Dangling Carrot

But those eyelashes!

Finally March is tomorrow.  Hannah will see Dr. Johnson for a weight check on Friday, and will get the second dose of synagis. Next Monday (!) is her swallow study and follow up with Dr. Smith.  I am so glad that it is finally here! I feel like each day and week is an eternity of waiting.  She was 10 lbs 12 oz on Monday, so I'm confident that by next Monday she will have reached the 11 lb milestone Dr. Smith was waiting for.

The game plan when we last saw Dr. Smith was to look at her throat and see if there was any improvement. If so, we would start oral feeding.

Come inside Jenny's brain for a moment.
Why would Hannah's esophagus get better on its own?  In some cases, the cricopharyngeal (CP) bar went away on its own.  If the sample size is only 5, and 2 out of 5 got better on their own, that isn't very impressive.  If 3 out of 5 got better on their own, that's more than 50%.  But still not impressive with such a small pool of data. And I don't usually ever win stuff, so I'm going to say that the odds are not in our favor.  I hear Hannah try to swallow every day. She does 5 or 6 loud swallow attempts in a row, with huge confused eyes, followed by a big grunt and sigh. Because it doesn't work. So yeah, I don't think it got better on its own.

Dr. Lexie at your service

And if it didn't get better on its own, Dr. Smith said he would do the dilation and botox again.
Ok, last time we did that, the dilation worked for about a day. After a day she couldn't eat again, and we had to go back to the hospital and get a feeding tube. And then we had to wait three whole weeks to see if the botox ever started working.  If Dr. Smith sees that the swallow study is no different than before, he'll say, "Lets do dilation and botox next week." which puts us in the middle of March. Let's say the dilation also works for a day or not at all, and Hannah still has a feeding tube. Let's say Dr. Smith wants to wait three weeks again to see if the botox worked, that brings us to the beginning of April. Let's say we get to the beginning of April and do another swallow study, and Hannah's esophagus STILL doesn't work, THEN will Dr. Smith do the myotomy? 

In my mind the myotomy surgery is a dangling carrot that keeps moving away from us every time we move forward a month or a week or 2 kilograms.  Every step is followed by, "Check back in 3 weeks to see if it worked." I feel like it will never happen.  Maybe I'm wrong and it's not as far off as I think and I'm being unfair.  But I am just so tired and I am so worried and I want my baby to get better right now. Yesterday. A month ago.

Tummy time

On Valentine's day, we were looking for pyloric stenosis. If a baby has pyloric stenosis, they would have corrective surgery within a day or two of being diagnosed.  Pyloric stenosis is an enlarged sphincter muscle at the bottom of the stomach, and prevents food from going through. The procedure to correct it is called a pyloromyotomy. So really, pyloric stenosis is almost the exact same problem as Hannah's except in a different place, and has almost the exact same surgical fix. If babies have surgery immediately to correct pyloric stenosis so that they can take food by mouth and grow, why wouldn't they also correct a cricopharyngeal "stenosis" immediately? Her problem not only prevents her from eating, it also compromises her breathing, which some would argue is kind of important.  And likewise, do they ever wait six months to see if a baby's pyloric stenosis goes away on its own? I think not!  This doesn't seem fair.  Just because ours is more rare, we get half a dozen swallow studies and months on a feeding tube, and so much waiting and wondering and worrying.  He only sees us and thinks about us once a month, but we do this every day and I think about that 3-weeks away appointment Every Day.

Every day and week and month that she is on a feeding tube she loses oral stimulation.  She loses elasticity in her stomach.  She has no hunger cues. She can't eat at the pace and volume she wants to set, that her body tells her to. She is 3 months old and should have tummy time every day and start pushing up and rolling over.  All of our babies have started crawling around 6 months old. How is she going to be mobile with a feeding tube in her nose? She would pull it out constantly.

The long-term alternative to an NG tube is a G-Tube, which is a tube through the abdominal wall directly into the stomach. Babies who still need enteral feeding after becoming mobile generally get a G-Tube that is easier to maintain than an NG tube. But it has to be surgically placed and requires general anesthesia or deep sedation. If we are withholding the myotomy surgery because of the risks of surgery and anesthesia to an itty bitty baby, it would seem counter-intuitive to surgically place a G-tube rather than to just do the dang surgery already and eliminate the need for tube feeding.

Every time she coughs and doesn't quite clear everything in her throat, I worry she is going to aspirate to her lungs or stop breathing. Every time she sputters up white foamy mucus and saliva, she chokes and screams, and I jolt out of bed, hold her upright and make sure she isn't turning blue.  Every time I lay her flat for a diaper change or for tummy time I worry that she will choke on her own spit.  Every time we leave the house or I hear someone sneeze I worry she will get sick again.

Oh yeah, that reminds me that Dr. Smith mentioned that he wouldn't do throat surgery if the baby has had a respiratory infection within six weeks because surgery is riskier if the throat is already irritated or inflamed. So here we are, sitting on our hands during an incredibly dangerous winter hoping that this baby doesn't get sick again, and if she does it will add at least 6 more weeks to the timeline I mentioned above.  Again, see the part about the baby needing to become mobile in the near future...

Its also inconvenient for me in a whole bunch of other ways, but I'm trying to focus on why surgery NOW is best for Hannah and not just me.

Maybe I will tell Dr. Smith everything I've just told you and he'll say "You're right! Lets do surgery tomorrow!" but I highly doubt it.

Still manages to smile even with
a tube in her nose!

Never a Dull Moment

Last Wednesday, Hannah had an ultrasound of her pyloric muscle to determine if she has pyloric stenosis. The ultrasound was rather fuzzy and we couldn't get a very clear picture, but a radiology consultant said "It's probably fine" and that was that.  I think we are still going to get a follow-up GI study done in the near future, because "probably fine" isn't super definitive in my opinion. We also had her nutrition levels checked, called a prealbumin blood test. It checks protein and nutrient levels, as well as organ functions. Gratefully, the test showed normal function and nutrition aside from low iron. We're going to give her an iron supplement and continue with her established feeding regimen.

I didn't take enough pictures this week

This Wednesday I was concerned that Hannah might be getting sick. She has a really wet cough and since her cough isn't very productive, I always worry she will aspirate to her lungs. There is a Facebook group, albeit small (about 20), of parents with babies who have CA, and many of them had aspiration pnemonia multiple times. Hannah does have some wheezing on both sides of her lungs but the doctor said it's not aspiration, and Hannah tested negative for RSV (Hallelujah!!)  We also got a one-week standing order for the suction clinic, which provides BBG suctioning outpatient at the hospital.  If I'm not able to clear her nose and throat with a bulb syringe or my NoseFrida, that is a logical next option for us. We went there once about a month ago, and it was very helpful. (PS The NoseFrida is awesome!) Her oxygen was excellent and she does not have lung retractions or a fever.  As long as she stays that way, we'll be OK. A respiratory infection will most likely delay her surgery by a month or more when the time comes.  It's so hard to keep the sicknesses at bay in a house full of people!  I have 3 kids in public school and a two-year old thumb-sucker, so who knows what germs are lurking around in here!

I had to un-sew Hannah's sleeves so she can flex her hands rather than make a fist constantly, and so that she can explore putting her hands in her mouth. I get serious anxiety whenever I think she's about to pull her NG tube out or the tape is coming loose. We are getting good at putting it back in but it is stressful for me and Hannah.  We probably have to put it back in and re-tape her face about once a week.  She is steadily gaining weight while getting 27 mL/hour through her NG tube. I am trying to go up incrementally, but she's picky about it, and her wet cough showed up right when I tried to go to 28 mL, so I went back 2 days later. I'll try again soon. 

Only one in diapers...that's a relief.

I gotta say, five kids is really busy and a lot of work.  And so loud. All my peeps want dinner every single night (the nerve!) but often complain about what is served, dishes are constantly piling up, laundry is an endless, eternal pain in the neck, and caregiving is exhausting. They have homework, church and school activities, I have a weeknight church assignment, we have several doctors appointments each week. We all have emotional needs. I'm pumping 4 times a day.  Hannah wants to be held constantly and seems to wake up the moment my head hits the pillow for a power nap. Last weekend I was pretty much running the show solo while Chris recovered from a minor surgical procedure, and I was also potty training Lexie, finally having a breakthrough on day seven.  (5 kids is plenty...I'll let you conclude what kind of surgery he had).  And did I mention the sheer volume of laundry? 

Our 2005 little red Honda is on its last legs (I guess cars don't have legs), and so Chris has been riding the train to and from Draper and leaving the car at the Draper train station to only go the 5 miles to and from his office. So rather than 250 miles a week commuting, he drives 50 miles. We just have to make that car last a little longer until we can replace it. But this means a longer commute, leaving earlier in the morning and getting home later, plus only one car at home to get us all those places I mentioned. 

I think its funny that they each have a
facial apparatus and tube of some sort. Haha!

I know they are Worth It and this is what I signed up for, but being Busy with a capital B is really hard. I have a to-do list ten miles long and some of it will probably never get done. I'm self-medicating with snickers bars and Mountain Dew.  Lets me honest, this momma needs therapy and anti-anxiety meds, and that's two more appointments to keep.  I wish I had more time to read, but whenever I have down time it's something mindless like Facebook, Netflix, or a game on my phone. I realized this morning that Leah will be a senior in High School when Hannah starts kindergarten.  This circus show is only getting started!  I keep thinking it should be getting easier to manage, but its only going to get harder and it will be like this for a long time even when Hannah is better.

I need a serious change of heart and mind, because I'm going to be doing this for many years to come, with increasingly busy kids with new and varied challenges. I have to figure out how to do this with a better attitude and more patience, more organization, etc. 

At least somebody likes the snow...

So far I have a few strategies for dealing with everything.  I have a notebook with all of Hannah's health information, growth progress, feeding changes, doctors appointments, phone numbers, and more... Doctors always seems surprised or impressed when they see me taking notes or referring to my notebook. I have a really good, no-fold laundry sorting set-up (which works great when I actually get around to it).  The kids are expected to put away their own clean, sorted laundry and help with taking out garbage and loading and unloading the dishwasher.  I'm recommitting to menu-planning to save on groceries and shopping frequency. Leah and Samuel are old enough to babysit the other kids and a sleeping Hannah if I need to run a quick errand. Chris is using FMLA leave a few morning hours per week in order to help get the kids out the door and allow me time for a shower.  I'm praying more (did you know that you can pray that a two-year old will pee in the potty and then she will?). I'm going to therapy and taking anti-depressants. I try to get out of the house for girls game night or a $1 movie. I got a cute new haircut.

I always feel like I need to end my posts on a positive note, so here goes. Hannah is healthy and growing. The other kids are (mostly) staying healthy and progressing in their respective spheres, whether it is a science test, times tables, reading new sight words, or going potty and moving to a big-girl bed. We are trying to still have meaningful experiences with them. Chris and I are re-learning how to manage our hectic household, although probably not very gracefully.  We have been incredibly blessed by friends and family and strangers, and countless prayers. My sister's homeschooling Co-op had a bake sale and decided to donate their proceeds to our YouCaring fundraiser, and they are in Illinois! My friend Stephanie donated her time and talents to take our family portraits. Ward members and friends have been babysitting and providing dinners and even helping clean my house as needed (a Ward is what we call our local church congregation).

Although fortunately no one has said this to me, I've often heard people say of someone else's trying circumstances "I could never do that" or "I don't know how you do it" and what I really think it means is "I don't want to have to do that" or "I'm glad I don't have to." And to be honest, I really wish I didn't have to either, but I tried not to ever say or think it because I knew in my heart that if I needed to I would Rise to the challenge and become a Warrior for my child.  And here I am clumsily working on my Warrior badge.

Shoot.  Someone just threw up. Gotta go.

Weight Check

All of our babies have been tall and skinny in their first 2 years-around the 75th percentile for height and 25th percentile for weight.  Hannah was fairly big at birth (8 lb 12 oz), but her growth took a serious hit with three hospitalizations and two respiratory viruses before she was six weeks old.  She finally regained her birthweight at six weeks, and was growing fairly steadily for a few weeks.  The goal is to gain about an ounce a day, and Dr. Smith wants her to be at least 11 pounds before doing the myotomy surgery.

On January 12 she was 9 lb 7 oz.  But at her checkup on January 22, she weighed 9 lb 9 oz, which was only 2 ounces in 10 days, and on February 5 she was 9 lb 12 oz. So while she had been growing an ounce a day for a while, she had now only gained 5 ounces in 3 weeks. 😱  At birth she was in the 50th percentile, and now she's in the 5th percentile! This is obviously very concerning.

Hannah has a really hard time whenever we try to increase the amount she gets in her tummy.  She gets really fussy and starts to regurgitate when we go up even 2 mL/hour.  It almost seems like she's in pain.  She gets a really wet cough, and there is the concern that she will aspirate into her lungs; aspiration pneumonia is not uncommon in babies with CA. Whenever we increase by 1 mL, I have to give her an entire day to adjust to the higher volume.  Mind you, 1 mL is a fifth of a TEASPOON!  This is why she has been getting a continuous feed 24 hours a day rather than bolus feeding, which more closely mimics natural eating habits like getting a 3 ounce bottle over a 15-20 minute period every few hours.

He just can't make a normal face!

Last month the doctor and I were working on trying to consolidate Hannah's feeding schedule away from 24 hours a day.  So in mid-January, I stopped Hannah's food pump for one hour a day and increased the volume on the other 23 hours so that she would still get the same total calories per day. I decided to eliminate the 2am hour, hoping she wouldn't even notice.  We did this for 3 1/2 weeks, increasing her hourly volume and adding 1 off-hour each Friday.  We were down to 20 hours a day at 25 mL per hour with breaks at 2am, 8am, 2pm, and 8pm. We did make the mistake of not increasing her daily caloric intake in that time, which is why she stopped gaining weight. So since her weight plateaued, on February 5th we went back to 24 hours a day and increased the volume to 27 mL/hour.  Now her total volume per day is almost 650 mL rather than 500.  Within a week she had already started gaining an ounce a day again. What a relief!

She's such a snuggle bug

This week, Dr. Henderson called me and recommended that I take Hannah to another pediatrician who has more experience with special cases like Hannah's.  Dr. Henderson has been incredibly helpful. First of all, he took me seriously on the Day Everything Hit The Fan (November 28) and got Hannah admitted to the hospital.  Every time I had nursed Hannah and she had turned purple, I got this panicky feeling that something was wrong.  It gave me anxiety and I was starting to dread feeding time because it was so scary for me to see her stop breathing.  At the same time, when she wasn't eating, she was perfectly fine, so I was puzzled and unsure of how seriously I thought it should be addressed.  Surely, she doesn't need to be hospitalized!  The hospital is for babies who are sick, and Hannah wasn't sick.  The first doctor told me it was nothing but reflux, but Dr. Henderson saw the warning signs that something more was wrong and escalated her case.  At the time I thought the hospital was going to be overkill and I was going to be embarrassed that nothing was wrong.  I'm so glad Dr. Henderson took me seriously and followed his instincts.  He very well could have saved her life. Dr. Henderson also does shifts at the hospital where we've been several times and was proactive about getting us the Synagis shot.  He's friendly and personable and has answered all my questions or consulted with other doctors and specialists if he didn't know the answer.  It has been nice to have the continuity of care, but I appreciate that he recognizes our case might be out of his scope. So while it is hard to switch doctors and say goodbye, I think it will be a good move.

We got in to see Dr. Johnson the very next day (yesterday).  I had made an appointment to see him right after New Year's, but had cancelled it in order to keep our continuity with Dr. Henderson.  So Dr. Johnson had already read Hannah's chart and knew all about her.  Right now the primary concern is getting Hannah to grow, and this involves finding the right feeding regimen.  Most normal babies are self-directed in how much food they take in.  They cry when they are hungry and eat until they are full, and it is just right for growing. Oh, how I miss this dynamic!  My baby gets fed constantly, a tiny bit at a time, and I have no way of knowing if she is getting enough or not.  I went to see Dr. Johnson with a list of questions about calories, ideal growth, bowel movements, and transitioning to bolus feeding, and he addressed all my questions before I even asked them!

All the kiddos.  I think they like her.

It was really good to learn the "formula" of how many calories she needs in order to gain weight and catch up: 110 calories per kg of weight. Now I can adjust her daily calories without having to ask a doctor how. I suppose I could have googled it, but I didn't think to. Dr. Johnson also ordered some blood work to check that all her internal systems are working properly and that her nutrition levels are balanced.  With regards to not being able to increase her hourly volume very quickly and without discomfort, he had a couple recommendations.  First, Hannah gets breastmilk with a little formula mixed in for added calories, and he switched us to a new formula that is easier to digest. Secondly, he prescribed a medication, Reglan, that helps the gut digest faster.  Hopefully these two changes will help her stomach empty a little faster and allow us to increase the hourly volume faster. Our first goal is to get to 32 mL an hour for 20 hours a day. The second goal will be to have 2 hours on and 1 hour off throughout the day, which is more like 41 mL/hour for 16 hours, but probably higher because she will weigh more by the time we get to that point.

Third, he ordered an ultrasound to check Hannah's pyloric muscle, at the bottom of her stomach. Babies who have pyloric stenosis have projectile vomiting as the main symptom. But Hannah doesn't ever have a "full" belly, and the cricopharyngeal bar blocks most of her reflux from coming up: she hardly ever spits up at all. So it is possible that she does have pyloric stenosis without that tell-tale symptom, and it would explain why she can't increase volume without significant discomfort. Incidentally, pyloric stenosis is an overly large sphincter muscle, just like her overly enlarged upper esophageal sphincter, and the surgery to correct it is also called a myotomy, a pyloromyotomy. It seems not a coincidence to me if she were to have both. Maybe she has some kind of disorder where her sphincter muscles are too big.  My mom says birth defects come in threes.

I put on pants! Survival mode here.
Celebrating the little things, people...

If she does have pyloric stenosis, she would have immediate surgery to correct it: same day or next day.  Our surgeon, Dr. Smith is hesitant to do Hannah's myotomy until she is bigger because of the risks associated with anesthesia.  But if we were going to be anesthetizing her to do the pyloric correction, couldn't we also do her cricopharyngeal myotomy while we're at it? I asked Dr. Johnson as much and he said yes and said he would call Dr. Schramm in Provo for his help.  I like that he is on the same wavelength as me: he seems to agree that the myotomy is Hannah's best option and had asked why we hadn't done it yet. Well, this was kind of exciting news for me and also a little overwhelming: Here it was 1:00pm on Valentine's day, and my kids are almost home from school.  I'm about to go get an ultrasound that could determine if we have surgery in the next 24-48 hours, surgery that I have been hoping for for months. And also, it would mean another chaotic balancing act while we have a hospital stay. Do I go home and pack a bag just in case I don't go back home? And oh, yeah, I've been trying to potty train Lexie this week (two year olds are so stubborn!).

Unfortunately, the ultrasound was inconclusive.  After trying for the better part of two hours, we were not able to get a good shot of the pyloric area with food movement, partly because the volume in Hannah's stomach is so small, partly because breastmilk is harder to see on an ultrasound, and partly because she had a lot of gas bubbles in her stomach and was really fussy.  (This baby does not burp!) So for a few hours I had an unsettled eagerness and anxiety that we might have imminent surgery, and then a huge let down when we didn't have an answer at all.  Talk about a roller-coaster! The next step will be to do a full study of the lower stomach and duodenum to put to rest or confirm the theory of pyloric stenosis.  We've already had various tests to look at her anatomy, but nothing below the stomach. I'm on pins and needles waiting for a call to go to radiology today, or maybe it won't happen until next week.

In all, I'm glad we have Dr. Johnson and that he has the same concerns as me and some new answers for us.  I'm glad he is up to speed on Hannah's condition and is proactive about testing. Hannah has enough calories and I have a game plan to keep her growing steadily.  And I'm preparing myself that any day could turn out completely different than how it starts.

Electronic Beep

Use an electric pump to extract milk from mom. Use a different electric pump to put that same milk in baby's tummy.  While effective, it is one of the least convenient or efficient ways to feed a baby.  (And so many parts to wash!)

Hannah has had an NG feeding tube for 2 months now-since she was two weeks old.  NG means "nasogastric" and goes in her nostril (naso-), down her throat, and directly into her stomach (-gastric).

Tubing goes through the pump valve and rotor

Starting at the top, we have a disposable "pouch" much like an IV bag, which gets filled periodically with fortified breast milk.  The tubing coming out of the bag passes through a Kangaroo Joey pump, being connected by a valve, going around a rotor, and then out again. Then there is about 6 feet of tubing. This bag and tubing is replaced every 24 hours. The bag is washed out and the tubing "flushed" with hot water every four hours when it gets refilled.  (This process takes at least 5 minutes and cannot be accomplished with one hand.  I have tried.  Its also much harder as a walking zombie at 2am.)

Hannah is sporting the NG tube in a soft purple,
the Kangaroo Joey pump in a black backpack,
and is accessorizing with two sizes of dosing
syringes that complement her tube and port.
She tops off the look with facial tape,
because who wouldn't?

This high-maintenance bag and tubing set is then connected to Hannah's NG tube that is taped to her face and goes into her body.  Her tube is much shorter, and extends out of her nose about 18 inches.  It has a purple push-on cap, and when uncapped, screws onto the end of the longer pump tube. This "port" has several uses. It allows us to change the bag and tubing every day, it allows us to administer medication directly into her NG tube with a matching screw-on syringe, and it allows us to cap and close off the NG tube when she is not getting fed.  We also flush her NG tube with 3 mL of warm water every 4 hours when we refill her bag to prevent her tube from getting clogged.

Unlike the feeding bag and tubing, Hannah's NG tube stays in 2-4 weeks without being removed or replaced.  It goes up into her nostril, at the back of the nasal cavity it turns downward and goes down her esophagus (past her cricopharyngeal bar), through the lower esophageal sphincter, and into her stomach.  The very last inch of this tube has a few openings similar to a soaker hose where the liquid nutrition is deposited in her tummy.

It is very uncomfortable for Hannah while we are putting the tube in, especially trying to "turn the corner" at the back of her nose to make it go downward.  But fortunately, once it's in all the way and we are done taping it to her face, she doesn't mind it at all and quickly goes back to being happy and smiley.  It usually takes me 2-3 tries, but Chris can usually get the tube in on his first try.  It is definitely a two-person job: one person to hold her down and put the tape on, and the other to put the tube in and hold it in place while it gets taped. It only takes about 10 minutes, but it sure gives me anxiety!

"NG Tube Insertion 101" is not a class we took when we became parents, so there was some learning and failing and quite a few tears before we got the hang of it.  It hurts my momma heart to pin a screaming baby down and torture her by putting things in her nose. It's disturbing when the tubing comes out her mouth instead of going down (eww!).  And its scary to realize you might accidentally get the tubing into her lungs rather than her stomach.  The tube has to be inserted the correct length, which depends on the size of the person.  It has to be taped at the right spot and in a way that the tape won't come off.

After we put the tube in and tape it, we have to check to make sure its in her stomach.  Using one of the screw-on syringes on the port end of the NG tube, we pull back slowly to see what comes out: if stomach content comes out, we're golden.  (Sorry! Kinda gross!)  Another way to check is by pushing air into the tube and listening with a stethoscope for a "whoosh" sound in her abdomen.  (Side bar: this whole process is kind of unfair. In the hospital, after an NG tube is inserted, they order an x-ray to make sure it is in the right place. I obviously don't have that luxury!  Why do they expect us to get it right when they have to use an x-ray to check their work?!)

The pump and food pouch go in a convenient little backpack which helps the baby stay portable, but it is still a pain in the neck.  Unless you bring the backpack with you, the baby can only go 6 feet. If you forget to bring the bag and you pull the tubing too far (which I do about once a day), the tubing gets dislodged and the pump beeps annoyingly at you until you turn it off and back on again.  If you pause feeding for some reason and then forget to turn it back on, it beeps at you.  If the tubing gets clogged or pinched, it beeps at you.  If the battery is low, it beeps at you.  If the bag tips over and gets an air bubble in the tubing, it beeps at you. When the bag is empty, it beeps at you.  Hannah sleeps 10 hours straight through the night, but I wake up to an electronic beep every 4 hours in order to refill her feeding bag.  Its so hard to get out of bed for an electronic beep!  I have actually fallen asleep standing up waiting for the bottle warmer...

Its easy to get tangled up her in tubing.  Its tricky to get her in her car seat, and her car seat in the car without pinching or pulling the tubing (or closing the car door on it).  Its impossible to wash and refill the bag while holding a screaming baby.  In short, the NG tube gives me anxiety, the pump is annoying, and the bag is high maintenance.  But I am so glad they exist.  They feed my baby and are helping her grow!  Without them, she would not have lived, so I am grateful for this work-around until Hannah can get her surgery and can swallow again. I would much rather feed my baby the regular way, but for now I'm glad I can even feed her at all.

Happy Feeding Tube Awareness Week!

(OK, seriously, I didn't know that there was a feeding tube awareness week, and I was already working on this post when I learned it was the second week of February.  Happy coincidence!)

Synagis run-around

Heinz needs to get some medication to save his wife's life. The pharmacist won't sell it for less than $2000 because he wants to make a profit off of his discovery. Heinz only has $1000 and the pharmacist won't budge, even though he knows the wife is dying. The question is if Heinz should steal the medication and why or why not.  This hypothetical moral dilemma is multi-faceted with myriad solutions, none of them right or wrong.

I have felt an awful lot like Heinz the past few weeks trying to get the Synagis shot for Hannah.  Synagis is an RSV prophylactic shot that is given to medically fragile babies during flu season.  Hannah qualifies for the medication because she has "a congenital anomaly that impairs the ability to clear secretions from the upper airway because of ineffective cough," and she is "at risk for a prolonged hospitalization related to lower respiratory tract infection."  Hannah was already hospitalized for influenza and needed to be on oxygen for 3 weeks afterward. I'm not sure how much this medication actually costs, but I've been told $2000 and I've been told $15,000.  Either way, the insurance company recognizes that the preventative medication is cheaper than another hospitalization.

In clinical trials, 10% of placebo recipients were hospitalized for RSV, and less than 5% of Synagis recipients were hospitalized for RSV, meaning that the medication cuts her chances of getting RSV by more than half.

This is on our front door.

We started this process on January 12 and it has been a total circus trying to get this prescription filled. We've had an especially dangerous RSV season, and Hannah's growth already took a hit with 2 respiratory viruses in a row.  What if she were to get sick while we were waiting?  We have hardly left the house at all, and I have been "screening" every visitor that comes over.  I've purposefully kept Lexie home from nursery at church, and opted not to take her to the mall or rec center or McDonald's play place even though she desperately needs to get out of the house and have a change of scenery.  Lexie and I are driving each other bananas. 

I had no idea how hard it would be to get this shot, and I have been stir-crazy for three weeks! Because its so expensive, there are quite a few hoops to jump through. Insurance has to have documentation from the physician of why the medication is necessary, and the pharmacy has to have prior-authorization information from insurance before they can proceed with dispensing.  Everything has to line up perfectly, all at once, in the right order, and not on a Friday because you can't overnight ship a refrigerated medication on a weekend. There's no checklist for worried parents to use to streamline this process.

It took an entire week of waiting before we even knew we had the wrong pharmacy, and then another week for the insurance company to catch up.  If I hadn't been making phone calls to insurance, doctor, and especially pharmacy on an daily basis, it might have never happened.

We started out on the wrong track, but here's a sort of check-list of how to get started and how our particular pharmacy really convoluted this process that I shall call:

THE SYNAGIS RUN-AROUND

First of all, make sure you know which "specialty" pharmacy your insurance company prefers to use for fancy medications like this.  Have the doctor send the prescription to this pharmacy and to the insurance company. It will still take the pharmacy a full week before they actually know who you are when you call. Maybe call the pharmacy every day and ask the "status" of your prescription. They will tell you they are waiting for information from the insurance company.  Meanwhile, the insurance company will automatically deny the prescription, and will say they need more information from the doctor about why the medication is necessary. 

Call the insurance company.  It might take them several days to realize that they need more information from the doctor.  They will say that they sent, or will be sending, a letter or fax to the doctors office asking for patient chart information. Call the doctors office and tell them to look for the letter from the insurance company and respond with the requested documentation. Then call insurance back to see if they got the info and ask them how long the review process will take before the medication can be authorized. They will probably say 2-3 days.  But it might help if you call them every day to ask the "status".  Also ask them what the next step will be.

Once the insurance company finally has the documentation they need, they will write a letter to the prescribing physician and it will have a "prior-authorization" number.  As soon as the insurance company has approved the medication, make sure they send this information to the physician. Then the doctor's office has to call the pharmacy and give them the prior-authorization information.  I recommend calling the the doctor's office to make sure they got it and remind them to call the pharmacy.  I asked insurance why they can't call or send this information straight to the pharmacy, and they said it has to do with privacy laws.

Side-bar: Every time I spoke with someone from the pharmacy about each step, I asked "how long should I expect that to take?" and usually they said 24-48 hours.  I personally think that is ridiculous. With this many steps and hoops to jump through, that is way too long.  If you had a regular prescription and a local pharmacy or if you were in the hospital, your prescription would be filled the very same day. I understand that special insurance authorization makes this process longer, but there is no reason it should take more than one week, TOPS! 

(We've already been working on this for 2 weeks at this point).  Now the pharmacy will the call the parent for "permission to ship" the medication.  I've already implied that his pharmacy is incredibly incompetent; when they called the parent for "permission to ship" they called and left a voicemail for my mother...who lives in Chicago.  Ummmm....okay?  How did they even get her number? I'm pretty sure she is listed as an emergency contact for us at the doctor's office, but I did not give that information to the pharmacy, so why would they have gotten it from the doctor?  *scratches head*

So, rather than calling me, I called them and asked, "What's the status?" and they told me they had already called the parent and left a message. "Uhhh, no you didn't..." says me.  I eagerly gave my permission over the phone.  They even asked me, "What day do you need it by?" and I legit said, "I needed it a week and a half ago." (So if I hadn't called them that day to ask the status, would the whole process have stalled until my mom-who was travelling and out of range-checked her voicemail?)  I asked them why they needed to call the parent for permission to ship. Of course you have my permission to ship! They said that sometimes the parent has changed their mind, or the child isn't going to get the shot after all because he or she already has RSV (GEE! I WONDER WHY!?!).

Then the pharmacy called the doctor AGAIN to set up the shipment. This means getting the address and business hours. The medication is refrigerated, so it has to ship overnight and be received the next day, which obviously can't be a weekend. They will also get the patient's weight, since the medication dosage is determined by weight. (Don't you think this process could be streamlined if all this information would have been collected at the very first encounter??)  Now, if the pharmacy calls the doctor's office and leaves a message, the ball is now in the receptionist's court again and the office has to call them back again... Usually, I would call the pharmacy and ask today's status and find out that they left a message, then I'd have to call the doctor's office and ask them to please return the message. Also, if the patient's weight has changed, the doctor has to send a new PRESCRIPTION!!! further frustrating the process, because now they have to call the parent and get permission to ship again.  Go ahead and ask me how I know...

Finally, a conference call. I got the pharmacy and a helpful nurse from the doctor's office on the phone at the same time and I didn't hang up until I knew that they had my permission to ship, the doctor's office had given a verbal confirmation of shipping address, business hours, child's weight, and dosing amount, and I was confident that all was in order to ship that day. This was Wednesday, January 31. I was told that the doctor's office would open the package immediately and put it in the fridge, and then call me to bring Hannah in for her shot. I wasn't about to count my chickens though, because I'm sure they could find some other way to screw this up, like shipping it to my house when I'm not home, or my mother's house, or putting the wrong name on the medicine, or some other unknown abomination.  I even called the pharmacy back later that evening to see if they had a tracking number for me, but they didn't yet. 

Proof. That is one hard-earned bandaid!

The next day, I waited anxiously all day for the call, not entirely certain if it would really happen. Finally at 3pm I called the doctors office to see if it had been delivered and they said no.  So I called the pharmacy for a tracking number, and they told me it had been delivered at 9:45 am and that someone named Clark had signed for it. I called the doctor's office again and sure enough, it was in the fridge with Hannah's name on it.  I was there within half an hour, and on Thursday, February 1, a full 3 weeks after the prescription was made, Hannah finally got her Synagis shot.

If every step takes days, even with daily prompting from a proactive parent, I can't imagine how long this would take if I had not been involved.  Each step would have been buried on someone's desk for a week at a time.  It has been completely ridiculous!  When you have a vulnerable baby-and a stray sneeze in her direction could send her to the hospital-you have to be proactive.  You want this medicine ASAP! Not after the spring thaw...  

Here are some tips if you are going through this process, and I hope you have a less incompetent pharmacy: Be as sweet as pie. Say please and thank you. Ask the name of the person you are talking to. Write down all the phone numbers you are calling, what days and times you called each place, who you talked to, and any new information you learned or information you need to get from someone else. You will spend a lot of time on hold; I recommend a hands free headset.  An impassioned monologue and some hearty tears on my behalf helped "escalate" our case. Let them hear that baby crying in the background. Make a friend at the doctor's office who knows the situation and can stay on top of the process with you.  (Thank you Linda!)

I count over 30 phone calls and over 4 hours of phone time (much of the time on hold).  This had better be more smooth next month when we get the second dose!! As my mom put it, when all is said and done, you will have made thousands of dollars per hour when you take into account the value of the medicine and the number of hours spent on the phone trying to get it. 

Lastly, give a big sigh of relief and snuggle that baby. And cover your cough, for crying out loud!