Ryli’s Journal

This page is a compilation of Ryli news over the past couple of years. If you plan to read it all, I suggest that you get yourself a nice cup of coffee. The information is presented in chronological order.  Enjoy.

The Journey Begins…

May 16, 2004

Ryli was seen at Swedish Medical Center Emergency Room due to muscle jerking.  She was looked over and diagnosed with Infantile Spasms, Bilateral hip dislocation (from birth) and possible blindness.  She was given a CTscan of her brain but nothing but a normal 3 month old brain was found. Ryli was transferred from Swedish Hospital and admitted to Children’s hospital where several tests were performed on her such as a Lumbar Puncture, countless blood work, Eye tests, CT Scans (which showed a normal 3 month old brain) and her first EEG.  Ryli was given an EEG to see what her brain activity was doing while she was having her Infantile Spasms.

 

 

ACTH

May 19^th thru July 3^rd, 2004

Ryli was fitted for a Pavlic Harness for her Bilateral Hip Dislocation.  You will see a band-aid on her leg where she received her first dose of ACTH.  ACTH is a Steroid that Ryli will receive to help her seizures go away.  Ryli is having up to 80 seizures daily.  ACTH can only be used up to 8 weeks and no longer.  ACTH is a one-time only treatment.

We want to give a little advice about ACTH for parents who may have a child with Infantile Spasms.  You will be told that there is no medication for this but ACTH or a similar drug from Canada, and there isn’t.  We gave our child the 8 week round of ACTH.  This is a very awful drug.  Ryli cried 24 x 7, threw up every time she ate, ACTH ate a hole in her stomach and her brain Atrophied which we were told after finishing ACTH that this is a side effect (Ryli’s condition could have caused atrophy as well—still unknown) Ryli relapsed 64 days after ACTH was done.  We tried 400 mcg of Folic Acid (200 mcg twice a day everyday) and it took away her seizures from the very first dose.  If you can spare a day or two of trying Folic Acid first, this just may help your child and ACTH may not have to be given.

 

 

Spica Cast

July 9, 2004

Ryli’s hips were not staying where they needed to be so her Orthopedic Surgeon Dr.Chang suggested that Ryli go to the Operating Room to be casted with a Spica Cast.  She will be in this cast for 6 weeks.

Also, Ryli is done with her ACTH shots.  These shots were so very hard on our little girl.  She cried 24x7, was rushed to the Emergency Room by ambulance due to throwing up blood.  The ACTH ate a hole in Ryli’s asophagus/stomach .  A CTscan was repeated and now is showing Brain Atrophy which means that Ryli’s brain shrunk and has a lot of space in between (brain tissue thinned out) along with Subdural Hematomas.  Her Neurologist stated that ACTH has been known to cause Atrophy but not Subdural Hematomas. 

Now, Ryli never cries.  Even when she is hungry.  She has forgotten how to suck her bottles but her home nurse Janice re-taught her.

 

 

 

Cast Removal

August 13, 2004

Ryli went into Surgery and underwent Anesthesia to have her Spica Cast removed. 

Her hips looked like they were where they needed to be and sitting pretty strong in the sockets.  She went from the Cast to a Rhino Splint. 

Also, she stopped sucking her bottles again.  The home nurse cannot get her to suck her bottles.  We had a Speech Therapist come out and help us to get Ryli to suck again but it just didn’t work.  We are now pouring Ryli’s formula into her mouth little by little.  Most times she swallows but sometimes chokes on it.

Ryli still never cries.  Not even when she is hungry.

 

 

 

Respiratory Problems

November, 2004

Ryli has been admitted in the hospital once in November and twice in December for Aspirating/Respiratory Distress. Ryli is not able to swallow now as well as cry or suck. Ryli’s Ctscans still show Brain Atrophy.  Her brain is growing, painfully slow but there is still thinning of the Brain tissue and a lot of space between the brain and scull.  Ryli had an ERG (vision test) and an Eye Biopsy done under Anesthesia in December.  The ERG shows that Ryli has vision but the amount is unknown at this time.  The Eye Biopsy did not show any Metabolical Disorders that her symptoms pointed to.

 

 

 

Ilfeld Brace

Christmas Eve, 2004

Ryli is now in an Ilfeld Brace.  The Rhino Splint should have been a faster way of getting Ryli’s hips to grow her socket but it was not working.  The Ilfeld Brace is just a different brace to try out.

 

 

 

Ryli's G-Tube Surgery
December 31^st, 2004
Ryli went in to Littleton Adventist Hospital on December 27th, 2004 with difficulty breathing due to mucus. They suctioned her out and got her breathing normally again. While she was there, they did a swallow study on her, and we determined that she was aspirating (inhaling into her lungs) her formula, and refluxing (pushing it back up from her stomach into her throat). Dr. Thane Blinman inserted a G-Button on her belly on New Year's Eve, so that we could feed her without risking her life every time. He also put a Nissen where her stomach connects with her esophagus. The Nissen prevents reflux. Ryli recovered quickly from this surgery, and began to gain weight at a rapid pace. She actually got back on the growth charts shortly following this surgery.

Ryli’s Nissen Fundoplasty makes her Retch (gag) so much that she aspirates her Salivations into her lungs.  She was given the Gtube with the Nissen so she couldn’t aspirate her food anymore but now her Salivations are putting her life in danger.  She underwent yet another surgery just 2 days after her First birthday (02-19-2005).

 

Ryli's ENT (Ear, Nose and Throat) Surgery
February 22^nd thru February 25^th, 2005
Ryli went in for her Adenoidectomy, Tonsilectomy, Neurectomy (cutting of nerves behind the ear drum), and Sinusitis Surgery (enlarging her nasal pathways) at Presbyterian/St.Lukes. Dr. Nigel Pashley perfromed the surgery as an attempt to lessen the amount of salivation, and allow her to swallow more easily. As you know, swallowing is a difficult process for Ryli, but one that most of us take for granted. A side benefit of the surgery is that colds will be easier for her to handle once she is older. This should have been an Outpatient Surgery but as you can see in the picture, this surgery was way too much for her little body.  She stayed in the hospital from the 22nd to the 25th of February, 2005.

 

 

Ryli's Intubation

April 13^th thru April 22^nd, 2005
Well, yet another scare. Ryli went to the ER at Littleton Adventist last Wednesday night. She was having a hard time breathing again due to the mucus that she cannot bring up on her own as her cough isn't that strong. Since she had to be intubated, Littleton had her transferred to Swedish since Swedish hospital is a Tier 1 hospital. Ryli was intubated from last Wednesday night (couldn't breath on her own or with oxygen) until Saturday. She had to stay in the Critical Care Unit after extubating her since she was still having troubles with her breathing.  The reasoning for this trip was Para Influenza Type #3 and Aspirated secretions into her lungs.

 
She is home now and doing pretty good. Still really mucousy but with her Meds, Nebulizer and Suction machine - hopefully we can stay out of the hospital for a while. It seems like every time she ends up in the ER due to her mucus, the procedures done on her becomes a little more severe. A Tracheotomy is a great possibility in the future if Ryli cannot bring up her own mucous but we'll cross that bridge when we get there. Please keep her in your prayers.
 
It’s hard to believe, but this picture is the day after she was discharged from Swedish Hospital.

 

 

 

CT Scan
August 8^th, 2005
Ryli had another CT scan this last Tuesday and her visit with her Neurologist yesterday to go over her CT scan. Ryli's Subdural Hematomas (bleeding on/around the brain) is completely gone. Her atrophy is still there but her brain is about the size of a 6 month old now where 3 months ago it was the size of a 4 month old. Slowly but surely Ryli seems to do a little better.

Orthapedic Appointment
August 10^th, 2005
Ryli had her orthopedic appointment yesterday. Her hips are perfect!!! Finally, 1 year and 3 months of working with casts and different harnesses and many, many, many X-rays - they are finally good. However, her spine's curve shows that surgery would definitely be needed but since she's still so small, her Orthopedic surgeon wants to try harnessing the spine for at least a year and then see what they have to work with...Ugh but hopefully her spine won't hurt her so bad anymore once it's forced into the position it should be in.

 August Hospitalization
August 13^th thru August 29^th, 2005
Ryli had another close call, and spent 16 days at Swedish Medical Center. Click here for details.

 

October Hospitalization

October 22^nd thru November 2^nd, 2005

Ryli ended up in PICU for another intubation.  Aspirated Pneumonia again.  Since Ryli cannot swallow anymore, she is aspirating her salivations into her lungs. This visit Ryli earned her Tracheotomy on October 27^th. This should make getting to Ryli’s salivations easier and we’re hoping for less hospitalizations.

 

January 16^th 2006

Shriners appointment in Salt Lake City, UT

We flew to SLC, UT to get Ryli’s spine and hips looked at. The Shriners come to Denver Colorado every other month for 1 day but in order to have Ryli become a patient—our 1^st appointment had to be in SLC. 

The Shriners talked about a “Titanium Rib” which is a bar placed in between Ryli’s rib cage from the left rib to the right rib keeping the ribs expanded so the spinal curve does not effect Ryli’s lung space.  Also, her hips are in their sockets but the sockets are not forming and are still shallow because of Ryli’s inability to walk or weight bare.

 

Pseudomonas

Year 2006

Ryli’s biggest battle with having a Tracheostomy is Pseudomonas.  This is a bacteria that lives around Ryli’s trach site because of the trach being a foreign object, moist and near air.  Since Ryli has this bacteria, she will always have it as long as she has her trach.  You cannot get rid of it.  Therefore, when Ryli’s Pseudomonas gets “high”, she is either on Tobramycin Nebulizer treatments twice a day for 30 days or Cipro which is an antibiotic.  Ryli has rotated these 2 every other month for the past entire year.  It’s rare that Ryli’s Pseudomonas level stays at a moderate level.  Ryli is colonized with this bacteria, even Ryli’s eyes are colonized with it.

 

RSV
December 22^nd 2006

Since Ryli has been on Toby or Cipro for the entire year, Ryli’s Pulmonologist doctor wanted us to go to the emergency room for chest xrays.  We did this and Ryli tested positive for RSV.  Even though Ryli gets her yearly Synagis (shots to help prevent RSV) she still gets this virus.  They wanted to transfer Ryli to another hospital for admission but I finally refused and took her home.  Ryli had wrapped presents under our tree and I didn’t want her to miss out on another holiday (Halloween admission).

We took Ryli home and she seemed very happy and perfect.

 

January Johns Hopkins Trip

January 31^st thru February 11^th 2007

Finally, we are going to Baltimore.  It took us a year and a half to get Ryli set up as out-patient at Johns Hopkins.  We have wanted this for a long time. We are bringing Ryli to have some Johns Hopkins doctors look at Ryli to help us figure out what is going on.  A diagnosis is our goal.

Ryli’s first appointment, February 1^st, was with Dr. Cohn, a geneticist at Johns Hopkins.  He could not think of any more tests to be done as Ryli’s test history is pretty thorough and complete.

February 2^nd was Dr. Thomas Crawford, Neurologist.  This is actually our current neurologists friend.  He spun Ryli around in circles to see how her eyes would respond.  Needless to say, Ryli’s eyes started bouncing back and forth about a minute after he was done spinning her.  This showed us that Ryli’s brain reacts very slowly—which we already knew this per her EEG’s in the past.  He ordered a Nerve Conduction test (info is on Latest News tab).

February 3^rd, Ryli wasn’t feeling well.  She was foaming from her trach, nose and mouth quite a bit that day and was just really not responsive.  We were hoping to keep Motrin in her and use her Airway Clearance Vest to loosen anything in her lungs.  I thought Ryli was shivering through the night because when we felt her forehead, she was nice and cool.  The pulseox that our DME company sent to Baltimore for us did not work so we couldn’t use it.  About 5am the next morning, the twitching began again, yet Ryli’s forhead felt cool.  I turned on the lights to find that Ryli was having a seizure.  The shivering throughout the night were actually seizures.  We called 911 and they rushed her to GBMC (greater Baltimore medical center).  Here is where we found out that Ryli had a temperature of 103.5.  Ryli’s forehead still felt really cool but her hands were on fire.  Since Ryli doesn’t regulate body temperature very well, her fevers come out in her hands.  Anyway, Ryli was full of blood outside of her mouth and nose due to suctioning that the hospital had to do.  Xray after suctioning and bagging Ryli showed a Pneumo-Thorax (collapsed lung).  Since this hospital was not a trauma hospital, a Johns Hopkins doctor and their own ambulance came to get Ryli.  She was transferred to Johns Hopkins.  Kind of our dream come true at this point.

Ryli was at Johns Hopkins PICU and on a breathing machine for several days.  During this time, all the out-patient appointments that we had, actually came to her. 

The orthopedic doctor evaluated Ryli’s spine and hips, the Opthamologist dilated Ryli’s eyes and examined her to find that the prescription glasses are probably accurate.  Ryli has visual problems but the amount is still unknown.  Dr. Cohn visited her almost on a daily basis.  He organized a skin biopsy as well as a muscle biopsy done by Dr. Crawford to be done the same time.  These doctors will test the muscle and skin for certain diseases/disorders and keep them for future testing.  All these tests came back negative. 

Ryli was discharged on oxygen February 11^th so we could come back home.  They wanted to keep her and didn’t want her to fly for another 4-6 weeks due to her pneumo-thorax but we needed to come home to our son. 

We got home and Ryli caught the stomach flu as her roommate in the hospital had the stomach flu.

She was on oxygen for about 2 weeks after getting home to Colorado and was finally weaned off.