Macy

Macy

Sunday, November 20, 2016

Transplant

Hi family and friends. Our "some day" has turned into a "today". Macy's counts have reached critical levels, and her Doctor from Minnesota called today telling us it was time.  Macy will be having her bone marrow transplant at the University of Minnesota Masonic Children's Hospital in December or January.  Until then she is in isolation.

Our minds are spinning and it feels like there are a million things we need to do to prepare right now.  We will be there for approximately 3-4 months. Macy is pretty disappointed that we will need to cancel our Disney trip for December, and that she can no longer go to school or dance :(  Its not safe for her to go anymore so we have no choice.  

Most of you on Facebook have received an "invite" to like Macy's page "Macy Against the Marrow Monsters." I'm still trying to figure out how adding a "page" works so please bear with me.  If you are interested in following Macy's transplant journey please "like" the page to see updates.

I will continue updating on Macy's FB page from now on, instead of here.

Take care,
Jenn

Monday, November 14, 2016

Isolation

Macy did end up having her surgery last Monday. We did bloodwork the Saturday before, and her platelets were 31,000 and ANC was 900.  Even though her platelets dropped, her ANC was the highest it has been in over 6 months, so it was a go!

The surgery itself went well. Macy stayed on the couch at home for about 3 days afterwards before she felt good enough to get up and walk around. We let her try a half day of school last Thursday, and she went all day on Friday. She was getting around better. Macy's FA Doctor from Minnesota wanted us to recheck her blood to make sure her counts were holding after surgery.

We checked it this past Saturday, and were really taken back by the results. Her platelets fell to 12,000 and ANC to 340. Just for some perspective on how bad those numbers are...there are many kids who have their bone marrow transplant before their numbers get that low.

Macy has been at home in isolation since we found out. We were sent to get her bloodwork today, and her ANC is still 340, and her platelets 18,000. We know this drop is from the surgery and are expecting them to increase eventually. I'm not sure if they will go back up to what they were, but should go up. When/if her ANC gets back up to 500 she will be able to return to school. Since we can't be around people we have been taking drives, and playing outside away from public areas.

Adam was able to take the boys up north for a few days so I can focus on Macy. I've been watching her close for fever and bleeding.

Her next check is next Monday. We are hoping for better news then.


Take care
Jenn

Friday, November 4, 2016

Surgery

Last summer (in the last post) we ended up having to cancel Macy's stomach surgery because her ANC was too low.  ANC is the 'absolute neutrophil count' which is a special (and very important) type of white blood cell that fights infection.  The typical persons is over 1500, and when they get sick it goes up higher because their body is fighting the infection.  Macy's was 400 the day before her surgery (and she wasn't sick), so we couldn't risk her getting surgery and getting an infection from it. Anything under 500 isn't really high enough for your body to be able to fight it. That's why when Macy gets sick (those with FA's ANC typically goes down when they get sick) and her ANC is under 500 she has to be hospitalized and put on IV antibiotics. 

Over the summer Macy had a few ENT appointments and we found the tubes she got put in her ears in February are not doing anything anymore.  One is coming out already, and the other isn't functional.  (sweet!) So more than likely she will need to get tubes again. Also, due to her FA one of her ear canals is extremely narrow (pretty common in FA kiddos) so she gets a ton of earwax built up in her ear.  It starts to affect her hearing and her speech when this happens so we've been going to the ENT to get her ear wax removed with special equipment he has. 

Macy's counts have continued to drop as well.  I'll have to look back on here to see where they were in my last few posts, but it feels like they are much lower now.  Her typical counts now are in the 30's for her platelets and her ANC has been 600-700. This seems to be her new normal which is putting her within grasp of transplant.  The ideal time for transplant is spring.  This doesn't mean that's when hers will be, but if you had to pick the 'perfect' time of year for it that would be it.  It's the least amount of school missed, and not during cold and flu season. It also makes travel out of state a bit less stressful. She will be getting her next bone marrow biopsy to check for leukemia/pre-leukemic cells, and cellularity (the volume of blood/cells her marrow is actually producing) in the first week of January. This will tell us a lot.

Since her last surgery was canceled Macy's stomach has gotten much worse.  Basically what happened was a year ago from last Feb. (Feb. 2015) we were given the ok by Macy's GI doc and pediatrician to remove her G tube (feeding tube) for good.  We were able to do it at home, and they said in almost all cases it will heal and fully close on it's own. We thought that happened.  We didn't notice any problems with it for about a year. Then last winter/spring once in a while we noticed where it healed on the outside of her body (kinda looks like another belly button) was getting a little bit of blood on it.  It looked like it was getting rubbed and irritated. But it only happened once in a great while, and it was a tiny spot. Then it started happening more and that is when we went into her GI's office and scheduled the surgery originally. Since then it has gotten to the point where it squirts out stomach fluid mixed with blood on a daily basis. It's ruining all of her clothes.  We change the bandage every day, but by the end of the day it leaks through onto her shirt, and she ends up with a wet circle on her shirt that turns black from the gastric juices. I'm not really sure why all of a sudden it's getting like this after being ok for a year.  Maybe it's because of her counts being so low now.  I'm not sure.  But we were able to reschedule her surgery for next Monday, Nov. 7th and I can't wait for it to be over. I just hope she doesn't get sick between now and then. We will get her bloodwork on Saturday to make sure her ANC is 500+ and hopefully we will be good to go. We just need to get through school tomorrow without her getting sick from someone. (please!)

That's most of what has been going on for now which is a good thing. When we start our transplant journey I'll be posting pretty much every day.  Mostly for my own benefit in terms of remembering everything, but also for family and friends to know what is going on as well.  It's difficult to update so many individuals.  It's emotional and takes too much time when you're in the thick of things.  So right now, I'm very thankful I don't need to update that often.

take care,
Jenn
                Emmet (2), Macy (5), and Reece (2)
****Macy has since turned 6 (Sept) and the twins 3 (Oct)

Wednesday, July 27, 2016

Appointments & Surgery


I wanted to post an update since Macy has had a few appointments recently and a surgery scheduled for tomorrow (maybe if her counts aren't too low). We have been lucky that this string of appointments are the first ones since March!! Not having any medical appointments for 3 months feels like eons, and it was great while it lasted. 

First up was Nephrology. Macy only has 1 kidney, and it's on her right side. For this appointment she gets an ultrasound then we meet with her Dr. after. Her kidney is looking really good. No signs of tumors, and it's growing and functioning well. As she gets older we have been able to increase the time in between appointments. She was up to every 9-12 months. But we can now push them out to every 2 years. Yeah!

Second was her Hematology/Oncology appt. She gets blood draws for this every 3 months. Every other blood draw is in addition to an appointment and every other appointment includes a bone marrow biopsy to check for signs of leukemia, pre-leukemia, and cellularity. Her counts came back lower than I would have liked, but she has been this low before. All we can do is wait to see if it will remain at these levels or continue to drop. 
WBC 5,710 <~~~I'm good with that since normal is 6,000+
ANC 850 <~~~getting a little lower than what I'm comfortable with since normal is 1500+ and Macy usually sits around 1,000 
Platelets 41,000<~~~also getting lower than what I'm comfortable with since normal is 150,000+ and Macy use to be around 50,000-70,000 this past year. 

Third appointment was Dentist. For those with Fanconi Anemia, dental visits are medical appointments. Even though most of the focus on FA is the blood disease/bone marrow failure part there is SO MUCH MORE that this disease encompasses. Cancer is a huge part of this disease. It's not a "cancer may happen someday" It's a "if I live past the bone marrow failure portion of this disease then I WILL get cancer...and young." One of the highest rates of cancer with FA patients is Head and Neck Squamous Cell Carcinoma. This a fast spreading and deadly form of cancer that starts in the oral cavity. So dental health is ESSENTIAL for Macy. We went and saw 'Finding Dory' in the theater about a month ago and Macy lost her first tooth in the middle of the movie! She was so excited (it had been loose for a few months). She was happy to show her tooth hole to the Dentist. She said Macy's 6 year molars are about half in. So at her next appt we will discuss putting sealants on them to protect them. 

Fourth appointment was with her G tube surgeon. We haven't been there in 4 years, and Macy's G tube was removed about 17 months ago. But recently it has been bleeding on and off so we brought her in. Her Dr. recommended he go in and close it surgically. So he will separate her stomach from the outer wall then close her stomach and the outside separately. This surgery is scheduled for tomorrow. Since her counts are low her hematologist wanted her to get her blood work today to make sure her counts are good enough to do the surgery tomorrow. 

Her Dr. office just called and her platelets are the same as before around 40,000. But her ANC has dropped to 400. <~~~~That's not a good number. What is worrisome is her ANC usually only gets that low when she is sick and she is not showing symptoms of being sick. No fever or anything. An ANC under 500 is critical. Any fever requires hospitalization and IV antibiotics to prevent sepsis, etc. If Macy got the surgery and developed an infection it would be serious. They are going to do a manual count (since it is usually a bit higher and more accurate than the automated one) and will call back after consulting with Macy's hematologist. But my guess is her surgery is going to need to be canceled tomorrow. It's not a pressing surgery so there's no reason to take unnecessary risks. 

So that's all the appointments for now. We are in the process of getting Macy to an eye specialist. She recently told us she sees red and green dots everywhere so I'm guessing she may need glasses. She also failed her hearing test that was given to her the last week at school. She goes for an ENT follow up in August so I will give him the paperwork then. She was just in in Feb and March and had her hearing tested with the ENT after her tubes were put in and it was fine, so I'm guessing the one at school was inaccurate. She tends to get a lot of ear wax build up, and her ear canal in the right side is very narrow due to her FA. But we will follow up none the less. 

I hope everyone is enjoying summer! ☀️


Take care,
Jenn

Saturday, April 30, 2016

Fanconi Anemia Awareness Day-May 1 2016


This is a picture of our daughter Macy. She's 5, and wants to be a Dentist when she grows up. Macy has a very rare genetic disease called Fanconi Anemia (FA). It's a very complex disease, but in a nutshell her body cannot repair DNA damage. This means every day things she's exposed to (like radiation from the sun, chemicals in clothes/the air, second hand smoke from a passerby, and even the by-products of natural cell turnover in her own body) are killing her. 
Kids with FA usually go into bone marrow failure by the time they reach 10 years old, thus needing a bone marrow transplant. This is NOT a cure. A lot of children with FA do not reach adulthood. Those who survive transplant often get cancer in their teens and 20's that the general population get in their 60's+ . Because they cannot handle high doses of chemo or radiation (meaning it will kill them) needed to fight cancer, they don't have many/if any treatment options. Macy wants to be a Dentist when she grows up. If we don't find a CURE there is a really good chance she won't live long enough to be the age I am today. This is HER reality. This is OUR reality. One of the challenges about "a very rare disease" is since most people have not heard of it it means they are NOT personally affected by it. It means they are NOT emotionally affected by it. It means they DON'T donate money to help find a cure. May 1st is International Fanconi Anemia Awareness Day. Our small group of families from all over the world are asking to please help us find a CURE for FA. Please help Macy have the chance to become a Dentist, or anything else she wants to be. Please take the $10, $5, $1 that you would normally spend today on something that DOESN'T matter, and spend it on something that DOES matter. Because Macy and all the other kids with FA deserve a chance to live. 
♥️ Please go to Fanconi.org and click on the donate button in the upper right-hand corner to donate❤️ OR you can text the word FANCONI to 541-515-7332 and you will be sent a link that brings you to a secure online form to make your donation. If you would like your donation to remain anonymous there is a box you can click that provides that option as well as an option to make your donation in honor of someone (aka: Macy). No donation is too small. All donations are tax deductible. 
Thank you
Jenn

Tuesday, April 12, 2016

April 2016 blood draw

Macy had her bloodwork done last weekend. It was her regularly scheduled draw that is without an appointment. Adam took her Saturday morning, and said she did pretty well. We used the emla cream this time with press n seal to hold it in place. It seemed to work well for her. She picked out a red, plastic, bendy snake as her poke prize. :) 
Her platelets have dropped a lot; pretty much by 50% since January. They are around 40,000. Her other counts look ok (around the same as they usually do these days). She is typically at her worst (in terms of counts) this time of year due to a school year full of exposure and illness. We will see what her June/July counts look like before I start to worry. Hopefully they will atleast hold steady and not go down too much. Looking forward to summer and staying away from large groups of people! 

Take care,
Jenn

Tuesday, February 9, 2016

Tubes

Tomorrow all 3 kids are getting tubes. Ear tubes that is. We have to be there at 7:30am and they will rotate the 3 in and out of surgery one at a time. Tomorrow will definitely be an interesting day. Adam's mom is coming down to help for the day so we can each concentrate on one kiddo, and give them the love and care they will need.

Up until last year (2015) none of our kids ever had an ear infection before. It was kinda strange that at 5 years old Macy was starting to get them each time she was sick. I wasn't sure if it was due to her low counts or what. But luckily, thanks to another FA mom I was given some answers. (Thank you by the way).  I want to take a quick pause to talk about how lucky and thankful we are to have our FA family (FAmily as we all call it). When Macy was first diagnosed we were so fortunate to have found the Fanconi Anemia Research Fund (FARF) right away. Many times when your child has a rare disease you are basically on your own. FARF is the only non profit in the USA that funds not only research to find a cure for FA, but family support services as well. We immediately had access to hundreds of FA families and individuals all over the world. We now also have a private FARF Facebook group as well. I can't even explain how helpful this group of people are. Not only in the emotional/mental way of support and community, but the unbelievable wealth of knowledge. If your kid is experiencing anything strange it's almost guaranteed there is someone there that has been through it. There are multitudes of families in every stage. From just being diagnosed, to beginning transplant...mid-transplant, cancer diagnosis, hospice, etc.
Another great thing about this group is its not just a bunch of strangers that never see each other in 'real' life. Not only do people get together in the regions in which they live, but a large number get together at Camp Sunshine in Maine every year. This is a camp specifically for children with serious life threatening medical conditions. There is a week every year dedicated to Fanconi Anemia. Not only do FAmilies attend but all of the leading researchers attend to discuss what they are working on, and where they are at in terms of treatments and a path towards a cure. Parents and kids meet year after year and become close friends. Its people you care deeply about and trust.
However, with this type of FAmily, there is also a great deal of pain. With the news of every child or FA adult's death, it brings such a sense of sorrow and defeat that is difficult to explain. It's also reality staring you in the face. Instead of trying to hide from difficult truths you have the reminder like a neon sign flashing constantly. Parents having to tell their child why their friend is not at camp this year. Parents reaching out to other parents asking how to go about starting a conversation with their child that they are going to die. It's hard, and those things really suck bad. But there's something about difficulty that brings people together. The support and willingness to help each other is unconditional. It is FAmily. And we are so grateful to have found these amazing human beings 5 years ago. I think I'd be a basket-case without them.

So as I was saying another FA parent told us of her daughter that had the same thing happen when she was a bit older. It turns out that due to growth issues that children with FA have, it can cause the tubes not to drain properly and can cause fluid build up behind the ear drum (which is what Macy has) this can cause ear infections. At the age of 5 Macy can also be a bit difficult to understand sometimes as well. She started getting speech services at school one day a week. Not to the extent that an IEP has been needed, but just enough to get some extra help in pronouncing words a bit more clearly. I really think getting rid of the fluid in her ears will help with this a lot. Because she'll actually be able to hear a lot better now.
The boys also got a couple ear infections last year so we decided it'd be easier just to do tubes with all of them and get it over with. Hopefully ear infections will be non existent this year! On a side note, our kiddos Pediatrician that they've had since the day Macy was born is moving to Detroit. :( So we have to get a new one. This makes me so sad. We love her. She's so great. She knows everything about us, and has been there from day one. It's going to be hard to transfer over to someone new that will have to completely start from scratch in learning about FA, etc. We have until March 7 so I better get on it. We will miss her but wish her the best of course.

This weekend is Disney on Ice!! Macy is so excited. We've gone for the last 3 years and I always buy the tickets during pre-sale and splurge and get the middle front row seats. We're about 2 feet from the ice. I brought Macy last year so Adam will bring her this year. She's already looking through all her princess dresses to figure out what one she's going to wear. We will see...

Take care
Jenn

Monday, January 11, 2016

Bone Marrow Biopsy Results & other Appt's

I realized today that I never updated on Macy's appointments last week. (Oops).

On Monday Macy had her bone marrow biopsy.  She fought them putting the IV in, but was fine after that. The nurses did a good job getting the IV in on the first try even after blowing her vein. They had that newer technology where they can scan the arm and it shows you exactly where the veins are so they can't miss. Pretty neat! The procedure went well, and Macy was back to jumping around later that day.
We received her labs that day, and surprisingly her counts were better than they have been in OVER A YEAR!!!! For her they were excellent. 
WBC 6.25
ANC 1290 <~~~~~her ANC hasn't been over 1,000 in a year
Hemoglobin 11.9
Platelets ---they counted these manually so they estimated between 90,000-110,000 <~~~~they haven't been over 70,000 in a year 

Her counts are freakin awesome for her. I am hoping that since this is her second year in school where she is exposed to so many germs that maybe her bone marrow is stabilizing for a little while. 

Later last week we got her biopsy results back and it was also really good news. Most importantly there is no sign of leukemia at this time. With Fanconi Anemia Macy will go into bone marrow failure and need a transplant whether she gets leukemia or not. And having FA makes her highly susceptible to developing leukemia. However, children with FA that develop leukemia before their transplant have much lower survival rates; which is why it's important to transplant before this happens. Her cellularity is at 40%. Cellularity is the amount of blood that your bone marrow makes (how many blood cells you have that make up your WBC, platelets, RBC, etc.). In the typical person your cellularity is usually 100 minus your age. As you get older your cellularity gets lower (which is why the elderly have a hard time fighting infection or healing after injury). At 40% it means Macy's body makes the same amount of blood as a typical 60 year old woman's. And she's 5 years old. Normally this would be alarming to someone; but for someone with FA it is expected and we are happy with that number. Especially since it hasn't gone down at all from last year's biopsy. 

So this means (and I don't want to jinx it) that we should be able to put off Macy's transplant for another year. Or we atleast shouldn't need to worry about it until next fall/winter. Now if for some reason at her next blood draw her counts drop dramatically and continue to fall a lot things could change. But I really feel like she's stable for now. One thing you'll find with a lot of FA families is that a lot of us don't make "plans" more than 9-12 months out. This is because a year from now things could be entirely different. Your child could be doing ok still, you could be in Minnesota, Cincinnati, or New York in the middle of transplant, your child could have developed a tumor or have had transplant already, or have passed away. You just don't know. So you take the time you have and run with it!!! I'm pretty confident we are good for this year. So we will focus on that, and run with it!

On Wednesday Macy had a dentist appt. For most kids this isn't big news, but one of the main reasons that life expectancy is so short for those with FA, EVEN if they received a successful bone marrow transplant is they will get cancer. Not in their 40's, or 50's or beyond. But in their teens, 20's, and the really lucky ones in their 30's. I only say lucky because most do not make it into their 30's. We are seeing more FA patients reaching their late 20's and early 30's and this is awesome. But it's still not the majority. There are a few known people that have made it to their 40's and 50's + but it has only been a handful at best, and almost all of them are mosaic. (Mosaicism is when a person has FA cells but they also have normal NON-FA cells in their body too). Anyways, point being the majority of the time the cancer that FA individuals get is Head and Neck Squamous Cell Carcinoma (aka: head and neck cancer) and it almost always starts in the oral cavity as oral cancer. So dental hygiene is extraordinarily important for Macy. She is 5 years old and already uses an electric toothbrush, flosses, and uses a tongue scraper. Her teeth and mouth are looking great right now. Her dentist said she has her 6 year molars on the bottom already. And her two middle teeth on bottom are loose!!! My reaction was "wow, already? She just turned 5!"  But Mother Nature is gonna do what she's gonna do when she's ready, I guess. Macy is excited about the tooth fairy! 
Macy loves the fish tank at the dentist. They have a Nemo and a Dory in there too! :)

Macy's last appointment last week was with an ENT (ears, nose, and throat). In 2015 she had 3 ear infections. Which was weird because she's never really had one before; even as a baby. I'm wondering if she's getting them now because of her counts. But she does have chronic fluid buildup behind her ear drum which is causing mild hearing loss even when she doesn't have an actual infection. Her brothers have also had a bunch of ear infections this past year so they will be seeing the ENT on the 21st, then we will be making an appointment for all 3 of them to get tubes put in. Fun stuff! The tubes should help a lot with the difficulty she is having hearing due to the fluid, because it'll keep it drained out, and will also prevent infections. 
Macy getting her hearing test 

So that's pretty much everything for now. I am hoping Macy doesn't get sick again to where she ends up in the hospital so we can go until March without a blood draw. She will get put under for the ear tube surgery, but other than that she has no appointments until March. :)

Take care
Jenn