Guess what Caleb got today?!?!?!?

Caleb got his hearing aid today, shown in the picture!!! He seems to be doing really well with it...not pulling at it or anything. We believe it has helped him hear better, of course, we will know more as he develops. It seems he is turning towards us know when he hears us rather than us moving into his line of sight...so we are all very hopeful!!! His hearing aid is on his right ear and he will get one for his left ear when he is a little bit more mobile.

Also, Caleb has been doing very well in his Bumbo chair as he has been slightly delayed due to being in the hospital so much. Both his Physical Therapist and Occupational Therapist were here today and were very happy with Caleb's progress. He is really a tough kiddo!!! He amazes us so much!!!!

Oh and also...Caleb got to wear clothes today!!!! How exciting. We have found out that if he wears outfits that snap or button up the front he can wear them and we can run his broviac line (IV line) out in between the snaps. He is looking pretty snappy if I do say so myself.

Update on how the Cyclosporine is working

Hi everyone, just wanted to touch base and let everyone know how things were going. Caleb was started on the Cyclosporine last Thursday. How this medicine works......what t-cells Caleb had were only a few of a couple types instead of a lot of a lot of types. Unfortunately, from many biopsies and blood tests we have found out what t-cells Caleb did have were attacking his gut and skin. Therefore, this immunosupressent that was started was hoped to relieve his skin, helping with the breakdown and itching issues, and to help his gut (hopefully stop the diarrhea). Well so far so good. Caleb's skin looks UNBELIEVABLY BETTER AND he has only had 1-2 bowel movements in the last three days as compared to 8 to 9 A DAY before the cyclosporine. Along with that some of his lymph nodes that have been swollen have gone done greatly in size. We are hoping this isn't just a coincidence. There are some side effects to Cyclosporine: high blood pressure (Caleb is already showing signs of this so he is on Atenalol to help that), kidney issues if the therapeutic levels of cyclosporine go to high, higher risk of infection as all of his immune system is now completely wiped out - however he only had a few t-cells functioning, hair growth-he will begin to grow hair so get ready for future pics.

He has developed an eye infection so we are treating that with eye drops. We hope he clears that infection with no problems.

In addition, through a friend in Wheeling we have met another family that has a child with CHARGE syndrome, as she refers to it another CHARGEr. She and her son have been so informative for Brian, Caleb, and I it has been wonderful. In addition, we have met other families with cute little CHARGErs and we are blessed to have met them. Thankfully, none of them so far have any of the immune issues that Caleb has. Please keep all of those families in your prayers too. I am hoping to update my sight soon and will hopefully have links to the other cute CHARGErs, of course with their parents' permission, blogs so you can read all about these tough kiddos. Well I will update soon...and hopefully with a little help from my friend Amy I will be able to post some video to this website soon too!!!! WOOHOOO

After second part of Duke Conference Call

Well the second part of the Duke Conference Call happened yesterday. We reviewed the consent papers and understand all the procedures, medicines, etc. that will be used. The papers will actually be signed once Caleb, my mom, and I arrive at Duke.

Unfortunately, Caleb's 4th central line worked it's way out yesterday. Therefore, they put another line, broviac, in lastnight that is a deeper and hopefully a more permanent line. This line allows the tissues to grow around the line to hold the line in better. This is also the line Dr. Markert wanted for when he is sent to Duke. Now, lets just pray that there are no line infections. He was uncomfortable lastnight and very dehydrated because he did not have any IV for several hours before the line placement, as he has no veins left for even a temporary peripheral line. So he had many boluses (larger quick amounts) of fluid lastnight to rehydrate him. In addition, he was given Tylenol and Dilaudid to help with the pain.

This morning he is still thirsty but needs lasix so he does not have fluid overload on his heart. In regards to his heart his hemoglobin levels have to be a little higher than normal to prevent his little heart from working a little too hard. He has already had two transfusions and they are ordering one for today. The good news is that Brian and I are a match to Caleb's blood type so we can donate. However, since he has the immune deficiency there are many viruses that people can carry that pose no risk to people with good immune systems. So the blood he gets is radiated and checked and double checked. There is one virus that 70% of the population carries that cannot be removed by radiation so all donated blood is checked for this virus, CMV. This virus can also pose risk at the success of the transplant so the blood he gets is very thoroughly examined.

These are the only updates I have at this time. As soon as I get more I will post them.

Duke Conference Call

Hello all, well we had our conference call today with the docs from Duke. It went well. They are going to start Caleb on a drug called cyclorsporine to help him with the skin breakdown issues. This drug can be hard on the kidneys if it reaches a certain level so they will be checking that. In addition, this drug makes the individual grow hair. This hair will eventually fall out once Caleb is off of it. We have been given the okay that if he responds to the cyclosporine well that we may be able to try baby food, wooohooo. We have been waiting because he has been having a lot of trouble digesting and absorbing his nutrition. We are hoping that this cyclosporine may help him enough that we can start baby food. We have found out that it will be approximately 5 weeks until Caleb will head to Duke. The thought now is that he will fly down on a jet, with a nurse and myself. Upon arrival Caleb will go through a serious of blood screens for about a month. At the same time, thymus tissue will go through a screening process to make sure it is a fit for him. Once they have the surgery scheduled, Caleb will go to the ICU and will have a drug infusion for 3 days that will completely deplete his body of T cells (the ones that he does have) so that his body does not fight infection. He will go to the ICU for this infusion to keep a close eye on his vitals as sometimes there can be spikes/drops in heart rate and blood pressure. Two days after the infusion Caleb will go to the Operating Room to get the transplant. Thymus tissue will be implanted into both legs in the muscle. They will make one incision on each thigh and place approximately 40 pieces of tissue. He will then be watched close for the two months. He will then have a biopsy to see if the Thymus tissue has begun to educate the T cells. If okay, Caleb could then be sent back to Children's in Pittsburgh to be watched for the next couple of months (great news that we were not expecting----closer to home---lets pray this is what happens for him). Then back at Children's they will watch all of his blood levels to see how the immune system is working. From there it is all up in the air as to when we would be home.

Long term possibilities. . . for the first year Caleb will be in Isolation (very clean home environment-we are writing Extreme Home Makeover to see if we can get help with a filter system, cleaning our basement from the flood to make sure it is safe, and better windows, and anything else they may be able to provide), he will also be on a drug Bactram (which he has already been on) for this time. The Bactram tries to prevent him from getting a pneumonia that can be life threatening to immuno-suppressed patients. They will then try to give Caleb a Tetnis vaccine, if his body responds then that is great news that his immune system is working. He will continue to be on the IVIG (Intravenous Immunoglobins mentioned in a previous blog) for the following 2 to 3 years, depending on how his Immune System is functioning. We do not know the frequency as to how often he will get the IVIG during this span.

We still have to finish our conference call tomorow to sign consent forms. If I have any more updates or pics :) I will post them!!!

Another update-medical diagnosis

Okay all..the last Duke conference call was cancelled because Dr. Markert is a very busy woman. We are on for a conference call tomorrow at 1:00 so hopefully we should know more then.
I also noticed I haven't been clear on some of the things they have found out Caleb has. First, we found out Caleb has a syndrome called CHARGE by genetic tests. According to the Charge Foundation a case of charge is about 1 in every 9-10,000 births. The letters in CHARGE stand for: Coloboma of the eye, Heart defects, Atresia of the choanae, Retardation of growth and/or development, Genital and/or urinary abnormalities, and Ear abnormalities and deafness. Sometime CHARGE effects the child with each of these things are just partially. To find out more about CHARGE please feel free to visit http://www.chargesyndrome.org/about-charge.asp Caleb's anomalies do fall within these categories with CHARGE. He does have colobomas of the eyes, the back of the eye didn't close completely, however, they don't think it is severe enough and at this time they do not think it will effect his vision. He does have heart defects, two ASDs (holes in the heart) which have caused pulmonary hypertension. He does have a slight atresia of the choanae, narrowing of the nasal canal but not what they think is a significant one at this time. He does not at this type pose any retardation of growth or development or genital or urinary abnormalities. He does have ears that are low-set and a significant hearing loss in his left ear but good hearing in his right ear. They think that with the help of hearing aids and the ability to read lips he should be able to communicate normally. The docs said they would like to try hearing aids first to see how they help. It has been explained to us that CHARGE acts in a snowball effect in utero. The one mutated gene effects other genes during development. Therefore, that is why there can be many anomalies. Along with the items described above Caleb does also have three brain anomalies: 1. Hydrocephalus-water on the brain that is being treated with a VP shunt that reroutes the draining of the spinal fluid off of the brain. 2. Kiari Malformation-the cerebellum is smaller than normal and sits low onto the spine. They are hoping that removal of the water of the brain will allow the cerebellum to grow normally and hopefully lift off of the spine. If it continues to sit low on the spine they can try a surgery to try to remove pressure on the spine. 3. Partial Agenesus of the Corpus Callosum-the corpus callosum is the major bridge between the left and right hemispheres of the brain. This is how both sides of the brain communicate with one another. Sometimes this agenesus effects the individual (shaky balance/gate, hand-eye coordination may not be at its maximum, high tolerance to pain, the communication between the left and right sides may not be at its optimum) other times it does not effect them at all. He also has a hypoparathyroid that effects his calcium and phosphorous levels in his body. If the calcium is too low it can pose a risk of seizures. This is being controlled with daily doses of calcium. Cleft Lip and Palate which is being treated with a NAM ( a mouthpiece) that moves the palate and nose to prepare him for surgery. He is doing great with that! He also had sever hip dysplasia of both hips at birth. This was treated with a hip harness for the first 4 months of his life and he is now out of the harness and the hips will be watched closely when he learns to crawl and walk and then will be followed for the rest of his life. All of these issues are being treated by the wonderful doctors at Children's Hospital in Pittsburgh.
Along with the CHARGE Caleb has also been diagnosed with Aytpical DeGeorge Syndrome. He is the sixth case in the world. This Syndrome prevented Caleb from developing a Thymus. The Thymus is the part of the body responsible for creating and educating the cells that are responsible for immune system development. Caleb is now on the list for a Thymus Tissue Transplant which he will receive at Duke Medical Center in Raleigh Durham, NC. This transplant will hopefully allow Caleb's body to have a functioning Immune System. We should be at Duke University for approximately 5-6 months to see how his body reacts to the transplant.
We are hoping all goes well with the transplant and that Caleb will have a full-functioning Immune System as he grows. You can see from a previous post the success rates of Dr. Markert's previous surgeries on Immuno Suppressed babies. To read more about Dr. Markert and her technology please feel free to visit http://www.dukemednews.org/news/article.php?id=7983 to read how the transplant works. Please note Caleb does not have Complete DeGeorge he has Atypical DeGeorge, however, in either case the babies may not have an immune system.
After the transplant, Caleb still needs to have his cleft palate and lip repair and a heart surgery (we are not sure at this time if it will be open heart or a catheter that can be placed through a vein in his groin and up to his heart).

The picture is Caleb in his bumbo chair, he has started to begin to hold his head up, very well I might add. He has been somewhat delayed for head control and sitting and crawling because he has been sick and has had many lines which sometimes prevent us from being able to do certain exercises with him that babies normally can do. He has very dry skin due to the malabsortion of fluids. Until he gets his transplant we have to very careful to treat skin breakdown so he does not get an infection. He is doing great! He is a fighter!

2/8/07

Okay all..first off the older website is on http://caleb-hlebiczki.blogspot.com/. I am hoping to join the two together on this website soon.

Great news!!!! Thanks to everyone's prayers and well-wishes we are going to DUKE!!!! We have a conference call with the docs here and the docs at Duke tomorrow. We should find out more details of what to expect and when we may be going. As soon as I have any more details I will update the blog.

Also, I have pics just need to download them to the computer as soon as I can I will download and put them on the blog.

THANKS EVERYONE FOR THE PRAYERS!!!