Chicago Trip Update

(This is the view from the efficiency apartment building
that is connected to Rush Hospital)


(Here is a shot of Josh sitting on the plane. We want to thank Southwest Airlines for providing the round trip tickets for us to see Dr. Weese-Mayer)


(Josh was monitored night and day in the sleep lab seen below)


(They placed many electrodes on Joshua's face and head while he slept and watched him via camera while he was in the sleep lab at night)


Hello all,

Joshua and I just got back from Chicago late this afternoon. We saw Doctor Debra Weese-Mayer and her group - who evaluated Joshua both in a Pediatric Intensive Care Unit and a Sleep lab setting and have a lengthy update to share with you. But first, I want to thank you all for thinking of us and praying for us during this trip. We sure needed them now - maybe more than ever!


Now, on to the update:


The team of doctors did confirm the diagnosis of Late-Onset Central Hypoventilation Syndrome with Hypothalamic Dysfunction.

They decided Josh requires (and probably has for some time) 24 hour mechanical ventilation. This means Joshua will have to be on his ventilator at all times.

They also adjusted his ventilator settings and changed his trach to a Bivona (adult sized) 5.0 tracheostomy tube with a tight to shaft (TTS) cuff. The cuff is like a balloon at the end of the tube to prevent air from coming back up the trachea and out the mouth and nose. It will only be used (or inflated) when he sleeps.

These findings mean we need to get nursing care in our home for Joshua. The doctor suggested we needed as close to 24 hour nursing care as possible. Joshua will also need to be tested thoroughly to see if he'll need growth hormones - and as soon as possible - so that he will develop as he should through puberty.

Speaking of development, Joshua had a very lengthy development study performed on him. The results indicate he suffered somewhere between 4-7 years of age and much of his development slowed or stopped at about that age. It is estimated his skills and mindset are that of about a 6 year old. The doctors are hopeful that with the ventilation needs being met and with a great deal of support at school and at home, Joshua should be able to make up much of the ground he has lost over these last few years. But it will take some time.

Joshua will also be on a strict diet. One of the functions of his hypothalamus is to maintain body weight, by sending messages when you are hungry or full. Because
that is not working properly (and probably because he did not grow taller during one growth spurt) Joshua has developed a very large belly - which he needs to slim down.
***so if you want to give him a snack, etc. please keep in mind it needs to be low-fat and low-sugar***

We will need to (ASAP) get Joshua's hearing aids checked and make sure they work properly and are fitting him as they should. The doctor wants to make sure we are all doing everything we can to make sure Josh doesn't miss a thing in his schooling or our speech to optimize his future development.

And finally, the pacer issue --- This was the greatest dissappointment of all for us.
We had hoped from the beginning of Joshua's getting a tracheostomy tube, that it was to be a temporary fix. Which it will NOT. Joshua could possibly qualify for pacers sometime in the future - BUT - we are told that the pacers will not mean he can EVER get rid of his trach tube and ventilator. That is because the pacers (which by the way are surgically implanted with 4 incisions into the trunk of the body) will help the diaphragm contract (if there is ample space to do so in the body) but will not create enough ventilation when sleeping or when sick. Because there are times when the pacers will not ventilate enough, Joshua would still need the ventilator - and thus, the trach (so he could hook up to it). There *is* a possibility that when he gets older, he might only require a face-mask type ventilation method and THEN he MIGHT be able to get rid of the trach.

This loss of immediate hope that we all held to for so long makes us very sad.

Struggling with wires and batteries and a heavy ventilator and vent stand are proving
to be other obstacles we are trying to work through right now.

Of course, I will also need to get with our insurance caseworker to try and work out
resources for the nursing care and obtaining a second ventilator and stand so we
can carry out all the orders the doctor gave us for Joshua.

Please keep praying for us - this has been a very hard week and it does not look to
be something that will get any easier very soon. We still, though, pray for a miracle healing for Joshua. An uncle and aunt of mine (Ronny & Molly) who happened to travel through Chicago today before we flew out) came by our room and laid hands on Josh and prayed for healing for him.

God bless you all and we look forward to talking to you all again very soon!

Vanessa

Swearing In Ceremony

The Chief of Police asks Josh about his plans for a career in law enforcement



Josh poses with the officers in his department



Proud Family

Aunt Amanda, Sister Steff, Dad James, Mom Vanessa with Officer Josh



Josh is now an official "reserve" Cibolo Police Officer - complete with badge, patch and credentials. His swearing in ceremony was held this morning at Cibolo City Hall.

Josh tells me two of the officers also came to his school today and ate lunch with him. And they plan to come again - and next time, they may have to bring some food in so they can make sure they'll all like what's on the menu. :)

Members of the Cibolo Police Department including McGruff the Crime Dog came by this evening to deliver a special birthday surprise to Josh.
They had a PSP and a couple of games for him. They also told him about a few other surprises they were still planning. They are arranging for him to come to City Hall later this week to swear him in as an honorary police officer, complete with an authentic badge and id "credentials." They said he will be welcome to ride along in their police car then to see what the officers do on patrol. They have also conspired with the San Antonio Spurs and are working on collecting several Spurs memorabilia including signed Spurs jerseys! They also promise a signed jersey from this year's brand new varsity football team at Cibolo's new high school.

The officers also had a surprise in the works for Steffany - who, by the way, they had pulled out of class today to ask for suggestions for a birthday present for Josh.
For Steffany, because they read she wants to be a pilot in the air force when she grows up, are working with Randolph Air Force Base to get her a flight suit and helmet, and want to send her up in a fighter jet!

Both kids were awe struck and very excited. And so are we! We just had to write and share all this great news with all of you!!!!

We will let you know when the swearing in will be and when the flight will take place as soon as we know more.


Vanessa & James

Birthday Party Day!



Josh turns 10 years old on Tuesday,
but since we have the trip to Chicago next weekend,
we went ahead and threw him a party today.



We had a great number of our friends
and church family turn out for the bash.








We enjoyed confetti cake, lemon cake,
homemade vanilla ice cream along with
other snacks and punch.

Josh felt pretty well for the party
and most importantly was home -
not in the hospital -
where he could enjoy all the company,
food and gifts. (Last year, Josh was
in the hospital during his birthday -
in isolation in the intensive care unit
and had just had his trach put in.
He wasn't yet able to eat the cake
and ice cream on his 9th birthday)

A Fundraiser to benefit Josh's trip
to see a specialist in Chicago
was headed up by a group of boys
Josh's age from our church.

These boys found that they could make a great difference
by organizing and operating a garage sale and lemonade stand.
The sale was held Saturday, Sept 16th at the home
of two of the boys in Schertz, TX.
The boys raised several hundred dollars!
Now who says you have to be a grown up
to do something important?

Thank you and may God bless you real good, boys!

Josh and I will travel to Chicago to Rush Hospital
next Sunday, Sept 24th. We hope the doctor there
will recommend a diaphragmatic pacemaker for Josh
so he can live without his trach and ventilator
for the first time in over a year.

Labor Day Weekend 2006

We had hoped this weekend to travel to Killeen, Texas and visit a family who used to live in our neighborhood and to go to their son’s birthday party. We had to put these plans aside because Joshua got sick last week with fever, low oxygen sats during the day and more dehydration. He also had some trouble a couple of days with the muscles in his legs, right arm and back tensing up. I even had to help him get up out of bed, get to the bathroom and to get dressed one morning.

We finally got Joshua in to see his primary doctor (who happens to be his pulmonologist) on Friday. She ordered some blood tests and a sputum culture (by suctioning some secretions from his trach). The blood test showed that his sodium level was way too high, and I believe the doctor mentioned his PH level was high, as well. The results from the sputum culture, which test for infection around his trach, will not be ready until after the holiday weekend. The doctor consulted with his endocrinologist and together they decided Joshua should start taking Cortef (a steroid) and increase his DDAVP (an anti-diuretic pill) to help him retain fluids hoping that will make his sodium levels go back down. The problem with that is it makes Joshua puffy.

The respiratory therapist at the doctor’s office is putting in an order for an oxygen concentrator because we have recently gone through so many oxygen tanks. That may actually be a big help when we go to Chicago, because some airlines will only allow oxygen concentrators – not oxygen canisters onboard.

Speaking of the trip to Chicago, that will be happening toward the end of this month. Josh will be admitted to Rush Hospital the 24th-28th so Dr. Debra Weese-Mayer can evaluate him. She has more experience with patients with Idiopathic congenital central hypoventilation syndrome than any in the San Antonio area. She is also the doctor who performed a test on Joshua when he was in the hospital here last year to check for a PHOX2b gene mutation. He did NOT test positive for it, and we have heard that is common among the patients with Late Onset Central Hypoventilation Syndrome, like Josh.

We are hopeful that Dr. Weese-Mayer will agree with Joshua’s pulmonologist here that he would be a good candidate to receive the diaphragmatic pacemaker. It’s a device implanted in the patient, that triggers his diaphragm to contract and make his lungs take deeper breaths. We think it will help him eliminate his need for a ventilator, which would mean he would no longer need his trach. After Josh gets rid of his trach and his neck heals up, he would be able to go swimming again (we hope) and to submerge in the bathtub again.

As I said before, we do not know what is causing Joshua to still run fevers, but he had a 100.4 temperature again this morning (Sunday). He seemed to feel pretty good and was happy and all. Then, the next time we checked, at lunch, he was down to 98.6 again. He was having trouble with bedwetting that no matter what we did he could not control. When he began taking DDAVP, that problem seemed to go away right away. Now that the doctor has increased how much he takes of it, he woke this morning with a wet bed again. Then, just now, with a b.m., Josh experienced some rectal bleeding – something that has never happened before. It didn’t last long – but is something we will have to keep an eye on and report to the doctor when we next see or talk to her (which I expect will happen on Tuesday after the holiday is over).