On The Road To Recovery

Yesterday was another half day spent at UCLA to see Bambino's Immunologists. He is just bouncing back from one of his worst break out this winter so he was all clear for the doctors. Fortunately, this doctor knows him well by now. He knows the intricacies of his disorder.

Whenever we see a new doctor and he is all clear, we would make it a point to take photos with us or the doctors would be wondering what the heck do we need their services for. Sometimes, when his skin is at his clearest, it is hard to believe that it was ever really bad.

These are photos from his most recent breakout. It's hard to believe it was just a mere one week ago.




He was red and peeling all over. His skin was painful to the touch. He was so miserable. Diaper changes were not happy events. He would be crying and screaming all throughout. I had to lather him up in thick layers of Aquaphor to keep his skin hydrated and that bothered him a lot.

We found out from yesterday's visit that he has lost about 7 ounces from last month. He is now back to 16 lbs even. His weight is just sliding backwards and it's heartbreaking. One of my frustrations was the difficulty in making an appointment with the GI doctor - something he really needs right now.

The Immunologists promised to help us get in to see the GI sooner. The last time we saw the GI, he insinuated that we might have to put in feeding tubes if he goes on like this - refusing to eat and continues to lose weight.

Since one of the current major concern is the itching, which usually happens at the tail end of a breakout (similar to a wound just about to heal), the docs prescribed a new antihistamine called Periactin. This rang a bell to me. I remember taking an appetite stimulant called Periactin-Vita as a child to help me put on some weight. I told the doctors that, and they said one of the "side-effects" indeed is increased appetite and weight gain.

It's an antihistamine so it helps with his itching and runny nose caused by allergies; it makes him drowsy so it will help him sleep better; and it's an appetite stimulant!

It sounded like a magic potion to me! It addresses practically every one of our concerns right now. I am hopeful.

Here's last night's photo of him. Besides the weightloss and the night time itching, he is back to his usual sunny disposition. And Mommy couldn't be happier.



Oh, and P.S.: as I'm typing this, he is halfway through his fourth 3oz-cup of formula! That is more than what he has ever consumed in a single day, and it's only 6pm! Does this mean then that the "side effect" was working? If I can make him drink 5 cups today, I would be the happiest Mommy on the block!

The $160 Cream

Dr. Justine Park at Children's Hospital prescribed a new cream for Bambino called Dovonex. We were surprised at how much it cost - a little more than $160! Insurance covered only $56 and we paid for the rest. The doctors said it's a prescription strength Vitamin D and only to be used every four days.

We're hoping that we would see results considering how expensive it is.



These are the the current topicals that I use on him: Derma-Smoothe on his scalp, Cutivate on affected areas, Bactroban on areas most susceptible to infection (diaper area & open sores), Nystatin or Ketoconazole when there's a possibility of fungal infection - especially while he is on oral antibiotics like now. I also use Refresh and Erythromycin ophthalmic ointment on his eyes to avoid keratitis. Epiceram is another costly cream we used for a day and had to stop because it seemed to sting.

During bad break-outs, I stop using steroids or any other harsh chemicals on him because of the danger of systemic absorption due to his compromised skin barrier.

These are what I use regularly, whether his skin is clear or otherwise:




Robathol Bath Oil added to his bath water, then I lather him up generously with Aquaphor while his skin is still damp from the bath.

Fortunately, Bambino gets a free supply of Aquaphor from this great company Beiersdorf. He goes through each jar so fast because he is practically coated with it all the time!

Leaving UCLA

Los Angles Children's Hospital Lobby

I've always considered ourselves lucky because of the fact that we live relatively close to renowned hospitals like UCLA, Cedars-Sinai and LA Children's Hospital. I thought: people from all over the globe fly to these Institutions to get better care and treatment and we just drive on the 405 to get there. How great is that? Unfortunately, not everything is great - I am not entirely pleased with our experiences from these institutions.

We stopped going to Cedars-Sinai when the Geneticist we were seeing there made us wait for three hours. We ended up leaving without being seen and after we've paid our co-payment. I reported the incident to their client service department but I didn't stay long enough to find out what came out of my complaint.

I've always been vocal of my discontent about UCLA Dermatology Department's service. It is extremely difficult to get in touch with them even on urgent issues. Not one of my calls were returned. And when I raised this issue to Dr. C, the main Derm, he told me he is best contacted through email. We did get one reply to the dozens of emails we sent to him. In order to get an audience, we had to get an appointment.

Being a teaching hospital, we get different team of fellows/residents who see us before the main doctor comes in. Consequently, we have to repeat Bambino's entire "skin story" to these fellows who we jokingly called "minions" every time we go for a visit so they can brief the main Derm. The main Derm is well acquainted with my baby's issues. He has been his primary Dermatologist ever before he was diagnosed of NS. In fact, after his diagnosis, Bambino became some sort of a cause celebre at UCLA. We've been invited to a couple of their grand rounds attended by dozens of doctors. We welcomed the attention, hoping that one of those 85 dermatologists who have seen him might be able to offer Bambino a real relief from his sufferings.

Not quite. They thanked us for coming and none of them returned our calls.

Don't get me wrong. I think they are all brilliant doctors. They are probably just too absorbed in other things in their residencies to bother with just one rare genetic "orphan" disorder. They saw how NS manifests, they saw the symptoms in flesh and blood - not just photos on thick textbooks anymore - and then they move on to other more common skin problems like psoriasis. There is nothing else they could do. Besides, cases like this would probably just make .01% of their future medical practices.

I have been tempted to switch to a private Dermatologists, but my Pediatrician pointed out that it will be difficult to find a Derm who will have the experience and skill my Bambino would require.

But I've had it with UCLA Dermatology.

Yesterday, we went to see the Dermatology team at Los Angeles Children's Hospital headed by Dr. Justine Park. My husband warned me not to expect any better because this is indeed another teaching hospital, with residents coming from USC.

I laid it out straight to the first fellow wo saw us, Dr. Goli. I told the young doctor that we've been seeing UCLA Derm but that we are not happy with how they run things over there and that we are actually looking for doctors who will return our calls and won't tell us to call for an appointment when we have questions about trivial stuff like dosages. I told her that they are welcome to turn us away if they think things are not going to be any different around here. She seemed to sympathize with my concerns and she left to brief Dr. Park about Bambino's case,

Three other fellows came in with Dr. Park after a while. They were as curious as the residents at UCLA and ganged up on me and Hubby with questions. They've never seen an actual NS case before. They conceded that they cannot give us the "private practice" kind of attention that we are seeking but they did refer us to one of their USC professors who also volunteers at Children's but has a private office in the Valley closer to our place.

Before we left, we got another invitation to appear at a USC medical conference for "show & tell". They were a bit hesitant to ask us - our wariness of these institutions was probably evident in our faces - but I assured them that we still are very much willing to help out the dermatology field in any way we can.

Although an impossibility, who knows, one of these doctors will find a cure.

Mommy & Me Mondays

Today is Monday. We're supposed to go to our Mommy & Me classes but since Bambino is still under the weather, we are staying in. I am currently typing with just my right hand because I have to support him with the other. Being extra-clingy when he's sick, he lives in my arms practically 24/7. I remember the sling that I used to carry him around in and I thought it would make our lives a little easier right now


Yup, easier 'cause I can have both hands free to do stuff. Plus he likes it better because he couldn't be any closer to me this way.

So since we are missing Mommy & Me today, I thought I'd post some of the photos I took from last Monday's class:



He liked riding the "Dinos" and he finds it amusing every time the girl beside him would shriek in joy.



He'd toss those dolls around. It's his first close encounter with baby dolls.



The boys in the "kitchen". Those two boys are about six months younger than him but he is the smallest.



Finally, the parachute ride which we do towards the end of the class. This is the first time we'd done it because we would always head home an hour before the class end - in time for his afternoon nap. Last week, he slept a little early. We were late for class but we were able to stay till finish. Oh but he didn't like this parachute group ride as evident in this photo. He was screaming along with a couple of other babies who disliked it as well.

Bad, Bad Couple Of Weeks

Just a quick entry:

Bambino is currently having one breakout after another. And as if this isn't enough, he's also been having low grade fever to contend with. Two days ago, I found out what's causing the fever: upper respiratory tract infection.

Because of this, he's lost his already waning appetite. He still wants to nurse, but the congestion in his chest makes it difficult to suckle. For the second night last night, he would wake up every hour crying because he finds it difficult to breath. He wakes up the entire household with his lusty cries - I'm surprised the neighbors didn't call 911.

This is his first bout with colds and it doesn't look like it's your garden variety colds. He would probably require antibiotics just to ward off any other infections.

His appearance right now is one of the worst we've seen. Majority of his face is beet-red, along with some areas of his arms, torso and legs.

I took some photos for the new set of Derms that we're scheduled to see at Childrens' Hospital on Wednesday.

A Session With the Feeding Therapist

I didn't even know that there's such a thing. I've heard of Occupational Therapists, and apparently FT (Feeding Therapist) is a sub-specialization of OT.

So Erin arrived at 10AM last Saturday, in time for Bambino's supposed breakfast - if he eats at all. He would usually just sit on his chair and play with his dry cereal, toss occasional pieces into his mouth, and when he gets bored with them, throws them on the floor to the dog. That last one which involves the dog is the highlight of his breakfast.






So Erin observed all these while asking me and Hubby all sorts of questions from her checklist. She did say, to our delight, that Bambino isn't as complicated a case as she initially expected. She acknowledged, though, that there is work to be done.




With the State of California's empty coffers and budget cuts all over, Erin couldn't tell how soon Bambino will get the services he requires, which includes a Nutritionist and Speech Therapist, but she promised to expedite her report and hopefully, the approval.

Still Not Gaining Weight

It could be because he has four molars coming out all at the same time, but Bambino refuses to eat anything but breast milk. He used to eat okay. Not good, but at least he was eating something else other than Mom's milk.

Consequently, he only gained 4 ounces in the past month. He weighed only 16lb4oz last time we visited the Immunologist.

Last week, we went back to the Gastroenterologist. He recommended doing a procedure that would measure his caloric/energy consumption. It's a tedious process that will take 20 minutes. The GI doctor is not discounting the possibility of nasogastric tube feeding if Bambino carries on like this.

Regardless, he is still the active, rambunctious toddler that he is, sometimes I wonder where he is getting all this energy. He loves to sing and dance while watching Barney and loves to run around and play ball with the dog.

Such a super trouper!