NoahDavid's Journey

Come unto me, ye who are weary and overburdened, and I
    will give you rest.
                                                                                 - Matthew 11:28

Spaghetti face...again!

Happy 3rd birthday Noah!

So far, so good...for the most part.  In January 2009, Noah did pretty good.  He had a few new problems with bleeding.  His tongue and lips were more sensitive than usual.  I put him to bed one night and blood was coming out of his mouth.  He had fallen asleep on my lap drinking on his sippy cup and when I laid him down, I noticed there was blood on my arm and coming out of the corners of his mouth.  I thought maybe he had bitten his tongue, but he didn't wake up if that was the case.  Then another day he was just talking to me and his lip started bleeding.  It didn't last long, but he hadn't hurt himself and it was weird.  

Noah's cheeks started to get thicker to the point that he was having a hard time using his back teeth without chewing on the inside of his cheeks.  His tongue was slowly getting bigger also.  It is formed to the roof of his mouth and is "hill shaped" in the middle.  You couldn't really tell that much difference on the outside, but it was slowly progressing. 

Because of the slow progression, we decided to ask the ENT again about having Noah's tonsils removed.  We had been putting it off because he has a large cyst behind his adenoids on the left side.  We were afraid that if it is too close to his adenoids and gets cut into by accident that it will make the LM grow.  We also wanted to give him a break from anesthesia.  He was still having lots of problems with sleeping because of the lack of room in his mouth. 

Noah had his first dentist's appointment in January also.  He was so scared, he wouldn't even let them clean his teeth.  It took them over 5 minutes just to talk him into letting them do an x-ray.  And they didn't get the 2 side mouth x-rays they needed.  However, Noah did let the dentist look in his mouth, which is what every doctor wants to see, so he's used to that. Well, the dentist didn't have good news.  He said that Noah has at least 4 cavities in his molars that he could tell.  He said it's very possible that he has more.  He went on to tell me that because of his age and being so scared, the only way he could fix them would be to put him under.  Of course, I wasn't happy about it and started bawling.  He was pretty sure that the local hospital wouldn't let him do it there because they would say that they didn't have the necessary ped. specialists to take care of him because of the LM and TRAPs.  (I wouldn't want them to do it there anyway.  We haven't had a good experience with the local hospital when it comes to Noah.)  So he said he would get ahold of his ENT and Rheum. docs in St. Louis and ask what they suggest.  He said he may be able to refer us to someone at Children's in St. Louis to do it for Noah, that way he will be in a hospital with his specialists.  I had to sign a ton of releases for medical information. 

In the beginning of February, Noah woke up and the tip of his tongue on the right side was turning bluish black, but it was underneath the white tissue on his tongue so it was hard to see what the problem was.   I thought maybe it was a blood pocket, but couldn't tell.  He said his tongue hurt and the blebs were red and purple so I thought we'd better take him in.  Doc said that he thought he had thrush and put him on nystation and fluconazole.  It seemed to help some, but the white stayed on his tongue.

The dentist called finally.  They said they had talked to Noah's doctor and they said it was fine to try valium, in house, to sedate him and try to do the work on his teeth.  I thought it was a pretty crazy idea myself, especially after they explained to me that they would write a prescription for it and I would give it to him at home an hour before I brought him in.  I was thinking, what happens if he has an allergic reaction and his throat swells up and I'm 45 minutes from the nearest crappy hospital?  Then I asked if they had talked to BOTH of his specialists, the LM doc AND the TRAPS doc.  That's when they said they weren't sure.  It was noted that they had talked to his TRAPS doctor.  I told the dentist's office that they needed to speak to both because they are totally different specialists that take care of two separate conditions.  I told her that there was no way that I would let them do it if they didn't have approval from the LM doctor.  So she said she'd check into it and get back to me. 

 In the middle of February, I got a call from Noah's ENT doc's nurse, almost frantic.  I missed the call and she left a message.  She said the dentist had called and left the wrong number so she couldn't call them back.  Then she made it very clear that his LM doctor did not want them to sedate him in the office in no way, shape, or form.  She said I needed to call her and she would get me a referral to one of the pediatric dentists in St. Louis. 

 I called her back when I got the message.  She said she and the doctor were confused why I would even consider that because of possible airway issues.  Then I explained to her that they were supposed to call and just get a referral on what they should do, and I explained that we weren't comfortable with it at all. 

 So she put me on hold to try to set up the dentist appointment.  She said that she talked to someone from dentistry and they were going to review Noah's medical history first and decide which dentist would be able to handle him best and call me back with an appointment.  This way, if there were other issues, his LM specialist can be there if they have to put him out.    I don't want him to have to be put under anesthesia just for dental work, but if it has to be done, at least we'll know he's in good hands.  

Noah had doctors appointments in St. Louis in late February.  He saw both his ENT and Rheumatology specialists.  Both appointments proved to be uneventful.

Rheumatology seemed to be confused by the fact that Noah has less severe fevers than most TRAPS kids they see.  They had not been consistent by any means, at least not for a long period of time. He was only getting them in the morning.  However, his flare-ups were consistent.  They happened approximately every 4 weeks.  One of the residing docs seemed to think that other symptoms he had were probably not related to the TRAPS unless he was having an active fever.  I was frustrated by this.  I asked on the TRAPS support group if anyone else had the same low grade fevers mainly in the morning and sure enough, Noah was not the only one.  Both of the other kids that had symptoms similar to Noah's have GI problems, which he had started experiencing on a regular basis.  He started taking Mirolax for constipation to try to help him. He was having a hard time having regular bowel movements unless he consistently took the medicine, and then they were so huge that I didn't know how he did it.  He was also having a lot of stomach pain that goes along with it. 

 ENT had a good report.  They did a speech test with him and were happy with the results.  I was very surprised at how well he did.  ENT said that he had a bit of an overbite which is consistent with kids like him with enlarged tongues.  He said to expect for him to have braces at some point down the road.  (I'm not sure quite how well that will work with his cheeks and tongue rubbing on them)  He said he did not want to go ahead with a tonsilectomy unless he started having sleep apnea.  He said letting him have positional breathing was okay as long as sleep apnea was not an issue.  He referred us to the Pediatric Dentist at Children's in St. Louis also.  He said he would not be comfortable with him being seen by anyone else.  (That means that I'm not just a paranoid mother!)  He said if he has to have a procedure to correct his dental problems that he would make himself available so that if he was needed, he could be there. 

Both ENT and Rheumatology said they didn't need to see him again for 6 months.  We are still on a wait and watch basis with the cystic hygroma until he's bigger.  We proceeded on with trying to get him into NIH for the TRAPS, especially since he was having bowel problems.  All of his records had been forwarded to them from St. Louis.

Noah had not had many problems with his tongue since the appointment.  He had a few episodes of it turning bright red for a day or two but no pain with it!  He had one day where his tongue almost looked totally normal-no discoloration, bumps, and normal size, but it was followed by two days of swelling in his cheeks and tongue with pain in his jaw.  Just can't win for losing!

In May, his TRAPS flared up again bad enough for him to run a fever for 5 days straight.  It was awful!  Kinda like old times-when he was younger.  He was in so much pain that all he would do is let me carry him around and scream between naps.  In June and July his flare was back to low grade fevers in the morning and joint pain.  We were able to take him off the Mirolax after he had been on it for almost 3 months. 

In late July, we got a call from NIH.  He was accepted to be seen!!  Maryland, here we come!  But not until November. 

At the end of August, Noah had follow-up appointments with Rheumatology, ENT, and saw a new dentist in St. Louis.  ENT said that they could do RFA (Radio Frequency Ablation) or laser treatments on his tongue to resurface it.  This would be mainly cosmetic, but would lessen the amount of bumps in his mouth that flare up.  He said it usually recurs after a year to year and a half.  The rheumatologist appointment was unremarkable.  The dentist said that he had 4-5 small cavities, and wanted to put him out so that he could get the work done all at once.  If we decide to have that done, the ENT can do the RFA at the same time.

On August 28th, Noah got a low grade fever and swelled up.  Although we were alternating Tylenol and Motrin, it was not enough to help with the pain.  By the 30th, his tongue became very swollen as well as his cheeks, gums, and bottom lip.  We took him to the ER where they put him on antibiotics and sent us on our way.  They said that if the swelling didn't get better to bring him back in and they would order him a steriod shot.  Later on that day, the pain and swelling persisted, so we called the ENT resident on call in St. Louis and they suggested that he be admitted into the hospital and put on IV antibiotics and steriods for at least 24 hours.   

While in the hospital, we got a call from the National Institue of Health.  Noah's appointment is on Nov. 17th and she told us that he will not only be seeing the TRAPS specialist, but they are also sending him to the ENT there for his lymphatic malformation.