MPS AWARENESS DAY

We are wearing Purple to raise awareness for MPS and all the different MPS diseases!

The boys have MPS II also known as (Hunter syndrome)

MPS II is one of the mucopolysaccharide diseases and is also known as Hunter syndrome. It takes its name from Charles Hunter, the professor of medicine in Manitoba, Canada, who first described two brothers with the disease in 1917. MPS II has a wide range of symptoms that vary in severity.There is no cure for MPS diseases, but there are ways of managing and treating the problems they cause, including enzyme replacement therapies.What causes the disease?Mucopolysaccharides are long chains of sugar molecule used in the building of connective tissues in the body.“saccharide” is a general term for a sugar molecule (think of saccharin)“poly” means many“muco” refers to the thick jelly-like consistency of the moleculesThere is a continuous process in the body of replacing used materials and breaking them down for disposal. Children with MPS II are missing an enzyme called iduronate sulfatase, which is essential in cutting up the mucopolysaccharides called dermatan and heparan sulphate. The incomplete broken down mucopolysaccharides remain stored in cells in the body causing progressive damage. Babies may show little sign of the disease, but as more and more cells become damaged, symptoms start to appear.How common is Hunter Syndrome?This is a rare condition affecting 1 in 100,000 to 1 in 150,000 males. There is an estimate in the United States that 1 in 25,000 births will result in some form of MPS.How is the disease inherited?MPS II occurs in boys, and girls may be carriers of the gene for MPS II. Although rare, MPS II has been diagnosed in girls.All families of children with MPS II should seek further information from their doctor or genetic counselor before planning to have more children.If the mother is a carrier, there is a 50% risk that any boy born will have the disease.The sisters and maternal aunts of a person with MPS II may be carriers of the disease and would also have a 50% chance of passing the syndrome to a son. There are tests available for carriers, so it is important for all female relatives on the mother’s side to seek advice from their doctor.Is there a Cure for MPS II?There is no cure but treatments such as enzyme replacement therapies can help make the disease more manageable. On July 24, 2006, the FDA granted marketing approval for ELAPRASE (idursulfase), the first FDA approved enzyme replacement therapy for the treatment for MPS II, also known as Hunter syndrome. For more information on the treatment, visit http://www.elaprase.com/.

Funds for Medical Bills

We are trying to raise some funds for the many medical bills that have accumulated over the last year and a half that pertain to the boys medical treatment and testing that has needed to be done.
http://www.gofundme.com/2vhdns

Every little bit helps. Thanks so much

May 15th MPS Awareness Day

Please wear purple this Wednesday to help raise awareness for Hunter, Kingston and Nash. All you need to do is tell one person about this rare disease. Please feel free to post a picture on their Facebook page. www.facebook.com/hopefortheclubbsboys

2/3 boys diagnosed between age 2-4 will be SEVERE

MPS 2 has always been labeled mild or severe, & you don't know if your child will be severe until they get there since it is a progressive disease. The latest study is showing that if your child is diagnosed between 2 and 4 that 2/3 boys will be severe!  This is really hitting home with me since they were diagnosed at 1 and 2. My pediatrician at the time picked it out in Kingston at age one.
I want to think the best and hope this disease doesn't attack their brains. 
At least there is Elaprase that helps their bodies. Praying/fingers crossed that the trial drug that goes right into their spinal cord that ENTERS their brain is put on the market soon. Can't bear to see my babies re progress.

This was Hunter as a two year old. You can't leave any of these boys alone even got a minute!

http://instagr.am/p/heAGy/
You just have to love him! 

Trouble

http://instagr.am/p/VodjLQFD8X/
He is the number 1 trouble maker you will know. Believe you me!  :)

Nash

http://instagr.am/p/VoojrJFD1p/
He's getting so big! 

Sweet baby Nash and his mamma!

http://instagr.am/p/VootWZlD12/
2 months old & 13lbs

First treatment=success

Nash's first treatment went very well. He started off a little slower than his brothers since they had to give him benedryl through iv first and then we had to wait half an hour before he could get his elaprase through iv. Other than that he did good. He slept the whole time other than waking up for feedings.
Hunter and Kingston did pretty well other than when they would get bored and thought they had enough of the tv. We some how managed to keep them in their beds, well Jeremy had to lay in bed with Kingston and keep him occupied while I sat beside hunters bed and the crib. Talk about in the way in such a small area. :)
Overall today was a success. No one had reactions and nash got his stitches out afterwards. Hopefully now we can get into a routine of things! 

Waiting for baby brother to get done.

Nash's Birth

December 11th 2012 Nash entered this world at 7lbs 13ozs 20 1/2 inches long. He was born at 37wks gestation. He swallowed fluid at birth and was TTP. He was transfered from Lady of Lourdes hospital to Wilson Memorial hospital the next morning after his birth. He came out of his tacipnic stage around 5 days old. He was off oxygen for two days about to come home and his o2 levels dropped that night after he was circumcised and had to go back on oxygen. He had a hard time coming back off the oxygen. He stayed in the NICU until he was 27 days old. 

The next week I took him with the other boys to their first doctor's appointment with their new geneticist at Albany Medical Dr. Adams. The week after that January 24th Nash had a port placed by Dr. Siskin at Albany Medical while Hunter and Kingston received their first infusions at Albany since their transfer from Upstate University in Syracuse.

Today January 31st Nash is going to be receiving his first enzyme replacement therapy (ERT) at 7 weeks old.

Wish us luck for no reactions and that we are able to control all three kids and that the day goes smoothly.

Ear tubes

Today 12/13/12 Hunter & Kingston are getting ear tubes at Upstate Medical. Im very excited for the possibility of better hearing and lesser ear infections.  :) pray for my strong boys, I know they will do great.

Nash Easton Cruze Clubbs

Nash Easton Cruze Clubbs made his appearance on December 11th 2012 at 3:08pm, 37 weeks gestation. He was 7lbs 13ozs & 20 1/2 inches long. 
I got to hold him for the first couple hours after birth. After that he was taken to the special care nursery to be put on oxygen for the night. We were told he had TTN of a newborn. His oxygen levels were up and down throughout the night. By morning he was needing more oxygen than when he came into the nursery. The doctor decided that it would be best to send him to the NICU over at Wilson since he was having a hard time under the hood. So yesterday 12/12 at 1pm he was admitted to the NICU. At 4pm he was down to 32% oxygen and not grunting as much. Praying that when I go and see him this morning that he has been weened off more oxygen and that he will be able to come home soon. 
Doctors are concerned because of the Hunters Syndrome that his body will take a little longer to heal than normal.  Im really hoping this is not the case. But we just have to wait and see.

MPS Conference Boston Plaza Hotel July 2012

The Transfer Process

        We have been in the process of transferring from Upstate Medical in Syracuse for over 4 months. It has been an interesting 4 months say the least. Our geneticists at Syracuse doesn't want us to transfer so she has been putting her foot in the door all along the way and making it as difficult as possible. She has gone as far as calling up the boys' other doctors and lying to them, and telling them that this was to much of a difficult treatment that they(the doctor) would not be able to handle it. We haven't gotten discouraged yet and we've been fighting for those boys. A 20 minute trip to the hospital will be so much nicer than the hour and a half trip we have been taking since April.
       We have had the hospital here at Lourdes trained in every which way possible. Their pediatrician does not seemed worried at all, and he also hasn't let the geneticist discourage him either. The pediatrician is coming out to watch the boys infusion on Thursday in Syracuse, hopefully our last infusion there. And we will know more after Thursday when we will be infusing at Lourdes Hospital. Hoping the next Thursday Nov 1st we will have everything set up. We then plan to do 2 or 3 infusions at Lourdes and then get everything set up for home infusions, so we will no longer have to travel for the two boys.
       Once the baby is here I will be taking him to Albany Medical which is 2 hours away for treatments. Which won't be as bad with only one child. That is where we have now found our new geneticist who seems to want the best for the boys and has already done more for this baby then Dr.Pellegrino ever did for Hunter and Kingston. So I am excited for this new step. Hopefully next year won't be as stressful for us since we will actually have a doctor on our side. :)
        We will be meeting with him November 2nd, after that we will have more answers about the baby and when infusions will be able to take place. So please keep us in thought for the next couple weeks we have A LOT of big decisions coming up!

We Will Fight Till The End... for all our boys!

I know there has been many questions regarding Clubbs Baby Boy # 3, so I'm hoping this post will inform everyone that has been wondering. We found out in May that we were expecting again, of course we had the scare that this baby would have the disease. So we did the early CVS testing at 10 weeks. It came back that he was a boy. A boy has a 50% chance that he would have the disease. A girl would n...

ot of had the disease, but a 50% chance of being a carrier of it. From there they sent out his DNA to California to a genetics lab where they tested for the X Linked mutation in his chromosomes.
At 14 weeks of pregnancy we got the phone call that this baby also had MPS 2. From there we had to decide what we were going to do. It broke my heart knowing that I was AGAIN going to have another boy with this life threatening disease but with all the fear and sadness I could never abort my unborn baby.
I would not trade my two boys for anything... YES I would ask for this disease to be gone but other than that I would not change anything about them. They are such a joy in all our lives. I could not imagine taking a life so pure that is going to be just like them.
Atleast now we know ahead of time and are prepared. And this baby will be able to get treatment much sooner, so the affects of the disease won't be as harsh on his body. So that is a big plus!

Ports aren't as easy as they seem!!

This past week has been a LONG LONG WEEK! Thursday July 19 was like any other Thursday. It was the 2nd week that my boys had their ports placed & 2nd infusion. They were still pretty pink because their bodies were not used to them yet and were not fully healed.
We started the morning by putting Emla cream on them and putting their bandages over them and heading to the hospital for their infusions. When we got their they took of their bandages to access their ports and by that time Kingston's port area was very bright red and tender to touch. The port was still accessed and his vitals were good for the next 4 hours.
After the infusion his port was still a bright red color. We left the hospital and about 45 minutes down the road Kingston woke up pulling at his ears, acting uncomfortable. By the time we got home and I got him out of his carseat and realized he was burning up. I got him in the house and took his temperature, it was reading 103.3 under the arm. I got my mom on the phone calling the doctors office as I headed out the door. By the time I got to the pediatricians I had got a text telling me they would see us right away.
We went right in and they took his temperature rectally and it was reading 105.3!! Wowza poor boy just wanted to be held by mamma and didn't want anyone else touching him. I had gave him Tylenol before we left the house and it was still rising.
The doctors office set us up at Wilson Hospital where we headed over there right away. All the doctors were scared that it was a line infection and would have to come right out...
That was just the start to our week long trip in the hospital(s)!!

FIRST Infusion with Ports!

Today is the first time in 4 weeks that the boys got their medicine. They were sick the first week, then vacation the 2nd, and last week they had their ports placed in.
I am so glad that we went the route we did with the ports! Today was such a breeze compared to the last 3 months.
We got there at 8:30 & they were hooked up to their medicine by 9:20! It was such a painless and more enjoyable process than getting ivs!
Everyone in the room had to put masks on, except the boys since they decided they weren't having anything to do with it. She then had to scrub them down and disinfect the area around their ports(under the left arm). She then put a needle that looked like a push pin into the port, which did not hurt since we put on the numbing cream before we left the house. She made sure it was accessed, which it was. Put tape on it and they were good to go. It maybe took 2-3 minutes to do all of that.
The boys were a little off edge because it was something new to them, but I'm sure next week they will realize it wasn't that bad and settle down some more. The hardest part for them was taking off the tape at the end when they were all done. And if that's the most pain they have to endure through this whole process, I think we can all handle that!! :)
Other than that treatment went very. The one nurse today told me that I have two very strong and healthy looking boys, which I am very proud of under the circumstances.

I still have boys on the bioplasma cell salts, and I see a HUGE difference when they take them. With all the traveling we did in the last month the boys didn't get their bioplasma cell salts & boy do I regret it! I saw a lot of storage in their faces. They got sick, which they haven't been since the winter time. I knew I needed to get them back on them religiously like they previously were. So I set my phone for three times a day to have the medicine and within a week their faced were starting to look better. I had family commenting on how good they were looking. Within two weeks of being on the cell salts they are having solid bowel movements, which I've noted in other posts how they've never had them till this medicine.
And now I know it's the cell salts because they haven't had their ELAPRASE, their therapeutic enzyme replacement since I've started this up again.
I always have the nurses asking about it or what I'm doing because they are looking so great! I'm really just glad that I found something that I KNOW HELPS THEM!! :)

Road Trip to Akron Ohio to see Dr. Hertle

Today July 3rd both of the boys had an eye appointment with Dr. Hertle. It has been 8 months since Hunter had eye surgery to correct his nystagmus also known as "dancing eyes" and to adjust his optic nerve to give him a more broader spectrum and to correct his head tilt.
Hunter has not been in glasses since January of 2012 when Dr. Hertle took him out of them because Hunter kept taking them off and they really seemed to be bugging him.

Before surgery Hunter was pretty much blind in his right eye and slightly blurred in his left. Specialists in Nashville TN and Binghamton NY both said they're was nothing that could be done. We thought else wise, and luckily we did because we found Dr. Hertle that is the nations top specialist in pediatric nystagmus and other pediatric eye diseases that require surgery to correct. He is actually the only doctor in the US that will perform this surgery for children. Luckily he is training a team so others will be able to perform this surgery in the future!

The boys had a few test done on them. They did not find any swelling or buildup behind the eyes. Hunters nystagmus wasn't cured from surgery just corrected. It's not noticeable by most people and only happens rarely. His vision has been corrected to the point where he does not need to wear glasses at this point in time. His right eye is now a +1.50 now, which is very good compared to the before. He should only need glasses come the school years where he will need them for reading. Other than than that it dies not in-pare his everyday activities. He does have an astigmatism in one eye that is not affecting his vision at all which may be corrected later with the glasses.
Kingston's cornea and optic nerve was very clear. His vision was excellent as well. He does have two astigmatisms that will be corrected at school age with glasses as well.

I'm very happy with my bits results and glad that their vision will not be holding them back in any way! :)