QUARANTINE UPDATE

Our family went into Quarantine on March 13th. The only person that has set foot in a grocery store or left for work would be Mark. I have done a few Walgreens drive through and today we did our first Kroger pick-up.

Broc has been struggling so much with low blood pressures, dehydration , sore throats, chest pain and lots of belly pain. His team have been amazing at helping us navigate things so that he can stay home. Unfortunately due to COVID 19 we are not able to go to Vanderbilt in July for our visit. It will now be pushed till later in the year or even next year. The team is sending out blood kits so that all the labs can be drawn at the home and they will start that process in the meantime.

His Immunologist was concerned about Broc having episodes all the time and how its making him feeling so we contacted the UDN team and spoke to them about letting Broc start his IVIG therapy in the mean time as we are still not sure how long it will be before we get to see them. The labs that they need will not be effected by the IVIG. Broc started IVIG therapy in the home in May.

We prep his sites with numbing cream.

I would draw up all the medication and fill the syringe. We also use some oils to calm him as the stress and anxiety of the needles is not fun for him. He also gets some premeds to counter act the some of the side effects he gets.

The infusion itself last about an hour. I check all his vitals every 15 minutes to make sure he's doing ok during the infusion.

Once we are done we take the needles out and cover with gauze and tape.

  

These are the bumps that are left behind as all the medicine is absorbed into his skin. Sometimes the bumps are smaller it just depends how the infusion goes and how his body decides to react that day. This way of infusing him is much nicer than being in the hospital all day. He typically chooses a movie and we all snuggle on the couch to watch a movie.We do his infusions once a week.

We have not seen any improvement in his episodes yet but it typically takes 6-8 months before we see any change. One nice thing about being in quarantine is that Broc hasn't had any hospital admits for viruses or flu. We are hoping that his IVIG will give his body the extra support it needs all around. IN the past the IVIG helped with his episodes, it made them easier to handle with not so many admits. It also helped with some of his lab numbers as well as helped manage his Tourettes Syndrome. 

  

This is one of those moments where you just have to capture it. Unfortunately it did make him ill and he paid for it dearly but he so enjoyed it. We have been loving the warmer weather. Broc not so much as its makes it difficult for him to breathe and control his temperature. The cooling towel does help a lot.

When it's just too hot outside there are always card games to play. Broc's favorite at the moment is Go Fish!

We also got some more caterpillars and watched them hatch into beautiful Painted Lady butterflies.

Unfortunately my buddy that was living in the pot plant did not make it but I think we gave it a few extra days in the garden. He eventually lost his whole wing on the one side and was unable to fly.

Broc was in some desperate need of some iron but his team was not comfortable sending him to the hospital for his infusion so they organised it as an in home infusion. It went very smoothly and we hope this will be something that we can continue to do at home. We do miss our nurses dearly but the less we expose Broc to the hospital the better. 

Broc did unfortunately have an ER visit for some severe pain that we couldn't control at home. His team felt that he needed to be evaluated. We discovered that Broc had an ilues with a viral infection along with a possible kidney stone. On further evaluation by his Riley urologist they are not sure that it was a stone that was seen so Broc will be going back to the hospital today for an abdominal, kidney and bladder ultrasound to rule out a stone or something else. He has still been in a fair amount of pain on and off. He is also having some pains in his lower rib area on the sides and towards his back. We are hoping to get some answers from all the imaging we are doing today. When we have an update on that I will be sure to post it as I know you all care about Broc as much as we do.

Now onto his legs. His poor legs. As many of you know his legs story started after his Open Heart Surgery in 2010. Since then Broc has had 2 vascular surgeries to give his right leg as much blood flow as it can to grow correctly. Unfortunately all those options have come to an end. His repair is looking great and his right leg has the same amount of blood flow as the left one does. When we went for his follow up last Monday his vascular team did all the tests they could to rule out any other options that could help his legs. Unfortunately his right leg suffered growth when it didn't have the correct blood flow. We were hoping that by doing the surgeries it would catch up. When we went for his appointment in December is leg length discrepancy was 1.2cm. When we measured him last week he has progressed to 1.7cm. He has grown 5mm in 6 months and continues to grow. Once the discrepancy reaches 2cm or more he will start having many back and hip problems. This Thursday we will be seeing an Orthopedic Surgeon at C.S.Mott for them to investigate and look at all the options we have. We have been told of 2 but hoping there maybe more. One is to cut the bones and lengthen the right leg with surgery and the other is to stunt the left leg by doing something to the growth plates. Either way neither of them sound like anything we would want for Broc to go through. We know we need to intervene. We, together with his team will make the best decision we can for Broc. This news has been very difficult for us as parents as we really thought his leg journey was over. Seeing Broc continually endure surgery after surgery no matter what part of his body it is is really difficult for us all. Especially the emotional toll it takes on everyone. The older Broc gets obviously he is more aware of the implications and the limitations he will have. Although we do not know what the treatment will be he has already started asking all the questions. Every year we hope that it will be a surgery free year but it doesn't seem that way.

Please keep us and his teams in your prayers as we try and make the best decisions we can for Broc. With him expected to be 6ft or taller we need to give him the best outcome possible.

We will continue to isolate and quarantine as long as we feel the need. Please respect our decisions.

Thank you for your continued love and support for our family.

🐸🐸🐸🐸    

A busy week

On Monday Broc and I spent 4 hours yes you read that right 4 hours at Dr Harris's office. we spoke about everything that Broc has been through since we saw him last which was the end of 2012. We also went through his diary and all the viruses and fevers that he has had too and the impact that these have on him. Not only does he deal with the fevers with these viruses but he also deals with gut shutdowns, crazy sensory issues which impact his schooling,his balance is also thrown off too which causes him to fall and bump into things.

Dr Harris decided to draw all his immune labs again and some others. We are hoping that one of these labs come back low so that insurance will finally help cover the treatment that we have been trying to get for him.It is really sad to hope that your child has a disease or something wrong in order to get the help you need. In 2011 we tried to get him a trial of treatment but ins denied coverage. One treatment alone will cost about $1000.00 that excludes all the monitoring that Broc will need during the treatment. He will have to be monitored in the hospital in case he were to have any reactions to the treatment. We don't know for sure that it will work, but if we don't try it we will never know.

He also has some other "out of the box" treatments in mind too. Some of the labs are not back yet so hopefully we should hear something next week.He also checked for Broc for any food allergies just to rule them out. It was not a very comfortable test but he did really well and tested neg for all of the food allergies.

On Wed Broc and I took a trip down to Riley. It was a very early morning. We left at 3.30 but that meant that we were home early enough to get Mark from school and he wouldn't have to ride the bus home. He really doesn't like it but will do it on days that I cannot get home early enough to get him. We had our lovely tech Nancy do Brocs echo. Mark and I will never forget her. She was the first person to take the time and explain everything on the monitor to us. She explained that if we see lots of colors like on a storm weather doppler then its bad. That was way back when Broc was only 8 weeks old and he was just dxed. That is a day that Mark and I will never forget. She was so happy to see Broc and how well he "looks". She did a fantastic job with his echo and got all the pics that she needed. She was also very interested in all that Broc has been through since we last saw her which was in 2010 when Broc has his OHS for his Aortic Valve repair.


Before her we went and had an EKG done too. Broc was able to pick out 2 prizes from the treasure box. He always picks one for his Buddy so he doesn't feel left out. I love the way they look out for each other. Then off to see Dr Hoyer. We caught him up to speed with everything that Broc has been through and dealt with. Also all the different surgeries and procedures that he has been through. We also discussed how we saw cardiology at Cincy and the not so great experience we had. He was great about that and said lets just forget you ever went there. Totally agree.

Onto Brocs heart. He feels that Dr Capannari is doing a great job of taking care of Brocs heart. He said that everything Dr C saw was what he saw on the echo. He did put Broc on a low dose heart med to help his heart along and prolong the time before his next OHS to replace his Aortic Valve. He said we could be looking at the next 3-4 years maybe a year or too longer if the meds help.
Initially we were hoping that Broc would be in his teens around 15-18yrs to have this surgery but its not looking that way.
We discussed all the different options that we have. Bovine,cadava,mechanical valves and also discussed the ROSS procedure as an option too. With all the rejection issues Broc has been dealing with from his bypass and now brain surgery this could be very tricky. It is imperative for us to find the source of the rejection and see if there is anything we can do to prevent it the next time. Rejecting a heart valve would not make for a very happy heart and a very sick little boy.
He also felt it best that since Broc is dealing with many other medical issues too he should see him too. So next year we will see Dr Capannari and then the following year we will see Dr Hoyer.
He did say the regurgitation is moderate to severe as well as some back flow over the aortic arch.

So now we have the rejection issue of his brain sorted for now as well as the cardiac clearance. I called CVC yesterday and they should be receiving all of Brocs heart info so that they can start scheduling his CTA.

For now he has had 2 weeks of low eos levels so we are thinking that we are done with this chapter for now. Broc has not been cleared for gym,recess etc yet. He will be cleared for school this afternoon. We have a very happy little boy who cannot wait to go back to school on Monday and one very happy Big brother who cannot wait to ride the bus with his brother again. Next week will be a short week for Broc as he will have school Monday and Tuesday and then Wed he has another app at Riley to see another immunologist.(Dr Boaz is hoping that he will be able to figure out the eos number issue.) Then Thurs and Fri are Fall Break. I think it will be a great way for him to start.

Thank you everyone for all your love and support
  

Cardiology and Immunology

I am sure you are all anxious for an update on how the past couple days have been so here it is.

We saw cardiology yesterday for Brocs anual checkup.We had it bumped up a little as he was showing some signs of fatigue and not been able to walk short distances without saying something.

Over all Dr C said that everything looked great.His numbers had not changed from last year June but he did say that his valve leakage from his Aortic Valve is now in the moderate range so it has got a little worse.He told us the serious signs to look for but hopes that we will catch it on echo before he would start to show the signs.

Eventually  we know that Broc will need his Aortic Valve repaired again or replaced. He does feel that his low endurance level is coming from his leg causing all the issues with not enough blood flow and that is causing him to be more fatigued. So we are hoping that once he has his ilio-femoral bypass surgery in June he should have more endurance and be able to run and play like his brother.We have been told that he will still have times that he will be tired and then we should let him rest. Dr C said that Broc knows his body very well and will be able to tell us when he needs to rest.

Broc is feeling much better and his fever has seemed to be gone.He is getting his energy back and went to school today.He wanted to go before Spring Break.

Now onto Immunology.At the beginning of the appointment we thought it was serious de ja vue as Dr N was saying the same old virus thing and that he will be fine. He then left the room to call Brocs ped and came back a changed man.I really believe that there was someone else helping us too as he knows the struggles we go through.

Dr N came back and went through all Brocs labs that he had done.He said that the EBV is no longer in his system which is great news.He also said that his immune antibody response is very good.That is the thing that puzzles him and many other Drs too.he has great repsonse but still gets sick all the time.He said that he has never seen anyone with such a high immune response but continues to get fevers with infections all the time. His is going to repeat his CBC next week to make sure that all his numbers have recovered.If they have not recovered then we will be looking at doing a bone marrow biopsy to see why they have not.So we are really hoping that his system bounce back to normal.

He also has never had a childs WBC and nuetrophil count drop that low everytime they have an infection.
We are not doing anymore antibiotics for Broc unless an actual bacteria is cultured as his risk of getting C-Diff again is extremely high and he is till suffering from the effects.It has given him irritable bowel syndrome which is causing him lots of pain when he eats and making him feel sick to his stomach a lot.He is taking pro-biotics for it.They said that his tummy will have to repare it self.

His genticist mentioned last week that there is a possibility of Broc having a mitochondrial disorder.Dr N is very interested in that and will be talking to Dr W the metabolic specialist to see if they can evaluate Broc.We also mentioned the MRI that he had done a few weeks ago and then he took off out of the room.He came back and said that the nuerologist is out of town and will be calling us when she gets back.He thinks that she will be a good Dr on Brocs care team as well.

BTW Dr S the nuerologist called me and wants to see us in 2 weeks.So we have another trip to make.

Dr N wants to follow Brocs fevers to see if there is a pattern to them so we are to take his temp in the morning and then again at 4pm in the afternoon.

The goodnews is that he will not need to have to wear a mask again unless we are in extremely crowded places such as assemeblies and conventions and maybe airtports too.We are so happy that everyone gets to see his precious face again.The mask was really not protecting him as he is still getting ill but in those places he said it will aid as a small protection.

So Broc is going to have a Care Team taking care of him which will consist of a Neurologist, Infectious Disease and Metabolic Specialist,Immunologist,Hematologist, and Geneticist.

I will have more updates once we see the Neurologist in 2 weeks.Till then thanks for supporting us n trying to help our little Buddy.

Another virus......

So Broc has not really had a break with being ill.Last week he had a small tummy bug ,then last Monday he had strep and now this Monday wakes with a fever of close to 104F.This is crazy.He was so lethargic so we took him to the ped and then for blood work.His WBC was low and so were a few other numbers.Fortunately his ANC level was higher than 1000.

Fast forward to today his fever is still up and down with Mortin.He is still lying around and not eating much.I took him for another blood draw this evening to see how his counts are doing.OH MY WORD.....
His WBC is lower and so is his RBC,Hgb,Hct. Now his ANC level is 490 something.

I called the ped and he said this is serious again.NO kidding. He thinks he may have the Parvo virus B19 again??? I really hope Dr Nelson has some good answers tom otherwise some sparks are seriously going to fly.

Our Buddy seriously needs a break.He has missed almost a whole month of pre-school if you add up all the days he has missed.He only started in the middle of December. He needs to be heatlhy with a good blood count for his major vascular surgery coming up in June.

As parents , we are seriously frustrated that Broc has to go through all these illnesses,blood draws,hospital stays,time away from his brother etc.That is not the life of a 4 year old.

We had never heard of Pravo virus until he tested positive for it last year and now they think he has it again.It is truly crazy , madeness.

I will update tom once we see Dr Nelson. We pray that he has some answers or solutions for us.If not we are thinking of taking Broc out of State to find help for him.


We thank you for all your prayers and words of comfort.