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Tuesday, June 30, 2015

copy of an email sent to provider looking for updates on Megan.

 Sorry for the delayed respond time.  It has been a rollercoaster around here between Maxim, nurses, all-nighters and Megan's GI and seizures.

Maxim is patching our nights but at this point each week I have been up 1 - 2 nights per week all night long which knocks me out for the following day to be good for anything.  Work and dr. appts have been being juggled around this.  
Maxim does not have ANY candidates for nites.  Sent one nurse who is waiting to be let go from current job then can come on board but when that will be we don t know. She thought by July 1.  She worked one nite so far along side of Another nurse but has never worked with Megan alone.  A couple of nurses have declined due to complexities - suctioning, etc.  Apparently they are intimidated.  

Megan has had ongoing GI issues - bleeding, vomiting.  Her HGB dropped and she went down to a 9.7 - heart rate, 02 sats and fevers have been everywhere.  She was on 02 for a couple of days and once the iron rx started to kick in she is managing 02 ok - but iron kills her stomach.

GI is increasing her seizure meds due to increase in seizures - seizures that are lasting a lot longer than normal for her.  We follow up in 4 weeks to see how adjustments are going.

Saw a new GI dr. last week too since the ones we have had for years are not doing anything for her.  Dr. Narwal is new one and he is well versed in Aero-digestive which is what she has needed all along but there has not been anyone here to access.  I was planning a trip to Cincinnati Childrens in Sep/Oct to see their team but that may not be necessary now with him here.  He is suggesting that we separate the G and the J and have one for each due to her motility and parastolosis (spelling?) - however she is too fragile right now for any procedures so we are seeing him again in 4 weeks.

Also just recultured yesterday to check for MRSA in airway again as she just finished another 21 day course of AB to treat it.  Should know by next week.

There are no plans for anyone in my family for the entire summer as we can not count on our nursing schedule for anything and Megan's  health isnt up to any outdoor or traveling activities.   WEhave two nurses going on vacation which means I am going to be doing days and nights from mid July through August 20 when other nurse returns.

Not doing real well with all of this as you can imagine.  It is really disheartening and depressing.  The quality of life around here is fair at best these days.

Friday, February 20, 2015

Letter for help...waiting to hear back with high hopes

I would like to start this email by thanking you for taking your time to read this and for any possible assistance/direction you can provide.  I would also like to establish that I am able and willing to travel to Ohio to seek any improvement to my daughter's  health.    I feel it is important to give you some  history on my daughter which I have summarized as detailed but briefly as possible as  to not take up too much of your time.  I can provide any additional information requested including pictures and video.

My dauughter, Megan,  is currently 8 years old.  To date her primary diagnosis is encephelopathy with white matter demylenation & disorder of mitochondrial function that is suspected to be seondary to a primary metabolic defect (lysosomal?).  I was doing some personal research on excess mucus production and came accross your article and felt very strongly about contacting you for assistance.  My daughter was born full term in October of 2006.  Pregnancy and birth were normal and all appeard to be fine with her.   Within the first 3- 6 months after birth she developed seizures and was diagnosed with a bilateral sensorinueral hearing loss.  She was 17 pounds at that time and to date has only reached 29 pounds and is 40 inches.
Shortly thereafter  we learned she is also cortically visually impaired and was not thriving despite dietary changes and she had a GTube placed at 15 months old for supplemental nutrition.    At slightly over 2 she suffered a sever and almost fatal reaction to ACTH used to treat the infantile spasms she developed .  She was intubated  in February of 2009 due to this reaction, but was unable to extubate due to hospital acquired infections,  excessive sedation and complication of a solumedrol medication error while inpatient.    She had a tracheostomy 6 weeks after intubation that allowed us to bring her home.  Although ventilator dependant at the time, she was completely weaned from the ventilator within 6 months of coming home.    Within another 6 months her trach was nothing more to her than a source of infection.  She was decannulated almost one year to the date (4/2010) that she had the initial tracheostomy and from a respiratory standpoint was doing well.  Respiratory was not the main concern for her.  Seizures and GI were on the forefront.  After being intubated Megan no longer tolerated eating by mouth so her GTube was used for continuos feeds.  Although motility was always an issue,  it progressed to gastroparesis and she was losing weight.  She had a trial of a gastric stimulator on 2/2012 and did wonderful with the outside temporary placement.  The permanent device was placed in April of 2012. After  initial placement the surgeons adjusted the settings to increase the pulses to allow for more motility at intervals of 4 approximately 4 weeks.  In June of 2012 Megan started producing mucus that seemed to be all upper airway.  There were large mucus balls that we were getting out of her stomach that would literally back up the feedings inhibiting the stimulator to do its job unless we cleared them out hourly.  The consistency was that of egg whites and it was constant.  She was having frequent episodes of atelectasis with increased heart rate, elevated body temperature, strained breathing, etc.   due to mucus in the upper airway.  Once th mucus plug was cleared all would resolve for a short while (a day).  She was also received regular neb treatments - every 2-4 hours consisting of pulmicort, albuuterol, saline, CPT vest.   It kept progressing and she eventually went onto adding mucomyst, pulmozyne and alternating Tobi nebs every other month such as a CF patient does.   This past spring (5/14) Megan's GI tract again was not tolerating feedings and she was losing 1 pound per week.  The physicians turned up the device and it was worse. I had her admitted through the ER to have the device shut and they placed a GJ Tube for emergency nutrition and shortly thereafter removed the device completely as they discoverd that the pyloric valve had almost sealed which was the cause of the gastric stimulator malfunctioning for her.  Megan has not lost any weight but is also not gaining either weight despite continous feeds at a rate of 53 ml per hour  with adequate caloric intake.  53 is the highest rate we have been able to achieve which is record setting for her.  Megan also has a history of GI bleeds as evidenced by the coffee grounds we  get in her GTube aspiration  contents and also sometimes in the oral suctioning.There is a dried bloody crust in her left nostril several times a week as well.  Endoscopy did not reveal the source of the bleed.  

All through this time I was searching with her physicians as to where this mucus was coming from and questioning everything.  She was inpatient several times for different tests and procedures to try and determine what was causing it as her quality of life had deteriorated to not being able to do anything but get  nebs and have suctioning.  We discovered she is IGG and IGA deficient and receives immunoglubulin therapy.  Every allergy for foods was weeded out and she is on a home made gluten, dairy, corn and soy free blenderized diet that I prepare daily for her.    I was hopeful that maybe the stimulator removal would give us some answers and maybe the nerve stimulation was creating this mucus.  We very rarely have the mucus in the stomach since it was removed but we are not feeding through the stomach any longer either so to say it was from the stimulator being removed I can not.  She still coughs copious amounts of mucus that is so viscous in nauture it chokes her and clogs the suction tubing.  It rolls in the back of her throat like an oyster when she is able to cough it up.  She  sometimes is too weak and tired to cough and the secretions are so thick we work for hours to thin them out with various treatments to get them to be able to "move".    Bronchoscopy and endoscopy revealed nothing.  Physicians were amazed at how good everything  looked and were taken back that she did not look like what they would have suspected by what they saw on the outside.  All I have gotten is to keep doing what we are doing.  Between GI, Pulmonology, ENT, Nuerology, Allergy and Immunology there has been no relief for her or answers to help.  If we could find some treatment for this mucus that is drowning her it would be life altering for her and our entire family.  

I saw several topics on acetlycholine and mucus and eventually somehow found my way to your article.   I am sure it is apparent that it peaked my interest and knowing what a wonderful institute Cincinnati Childrens is I felt confident that if there is help for my daughter to be found this was a reliable source of information.

Once again,  thank you for receiving and reviewing my email to you. 

Megan's Movie