What Is Isolation All About?

As we go through the bone marrow transplant journey and are into the post 100+ days period (we are at Day 126), family and friends are wondering why they still cannot visit or come to our home for playdates when Kate does get home. I thought an explanation here might help:

To prepare Kate’s body for a bone marrow transplant, she was required to undergo ~9 days of intensive preparatory chemotherapy and immune suppression. From the moment we walked into her room on the 3rd floor of Ste.Justine’s hematology oncology building, we were masked, gowned, and became obsessive hand washers. Anything that came into Kate’s room had to be sanitized – toys, clothes, bedding from home, iPads, cell phones etc. ‘Visitors’ were limited to a finite list of 7 people that were very well briefed on protective isolation protocol. We ensured that list was comprised of people who could commit to helping us care for Kate while she was undergoing her bone marrow transplant, and we negotiated for her brother to be added as a #8 to that list. (He was amazing with adhering to the protocols – very conscientious).

We were required to maintain this ‘protocol’ from the moment Kate started her preparatory regimen until her neutrophils were back above 0.5

Neutrophil granulocytes (also known as neutrophils) are the most abundant (40% to 75%) type of white blood cells in mammals and form an essential part of the innate immune system. They are formed from stem cells in the bone marrow. They are the ‘first responders’ in case of a bacterial infection. (Interesting fact: they are the predominant cells in pus, accounting for the yellowish/whitish appearance.)

Once above 0.5 we were allowed to remove our gowns and masks, but hospital personal and anyone else entering her room had to keep masks on. Hand washing and sanitizing were still very important. With her neutrophil count above 0.5 Kate had limited protection against  bacteria, but still had no immunity against common viruses (e.g. the common cold), or fungus (fungal infections). Isolation was very important.

As Kate recovered her blood counts her ‘neutropenia’ (neutrophils below 1.0) continued to have ups and downs. Her body was weak and needed ongoing protection. She was also taking heavy doses of methylprednisone to control her graft versus host disease, and cyclosporine to suppress her new immune system (to prevent GVHD). When she exited her room she was required to wear a N95 mask to protect her from airborne viruses. She was also to stay away from other people as much as possible. Her room continued to be kept as a ‘clean space’ and her home was also meant to be a ‘clean space’ with limited visitors (at our discretion), because of her immune suppression.

As you all know, Kate wasn’t home long, she was back in hospital less than 72 hours after being discharged to home. When she returned to hospital, she was again confined to her room and protective isolation protocols were put in place again. Mask, hand washing, limited visitors (immediate caregivers and helpers only).

Now that we are back at Ste.Justine, on the post-BMT unit, this protective isolation protocol remains in place. We have GVHD, and the treatment of this is immune suppression which makes Kate more vulnerable to infection. Kate is allowed to leave her room her, but can only ‘play’ on the 3rd floor BMT unit. She can’t visit the playroom on her floor (post BMT recovery), or be near any other children or parents on the hematology oncology unit. Her room is limited to her nurses and to visitors that are ‘approved’. This means that as her parents, we can choose who comes into her room, but we have been educated to be cautious and limit how may people interact with Kate from the outside. It is for her protection as she continues to be immune suppressed.

We are so grateful for the offers of help and support. Many of you have been so willing to come and see us in Montreal, and it is sooo difficult to say no. But we have to. Believe me, we would love the help and assistance, as there is no doubt there is a lot fatigue among Brian and I and those who have been helping us. But to protect Kate, I have to say no.
We have limited our ‘caregiving team’ here to what is essential and to those that have been trained and understand the isolation protocols and we are grateful for their continued support.

Once Kate returns home, she will continue to be immune suppressed and we won’t be able to have visitors at our home. She will get to go outside and see people from a distance, but not much more. Crowded places are a risk for her. This protective isolation will end when her immune suppression ends and when her CD4 +T helper cells start to recover. This will be another important milestone for us as it will signal that she has better protection against viruses and fungal infections.

 CD4+ T helper cells are white blood cells that are an essential part of the human immune system. They are often referred to as CD4 cells, T-helper cells or T4 cells. They are called helper cells because one of their main roles is to send signals to other types of immune cells, including CD8 killer cells, which then destroy the infectious particle. If CD4 cells become depleted, for example in untreated HIV infection, or following immune suppression prior to a transplant, the body is left vulnerable to a wide range of infections that it would otherwise have been able to fight.

We are watching what is called the CD4 count in Kate’s bloodwork. A consistent count above 200 is the goal.  We anticipate that being sometime in October/November. At that point restrictions will be lifted, but we still need to be cautious as Kate’s new immune system will have not been exposed to viruses that are common and will have to be revaccinated against childhood disease. 

Julie

The Art of Imperfect Decision Making

A Recipe for Making Decisions

How to make an impossible decision in 7 easy steps 

 

Ingredients:

Information and Research

Gathering of knowledge from ‘the experts and specialists’

Analysis

Questions

Discussion

Weighing of Opinion

Pros/Cons

Evaluation of information

Decisiveness

Gut Instinct

Support

 

Method:

1. Gather as much information as you can through research. Mix this with knowledge and expertise from many sources (e.g. experts, specialists, others who have made a similar decision), placing an emphasis on those who are leaders in the area/topic on which you are trying to make a decision. Be prepared to search this information out internationally.

2. Mix all of this information, knowledge and expertise together and conduct an analysis by comparing and contrasting everything you now know. Where is the information similar, where is it contradictory, what are the gaps in your information, what other sources of information and expertise might be available, and find out what you do not know that you need to know. After this step in the process you can move to formulating your questions and identifying what more you need to know or who else you might need to speak to about your decision.

3. Your next step will require several discussions about the decision you are trying to make. The structure of these discussions will vary, and your recipe for decision making could include: 1 to 1 discussion, informal conversations with trusted friends/family/professionals, formal multi-disciplinary team discussions, discussions with specialists in the field. You might conduct your discussions over the phone, face to face, or over video-conferencing. You may also need to consider travel to other destinations in order to conduct your discussion in person. Consider the following when organizing and preparing for discussions related to your decision-making:

– Bring a friend who can listen and take notes. It is difficult to take all the information in when you are in a very important discussion/conversation. Having another person to assist in the task of listening and note taking can be very helpful to your decision-making process

– Ask if you can tape record the conversation. This can be helpful so that you can hear the conversation again.

– Prepare in advance. Write down a list of questions and concerns related to your decision and send them to the meeting participants in advance so that they are prepared to address them. (Sometimes sharing this list with knowledgeable friends or professionals in advance can help you to refine your list of questions).

– Bring a notebook and paper.

– Ask another person (social worker, nurse, trusted professional) to facilitate the meeting so that you can focus on the discussion and not on managing the meeting.

4. Analysis and Weighing of Opinion.

You will need time after the gathering of information and knowledge, analysis and discussion to weigh the information you have been provided with. A helpful tool  in this step of the Decision Making Recipe is to develop a Pros vs. Cons list and then to add a value or weight to each of the Pros and Cons. An example of this can be found here, Ottawa Family Decision Guide.

It might also be helpful in this step of the process to engage a Decision Making Facilitator or Decision Making Services, a third party who guide you through this process. This is particularly important to ensure there is open communication and that all parties of the Decision Making Recipe are working together and communicating well. In intense medical decision-making, this can sometimes get lost – this is where facilitated sessions can help.

Professionals can help guide you through the weighing of opinions and information, and can help identify where your priorities lie with respect to the decision you are trying to make.

5.  You may have to repeat Steps 1-4 more than once in order to feel decisive about your decision.  It is important to know that important decisions cannot be rushed, and you should feel supported in taking your time to make a decision.

6. Gut Instinct.

Your gut instinct needs to factor into to your decision-making. If it feels wrong, it probably is. In my experience, my maternal instinct has been referred to by our medical team and decision-making team, and I have been told to listen to it and factor it into my decision-making.

But what if your gut instinct isn’t clear? What if it isn’t saying anything at all?

I call this the vortex of decision-making. Circling and circling because the information, expertise, knowledge that informs the decision you are trying to make is imperfect and incomplete. How do you make a decision with imperfect and incomplete information? You make the best decision you can with the information you have.

The vortex of decision-making, also known as “indecisiveness” can be debilitating. It can eat away at your soul. It leads to stress, anxiety, possibly even depression and should be avoided at all costs. You avoid it by asking more questions, asking repeated questions, clarifying as much as possible and eventually coming to a decision, even if you are making that decision with imperfect information. A recommendation for your Decision Making Recipe is to set a timeline for making your decision (if one has not already been set for you). Living with an ongoing decision is very limiting and difficult. Once it is made you will find yourself ‘unstuck’ and able to move forward.  The decision can be changed, but at some point you will have to commit one way or another.

7. Once you have made your decision, sit with it. Let it absorb, start to live it and get as comfortable with it as you can. Shift your mind-set toward the decision you have made. Then push the ‘Send’ button and let the professionals know the decision has been made.

8. Once you’ve made your decision you will slowly need to start sharing it. Make it clear to others that you expect to be supported in your decision. Those that are not providing support need to play a more minor role in your life. They cannot contribute to the vortex of indecisiveness. They need to be ‘on-board’. Finding those people and giving the important role of supporting your decision is a last and most important step in this process.

 

Other Things to Consider:

– Others cannot and will not make the decision for you, but you can still directly ask your trusted team “What would you do?”

– Many decisions are imperfect because there is not enough information available or there is no obvious choice between the decision(s).

– Decisions can be changed/reversed to a certain point. 

– You should never feel pressured about making a decision.

– Include others in supporting you once your decision is made.

 

Julie

Note: Our family has been going through the process of a very difficult medical decision for Kate for the past 2 years. The Decision Making Recipe is based on our experience. We have made our decision  and it is imperfect and lacks all the information we need, but we are moving forward. 

I wish you the best of luck with yours. 

How Did This Happen?

There has been a story out there in the news media for close to a year now about a young girl who was seized by the state children’s protective services in Massachusetts after a medical disagreement between parents, doctors, and two different hospitals. The Justina Pelletier story.

As with any story that has been filtered, and the details are not all there – citing privacy and confidentiality issues. But the details that are available tell a chilling story about abuse of power. A child with a condition that is not well-known or understood, a disease that has no standard treatment plan or cure, and a lack of consultation with her treating physicians, can be taken from her parents, taken off all of her current medications and treatments, and kept in the psychiatric unit of a hospital with little to no visitation with her family or friends, and the parents accused of medical child abuse.

Horrifying. Terrifying.

 

My visceral reaction is disgust at the doctors and staff who did this to this young girl and her family. I am astounded that there will be no repercussions to any of them for the trauma they have caused this young girl and her family. But my instinct kicks in, and despite the questions I have about what these doctors were thinking, and how could they do this, I start to think of our own situation.

Rare. Unknown. No standard treatment. Many specialists involved. Many medical institutions involved.

I wonder if this could have happened to our family when we were searching for a confirmed diagnosis for Kate.

Could this happen to other children who are rare, without a confirmed diagnosis, medically complex, medically fragile, or perhaps to parents who are not savvy about how to ‘behave’ within the system.

I am a strong advocate for Kate. I feel that I am part of her medical care team and have an equal voice at the decision-making table. I voice my opinion. I appreciate the doctors opinions, but I also take into account my own knowledge and expertise as the expert on Kate. That is patient engagement and patient and family centred care right? Am I naive about the rights that I have to make decisions as her parent?

What makes the Justina Pelletier case hit home for me is that Justina has mitochondrial disease. A disease that few know about, where little research is done, where donation dollars and support don’t flow, where the medical community is still learning, and where there are few experts.

How does something like this happen?

How could the system change so it doesn’t happen again?

 

Julie

Random Thoughts About Jenny McCarthy

So vaccinations are in the news again, with outbreaks of measles in Ontario and in BC, and my mind wanders to thoughts about Jenny McCarthy. I hold the view that Jenny is point zero of the ‘outbreak’ of the anti-vaccination movement.  A few months ago there was an online article that was claiming she had retracted her son’s autism diagnosis. She has since come out with an official statement that discounts this article and plans to take legal action against the online media source. Irealize many have Jenny on a pedestal and believe she has raised awareness of the terrible consequences of vaccinations. I feel sad for Jenny.

 

vaccionation 2

 

Vaccinations are in the news again.

So why should we care?

To be honest Jenny influenced me – just a little bit. At the time that Jenny was standing on her soap box and proclaiming the evils of immunization, we were trying to figure out what was going on with our child and her acute illnesses, her mounting medical conditions, and her diagnosed but unattributed global developmental delay.

Kate does not have autism. In the early days of working on a diagnosis for Kate, there was some discussion about her ‘global developmental delay’ and possible autism. We kept asking the question because we were struggling to define her developmental delay and identify how to support her and what services/interventions/therapies might work best for her.

I wanted answers to help Kate and as she was still quite young, I questioned anything and everything that we put into her body – including immunizations. Kate was considered fragile and there was little known about the unanticipated impact immunizations could have on her and her undiagnosed condition; would it work, would it not, or would it complicate her medical condition. As I was exploring the question of autism, I started to read and found Jenny (McCarthy). I read her positions and I even read (scanned through) her book about how she ‘cured’ her son Evan. I am very capable of critical thinking and analysis, but I think the very exposure to all things Jenny as it related to immunizations and autism influenced my thinking.

I could blame pop culture and my occasional watching of E-Talk or Entertainment Tonight or Oprah. We can all blame Jenny in some way for the anti-vaccination movement, and that blame is coming in spades now with the discounting and a full retraction of the one ‘scientific’ article which supported her theory of immunization causing autism, and which has ignited a social revolution that is anti-vaccination. But I am not sure she is to blame. I do think she could have had more awareness that her impact as a pop culture personality would have on the broader population. I do think she had a responsibility to temper her public position with true evidence and research and support proper scientific discourse about her ideas and claims. I don’t blame her because she did what we all have done and what those of you who haven’t had to yet would do for your own children. She fought for her child. She did what she thought was best.

All she knew is that something was going horribly wrong with her son and she needed someone to listen. She needed help for Evan. She pushed for answers where there were none, and in the pursuit for answers she found a diagnosis that made sense and seemed to fit. Something that occurs more commonly than not in the rare and undiagnosed disease community. When she had that diagnosis, she did everything in her power to ‘fix’ what was wrong with Evan. She went everywhere and talked to everyone – again in the pursuit of fighting for her child. Where she went wrong was losing sight that what might work for Evan, and how she thinks she ‘cured’ him wasn’t based on evidence, at best it was anecdotal. She has a right to pursue her instinct for Evan, but she was wrong it trying to ‘sell’ it to the rest of the world, and those working with her were wrong as well. She lost her way as a mama bear and became an unfortunate advocate. Rather than working with the scientific community to prove the theory she feels so strongly about, she worked against known evidence and science and she has created a very difficult and dangerous situation for the world community.

It is hard to believe that someone like Jenny McCarthy can wield that kind of power. But when you have any element of celebrity and access to the talk show circuit with moms watching and a scary story to tell, watch out.

I really don’t blame Jenny, but I do blame people for not gathering the proper information and thinking critically for themselves. I feel public health officials and agencies should shoulder some blame for not being in front of the tidal wave of public worry about vaccinations and showing more leadership. Vaccinations are scary, you are putting a needle in your baby’s arm on a regular basis, and the public health message has not been  consistent and informative about why they are so important for well children, for chronically unwell children and most importantly, for those that can’t be vaccinated.

Parents Want Information

Your doctor tells you to get vaccinated, so you do, but that doctor/patient relationship is changing. Parents and patients are asking more questions of their doctor’s, they are thinking for themselves and misguided or not, they listen/watch social media, and celebrities with a cause, and are influenced by anecdotal opinion. This generation of parents hasn’t lived through epidemics of measles and mumps, or lost a pregnancy due to rubella. In my opinion, there is a responsibility for the public health community to better educate and increase awareness, by sharing more information about vaccinations, and what can happen if we stop vaccinating. Simply being told that they are safe by your doctor is not enough anymore. (Perhaps public health need a celebrity spokesperson of their own).

We had to suspend Kate’s vaccination schedule at one time, while her underlying undiagnosed disease was being investigated, but not because I thought they had caused autism (though I admit did worry in the back of my mind if her developmental delay had been impacted in someway by vaccinations).  Now, as it turns out, we no longer have a vaccination choice. Kate’s b-cell hypogammagloblunemia caused by her mitochondrial disease, hampers the effectiveness of vaccinations for her. She doesn’t mount the immune response a typical child does. As a result she has been re-vaccinated with vaccinations she has already received, beyond the normal vaccination ‘schedule’, in hopes that she will have some degree of protection. We’ve since learned, that the vaccination response has not been sustained in Kate, and she again has no protection.

My daughter is at high risk for contracting measles, mumps, rubella, diphtheria, pertussis, tetanus, meningococcus and pneumococcus should she be exposed.

The bottom line for me in my decision-making process is that if Kate acquires any one of these diseases she can become very sick. It was better to make her safe based on the scientific evidence than to risk her contracting such diseases which could seriously complicate her mitochondrial disease. But I no longer have a choice to vaccinate her, and because others are not vaccinating their children – what I call ‘the Jenny McCarthy effect’ – Kate is at higher risk as the rate for diseases controlled through vaccination are on the rise.

The ‘herd effect’ is less effective.

Yes, we have been protected thus far, because we have worked together as a society to vaccinate and reduce the prevalence of these diseases, but that is now showing cracks and being compromised. I think this is a point that many parents don’t realize, or don’t consider. We did not live through the time when these vaccinations were not available and these diseases killed children, or caused them significant harm, and we are now travelling the world more than ever before and visiting areas that have endemic disease. By not vaccinating your child because of unsupported claims, you are putting others at risk.  On the other hand, you probably have vaccinated families to thank for you child not having yet contracted one of these terrible diseases by minimizing your unvaccinated child’s exposure.

Not everyone has the choice to be vaccinated, and the risk of these diseases to children who are already medically fragile and who don’t have a vaccination choice can be devastating.

I understand that I am asking you to vaccinate your child to protect mine. But I am asking you to make a reasonable choice – a proven and effective choice – to protect your child also. I am not asking you to put your child at risk. Vaccinations do not cause autism. I challenge anyone to find a scientific article that can state that they do as scientific fact and published in a reputable medical journal. Vaccinations rarely cause adverse events, in Ontario last year there were 7.2 serious events for every 1 million vaccines. Measles kills 1 in 1000.

 

My last random thought about Jenny McCarthy is I feel sad for her. There is a lot of blame pointed in her direction, and she has been sorely misguided. I understand where she started in this journey, but I don’t support where she took it.

 

Julie

Moment to Moment

Disclaimer: Hoping this post reads well. The neurons are not firing on all cylinders these days.

Something happens when Kate has an acute episode of illness related to her mitochondrial disease. Life seems to slow down immensely and takes on a very focussed purpose. The analogy of ‘putting on foot in front of the other’ is the best I can use to describe the sensation. Managing her through each moment of her vomiting – cleaning her off, changing her bed, taking sheets, towels, pyjamas to the laundry room, administering meds (and deciding which ones to give), trying to settle her and repeating it all again throughout the night and into the next day. Each of those steps is a moment. Eventually deciding we need to go to CHEO for care and treatment, and absorbing that decision – that we make so often – and the implications and the stress that it brings with it – it becomes another moment. I pack her bag like I have done so often before. I cancel the week of appointments for the entire family, I call school, I cancel therapy, I cancel personal plans like the long awaited visit to the hairdresser or dentist.  I call into work and tell them I won’t be in.

Everything that is not about this moment of Kate being sick is no longer relevant. And then I dive into the world of acute illness and supporting Kate. I turn inward and rally my strength, contain my tears, and help her get through yet another round of bloodwork, procedures, and IV pokes. I sit with her and stroke her face or hold her and rock her just being in the moment. Our medical team comes and goes, our social worker, a therapist, another CHEO friend may stop by – they help to give a brief reprieve from the moment – and then the focus is back to Kate.

Those first few days when she is acutely unwell she sleeps most of the day. We are usually in a room with no windows. I turn the lights off to let her rest. It is like a cocoon and any sense of what I should be doing that day falls away as I watch her sleep. Over a few days she starts to feel better and then managing a little girl who wants out of the hospital, but still needs to be there, becomes my moment. Minute to minute, builds into hours of entertaining her and keeping her in her bed – maybe breaking things up with a walk and some visiting to the snack shop or cafeteria. The routine is usually the same. Then we get home, and life still does not return to normal because Kate is still recovering and not feeling like herself. Crying, whining, a need for constant attention become the new routine. My six year old who is more like a 3 year old. Carrying her because she wants to be held. Constantly waking at night for comfort or because she cannot sleep due to some sort of unidentified discomfort that she cannot tell me about. But the act of living in the moment cannot be sustained forever, and though she is still unwell and needing care and attention, life slowly starts to creep back in and the pace steps up. All those things that were set aside now return immediate attention. Not little things like the hairdresser and a coffee date with a friend that were cancelled, big things like the urgent priority at work, medical appointments for Kate, banking and taxes, booking summer camps, commitments made that were delayed, groceries. It doesn’t matter that I am exhausted, that I haven’t slept, that my child is still not well. Life has to start back up and demands my attention.

 

Out of Order

Coming back from the month of February has been an act of floating and sinking at the same time for me. Kate was steadily unwell from early December and undergoing regular tests investigating what was going on with her. February resulted in two typical childhood illnesses, a mild stomach flu and a common cold, that landed Kate in CHEO and caused her to be very unwell. The month was spent in and out of hospital, and sleepless due to constant care day and night. I started floating, living in the moment of caring for her – which is the only way it can be done. At the same time, I was sinking as responsibilities piled up that I did not have either the time or energy to address. Once Kate was home and feeling better, I thought I should set aside the time in the evening to catch up, but found I simply couldn’t focus because I was so tired. My brain is simply tired and thinking through the tasks of my ‘other job’ having just arrived home from work feels impossible.  The effect of fatigue is profound. I sink and the pile builds. Kate has pulled me into a pattern of not sleeping, so sugar and caffeine have become my companions struggling to find the energy I need wherever I can find it. I get done in my day what I must, and sometimes I get done what I need to do to get by. I start considering what responsibilities I can set aside.

What I know I need, I cannot figure out how to get. Respite, time from Kate to catch up on our life, to feel settled again, to get into a routine again so that I can catch up on my rest. The resources for respite are slim, and often already booked. Rogers House has been an amazing resource for us, but I don’t leave Kate there when she is unwell. I don’t feel it is fair to her when she needs us.  In fact, I did book Kate at Rogers House this weekend and was so looking forward to it, but we were bumped for an emergency admission. My heart sank.

There is a great motivator for me to get back to a better state, and that is Kate. This disease is endless and it is relentless, and above all it is predictable to the point that we know she will be sick again and soon, we just don’t know when. Our family has lived with SIFD for six and half years. It hasn’t changed, it hasn’t been cured, it is not something Kate will grow out of (my least favorite question). It is with us, it is part of our life and implicates itself into any and all of our plans. I have to find a way to deal with the sinking and the floating. I don’t think the answer can be living in the moment. I think it needs to be planned and systematic and disciplined – but how do that amidst the exhaustion and the sinking/floating feeling. Sometimes you are just to tired to know what you need.

I can see how long term caregiving wears on families and on individuals. I worry about that for me. The comment ‘I don’t know how they do it’ comes to mind, but I already know the answer to it, ‘Because you have to’.  It has been six years, what will the next six look like for me? I know I need a better plan.

How will I deal with constant trauma and stress that comes with parenting and loving a child with an ultra-rare disease which will likely take her from me?

Is it really just a matter of moving from moment to moment.

 

Julie