Another test behind us

Joshua is doing well after his endoscopy and colonoscopy this morning.  He recovered from anesthesia ready to roll!  He was (unsteadily) roaming the halls of Day Surgery within minutes of waking up.  The doc said most everything looked normal for a short gut kid from the naked eye, but the biopsies and cultures collected today will tell the real story.  Those results should be in next week.  We have another clinic appointment scheduled next Friday afternoon to discuss all of the results and potential steps moving forward. 

The doctor did see a "miniscule superficial erosion" in the distal portion of the duodenal wall consistent with inflammation of the mucosal lining due to an allergic response or bacteria - that's the million dollar question.  He also found an area of lymphonodular hyperplasia in the duodenal bulb which basically means his lymph nodes are overly active in this spot.  This is apparently not uncommon in short gut kids because what little intestines they have are forced to work extra hard and this is a constant source of irritation which stimulates the lymph node activity.  Both areas were biopsied so we will know more from the histology reports next week. 

The real thrill and surprise for me this morning was learning the doctor was able to collect an aspirate of gastric fluid to perform a gut culture.  I have been fighting to get the gut culture protocol reinstated at CHOA for months because this test is helpful in identifying the specific types of bacteria causing overgrowth problems.  This would allow us to tailor Joshua's antibiotic regimen to the bacteria affecting him and not play antibiotic roulette using a standard protocol for all short gut kids.  It would also help us rule on whether bacterial overgrowth or an allergic response is the bigger culprit in terms of the inflammation and malabsorption Joshua suffers from and allow us to pinpoint a proper treatment plan.  I learned through my support group that gut cultures are routine at premier children's hospitals in Boston, Pittsburgh, and many others.  I was floored when our doctor told me this test was not an option at CHOA - the best children's hospital in the southeast!  I eventually figured out it used to be a protocol 8 years ago, but the one employee in the lab that read the complex culture results left and they just dropped the test altogether rather than training a new employee.  Unacceptable!  I hounded our GI doc for months about it and stalled the endoscopy until this additional test was included.  Our doctor went to bat for us cutting through some significant red tape and now the gut culture test will be available to any GI patient that needs it at CHOA.  I am pretty proud I was able to instigate this hospital policy change to benefit Joshua and future GI patients!  I am nervous and anxious to learn the results next week.  The gut culture cannot always be collected if enough fluid is not present, luckily our doctor was able to get a good specimen.  Growing out and reading the culture can also be tricky because there are typically multiple types of bacteria and you have to figure out which specific type is overgrowing by counting colonies.  The test is not perfect, but I know dozens of families that have benefited from it and having more information is always better.

I already know our doctor wants to order a gastric emptying test later this month to see how well Joshua's stomach contents are emptying into his duodenum because of his recent vomiting.  This involves Joshua eating radioactively labeled food (it better include bananas) and having a series of x-rays taken over the next few hours.  Sounds like a fun-filled afternoon for Mom & Josh!

Thank you to everyone for your extra thoughts and prayers this week for our little warrior!  He continues to amaze us every day with his strength, bravery, and sweet spirit!!

Joshua's next procedure

Joshua will be traveling to CHOA this week on Thursday for a pre-op visit and lab work and then again on Friday for an endoscopy with biopsies.  I am anxious to get through this latest procedure so we have more information in hand to better evaluate Joshua's condition and determine future treatments and so I can have the part where we have to say goodbye to our baby as he is wheeled off to the OR behind us once again.  We have been putting this scope off for months because we hate to put him under general anesthesia and we still aren't convinced we will obtain valuable enough information to outweigh the risk of the anesthesia.  But Joshua's belly pains at night have only worsened and his body is not able to tolerate as much solid food as one would expect, so at this point the discomfort for Joshua and the need to know what is going on inside him are pushing us forward with the procedure. 

Joshua's last clinic appointment was March 7th.  We were expecting to receive the great news that his weight gain had continued and it was time to start lowering his hourly rate of IV fluids.  This is the first step in slowly weaning down on the fluids over a period of months (or years) until his bowel can tolerate the amount of food necessary to thrive and grow so he is no longer dependent on the IV nutrition supplementation.  Unfortunately, we were very disappointed to learn that while Joshua did gain weight, he did not maintain the same rate of growth as the last appointment so the doctor did not feel comfortable lowering his IV fluids.  We were pretty shocked because Joshua seems to be heavier and taller all the time, he is still maintaining in the 50th percentile for weight.  Having gone through the scare we did last year when we tried to push his system into handling more than it could and the disastrous effects that caused, I do understand that taking a cautious approach is the best course of action.  I firmly believe slow and steady wins this race.

At this same appointment, we were talking with our nutritionist about how picky Joshua has become with not eating certain types of foods like meats and veggies.  His favorite combination is a cut up banana and a rice cake crushed and mixed together.  He will pick through any bites of meat or different colored veggies and put them to the side on his tray.  I have a tough time coming up with new options for him to try because of his dairy and wheat allergies and the other list of no-no foods like nuts, all fruit besides banana, soy, and anything processed or with too much sugar.  That counts out a lot of foods!  I am now that person in the grocery aisle that reads every word of nutrition labels in the search for dairy-free, gluten-free, nut-free, fruit-free, and low sugar items. 

After learning about Joshua's new picky behaviors, the nutritionist suggested that it may be time for him to have a g-tube placed to receive tube feedings directly into his stomach.  I am incredibly resistant to this idea for many reasons.  Joshua has managed to make it this long without a feeding tube which is highly unusual given his condition.  Because he has been allowed to eat orally continuously from a few weeks old (except for periods after surgeries), he has not developed the oral aversions that are so common with short gut kids.  These can be hugely detrimental and hideously difficult to overcome, requiring years of feeding therapy with varying levels of success.  I absolutely do not want to take away any of his opportunities for oral feeding.  A g tube is only good for getting an amount of food that Joshua's bowel can process and absorb into his stomach because he won't take it in orally.  Another major issue I have is that we have proven from trial and error that Joshua's intestines cannot handle an increased amount of food at this time so a g tube is not indicated for his current condition. 

I was determined to prove the nutritionist wrong and get Joshua eating a more varied diet with protein and veggies.  I was successful in increasing the size and variety in Joshua's meals adding chicken, eggs, green beans, and corn.  He loves to eat, but it is very easy for him to take in more than he can handle and he ends up "dumping" the food out in a diaper without ever absorbing it.  This past week he also vomited all over me on Tuesday and Saturday nights during or after his bedtime bottle.  Our GI doc thinks he is so full from the solid food at dinner that the formula an hour or two later has nowhere to go.  He suggested that we may need to do a gastric emptying study after we get the results from the endoscopy to confirm whether Joshua's stomach is emptying at a normal rate. 

So we continue to walk a very thin tightrope attempting to increase Joshua's food and formula intake in order to get him off the IV fluids, but without exceeding his body's capacity to process the increased amount lest we take giant steps backwards with episodes of vomiting, diarrhea, cutting back on feeds, and ultimately weight loss.  To say it's an every day challenge is putting it mildly.  But, we're doing it... however slow and bumpy the road may be.

We cleared a huge hurdle this week when we finally received Joshua's long sought after renewal for the Katie Beckett deeming waiver.  The paperwork battle this year was tougher than last and we were down to our last rejection notice before having to go through the appeal process which I am sure is even more torturous.  Letters from me and our GI doctor turned the tide in our favor.  Our approval notice was met with immense relief and a grim sense of satisfaction for besting the bureaucratic process this time around.  Now I get an eight month reprieve before the battle begins again in November for next year's coverage. 

Please keep Joshua in your thoughts this week as he undergoes yet another invasive procedure.  Let us hope for the doctors to find the information they need in order to develop a targeted treatment plan for Joshua's condition.  Slow and steady win the race - we just need more of the steady!