Your questions answered!

Q: How long will Lily have the tube?

A: Unfortunately there isn't an exact answer. She will have the tube as long as she needs it to survive. At this point she isn't improving at all in her dysphagia, actually she is getting worse. If she keeps getting worse they are gonna take oral feeding completely away. And over the past couple weeks she has started refusing again. It took 9 months to get her to accept more than 3 oz at a time, and now she is back to 3 again. So, as of right now there is no end in sight as far as the tube feeding goes.

Q: How long do her feeds take/can she move around while being fed?

A: She gets fed for 10-12 hours continuously every night while she is sleeping, and for a while after she wakes up in the morning. She is on a relatively slow rate of 55ml(approx. 1.8 oz) an hour. During the day she has a goal of 12oz by mouth, I try and give her 3 bottles of 4oz each, but as she refuses I then have to bolus feed her (put the formula in a giant syringe and push it slowly through her tube). Occasionally it isn't safe for her to eat by mouth at all (like when she is super sick) So she sometimes has to go on continuous 24 hour feeds. She can move around while being fed I just have to follow her around holding the pump. As she gets older and hopefully stronger there is a backpack she can wear to make the pump more mobile.


Q: Can she be potty trained when she is old enough?

A: Maybe...kinda. It all depends on how weak her core muscles are and  weather she will have the ability to strengthen her muscles enough to be potty trained. Then there is the feeding tube. If she is strong enough there is still the issue of her being fed continuously while she is sleeping. From what I understand it is sometimes possible to potty train tube fed kids during the day but not a realistic goal for over night potty training. She will need diapers at least overnight as long as she is fed overnight...

Q: Does her tube hurt her/is it sensitive?

A:The tube doesn't hurt her unless she tugs on it or it gets caught on something. I have to spin it daily (which she doesn't even notice) and We have recently discovered she is super ticklish all around it and will actually laugh out loud when we poke her skin around it (laughing out loud is exceedingly rare for her).


Q: What is it that makes her able to handle some foods and not others?

A: Eosinophilic Gastroenteritis is the main culprit for her being so sensitive to so many foods. Because of her Eoe, she has many many allergies/intolerance's. Then there is also the issue of her dysphagia/silent aspiration, For things that are safe to eat from an allergy stand point, they still aren't safe for her to swallow. Like water for example, every bit of water that is swallowed goes into her lungs, so if I were to give her a 6 oz bottle of water she would drink it and would drown. On days when she is teething and has excessive amounts of drool we have to constantly listen to her lungs because it goes to her lungs and she doesn't consistently cough it up (silent aspiration).


Q:Will Lily be able to attend regular school?


A: Honestly, probably not. Her immune deficiency makes public school way to dangerous. Plus the nature of Mito is, it is progressive, and so by then she most likely will be sicker than she is now. Arizona's special education programs (in my opinion) are sorely lacking. She will either be home-schooled, or preferably she will go to a private school where they are equipped to handle medically complex children. So far it looks like she is cognitively fine, even gifted (YAY!) as far as intelligence goes, so that also poses a challenge for schooling her because finding a program that can tailor to her physical needs but still challenge her intellectually is hard to come by.


Q: Is Lily a million dollar baby? (Do her medical bills before insurance equal one million yet)?

A: If my calculations are correct, then yes. Just between her hospital stays and her IVIG alone equal 1 million. That doesn't even include all her doctor appointments, medicines, equipments, therapies, etc. 


Q:Are we considering having more kids?


A: This is a tough one. We definitely will have more kids there is not question in our minds about that, but as of right now it looks like we will be adopting. The percentage of having another child with Mito is extremely high, and well, lets just say it is not something we are taking lightly. The decision will be made through much prayer and meditation on the subject and ultimately we will do our absolute best to do God's will. 
 


Q:What does the future hold in store for Lily.


A: This is what my nightmares are made of now. Mito is progressive that much we know. But the thing we do not know is how fast it will progress or what will be affected next. Most common symptoms include:

• brain: confusion, memory loss, headaches, seizures, developmental delays, and stroke-like episodes
• nerves: pain caused by nerve abnormalities (neuropathic pain), gastrointestinal problems linked to nerve abnormalities, abnormal sweating, and fainting
• skeletal muscles: muscle weakness, muscle cramping, muscle pain, loss of coordination, exercise intolerance, and poor growth
• liver: liver failure and low blood sugar (hypoglycemia)
• heart: heart muscle weakness and disturbed electrical signals in the heart (called heart block)
• kidneys: abnormalities that cause difficulty with absorbing nutrients and electrolytes back into the body (called Fanconi syndrome)
• ears: hearing loss
• eyes: eye muscle paralysis, progressive loss of vision
• pancreas: diabetes (a group of conditions characterized by excessive urine excretion and persistent thirst) and pancreatic failure
• Sensory processing disorders, or autism

Other symptoms include failure to thrive in infants, poor growth, short stature, fatigue, respiratory disorders, swallowing difficulties, and increased risk of infection.

Read more: http://www.answers.com/topic/mitochondrial-disorders#ixzz1mV1wSD4r

Only 20% of children with infant onset Mito will make it to adulthood, and out of those 20% very very few will see their 30th birthday. It is a cruel and sinister disease.

Not two people get mito exactly the same way, and just because somethings aren't listed here doesn't mean it can't be caused by mito. Mito is a guessing game at best, and since there is not cure and no real treatment we just take one day at a time and we and her doctors are forced to watch every little change closely and decide if it is a symptom or a personality quirk or a "typical baby thing". Kids with mito can go from acting and seeming fine to complete system shutdown within hours. That is why everything is watched so closely. The sooner we catch a new symptom the sooner we can stop it or at least slow it down, if possible.

The future terrifies me. I am doing my best to live here and now, because that is where we are and that is where I have her safe in my arms. We have to just keep swimming. And we will accept God's will in ALL things. He is in control and He knows whats best in EVERYTHING. I trust him with my life, and soul. And I trust him With Lily's as well. I will have her as my daughter forever. That I know. And no matter how long any of us are on this Earth we will be together forever and ever in Heaven. 

GI diagnosis'

From the minute Lily was born she has had trouble with feeding. Her GI issues are vast and complicated to say the least. I will start with her first actual diagnosis.

GERD: Gastero Esophogeal Reflux Disease aka Reflux. Lily was diagnosed with "an extremely severe case of Gerd at 3 months old. Basically everything that went into her stomach came right back up. I mean basically EVERYTHING! She was 6 lbs 8 oz at birth and by 4 months old she weighed barely 8 lbs. She had only grown 1 inch longer as well. I could see every bone in her frail little body. She was Diagnosed as Failure To Thrive (FTT). By the time they gave her her first feeding tube she couldn't lift her head, all facial expressions had gone away. She was almost lifeless.The doctors were worried about brain damage from malnutrition. She was starving to death and no matter how much i fed her it made no difference. She got her Ng tube when she was 4 months old. The feeding tube saved her life. I will go more into feeding tubes in a later post.

 Sandifer syndrome: In super rare cases of super severe GERD Sandifer Syndrome can occur. When she was 6 months old she was admitted to PCH for apneas(not breathing) and "seizure like activity". About 10 times a day Lily's right arm would start flailing uncontrollably, her tongue would start thrusting, her head would roll back, her back would arch over and over and her eyes would roll back in her head. And she would scream. After 2 brain MRI's, and 2 EEG's, a Cat scan, and an ultrasound of her brain through her fontanel. They ruled out seizures. So I started researching and found a YouTube video of a little boy with "confirmed sandifer syndrome" It was so similar to Lily. We went to a doctor who referred us to another doctor who referred us to another doctor who confirmed Sandifer Syndrome. Apparently when reflux is severe enough it affects the nerves that also control movement and causes spasms. It is controlled only when the reflux is controlled. So we just give her a cocktail of anti-reflux meds and it seems to work. So if I forget to give her her medicine Its OBVIOUS because she has "sandifer fits". Luckily this diagnosis doesn't cause any physical harm its just a bit embarrassing for her as she gets older.


Silent aspiration: This one is simple to explain but it scares me. Basically anything Lily swallows that is thinner than the thickness of Honey goes into her lungs. Water or food in lungs = BAD!  She can't drink water. Her formula must be thickened to a "Honey Thick consistency" with rice cereal. And she has to drink it all from a gerber med flow bottle nipple. I tried to drink honey-thick liquid through a med flow nipple and ITS HARD!. My cheeks were sore. Its no wonder she gets exhausted after just 4 oz. 

Dysphagia: Dysphagia is a weak or non existent swallow. Lily's is weak. When she was born it was non existent. It is treated with a feeding tube. A short documentary on You Tube explains it so much better than I can. Here's the link: http://www.youtube.com/watch?v=MrbEUDO6S5U&sns=fb
(copy and paste it into your browser)


Eosinophilic Gastroenteritis: An Eosinophil is a white blood cell. In EoE patients(that's the abbreviation for Eosinophilic disorders) eosinophils gather in large quantities in either the esophagus, stomach, or bowels. In Lily its the stomach and bowels. It causes inflammation and damage to the stomach and bowels. It causes severe pain, vomiting, and diarrhea. In severe cases it is treated with a feeding tube. EoE patients are on very restrictive diets. In Lily's case just Beech Nut rice cereal, And Neocate, with an occasional and very conditional bit of thickened sweet potato baby food. There is no known cure.

Laryngomalacia: basically the vocal chords are too soft an floppy. They also don't always move. There are times when they are barely moving and that is why she has stopped breathing so many times.If your vocal chords freeze up then air cannot get to the lungs. She was on an apnea monitor until she was 10 months old. She used to stop breathing and actually change color up to ten times a day. She had recently gone 4 months without an apnea until last week. :(   It also affects her speech. Some times she can say Mama and other times she can only mouth the words and no sound comes. We don't know how long this will affect her. It is quite possible she will go through life being able to speak some days and not on others. Only time will tell. There is a surgery that can fix it but its incredibly invasive and dangerous and she would have to have a trach for a while. We would really like to avoid that...


Allergies:Lily has many many allergies. Allergies go hand in hand with EoE and also because she is missing The IgM immunoglobulin. IgM is what deals with allergies and allergic reactions. We know she is allergic to many many foods, we just dont know how many or which ones yet because she is on such a limited diet and it is not safe for her to try foods because of her swallowing issues.  We do know she is allergic to milk and soy. She is allergic to most adhesives especially bandaids, and most medical tapes. We know she is NOT allergic to Beech Nut rice cereal and Sweet potatoes.  She has a bad reaction to any other brand of rice cereal like Gerber for example. Only time will tell what she can have and which allergies (if any) she will grow out of.

I think that about sums up her GI diagnosis'. Sorry this post was pretty bland. I wanted to get the technical descriptions outta the way. She has a few more non-GI diagnosis I will go into on another day.