsure what was wrong with our roommate’s baby.

Around one pm, long after our room had settled down, I pulled out my bed for riposo, the Italian version of a siesta. Some time later, a man, tall, dark, and handsome, wearing black slacks and a leather jacket woke me from my slumber, saying he had brought goodies for me. He had gone to the store and purchased my favorite powerbars, brought some homemade foods, more clothes for me and the little one, and upon asking him if he brought a towel so I could shower he replied, “Babe, I brought two.” (Always such an overachiever! I’m a lucky woman) It was great to have him there and have some company, as well as someone to watch Adam so I could shower and get some things done. 

            Troy left very late that night with a small coffee in hand, disappearing into the flurry of snowflakes that had been falling all evening. He made the almost two hour one-way commute for five of our six day stay, on top of going to work.
           On Friday we were informed that we were going to have stay until at least Monday, possibly longer. The days were busy with the pattern of weighing and measuring the baby, taking his blood pressure, administering medicine, taking blood every four hours etc. In between there was never a breather of visitors. I got a visit from a nun, who in her broken English told me about the time she got to go to Hawaii. She commented on how large and grand everything is in the United States. I also got a visit from a very old woman named Norina. I don’t know if she was a volunteer or if it was her job, but she was the “baby holder”. She offered to watch the kids so mothers could have a break. At any other time this would have been great, however Adam was hungry at the moment and he would only scream and cry if I left him with someone else. I thanked her and kindly turned her down, but the old woman looked very sad. She gave Adam many kisses before leaving and heading to my neighbor next door. I realized later that I had the smallest/youngest baby in the ward by far, and she had likely been looking forward to holding a little one vs keeping an eye on a toddler. I felt badly, but there wasn’t much I could do about it.

We also got a visit from an American doctor, working at an Army base nearby. I felt badly because no one had informed me he was going to visit and he had traveled some distance to come and see us. I had had people coming and going every other moment and the visits were starting to blur together. I’m still not entirely sure what his specific function was, since we had doctors on the Air Force base, but he spoke fluent Italian and had had experience with this hospital and had brought a bunch of reading on PKU for me to look through. He gave me his personal cell and told me to call or visit if we were ever in the area and to let him know if we needed anything. That was very kind of him and I definitely benefitted from the information he brought.

Every day a nurse would roll by with a cart of diapers and ask, “Pampers?” I believe the Italians use the word Pampers for diapers, like some people say, “Grab a Kleenex” when really they mean “Grab a tissue” as Kleenex is actually a specific tissue brand. So far, every diaper seems to be a “Pampers”. She would deliver Adam's diaper allotment for the day and roll away. In the evenings there were programs for the kids. On Friday evening clowns came through. They were also very excited to see a baby so small and were thrilled when I let them hold him. They then proceeded to make us some balloon art as they told me of the time they got to meet Patch Adams. I was fairly certain balloons were a choking hazard, but the Italians seemed unconcerned as they placed balloon creatures in Adam's crib and the bed of my roommate’s baby as well. I thanked them and removed the balloon once they were out of sight.

A balloon heart with a mother and baby inside. 

Two of the many clowns. 

          My roommate was very sweet, and she helped me obtain Internet by translating the pages I encountered (so I could finally email family back home, and post this blog post!). She spoke some English and we managed to have several good conversations together. She had difficulty communicating what was happening with her son, as she herself was unsure and the translation hard to manage, but I understand he was having some sort of trouble with sugar and they were also testing various medicines and diets on him. Having a roommate was nice, as we were able to watch one another’s babies so the other could use the restroom or grab a cup of hot cocoa from the hot drink vending machine. She told me that after our hospital stay we were welcome to come and visit her, and that her family lived on the largest lake in Italy. Troy and I hope to take her up on that offer.

As this hospital was a research hospital at a university, there were many young medical students there. A few in particular stopped by my room quite often. One fellow named Alfonso seemed to stop by more than was common, and I was getting concerned we were getting special treatment of some sort just because we were American. However I noticed that each time he left, he would use a different form of goodbye. “See you later!, Until next time!, Have a good evening!” etc. I concluded that he, and many of the others who stopped by frequently, desired to practice their English, as it was very rare they had an American family stay at this section of the hospital. I had many nurses tell me that if we needed anything to ask for them in particular as they spoke the best English in the ward. Lol. Needless to say we were not short of kind people willing to offer us aid. I learned to respond with similar answers to the various goodbyes Alfonso gave me, in the hopes that he would feel validated in his English and confident that those were sayings Americans actually do use and not something simply taught in the classroom.

The Italians were all very kind and eager to help. Every small question we asked they would find an answer to. One evening my hubby and I asked Alfonso if there were any good pizza places with take-out nearby. We had meant it as an informal personal question, as we figured since he worked here he had likely eaten at places nearby or heard of them from other coworkers. “I can ask!” he said, turning on heel and flying out the door. (That’s something he was famous for saying, “I can ask!” haha) He soon returned with pamphlets he had taken from the front desk downstairs. I believe they were someone’s personal pamphlets as they were quite worn and he asked that we return them to someone specific.

Apparently, this is a typical Italian breakfast. 

One morning, after receiving that same two pieces of toast and coffee for breakfast I decided to ask him: “So, do Italians not eat breakfast?” “Si, si, we have breakfast”. He responded smiling. Silly me, I asked the wrong question. “Is this a typical Italian breakfast?” I asked, indicating to the tray of coffee and biscotti in front of me. “Si, si.” He responded. I then later asked my roommate what she and her family had for breakfast every morning, and she said they would have café and biscotti. Oh my. Perhaps that’s why Italians are more laid back and leisurely moving throughout the day, they get a slow start on such small nutrient intake! She asked what we normally had for breakfast and I informed her that a couple eggs and bacon with orange juice were a staple, sometimes with pancakes or fruit or veggies, depending on what we were craving and the food we had on hand. Her eyes were wide. “Oh my, such a big breakfast!” she said. I explained that in the states it was thought that breakfast was the most important meal of the day. She said that dinner was often the largest and most important. (Hence the many courses perhaps?) I’m not sure how I would get going on such small sustenance every morning.

Saturday morning our blood results from the past three days were in, so my hubby was there bright and early so the doctor could pull us out of our room and into the blood lab to discuss them. I held my breath hoping for good news. This time, we got some. Adam had responded quite well to the medicine and his Phenylalanine levels were in a normal, healthy range. There are three different forms of PKU and it was concluded that Adam had mild PKU. (Hallelujah!) The doctor said that children with mild PKU could follow a more relaxed diet than those with moderate or classic (the most severe form of PKU), and that this medicine would assist even further. I was ecstatic that he had responded well, would not be suffering any brain damage due to the brief exposure of toxic Phenylalanine levels, and that he could enjoy bacon with his dad and a sliver of birthday cake every year. I was permitted to continue to nurse him so long as he kept up on the medicine daily. He would need monthly blood tests as he grew up, particularly when he went through growth spurts or we added new foods to his diet, but the breadth of what he could eat looked brighter than before. We worked out some more details and were informed that we would be good to go home come Monday evening and that we were to be given our own room for the weekend. I had quite enjoyed my second roommate, but I wasn’t opposed to having our own room either.

We walked back to our former room elated, and prepared to move our possessions into the new room. Upon reaching the room, I found my roommate crying over her baby’s crib. I asked her what was wrong and she said that her Niccolo wasn’t responding to anything they were trying, and the doctors feared he was going to suffer long-term brain damage. We hugged and cried. She asked what the doctor had said about Adam and if he was going to be okay. I don’t know if I’ve ever felt so guilty for receiving good news as I told her that the medicine worked and we would be going home soon. It didn’t seem fair, and I felt her pain as she stared at her small Niccolo, wondering how this happened and if he would end up okay.  

My hubby and I are very thankful for how everything turned out. Adam is well and the issue had been caught within his first two weeks of life, so there hadn’t been time for much, if any, development to be hindered. As my hubby and I discussed it, we found it interesting how many coincidences have occurred through this process that we were unaware of previously. Just weeks after conceiving little Adam, my DH was offered the option of going overseas to Italy if he reenlisted. My DH had planned to get out and use his GI bill for college, but at the last chance chose the opportunity of reenlisting and going to Italy instead. Here in Italy we are two hours from one of the best hospitals and doctors for metabolic and genetic disorders, and according to the American Dr. Novek (who came to visit me) the Italian Dr. Burlina often goes to the states to speak on such matters, and that we would have been hard pressed to find better or closer care in the states. Also, since we are still in the military, the military health insurance will pay for the medicine Adam needs, which costs $800-$1000 a month. These, and many other things we noticed have woven together up to this moment to let us know that we’re right where we’re supposed to be, and that someone is watching after our Adam, and he couldn’t be in better hands.   

Adam with his Daddy <3

If you want more detailed information on PKU, here are some good links:

• http://www.intelihealth.com/IH/ihtIH/WSIHW000/32193/48811.html
• http://www.mayoclinic.com/health/phenylketonuria/DS00514