Hope is a Journey….

Well, it is upon us….Cold and Flu Season is unfortunately here.

This is the time of year that most families that battle Primary Immunodeficiency Disease (PIDD) or other serious health conditions dread.

Why?

Because people sometimes don’t think.

People sometimes forget that their choices and decisions have a larger reach than they think they do…

People don’t realize how one simple thing for them can create a devastating domino effect in the lives of others.

And sadly…there are even some people who are just plain selfish and don’t care about the consequences of their actions.

So, for the sake of just putting it all our there…..I’m taking the time to try to explain to friends (and yes, even some of our extended family still don’t grasp this yet) about why it is SO important to keep your germs to yourself!

Will and Sasha have Primary Immunodeficiency Disease (PI)

Individuals with Primary Immunodeficiency Disease have immune systems that do not function properly.

What does that truly mean though?

For Will and Sasha, they lack a few things that are critical for proper immune function:


1. IgG – This part of the immune system is supposed to react in response to invasions by bacteria, fungi, and viruses.  When there aren’t enough IgG, the system cannot react properly…and sometimes, it doesn’t react at all.
(Click HERE for more information)

2.  IgA – IgA is a major antibody in the saliva and in the mucous membranes of the intestines and the bronchi. It protects against bacterial and viral infections.  It is the first line of defense against infection.  If there aren’t enough IgA antibodies, the individual isn’t protected against infection.

3.  Poor Vaccine Response:  This means that, even if they are vaccinated against something, the body has no memory – or doesn’t remember it well enough to fight it off if exposed to it…thus defeating the purpose of vaccinations.

For example, Will was vaccinated against Pertussis and ended up extremely ill and hospitalized from it while we were on vacation in Myrtle Beach.  While this vaccine isn’t 100%, most people vaccinated against pertussis are protected from it…Will was not.

Will getting a breathing treatment in the hospital when he had Pertussis

Because both Will & Sasha have ‘broken’ immune systems, they receive weekly infusions of Gammaglobulin (blood product derived from thousands of plasma donors) to help replace the things that are missing in their bodies.

Being on the infusions helps protect them as it gives them the missing antibodies weekly thus taking over for the fact that their own immune system cannot do this naturally.

The other thing that needs to be considered is that, because their incidence of bacterial illnesses are more frequent AND the fact that they need to be on a daily low dose antibiotic (and, for Will – the fact that he is allergic to several classes of antibiotics) – we try to avoid any illness because it could lead to the need for full dose strength and/or other heavy duty antibiotics.

The children are very young still – and, if we continue on this pace – by the time they are teens or in their 20’s, their antibiotic options may be extremely slim (or almost none in Will’s case as we are already running out of options) – or they may end up becoming resistant to them.  We could find ourselves in some seriously treacherous waters with the kids.

It also means that illnesses that they have gotten before, they can get again with the same level of intensity because the body does not remember the past exposure like a normal person’s body would.  For example, when exposed to something as simple as the common cold, the system does not recognize it (you can build antibodies to certain viruses and bacterial infections and actually fight it off if you are exposed to that same strain of bacteria or virus a second time OR you can get a much lesser version of it if exposed to it again).

It means that vaccinations they get are often ineffective (we still get our flu shot even though there’s a good chance it won’t work…it’s worth a shot – no pun intended – to hope that it will).   Individuals with PI should NOT be getting live vaccinations (like the MMR and Chicken Pox vaccine) because the person with the PI cannot fight off virus/bacteria even in small doses – and it causes actual sickness within the individual (this happened to Will with the MMR shot, he got very seriously ill from it – we didn’t know at the time about not getting live vaccinations because he was just newly diagnosed and we were still in the process of learning about the disease and the initial doctor we had was a joke).  If others living in the same household receive live vaccinations, they actually are not supposed to come into close contact with those with PI until the risk of exposure to the individual with the PI is gone (with each live vaccine, that time frame varies – with the chicken pox, it’s a LONG time!).


When it comes to viral illnesses, we need to be careful….not because we worry about the cold, but because ‘just a cold’ to our children becomes a sinus infection, or bronchitis, or pneumonia.

Here are 2 perfect examples of why we worry – the first occurred several years ago – it was Thanksgiving. One family brought their kids along and they were sick with ‘just a cold’ – I was upset about it, but I did nothing about it because I figured it was a moot point since we had been around them for a few hours before it was clear they were sick AND both of our kids were on low dose daily antibiotics to prevent bacterial infections.  Several days later, Sasha and Will both became ill with the cold.  Almost week into it, Sasha was diagnosed with a sinus infection (despite being on preventative antibiotics) and placed on a high dose 21 day course of Augmentin.  She continued to cough and be unwell, and…a week before Christmas, Sasha was so very sick that we had to rush her to the ER – turns out she had bilateral bacterial pneumonia (despite being on the Augmentin for 18 days at that point).  It was very scary for us and we ended up missing all the ‘Christmas’ activities because Sasha was sick.  Meanwhile, the child (the one with ‘just a cold’) who brought the illness to us was completely fine within a week of our initial exposure over Thanksgiving.


The second example that highlights this occurred just before Halloween 2 years ago.  At another holiday gathering, a family brought their child who they felt was sick with ‘just a cold’ to join the festivities.  Despite knowing our situation and that our children (who were not sick at the time) would be there – they went anyway.   Their child had had a little fever (low grade) and some congestion, so they gave her antihistamines and Tylenol prior to arrival.  Several hours later, the medicine wore off and it was clear she was not well.  When I approached the parent about the clearly unwell child, I was told it was,  ‘just a cold.
To say we were upset was an understatement.

Sure enough, a few days later, both kids weren’t just a little sick…they were VERY sick.  Turns out that the child had the the start of the flu and the parents didn’t realize it was the flu at the time. What these parents felt was just the start of something harmless turned out to be something very serious.

Will and Sasha had to be quarantined (Swine Flu) and it was a total nightmare.  They had to be started on Tamiflu and also placed on more antibiotics and missed quite a bit of school.  It was a long haul and they were STILL sick at Christmas from secondary infections.  It was a really rough ending to the year for them that year.

On top of that, Will, in particular – has low neutrophils and is considered ‘chronically neutropenic’ and his level of deficit is moderate.
So, what does that mean?

It means that his primary defense against bacterial infections are low.  Sasha is also ‘chronically neutropenic’ but her deficit is considered mild – her numbers are only marginally better than his and have been on the decline – so that ‘mild’ might become categorized as ‘moderate’ in December when we see the immunologist.
Sometimes, the reality is that their little bodies just aren’t strong enough….

What does it mean in a nutshell?

If their defenses are low enough, they can get pretty sick from bacteria that other people carry and aren’t even sick from.  The most common type of infections seen in neutropenic patients are caused by bacteria normally found on the skin (such as Staphylococcus aureus) or from the gastrointestinal and urinary tract. Fungal infections are also more frequent in patients with neutropenia. The infections may be limited to certain areas of the body (commonly the oral cavity, genital area, and skin) or may spread via the bloodstream to the lungs and other organs in severe, prolonged neutropenia.  Funny enough, Sasha has WAY more issues with Staph infections, in particular, than Will does – and his numbers are lower.

SO…99% of the time, we are extremely careful about ANY illness exposure.

I will admit though, that there have been times that we should have been more cautious and were not – some of it was sheer irresponsibility on our part and some of it was hoping that we could have a little normalcy and just enjoy life without the worry.  Most of the times we have played our cards this way, it has come back to bite us.


So, we’ve learned some hard lessons because of just wanting to have some ‘normalcy.’

Even at times, because we were selfish for wanting that normalcy –

they’ve paid the price…definitely unfair of us to do to them.

And, at this point – we have found what works to keep our kids as healthy as possible and have learned our lesson from taking chances.

Here are the general guidelines that we use:


1.    If you are sick or anyone in your house is sickwe should not be in contact with each other.  Often times, if it is not bacterial, we give it a few days even AFTER all are well because you could still be shedding the virus even though you feel okay.   That’s why it’s good to know what we’re up against because we then are able to research it online and see if we’ve been exposed and also how long that particular virus is shed for to avoid exposure.  For example,  even different types of stomach flu have different incubation periods and different lengths of viral shedding (http://www.mayoclinic.com/health/stomach-flu/AN01758).


2.    We are super crazy about anyone entering our home being vaccinated with the flu shot.  The flu shot (not to be confused with the flu-mist) is a killed virus and poses no risk to you or to us.  (http://www.cdc.gov/flu/protect/keyfacts.htm).  All too often I hear conversations about people not getting the flu shot because they think it contains mercury – so I’ll also include some info on how this assumption can be wrong as well HERE.


3.    The cold virus and the flu virus are 2 VERY different beasts.   They are 2 separate viruses.  I know that some are under the misconception that they are the same, but they are absolutely not.  We take the flu VERY seriously, especially after our run in with it a few years ago.  (http://www.scientificamerican.com/article.cfm?id=cold-flu-difference)


4.    We are SUPER vigilant during cold and flu season – for reasons listed above in #3 and also the example we gave with our encounter with the flu.  We stress A LOT of hand washing and avoid crowded areas usually beginning at the end of October (or whenever we see the Flu cases in our state start to rise) through April (or later if the state health department indicates on their website that cases are still occurring in significant enough numbers) in particular.  We follow that on the state website here: http://publichealth.blog.state.ma.us/h1n1-swine-flu/ (it gives you links to graphs and also gets in great detail as the season progresses), and also the CDC site here: http://www.cdc.gov/flu/weekly/ (yes, these are the things we do that often don’t get spoken of – but are happening for the well-being of the kids).


5.    While school and going to the doctors’ offices and such are calculated and necessary risks, avoiding ANY known illness (no matter how seemingly harmless) or possible illness is critical for us.  We do not take any chances and the physicians have also instructed us not to, which is why – when the H1N1 first came out (and almost 6 months before they actually contracted it from someone at a holiday gathering), Will and Sasha were pulled from school at the direction of our immunologist and they were tutored from home for the last few months of school.


6.    To complicate matters more, because several people in our home have been diagnosed with Mitochondrial Disease, we have to also be extra cautious of how this complicates that picture for them.  The greatest risk to anyone with Mitochondrial Disease is the period during the illness and 2 weeks after the illness because the mitochondria are stressed and unable to keep up with the demands of the vital organs.  This excerpt is taken directly from the UMDF’s website:  “The child or adult is at highest risk for neurological and organ damage during and for the two weeks following an illness.  Therefore even a simple flu or cold virus can have devastating effects on the patient, even death.  Any illness must be treated immediately with medical interventions, like IV fluids and IV antibiotics.”  Click HERE for the entire excerpt.


So, as you can see, we have SO many factors and reasons that we have to consider on a daily basis, and – right now, especially given that we had stressed both kids physically by traveling (which took a toll on Sasha towards the end of the trip), we really need to be extra cautious.  Going further on this point, while *I* do not have the same immune deficiency as them and do not require infusions (though it has been discussed), my body is so compromised and weakened from the mitochondrial disease that *I* do not handle anything respiratory or GI well at all.  For example, 2 years ago I was in the hospital for an entire week JUST because I had a GI bug that only caused me to throw up ONE time.

One of the most dangerous things to do is to assume it’s ‘just a cold’ or it’s ‘just allergies’ – if you don’t feel right – let us know and let us decide whether it’s better to be safe than sorry as it is our family that will have to deal with the fallout if it is in fact an illness.


I hope that this helps to clear things up for some of you – and I apologize to you all for not doing this sooner….

I realize that this is a lot of information to take in and digest – we can appreciate this.  I just wanted to give you all the pieces to our puzzle so you can realize how complicated this is for us and why we have to be so strict about things.

The same consideration should be given to ANYONE you come in contact with as you just NEVER know what battles they (or someone they live with or care for) are facing and how far the ripples from your actions will reach.

Here are some things you can do for the benefit of ALL:


1. Wash your hands! This is the most effective way to stop the spread of many illnesses!

2. Get plenty of rest – rest allows your body to heal and rejuvenate itself.  If you don’t get enough rest, you are susceptible to illness.

3. When you’re sick, visit your doctor!  One of the worst things you can do when you’ve been sick for several days, or – if your sickness is more severe or different than the ‘usual‘ sicknesses you’ve had is WAIT….that allows for possible secondary infections to brew and then spread to others.

4. Eat healthy and don’t skip meals! Your body needs healthy sources of energy to function properly and be able to defend itself from illness and disease.

5.  If you’re sick, STAY HOME! Despite the old saying, “Misery Loves Company,” I can guarantee you that no one wants to join you in your sneezing, coughing, body aches, boogers, vomiting, and other unpleasant bodily nuisances that might occur.  There are SOME things you MUST do (like go to the drug store or food store to pick up necessities) when you are not well, but other things (like going to the movies, running errands,  and even going to work) should wait.  That being said, I understand that some people don’t have the luxury of ‘staying home‘ – but, in an ideal world, it is what we ALL should do when we are sick.

6. If your child is sick DO NOT send them to school where he/she can infect the rest of his/her classroom.  Again – remember, to your child, illness may be no big deal – but what if another classmate is immune compromised or has a parent or grandparent who is fighting cancer or is just too weak to fight off infections?  Think beyond yourself for a moment an imagine if YOU were the parent of that immune compromised child…or you yourself were the one who wasn’t able to fight off illness and had to then care for your child (who got sick from another kid at school).

7. Cover your cough/sneeze.  When you are sick, sometimes you can spread your illness within the tiny droplets that are emitted during coughing and sneezing.  It’s pretty simple folks – click HERE for a visual if you need it….

8. Don’t forget to exercise!  Research shows that engaging in moderate physical activity can help protect your immune system.

If you just follow the simple helpful tips above, you could make a HUGE difference not only in your own life, but in the lives of those around you! 

For some….

In the end, there really are NO EXCUSES to not think about how your actions impact those around you…loved ones and strangers.

Please do forgive us if we get upset or worked up about even the little illnesses (especially during cold and flu season) as it can be hard for us to relax about things that are seemingly ‘non issues’ for the typical healthy person.

I do ask for you to please understand that we are not trying to be difficult with you or be jerks about anything – we just are trying to keep our family as healthy and illness free as possible.

Will and Sasha are blessings in our lives and we will do anything and everything in our power to keep them happy, healthy, safe and as illness free as possible.  I hope you’ll join us in our mission not only to help keep Sasha and Will happy, healthy, and safe – but all those around us as well….because THIS….

and this….

Is NO way to live…..

THIS….

is LIVING!

And isn’t that what life is all about????

Love and Light,

Stefani

Comments on: "It’s not just about you….." (2)

  1. Shelly Dowdle said:

    My son does not have the same condition, but he did have a kidney transplant. His immune system is less suppressed than it was right after transplant (he had it 16 years ago), but I still worry. We all get the flu shot every year as well. When my younger son had his vaccinations (MMR and chicken pox) I was REALLY worried about him passing it on to my older son, but thankfully he was already pretty far out from transplant and we were told as long as he had limited contact and did not change diapers or anything like that. Also LOTS of hand washing! I really hope that people with start to think about others after reading this!

  2. My son has PIDD. He was diagnosed at age 7 – 10 yrs ago. We are blessed in that he hasn’t had any severe illnesses. He does receive a weekly infusion go IGG. Your article was great. So many people have no idea what we deal with.

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