Also New to the Group

Hi, my name is ashley, i am a stay at home mother of 3. 7,21m,and 6m. my 21month old has digeorge syndrome. we found out when he was13 months old. here is his story.
thomas michael was born in kansas city mo on 12/22/05 naturally. and appeared to be heathly. i was a first time breast feeding mom. like most new borns (and it had been like 5years since i had one) he ate often and slept alot. he was gaining just slowly. at his 5wk check up our nurse heard the mumor that was on a friday. because of his slow gaining she asked me to switch to formula so we could measure his intake and sent me to see the cardioligist. on monday he saw the heart doctor and he had a vsd. by that friday he quit eating. we spentall in the er at st.joe and they said he was in mild heart failure. we went home early in the morning and by 10 he threw up, i called the doctor and said to check us in to childrens mercy i was staying until he was fixed. our first stay was over a month. they put a tube in his nose to feed him and on his 3m birthday they did his heart surgery, vsd,asd,and pda. all seem to be fine now. we went home a couple weeks later, but he still would not eat. we were hoptialized two more times for illness and failure to thrive. in july that year about 6m old he got his g-tube. then he finally started to gain. that september he came down with a cough that lasted for months. in december our ent did a broncosopy and found he had horrible broncitis. in febuary they did the second broncosopy and found he has a type for cleft palate. looking at him he looks fine. but he has speech delays he only says mama,dada and nothing often. his only normal sounds are grunting and growling. though i must rejoice that all the signing is paying off, he started to catch on about a week ago. we still doesnt really eat though. he seems to go through spurts were he will eat and then he just stops. he has to have every thing thick and they have him on a high calorie diet but for the last three months he has gained nothing. he is 32 in tall and only weighs 24 pounds. they cannot seem to figure out why. the stress as you all know is at times overwhelming. he has had over 10 hospital stays and the last half of those i was ready to give birth to our new daughter. we worry about him all the time and we worry about the future since i know i journey has just began and there is so much more that could change or happen. i just want help and an ear. i want to know i am not the only one.
ashleypagekcmo

Hi Ashley. I’m Ronda, my granddaughter was born one year ago with digeorge. She had tetrology of fallot and had two heart surgeries to correct it. Fortunately her heart is great now. She has developmental delays. She does grunt and growl a lot.I was wondering if speech would be a problem for her. I am reading more and more of there being speech delays and speech therapy in these kids with digeorge. I am also wondering about her palate. In your case there was a problem that was not noticed or visible. That is my concern for her. You can’t see a problem. So how was your daughters diagnosed? By the way your daughter and my granddaughter share the same birth month. Her birthday is Dec.2nd. You really have your hands full with 3 little ones and one with health problems. Any info.that you have or resources, books etc. that you know of would be appreciated. It is so hard to find info on this. I keep coming up with the same stuff.

Hi Ashley, my son is 7 months old and he has DGS and TOF at 3 months he had the repair I’m not really sure if my son has fullblown or not but so far he has been ok on develpment but slowly starting to get behind. He has his thymus and his plalet and everything is ok. After reading alot of stuff on here alot of kids with DGS have alot of speech problems. My son growls and grunks alots also I just wonder if that’s something that all DGS kids do. I’m scared because this is my first child and I really don’t know what’s going to be down the road for both of us. AS of right now we don’t know if it came from me or my hubby. This is the first in either of our familys that we know of.

Hi Ashley,

You are not alone. We have three boys 5, 3, and 4 months.

Our son Caden (the 3 year old) was diagnosed with DiGeorge during my wife’s pregnancy with him. He has had a repair of Interrupted Arortic Arch, VSD, ASD, and has a pacemaker. He had a Nissen Fundalplication for massive nasal reflux at 8 months and has had numerous other procedures to work on swallowing and speech issues. The most recent surgery was a sphincter plasty. Our Caden is still 100% tube fed via G-tube even for hydration. He is not even able to sallow his own saliva. We carry a feeding pump and suction machine everywhere we go to help Caden clear secretions to prevent aspiration. Oh…He also has been diagnosed with scoliosis. (40 degree curvature of the spine) He sees speech, physical, and occupational therapy weekly.

On grunting. Caden does make some weird non verbal sounds that he seems to entertain himself with. He is much like your little guy in that Caden only says mama, nana, and little else clearly. He does know and use over a hundred signs. However, it has been difficult to teach him how to initiate a conversation with signs to let us know what he wants. Caden resorts to whining and crying when he needs to get our attention from another room, like at night when he get really congested and needs suctioning.

Once again, you are not alone. I started blogging the day that Caden was born. Since we are 150 miles from our nearest relative, the site was a way to keep people posted on Caden’s progress. It turned into kind of a therapy tool for me. Now the site serves as a place for parents to connect with us after searching on issues related to 22q11.2.

http://www.hoei.com/caden/blog

evensmom,

You said, "AS of right now we don’t know if it came from me or my hubby. "

Did you know that most cases of DiGeorge don’t come from the parents? Only about 10% to 15% of the 22q11.2/DiGeorge cases are found to originate from a parent. My wife and I were tested to help us understand the risks before having more children. Neither of us were 22q11.2. There are supposedly higher chances of a repeat with other children even if you or your husband do not have 22q. We were told that the chances double. So instead of the estimated 1 in 4000 occurrences of 2q11.2 you would have 2 in 4000 chances of having the next child with DiGeorge/VFCS/22q11.2.

A great resource for learning more is at http://www.vcfsef.org/

Hi Ashley,

Sierra, my 5 1/2 yr old daughter with DiGeorge Syndrome, currently uses Sign to talk she is able to mama, danny (which is daddy), more, and a few other words that only i am able to understand. She was just diagnosed with developmental dyspraxia with language delays. She know sign (her two brothers ages 9 and 7 also learn the sign along with her father and I) but there are times that both her and I get frustrated because we can’t tell what it is that she wants as she doesn’t know the sign for it. She has just completed an evaluation for a speech device ( it has pictures that she points to that speeks the word that she is trying to say), we were told that she she is a prime candidate for one as the evaluation showed that she was able to use one to communicate what it was they were trying to get her to say.

We are waiting to see if her insurance will pay for the sound device for her. Even though she is really good with sign, there are very few people in our community who know sign (and that is including her speacial education teachers). She currently attends a school for children with disabilities. It’s hard seeing her with other children trying to talk and play with them and having none of them know what it is she is trying to say. Hopefully this speech device will help bridge the communication gap so that she can finally let people know what it is she is trying to say.

I don’t know what program, if any, anyone uses to learn sign language, but I can you that we have been using “Signing Time” to learn sign language. Sierra connests with it so well and she catches on quite fast with it. You can go to signingtime.com and they also have clips of some of the videos and signs that are on there. When my daughter had first seen it online, I knew instantly that was what we needed to do with her as she started picking up signs from it right away.

I hope this helps,
Bonnie

Hello
My son is now 7 mths old. I had a normal pregnancy and all was well. In the delivery room, I noticed he had a very odd squeaky high pitched cry. His colour was not good and had trouble breathing but he was fine after alot of suctioning and some oxygen.
While in hospital he was transferred for observation to the NICU for blue spells when he got upset, they looked exactly like he was holding his breath in a temper tantrum and turning red, purple, blue then black. Instantly resolving once he stopped being annoyed. Everything looked fine. He did have an echo done of his heart and it showed a very small VSD.
The VSD closed on it’s own by the next follow up echo in 2 mths.

We went home, he was doing well, but I always felt a little ‘unsettled’ about him. I’m a pediatric ICU RN , so that cry and his ‘breath holding’ made me nervous. When he would cry, you can’t really hear him, until he really got going and then he sounded like a tea kettle. A few times he would have formula coming leaking out of his nose with bad crying.
At 5 wks he started with some mild stridor and noisy breathing, sounded like mild laryngomalacia. Ok, he’ll grow out of that. At 6 wks he got thrush and it took 2 mths to treat, nystatin didn’t do anything, gentian violet didn’t either, it took a month of fluconazole .
The breath holding blue spells resolved on it’s own at about a month old.

At his 2 mth appmt I mentioned his odd cry and the laryngomalacia and my ped referred me to ENT to take a look. There they were surprised to find a laryngeal web ( tissue between his vocal cords, which narrows his airway, it also causes the high pitched cry ) They said it’s pretty rare. They said that because there was a study that came out linking webs to Digeorge that they should do a FISH test, but they didn’t think he had it. Everyone was in shock when it came back positive. I was in shock and heartbroken. I was alone when they told me at a follow up ENT appmt , and I remember crying all the way home.

He is now 7 mths and he is doing well. He has been seen by the genetics , immunology , clinic at the ped hospital here ( where I work as well !) , his development is on track, he is rolling over, sitting up though not really great yet, he still leans forward a bit and needs steadying… falls over if he turns to look at something etc, but is improving every day. His tone they said is a little low but still pretty normal. One thing I’ve noticed is that he doesn’t ‘kick’ up , when his feet are put down on a flat surface. His legs are strong and will play and kick lying down, but seems to just sink down when put in a ‘standing’ position. They said some kids are just like that. I have noticed it’s getting better esp since he’s been playing in the exersaucer.

His PTH and calcium seem fine, though they said to monitor that with other bloodwork yearly. His thymus function they said is typical of a 22q11. His CD8 lymphocytes are on the low side, with 2/3 function , he is not to have live vaccines and needs to be ZVIG’d if exposed to chickenpox for now. But they said usually most of these kids will have normal immune function as their thymus matures within 2-3 yrs.

He is a bright and happy little guy. He has no facial features, his VSD is already closed, he is due to have a kidney u/s soon though his function seems to be fine, he is making alot of noises but cooing and laughing mostly. I don’t know if that is mostly due to his web that he can only make the higher notes. I have him on the list for speech therapy already as there is a bit of a wait. His web they wanted to wait until he was over a year to laser it, but because he had a serious incident a few weeks ago where he couldn’t breathe ( he woke up in the middle of the night with an acute attack of severe resp distress, couldn’t breathe in, they dont’ know if it was a laryngospasm , or he had some mucous blocking it, or some reflux? ) they are doing it sooner, in August right before he is 10 mths old.

I know how overwhelming it is. So much to wonder about. I worry everyday about what the future holds for him. There can be such variance in this disorder from one individual to the next and the internet can be a scary place. Sometimes I wish we never did that test, no one would have known , but now that I do, I know that I have to try to give him the best opportunities to reach his full potential and I am grateful. It’s a process to come to terms with it yourself I think.

Hugs to all of you dealing with this and best wishes for all of you.

My husband and I haven’t been tested and are weighing the pros and cons, we weren’t planning more children , Jonah was my 2nd son, that’s what we wanted 2 boys. Any thoughts on it? Are there any drawbacks? Insurance? etc?

For anyone living in Utah. We have a support group for 22nd chromosome syndromes. It’s very broad as of right now nut we would like to be a resource to families. Please feel free to pass this information along. To contact us send an e-mail to q22.deletion@yahoo.com .

Cassaundra