Blood Draw

The blood-draw went well as well as could be expected today. The lab tech used a butterfly and got the vein on the first stick in Anna's left hand. She took 6 tubes of blood! Our little trooper cried quite a bit and I had to hold her tightly on my lap, but by the time the tech was on the third tube Anna had quieted down. I don't think she was too traumatized-- but her mother was --  and she wasn't even there!
Steve

Anna Update

Anna's cough seems to have nearly disappeared! We are so very grateful that she is going to make it through this one (a cold) without much stress on her system. 

We hope the upcoming holidays will continue to find us in good health. It was last year during the holiday season that she caught the cold that put her in the hospital by February.  The CF nurse said recently that because Anna is older she will be better able to fight off colds.  I grab on to any positive statements like that because it lifts my hope.

Tomorrow we have to take Anna in for a "routine" blood draw.  It is our first time.  They are testing for her general health but also to make sure she is absorbing the special vitamins that we give her twice a day.  I have dreaded this day since I found out we had to do it at our October CF clinic visit. I do not know if I can muster up the courage to go- and may send her dad with her instead- but then I know it is at times like this in our lives that we really need our mothers.

From Anna's Mom

Anna is on antibiotics again

I guess I knew it would end. We had such a good run without sickness. Life

felt almost “normal” and one could forget, on some days anyway, that Anna

has CF.  It was August that Anna last had antibiotics.  There was one cold in

there, but it stayed in her upper respiratory area and she got through it herself. 

Though I try to be realistic about how sickness will come into our lives, I still

felt great disappointment when I saw that runny nose and heard that first cough.

Somehow I hoped we could avoid germs if we used enough disinfectant

on our hands and prayed hard enough.  And, I can’t help to feel that somehow

I didn’t protect her enough….   As I write this, she is licking the sliding glass door!

What can a person do?

 

Somewhere out there is a balance that I need to find. The balance between still living

your life and being smart about staying healthy.  I had a horrible dilemma in November

trying to decide whether to let the kids go on a field trip with their friends to Lego Land

( a Lego themed children’s museum).  I want them to have great experiences but sending

them to a “germ park” was a scary thought. What if they brought home a cold? the flu?

I would feel responsible because I made the decision that they could go. After great

mulling and worrying we decided on a compromise- Steve took off of work and took

the three older kids to Lego Land while I stayed home with the two younger. I told Steve that he had to disinfect their hands every 10 minutes and watch them for sticking their fingers in their mouths (unfortunately, all three older kids have oral fixations).  As it turned out they didn’t get colds. But I think that the present one that Anna has came from a trip to a museum last week….   So, where is the balance? Perhaps it will take a few years, or more, if ever, to figure it out.

Wende

Entering cold and flu Season again.. a new blog

Thank you for keeping up with my blog and keeping Anna in your prayers and thoughts. It is evidently working because this little girl of our continues to thrive.

I set up a new blog dedicated to the more technical side of parenting a child with CF -- particularly the health and wellness side of things. We have learned a lot this last year and I thought this would be a good way to share some of the good information Wende and I have come across.  

Anna is doing really well --  she is down to one therapy session a day! Her lungs have been clear, her respiration rate has been good and she continues to thrive. She has a healthy glow about her and I am perpetually amazed by her curly hair. I find myself marvelling at Anna and her radiant personality, contemplating the divine interplay of this gift of CF into our lives. I often wonder how this cross will form her as a grown woman.

Cutting out the evening therapy has been a big deal for us, since that treatment session would typically coincide with Pajama Time for the other four kids.. oi vay...

Thanks again for all the prayers and support.

Today Anna turns 10 months old. Her favorite thing to do is stand up on her own two (incredibly tiny)feet and doing the wobble-walk across the floor while Mommy holds her by the hands. She has a look of total satisfaction on her face like the cat who ate the mouse.  She had her first walk in the park yesterday!

Her least favorite thing to do is therapy, which she is back to doing four times a day now. Simon, Jesse and Luke all have colds--  and now so does Anna. She  swats at her mask incessantly and wiggles, kicks and screams during therapy time. Sometimes we have to restrain her which is slightly traumatic and unpleasant for all.  Needless to say we are worried once again--- still haunted by her last cold which ended up having her hospitalized for a week-- 6 months ago today as a matter of fact. Sorry to be a downer here.  The purpose of this blog is not to make you worried but to pray for Anna.

Anna is 9 months old!

It is hard to believe, but Anna is 9 months old already. She really likes being outside and can sit up and play by herself for long periods. She has two therapy sessions a day and we strive to get as many calories in her as possible. She is still nursing, supplemented with formula and extra salt. She is eating solid foods augemented with extra fat (usually vegetable oil or butter) and takes CF specific vitamins and an ever-increasing amount of digestive enzymes. Anna is doing her job-- she is thriving. Her visit with our CF team a couple weeks ago went well and they are pleased with her progress. Thank you for the prayers and support. Please keep it going.

Anna with her (almost) 3 year old brother Luke who likes to keep her company and, yes, he really DOES need the life vest when sitting in 4 inches of water.

At the Fourth of July Parade with Mommy

Just a little update here. Anna is doing well. We still do two therapy sessions a day: albuterol and pulmozyme by nebulizer followed by chest percussion. Each treatment session takes around 50 minutes. She still has a cough with feedings but our CF team is thinking it is not related to the Cystic Fibrosis-- perhaps a little reflux. She is gaining weight and is thriving. Other than expressing some irritability cutting her first tooth, she is showing an amazingly cheerful and easy-going disposition. Thank you for your prayers and please continue to pray. 

More of the same

Last Thursday we took the kids to the Naperville Children's Museum, otherwise known as the Naperville Germ Park and are paying the consequences.  Our two year old's nose is a virtual faucet at the moment -- he had a fever a few days ago too. The seven year old has a fever and a cold and the five year old is hacking and sniffling constantly. He has perfected the art of wiping his nose with his bare palm. We have run out of kleenex in the house. Anna has gotten the bug too, unfortunately, and coughing more frequently. She had a fever a few days ago but was gnawing on her gums like crazy we chalked it up to "teething." It is disturbing to have so many sick people in the house at the moment. Anna has a doctor's appointment tomorrow and we'll see what they have to say.

Anna still has a cough. Some days are better than others and today happens to be a good day. The good news is her respiration rate is on her low side, she does not have retractions and her lungs sound clear after therapy. She has a cough, but she is not struggling to breathe. Anna is still getting 3 therapy sessions daily and our goal is to get her down to one session a day. Wende and I are able to tell when it is getting time for a therapy session: her breathing gets noisy.

Our doctor wants us to look into improving the air quality in our home speculating she may have allergies.  I have an allergen highest rating air filter in our furnace blower and have covered the vents with filters as well.  I am looking into buying a couple air-filtration units for the house. Does anyone have any experience with these and can recommend a particular brand or type?

On another note, you may have noticed I have allowed ads on this blog--  please click on them. These ads are helping to fund Anna's medical bills. Thank you.

Also, please enter your email address to the right and when I update the blog it'll show up in your email.

Finding Normal

Thus far we have been sharing with you our struggle as parents with Anna's Cystic Fibrosis and the rollercoaster it has been. Thank you for your continued thoughts and prayers. The primary purpose of this blog is to remind everyone to continue to pray for Anna (and her family) and to keep eveyone updated on how she is doing. As a side note, if you are not accustomed to praying for someone, please check out this article and give it a whirl. If you are not sure how or what to pray for, give me a holler.

Wende and I feel strongly that Anna not be known as the "sick" child. She is a fun and particularly joyful little child. With the five children, our house is "high-energy"  and she is the cause of so much joy and delight in it. As I type this, Anna is sitting up in her bouncy seat talking to her 5 year old brother-- who is delicately listening to Anna's heart with his stethescope. He is reporting to me that her heart sounds good.

Here are some observations from Wende:

-Anna likes to do a full body stretch while I am changing her first diaper after getting

  her out of her bed in the morning. Her legs go straight out as far as they will go.

- Grandma Foht has declared that she has the “Foht smile”- because her upper lip

   disappears when she flashes you her cute little smile.

- We were told by the pediatrician to give her more “tummy time” to strengthen her

   upper body.

- She actually responds to “sshhh” when you are trying to sooth her.

- She attempts to hold her bottle and the nebulizer.

- She displayed some “stranger anxiety” when a friend came over for a visit.

- She had her first conversation on the phone with her dad.

- While on the diaper table she usually puts both feet straight up in the air.

- Everyday she soils one or two outfits that I end up scrubbing in my sink.

- Her eyes follow her mommy everywhere.

Here the Battle Line is Drawn

I never thought we would become germophobes. The daily battle against Anna's cystic fibrosis is in preventing ANY respiratory infection. She is not immunocompromised. Her immune system is completely normal,  however her body's normal response to a cold or flu is the problem. Most kids can clear their lungs of fluid or mucus, eventually recover from the infection and move on. Kids with cystic fibrosis cannot clear their lungs on their own and during a "pulmonary exacerbation" i.e. a flare-up, they essentially begin to get clogged by their own mucus. Because there is always mucus in their lungs, once a certain bacteria (pseudomonas aurigenosa) arrives it never leaves because the evil critters are able to hide in the mucus from antibiotics. The word fibrosis refers to the damage and scarring that occurs in the lungs as a result of the long-term infection and inflammation.

Anna was four months old with her first hospitalization because of a pulmonary exacerbation that started with a cold..    ..this is war.

And here the battle line is drawn. With other small children in the house, the daily struggle against the spread of germs takes has taken on epic proportions. Our two year old does not understand physical boundaries and  evidently thinks Anna is the coolest thing around. He is very affectionate and gentle with his little sister. To our alarm he loves to come up and put his face just microns from her face, and he is very entertained by playing with her hands. Our five year old has a runny nose currently, and has the unconscious habit of wiping his nose with his bare hand. To my horror I have seen him wipe his nose and immediately touch Anna's hand and then have her place her hand in her mouth!

So we are forced to make some lifestyle changes. We have had to cancel some social engagements because others attending were sick. Our CF center has taught us to use hand sanitizer before picking Anna up. We have invested heavily in hand sanitizer and are trying to get the children into the habit of washing their hands immediately when coming in the house from outside and using hand sanitizer before touching her.  It is a little awkward requesting others to do the same.

A month after being out of the hospital, we are still a little freaked out-- obviously.  Forming good habits for our family is ultimatley what needs to happen and then we can move on. We are not holed up in isolation nor avoiding people, but we do need to be more cautious for Anna's sake. We know we will find a balance between being overprotective and putting her at risk. It's just going to take a little time.

Ups and downs

Anna is doing better. Our little hero is off all antibiotics after 3 months of continuous use. We have gotten into a daily rhythm with adding Anna's special needs into the daily routine. She is down to two treatments a day of nebulizer (Albuterol followed by Pulmozyme) and then 20 minutes of chest percussion. We've been doing one session in the morning and once before bed. we were told that if she continues to improve she will back down to one treatment session a day as her daily maintenance program. However, as of yesterday she has started coughing again... so we need to call our team and see what the plan is today. I listened to her lungs this morning and there is some congestion again.

The rallying support we received from our family, friends, co-workers these last several weeks has been overwhelming. Thank you to everyone. The army of people praying for Anna and our family has been amazing and edifying. Please do not stop praying.  Until there is a cure for CF, she will not know life without daily treatment and taking capsules of enzymes every time she eats. Please pray that the new medications in the research and development "pipeline" are safe, effective and on the market soon. Please pray that her symptoms stay under control.

Welcome to Cold and Flu Season

Sometime in December Anna got a cold-- just like the rest of the kids. She began to get a cough which didn't go away. As time progressed, so did her breathing rate and "rattles" in her chest. I think the progression was so gradual it was hard for us to notice how bad it was getting. On one of our visits to the CF center, Dr Akhter noticed she was having "retractions" in her ribs and above her stomach. She seemed to be having them in her neck as well. He explained that this was an indication that she was struggling to breathe. At the time her oxygen saturation was good, but the cough persisted and her lungs were congested. Anna was vomiting a large amount of her food and it seemed to contain quite a lot of mucus.

We were becoming more and more alarmed as we realized Anna was not getting better. We were already doing therapy (nebulizer & chest percussion for 20 min) three times a day at home.  (I say "we" but it is really Wende who is the hero in this story. I get to leave the house and hang out in the world of grown-ups all day at work. She is the one taking care of EVERYTHING-- using her supernatural gift to organize, educate and provide a sense of order and foundation amid the chaos of our infant and the other 4 children whom I affectionately refer to as Madame Curie and the Three Stooges. While Padre Pio may have had the supernatural gift of bilocation, Wende has the gift of being organized, and as St. Josemaria Escriva said "being organized multiplies your time." But I digress...) Anna had been on three different rounds of oral antibiotics for the last 6 weeks. Was this "just the CF" symptoms starting to show up or was this an infection? If it was viral, why was it being aggressively treated with antibiotics? Why wasn't she getting better? Dr Akhter informed us that he would like to have her hospitalized, put on IV antibiotics and receive more intensive treatment. Wende and I initially said no and wanted to give it another week. We were caught off guard, not to mention that there are logistics involved concerning the other 4 kids.

 We were told if Anna was not significantly better by the following Thursday she would be admitted into the hospital, so we should "have our bags packed" when we came follow-up visit. My feelings of alarm had turned into fear. 



That next week was spooky. The blizzard hit, I stayed home from work for a few days. Anna's labored breathing continued and her chest and neck retractions were as plain as day. By the time Thursday rolled around they had finally plowed our street (it took 2 days after the snow stopped) and we could get out of our driveway. Wende and I were both mentally prepared to have her hospitalized. We had also arranged the logistics for the other kids.

Getting Anna into the hospital ended up being an all-day event. When we arrived at Hope Children's Hospital to meet the CF team before she was to be admitted, we were told that they couldn't admit her. There were no beds available. Probably not that day. Maybe not even Friday. I couldn't believe we were being sent home. We went back to pick up the kids and waited at my mom's house for a few hours. I called the "bed coordinator" and pleaded with her if there was anything she could do to get us in. She informed me that there were 8 other children ahead of us waiting for a bed. It was late, so we drove home to Joliet, and as we were pulling in to the driveway, I received a call that they could put Anna in the ICU if we turned around and came in immediately. We turned around and finally got her in.

Anna was at Hope Children's Hospital for a week. Wende stayed with her and did not set foot out of the hospital that whole time. Our doctor showed us her chest xrays compared to her normal, baseline films taken just 1 month prior. I was shocked at how dramatically different the two films were. The radiologist who read her films called it pneumonia, and I suppose that is a way to describe what her symptoms. And evidently that is an academic debate and a matter of semantics as our pulmonologist said  it is not "pneumonia," it is a "pulmonary exacerbation."  "P.E. is the code-word now."    ...got it.

So, to put the pieces of the puzzle together: the cold or virus that Anna had evidently caused her cystic fibrosis mucus-thickening-can't-get-it-out-of-you mechanism to kick-in high-gear. Dr Akhter told Wende that he had not seen such severe CF symptoms in someone so little. That was tough to hear. You never like to hear a specialist tell you news like that. CF treatment and management is supposed to be a long-term game plan, not a "4th quarter and the team is down 14 points" game plan.

Anna's therapy was upped to 4 times a day. She was put on Pulmozyme, which breaks down mucus and helps people with CF cough it out-- but most CF patients aren't on this medicine until later in life. She was on IV antibiotics even though her cultures did not show anything bacterial.



Worst case scenario would be that she came up positive for a type of Pseudomonas -- a bacterium that people with CF get-- the proverbial "CF Bug" that never leaves once they get it. God Be Praised they didn't find that.

Over the week her oxygen saturation improved and her respiration rate became more normal. The chest retractions improved too. Except for the nurses who changed her IVs, Anna greeted everyone with a smile. This is not the Proud Pappa making overly sentimental platitudes-- she really smiled. On more than one occasion Wende was told by the staff that it was "nice to have a happy baby." I wish Anna had words. In her own little way she evidently brightened the day for a few others.

Anna was discharged from the hospital last Thursday and we see our CF team tomorrow. She'll have a follow-up chest xray in a couple weeks to see how her lungs are looking.  Wende told me today that she could barely notice the chest retractions. Thank you everyone for your prayers. Please keep it up.
.

Our Introduction to CF

It was the end of October. Anna was two weeks old and we were still getting used to having a little baby around the house again. Wende and I had just put the other four kids to bed and were enjoying that blessed peace and silence that ensues once the kids finally fall asleep.  The phone rang. It was Dr Reicherts, our pediatrician and I was alarmed by the fact that he was calling us so late in the evening for no apparent reason. He sounded very concerned. He was calling to inform us that the State of Illinois now screens all newborns for Cystic Fibrosis, and Anna had two genetic markers for the disease: F508 and G542X. He had just received the fax from the State and was calling us to let us know the results. He recommended we find a Cystic Fibrosis Center and set up an appointment for follow-up testing.  I hung up the phone and shared with Wende what the doctor had just told me. I was in disbelief. Surely they were wrong. Anna was not sick.

The next day I spoke with Dr Akhter, a pediatric pulmonologist. His office made arrangements for Anna to have a Sweat Test two days later, which would determine if Anna really had Cystic Fibrosis (CF). We had an appointment to meet Dr Akhter and his team after the sweat test to discuss the results. I was looking forward to having some peace of mind knowing that though she may have the gene, she did not have the disease, and we could put this little scary episode behind us.

We went to Hope Children's Hospital in Oak Lawn and Anna had her sweat test. We met with Dr Akhter and his team at the Cystic Fibrosis Center to discuss the results. The tests was strongly positive for CF. My disbelief had turned into shock: that sudden alarming type of shock that takes your breath away, like how it feels having jumped into freezing cold water and you can't get out fast enough. They did another test to check her pancreas which showed she was producing almost no digestive enzymes whatsoever. She was already in a state of malabsorbtion and not getting sufficient nutrition.

The doctor went on to explain that Anna will have "typical" CF, which meant that we could presume she will manifest lung and pancreas symptoms, but there was no way of knowing how severe her symptoms would be. The doctor and the CF team presented Anna's diagnosis in a hopeful, positive manner, but neither Wende or I were able to appreciate what he was saying. He was very optimistic about the future of people with CF in light of the early detection in newborns and new treatments on the horizon--  but it still felt like a death sentence for Anna. It was going to take some time for Wende and I to accept the bomb that was just dropped on us.

We met with the entire CF team every Thursday. The team consisted of Dr Akhter, his nurse Jean, a nutritionist, respiratory therapist and a social worker. They monitored her status and began to give us instructions for her daily treatment.  First, we began giving pancreatic enzymes with every feeding so her body could absorb the nutrients in her food.

This was achieved by opening a capsule of Creon and dumping the contents into Anna's mouth immediately before feeding. The goal was to have her in the 75th percentile for weight of children her age. I wanted to see fat baby thighs. Anna also began to receive  a bottle at night with salt and fat-soluble vitamins. We were taught how to daily chest percussion as a means to keep her airway as clear of mucus as possible. Several weeks later we began giving her a daily dose of albuterol medication using a nebulizer, followed by CPT(just like in this video clip). Over the course of a few weeks we began to get into the routine.


Anna is now 4 months old and is showing her personality already. She likes to talk and she is extremely generous with her smiles!